Cardiac Surgery Operations On The Heart and Great Vessels in Adults and Children PDF
Cardiac Surgery Operations On The Heart and Great Vessels in Adults and Children PDF
Cardiac Surgery Operations On The Heart and Great Vessels in Adults and Children PDF
Axel Haverich
Editors
Cardiac Surgery
Operations on the
Heart and Great
Vessels in Adults
and Children
123
Cardiac Surgery
Gerhard Ziemer
Axel Haverich
Editors
Cardiac Surgery
Operations on the Heart and Great Vessels
in Adults and Children
Editors
Gerhard Ziemer Axel Haverich
Department of Surgery HTTG - Surgery
The University of Chicago Medizinische Hochschule Hannover
Chicago, IL Hannover
USA Germany
Preface
The majority of the various comprehensive the third and latest edition of this German
textbooks available cover either cardiac textbook in 2010 [3].
surgery in adults, mostly synonymous for In the spirit of our German teacher
acquired heart disease, or pediatric cardiac Hans Borst’s honored guest’s address at the
surgery, synonymous for congenital heart 65th Annual Meeting of the American
defects. This separation of the spectrum of Association for Thoracic Surgery in New
cardiac surgery is not appropriate anymore, Orleans 1985: Hands across the ocean,
especially as the majority of children oper- German-American relations in thoracic
ated for congenital heart disease reach surgery [4], we expanded the project of our
adulthood, potentially needing further sur- German textbook and planned for an
gical attention. On the other hand, although updated English language edition.
a rare phenomenon in the Western world, Among the authors and co-authors
cardiac surgeons have to deal with acquired contributing to the latest German edition
heart disease in children as well. (mainly from Germany, Austria, and Swit-
Surgery and catheter interventions, zerland), many had received at least part of
competing therapeutic strategies in the their specialty and subspecialty training in
beginning, have become complementary: clinical cardiac surgery or research in
interventional valve implantation may be English-speaking countries, most of them
performed by cardiac surgeons, and aortic in North America, but also in the United
stent implantation is well established in Kingdom, Australia, and South Africa.
the thoracic domain. This development This our first English language edition
also blurred the boundaries of involved entitled Cardiac Surgery: Operations on the
specialties, necessitating close coopera- Heart and Great Vessels in Adults and Chil-
tion not only between cardiologists and dren comprises the view of 71 authors not
cardiac surgeons, but also involving vas- only from 30 German, Austrian, Swiss,
cular surgeons, radiologists, and others. French, and Luxembourgian Cardiac Units,
Furthermore, hybrid procedures per- but also from Boston, MA; Chicago, IL;
formed in parallel or in sequence with Greenville, NC; Houston, TX; Los Angeles,
open surgery and catheter intervention in CA; Miami, FL; Milwaukee, WI; St. Peters-
the same setting by different groups of burg, FL; Toronto, ON; and Washington, DC.
specialists (e.g., surgeons and catheter Starting rather traditionally, with a
interventionalists) have changed the tra- chapter on the history of cardiac surgery,
ditional field of surgery. we continue with risk scores and principles
Only a few textbooks cover such a of quality assurance, followed by quality
broad spectrum of cardiac surgery. Our control in cardiac surgery in the United
predecessors as editors, Hans Georg Borst, States, and an overview of databases in car-
Werner Klinner, and Åke Senning, started diac surgery.
this endeavor, when they published the This introduction is completed with a
first and only German textbook on cardiac section of chapters on technical prerequi-
surgery, “Herz + herznahe Gefäße” 1978 sites for cardiac surgery in which we focus
[1], the year, in which both of us (G.Z. and separately on postoperative critical care for
A.H.) just finished medical school. adults and children. There also is a chapter
For the second edition, published in on tissue engineering in cardiac surgery.
1991, now named “Herzchirurgie” [2], The major part of this book is divided
edited by Hans Borst, Werner Klinner, and into sections on congenital anomalies and
Hellmut Oelert, we could contribute as those for acquired diseases, also addressing
junior faculty in our primary fields of minimally invasive cardiac surgery as well as
interest. This led us to become editors of endovascular treatment of aortic diseases.
Preface
vi
Gerhard Ziemer
Chicago, IL, USA
Axel Haverich
Hannover, Germany
vii
Contents
I Introduction
1 The History of Cardiac Surgery............................................................................................. 3
Knut H. Leitz and Gerhard Ziemer
II Technical Prerequisites
6 Extracorporeal Circulation and Myocardial Protection
in Adult Cardiac Surgery........................................................................................................... 111
Christof Schmid
Service Part
Index.................................................................................................................................................... 1139
xi
Contributors
Introduction
Contents
References – 24
Chapter 1 · The History of Cardiac Surgery
5 1
1.1 From the Beginning heart can be sure to be definitively discredited by
his colleagues.» This quotation is found in
September 9, 1896, is generally agreed upon as the K.H. Bauer’s Aphorismen und Zitate in der
date of birth for clinical cardiac surgery. This was Chirurgie [Aphorisms and Citations in Surgery]
the day when in Frankfurt/Main, Germany, (Bauer 1972). Karl-Ludwig Schober, in his work
Ludwig Rehn (1849–1930) decided to operate on on early history of surgery of the thorax and its
a 22-year-old gardener who suffered from a stab organs, has his doubts about this being a proper
wound to his chest. He had been treated for 1 day citation of what Billroth actually said. He rather
conservatively with icepacks and camphor injec- thinks that this notion is a misquotation, originat-
tions. When the patient’s clinical condition rap- ing from hearsay or from primary mistakes in
idly deteriorated, Rehn performed a left citation (Schober 1981, 1993).
thoracotomy and opened the pericardium. He Among crucial points for the early years of
found a 1.5 cm stab wound in the right ventricle, cardiac surgery, Rehn’s experience (Rehn 1913) is
which he closed with three single stitches. The repeatedly quoted by Herrmann Küttner, who
postoperative course was complicated by fever wrote the chapter «Cardiac Surgery» for the first 6
and frank pus draining from the thoracotomy editions of the Textbook of Surgery edited by Bier,
wound; however, the patient eventually recovered Braun, and Kümmel, first edition 1912 (Schober
and was discharged from hospital. When Rehn 1993):
presented this case at the 26th Meeting of the
German Society for Surgery in 1897, he con-
»» It is important not to take shallow bites on
both wound edges, as the sutures need to be
cluded: «From now on, there should not be any tied without cutting through the fragile tis-
more doubt about the feasibility of a heart suture.» sue of the heart muscle.
He hoped his successful case would encourage
others to continue the work in the field of heart Referring to the issue of continuous versus
surgery (Rehn 1897). interrupted single sutures, Rehn clearly pleaded
Rehn’s pioneering effort was not an ingenious, for interrupted single sutures.
spontaneous flash of inspiration. In 1868 Georg
Fisher from Hannover, Germany, had published an
»» If the heart shows signs of fatigue and starts
working in an irregular fashion while the
analysis of medical discharge summaries of 452
suturing is performed, give it a rest to recover.
patients with heart wounds (Fisher 1868). He found
Therefore one should put the heart back into
the recovery rate to be 10 %. This meant not all
its original, normal position and refrain from
patients died immediately from wound to the heart,
any manipulation for some time.
which at least for some should allow for surgical
intervention. Reports on successful experimental Most of this advice is still up to date!
and unsuccessful clinical cases of stab wounds to Ludwig Rehn was a self-made man who had
the heart had been presented by Block from Gdansk not been trained at any of the leading centers for
(then Germany) and also by Norwegian and Italian surgery at that time. However, he was a
surgeons (Bircks 2002; Block 1882). thoroughbred surgeon who caught the momen-
The first surgery for a stab wound to the heart tum and acted accordingly. He became not only
in the United States was performed in Montgomery, the first to successfully perform cardiac surgery,
Alabama, by Luther Hill on September 14, 1902, he also was the first cardiac surgeon, who devel-
on a 13-year-old boy, who had suffered numerous oped and controlled his surgical techniques in
stab wounds to his chest. Surgery was performed animal experiments. This made him the founder
under chloroform anesthesia. One single stitch of academic cardiac surgery (Mueller 2007;
had to be done on the myocardium, and the Schmieden 1931).
patient survived surgery without complications Three other surgeons became the leaders of
(Westaby 1997). In 1903, Ricketts from Cincinnati, experimental cardiac surgery before World War I
Ohio, reported on 56 cases of cardiac sutures, with and provided the base for its further clinical appli-
success in 20 (Ricketts 1903). cation (Schober 1993; Vaubel 1980; Westaby 1997):
There has been controversy about a notion 55 Alexis Carrel (1873–1944)
attributed to Theodor Billroth (1829–1894), «The 55 Ernst Jeger (1884–1915)
surgeon who will try to suture a wound of the 55 Rudolf Haecker (1878–1957)
6 K.H. Leitz and G. Ziemer
Alexis Carrel was born in Lyons, France. After «The Current State of Blood Vessel Surgery» (Jeger
1 graduating in medicine from the University of 1914b), Jeger died in 1915 of typhus as a prisoner of
Lyons, he left France for Montreal, Canada, but war in Russia (Schober 1993).
joined shortly afterward the Department of Ernst Jeger’s main interest was experimental
Physiology at the University of Chicago, where he cardiovascular surgery. In 1913 he reported on
mainly worked with Charles Guthrie (1880– reimplantation of the renal vein into the inferior
1963). They perfected the technique of vascular vena cava (Jeger and Israel 1913). In Breslau he suc-
anastomosis. The triangle method for vascular ceeded in bridging resected aortal segments by
anastomosis developed by Carrel in 1902 is still a end-to-side anastomoses of vein grafts (Jeger 1913).
standard today. They also reimplanted limbs and For treatment of portal hypertension, he proposed
performed autotransplantations of kidneys, ova- the mesenteric-caval shunt (Jeger 1914a). While in
ries, and thyroid glands. war, he took care of vascular injuries and was suc-
In 1906 they separated; Charles Guthrie went cessful in six out of eight cases (Jeger 1914b).
to Washington University in St. Louis, Missouri, In 1913, as part of a lecture at the 42nd Congress
and Alexis Carrel joined the Department of of the German Society of Surgery, he anticipated
Experimental Surgery at the Rockefeller Institute the later Blalock-Taussig shunt as a way to arterial-
in New York City. There he replaced segments of ize the pulmonary circulation, without having any
the descending thoracic aorta with caval vein idea about the clinical significance at that time
grafts, being already aware of the risk of paraple- (Jeger 1914a). Ernst Jeger’s early death was not only
gia in this type of surgery. He did experiments on a personal tragedy but also a step back for the
techniques of mitral commissurotomy and car- development of the European cardiac surgery.
diac aneurysmectomy, and he also did research Rudolf Haecker is the third surgeon who has to
on coronary artery bypass surgery. be mentioned here. As an assistant to Paul
Another one of his areas of interest was heart Leopold Friedrich in the Department of Surgery
transplantations. He transplanted hearts from lit- at the University in Greifswald, Germany, he pub-
tle animals to the neck vessels of bigger animals lished on experimental cardiac pathology and
(Carrel and Guthrie 1905). In 1912 Carrel was surgery (Haecker 1907). His experiments on caval
named the Nobel Laureate for Physiology and inflow occlusion in normothermia showed that
Medicine. After the World War I, he continued his dogs survived this for no more than 3 min with-
research on organ preservation and worked with out cerebral damage. Therefore, normothermic
Charles Lindbergh on a mechanical pump which caval inflow occlusion was defined as a method to
should support the circulation when the heart be used only for a very short period of time when
needed to be stopped for surgical procedures treating wounds of the heart. This was confirmed
(Edwards and Edwards 1974; Westaby 1997). by Ferdinand Sauerbruch and Ludwig Rehn
Ernst Jeger (1884–1915) was born in Vienna, (Rehn 1913; Sauerbruch 1907).
Austria. There he received his training in surgery The next steps in the development of cardiac
from Professor Eiselsberg and Professor surgery included success in closed-heart surgery;
Zuckerkandl. A short time after that, he went to the the heart was operated from the outer surface,
Physiological Institute in London, England, and «trying not to disturb and certainly not interrupt
then to Berlin, Germany, to Professor Bickel in the the heart’s pump function».
Department of Experimental Biology at the Charité.
In between, he spent 6 months studying with Carrel
in New York City. In June 1913 he started working 1.2 Operations on the Pericardium
as a volunteer in the Department of Surgery at the
University of Breslau with Hermann Küttner. Being At the beginning of the twentieth century, it was
a foreigner from Austria, taking this unpaid posi- not possible to treat inflammatory pericardial dis-
tion was his only chance to work there without hav- ease in the state of purulent, especially tubercu-
ing to pass a new medical examination for the lous pericarditis; the consequence often was a
German Reich. When World War I began, he had to severely contracting pericarditis leading to the
join the Austrian army and had to defend the end stage of constrictive pericarditis. In 1902
Przemysl fortress against the Russians (Schober Ludolf Brauer inaugurated what he called cardi-
1993). Just before he could publish his overview olysis: resection of the ventral parts of the calcified
Chapter 1 · The History of Cardiac Surgery
7 1
pericardium together with segments of the tho- East Prussia/Germany (Kirschner 1924). Thereafter,
racic wall (Brauer 1903). The heart was indirectly successful Trendelenburg operations were reported
unwrapped. The first successful pericardectomies by Arthur W. Meyer (1885–1933) from Berlin,
were performed in 1912, again by Ludwig Rehn in Germany (Meyer 1928, 1931) and by Clarence
Frankfurt/Main, and in 1913, by Ferdinand Crafoord and G. Nystrom from Sweden (Crafoord
Sauerbruch in Zurich, Switzerland. Later, the 1929; Nystrom 1930).
technique of pericardectomy was mastered by
Viktor Schmieden (1874–1945) in Halle/Saale
and later in Frankfurt/Main, where he had become 1.4 Resection of a Right
Rehn’s successor in 1919. There he later continued Ventricular Aneurysm
to work with the internist Franz Volhard (1872– by Sauerbruch (1931)
1950), who came to Frankfurt in 1927 after having
been professor in Halle/Saale (Bircks 2002; With the diagnosis of a mediastinal tumor, the big
Schober 1993). The first pericardectomy in the mass was punctured intraoperatively. When
United States was performed in 1928 by Edward bleeding occurred, the tissue had to be clamped;
D. Churchill in Boston on an 18-year-old girl however, it continued tearing and bleeding even
(Churchill 1929). In Cleveland, Ohio, Claude more. It eventually could be controlled by com-
Beck worked experimentally on the clinical symp- pression with a finger. Sutures were laid over the
toms and the surgical therapy of constrictive peri- compressing finger in situ and tied. Histopathology
carditis (Westaby 1997). revealed an aneurysm of the wall of the right ven-
tricle (Sauerbruch 1931).
..Fig. 1.3 After September 1942, ductus ligation became a rarity, almost exclusively replaced by ductus division
(Dr. Goss’s surgery case logbook #1, «Patent Ductus» list, pp.174/5, Sept. 1944 – Oct. 1945) (Courtesy Dr. Robert and
Carol Replogle)
1.6 Blocked Developments the pressure ventilation, the lung is expanded and
and Missed Opportunities breathes very calmly and regularly. When the
pressure pipe is disconnected from the endotra-
1.6.1 Endotracheal Intubation cheal tube, the lung collapses.» Kuhn saw the
advantages of this method for an undisturbed gas
Friedrich Trendelenburg (1844–1924), at that exchange and superior kinetics of the inhaled nar-
time still at the University of Rostock, Germany, cotic agents, which lead to a better control of nar-
used tracheal intubation in a patient for the first cosis. In describing his experiments, he obviously
time in 1869. The rationale was to avoid aspira- saw also the advantage of avoiding pneumothorax
tion of blood and secretions during oral surgery during his open chest experiments; however, he
(Trendelenburg 1871). At the end of the nine- was not a thoracic surgeon (Schober 1993).
teenth century, other surgeons reported success- At almost the same time, Franz Kuhn was per-
ful narcosis employing tracheal intubation, like forming his endotracheal intubation experiments,
Karel Maydl (1853–1903) in Prague, Bohemia in 1904 Ferdinand Sauerbruch (1875–1951), assis-
(Maydl 1892); Viktor Eisenmenger (1864–1932) tant to Johannes (born: Jan) von Mikulicz-Radecki
in Vienna, Austria (Eisenmenger 1893); and (1850–1905) in Breslau, Germany, performed
Theodore Tuffier in Paris, France (Schober 1993). open chest surgery on a lung in a low-pressure
A cuffed rubber tube, which also had a pilot bal- chamber developed and constructed by himself
loon, was already used by Eisenmenger in 1893 (Sauerbruch 1904b, c). To avoid pneumothorax,
(Goerig and Schulte am Eich 2003). he employed a low-pressure technique he devel-
Franz Kuhn (1866–1929), working as a gen- oped, in which the patient’s head was exposed to
eral surgeon in Kassel, Germany, was a proponent normal atmospheric pressure outside the box,
of tracheal intubation (Kuhn 1901). He employed while the other parts of the body were in subat-
flexible metal pipes. In March of 1905, he mospheric pressure within the low-pressure
described his experiments on dogs as follows: «In chamber. When opening the chest in the low-
deep narcosis the right thorax is entered. Due to pressure chamber (in which, in addition to the
10 K.H. Leitz and G. Ziemer
patient, the whole surgical team had to fit), the surgeon in Germany. In his comments he did not
1 lung did not collapse, and the patient could favor one technique over the other (Goerig and
breathe spontaneously. Sauerbruch, who got Schulte am Eich 2003; Schober 1993).
worldwide attention for this discovery, rejected High-pressure and low-pressure techniques,
the endotracheal positive pressure ventilation as as well as the insufflation techniques, illustrate the
suggested by Kuhn, which he viewed to be struggle of the different schools for the best
unphysiological (Sauerbruch 1904a, b, and c; method to avoid pneumothorax and at the same
Schober 1993). In his mind, positive pressure ven- time provide the best gas exchange during tho-
tilation represented a permanent Valsalva maneu- racic surgery. The superior solution that was ulti-
ver, potentially leading to circulatory disturbance. mately accepted, however, had already been
On another occasion he wrote, «The low pressure published in 1886 by the French surgeon Théodore
procedure creates the pressure difference (neces- Tuffier (1857–1929). Employing a snugly fit endo-
sary to keep the lung expanded) by thinning the tracheal tube, he intermittently delivered high-
air on the lung surface, while the positive pressure pressure air. This optimal French solution was
procedure increases the air pressure in the lung» only reluctantly accepted in Germany (Goerig
(Schober 1993). After a visit in New York City in and Schulte am Eich 2003; Schober 1993).
1908, bringing over his low-pressure chamber At this point we like to briefly focus on
from Germany for demonstration, Sauerbruch left Ferdinand Sauerbruch and his professional career.
it with Willy Meyer (1858–1922) at Lennox Hill After medical school education at the universities of
Hospital. He may not have liked when Willy Marburg, Greifswald, Jena, and Leipzig, he took his
Meyer and his brother, who was an engineer, later first resident position in Breslau 1903. Six days after
tried to reverse the low pressure into a high- he finished his PhD thesis on experimental thoracic
pressure chamber («universal chamber») with esophageal surgery, he had to attend the funeral of
having the patient’s head inside and the surgical his teacher, von Mikulicz in 1905. He went to work
team operating on the patient’s chest outside with Paul Leopold Friedrich (1864–1905) in
under normal pressure. Greifswald. Friedrich was already a respected tho-
The air insufflation method, developed by John racic surgeon at that time (Cherian et al. 2001;
Auer (1875–1948) and his father-in-law James Dewey et al. 2006; Schober 1993). When he took a
Meltzer (1851–1920) in New York City, also was university chair in Marburg in 1907, Sauerbruch
rejected by Sauerbruch (Meltzer 1910; went with him and was appointed Professor in
Schober1993). Oxygenation was achieved by a con- Marburg. In 1910 his reputation in lung surgery
tinuous flow of air via a small pipe introduced into made him chairman and Professor of Surgery at the
the patient’s upper airway. In animal experiments University of Zurich, Switzerland, the country of
the depth of insertion of that pipe into the trachea tuberculosis hospitals and spas. He published the
correlated with the length of survival. In 1910, the standard text book Techniques of Thoracic Surgery
neurosurgeon Charles Elsberg (1872–1948) in 1911 (Sauerbruch and Schumacher 1911). In
employed the Meltzer/Auer method successfully in 1918 Ferdinand Sauerbruch went to become
a patient at Mount Sinai Hospital in New York City Professor in Munich, Germany, just in time to
(Schober 1993). Not surprisingly, Sauerbruch also receive the title of Geheimer Hofrat (privy council-
rejected the concept of high-pressure respiration, lor) from the last Bavarian King (Cherian et al.
as suggested by the internist Ludolf Brauer (1885– 2001; Dewey et al. 2006). There he founded what
1951) in Marburg, Germany (Brauer 1904). In was called the Sauerbruch school, which later
high-pressure respiration, the patient had to exhale peaked at the Charité in Berlin (1928–1949).
against a high airway pressure delivered by an air- Nobody could overrule his judgement. In
tight face mask. With the overwhelming authority Sauerbruch’s defense, Rudolf Nissen stated in his
of Sauerbruch in opposition, endotracheal ventila- book Real-life in Thoracic Surgery (Erlebtes aus der
tion and endotracheal anesthesia had no chance to Thoraxchirurgie):
develop in Germany at that time. Interestingly,
both Ferdinand Sauerbruch’s and Ludolf Brauer’s »» Sauerbruch’s rejection of the endotracheal
papers were published in the same journal, both intubation today seems unbelievable to us.
receiving editorial comments by von Mikulicz- However, it was only in a small portion
Radecki, the authoritative contemporary thoracic related to stubbornness. The main reason
Chapter 1 · The History of Cardiac Surgery
11 1
was a lack of an organized management for an inactivity in progress in German medicine
anaethesia. Sauerbruch opposed the estab- occurred. This cannot be explained with the war
lishment of anaesthesia as a specialized and its consequences alone (Schober 1993;
discipline. He saw it as a step forward to the Wachsmuth 1985b). German medicine and surgery
decomposition of the discipline of surgery fell below the level of the leading nations. The North
into different subspecialties. (Nissen 1955) American, British, and Scandinavian proponents of
thoracic surgery took over the field (Schober 1993).
Looking through the congress communications
1.6.2 Cardiac Catheterization of the German Society of Surgery after 1920, the suc-
cessful Trendelenburg operation in 1924 performed
Similarly, cardiac catheterization was not recognized by Martin Kirschner and the aneurysmectomy done
as diagnostic tool. There was a lack of by Ferdinand Sauerbruch in 1931 were the only
clinical questions and therapeutic options. Werner highlights. Several reasons are named and discussed;
Forssmann (1904–1979) was a young resident in the others may be added; many of them may be interde-
hospital of Eberswalde, a small town near Berlin. pendent (Sauerbruch 1924; Schober 1993):
Motivated by a picture he saw in a textbook of phys- 55 Fear of splitting the field of surgery into inde-
iology, in 1929 he pushed a thin, well-lubricated pendent subspecialties: The best will only
urine catheter through a cubital vein directly into his become master in a subspecialty (Nissen 1955).
heart (Lichtlen 2002). He went to radiology to docu- 55 Lack of diagnostic options (only very few
ment this experiment on himself by x-ray. His idea internists were willing to expose their
was to later inject drugs directly into the heart and patients to risky therapies).
immediately study the response (Forssman 1929). In 55 Exclusion of the German Society of Surgery
1931 he published an article about contrast images from the Societé Internationale de Chirurgie
of heart cavities with the same experimental setup after World War I.
(Forssmann 1931). The importance of Forssmann’s 55 Lack of cooperation within Germany, espe-
experiments was recognized by his chief, Dr. cially as compared to the situation in the
Schneider. He advised Forssmann to go to the most United States.
authoritative surgeon in Germany, who happened to 55 Both World wars with tremendous human
work in the close-by Charité. Sauerbruch had not sacrifice and waste of material resources and
the slightest recognition of the potential of wealth.
Forssmann’s work, and he dismissed the young col- 55 The hierarchical structure of the German
league with the words, «With this you cannot do society, in which only a few opinion leaders
anything at all in surgery.. . . . for such tricks you may had the say (Forssmann 1972).
receive your PhD in a circus but not at a respectable 55 Decline of the German language as the scientific
German medical department» (Forssmann 1972). language. Most scientific publications were now
In 1956 Forssman and the Americans André in English, a language only very few of the
F. Cournand (1896–1988) and Dickinson Richards German opinion leaders spoke (Leitz 2005).
(1895–1973), both working at the Bellevue Hospital 55 The therefore difficult information transfer as
in New York City, became Nobel Laureates in compared to today (e.g., when the German
Medicine for their pioneering work in cardiac cath- surgeon Werner Wachsmuth was prisoner of
eterization (Bircks 2002; Lichten 2002). war in England after 1945, he was asked
whether the Germans used penicillin: He had
never heard about it before, even though
1.6.3 ecline of German Surgery
D Alexander Fleming had already discovered
Pre-World War II penicillin in 1928, and the Allied Troops used
it since 1940 routinely (Wachsmuth 1985a).
It became evident that after the stunning develop- 55 The exodus and murder of a vast number of
ments up to the beginning of World War I in scientists with Jewish background due to the
1914—a period during which the names of Ernst Nazi racial discrimination politics, which had
von Bergmann, Curt Schimmelbusch, Robert been sharpened with the law launched April
Koch, August Bier, Rudolf Virchow, Konrad 7, 1933, to restore the civil servants career
Röntgen, and Karl Landsteiner can be mentioned— with the Arian paragraph (Nissen 1969).
12 K.H. Leitz and G. Ziemer
1.7 Heart Valve Surgery as the access to the heart and the left auricle as the
1 in the Time Before entry for the digital disruption of the mitral valve.
Extracorporeal Circulation After World War II, it was Russell Brock (1903–
1980) in London who tried to treat aortic valve ste-
The first operation on a heart valve is attributed noses. Coming either from the brachiocephalic
to Theodore Tuffier (1857–1929). On July 13, trunk, the subclavian artery, or through the left
1912, he operated a patient with aortic valve ste- ventricular apex, he inserted valvulotomes through
nosis. After consulting Carrel, who was present stenotic aortic valves to relieve stenotic aortic
at the operation in Paris, France, he did not valves. The results, however, were bad (Brock
incise the aortic wall but tore the aortic valve as 1950). Horace Smithy (1914–1948) in Charleston,
he invaginated the aortic wall with the index fin- South Carolina, had similar experiences. He tried
ger, tearing the valve open in this way (Tuffier to perforate the mitral valve with a punch, which
1914). The idea of bursting stenotic valves domi- rarely went smoothly. However, five out of his
nated the surgical circles of that time. seven patients with mitral stenosis survived.
Valvulotomes and cardioscopes were con- Smithy himself died of rheumatic aortic valve ste-
structed, e.g., by E. Cutler, S. Levine, and C. Beck nosis. His paper on his surgical achievements was
but also by others (Westaby 1997). But cardio- published shortly thereafter (Smithy et al. 1950).
scopes were never successful due to the bad Just before he died, he had visited Alfred Blalock in
viewing conditions. Baltimore, who offered to operate on him after he
Encouraged by his experimental experience would have assisted Dr. Smithy operating on a few
with animals, in 1923 Elliot Cutler (1888–1947) patients there. The first patient fibrillated after tho-
operated a 12-year-old girl with hemoptysis at racotomy and died, and Blalock refrained from
Peter Bent Brigham Hospital in Boston, further operations like this (Crawford 2010).
Massachusetts. Via a modified sternotomy, he At Hahnemann Hospital, Philadelphia,
exposed the heart without opening the pleurae Pennsylvania, Charles Bailey (1910–1993) took
and drove a valvulotome from the apex of the left his chance. After numerous animal experiments,
ventricle through the mitral valve and burst it he operated on five patients with mitral stenosis,
open. The girl survived for four and a half years, but only one patient survived (Bailey 1949). For
the hemoptysis diminished (Cutler and Levine disrupting the mitral valve, Bailey utilized little
1923). Cutler abandoned this type of surgery after knives mounted to his index finger. Due to his
further clinical treatments failed. failure, he was referred to as the «butcher of
Sir Henry Souttar (1875–1964), a surgeon Hahnemann Hospital» and was prohibited by the
from London, England, operated in 1925 on a administration of his hospital from operating on
19-year-old woman with rheumatic mitral steno- any other patients for mitral stenosis (Stephenson
sis through a left thoracotomy. Employing posi- 1997). Therefore, he in part started operating in
tive pressure ventilation and ether anesthesia, he other hospitals. At the meeting of the American
dissected the heart and inserted his index finger College of Chest Physicians in Chicago, 1948, he
into the left atrium. In this way he divided the presented his only successful mitral commissur-
leaflets of the mitral valve; however, this caused otomy case, a young woman (Stephenson 1997).
regurgitation (Souttar 1925). The women was Shortly after Bailey’s success with his first case,
hospitalized again in 1930 but died of multiple Dwight Harken (1910–1993) in Boston,
cerebral embolisms and heart failure. Massachusetts, was also successful with his first
Souttar performed mitral valvulotomy only mitral commissurotomy. When his next six con-
once, and when asked why, he explained his deci- secutive patients died, Harken decided to quit
sion in a letter to Harken: «. . . The physicians cardiac surgery; however, colleagues persuaded
declared that it was all nonsense and in fact that him to continue. In England, Russell Brock
the operation was unjustifiable. In fact it is of no reported in 1950 on six successful closed mitral
use to be ahead of time. . . .» (Harken and Curtis commissurotomies (Baker et al. 1950).
1967; Westaby 1997). 1948 was the year of the closed mitral com-
In summary, it may be concluded that the first missurotomies (Westaby 1997) (see . Table 1.1).
attempts to divide heart valves were not very suc- It was Brock, DuBost (1914–1991), and Tubbs
cessful. But these attempts established sternotomy (1908–1993) who earned the credit to have
Chapter 1 · The History of Cardiac Surgery
13 1
allowing interruption of the circulation for some 1955a; Lillehei et al. 1955b). Usually an adult served
1 time to have a direct look into the heart and eventu- as the «circulation donor» who continuously sup-
ally perform a procedure. Animal experiments ported with part of his arterial blood the perfusion
showed that oxygen consumption could be only of the arterial system of a usually much smaller
reduced linearly with body temperature, when patient. The blood flow was controlled by a pump.
muscle shivering was suppressed (Bigelow et al. The venous blood of the patient was pumped back
1950a). Bigelow also learned that an adult labora- to the donor’s venous system. The donor only
tory animal can be cooled down without risk of required his groin vessels to be cannulated. With
adverse sequelae to 20 °C body temperature. At adequate cannulation of the patient, the heart was
20 °C body temperature, oxygen consumption was taken out of the circulation, and the cardiac cavities
only 20 % as compared to 37 °C; 20 min would be could be opened for direct vision heart defect
allowed to perform controlled heart surgery under repair (Lillehei et al. 1955a; Lillehei et al. 1955b)
direct vision (Bigelow et al. 1950b, 1969). (see also 7 Chapter «Surgery for Congenital Heart
In Bigelow’s experiments the anesthetized and Defects: A Historical Perspective», Sect. 11.2).
ventilated animals were placed on cooling blankets With this approach, Lillehei and his team set
and cooled down to 20 °C. After thoracotomy, caval the following milestones in surgical repair of con-
inflow occlusion was initiated, and the right atrium genital heart disease (Lillehei et al. 1955a; Lillehei
was opened. It was closed again after 15 min. et al. 1955b):
Survival was 50 % in these laboratory animals 55 Ventricular septal defect (1953)
(Bigelow 1950a). Bigelow presented his experimen- 55 Complete atrioventricular canal (1954)
tal results in 1950 at the annual meeting of the 55 Tetralogy of Fallot (1954)
American Surgical Association (Westaby 1997).
F.J. Lewis and M. Taufic at the University of Lillehei endured almost hostile opposition.
Minnesota in Minneapolis were the first to clini- Critics talked about the potential of a 200 % mor-
cally apply hypothermia and caval inflow occlusion tality, as two persons were operated upon for only
after having done numerous experiments them- one patient to be treated. But the head of the
selves. On September 2, 1952, they operated on a Department of Surgery in Minneapolis, Owen
5-year-old girl after cooling her down to 28 °C rec- H. Wangensteen, always protected him and
tal temperature. The atrial septum defect they greatly supported his intellectual and surgical
found measured 2 cm and has been closed directly. enthusiasm (see 7 Chapter «Surgery for Congenital
After five and a half minutes, the inflow to the heart Heart Defects: A Historical Perspective», Sect. 11.2).
was opened again; the heart started beating (Lewis Hypothermia and cross circulation had opened
and Taufic 1953). Shortly thereafter, Henry Swan the window of direct vision-controlled heart sur-
from Denver, Colorado, reported on 13 patients he gery. The real breakthrough, however, only came
had operated upon in the same way (Swan et al. when finally the heart-lung machine became avail-
1953). See also 7 Chapter «Surgery for Congenital able for support of extracorporeal circulation. It
Heart Defects: A Historical Perspective», Sect. 11.2. was John Gibbon (1903–1973) who worked his
Ernst Derra (1901–1979), University of whole professional life, together with his wife Mary,
Düsseldorf, Germany, was the first in Europe to on the development of a machine to support extra-
perform open-heart surgery employing surface corporeal circulation. As early as 1937 he could
hypothermia; when in 1955, he closed a secundum demonstrate that heart and lung would, in princi-
atrial septum defect (Derra et al. 1965; Bircks ple, resume their full function after their work had
2002). Without any doubt, Derra and his group been temporarily taken over by an artificial pump
have set the standard in employing surface hypo- and oxygenator. However, only a few animals sur-
thermia for open-heart surgery in atrial septum vived and those only for a few hours (Gibbon
defect and pulmonary stenosis. Ranging well into 1939). Gibbon became more successful when, after
the 1960s, Derra’s group performed the largest World War II, he met Thomas Watson, Chairman
single hospital series of surface hypothermia in the of IBM, who was fascinated by Gibbon’s ideas and
world, comprising 1851 patients (Schulte 2001). promised to help him. A heart-lung machine was
At the University of Minnesota in Minneapolis, built with modified DeBakey roller pumps and
C. Walton Lillehei (1918–1999) developed the so- with a grid oxygenator (Stephenson 1997).
called «controlled cross circulation» (Lillehei et al. Employing this new Gibbon-IBM heart-lung
Chapter 1 · The History of Cardiac Surgery
15 1
machine, he successfully closed an ostium secun- thin blood film which provides a rapid gas
dum atrial septal defect in an 18-year-old girl on exchange. The disadvantage of both film oxygen-
May 6, 1953 (Gibbon 1954). When his next two ators is the complicated maintenance and assem-
patients died, he retired to investigate the potential bly, which requires a lot of time and personnel.
causes of the failure of his heart-lung machine. Extracorporeal circulation was simplified
Later he retired completely from cardiac surgery when a new, less complex and therefore easier to
and exclusively worked on issues in thoracic sur- set up oxygenator, the bubble oxygenator, was
gery (Stephenson 1997). finally developed by DeWall and Lillehei in 1955
As other groups also worked on the develop- (Lillehei et al. 1956; DeWall and Lillehei 1958).
ment of a heart-lung machine, so did C. Dennis This development was made possible by LC
(1902–2005) in Minneapolis; he, however, was Clark’s findings published 1950, who had demon-
unsuccessful with his first clinical case (Dennis strated that excess gas bubbles could be elimi-
et al. 1951). F. Dodrill (1902–1997) in Detroit, nated from blood by adding silicone components
who had built a heart-lung machine with the sup- to the oxygenator setup (Clark et al. 1950). The
port of General Motors, successfully used it as a previous inability to get rid of these bubbles, to
left heart bypass in a mitral valve operation defoam the oxygenated extracorporeal blood, had
(Dodrill et al. 1952). Shortly thereafter he also delayed the development of this type of oxygen-
used it as a right heart bypass in pulmonary valve ator significantly.
surgery (Dodrill et al. 1953). When Vincent Gott succeeded in building a
In Europe, among others, Clarence Crafoord one-way bubble oxygenator and have it produced,
(1899–1984), Viking Björk (1918–2009), and Ake it made preparation for cardiac operations much
Senning (1915–2000) in Sweden (Andersen and easier and significantly less time consuming (Gott
Senning 1946; Björk 1948), J. Jongbloed in et al. 1957). From then on, the DeWall-Lillehei
Holland (Jongbloed 1949), and AM Dogliotti in oxygenator became a standard element in the
Italy (Dogliotti 1951) experimented with extra- setup for extracorporeal circulation until the late
corporeal circulation. The world’s second success- 1970s. IH Rygg in Denmark succeeded in con-
ful operation with a heart-lung machine was the structing a similar device (Rygg and Kyvsgaard
excision of a left atrial myxoma in Stockholm, 1958). WJ Kolff and SA Clowes developed the first
Sweden (Senning 1954). membrane oxygenators (Clowes et al. 1956; Kolff
The final breakthrough in using the heart-lung et al. 1956). They came into practical use once
machine as a routine tool in open-heart surgery appropriate membranes could be produced suc-
was accomplished by John W. Kirklin (1917– cessfully with the reliability required. AJ Lande
2004) in the Mayo Clinic. After having visited described the first commercially available one-way
Gibbon and Dodrill, the Mayo group decided to membrane oxygenators for routine clinical use
build their own oxygenator-pump, based on the (Lande et al. 1967). Frank Gerbode and his group
work they were shown. During the winter of later reported success with other types of mem-
1954/1955, nine of ten experiments in dogs brane oxygenators (Gerbode et al. 1967; Hill et al.
employing the new Mayo-Gibbon heart-lung 1972; Zapol et al. 1979). See also 7 Chapter
machine were successful. The group decided to «Advances in Cardiopulmonary Bypass for the
choose eight patients to be operated upon. All of Neonate and Infant», Sect. 7.2.1 and chapter
these patients would be operated, even if the first «Surgery for Congenital Heart Defects: A Historical
seven surgeries had a fatal outcome. Four patients Perspective», Sect. 11.2).
survived, and with this the success story of cardiac Further innovations in extracorporeal circula-
surgery began (Kirklin et al. 1955; Kirklin 1989). tion comprised hemodilution, respectively, non-
The Mayo-Gibbon oxygenator represented a blood prime, when 5 % glucose or dextran was used
stationary grid oxygenator, in which the blood as reported by Zuhdi, Long, Cooley, and Neville in
passes along stiff narrowly spaced grids. Another the early 1960s (Westaby 1997). Another new fea-
principle is that of a cylinder oxygenator described ture was the integration of a heat exchanger into the
in 1953 by Denis Melrose (1953) or the rotating arterial side of the circuit (Sealey et al. 1958, 1959).
disk oxygenator described in 1956 by E. Kay and With this, the advantages of hypothermia could be
FS Cross (Cross et al. 1956; Kay et al. 1956). In integrated into the concept of extracorporeal circu-
both types of oxygenators, rotation produces a lation. The combination of deep hypothermia and
16 K.H. Leitz and G. Ziemer
extracorporeal circulation was developed by Hans different degrees lead to bleeding tendency, throm-
1 Borst in Germany for aortic arch surgery (Borst boembolism, fluid retention, and primary or sec-
1959; Borst et al. 1964) and by Y Hikasa in Japan ondary organ damage (Edmunds 1998). See also 7
and Brian G Barratt-Boyes in New Zealand for sur- Chapter «Extracorporeal Circulation and Myocardial
gical correction of congenital heart defects (Hikasa Protection in Adult Cardiac Surgery», Sect. 6.1.4.
et al. 1967; Barratt-Boyes et al. 1972).
A prerequisite for extracorporeal circulation
and whole-body perfusion had been the discov- 1.9 Development of Heart Valve
ery of heparin by J McLean in 1916 (McLean Surgery in the Era
1916) and protamine by E Chargaff and KB Olson of Extracorporeal Circulation
in 1937 (Chargraff and Olson 1937).
In order to limit the ischemic time of heart on The first aortic valve replacement with an artificially
one side, and still having sufficient time to operate manufactured valve was accomplished in 1960 by
on the immobilized heart, various methods were Dwight E. Harken (1910–1993) in Boston,
developed. Ake Senning (1952) very early on used Massachusetts. He implanted a prosthesis consisting
electrically induced ventricular fibrillation. For of a ball in a steel cage. It was implanted with single
aortic valve replacement, Dwight McGoon interrupted sutures into the aortic anulus. As he
(1925–1999) utilized selective coronary perfusion thought that the aortic wall could interfere with the
employing special cannulas (McGoon 1976). In ball in the cage and thus be affected, his valve had a
the beginning many groups used intermittent double cage (Harken 1989). For the implantation, he
interruption of coronary circulation by used extracorporeal circulation at a temperature of
intermittent aortic cross clamping. The time inter- 26 °C (Harken et al. 1960). Albert Starr (born 1926)
vals provided by this way were 15–20 min, which in Portland, Oregon, constructed a similar prosthe-
seemed to be sufficient for some (Cooley et al. sis for the mitral position in cooperation with the
1958a). But Denton Cooley also described the retired engineer Lowell Edwards (1898–1982). Their
«stone heart» (Cooley et al. 1972). Further toler- cooperation started in 1958. As did so many others,
ance to ischemia was achieved by topical cooling they started out to develop a bileaflet valve, which,
as suggested by the Stanford group (Shumway et in most cases, completely thrombosed within
al. 1959). Simple cardioplegia solutions were ready 2–3 days in their dog experiments. Therefore, they
for clinical use as early as 1955, like the potassium had to go back to the conception of the caged ball
citrate cardioplegia of Melrose (Melrose et al. valve, the concept used already in Hufnagel’s first
1955), the magnesium cardioplegia of Kirsch off-pump descending aortic valve implantation in
(Kirsch et al. 1972), or the sodium extraction car- 1952 (see 7 Sect. 1.7, «Heart Valve Surgery in the Time
dioplegia of Bretschneider (1964, 1980). Before Extracorporeal Circulation»).
Bretschneider’s solution was clinically tested by Ball valves absolutely did not resemble a natural
Sondergaard (Sondergaard and Senn 1967). From valve, but flawless function was more important
1975 on, experimentally proven solutions were than the shape and design. An advantage of the ball
commonly available, like the crystalloid HTK valve concept was the fact that the ball was not fixed
solutions after Bretschneider (1980) and the St. to the ring, and in this way thrombus formation
Thomas solution (Hearse et al. 1978) or blood car- beyond the ring, especially in mitral position, was
dioplegia after Buckberg (1982), which later was completely eliminated. In addition, the constant to-
modified by Calafiore (Calafiore et al. 1994). and-fro oscillation of the ball had a cleansing effect
Although extracorporeal circulation was and is on the ring. Furthermore, dogs with ball valve
an integral part of the success story of cardiac sur- implants in the mitral position showed clearly less
gery, it had to be learned that through temporary problems, and many of them became long-time
whole-body perfusion with extracorporeal survivors (Matthews 1998). The model chosen for
devices, harm can also be produced (Blackstone human use consisted of a cage of steel, a silastic ball,
et al. 1982; Kirklin et al. 1986; Kirklin and Barratt- and a Teflon sewing ring. After the first patient died
Boyes 1993; Edmunds 1997). The contact of blood 10 h postoperatively caused by an air embolism,
with artificial extracorporeal surfaces activates Albert Starr successfully implanted this type of ball
about five plasma proteins and five cell systems, valve in mitral position in the eight following
which through a cascade-type activation can to patients between July 1960 and February 1961.
Chapter 1 · The History of Cardiac Surgery
17 1
With the support of extracorporeal circulation, he the first time were reported in 1978, the convex-
operated through a right thoracotomy. Six patients concave valve was taken off the market in 1986
became long-term survivors, with two even going (Lindblom et al. 1986; Ostermeyer et al. 1987). A
back to work (Starr and Edwards 1961; Pluth 1991; follow-up model, the so-called monostrut valve
Matthews 1998). The original model of the Starr- (Björk and Lindblom 1985), which was manufac-
Edwards valve has been modified several times. tured out of one block of metal, therefore not
Sheathing the brackets of the cage with Teflon cloth requiring weld joints anymore, was introduced in
in order to reduce the incidence of thromboembo- response to the failures. Success was limited; when
lism did not prove successful. Only model 1260 and due to the worldwide problems with the old valve
model 6120 received subsequent FDA approval and the consecutive law suits, Shiley’s company
(Akins 1991), which entered the medical manufac- finally collapsed after a settlement (Westaby 1997).
turing environment in 1976 (Siposs 1989). Another Walt Lillehei also participated in the develop-
caged ball valve model is the Smeloff-Cutter valve, ment of tilting disk valve prostheses. In 1966 the
which was introduced on the market in 1966. Lillehei-Nakib toroidal discoid prosthesis was
Remarkable is its double- cage construction; the described, and it was followed in 1967 by the
smaller dimensions of the cage were favorable, improved Lillehei-Kaster pivoting disk valve
especially for implantation in the mitral position, as (Lillehei et al. 1974, 1977, 1989). Almost a cross-
this caused left ventricular outflow tract obstruc- breed between the Björk-Shiley and the Lillehei-
tion less often and to a lesser degree (Smeloff 1989). Kaster valve is the Medtronic-Hall tilting disk
Numerous artificial heart valves were developed, prosthesis, inserted for the first time in a patient
but did not find widespread acceptance or had been in June 1977. It was developed by the Norwegian
implanted only by a few, like the Barnard-Goosen Karl Victor Hall (1992, 1989) and received world-
valve or the Gott-Daggett valve (Westaby 1997). wide recognition (Akins 1995).
Progress came with the development of the tilt- It also was in Lillehei’s laboratories where the
ing disk heart valve prosthesis. Its design concept, a design of the first artificial double leaflet valve, the
mobile disk occluder contained in a ring, was devel- St. Jude valve, was thought of. In these models
oped by Juro Wada (1922–2011) from Sapporo the suture ring as well as the leaflets were made of
Medical College, Japan, in 1966. The Wada-Cutter synthetic nonthrombogenic pyrolytic carbon. The
valve was characterized by a very low profile, in leaflets, which are mounted into articular caves in
addition it had a significantly lower-pressure gradi- the valve prosthesis ring, open up to 85° and
ent compared to all other artificial heart valve mod- allowed for almost laminar blood flow. The first
els available at that time, as the central blood stream clinical implantation was on October 3, 1977, by
was not obstructed (Wada et al. 1989). Viking Björk DM Nicoloff in Minneapolis, Minnesota (Emery
(1918–2009) in Stockholm, Sweden, visited Wada et al. 1978; Lillehei et al. 1989).
in Japan and immediately started implanting the Only a few of the 32 mechanical heart valve
Wada-Cutter valve. He compared its performance prostheses that were developed from the mid-
with the Kaye-Shiley valve, which revealed a sig- 1960s to the end of the 1980s received FDA
nificantly higher-pressure gradient (Westaby 1997). approval and survived (Akins 1991). These are the
Therefore, Björk convinced Don Shiley to design Starr-Edwards valve, the Medtronic-Hall valve,
and build a prosthesis according to Wada’s concep- the St. Jude Medical valve, and the Omniscience
tion. Shiley was a former Edwards Laboratories valve (Akins 1991). In 1993 the Carbomedics
engineer, who later worked independently. The valve was approved as the second bileaflet valve in
result was the Björk-Shiley valve, which was the United States (Akins 1995).
implanted for the first time on January 16, 1969, in The era of homografts (postmortem-harvested
Stockholm by Björk himself (Björk 1969, 1984; human heart valves) began in 1962 with the
Westaby 1997). The original model consisted of a implantation of homografts by the Toronto group
ring with two brackets which lay within the ring (Heimbecker et al. 1962). Donald Ross (1922–
plane, in between which the disk moved. The open- 2014) in London, England, on July 24, 1962, and
ing angle originally was 60°. The disk underwent independently Brian Barratt-Boyes in Auckland,
various modifications, and from 1976 it became a New Zealand, on August 23, 1962, performed
convex-concave shape (Björk 1978). After a signifi- the first aortic valve replacements with aortic
cant number of fatal bracket fractures, which for homografts (Ross 1962; Barratt-Boyes 1965).
18 K.H. Leitz and G. Ziemer
Experimentally Alfred Gunning (1918–2011) and Shiley and Carpentier- Edwards biological heart
1 Carlos Duran in Oxford, England (Binet et al. valves (Carpentier 1972). Warren Hancock,
1971) worked on the extraction, conservation, employed at Edwards Laboratories until 1967,
and implantation of homografts but also of developed a porcine valve, which was implanted
heterografts (Duran and Gunning 1962). They
clinically by Robert Litwak (Kaiser et al. 1969).
suggested the technique Ross employed in his first Later models incorporated flexible stents (Westaby
successful clinical case (Ross 1962). As the implan- 1997). Because of transvalvular obstruction, espe-
tation of homografts was technically considerably cially in the smaller porcine bioprosthesis, a tech-
complex, Angell mounted homografts to a stent nique was devised to remove the native right
which made a mitral valve replacement possible coronary cusp, which contains in pigs a bar of ven-
(Binet et al. 1971). Instead of homografts, Ake tricular muscle. The removed cusp was substituted
Senning in Zürich and Marion Ionescu in Leeds, by a cusp of a second animal. The first Hancock
U.K., used valves constructed out of autologous modified orifice valve was used clinically in 1976
fascia lata and mounted them on a stent (Senning (Cohn et al. 1989).
1967; Ionescu et al. 1970). These fascia lata valves, During the 1980s, the early enthusiasm to use
however, had a high failure rate and were soon glutaraldehyde-treated bioprostheses cooled
abandoned (Thiede et al. 1971). Donald Ross in down since bioprostheses calcified with time and
1967 for the first time used the pulmonary artery the decreased durability became an established
valve of a patient as an aortic valve replacement fact (Carpentier et al. 1984). This opened up the
and restored the continuity between the right ven- development and use of stentless bioprotheses
tricle and the pulmonary artery with an aortic or a experimentally and in humans (David et al. 1992).
pulmonary homograft (Ross operation) (Ross To preserve the mitral valve in clinical mitral
1967). The first successful Ross operation in a neo- insufficiency had already been suggested by Walt
nate, employing the autograft’s growth potential, Lillehei (Lillehei et al. 1957) and later on by GH
was performed by Gerhard Ziemer in 1989 in Wooler (Wooler et al. 1962), GE Reed (Reed et al.
Hannover, Germany in a 2.7 kg baby (Ziemer 1965), and JH Kay (Kay et. 1978). But it is Alain
1992). After root remodeling surgery at 13 years, Carpentier who is entitled to the leadership of
the autograft got finally replaced at 26 years of age reconstructive mitral valve surgery. Since 1971, he
with a mechanical valve. concentrated his work on the anatomical changes
In 1964 Duran and Gunning in Oxford clini- in mitral regurgitation and published a classifica-
cally implanted for the first time a porcine valve tion based on the involved segment of the valve. It
prosthesis mounted on a stent (Binet et al. 1971). In dates back to him developing the understanding
Paris Binet and Carpentier implanted heterografts/ that mitral valve anulus dilatation primarily relates
xenografts mounted on a stent including the first to the posterior portion of the anulus. He was the
stented mitral heterograft prosthesis in 1967 first to suggest a rigid ring for stabilizing the mitral
(Carpentier 1972). Originally, the animal valves valve anulus and developed various technical
used, mainly porcine valves, were preserved in details for reconstructive mitral valve surgery. He
organic mercurial salt solution or 4 % formalde- summarized his experience in the famous paper
hyde, from 1968 on in glutaraldehyde. Glutaral published in 1983: Cardiac Valve Surgery-The
dehyde was introduced by Alain Carpentier. As an French Correction (Carpentier 1983).
associate Professor of Surgery, he interrupted his
surgical activity 2 days a week to study chemistry
and discovered glutaraldehyde (Spencer 1983; 1.10 Development of Coronary
Carpentier 1989). Glutaraldehyde diminishes anti- Surgery
genicity and avoids denaturation of collagen fibers,
strengthening the tissue by intense collagen cross- The first surgical attempts to treat coronary heart
linking at the same time. Carpentier summarized: disease were sympathectomy of cervical ganglia to
«The valve substitute obtained on this way is a bio- achieve analgesia and thyroidectomy to lower
prosthesis rather than a heterograft» (Binet 1989; metabolism and, in this way, cardiac oxygen con-
Carpentier 1989; Stephenson 1997). These experi- sumption (Parsons and Purks 1937; Mueller et al.
mental and first clinical experiences lead to the first 1997). Claude Beck, professor at the Western
commercially available bioprostheses: Ionescu- Reserve University in Cleveland, Ohio, went a step
Chapter 1 · The History of Cardiac Surgery
19 1
further following the idea of creating collateral ves- begun. Not only new diagnostic means to detect
sels to increase myocardial blood flow (Beck and the pathology of coronary artery disease were cre-
Tichy 1934; Beck 1935). He employed mechanical ated, but also the efficacy of the utilized surgical
irritation to create adhesions between the pericar- treatment modalities could be directly evaluated
dium and epicardium. He also interposed pectoral (Sones and Shirey 1962; Lichtlen 2002).
muscle or omentum to induce neovascularization Lillehei (Absolon et al. 1956) and Bailey
(Beck 1935). Similar procedures were reported by (Bailey et al. 1957) were the first to envision direct
O’Shaughnessy in London, England, and by coronary artery surgery with their experimental
A. Lezius in Hamburg, Germany (O’Shaughnessy open and closed coronary thromboendarterec-
1936; Lezius 1938). To achieve the same result, tomy. Longmire in Los Angeles, California
Lezius also sewed the lung to the epicardium, a pro- (Longmire et al. 1958), and Ake Senning in
cedure he called cardiopneumopexy (Lezius 1951). Stockholm, Sweden (Senning 1959), were the first
Arthur Vineberg (1903–1988) from Montreal, who applied these techniques in clinical cases.
Canada, went a step further when, in an experi- Rene Favaloro (1923–2000) from the Cleveland
mental setting in 1947, he implanted the mammary Clinic reported about 163 patients who under-
artery into a tunnel made into the left ventricular went open coronary thromboendarterectomy and
myocardium. To his surprise, there was no hema- vein or pericardial patch plasty between January
toma formation, as he had seen after implantation 1962 and May 1967. These procedures were per-
of an artery into a skeletal muscle in the same way. formed on a fibrillating heart with extracorporeal
So the myocardium seemed to have properties like circulation at a temperature of 30 ° C. It was pri-
a sponge. He explained this with sinusoids known marily the right coronary artery which was
from embryology. In cases of ischemia caused by treated. Twenty-eight patients died during hospi-
coronary disease, the sinusoids were supposed to tal stay (17 %). Of 94 patients who underwent
open up. He proved the existence of the assumed postoperative angiography, the result was consid-
connections by stain injections. Clinically he per- ered good in 53 (Favaloro 1970).
formed this procedure in 1950 for the first time and As these results were not satisfying, Favaloro
reported his 140 operations 1964 with a final mor- proceeded with direct coronary bypass surgery.
tality of 2 % (Vineberg 1946, 1958; Vineberg and On May 9, 1967, he implanted an aortocoronary
Buller 1955; Westaby 1997). Vineberg had his late venous bypass in a 51-year-old female patient
satisfaction through Mason Sones, who proved by who had proximal obstruction of the right coro-
angiography that most internal mammary artery nary artery on angiography. The left coronary
implants remained open and even developed anas- artery was unobstructed and fed via collateral ves-
tomoses to the native coronary arteries in 70–80 % sels the peripheral right coronary artery which
of the patients (Effler et al. 1963). had no further artheriosclerotic changes. On this
The evolution from the indirect to direct pro- healthy periphery, Favaloro anastomosed the
cedures in coronary surgery would not have been bypass vein. Twenty days later, the patient under-
possible without the development of coronary went postoperative coronary angiography. The
angiography by Mason Sones (1918–1985) at the aortocoronary artery venous bypass was open
Cleveland Clinic in Cleveland, Ohio. Before 1959 (Favaloro 1968, 1970). Between May 1967 and
coronary arteries were visualized only nonselec- December 1968, 171 coronary artery venous
tively by injecting contrast through a catheter bypass surgeries were performed. From this point
positioned in the aortic root. On October 30, on, the success story of coronary artery bypass
1958, the catheter migrated unnoticed into the surgery took off. Favaloro summarized the advan-
right coronary artery during contrast injection tages of the bypass graft technique as follows:
and an image was taken; the error was only 55 Low mortality
noticed after the angiography film was developed 55 Accessibility of all regions of the coronary
and ready to be inspected. The patient went artery system and hence
through the nonvoluntary maneuver without sub- 55 Enlarging the indication for coronary surgery
jective or even objective harm (Westaby 1997).
After repetition of direct coronary angiography Next to Favaloro, it is Dudley Johnson from
on 1,020 patients, Sones stated correctly that now Milwaukee, Wisconsin, who was part of the inau-
the era of selective coronary angiography had guration of routine coronary surgery. In 1969 he
20 K.H. Leitz and G. Ziemer
reported on 301 coronary patients whom he had Off-pump resections of ventricular aneurysms
1 operated upon since February 1967 and who in by simply applying big clamps between aneurys-
most cases received a vein graft (Johnson et al. mal sac and left ventricle were described by Likoff
1969). His conclusions were similar to Favaloro’s. and Bailey (1955). It was Denton Cooley in 1957
Johnson noted that there have been others who who for the first time employed extracorporeal
reported on sporadic cases of coronary artery circulation to resect a ventricular aneurysm
bypass surgery between 1962 and 1967. But none (Cooley et al. 1958b). A further development in
of these publications had any influence on further cardiac aneurysm surgery came from Vincent
development. Among those, for example, was a Dor, Monte Carlo, Monaco, who suggested an
venous bypass to the left anterior descending cor- endocardial patch plasty to maintain and receive a
onary artery in 1964, which, however, was better remodeling of the heart leading to improved
reported only much later (Garret et al. 1973). function (Dor et al. 1989).
Originally, coronary bypass surgery favored The first closure of a postinfarction ventricu-
vein grafts. Years later, Loop and Lytle, also from lar septal defect was performed by Cooley in 1957
the Cleveland Clinic, demonstrated the superior- (Cooley et al. 1957). The early operations were
ity of arterial grafts. Consequently, this made arte- performed only on hemodynamically stable
rial revascularization the technique of choice, patients through an incision in the right ventricle.
especially in young patients (Loop et al. 1986; Later on, emergency patients with huge left to
Lytle et al. 2004). The use of the internal mam- right shunts were also operated on. Heimbecker
mary artery as a bypass graft was reported as early et al. (1968), Dagett et al. (1970, Dagett (1990),
as 1967 by Vasilii Kolesov from St. Petersburg, Kitamura et al. (1971), and David et al. (1972) are
Russia. He operated through a left thoracotomy among those who developed the techniques cur-
on the beating heart without extracorporeal circu- rently used with incision through the infarcted
lation. Donald Effler (1915–2004), cardiac area and application of synthetic patch material.
surgeon-in-chief at Cleveland Clinic, discussed
this technique and suggested a pre- and postop-
erative angiographic control (Kolessov 1967). In 1.11 Pacemaker, Defibrillators,
the United States, Goetz, Bailey, and Green worked and Arrhythmia Surgery
using the mammary artery (Borst and Mohr
2001). But its widespread use started only after the P.M. Zoll opened the modern era of pacemaker
convincing publication out of the Cleveland therapy in 1951, when he treated Adam-Stokes
Clinic. The radial artery as a bypass graft was syndrome with externally applied stimulation
brought into discussion by Carpentier in 1973 (Zoll 1952). He fixed electrodes precardially to
(Carpentier and Guernonprez 1973). Because of the skin and stimulated the heart with intermit-
frequently seen graft spasms, the radial artery did tent electrical impulses. Skin changes were caused
not gain acceptance at the beginning, but rather and patients complained about bad pain.
experienced a renaissance in recent years. An important push to the development of
Following Dudley Johnson’s dictum: «anasto- pacemarkers came from Lillehei’s group in
moses on small vessels can’t be performed on a Minneapolis. Following VSD surgery, they repeat-
moving object» (Johnson et al. 1969), it was edly had to deal with the problem of complete AV
through the late 1990s that employing extracor- block. In experiments on dogs, they created com-
poreal circulation in coronary surgery seemed to plete heart block and applied low voltage current.
be mandatory. Nevertheless, there were already This leads to continuous heart beats again and
large off-pump coronary surgery series reported reestablished normal blood pressure.
showing acceptable results (Buffolo et al. 1985). Lillehei immediately put this laboratory expe-
SIRS (systemic inflammatory response syndrome) rience into clinical practice. He must have been
frequently is a response of the immune system to the first to implant temporary pacemaker wires
blood contact with artificial surfaces. In order to on the right ventricle. They could be removed by
avoid SIRS whenever possible, many groups simply pulling them once postoperatively the
almost exclusively embarked on off-pump coro- patient’s heart rhythm was stabilized. Lillehei also
nary surgery (Calafiore et al. 1996; Subramanian realized that wires could be introduced through
1997; Borst and Mohr 2001). cannulas into the closed chest and placed on the
Chapter 1 · The History of Cardiac Surgery
21 1
right ventricle, so that patients with Adam-Stokes designed for the surgical therapy of arrhythmias
syndrome could be helped without further surgi- became obsolete. The complex surgical procedures
cal interventions (Westaby 1997). Together with for atrial fibrillation, developed by Gerard
Earl Bakken, he built a pacemaker generator as Guiraudon in France and by James Cox in St. Louis,
big as a cigarette box, which could be carried Missouri, the so-called Maze procedure, under-
around outside the body. Attached to suspenders, went multiple variations following the develop-
it hung over the shoulder. The steel wires being ment of various ablation devices (see 7 Chapter
connected to the right ventricle and transmitting «Surgical Therapy of Atrial Fibrillation», Sect. 29.6).
the impulse were isolated with Teflon (Lillehei
et al. 1960). In this way pain and skin changes
which annoyed Zoll’s patients were eliminated. 1.12 Thoracic Organ
The first totally implantable pacemaker was Transplantations
inserted by Senning and Elmquist in 1958 in
Stockholm, Sweden (Elmquist and Senning 1959). After initial experimental work by Carrel and
The electrical switch had been embedded in epoxy Guthrie (1905), Demichov (1962), and Mann et al.
resin. The patient had a total AV block with fre- (1933), Richard Lower (1930–2008) and Norman
quent Adam-Stokes attacks. As the quicksilver bat- Shumway (1923–2006) at Stanford University in
teries used at that time had a short life span, a California developed the experimental foundation
patient might have to experience quite a lot of bat- for clinical heart transplantation (Lower and
tery changes. In the United States, it was William Shumway 1960; Lower et al. 1962). Starting from
Chardack in 1960 who implanted the first pace- the experiments on surface cooling for myocardial
maker in Buffalo, NY (Chardack et al. 1960). He protection, they perfected the surgical technique of
worked together with Wilson Greatbatch, an elec- orthotopic heart transplantation. With the recipi-
trical engineer. Other types of implantable pace- ent animal being on extracorporeal circulation in
makers were developed by Zoll in 1960 (Zoll and mild hypothermia for excision of the «sick» heart,
Linenthal 1960). A further milestone in pace- they developed the technique of leaving portions of
maker therapy was the development of tranvenous the posterior wall of the left and right atrium as a
electrodes, which avoided surgery to expose the cuff to be anastomosed to the donor heart.
right ventricle. Both in 1962 it was Parsonnet in Thereafter, aorta and pulmonary artery were anas-
the United States (Parsonnet et al. 1962) and tomosed. Some of their experiments were auto-
Ekestrom and Lagergren in Sweden (Ekestrom transplantations; others were heterotopic
et al. 1962) who were the first to implant transve- transplantations in dogs. When in 1967 they got
nous electrodes. Not only the demand function in the first dogs to survive more than 250 days with
1964 but also the principle of bifocal, AV- the aid of immunosuppressive drugs (azathioprine,
sequential stimulation in 1969, was developed by cortisone), they thought to be ready for the first
Castellanos et al. (1964) and Berkovitz et al. (1969). clinical heart transplantation (Lower et al. 1965).
The cumbersome short life spans of pace- The first homologous human heart transplan-
maker generators could be significantly prolonged tation in the world took place on December 3,
when W Greatbatch developed lithium batteries, 1967; however, it was not performed at Stanford
which have a life span five to ten times longer than University, California, but at Groote Schuur
quicksilver batteries (Greatbatch et al. 1978). Hospital in Cape Town, South Africa. The donor
The first implantable defibrillator (automatic was a young woman who died from a car accident,
internal cardioverter/defibrillator: AICD) was the recipient was a 54-year-old grocer named
implanted by M Mirowsky at Johns Hopkins Louis Washkansky, who had end-stage ischemic
Hospital in Baltimore, Maryland (Mirowsky et al. cardiomyopathy after multiple myocardial infarc-
1980). The first systems required a thoracotomy to tions, being considered inoperable by various
implant patch electrodes on the heart; however, authorities. He was in irreversible heart failure.
from 1991 on the transvenous implantation After donor brain death was confirmed, the trans-
became possible (Moore et al. 1991). plantation process started, in which a team of 30
As a result of the rapid development in pace- people under the leadership of Christiaan Barnard
maker and defibrillator technology, including less (1922–2001) participated. Before this, Barnard
invasive catheter methods, many of the techniques had visited Richard Lower’s laboratory to get first-
22 K.H. Leitz and G. Ziemer
hand information about the transplantation tech- sporin A, which was available to the Stanford
1 nique developed by Shumway and Lower, the group in 1980 (Oyer et al. 1983).
technique he employed (Barnard 1967). 55 The concept of retransplantation for acute
After the donor heart was cooled to 16 °C, it and chronic rejection was established
was put in 10° cold saline and transported to the (Copeland et al. 1977).
next door recipient operating room, where it was
perfused with blood. The ischemic time was Due to the new facilitating supportive fea-
4 min. Barnard described the moment after he tures, the number of heart transplantations rose
had excised the diseased recipient’s heart that for up to almost 4,500 worldwide cases in 1995. By
the first time in his life he looked into an empty that time, heart transplantation had become an
chest (Schmid et al. 2003). Louis Washkansky established surgical treatment for end-stage car-
died on December 21, 1967, of pneumonia. diac failure (see 7 Chapter «Heart and Heart-Lung
The second heart transplantation in the world Transplantation», Sect. 37.1.1).
was performed 3 days later, on December 6, 1967, The first attempts of combined heart and lung
by A. Kantrovitz at Maimonides Hospital in transplantation were undertaken 1968 by Cooley,
Brooklyn, New York. But the recipient, a 2-day- 1969 by Lillehei, and 1981 by Barnard.They all
old baby, died immediately after the operation were not successful (Westaby 1997). It was Bruce
(Schmid et al. 2003). Reitz in Stanford, California, who, after exhaus-
On January 2, 1968, Barnard transplanted the tive animal experiments on monkeys (Reitz et al.
heart of an organ donor who died of subarach- 1980) based on Castañedas heart-lung autotrans-
noid bleeding to the 58-year-old dentist Philip plantation experiments in baboons (Castañeda
Blaiberg. Blaiberg survived for 18 months. He et al. 1972), succeeded in applying this surgery on
finally died of chronic rejection (Westaby 1997). a 45-year-old woman with primary pulmonary
Soon thereafter, other heart surgeons followed hypertension on March 9, 1981. The woman was
with their first clinical heart transplantations: extubated 48 h after surgery and survived for
Shumway in Stanford; again Kantrovitz in more than 5 years. Reitz’s second patient, who had
Brooklyn; Sen in Bombay, India; Cabrol in Paris, Eisenmenger’s syndrome caused by a VSD, was a
France; Ross in London, England; and indepen- long-term success, also (Reitz et al. 1982).
dently Cooley as well as DeBakey, both in The first isolated lung transplantation was per-
Houston, Texas. The results of the more than 100 formed by James Hardy in 1963. His patient died
patients transplanted within a year after Barnard’s 7 days after surgery (Hardy et al. 1963). More suc-
first operation were very poor. More than 60 cess had Fritz Dermon (Dermon et al. 1971) from
patients died within the first postoperative week; Belgium, whose patient survived for 10 months.
average survival for the remaining patients was The final scientific and clinical breakthrough in
less than 30 days (Westaby 1997). The problems of lung transplantation surgery was achieved by Joel
indications, definition of brain death, myocardial Cooper’s group in Toronto, Canada. They reported
protection, as well as rejection and its diagnosis on two patients who had returned to normal life
diminished the initial enthusiasm. Most of these activities after surgery and survived lung trans-
problems were solved in the following years, plantation 14 and 26 months (Cooper et al. 1986).
mainly by the Stanford group: Since then, the number of lung transplantations
55 Exclusion criteria for heart transplantation performed yearly has progressively risen all over
were specified (Jamieson et al. 1982). the world. In 2013, lung transplantation was per-
55 Transvenous biopsy was introduced for rejec- formed in more than 4000 patients (see 7 Chapter
tion diagnosis (Caves et al. 1973). «Lung Transplantation», Sect. 36.1, . Table 36.1).
55 New concepts for myocardial protection made
long-distance organ procurement possible.
Ischemia of up to 4 h could be accepted 1.13 Circulatory Assist Devices
(Thomas et al. 1978).
55 Improvement of immunosuppressive medica- The idea of mechanical circulatory assist devices is
tion, first by human antithymocyte globulin old. Different approaches were followed. The con-
with introduction of T-cell monitoring (Bieber cept of diastolic augmentation to improve coronary
et al. 1976), thereafter by introduction of cyclo- perfusion was developed in experimental animal
Chapter 1 · The History of Cardiac Surgery
23 1
studies by the brothers Kantrowitz (1953) and two more patients (Campbell et al. 1988). PE
Kantrowitz and Kinnen (1958). Internal counter- Oyer in Stanford did the first successful «bridge
pulsation was employed by Claus and coworkers to transplant», when he successfully trans-
(1961). Thereby the blood was aspirated from the planted a heart after having implanted a
aorta during systole and injected back during dias- Novacor left heart assist system before (Portner
tole. With modifications and, most importantly, the et al. 1985), see also 7 Chapter «Cardiac Assist
availability of inflatable latex balloons, Moulopoulos Devices and Total Artificial Heart», Sect. 38.2.
and coworkers succeeded in 1962 in the develop- The patient Barney Clark was the first to
ment of the intra-aortic balloon pump (IABP) undergo a complete artificial orthotopic heart
(Moulopoulos et al. 1962). Inflating and deflating replacement. In December 1982, a Jarvik Type 7
of gases, at first C02, later helium, were ECG trig- total artificial heart was implanted in the
gered. The first clinical successes were reported by University of Utah by William DeVries. The
Kantrowitz in 1968 (Kantrowitz et al. 1968). Since patient was extubated on the second postopera-
1979, the Seldinger technique allowed insertion of tive day, but he died on the 112th postoperative
the IABP balloon pump from the periphery day after multiple, mostly thromboembolic, com-
through the femoral artery (Bregman and plications (De Vries et al. 1984; Westaby 1997).
Cassarella 1980). The intra-aortic balloon pump, in The idea of using peripheral muscles to aug-
addition to increasing coronary perfusion in dias- ment cardiac performance came from Kantrowitz
tole, also causes systolic afterload reduction. and MiKinnen (1958). But the idea could only be
Extracorporeal membrane oxygenation realized once the fatigability of peripheral muscles
(ECMO), developed by Hill et al. (1972) and Zapol was understood and managed (Macoviak et al.
et al. (1979) and modified by Gattinoni (Pesenti 1982). Of same importance was the knowledge to
et al. 1993), is another principle for treating post- correctly stimulate the skeletal muscle
cardiotomy heart failure. The ECMO principle is a (Drinkwater et al. 1980). The first one to apply the
long-term extracorporeal circulatory and respira- cardiomyoplasty clinically was Carpentier in 1985
tory support, derived from its use during cardiac with a transformed latissimus dorsi muscle
surgery. As left ventricular afterload cannot be wrapped around the heart (Carpentier and
reduced with the ECMO system alone, the combi- Chachques 1985). See also 7 Chapter «Left
nation of both support systems, ECMO and IABP, Ventricular Reconstruction and Conventional Surgery
has been suggested (Bavaria et al. 1990). for Cardiac Failure», Sect. 23.3.4.
The first realistic attempts to replace the heart
with an artificial implant are attributed to Willem
Kolff and his coworkers T. Akutsu and Y. Nose at 1.14 The History of (Thoracic) Aortic
the Cleveland Clinic. They implanted in the mid- Surgery
1960s four-chambered air-driven artificial hearts
into calves in an orthotopic position. The animals The first surgeons who concentrated their efforts
could be kept alive for more than 24 h. In 1967 on the thoracic aorta and its branches were HB
Kolff became head of the Division of Artificial Shumacker (1947), Henry Swan et al. (1950), Henry
Organs and Institute for Biomedical Engineering Bahnson (1953), as well as Michael DeBakey and
at the University of Utah. He worked with Kwan- Denton Cooley (1953). The first infrarenal aortic
Gett and Robert Jarvik on the design of a com- aneurysm was resected by Charles DuBost in 1951
pletely implantable artificial heart (Westaby 1997). (DuBost et al. 1952). They all replaced the aortic
In Houston, Texas, Domingo Liotta (born segments resected with aortic tube homografts.
1924) designed artificial ventricles, planned to The first synthetic tube graft, made from
be used as pumps for extracorporeal circulation Vinyon- N, was implanted in 1952 by Arthur
or as an artificial heart. Already in 1966 Michael Voorhees (1921–1991) at Columbia University in
DeBakey (1908–2008) successfully employed New York, NY (Voorhees et al. 1952). They had
an artificial Liotta ventricle as part of a left heart found that synthetic grafts were accepted by the body
bypass in a 37-year-old woman for postcardiot- as a vessel substitute, when the material used had a
omy heart failure. After 10 days, she was weaned rather porous structure and was biologically indiffer-
off bypass and became a long-term survivor ent. Around the same time, DeBakey started using
(DeBakey 1971). DeBakey was successful with Dacron, with the first grafts being manufactured
24 K.H. Leitz and G. Ziemer
Churchill ED (1929) Decortication of the heart for adhesive David H, Hunter JA, Najafi H et al. (1972) Left ventricular
1 percarditis. Arch Surg 19:1457–1469
Clark LC, Gollan F, Gupta VB (1950) The oxygenation of
approach fort the repair of ventricular septal perfora-
tion and infarctectomy. J Thorac Cardiovasc Surg 63:14
blood by gas dispersion. Science 111:85–87 David TE, Bos J, Rakowski H (1992) Aortic valve replace-
Clauss RH, Birtwell WC, Albertal G et al. (1961) Assisted cir- ment with the Toronto SPV bioprothesis. J Heart Valv
culation: I. The arterial counterpulsator. J Thorac Dis 1:244–248
Cardiovasc Surg 41:447 DeBakey ME (1971) Left ventricular bypass pump for car-
Clowes SA, Hopkins A, Neville WE (1956) An artificial lung diac assistance. Am J Cardiol 27:3
dependent upon diffusion of oxygen and carbon DeBakey ME, Cooley DA (1953) Surgical considerations
dioxide through plastic membranes. J Thorac Surg and excisional therapy for aortic aneurysms. Surgery
32:630 34:1005–1020
Cohn LH, Disesa VJ, Collins JJ (1989) The Hancock modified- DeBakey ME, Crawford ES, Cooley DA et al. (1957) Successful
orifice porcine bioprosthetic valve: 1976–1988. Ann resection of fusiform aneurysm of aortic arch with
Thorac Surg 48:S81–S82 replacement by homograft. Surg Gynecol Obstet 105:657
Cooley DA, DeBakey ME (1956) Resection of entire ascend- DeBakey ME, Cooley DA, Crawford ES, Morris GC (1958)
ing aorta in fusiform aneurysms using cardiac bypass. Clinical application of a new flexible knitted dacron
JAMA 162:1158 arterial graft. Am Surg 24:862–869
Cooley DA, Belmonte BA, Zeis LB, Schnur S (1957) Surgical Demichov VP (1962) Experimental transplantation of vital
repair of ruptured interventricular septum following organs (authorized translation). Consultants Bureau
acute myocardial infarction. Surgery 41:930 Enterprises, New York
Cooley DA, Collins HA, Giacobine JW et al. (1958a) The Dennis C, Spreng DS, Nelson GE et al. (1951) Development
pump oxygenator in cardiovascular surgery: observa- of a pump oxygenator to replace the heart and lungs:
tions based upon 450 cases. Am Surg 24:870–882 an apparatus applicable to human patients and appli-
Cooley DA, Collins HA, Morris GC et al. (1958b) Ventricular cation to one case. Ann Surg 134:709
aneurysm after myocardial infarction: surgical exci- Derom F, Barbier F, Ringoir S et al. (1971) Ten-month sur-
sion with use of temporary cardiopulmonary bypass. vival after lung homotransplantation in man. J Thorac
JAMA 167:557 Cardivasc Surg 61:835
Cooley DA, Reul GJ, Wukasch DC (1972) Ischemic contrac- Derra E, Grosse-Brockhoff, Loogen F (1965) Der
ture of the myocardium “stone heart”. Am J Cardiol Vorhofseptumdefekt. Ergeb Inn Med Kinderheilkd
29:575 22:211–267
Cooper JD, Ginsber RJ, Goldberg M et al. (1986) Unilateral DeVries WC, Anderson JL, Joyce LD et al. (1984) Clinical use
lung transplantation for pulmonary fibrosis. N Engl of total artificial heart. N Engl J Med 310:273
J Med 314:1140 DeWall RA, Lillehei CW (1958) Design and clinical applica-
Copeland JG, Griepp RB, Bieber CP et al. (1977) Successful tion of the helix reservoir pump-oxygenator system for
retransplantation of the human heart. J Thorac extracorporeal circulation. Post-Grad Med 23:561–573
Cardiovasc Surg 73:242 Dewey M, Schagen U, Eckart WU, Schönenberger E (2006)
Crafoord C (1929) Two cases of obstructive pulmonary Ernst Ferdinand Sauerbruch und seine ambivalente
embolism successfully operated upon. Acta Chir Rolle während des Nationalsozialismus. Mitteil Dtsch
Scand 64:172–186 Ges Chir 35:325–333
Crawford FA (2010) Horace Smithy: pioneer heart surgeon. Dodrill FD, Hill E, Gerisch RA (1952) Temporary mechanical
Ann Thorac Surg 89:2067–2071 substitute for the left ventricle in man. JAMA 150:642
Crawford ES, Saleh CA (1981) Transverse aorta and aneu- Dodrill FD, Hill E, Gerisch RA, Johnson A (1953) Pulmonary val-
rysm: improved results of treatment employing new vuloplasty under direct vision using the mechanical
modifications of aortic reconstruction and hypother- heart for a complete bypass of the right heart in a patient
mic cerebral circulatory arrest. Ann Thorac Surg with congenital pulmonary stenosis. J Thorac Surg 25:584
194:180–188 Dogliotti AM (1951) Clinical use of the artificial circulation
Crawford ES, Coselli JS, Safi HJ (1987) Partial cardiopulmo- with a note on intra-arterial transfusion. Bull Johns
nary bypass, hypothermic circulatory arrest, and pos- Hopkins Hosp 90:131
terolateral exposure for thoracic aortic aneurysm Dor V, Saab M, Coste P et al. (1989) Left ventricular aneurysm:
operation. J Thorac Cardiovasc Surg 94:824–827 a new surgical approach. Thorac Cardiovasc Surg 37:11
Cross FS, Berne RM, Hirsoe Y, Kay EB (1956) Description and Drinkwater D, Chiu RC, Modry D et al. (1980) Cardiac assist
evaluation of a rotating disc type reservoir oxygen- and myocardial repair with synchronously stimulated
ator. Surg Forum 7:274 skeletal muscle. Surg Forum 31:271
Cutler EC, Levine SA (1923) Cardiotomy and valvulotomy Dubost C, Allary, Oeconomos N (1952) Resection of an
for mitral stenosis. Boston Med Surg J 188:1023 aneurysm of the abdominal aorta: reestablishment of
Daggett WM (1990) Postinfarction ventricular septal defect the continuity by a preserved human arterial graft,
repair: retrospective thoughts and historical perspec- with results after five months. Arch Surg 62:405
tives. Ann Thorac Surg 50:1006 Duran CG, Gunning AJ (1962) Heterograft in aortic posi-
Daggett WM, Burwell LR, Lawson DW, Austen WG (1970) tion. Lancet 2:486
Resection of acute ventricular aneurysm and ruptured Edmunds LH (1997) Extracorporeal circulation. In:
interventricular septum after myocardial infarction. N Edmunds LH (ed) Cardiac surgery in the adult.
Engl J Med 283:1507 McGraw-Hill, New York, pp 272–282
Chapter 1 · The History of Cardiac Surgery
27 1
Edmunds LH (1998) Inflammatory response to cardiopul- Griepp RB, Stinson EB, Hollingsworth JF et al. (1975)
monary bypass. Ann Thorac Surg 66:12–16 Prosthetic replacement of the aortic arch. J Thorac
Edwards WS, Edwards PD (1974) Alexis Carrel: visionary Cardiovasc Surg 70:1051–1063
surgeon. Charles C Thomas, Springfield, p 93 Gross RE, Hubbard JP (1939) Surgical ligation of a patent
Effler DB, Groves LK, Sones FM et al. (1963) Increased myo- ductus arteriosus. Report of first successful case. JAMA
cardial perfusion by internal mammary artery implant: 112:729
Vineberg’s operation. Ann Surg 158:526 Haecker R (1907) Experimentelle Studien zur Pathologie
Eisenmenger V (1893) Zur Tamponade des Larynx nach und Chirurgie des Herzens. Arch Klin Chir 84:
Prof. Maydl. Wiener Med Wochenschr 43:199–200 1035–1098
Ekestrom S, Johansson L, Lagergren (1962) Behandling av Hall KV (1989) The Medronic-Hall heart valve: background,
Adams-Stokes-syndrom med en intracardiell pace- latest results, and future work. Ann Thorac Surg 48:S47–
maker electrod. Opusc Med 7:1 S48
Elmquist R, Senning A (1959) An implantable pacemaker Hall KV (1992) The Medtronic-Hall valve: a design in 1977 to
for the heart. Proc 2nd intern conf med electronics. improve the results of valve replacement. Eur
Paris, Iliffe& Sons, London J Cardiothorac Surg 6(Suppl1):S64
Emery RW, Palquist WE, Mettler E, Nicoloff DM (1978) A Hardy JD, Webb WR, Dalton ML et al. (1963) Lung homo-
new cardiac prosthesis: in vitro results. Trans Am Soc transplantations in man: report of the initial case. JAMA
Artif Intern Organs 24:550–556 286:1065
Favaloro RG (1968) Saphenous vein autograft replacement Harken DE (1989) Heart valves: ten commandments and still
of severe segmental coronary artery occlusion. Ann counting. Ann Thorac Surg 48:S18–S19
Thorac Surg 5:334–339 Harken DE, Curtis LE (1967) Heart surgery-legend and a long
Favaloro RG (1970) Surgical treatment of coronary arterio- look. Am J Cardiol 19:393–400
sclerosis. Williams & Wilkins, Baltimore Harken DE, Soroff HS, Taylor WJ et al. (1960) Partial and com-
Fischer G (1868) Die Wunden des Herzens und des plete prosthesis in aortic insufficiency. J Thorac
Herzbeutels. Arch Klin Chir 9:571 Cardiovasc Surg 40:744
Forssmann W (1929) Die Sondierung des rechten Herzens. Hearse DJ, Stewart DA, Braimbridge MV (1978) Myocardial
Klin Wochenschr 8:2085–2087 protection during ischemic cardiac arrest: importance
Forssmann W (1931) Über die Kontrastdarstellung der of magmesium in cardioplegic infusates. J Thorac
Höhlen des lebenden rechten Herzens und der Cardiovasc Surg 75:877
Lungenschlagader. MMW 78:489 Heimbecker RO, Baird RJ, Lajos RJ et al. (1962) Homograft
Forssmann W (1972) Selbstversuch, Erinnerungen eines replacement of the human valve: a preliminary report.
Chirurgen. Droste, Düsseldorf Can Med Assoc J 86:805
Frey EK (1978) Rückschau und Umschau. Demeter, Heimbecker RO, Lemire G, Chen C (1968) Surgery for mas-
Gräfelfing sive myocardial infarction. Circulation 11–3(Suppl 2):37
Frey VM, von Gruber M (1885) Untersuchungen über den Hikasa Y, Shirotani H, Satomura K et al. (1967) Open heart
Stoffwechsel isolierter Organe: Ein Respirations-Apparat surgery with the aid of hypothermic anesthesia. Nippon
für isolierte Organe. Virchow’s Arch Physiol 9:519–532 Geka Hokan 36:495
Galletti PM (1993) Cardiopulmonary bypass: a historical Hill JD, O’Brien TG, Murray JJ et al. (1972) Prolonged extra-
perspective. Artif Org 17:675 corporeal oxygenation for acute post-traumatic respira-
Galletti PM, Brecher GA (1962) Heart-lung bypass. Grune tory failure (shock- lung syndrom): use of the Bramson
and Stratton, New York membrane lung. N Engl J Med 286:629
Garrett HE, Dennis EW, DeBakey ME (1973) Aortocoronary Hufnagel CA (1951) Aortic plastic valvular prosthesis Bull
bypass with saphenous vein: seven year follow up. Georgetown. Med Cent 4:128–130
JAMA 223:792–794 Hufnagel CA, Harvey WP, Rabil PJ et al. (1954) Surgical cor-
Gerbode F, Osborn JJ, Bramson ML (1967) Experience in rection of aortic insufficiency. Surgery 35:673
the development of a membrane heart-lung machine. Ionescu MI, Ross DN, Wooler G et al. (1970) Replacement of
Am J Surg 114:16 heart valves with autologous fascia lata. Br J Surg
Gibbon HJ (1939) Maintenance of life during experimental 57:437
occlusion of the pulmonary artery followed by sur- Jakoby C (1895) Ein Beitrag zur Technik der künstlichen
vival. Surg Gyenecol Obstet 69:602–614 Durchblutung überlebender Organe Arch. Exp Path
Gibbon HJ (1954) Application of a mechanical heart and lung (Leipzig) 31:330–348
apparatus to cardiac surgery. Minn Med 37:171–180 Jamieson SW, Oyer PE, Reitz BA et al. (1982) Cardiac trans-
Goerig M, Schulte am Eich J (2003) Die Anästhesie in der plantation at Stanford. Heart Transplant 1:36
ersten Hälfte des 20. Jahrhunderts. In: Schüttler L (ed) Jeger E (1913) Die Chirurgie der Blutgefäße und des Herzens.
50 Jahre Deutsche Gesellschaft für Anästhesiologie August Hirschwald, Berlin
und Intensivmedizin. Springer, Berlin, pp 27–65 Jeger E (1914a) Der gegenwärtige Stand der
Gott VL, DeWall RA, Paneth M et al. (1957) A self-contained Blutgefäßchirurgie. Berl Klin Wschr 51:1645–1648,
disposable oxygenator of plastic sheet for intracardiac 1667–1669
surgery. Thorax 12:1–9 Jeger E (1914b) Kriegschirurgische Erfahrungen über die
Greatbatch W (1978) Pacemaker energy sources old and Gefäßnaht. Berl Klin Wschr 51:1907–1911
new. In: Thalen HJ, Harthome JW (eds) To pace or not Jeger E, Israel W (1913) Über Neoimplantation der V. renalis
to pace. Martinus Nijhoff, The Hague, p 105 in die V. cava. Arch Klin Chir 100:893–924
28 K.H. Leitz and G. Ziemer
Johnson WD, Flemma RJ, Lepley D, Ellison EH (1969) ment of the ascending aorta and aortic valve. J Thorac
1 Extended treatment of severe coronary artery disease: a
total surgical approach. Ann Surg 171:460
Cardiovasc Surg 92:691–705
Krüger H (1909) Ein nach Trendelenburg operierter Fall
Jongbloed J (1949) The mechanical heart-lung system. von Embolie der Lungenarterie. Zentralbl Chir 36:
Surg Gynecol Obstet 89:684 757–762
Kaemmerer H, Meisner H, Hess J, Perloff JK (2004) Surgical Kuhn F (1901) Die perorale Intubation. Zbl Chir 28:1281–1285
treatment of patent ductus arteriosus: a new historical Lande AJ, Dos SJ, Carlson RG et al. (1967) A new membrane
perspective. Am J Cardiol 94:1153–1154 oxygenator-dialyzer. Surg Clin North Am 47:1461–
Kaiser GA, Hancock WD, Lukban SB, Litwack RS (1969) 1470
Clinical use of a new designed stented xenograft heart Leitz KH (2005) Videoclip, aus der Reihe Zeitzeugen berich-
valve prosthesis. Surg Forum 20:137 ten Prof. HG Borst DGTHG. Berlin
Kantrowitz A, Kantrowitz A (1953) Experimental augmen- Lewis FJ, Taufic M (1953) Closure of atrial septal defects with
tation of coronary flow by retardation of arterial pres- aid of hypothermia: experimental accomplishments
sure puls. Surgery 34:678 and the report of one successful case. Surgery 33:52
Kantrowitz A, MiKinnen WMP (1958) The experimental use Lezius A (1938) Die anatomischen und funktionellen
of the diaphragm as an auxiliary myocardium. Surg Grundlagen der künstlichen Blutversorgung des
Forum 9:266 HerzmuskelsdurchdieLungebeiKoronararterienverschluß.
Kantrowitz A, Tjonneland S, Freed PS et al. (1968) Initial Arch klin Chir 191:101
clinical experience wirh intraaortic balloon pumping Lezius A (1951) Die Behandlung der Angina pectoris und
in cardiogenic chock. JAMA 203:135 der Koronarinsuffizienz durch die Kardio-
Kay EB, Zimmerman HA, Berne RM et al. (1956) Certain Pneumopexie. Langenbecks Arch 267:576–580
clinical aspects in the use of the pump oxygenator. Lichtlen PR (2002) Geschichte der koronaren
JAMA 162:639 Herzerkrankung. In: Lüderitz B, Arnold G (eds) 75 Jahre
Kay JH, Zubiate T, Mendez MA et al. (1978) Mitral valve Deutsche Gesellschaft für Kardiologie-Herz- und
repair for significant mitral insufficiency. Am Heart Kreislaufforschung. Springer, Berlin, pp 269–306
J 96:243 Likoff W, Bailey CP (1955) Ventriculoplasty: excision of
Kazui T, Kimura N, Yamada O, Komatsu S (1994) Surgical myocardial aneurysm. JAMA 158:915
outcome of aortic arch aneurysms using selective Lillehei CW (1977) Heart valve replacement with the pivot-
cerebral perfusion. Ann Thorac Surg 57:904–911 ing disc prosthesis. Appraisal of results and description
Kirklin JW (1989) The middle 1950s and C Walton Lillehei. of a new all-carbon model. Med Instrum 11:85–94
J Thorac Cardiovasc Surg 98:822 Lillehei CW, Cohen M, Warden HE et al. (1955a) The result of
Kirklin JW, Barratt-Boyes BG (1993) Cardiac surgery. direct vision closure of ventricular septal defects in
Churchill-Livingstone, New York eight patients by means of controlled cross circula-
Kirklin JW, DuShane JW, Patrick RT et al. (1955) Intracardiac tion. Surg Gynecol Obstet 101:446
surgery with the aid of a mechanical pump-oxygenator Lillehei CW, Cohen M, Warden HE et al. (1955b) The direct
system (Gibbon type): report of eight cases. Mayo Clin vision intracardiac correction of congenital anomalies
Proc 30:201 by controlled cross circulation. Surgery 38:11
Kirklin JK, Chenoweth DE, Naftel DC et al. (1986) Effects of Lillehei CW, DeWall RA, Read RC et al. (1956) Direct vision
protamine administration after cardiopulmonary intacardiac surgery in man using a simple disposable
bypass on complement, blood elements and the artificial oxygenator. Dis Chest 29:1
hemodynamic state. Ann Thorac Surg 41:193 Lillehei CW, Gott VL, DeWall RA et al. (1957) Surgical correc-
Kirsch U, Rodewald G, Kalmar P (1972) Induced ischemic tion of pure mitral insufficiency by annuloplasty under
arrest. Clinical experience with cardioplegia in open direct vision. Lancet 77:446
heart surgery. J Thorac Cardiovasc Surg 63:121–130 Lillehei CW, Gott VL, Hodges PC et al. (1960) Transistor
Kirschner M (1924) Ein durch die Trendelenburgsche pacemaker for treatment of complete atrioventricular
Operation geheilter Fall von Embolie der Arteria dissociation. JAMA 172:324–328
Pulmonalis. Arch Klin Chir 133:312 Lillehei CW, Kaster RL, Coleman M et al. (1974) Heart valve
Kitamura S, Mendez A, Kay JH (1971) Ventricular septal replacement with Lillehei-Kaster pivoting disk pros-
defect following myocardial infarction: experience thesis. NY State J Med 74:1426–1438
with surgical repair through a left ventriculotomy and Lillehei CW, Nakib A, Kaster RL et al. (1989) The origin and
review of the literature. J Thorac Cardiovasc Surg development of three mechanical valve designs:
61:186 Toroidal disc, pivozing disc, and rigid bileaflet cardiac
Kolessov VI (1967) Mammary artery-coronary anastomosis prostheses. Ann Thorac Surg 48(S):35–37
as a method of treatment for angina pectoris. J Thorac Lillehey CW (1993) Historical development of cardiopulmo-
Cardiovasc Surg 54:536–543 nary bypass 1–26. In: Cardiopulmonary bypass: princi-
Kolff WJ, Effler DB, Groves LK et al. (1956) Disposable mem- ples and practice. Williams & Wilkins, Baltimore,
brane oxygenator (heart-lung machine) and its use in p 1993
experimental surgery. Cleve Clin Q 23:69 Lindblom D, Björk VO, Semb BH (1986) Mechanical failure of
Kouchoukos NT, Marshall WG, Wedige-Stecher TA (1986) the Björk-Shiley valve. J Thorac Cardiovasc Surg
Eleven- year experience with composite graft replace- 92:894–907
Chapter 1 · The History of Cardiac Surgery
29 1
Longmire WP, Cannon JA, Kattus AA (1958) Direct vision Mueller RL, Rosengart TK, Isom OW (1997) The history of
coronary endarterectomy for angina pectoris. N Engl surgery for ischemic heart disease. Ann Thorac Surg
J Med 259:993–999 63:869–878
Loop FD, Lytle BW, Crosgrove DM et al. (1986) Influence of Nissen R (1955) Erlebtes aus der Thoraxchirurgie. Thieme,
the internal-mammary-artery graft on 10-year survival Stuttgart
and other cardiac events. N Engl J Med 314:1–6 Nissen R (1969) Helle Blätter–Dunkle Blätter: Erinnerungen
Lower RR, Shumway NE (1960) Studies on orthotopic trans- eines Chirurgen. Deutsche Verlags Anstalt, Stuttgart
plantation of the canine heart. Sur Forum 11:18 Nystrom G (1930) Experiences with the Trendelenburg
Lower RR, Stofer RC, Hurley EJ et al. (1962) Successful operation for pulmonary embolus. Ann Surg 92:
homotransplantations of the canine heart after anoxic 498–532
preservation for seven hours. Am J Surg 104:302 O’Shaughnessy L (1936) Experimental method of providing
Lower RR, Dong E, Shumway NE (1965) Long-term survival collateral circulation to the heart. Br J Surg 23:665–670
of cardiac homografts. Surgery 58:110–119 Ostermeyer J, Horstkotte D, Bennet J et al. (1987) The
Lytle BW, Blackstone EH, Sablik JF et al. (2004) The effect of Björk-Shiley 70° convex-concave prosthesis strut frac-
bilateral internal thoracic artery grafting on survival dur- ture problem. J Thorac Cardiovasc Surg 35:71
ing 20 postoperative years. Ann Thorac Surg 78:2005–2014 Ott DA, Frazier OH, Cooley DA (1978) Resection of thee
Macoviak JA, Stephenson LW, Armenti F et al. (1982) aortic arch using deep hypothermic and temporary
Electrical conditioning of in situ skeletal muscle for circulatory arrest. Circulation 58(Suppl1):227–231
replacement of myocardium. J Surg Res 32:429 Oyer PE, Stinson EB, Jamieson SW et al. (1983) Cyclosporine
Mann FC, Priestly JR, Markowitz J et al. (1933) Transplantation in cardiac transplantation: a 2,5 year follow up.
of the intact mammalian heart. Arch Surg 26:219–224 Transplant Proc 15:2546
Matthews AM (1998) The development of the Starr- Parsonnet V, Zucker IR, Gilbert L et al. (1962) An intracar-
Edwards heart valve. Tex Heart Inst J 25:282–293 diac bipolar electrode for interim treatment of com-
Maydl K (1892) Über die Intubation des Larynx als Mittel plete heart block. Am J Cardiol 10:261
gegen das Einfließen von Blut in die Respirationsorgane Parsons W, Purks WK (1937) Total thyroidectomy for heart
bei Operationen. Wien Med Wochenschr 43:102 disease. Ann Surg 105:722
McGoon D (1976) Valvular replacement and ventricular Pesenti A, Gattinoni L, Bombino M (1993) Long term extra-
function. J Thorac Cardiovasc Surg 72:225–263 corporeal respiratory support: 20 years of progress. Int
McLean J (1916) The thromboplatic action of cephalin. Am Crit Care Dig 12:15
J Physiol 41:250 Pluth JR (1991) The Starr valve revisited. Ann Thorac Surg
Melrose DG (1953) A mechanical heart-lung for use in man. 51:333–334
Br Med J 2:57–66 Portner PM, Oyer PE, McGregor CG et al. (1985) First human
Melrose DG, Dreyer B, Bentall BB et al. (1955) Elective cardiac use of an electrically powered implantable assist sys-
arrest: preliminary communication. Lancet 2:21–22 tem. (Abstract). Artif Organs 9:36
Meltzer SJ (1910) Die Methode der intratrachealen Reed GE, Tice DA, Claus RH (1965) A symmetric, exaggerated
Insufflation, ihr wissenschaftliches Prinzip und ihre mitral annuloplasty: repair of mitral insufficiency with
praktische Anwendung. Berl Klin Wschr 47:566 hemodynamic predictability. J Thorac Cardiovasc Surg
Meyer AW (1928) Eine weitere erfolgreiche 49:752
Trendelenburgsche Lungen-Embolie-Operation. Arch Rehn L (1897) Über Herzwunden und Herznaht. Verh 26
Klin Chir 152:97–98 Kongr Dtsch Ges Chir I: 7277; II: 151–165
Meyer AW (1931) Eine weitere erfolgreiche Rehn L (1913) Zur experimentellen Pathologie des Herzens.
Trendelenburgsche Lungen-Embolie-Operation. Arch Verh 42 Kongr Dtsch Ges Chir II: 339
Klin Chir 167:136–137 Reitz BA, Burton NA, Jamieson SW et al. (1980) Heart and
Mirowski M, Reid PR, Mower MM et al. (1980) Termination lung transplantation: autotransplantation and allo-
of malignant ventricular arrhythmias with an transplantation in primates with extended survival.
implanted automatic defibrillator in human beings. N J Thorac Cariovasc Surg 80:360–372
Engl J Med 303:322 Reitz BA, Wallwork JL, Hunt SA et al. (1982) Heart-Lung
Moore FD, Folkman J (1995) Robert Edward Gross July 2, transplantation: successful therapy for patients with
1905–October 11, 1988. A biographical memoir. pulmonary vascular disease. N Engl J Med 306:557
National Academy of Sciences p. 131–149. National Ricketts (1903) Injuries of the heart. N Y Med J 77:993
Academies Press, Washington D.C. Ross DN (1962) Homograft replacement of the aortic valve.
Moore SL, Maloney JD, Edel TB et al. (1991) Implantable Lancet 2:487
cardioverter defribrillator implanted by nonthoracot- Ross DN (1967) Replacement of aortic and mitral valves
omy approach: initial clinical experience with the with a pulmonary autograft. Lancet 2:956
redesigned transvenous lead system. PACE 14:1865 Rukosujew A, Fugmann M, Scheld HH (2007) Die extrakor-
Moulopoulos SD, Topaz S, Kolff WJ (1962) Diastolic balloon porale Zirkulation: Historische Aspekte der Entwicklung-
pumping in the aorta- a mechanical assistance to the Teil I. Thorac Cardiovasc Surg 55:279–283
failing circulation. Am Heart J 63:669 Rygg IH, Kyvsgaard E (1958) A disposable polyethylene
Mueller U (2007) Herznaht wider ethische Bedenken. oxygenator system applied in heart-lung machine.
Dtsch Ärztebl 104:A26–A28 Acta Chir Scand 112:433–437
30 K.H. Leitz and G. Ziemer
Sauerbruch F (1904a) Über die physiologischen und physi- Siposs G (1989) Memoirs of an early heart-valve engineer.
1 kalischen Grundlagen bei intrathorakalen Eingriffen in
meiner pneumatischen Operationskammer. Verh
Ann Thorac Surg 48:S6–S7
Smeloff EA (1989) Comparative study of heart valve design
Dtsch Ges Chir 33 II:105–115 in the 1960s. Ann Thorac Surg 48:S31–S32
Sauerbruch F (1904b) Über die Ausschaltung der schädli- Smithy H, Boone JA, Stallworth JM (1950) Surgical treatment
chen Wirkung des Pneumothorax bei intrathorakalen of constrictive valvular disease of heart. Surg Gynecol
Operationen. Zbl Chir 31:146–149 Obstet 90:175–192
Sauerbruch F (1904c) Zur Pathologie des offenen Sondergaard T, Senn A (1967) Klinische Erfahrungen in der
Pneumothorax und die Grundlagen meines Verfahrens Kardioplegie nach Bretschneider. Langenbecks Arch
zu seiner Ausschaltung. Mitteil a d Grenzgeb d Med u Chir 319:661
Chir 13:399–482 Sones FM, Shirey EK (1962) Cine coronary arteriography.
Sauerbruch F (1907) Die Verwendbarkeit des Unterdruc- Mod Concepts Cardiovasc Dis 31:735–738
kverfahrens in der Herzchirurgie. Verh Dtsch Ges Chir 26 Souttar H (1925) Surgical treatment of mitral stenosis. Br
II:245–253 Med J 2:603
Sauerbruch F (1924) Der Stand der Chirurgie der Brustorgane Spencer FC (1983) Intellectual creativity in thoracic sur-
auf Grund der Entwicklung in den letzten 20 Jahren. geons. J Thorac Cardiovasc Surg 86:163–179
Verh 48 Kongr Dtsch Ges Chir I: 9–29; II: 277–311 Starr A, Edwards L (1961) Mitral replacement: clinical experi-
Sauerbruch F (1931) Erfolgreiche operative Beseitigung ence with a ball valve prosthesis. Ann Surg 154:726–740
eines Aneurysmas der rechten Kammer. Arch Klin Chir Starr A, Edwards L, McCord et al. (1963) Aortic replace-
167:586 ment. Circulation 27:779
Sauerbruch F, Schumacher ED (eds) (1911) Die Technik der Stephenson LW (1997) History of cardiac surgery. In:
Thoraxchirurgie. Springer, Berlin Edmunds LH (ed) Cardiac surgery in the adult. Mc
Schmid C, Schmitto JD, Scheld HH (2003) Herztransplantation Graw Hill, New York, pp 3–33
in Deutschland, ein geschichtlicher Überblick. Subramanian VA (1997) Less invasive arterial CABC on a
Steinkopff, Darmstadt beating heart. Ann Thorac Surg 63:68–71
Schmieden V (1931) Nekrolog auf Ludwig Rehn. Verh 55 Swan HC, Maaske M, Johnson M et al. (1950) Arterial homo-
Kongr Dtsch Ges Chir I: 4 grafts: resection of thoracic aneurysm using a stored
Schober KL (1981) The quotation about the heart. human arterial transplant. Arch Surg 61:732
Comments on the Billroths attitude towards cardiac Swan H, Zeavin I, Blount SG, Virtue RW (1953) Surgery by
surgery. Thorac Cardiovasc Surg 29:131 direct vision in the open heart during hypothermia.
Schober KL (1993) Wege und Umwege zum Herzen. Thorac JAMA 153:1081
Cardiovasc Surg 41(SuppI I):1–221 Thiede A, Müller-Hermelik HK, Xankah AC et al. (1971)
Schulte HD (2001) A reflection on the life and legacy of Ernst Strukturelle Veränderungen von Herzklappen aus
Derra on his 100. birthday. Thorac Cardiovasc Surg autologer Fascia lata. Thoraxchirurgie 19:386
49:251–253 Thomas FT, Szentpetery SS, Mammana RE et al. (1978)
Schumacher ED (1913) Beiträge zur operativen Behandlung Long distance transportation of human hearts for
der Lungenembolie. Verh 42. Kongr Dtsch Ges Chir II: transplantation. Ann Thorac Surg 26:346
169–183 Trendelenburg F (1871) Beiträge zu den Operationen an
Sealy WC, Brown IW, Young WG (1958) Report on the use of den Luftwegen. Tamponade der Trachea. Arch Klin
both extracorporeal circulation and hypothermia for Chir 12:121–133
open heart surgery. Ann Surg 147:603613 Trendelenburg F (1908) Zur Operation der Embolie der
Sealy WC, Brown IW, Young WG et al. (1959) Hypothermia Lungenarterien. Zentralbl Chir 35:92
and extracorporeal circulation for open heart surgery. Tuffier T (1914) Etat actuel de la chirurgie intrathoracique.
Its simplification with a heat exchanger tor rapid cool- Trans internat congress med 1913 London, section VII,
ing and rewarming. Ann Surg 150:627–638 surgery, pp 247–327
Senning A (1952) Ventricular fibrillation during extracorpo- Vaubel E (1980) Ernst Jeger. Gefäßchirurg vor und in dem
real circulation used as a method to prevent air- Ersten Weltkrieg. Mitt Dtsch Ges Chir 1:17
embolism and to facilitate intracardiac operations. Acta Vineberg AM (1946) Development of anastomosis
Chir Scand Suppl 171:1–79 between coronary vessels and transplanted internal
Senning A (1954) Extracorporeal circulation combined with mammary artery. Canad M Ass J 55:117
hypothermia. Acta Chir Scand 107:516 Vineberg AM (1958) Coronary vascular anastomosis by
Senning A (1959) Strip-graft technique. Acta Chir Scand internal mammary implantation. Canad M Ass J 78:871
118:81–85 Vineberg A, Buller W (1955) Technical factors which favor
Senning A (1967) Fascia lata replacement of aortic valves. mammary-coronary anastomosis. J Thorac Surg 30:411
J Thorac Cardiovasc Surg 54:465 Voorhees AB, Janetzky A, Blakemore AH (1952) The use of
Shumacker HB (1947) Surgical cure of innominate aneu- tubes constructed from Vinyon “N” cloth in bridging
rysm: report of a case with comments on the applicabil- defects. Ann Surg 135:332
ity of measures. Surgery 22:739 Wachsmuth W (1985a) Ein Leben mit dem Jahrhundert.
Shumway NE, Lower RR, Stofer RC (1959) Selective hypo- Springer, Berlin
thermia of the heart in anoxic cardiac arrest. Surg Wachsmuth W (1985b) Reden und Aufsätze 1930–1984.
Gynecol Obstet 104:750 Springer, Berlin
Chapter 1 · The History of Cardiac Surgery
31 1
Wada J, Komatsu S, Teruhisa K (1989) Wada-Cutter heart Zapol WM, Snider MT, Hill JD (1979) Extracorporeal mem-
valve: overall experience at the Sapporno Medical brane oxygenation in severe respiratory failure. JAMA
College. Ann Thorac Surg 48(S):38–40 242:2193
Westaby S (1997) Landmarks in cardiac surgery. Isis Ziemer G (1992) Discussion of Ross D, Jackson M, Davies J
Medical Media, Oxford (1992) The pulmonary autograft-a permanent aortic
Wheat MW, Wilson JR, Bartley TD (1964) Successful replace- valve. Eur J Cardiothorac Surg 6:117
ment of the entire ascending aorta and aortic valve. Zoll PM (1952) Resuscitation of the heart in ventricular
JAMA 188:717 standstill by external electric stimulation. N Engl
Wooler GH, Nixon PG, Grimshaw VA et al. (1962) Experiences J Med 247:768
with the repair of the mitral valve in mitral regurgita- Zoll PM, Linenthal AJ (1960) Long-term electric pacemaker
tion. Thorax 17:49 for Stokes-Adams-disease. Circulation 22:341
33 2
2.4 Summary – 63
References – 63
isolated aortic valve replacements, and the combi- 2.2.4 isk Adjustment in Congenital
R
nation of both (n = 66,365 in 2011), as well as Heart Surgery, 1st Version
transplantations of the heart, lungs, and both. The (RACHS-1)
2 institution uses a set of well-defined risk fac-
tors. A random validation of source data was RACHS-1 (Jenkins et al. 2002, 2004) is a score
performed and did not reveal any evidence of that groups most of the procedures performed in
purposeful manipulation of entry of data. The patients with congenital cardiac malformations
most recent data set used to develop regression according to their risk of postoperative in-hospital
models for in-hospital mortality is based on mortality. The developers of RACHS-1 wanted to
patients operated on in 2010. The regression coef- keep the score relatively as simple, with minimal
ficients and the constant of the models derived entry of data that could all be obtained from the
from this huge data set can be downloaded from usual hospital discharge data sets. The developers
the following website hyperlinks: of RACHS-1 used a data set of 3,709 surgical pro-
7 http://www.sqg.de/ergebnisse/leistungsbereiche/ cedures performed in 1996, including patients
koronarchirurgie-isoliert.html from 32 institutions. The procedures were first
7 http://www.sqg.de/ergebnisse/leistungsbereiche/ classified by their estimated mortality risk, and
aortenklappenchirurgie-konventionell.html then the group assignment was adapted to reality.
7 http://www.sqg.de/ergebnisse/leis- Finally, a validation using 3,419 discharge data
tungsbereiche/kombinierte-koronar- sets was performed and confirmed good calibra-
undaortenklappenchirurgie.html tion and discrimination. For 5 of the 79 proce-
dures (. Table 2.2), an age split was introduced.
. Table 2.1 gives the 2011 results for isolated The 79 procedures are divided into six categories.
cardiac surgery based on data of 40,353 such The applicability of this simple scoring system for
operations. dividing patient populations into groups with sig-
Unlike for the EuroSCORE, data for this nificantly differing operative risk has been shown
KCH-SCORE 3.0 model are specific for the repeatedly in various countries (. Table 2.3)
described procedures and come from German RACHS-2, an updated version of RACHS-1,
centers only, so they should me more specific for has not yet been published. In the future, increas-
comparisons with or within Germany. The ing availability of computer power and larger data
AQUA-Institut updates the regression models in sets may lead to the development of procedure-
short intervals, making current factor weights specific mortality prediction models based on the
available. When using these models outside primary procedure of the index operation carried
Germany, it is advisable to consider the usual out during a hospital stay. Such a procedure-
postoperative length of hospital stay in German specific mortality prediction model may be appli-
hospitals (isolated aortic valve replacement: 5 % cable for common operations but will likely not
up to 7 days, 56.5 % 8–14 days, 22.2 % 15–21 days, obviate the need for a grouping process such as
15.8 % >21 days; isolated CABG: 9.5 % up to RACHS-1 to deal with the multiple rarer opera-
7 days, 61.7 % 8–14 days, 17.6 % 15–21 days, tions. The large databases for congenital heart
11.2 % >21 days; CABG plus aortic valve replace- surgery (STS Congenital Heart Surgery Database
ment: 6.0 % up to 7 days, 48.2 % 8–14 days, 25.2 % and European Association for Cardio-Thoracic
15–21 days, 20.5 % >21 days). Surgery [EACTS] Congenital Heart Surgery
Database) can provide the large numbers required
for both procedure-specific mortality prediction
model and complexity stratified models.
The KCH-SCORE 3.0 is very useful to compare
results of isolated coronary artery surgery,
aortic valve replacement, or both of them to
actual expectations in Germany. The RACHS-1 is suited to group pediatric cardiac
KCH-SCORE 3.0 is best used to compare surgery patients according to their risk of
outcomes with or within Germany. mortality after cardiac surgery.
Chapter 2 · Risk Scores in Cardiac Surgery
37 2
..Table 2.1 Risk-adjusted in-hospital mortality according to the log. (KCH-SCORE 3.0)
(Constant) – −5.7503853602647300 = β0
71–75 years 0.6471051658100260 = β2
76–80 years 0.8592868665625700
80–85 years 1.2456627767548900
>35 0.4181980662086110 = β5
30–50 % 0.5526803957590370 = β12
Add the constant (in this case: −5.7503853602647300), name the result «R,» and replace «R» with the result in the
following formula:
2.718259R/ (1+2.718259R)
4 Aortic valvotomy or valvuloplasty at age The products are added, and their sum indicates the
≤30 days number of expected deaths. This number can then
be expressed as percentage of the entire group of
Konno procedure operated patients: the mortality rate expected if
Repair of complex anomaly (single ventricle) mortalities were equal in the observed and the
by ventricular septal defect enlargement reference population
Repair of total anomalous pulmonary veins Comparison: The quotient of observed and
at age ≤30 days expected mortality rates (in %) shows the
relationship of observed and expected mortality.
Atrial septectomy Another intuitive measure of comparison is to
indicate the mortality rate difference: it shows how
Repair of transposition, ventricular septal
many lives per 100 were saved (or lost) in one
defect, and subpulmonary stenosis (Rastelli)
group (hospital, country) in respect to the
Atrial switch operation with ventricular reference group
septal defect closure
(From Jenkins et al. (2002), Jenkins (2004); used
Atrial switch operation with repair of with permission)
subpulmonary stenosis
..Table 2.3 Various published mortality rates with RACHS-1 stratified RACHS-1 groups
Literature Population Treatment Operations Mortality [%] in the different RACHS-1 categories
period
1 2 3 4 5 6
Jenkins et al. (2002) PCCC, USA 1996 4370 0.4 3.8 8.5 19.4 – 47.7
Boethig et al. (2004) Bad Oeynhausen, Germany 1996–2002 2386 0.3 4.0 5.6 9.9 50.0 40.1
D. Boethig and J.P. Jacobs
Larsen et al. (2005) Aarhus, Denmark 1996–2002 957 1.1 3.1 8.5 17.0 – 57.0
Kang et al. (2004) London, England 200–2003 998 0 1.3 5.0 11.1 – 36.5
Welke et al. (2006) CHSS members 2001–2004 12.672 0.7 0.9 2.7 7.7 – 17.2
Al-Radi et al. (2007) Toronto, Canada 1999–2004 2397 0 1.0 4.0 7.0 17.0a 17.0a
Nina et al. (2007) Maranhao, Brazil 2001–2004 145 3.8 26.0 60.0 – – –
Larrazabal et al. (2007) Guatemala City, Guatemala 2003–2004 537 0.5 7.4 23.3 25.0 – –
Mildh et al. (2007) Helsinki, Finland 2000–2004 1001 0 2.1 3.2 9.7 – 14.3
Padley et al. (2011) Sydney, Australia 2005–2010 1745 0 1.1 1.6 6.2 0 25.8
Pasquali et al. (2010) 38 USA centers 2003–2008 46.730 0.8 1.3 2.7 7.0 17.1a 17.1a
Vijarnsorn et al. (2011) Bangkok, Thailand 2005 230 0 1.7 11.1 25 100 100
Bojan et al. (2007) Paris, France 2007–2009 1384 0 1.7 3.0 7.1 100 57.9
Magliola et al. (2011) Buenos Aires, Argentina 2004–2009 2942 0.4 2.4 7.1 14.0 34 34
CHSS Congenital Heart Surgeons Society, HDD Hospital Discharge Data Sets, PCCC Pediatric Cardiac Care Consortium, RACHS-1 Risk Adjustment in Congenital Heart Surgery,
1st version
aRACHS-1 categories 5 and 6 were combined to calculate the mortality rate
Chapter 2 · Risk Scores in Cardiac Surgery
41 2
year 350 BCE, in Rhetoric, Book 1, Aristotle stated, from complex procedures, such as Sinzobahamvya
«When there is no scientific answer available, the et al. (2006), Miyamoto et al. (2005), Artrip et al.
opinion (Doxa) perceived and admitted by the (2006), and Derby et al. (2007).
majority has the value of truth.»
Lacour-Gayet and the Aristotle Committee
have differentiated the concepts of complexity and The Aristotle Score is suited to group
risk and have stated, «Complexity is a constant pediatric cardiac surgery patients according
precise value for a given patient at a given point in to their risk of mortality after cardiac surgery.
time; performance varies between centers and
surgeons. In other words, in the same exact patient
with the same exact pathology, complexity is a
constant precise value for that given patient at a 2.2.6 he STS–EACTS Congenital
T
given point in time. The risk for that patient will Heart Surgery Mortality Score
vary between centers and surgeons because per- (STAT Mortality Score)
formance varies between centers and surgeons.»
Under the leadership of Lacour-Gayet and the First published in 2009 by O’Brien et al., the
Aristotle Committee, the Aristotle Basic Complexity STS–EACTS Mortality Score (later abbreviated as
Score was developed by a panel of experts, made up STAT Mortality Score: Society of Thoracic
of 50 surgeons who repair congenital cardiac Surgeons – European Association for Cardio-
defects in 23 countries and represent several major Thoracic Surgery Congenital Heart Surgery
professional societies. The Aristotle Basic Mortality Score) was derived from 77,294 con-
Complexity Score allocates a basic score to each genital heart surgery data sets. They were pooled
operation, varying from 1.5 to 15, with 15 being the from the databases of STS and EACTS, collected
most complex, based on the primary procedure of a between 2002 and 2007, and describe outcomes of
given operation. The Aristotle Basic Complexity 148 different operations. Similar to RACHS-1,
Score represents the sum or aggregate of scores only the most complex component procedure of a
assigned to a given procedure for the three compo- given operation is considered (Jacobs et al. 2009b).
nents of complexity—potential for mortality, To create the STAT Mortality Score, at first the
potential for morbidity, and technical difficulty— observed hospital discharge mortality rates were
each of which varies from 0.5 to 5. To facilitate adjusted by approaching the mortality rates of
analysis across large populations of patients, each rarely encountered procedures to the average mor-
procedure is then assigned an Aristotle Basic tality, in order to limit over- or underestimation by
Complexity Level, which is an integer ranging from chance. The applied Bayesian algorithm modified
1 to 4 based on the Aristotle Basic Complexity the observed rates stronger if the total case number
Score (. Table 2.4) (O’Brien et al. 2007). was lower. Then, the adjusted mortality rates were
The Aristotle Comprehensive Complexity transformed by shifting and rescaling, in order to
Score adds further discrimination to the Basic fit into a scale ranging from 0.1 to 5. The result is
Score by incorporating two sorts of patient-specific the «STS–EACTS Congenital Heart Surgery
complexity modifiers: (1) procedure- dependent Mortality Score (O’Brien et al. 2009).»
factors, including anatomic factors, associated In a further step, these score values were grouped
procedures, and age at procedure, and (2) proce- into the five «STS–EACTS Congenital Heart
dure-independent factors, including general fac- Surgery Mortality Categories (STAT Mortality
tors such as weight and prematurity, clinical factors Categories).» The comparative validation on 25,106
such as preoperative sepsis or renal failure, extra- data sets collected (2007–2008) from the same data-
cardiac factors such as duodenal atresia and base showed optimal discrimination of the STAT
imperforate anus, and surgical factors such as Mortality Score for the procedure-specific mortality
reoperative sternotomy. Additional points, up to a rates (C-index = 0.787), followed by the STAT
maximum of 10, are added to the Basic Score to Mortality Categories (C-index = 0.778), then the
account for the added complexity and challenge RACHS-1 categories (C-index = 0.745), and then
imputed by these modifying factors. The Aristotle the Aristotle Basic Score (C-index = 0.687). Addition
Comprehensive Complexity Score has been used of patient-specific variables improved the C-indexes
by numerous investigators to analyze the outcomes to 0.816, 0.812, 0.802, and 0.795, respectively.
42 D. Boethig and J.P. Jacobs
..Table 2.4 The Aristotle basic complexity score (ABC score) and the Aristotle basic complexity levels
(ABC levels) (January 1, 2010)
Complexity
1.5–5.9 1
6.0–7.9 2
8.0–9.9 3
10.0–15.0 4
Total Complexity
(Basic
Procedures (Basic score) Mortality Morbidity Difficulty
level)
Pleural drainage procedure 1.5 1 0.5 0.5 0.5
Bronchoscopy 1.5 1 0.5 0.5 0.5
Delayed sternal closure 1.5 1 0.5 0.5 0.5
Mediastinal exploration 1 0.5 0.5 0.5
Sternotomy wound drainage 1.5 1 0.5 0.5 0.5
Intra-aortic balloon pump (IABP)
2.0 1 0.5 1.0 0.5
insertion
Explantation of pacing system 2.5 1 1.0 1.0 0.5
PFO, primary closure 3.0 1 1.0 1.0 1.0
ASD repair, primary closure 3.0 1 1.0 1.0 1.0
ASD repair, patch 3.0 1 1.0 1.0 1.0
ASD partial closure 3.0 1 1.0 1.0 1.0
Atrial fenestration closure 3.0 1 1.0 1.0 1.0
Pericardial drainage procedure 3.0 1 1.0 1.0 1.0
PDA closure, surgical 3.0 1 1.0 1.0 1.0
Pacemaker implantation, 3.0 1 1.0 1.0 1.0
permanent
Pacemaker procedure 3.0 1 1.0 1.0 1.0
Shunt, ligation and takedown 3.5 1 1.5 1.0 1.0
ASD, common atrium (single
3.8 1 1.0 1.0 1.8
atrium), Septation
AVC (AVSD) repair, partial
4.0 1 1.0 1.0 2.0
(incomplete) (PAVSD)
Chapter 2 · Risk Scores in Cardiac Surgery
43 2
. Table 2.4 (continued)
Total Complexity
(Basic
Procedures (Basic score) Mortality Morbidity Difficulty
level)
Coronary artery fistula ligation 4.0 1 1.0 2.0 1.0
Aortopexy 4.0 1 1.5 1.5 1.0
ICD (AICD) implantation 4.0 1 1.5 1.0 1.5
ICD (AICD) (automatic implantable
4.0 1 1.5 1.0 1.5
cardioverter defibrillator) procedure
Ligation, thoracic duct 4.0 1 1.0 2.0 1.0
Diaphragm plication 4.0 1 1.0 2.0 1.0
ECMO decannulation 4.0 1 2.0 1.0 1.0
ASD creation/enlargement 5.0 1 2.0 2.0 1.0
Atrial septal fenestration 5.0 1 2.0 2.0 1.0
AVC (AVSD) repair, intermediate
5.0 1 1.5 1.5 2.0
(transitional)
PAPVC repair 5.0 1 2.0 1.0 2.0
Lung biopsy 5.0 1 1.5 2.0 1.5
Ligation, pulmonary artery 5.0 1 1.5 2.0 1.5
Decortication 5.0 1 1.0 1.0 3.0
ASD repair, patch + PAPVC repair 5.0 1 2.0 1.0 2.0
PAPVC repair, baffle redirection
to the left atrium with systemic vein
translocation (Warden) (SVC sewn 5.0 1 1.0 2.0 2.0
to the right atrial appendage)
ECMO cannulation 5.0 1 2.0 1.0 2.0
Pectus repair 5.3 1 2.0 1.0 2.3
Aortic stenosis, supravalvar, repair 5.5 1 1.5 2.0 2.0
Valvuloplasty, pulmonic 5.6 1 1.8 1.8 2.0
VSD repair, primary closure 6.0 2 2.0 2.0 2.0
VSD repair, patch 6.0 2 2.0 2.0 2.0
AP window repair 6.0 2 2.0 2.0 2.0
Valve replacement, truncal valve 6.0 2 2.0 2.0 2.0
Cor triatriatum repair 6.0 2 2.0 2.0 2.0
Valve excision, tricuspid (without
6.0 2 2.0 2.0 2.0
replacement)
PA, reconstruction (plasty), main
6.0 2 2.0 2.0 2.0
(trunk)
Pericardiectomy 6.0 2 2.0 2.0 2.0
Coarctation repair, end to end 6.0 2 2.0 2.0 2.0
Coarctation repair, subclavian flap 6.0 2 2.0 2.0 2.0
Coarctation repair, patch aortoplasty 6.0 2 2.0 2.0 2.0
Vascular ring repair 6.0 2 2.0 2.0 2.0
(continued)
44 D. Boethig and J.P. Jacobs
Total Complexity
2 (Basic
Procedures (Basic score) Mortality Morbidity Difficulty
level)
PA banding (PAB) 6.0 2 2.0 2.0 2.0
PA debanding 6.0 2 2.0 2.0 2.0
ECMO procedure 6.0 2 2.0 3.0 1.0
Aortic stenosis, subvalvar, repair 6.3 2 2.0 1.8 2.5
Shunt, systemic to pulmonary,
modified Blalock–Taussig shunt 6.3 2 2.0 2.0 2.3
(MBTS)
RVOT procedure 6.5 2 2.0 2.0 2.5
Valve replacement, pulmonic (PVR) 6.5 2 2.0 2.0 2.5
Shunt, systemic to pulmonary,
central (From the aorta or to the 6.8 2 2.0 2.0 2.8
main pulmonary artery)
Valvuloplasty, truncal valve 7.0 2 2.0 2.0 3.0
Anomalous systemic venous
connection repair 7.0 2 2.0 2.0 3.0
Total Complexity
(Basic
Procedures (Basic score) Mortality Morbidity Difficulty
level)
Conduit placement, RV to PA 7.5 2 2.5 2.0 3.0
Sinus of Valsalva, aneurysm repair 7.5 2 2.5 2.0 3.0
Valve replacement, mitral (MVR) 7.5 2 2.5 2.0 3.0
Coronary artery bypass 7.5 2 2.5 2.0 3.0
Bilateral bidirectional
cavopulmonary anastomosis 7.5 2 2.5 2.0 3.0
(BBDCPA) (bilateral bidirectional Glenn)
Conduit placement, Other 7.5 2 2.5 2.0 3.0
Hybrid approach "Stage 1",
Application of RPA and LPA bands 7.5 2 2.5 2.5 2.5
Arrhythmia surgery—atrial,
surgical ablation 8.0 3 3.0 2.0 3.0
Arrhythmia surgery—ventricular,
surgical ablation 8.0 3 3.0 2.0 3.0
(continued)
46 D. Boethig and J.P. Jacobs
Total Complexity
2 (Basic
Procedures (Basic score) Mortality Morbidity Difficulty
level)
Pulmonary embolectomy 8.0 3 3.0 3.0 2.0
Pulmonary embolectomy, acute
pulmonary embolus 8.0 3 3.0 3.0 2.0
Total Complexity
(Basic
Procedures (Basic score) Mortality Morbidity Difficulty
level)
Congenitally corrected TGA repair,
VSD closure 9.0 3 3.0 3.0 3.0
(continued)
48 D. Boethig and J.P. Jacobs
Total Complexity
2 (Basic
Procedures (Basic score) Mortality Morbidity Difficulty
level)
Hybrid Approach “Stage 2”,
aortopulmonary amalgamation +
superior cavopulmonary 10.0 4 2.5 3.5 4.0
anastomosis(es) + PA debanding +
without aortic arch repair
Hybrid approach “Stage 1”, stent
placement in arterial duct (PDA) + 10.0 4 3.0 3.0 4.0
application of RPA and LPA bands
Valve replacement, common
atrioventricular valve 10.0 4 3.5 3.5 3.0
Ross procedure 10.3 4 4.0 2.3 4.0
DORV, Intraventricular tunnel
repair 10.3 4 3.3 3.0 4.0
Valvuloplasty converted to valve
replacement in the same 10.3 4 3.5 2.5 4.3
operation, aortic
Ventricular septation 10.5 4 3.5 3.5 3.5
Valvuloplasty converted to valve
replacement in the same 10.5 4 4.0 2.5 4.0
operation, mitral
Interrupted aortic arch repair 10.8 4 3.8 3.0 4.0
Truncus arteriosus repair 11.0 4 4.0 3.0 4.0
TOF–AVC (AVSD) repair 11.0 4 4.0 3.0 4.0
Pulmonary atresia–VSD–
MAPCA (pseudotruncus) repair 11.0 4 4.0 3.0 4.0
Total Complexity
(Basic
Procedures (Basic score) Mortality Morbidity Difficulty
level)
Partial left ventriculectomy (LV
volume reduction surgery) (Batista) 12.0 4 4.0 4.0 4.0
(continued)
50 D. Boethig and J.P. Jacobs
Total Complexity
2 (Basic
Procedures (Basic score) Mortality Morbidity Difficulty
level)
Total Complexity
(Basic
Procedures (Basic score) Mortality Morbidity Difficulty
level)
Cardiovascular catheterization
procedure, therapeutic, stent redilation
Cardiovascular catheterization procedure,
therapeutic, perforation (establishing
interchamber and/or intervessel
communication)
Cardiovascular catheterization procedure,
therapeutic, transcatheter Fontan
completion
Cardiovascular catheterization procedure,
therapeutic, transcatheter implantation
of valve
Cardiovascular catheterization
procedure, therapeutic adjunctive therapy
Cardiovascular electrophysiolog
catheterization procedure
Cardiovascular electrophysiolog
catheterization procedure, therapeutic
ablation
Other miscellaneous, not scored:
(Either too vague or not a primary
procedure)
Atrial baffle procedure, NOS
Tracheal procedure
(continued)
52 D. Boethig and J.P. Jacobs
Total Complexity
2 (Basic
Procedures (Basic score) Mortality Morbidity Difficulty
level)
Peripheral vascular procedure, other
Pericardial procedure, other
PDA closure, NOS
Palliation, other
PA, reconstruction (plasty), NOS
Other
Organ procurement
Miscellaneous procedure, other
Mediastinal procedure
Fontan, TCPC, lateral tunnel, NOS
Fontan, other
Fontan, NOS
Esophageal procedure
DORV repair, NOS
Diaphragm procedure, other
Coronary artery procedure, other
Congenitally corrected TGA repair,
other
Congenitally corrected TGA repair, NOS
Conduit placement, NOS
Coarctation repair, other
Coarctation repair, NOS
Cardiotomy, other
Cardiac procedure, Other
AVC (AVSD) repair, NOS
ASD repair, NOS
Arrhythmia surgery, NOS
Other anular enlargement procedure
Fontan, TCPC, external conduit, NOS
VATS (video-assisted thoracoscopic
surgery)
Minimally invasive procedure
Bypass for non-cardiac lesion
Valve replacement, aortic
Chapter 2 · Risk Scores in Cardiac Surgery
53 2
This classification had been also applied to 111.494 often left to some leader’s gut feelings; however,
pooled data sets from the STS Congenital Heart scores are an alternative and more objective
Surgery Database and the EACTS Congenital Heart methodology of outcome research (Jacobs et al.
Surgery Database for patients operated between 2005). A comprehensive comparison of risk-
2006 and 2009 (Jacobs et al. 2012). adjusted, expected, and observed results informs
In contrast to RACHS-1 and the Aristotle about the current state and its development, both
Score, the STAT Mortality Score and the STAT before and after implementation of structural or
Mortality Categories (O’Brien et al. 2009) are not procedural changes. Scores address a broad vari-
based primarily on expert opinion, but are instead ety of end points and are quite helpful for such
based on objective data (77,294 observations) and endeavors (Jacobs 2008).
their exact evaluation.
Using methodology similar to that used to
develop the STAT Mortality Score and the STAT 2.3.2 Quality Monitoring
Mortality Categories, the STAT Morbidity
Categories have recently been developed to facili- Differences between expected and achieved
tate grouping pediatric and congenital cardiac results are an appropriate tool to assess quality
operations into five categories associated with development. Professor Paul T. Sergeant explains
operative morbidity (Jacobs et al. 2013). very clearly how to deal with such expectations
The role of preoperative morbidities as well as and their fulfillment, using the analogy of a bank
of concomitant procedures is currently being ana- account (Sergeant et al. 2001):
lyzed by the STS and EACTS Congenital Heart
Surgery Databases (. Table 2.5). »» A successfully performed procedure adds a
certain sum to the surgeon’s «life bank
account». The sum equals the average risk of
mortality of the patient. If the procedure had
The STS–EACTS Congenital Heart Surgery
a mortality risk of 20% and the patient sur-
Mortality Score (O’Brien et al. 2009) (STAT
vived, the surgeon gains 1/5 life on his
Mortality Score) is a grouping system for
account. In contrary, if the surgeon loses one
pediatric and congenital cardiac surgery
of such patients, the probability of survival
mortality comparisons that encompasses a
(80%) gets withdrawn from his account. So,
wide range of surgical procedures. The tool is
if one of 5 patients with a 20% risk of mortal-
contemporary, observation based, validated,
ity dies and four survive, the surgeon has
and simple to use.
saved up 0.8 lives and withdraws 0.8 for the
lost patient from his account. His perfor-
mance corresponds to the average of the
reference population. Using this method,
2.3 Appropriate Use of Risk Scores patients with inhomogeneous risk can be
summarized objectively, just by adding the
. Table 2.6 summarizes the appropriate and inap-
risks of mortality of the survivors and sub-
propriate use of risk scores. These topics are then
tracting the probabilities of survival of the
discussed in more detail.
patients that die.
..Table 2.5 STAT mortality score and the STAT mortality categories
Procedure Mortality
2 Score Category Unadjusteda Model basedb
AVC (AVSD) repair, partial (incomplete) 0.1 1 0.3 % (0.1–0.8 %) 0.5 % (0.2–0.9 %)
(PAVSD)
ASD repair, patch + PAPCV repair 0.2 1 0.2 % (0.0–1.3 %) 0.6 % (0.2–1.4 %)
Congenitally corrected TGA repair, VSD 0.5 2 0.0 % (0.0–16.1 %) 2.6 % (0.3–8.8 %)
closure
ASD, common atrium (single atrium), 0.6 2 2.3 % (0.1–12.0 %) 3.1 % (0.5–8.3 %)
septation
Aortic root replacement, valve sparing 0.6 2 2.7 % (0.1–14.2 %) 3.4 % (0.6–9.2 %)
Partial left ventriculectomy (LV volume 0.7 2 3.8 % (0.1–19.6 %) 4.1 % (0.7–11.3 %)
reduction surgery; Batista)
Arterial switch procedure + aortic arch 1.4 4 11.1 % (1.4–34.7 %) 8.0 % (1.7–20.6 %)
repair
(continued)
58 D. Boethig and J.P. Jacobs
Aortic arch repair + VSD repair 1.7 4 10.1 % (7.1–13.8 %) 9.8 % (6.9–13.1 %)
Congenitally corrected TGA repair, VSD 1.7 4 16.7 % (2.1–48.4 %) 10.1 % (2.0–25.9 %)
closure and LV to PA conduit
Arterial switch procedure and VSD 2.4 4 15.0 % (9.0–23.0 %) 14.0 % (8.5–20.5 %)
repair + aortic arch repair
Procedures and their mortality scores, categories, rates as observed, and «smoothed» model based rates – accord-
ing to the STS–EACTS scoring system
ASD Atrial septal defect, AVC atrioventricular canal, AVSD atrioventricular septal defect, PAVSD partial atrioven-
tricular septal defect, PAPVC partial anomalous pulmonary venous connection, ICD implantable cardioverter defi-
brillator, AICD automatic implantable cardioverter defibrillator, DCRV double-chambered right ventricle, VSD
ventricular septal defect, PFO patent foramen ovale, AVR aortic valve replacement, PVR pulmonary valve replace-
ment, TOF tetralogy of Fallot, TCPC total cavopulmonary connection, PDA patent ductus arteriosus, PA pulmonary
artery, LV left ventricle, RVOT right ventricular outflow tract, TGA transposition of the great arteries, AP aortopul-
monary, TVR tricuspid valve replacement, RV right ventricle, MAPCA major aortopulmonary collateral artery, ASO
arterial switch operation, REV reparation a` l’e´tage ventriculaire (REV procedure), MVR mitral valve replacement,
DOLV double-outlet left ventricle, MBTS modified Blalock–Taussig shunt; HLHS, hypoplastic left heart syndrome;
TAPVC, total anomalous pulmonary venous connection, IAA interrupted aortic arch
aDenotes 95 % exact binomial confidence interval
Denotes 95 % Bayesian credible interval (Table 2.5 derives from O’Brien et al. (2009))
Another application of such data would be the Computers are required in order to capture
construction of plots showing the development of all relevant data necessary for complex scores
cumulated risk-adjusted mortality (CRAM plots, with reasonable (van Gameren et al. 2011)
. Fig. 2.1) corresponding to the running total a life effort and reliability. Unfortunately, a com-
bank account on which mortality risks of survi- pletely automatized system of data entry and
vors are deposited and survival chances of nonsur- analysis is currently not freely available. Such a
vivors are withdrawn. Continuous performance completely automatized system of data entry
monitoring, based on any group that has published and analysis would include the following
its data, is made feasible by applying CRAM plots. components:
Confidence limits for such curves can be calcu- 1. A standardized and complete methodology
lated according to Kang (2006). of data collection that would be applicable
Alternative approaches to assessing the per- in the heterogeneous environment of a
formance of hospitals in the context of multi- large number of participating units
institutional performance have been presented for 2. A reliable centralized system of storage
adults with acquired heart disease, using quar- Zof data
terly overall adjustment (Jin et al. 2010), and for 3. The generation of clinically relevant,
congenital cardiac surgery, using funnel plots well-founded, and statistically correct
(Jacobs et al. 2011, 2012b). information from these data
2
60
..Table 2.6 Appropriate use of risk scores and associated potential limitations
Individual prognoses Scores ought to enable the surgeon to objectively Score-based therapy decision-making might promote a shift from being
select the most promising therapy patient centered to literature centered; scores might consider only a subset
of a of a patients’ risk factors
Comparison of therapya Inhomogeneous groups become better comparable The risk of ignoring (locally) important factors grows
Quality assessment (CRAM plots; Kang Early recognition of (un)favorable treatment options Premature assumption of significance; statistical efforts required; possible
D. Boethig and J.P. Jacobs
Effort Recognition of risk bearing or inappropriately In case of already high efficacy of a unit that does not do scoring, the time
expensive treatment options or institutions and money spent for quality or risk assessment (that are detracted from
patient care) are theoretically wasted. However, the act of participating in
a database and benchmarking can lead to further increase in quality even
in a high performing unit
Inadequate score Suboptimal tools are more useful than no tools at all Neglecting of relevant or rare factors; new developments might shift risk
factor weight before being integrated in a new version
Manipulationa Score defects might be circumvented «Upcoding» as well as selection of patients and data might compromise
the treatment of severely ill patients (Epstein 2006; Steinbusch et al. 2007),
obfuscate underperforming units, and distort score development itself
Usefulnessa Personnel and money can be allocated more A general lack of resources cannot be overcome by redistribution
effectively
External vs. internal quality assurancea More objective (knowledge-based) evaluation Risk of inadequate interpretation and reactions
Publication (public reporting)a Large patient numbers might be guided to better In case of manipulations or errors, patients might be misled (www.health.
performing institutions state.ny.us). Risk aversion can develop, but can be prevented with
appropriate risk adjustment
Health politicsa Sanctions can be appropriate, closing (Severe) sanctions might be applied based on scores that disregard
underperforming units can be beneficial for patients important locally specific risk factors
Publicitya Evidence-based information instead of «smart» Information might be selected or difficult to interpret; «evidence» may not
advertising might direct patients to better treatment be absolute truth
Especially in times of restricted funding for an inappropriately high amount is subtracted from
health care, outcomes analysis and quality assess- the «life accounts» of the surgeon and hospital.
ment using scores require a shift of money away Such errors are likely to remain uncompensated.
from patient care toward outcomes analysis and Furthermore, such errors can have increased sig-
quality assessment. Although many diagnoses and nificance and impact in low- volume programs
procedures must be coded anyway at various institu- where patient numbers are small.
tions in order to comply with regulatory require-
ments and generate documentation for billing, every
cent that is needed to fund additional collection and 2.3.4 Manipulation
analysis of data often has to be generated by and
diverted from the direct care of patients. We must Score-based results are at risk to be manipulated.
keep in mind that the results from any work with Such alterations might be triggered by threats with
outcomes analysis and quality assessment using undesired consequences such as closure of depart-
scores must have benefits that outweigh the resources ments or simple comparative publication of results
used to construct and apply them. Otherwise, we can (Epstein 2006; Steinbusch et al. 2007; 7 www.health.
harm patients by wasting their money. state.ny.us). On the other hand, MELD scores used
for liver allocation were reported to have been
intentionally worsened in order to bypass patients
2.3.3 Inadequate Score on the waiting list (7 http://www.aerzteblatt.de/
archiv/128320/Transplantationsskandal-a n-der-
Even the most sophisticated score uses only a part Universitaet-Goettingen-Erschuetterndes-Mass-an-
of the information pertaining to a patient: the ele- Manipulation; 7 http://www.wseas.org/multimedia/
ments of data that have been defined and are journals/biology/2012/54–918.pdf; Sumeet 2010).
included in the score. It is simply not possible to Manipulation of data can influence several
create and use a scoring system that considers all domains: quality improvement, «upcoding,» «out-
possible circumstances and elements of data; such coding,» and selection of patients:
a system would drive the effort of acquisition to an 55 Quality improvement. The ideal consequence
impossible level. Meanwhile, scores that disregard of outcomes analysis and quality assessment is
important factors do not reflect reality well and improvement of the quality of treatment
might lead to distorted conclusions. The risk of (Jacobs et al. 2007). This goal can be achieved
mortality of patients with important disregarded frequently, but often might be difficult to
factors is underestimated, and if the patient dies, reach. Frequently, the necessary measures are
62 D. Boethig and J.P. Jacobs
MJ, Mavroudis C (2005) Initial application in the STS Jacobs ML, O’Brien SM, Jacobs JP, Mavroudis C, Lacour-
congenital database of complexity adjustment to Gayet F, Pasquali SK, Welke K, Pizarro C, Tsai F, Clarke DR
evaluate surgical case mix and results. Ann Thorac (2013) An empirically based tool for analyzing morbid-
Surg 79:1635–1649 ity associated with operations for congenital heart dis-
2 Jacobs JP, Jacobs ML, Mavroudis C, Maruszewski B, ease. Thorac Cardiovasc Surg 145:1046–1057
Tchervenkov CI, Lacour-Gayet FG, Clarke DR, Yeh T, Jacobs JP, Pasquali SK, Austin E. Gaynor JW, Backer C,
Walters HL 3rd, Kurosawa H, Stellin G, Ebels T, Elliott MJ, Romano JCH, Williams WG, Caldarone C, McCrindle BW,
Vener DF, Barach P, Benavidez OJ, Bacha EA (2006a) Graham K, Dokholyan RS, Shook G, Poteat J, Baxi M,
What is operative morbidity? Defining complications Karamlou T, Morris B, Blackstone EH, Mavroudis C,
in a surgical registry database: a report from the STS Mayer, Jr JE, Jonas RA, Jacobs ML (2014) Linking the
congenital database task force and the joint EACTS- congenital heart surgery databases of the Society of
STS Congenital Database Committee. Ann Thorac Surg Thoracic Surgeons (STS) and the Congenital Heart
81:1937–1941 Surgeons’ Society (CHSS): part 1—rationale and
Jacobs ML, Mavroudis C, Jacobs JP, Tchervenkov CI, methodology. World J Pediatr Congenit Heart Surg
Pelletier GJ (2006b) Report of the 2005 STS congenital 5:256–271
heart surgery practice and manpower survey: a report Jenkins KJ (2004) Risk adjustment for congenital heart sur-
from the STS work force on congenital heart surgery. gery: the RACHS-1 method. Semin Thorac Cardiovasc
Ann Thor Surg 82:1152–1158 Surg Pediatr Card Surg Ann 7:180–184
Jacobs JP, Wernovsky G, Elliott MJ (2007) Analysis of out- Jenkins KJ, Gauvreau K, Newburger JW, Spray TL, Moller JH,
comes for congenital cardiac disease: can we do bet- Iezzoni LI (2002) Consensus-based method for risk
ter? Cardiol Young 17(Suppl 2):145–158 adjustment for surgery for congenital heart disease.
Jacobs JP, Cerfolio RJ, Sade RM (2009a) The ethics of trans- Thorac Cardiovasc Surg 123:110–118
parency: publication of cardiothoracic surgical out- Jin R, Furnary AP, Fine SC, Blackstone EH, Grunkemeier GL
comes in the lay press. Ann Thorac Surg 87:679–686 (2010) Using Society of Thoracic Surgeons risk models
Jacobs JP, Jacobs ML, Lacour-Gayet FG, Jenkins KJ, for risk-adjusting cardiac surgery results. Ann Thorac
Gauvreau K, Bacha E, Maruszewski B, Clarke DR, Surg 89:677–682
Tchervenkov CI, Gaynor JW, Spray TL, Stellin G, O’Brien Kang N, Cole T, Tsang V, Elliott M, de Leval M (2004) Risk
SM, Elliott MJ, Mavroudis C (2009b) Stratification of stratification in paediatric open-heart surgery. Eur
complexity improves the utility and accuracy of out- J Cardiothorac Surg 26:3–11
comes analysis in a multi-institutional congenital Kang N, Tsang VT, Gallivan S, Sherlaw-Johnson C, Cole TJ,
heart surgery database: application of the Risk Elliott MJ, de Leval MR (2006) Quality assurance in
Adjustment in Congenital Heart Surgery (RACHS-1) congenital heart surgery. Eur J Cardiothorac Surg
and Aristotle Systems in the Society of Thoracic 29:693–697
Surgeons (STS) Congenital Heart Surgery Database. Lacour-Gayet F (2002) Risk stratification theme for con-
Pediatr Cardiol 30:1117–1130 genital heart surgery. Semin Thorac Cardiovasc Surg
Jacobs ML, Daniel M, Mavroudis C, Morales DLS, Jacobs JP, Pediatr Card Surg Ann 5:148–152
Fraser CD, Turek JW, Mayer JE, Tchervenkov C, Conte JV Lacour-Gayet F, Clarke D, Jacobs J, Comas J, Daebritz S,
(2011) Report of the 2010 Society of Thoracic Surgeons Daenen W, Gaynor W, Hamilton L, Jacobs M,
Congenital Heart Surgery Practice and Manpower Maruszsewski B, Pozzi M, Spray T, Stellin G, Tchervenkov
Survey. Ann Thor Surg 92:76276–76279 C, Mavroudis C, Aristotle Committee (2004a) The
Jacobs JP, Jacobs ML, Maruszewski B, Lacour-Gayet FG, Aristotle score: a complexity-adjusted method to
Tchervenkov CI, Tobota Z, Stellin G, Kurosawa H, evaluate surgical results. Eur J Cardiothorac Surg
Murakami A, Gaynor JW, Pasquali SK, Clarke DR, Austin 25:911–924
EH 3rd, Mavroudis C. (2012a) Initial application in the Lacour-Gayet FG, Clarke D, Jacobs JP, Gaynor JW, Hamilton
EACTS and STS Congenital Heart Surgery Databases of L, Jacobs ML, Maruszewski B, Pozzi M, Spray T,
an empirically derived methodology of complexity Tchervenkov CI, Mavroudis C, the Aristotle Committee
adjustment to evaluate surgical case mix and results. (2004b) The Aristotle score for congenital heart sur-
Eur J Cardiothorac Surg. 42:775–779 gery. Semin Thorac Cardiovasc Surg Pediatr Card Surg
Jacobs JP, O’Brien SM, Pasquali SK, Jacobs ML, Lacour- Ann 7:185–191
Gayet FG, Tchervenkov CI, Austin EH 3rd, Pizarro C, Larrazabal LA, Jenkins KJ, Gauvreau K, Vida VL, Benavidez
Pourmoghadam KK, Scholl FG, Welke KF, Gaynor JW, OJ, Gaitán GA, Garcia F, Castañeda AR (2007)
Clarke DR, Mayer JE Jr, Mavroudis C (2012b) Variation Improvement in congenital heart surgery in a devel-
in outcomes for risk-stratified pediatric cardiac surgi- oping country: the Guatemalan experience.
cal operations: an analysis of the STS Congenital Heart Circulation 23:1882–1887
Surgery Database. Ann Thorac Surg. 94:564–71 Larsen SH, Pedersen J, Jacobsen J, Johnsen SP, Hansen OK,
Jacobs JP, O’Brien SM, Pasquali SK, Jacobs ML, Lacour- Hjortdal V (2005) The RACHS-1 risk categories reflect
Gayet FG, Tchervenkov CI, Austin EH 3rd, Pizarro C, mortality and length of stay in a Danish population of
Pourmoghadam KK, Scholl FG, Welke KF, Mavroudis C. children operated for congenital heart disease. Eur
(2011) Variation in outcomes for benchmark opera- J Cardiothorac Surg 28:877–881
tions: an analysis of the Society of Thoracic Surgeons Lee JW, Um SH, Lee JB, Mun J, Cho H (2006) Scoring and
Congenital Heart Surgery Database. Ann Thorac Surg. staging systems using Cox linear regression modeling
92: 2184–91 and recursive partitioning. Methods Inf Med 45:37–43
Chapter 2 · Risk Scores in Cardiac Surgery
65 2
Magliola RH, Althabe M, Moreno G, Lenz AM, Pilan ML, Pasquali SK, Hall M, Li JS, Peterson ED, Jaggers J, Lodge AJ,
Balestrini M, Charroqui A, Landry LM, Krynski M, Marino BS, Goodman DM, Shah SS (2010)
Salgado G, Martin A, Barretta J, Garcia Delucis P, Corticosteroids and outcome in children undergoing
Cornelis J, Suarez J, Laura JP (2011) Congenital heart congenital heart surgery: analysis of the Pediatric
disease: surgical results in a public hospital in Health Information Systems database. Circulation
Argentina. [Engl. abstr., article in Spanish] Arch Cardiol 122:2123–2130
Mex 81:178–182 Rosenbaum P, Rubin DB (1983) The central role of the pro-
Mavroudis C, Jacobs JP (2000) International congenital pensity score in observational studies for causal
heart surgery nomenclature and database project. effects. Biometrika 70:41–55
Ann Thorac Surg 69(S1):1–372 Sergeant P, de Worm E, Meyns B, Wouters P (2001) The chal-
Mildh L, Pettilä V, Sairanen H, Rautiainen P (2007) Predictive lenge of departmental quality control in the reengi-
value of paediatric risk of mortality score and risk neering towards off-pump coronary artery bypass
adjustment for congenital heart surgery score after grafting. Eur J Cardiothorac Surg 20:538–543
paediatric open-heart surgery. Interact Cardiovasc Shahian DM, Edwards FH, Jacobs JP, Prager RL, Normand
Thorac Surg 6:628–631 SL, Shewan CM, O’Brien SM, Peterson ED, Grover FL
Miyamoto T, Sinzobahamvya N, Kumpikaite D, Asfour B, (2011a) Public reporting of cardiac surgery perfor-
Photiadis J, Brecher AM, Urban AE (2005) Repair of mance: part 1-history, rationale, consequences. Ann
truncus arteriosus and aortic arch interruption: out- Thorac Surg 92(3 Suppl):S2–S11
come analysis. Ann Thorac Surg 79:2077–2082 Shahian DM, Edwards FH, Jacobs JP, Prager RL, Normand
Nashef SA, Roques F, Michel P, Gauducheau E, Lemeshow S, SL, Shewan CM, O’Brien SM, Peterson ED, Grover FL
Salamon R (1999) European system for cardiac opera- (2011b) Public reporting of cardiac surgery perfor-
tive risk evaluation (EuroSCORE). Eur J Cardiothorac mance: part 2-implementation. Ann Thorac Surg 92(3
Surg 16:9–13 Suppl):S12–S23
Nashef SA, Roques F, Sharples LD, Nilsson J, Smith C, Sinzobahamvya N, Photiadis J, Kumpikaite D, Fink C,
Goldstone AR, Lockowandt U (2012) EuroSCORE II. Eur Blaschczok HC, Brecher AM, Asfour B (2006)
J Cardiothorac Surg 41:734–744 Comprehensive Aristotle score: implications for the
Nilsson J, Algotsson L, Höglund P, Lührs C, Brandt J (2006) Norwood procedure. Ann Thorac Surg 81:1794–1800
Comparison of 19 pre-operative risk stratification Steinbusch PJ, Oostenbrink JB, Zuurbier JJ, Schaepkens FJ
models in open-heart surgery. Eur Heart J 27:867–874 (1995) The risk of upcoding in casemix systems: a com-
Nina RV, Gama ME, Santos AM, Nina VJ, Figueiredo Neto JA, parative study. Health Policy 81:289–299
Mendes VG, Lamy ZC, Brito LM (2007) Is the RACHS-1 Tischendorf JJ, Hecker H, Krüger M, Manns MP, Meier PN
(risk adjustment in congenital heart surgery) a use- (2007) Characterization, outcome, and prognosis in
ful tool in our scenario? Rev Bras Cir Cardiovasc 273 patients with primary sclerosing cholangitis: a
22:425–431 single center study. Am J Gastroenterol 102:107–114
O’Brien SM, Jacobs JP, Clarke DR, Maruszewski B, Jacobs Tu JV, Jaglal SB, Naylor CD (1995) Multicenter validation of
ML, Walters HL III, Tchervenkov CI, Welke KF, Tobota Z, a risk index for mortality, intensive care unit stay and
Stellin G, Mavroudis C, Lacour-Gayet FG (2007) overall hospital length of stay after cardiac surgery.
Accuracy of the Aristotle basic complexity score for Steering Committee of the Provincial Adult Cardiac
classifying the mortality and morbidity potential of Care Network of Ontario. Circulation 91:677–684
congenital heart surgery procedures. Ann Thorac Surg van Gameren M, Putman LM, Takkenberg JJ, Bogers AJ
84:2027–2037 (2011) Risk stratification for adult congenital heart sur-
O’Brien SM, Clarke DR, Jacobs JP, Jacobs ML, Lacour-Gayet gery. Eur J Cardiothorac Surg 39:490–494
FG, Pizarro C, Welke KF, Maruszewski B, Tobota Z, Miller Vijarnsorn C, Laohaprasitiporn D, Durongpisitku K,
WJ, Hamilton L, Peterson ED, Mavroudis C, Edwards FH Chantong P, Soongswang J, Cheungsomprasong P,
(2009) An empirically based tool for analyzing mortal- Nana A, Sriyoschati S, Subtaweesin T, Thongcharoen P,
ity associated with congenital heart surgery. J Thorac Prakanrattana U, Krobprachya J, Pooliam J (2011)
Cardiovasc Surg 138:1139–1153 Surveillance of Pediatric Cardiac Surgical Outcome
Overman D, Jacobs JP, Prager RL, Wright CD, Clarke DR, Using Risk Stratifications at a Tertiary Care Center in
Pasquali S, O’Brien SM, Dokholyan RS, Meehan P, Thailand. Cardiology Research and Practice 7 http://
McDonald DE, Jacobs ML, Mavroudis C, Shahian DM dx.doi.org/10.4061/2011/254321
(2013) Report from The Society of Thoracic Surgeons Welke KF, Shen I, Ungerleider RM (2006) Current assess-
National Database Work Force: clarifying the defini- ment of mortality rates in congenital cardiac surgery.
tion of operative mortality. World J Pediatr Congenit Ann Thorac Surg 82:164–170
Heart Surg World J Pediatr Congenit Heart Surg
4:10–12
Padley JR, Cole AD, Pye VE, Chard RB, Nicholson IA, Internet Sites
Jacobe S, Baines D, Badawi N, Walker K, Scarfe G,
Leclair K, Sholler GF, Winlaw DS (2011) Five-year analy- 7 http://66.89.112.110/STSWebRiskCalc261
sis of operative mortality and neonatal outcomes in 7 http://de.wikipedia.org/wiki/Delphi-Methode
congenital heart disease. Heart Lung Circ 20:460–467 7 www.bqs-outcome.de/2006/ergebnisse/leistungsbe-
Parsonnet V (1995) Risk stratification in cardiac surgery: is reiche/koronarchirurgie/buaw/risiko
it worthwhile? J Card Surg 10:690–698 7 www.dict.cc/?s=score
66 D. Boethig and J.P. Jacobs
7 www.eactscongenitaldb.org 7 www.krankenhausspiegel-hannover.de/lang/de_DE/
7 www.eactscongenitaldb.org/db/public-reports.py?fnc= clinical.picture.php?cpid=3&qiid=27
r42&dbname=database 7 www.ncbi.nlm.nih.gov/sites/entrez
7 www.ebm-netzwerk.de/netzwerkarbeit/jahrestagun- 7 www.rand.org
2 gen/pdf/wegscheider_Kongress04.pdf 7 www.bqs-online.com/public/leistungen/exqual/lbs/2007/
7 www.etymonline.com/index.php?search=scoru&search musteraw (“Koronarchirurgie isoliert”, PDF, p. 80)
mode=none 7 www.bqs-outcome.de/2006/ergebnisse/leistungsbe-
7 www.health.state.ny.us/diseases/cardiovascular/heart_ reiche/koronarchirurgie/download, p. 95
disease/docs/cabg_2002–2004.pdf
67 3
Principles of Quality
Assurance and Risk
Management Risk
Juergen Ennker and Tobias Walker
3.1 Introduction – 69
References – 83
Chapter 3 · Principles of Quality Assurance and Risk Management Risk
69 3
3.1 Introduction binding requirement for systematic quality assur-
ance was introduced, and it has become the govern-
Providing high-quality medical services was and still ing concept in this professional field. The applied
is the self-imposed demand physicians and hospitals quality assurance measures comprising not only
wish to be measured by. However, now it is no longer internal and external quality assurance with mor-
sufficient to merely formulate high demands; medi- tality and morbidity conferences but going far
cal service providers also have to document these beyond this concept.
services to achieve comparability. Exacerbated by
increasing economic constraints and an ever-increas-
ing competitive situation, on the one hand, as well as 3.2.1 Quality Assurance as an
increasing patient expectations, on the other hand, Essential Part of Quality
physicians and hospitals can no longer get by without Management
the introduction and permanent establishment of
quality management systems (Korenstein et al. 2012). Quality assurance is an essential part of the qual-
It has been shown in the recent past that in ity management system. Quality assurance mea-
addition to an improved medical reputation, the sures in hospitals, medical facilities, and practices
pursuit of maintaining and improving patient care have the aim of increasing the trust of all interest
also offers economic advantages. Documentation groups («stakeholders») by fulfilling precisely for-
and the evaluation of the services rendered, mulated requirements.
namely, quality assurance, as well as the identifi- Medical quality assurance and patient interest
cation and prevention of risk situations and risk are thus closely linked with one another.
management, play a very essential role. For both
tools, medical care oriented toward patient needs
and demands is central. The aim of medical quality assurance is an
From the perspective of a hospital setting, we improvement of treatment quality and as a
would like to elucidate the essential elements of result patient satisfaction—one of the strongest
quality assurance and present the key elements of arguments in quality management systems.
a functional risk management system in everyday
hospital routine.
The common target of the various quality
management systems is to bring more transpar-
3.2 Fundamentals of Quality ency into the quality of medical care and to ensure
Assurance the long-term survival and continuous improve-
ment of physician practice (Lack and Schneider
In 1988, the German Physician’s Board included the 2005; Lack and Gerhardinger 2010).
obligation of all physicians to participate in quality The wish for continuous improvement in medi-
assurance measures in their own professional code cal services has been present in the medical pro-
of conduct. This decision has been implemented by fession for a long time. It goes beyond the
all State Medical Boards in the professional regula- measures for medical self-regulation (medical
tions. For this reason, participation in quality assur- case discussions, conferences, hygiene commis-
ance programs has become a part of the ordinary sions, drug commissions, clinical studies, semi-
professional duties of the physician. The obligation nars, and autopsies) established to date.
of a physician to participate in quality assurance is,
however, also defined by Articles 135–137 of
Volume V of the German Code of Social Law V 3.2.2 Excursus into Quality
(SGB V) by the legislator. In this code, it is stated Management
that: «Approved hospitals, inpatient care facilities
and inpatient rehabilitation facilities are committed According to the definition of the International
to introduce and further develop internal quality Organization for Standardization (ISO), quality is
management» (Ennker et al. 2004). «. . . the degree to which a set of inherent
In surgery departments, quality assurance mea- characteristics meets the requirements . . ..» The
sures have been a constant factor since the 1970s; a term «quality management» (QM) is defined as «. . .
70 J. Ennker and T. Walker
a process within an organization, which attempts to management and to receive certification. DIN stan-
achieve continuous improvement of a process or a dards are generally a universal standard that is famil-
service . . . » (7 www.iso.org/iso/iso_9000). iar to many people outside the medical field and
However, quality management established in whose merit and significance have achieved general
practice goes beyond these measures. For application acknowledgement. Additionally, the quality man-
in a hospital, there are several quality management agement system in accordance with DIN EN ISO
3 models. Despite differently weighted target parame- 9001:2000 can be applied to complex hospitals and
ters (patient and referring physician satisfaction, pro- university clinics as well as in their departments and
cess optimization, and responsibility of the sections, which can then be certified accordingly.
management), all current concepts in Germany have The central issue of the DlN-ISO standard is the
one central pillar, the PDCA cycle. This cyclic process written documentation of the essential procedures of
was formulated and described by William Edwards a facility (hospital, laboratory area, and medical care
Deming and Walter Andrew Shewhart in the 1980s center) in a QM handbook. Based on this electronic
(Deming 1982, 1986). PDCA stands for the terms or printed document, everyday hospital processes
«plan,» «do,» «check,» and «act» and describes the are made comprehensible and transparent to third
planning of a process or the improvement of an exist- parties. Moreover, the QM handbook also includes
ing process («plan»), implementating a change («do»), instructions and stipulations as the permanent focus
checking the results achieved («check»), and correct- of the overall work process. The QM handbook
ing based on the results of the process («act»), which according to DIN ISO is divided into five chapters,
can in turn lead to a new plan («plan»). This cycle has which inquire into the different areas systematically
also entered into the literature as the Deming Cycle. (quality management system requirements, defini-
tion of management responsibilities, resource man-
3.2.2.1 uality Management Models
Q agement, product realization, and nature of the best
in the Hospital possible form of continuous process improvement).
Several quality management models have been
developed for application in hospitals, which 3.2.2.3 Cooperation for
each have different focuses. This partly has his- Transparency and Quality
torical origins because the models were not in the Hospital (CTQ) and
originally developed for the medical field or for proCum Cert
pragmatic reasons because they place particular The Cooperation for Transparency and Quality
emphasis on certain specializations and medical (CTQ) model is a quality management system
disciplines. The most frequently used models of developed by one of the federal associations of the
quality management in Germany are as follows: health insurance funds, the German Hospital
55 DIN EN ISO 9001:2000 (DIN: German Federation, the Chambers of Physicians, and the
Institute for Standardization; EN: European German Nursing Council; it was developed explic-
Standard; ISO: International Organization for itly for implementation in hospitals. ProCum Cert
Standardization) is closely based on CTQ but was adapted for
55 Cooperation for Transparency and Quality in denominational sponsoring organizations.
the hospital (CTQ) with proCum Cert CTQ is based on a structured hospital self-
55 European Foundation for Quality assessment in accordance with defined prescribed
Management (EFQM) criteria. The report is divided into six criteria, and
55 Joint Commission on Accreditation of the hospital is assessed on the basis of the PDCA
Healthcare Organizations (JCAHO) cycle by the applicants themselves. This is followed
by an external assessment (inspection) by external
All four models are to be illuminated briefly in CTQ inspectors where points are awarded and
the following. facilities must reach a certain level to achieve certi-
fication. This may involve some areas that are
3.2.2.2 DIN EN ISO 9001:2000 assessed at a lower level being compensated for by
DIN EN ISO 9001:2000 is a quality management higher-rated partial criteria. However, in all areas,
standard and stipulates what requirements the over- at least 55 % of the possible points must be achieved.
all management of an enterprise must fulfill to meet In the case of proCum Cert, this model has
a certain standard in the implementation of quality been extended by a Christian mission statement
Chapter 3 · Principles of Quality Assurance and Risk Management Risk
71 3
and a Christian self-understanding within the In the JCAHO handbook, 562 different stan-
CTQ requirements: dards are defined for medical performance assess-
55 Pro: for the patients and for good and proven ments. Upon fulfillment of these standards,
quality in denominational hospitals. JCAHO certificates are awarded. These certifi-
55 Cum: with the patients and their concerns cates are, however, graduated and correspond to
and wishes and with other denominational accreditation steps, each time imposing more
hospitals. stringent requirements on the hospital. In 2000,
55 Cert: structures, standards, and procedures, the first quality standards for hospitals valid
which have to exist, are laid down by an worldwide were published. For the first time,
«Expert Advisory Committee» and monitored these standards take the different economic, polit-
by trained inspectors. ical, and cultural peculiarities of the countries
into account.
3.2.2.4 European Foundation
for Quality Management
(EFQM) 3.3 External Quality Assurance
The underlying quality management concept of the
European Union (European Foundation for The measures outlined above all have a quality
Quality Management, EFQM) was initially focused assurance character, but do not, however, guaran-
on facilities in health care. Originally its intention tee optimal therapeutic quality. The diversity in
was to comprise a concept for industrial working medical therapeutic quality principally arises
areas and service providers. Nine criteria were from the skills and ability of the physicians and
defined for application in hospitals. The basic idea the health care staff as well as the experience and
of the concept is the requirement that the best in a motivation of the team assigned to the patient.
branch or field set the standard and competitors The compliance of the patient also contributes to
are benchmarked according to this standard. More diversity in care. Therefore, therapeutic results
specifically, the EFQM comprises five eligibility cri- obtained under study conditions can also not
teria, which show the nature of the services an always be reproduced and therefore can differ
organization provides. The results achieved by the from clinic to clinic (Moreland 1999).
organization are presented in four outcome criteria. With the help of external quality assurance, an
In this model, the petitioner also initially gives a attempt can be made to record the therapeutic qual-
self-assessment. In the event of a prize being attrib- ity of the participating facilities, thereby making
uted, this assessment is checked by the EFQM them comparable. The objective of such measures
within the scope of a visit (external assessment). may simply be the documentation of an applied
The organizations with the highest point scores can standard. However, they can also be used to achieve
receive the European Quality Award (EQA) from transparency and to provide comparability for
the European Organization for Quality (EOQ). referring physicians, health insurance companies,
Since the criteria for this award are very compre- patients, and relatives (hospital clients).
hensive, this model of quality management is princi-
pally suitable for large hospitals and not necessarily
for subsections or individual departments of a hospi- Additionally, with the aid of external quality
tal. Similarly, this model is recommended for enter- assurance, competition can develop between
prises that already have experiences with quality the participating enterprises and can result in
management systems and have already experienced the initiation of an improvement of
certification procedures (Kastenholz et al. 2011). therapeutic quality.
IT To carry out a statistically valid data analysis, it is necessary to put together several sources of
documentation data within a hospital. In practice, this often represents much difficulty:
3 and data
The data sources in autonomous departments are often unlinked
acquisition
Date acquisition is often of a redundant nature
The individual departments as «owners» of the data sources insist on their autonomy
Acquisition of For a more detailed, postoperative follow-up, observation data must be accessed. To do this,
patient data it is necessary to develop and implement a multitiered system. This enables continuous
following documentation of the important anamnestic perioperative data. It is recommended that, in
transfer addition to the current daily routine work, a follow-up collection of data should be carried
out 6 months and 1 year postoperatively. This includes follow-up of physicians and hospitals
(hospitals for follow-up treatment, rehabilitation clinics, etc.) as well as the patients
themselves
Risk adjustment To evaluate the results of new therapies as well as the individual surgeons and departments,
it is necessary to compare the risk profiles of the patient population adequately. This is
possible with the help of established risk models such as Euroscore or Parsonnet Score. In
the representation of hospital-specific risk profiles, however, they are sometimes insufficient
for adequate presentation and can be supplemented by additional and, in some cases,
specific models. Moreover, it has to be taken into consideration that the collected data can
only be acquired retrospectively and can potentially result in error. Using modern statistical
methods such as balancing scores enables an adjustment of the patient characteristics and
hence leads to comparability between different institutions
Assessment As the results of surgical interventions are subject to multifactorial influences, the
plausibility elucidation of a cause and effect relationship is desirable. Depending on the method,
statistical methods alone can only show correlations and may not provide any actual
evidence. Therefore, the evaluated results must be subjected to a plausibility test
Due to the mass of collected data and the complex- referring physician, health insurance associations,
ity of the analysis, this internal quality assurance and patients, presentation within the scope of an
task is only possible with the help of computer- Internet website is also recommended.
assisted systems. Core issues of such a form of data
acquisition are summarized in . Table 3.2.
In the current conflict of dwindling financial 3.7 Risk Management
resources, computer-assisted data acquisition
represents a great challenge as an internal quality 3.7.1 Fundamentals of Risk
assurance tool. Thus, it is essential when setting Management
up and permanently maintaining such a system to
combine the multiple ranges of applications with In 1998, the Control and Transparency in
a high standard of quality. Further, one should Enterprises Act (ConTraA) took effect in
also not shy away from communicating the data Germany. According to this act, the board of an
gained within the scope of transparency and com- incorporated company must «. . . adopt appropriate
parability in the health care sector to the outside. measures to identify developments endangering
In addition to the written information for the the continued existence of the corporation at an
Chapter 3 · Principles of Quality Assurance and Risk Management Risk
75 3
early stage.» This legal requirement also refers to hysicians, practices, and hospitals. It is precisely
p
limited liability companies (Ltd) and its CEOs and modern medicine which involves a great potential
applies to a number of privately sponsored hospi- for complications, mistakes, and damage.
tal associations. The problem is accentuated by the demand of
Derived from this act is the contractual the legislator to reduce treatment costs. Ultimately,
accessory obligation of the hospital manage- this leads to a shortened hospital stay of the
ment to create and permanently guarantee the patient and to increased pressure of time for the
organizational principles for a smooth course treatment of the patient group involved.
of treatment. In concrete terms, for the hospital All these criteria taken together lead to an
owners and hospital management, this means increased risk for the patient.
that an appropriate department for legal as well This became clearly apparent when the
as technical-medical guidelines must be estab- Institute of Medicine (IOM) published its report
lished. This begins with the decision for a spe- «To Err Is Human» in 2000 (Institute of Medicine
cific legal form of the enterprise progressing up 2000; Leape and Berwick 2005). According to
to the allocation of departmental responsibilities. their definition, the term «patient safety» means
Furthermore, the hospital management also has the absence of adverse events during treatment.
the obligation to ensure the organizational struc- However, about 10 % of all hospital patients suffer
tures become effective in everyday medical treat- from adverse events, of which approximately half
ment and, if necessary, to intervene. The hospital can be attributed to direct errors in hospital activ-
manager is also responsible and accountable for ity and would have been avoidable (Institute of
the financial, spatial, personnel, and equipment Medicine 2000, 2001). As a result, in the USA
planning of the hospital. alone, up to 100,000 deaths per year have to be
In 1952, the BGH formulated a «Postulate of expected, which in principle would have been
Jurisdiction»: «Responsibility will be held for avoidable. There are no exact figures available for
organizational errors irrespective of financial, Germany but is may be assumed that they are of
structural, personnel or material bottlenecks similar dimensions.
because protection and safety of the patient has The topic «patient risk, patient safety, and
absolute priority over all other issues.» improvement of risk safety» is of enormous
importance for patients, hospital staff, and insur-
ance companies. Accordingly, there is also in
Emerging from the legislation described
Germany an ever-increasing ambition both on
above is the legal obligation of a medical
the part of the insurance companies as well as for
care facility to define a corporate strategy for
patients and physicians’ representatives to make
the enterprise to prevent damage and risks
important aspects of hospital activity a part of a
and to minimize any damage that may occur.
functioning risk management system.
On the other hand, the development within 3.7.2 What Is Understood by Risk?
the health care system has led to manifold
advances in therapy options. As a result, a marked The term «risk» can be characterized as the
improvement in the therapeutic results could be probability of damage occurring in the negative
achieved (Ulrich 2012; Fehr et al. 2012). At the case or benefit in the positive case. How the
same time available medical technology has con- damage or benefit is understood depends on the
tinued to develop so that therapeutic results values and objectives of the organization con-
which were still unthinkable several years ago are cerned in each case. In the area of risk manage-
taken for granted today. Through the publication ment, this approach, however, is limited
of data, the general awareness and demands of exclusively to the negative viewpoint, that is, the
patients are growing accordingly. potential damage to the patient or financial loss
On the other hand, the increased frequency of for the organization.
high-risk operations also leads to an increasing There are often several concomitant risks that
number of civil proceedings with increasing may jeopardize a patient’s therapeutic success.
claims for damages and compensation from One problem regarding risk assessment is that not
76 J. Ennker and T. Walker
Training Experience
Organization Communication
Chapter 3 · Principles of Quality Assurance and Risk Management Risk
77 3
..Fig. 3.2 Damage
pyramid according to
Heinrich
1 Catastrophe
29 Accident
300 Incident
..Fig. 3.3 Risk
management cycle
according to Ennker et al.
(2006) 1. RISK IDENTIFICATION 2. RISK ASSESSMENT
legal implications are feared. Ultimately, risk anal- usually does not lead to the discovery of the actual
ysis is a purely retrospective process, so that causes and the error analysis also does not lead to
important aspects may simply become forgotten. a real improvement in the causative process.
This means that in many cases, the «catastro- The origin of errors and the erroneous handling
phe» has already happened and only then is an as a result is a multifactorial process, whose causes,
investigation started to determine how this calam- as a general rule, are to be sought in different work-
ity could occur (Duthie et al. 2005). ing areas and only in the rarest cases can they be
The most common reaction to a major calam- reduced to a direct, exclusively human cause
ity is the provision of prevention measures, so that (Carthey et al. 2001). The principle possible causes
this particular catastrophe can never happen are frequently ignorance and/or i ndifference, a heavy
again. The essential aspect of far-reaching risk pre- or excessive workload, communication deficiencies,
vention, however, is that the calamity is often only monitoring problems, inadequate resources, and in
the tip of an iceberg and under the surface lays the the health care system often patient factors. They
hidden potential for a complete series of further may make a contribution toward the occurrence of
and different calamities that cannot be resolved by an error on an individual basis or in their entirety.
the specifically tailored individual measures.
Whereas in Anglo-Saxon countries the assess-
ment of errors and causes of errors has changed,
The vast majority of errors here is not to be
in Germany errors are still primarily considered
ascribed to an individual person but often
under the aspect of personal responsibility.
arises at communication interfaces between
Although physicians are often threatened with
different service areas and types of service.
sanctions, those concerned usually accept the
consequence of, if possible, covering up the mis-
takes or not revealing their own involvement. Due
to the paucity of information, the consequence of However, in order to learn from mistakes and to
such behavior is the exact causes of the emergence avoid the repetition of the same mistake, errors and
of the error cannot be or can only inadequately be previous incidents must in the first instance be docu-
recognized (Department of Health 2000). mented in a reproducible system. When doing this, it
In the aviation transport or the atomic indus- is of decisive importance that reporting is performed
try, errors that occur are not always the result of free of sanctions and anonymously. Only when it has
human error. This erroneous assumption then been accepted in an organization that everyone is
Chapter 3 · Principles of Quality Assurance and Risk Management Risk
79 3
capable of making mistakes and that this may consti- incidents (incidence reporting system), an attempt
tute a part of normal working procedures can a valid is made to record all incidents independent of their
system be established to identify the sources of potential repercussions and to derive suitable mea-
error—the prerequisite for reliable prophylaxis sures from this for organizational processes
(Leape 2002; Emanuel et al. 2008). In the following, a (Gausmann and Schmitz 1998). The underlying
selection of such systems, which have already proved principle behind this form of recording is based pri-
their validity in practice, are presented. marily on the abovementioned statistical relation-
ship between a multitude of preceding incidents of
3.7.6.1 Adverse Occurrence smaller significance and a catastrophe ultimately
Screening resulting from these (cf. Heinrich’s Law).
Using adverse occurrence screening and a targeted
search for previously defined adverse events, it is pos- 3.7.6.4 Complaint Management
sible to make a risk assessment of defined sections of The systematic introduction of a functional com-
a process or a service (Pagnamenta et al. 2012). plaint management system is based on the follow-
In a hospital, for example, this can be the death of ing observation: a patient who is satisfied with his
a patient, unplanned readmission into a hospital, treatment tells an average of three additional per-
unplanned return transfer to the intensive care unit, sons about his/her experiences. A patient who is
an unplanned operation, or hospital stay of more dissatisfied with his treatment informs nine addi-
than 30 days. With the help of a computer-assisted tional persons about this, and a patient who was
database, an assessment of the occurrence and dissatisfied who could then be satisfied again (e.g.,
chronological sequence of defined risks may be with the help of a functional complaint manage-
gained. The disadvantage of this method is, however, ment system) recounts this very satisfying experi-
the fact that only retrospective registration of the ence for him/her to 20 additional persons.
defined target variables is possible, thus hampering In this example, it is evident that the reputation
the search for the causes due to delays (Reason 1995). of a hospital can be dependent on positive and
negative incidents and that it is important to react
3.7.6.2 Sentinel Event Report appropriately to the complaints made by patients.
Certain events in a hospital can be considered as
key events, based on a combination of similar error
Every complaint must be seen as being an
structures. Examples of this are (Leape et al. 1995):
opportunity for improvement. Patients who
55 Operation of the wrong patient or the wrong side
express complaints are in principle interested
55 Suicide of a patient during the hospital stay
in contact with the hospital and/or its staff.
55 Unexpected death of a patient
55 Reoperation due to forgotten material or
surgical instruments
55 Blood transfusion despite blood group Patients who have the feeling that their com-
incompatibility plaints are handled attentively and satisfactorily
55 Medication mix-ups as the direct cause of believe that their decision to choose this hospital
death of a patient has been confirmed, and they then communicate
their experience to others.
3.7.6.3 Incidence Report Further, important avenues for internal qual-
This reporting system involves the acquisition and ity management can be derived from where there
assessment of all error incidents within an organiza- are deficits in the everyday routine or where
tion, independent of whether they are severe or only potential for improvement exists.
minor incidents (WHO 2005). In such a complex
organization such as hospital, errors and near errors 3.7.6.5 Patient Questionnaire
are an integral part of the daily routine. Although The aim of patient questionnaires is to improve
several of the incidents rated as especially serious patient satisfaction and the competitiveness of the
are discussed within the scope of ward handovers or facility concerned. One of the greatest challenges
medical rounds, a large majority of these do not, in the implementation of this tool consists initially
however, undergo any further processing as they are in detachment from an antiquated doctor-patient
not really considered to be relevant, as «nothing relationship. A patient generally possesses only lim-
happened.» With the help of a reporting system for ited medical competence and must be considered
80 J. Ennker and T. Walker
as a partner who is involved in relevant decisions. 55 Checking the effectiveness of the measures
To be a partner, he/she should be put into the posi- taken to prevent errors
tion by the medical personnel to be able to properly 55 Reassessment of the risk after the corrective
judge his/her own clinical situation (Merten 2005). measure based on the abovementioned key
If attention is given to patient questionnaires which points (gives a new risk priority number, which
particularly describes situations that include risk in turn regulates the further need for action)
3 and regards potential for improvement, then imple-
menting a questionnaire leads to gaining important A comparative analysis of the risk priority
information that may have escaped the attention of numbers gives the possible risk reduction and the
the staff. From this point of view, patient question- most suitable measure to prevent the occurrence
naires represent an important source of informa- of a specific error and damage associated with it
tion for exposing risk potentials. (cf. Stauss and Seidel 2002).
In order to gain as much information as pos-
sible from such questionnaires, a patient
questionnaire should be constructed in such a 3.7.7 Risk Analysis and Risk
way that it allows not only yes and no answers but Assessment
also allows rating. This is usually achieved by an
evaluation of the predefined statements or scaling Following risk identification is an analysis, an assess-
of «not/never true» up to «completely/always ment, and an estimation of the risks, and potential
true,» usually in five to six steps. risk areas are discovered. These are attributable the
product of the probability of the occurrence of the
3.7.6.6 Failure Mode and Effects analyzed error and the degree of the damage associ-
Analysis (FMEA) ated with it. This relationship can be depicted graph-
The FMEA is an analytical method designed to ically as in . Fig. 3.4. The risks here cannot always
find potential weaknesses in a workflow procedure, be seen as single, independent points, but rather
a process, or an organization. Within the frame- they may include larger areas which are then
work of risk management, the FMEA can be imple- described as «risk areas.» In certain special cases,
mented to avoid errors and to act as a preventative these areas may also shift into areas that exceed a
measure. The FMEA is premised on precautionary predetermined risk threshold. A section of the risk
accident prevention as opposed to error detection which is not located within an acceptable range
and correction (error management), emphasizing must be considered separately and re-analyzed.
early identification of potential causes of error. In At the beginning of a risk analysis, the probabil-
practice, this usually functions by a team of ity of the occurrence of the risk should be deter-
employees from the departments concerned hav- mined. This is especially important because risks
ing a discussion on errors and the assessment of the that occur regularly show a habituation effect and as
errors, which should take place in predefined steps: a result are underestimated in the future. It is there-
55 Identification of potential error possibilities fore recommended to follow a time frame scheme.
55 Evaluation of the possible error consequences An example of this is presented in . Table 3.3.
55 Analysis of the causes Following the determination of the probabil-
55 Risk assessment according to the estimated ity of occurrence are an evaluation and classifica-
probability of the occurrence of the error tion of consequential damage. For a hospital, this
concerned and classification in a mathematical can be presented as in . Table 3.4.
scale system From . Tables 3.3 and 3.4, it is possible to set
55 Classification of the possible damage up a risk rating matrix, and the classified events
55 Classification of the probability of can be assigned to specific levels of activity, which
discovering this error must be implemented by those bearing responsi-
55 Multiplication of the values of occurrence, bility in order to prevent the future occurrence of
significance, and discovery (this gives a these risks and/or damage (. Table 3.5).
grading system with a risk priority number Based on this structured risk estimate and the
which regulates the urgency of the further potential consequence, a ranking list can be created,
need for action) in which ranking of the potential consequences and
55 Result-oriented discussion of measures to error sources takes place. A catalog of measures can
make the occurrence of the error impossible then be derived from this list to deal with risks.
Chapter 3 · Principles of Quality Assurance and Risk Management Risk
81 3
Probability
of occurence
Threshold for
acceptance
moderate
Repercussions
..Fig. 3.4 Relationship between probability and damage (From Ennker et al. 2006; used with permission)
Consequential damage
Very common 1 1 2 3 3
Common 1 1 2 3 3
Uncommon 1 2 2 3 4
Rare 1 2 3 4 4
Very rare 2 3 3 4 4
The numerical values given correspond to the different levels of consequences with the corresponding measures derived from these, which are listed below
Level 1: Unexpected death or persistent dysfunction that is not normally related to the disorder. The immediate intervention of the functionaries is required here. For a hospital,
especially in private ownership, such events threaten its very existence. A single occurrence here may also be sufficient to lead to serious consequences for the hospital
Level 2: An event that is fraught with a high risk or an incident fraught with a high risk which may potentially lead to death, severe complications, or a permanent dysfunction of
the patient. Immediate intervention of the functionaries is necessary
Level 3: Events that lead to health impairment of the patient and are related to inadequate handling of medical care, clinical practice, and quality of the operation. A report to those
bearing responsibility is required that stipulates appropriate measures to prevent future repetitions
Level 4: Events which involve a minor health impairment, but which harbor a risk potential. A remedy for this may often already be achieved by means of a qualitative improve-
ment in routine procedures
Chapter 3 · Principles of Quality Assurance and Risk Management Risk
83 3
In this way, within the scope of a risk avoid- increasing competition is becoming more and
ance scheme, a patient can be transferred to a spe- more important and is essential to the survival of
cialty hospital when adequate treatment cannot the enterprise and the employees working there.
be guaranteed in an organization’s own hospital.
References
Therefore, to deal with risks, it makes sense to
implement error-tolerant systems and to Albert AA, Walter JA, Arnrich B et al. (2004) On-line variable
organize the everyday clinical routine in a live-adjusted displays with internal and external risk-
number of stages, so that it is possible to adjusted mortalities. A valuable method for bench-
intervene in a regulative manner at different marking and early detection of unfavourable trends in
cardiac surgery. Eur J Cardiothorac Surg 25:312–319
interfaces.
Borracci RA, Rubio M, Ahuad Guerrero RA, Barrero C, Mauro V,
Fairman E (2007) Continuous monitoring of cardiac sur-
gery quality indicators. Arch Cardiol Mex 77(4):275–283
Often the demands of a functional risk pre- Carthey J, de Leval MR, Reason JT (2001) The human factor in
cardiac surgery: errors and near misses in a high tech-
vention scheme are also considerably easier. In
nology medical domain. Ann Thorac Surg 72:300–305
this way, errors attributable to overtiredness in the Deming WE (1982) Quality, productivity, and competitive
physician sector can be minimized by compliance position. Massachusetts Institute of Technology,
with legal regulations. Cambridge, MA
In certain cases, however, a risk cannot be pre- Deming WE (1986) Out of the crisis. Massachusetts
Institute of Technology, Cambridge, MA
vented completely by regulations and measures.
Department of Health (ed) (2000) An organization with a
This residual risk must be accepted, and its conse- memory—report of an expert group learning from
quences must as be cushioned, for example, by adverse events in the NHS. Department of Health, Norwich
transferring the risk, such as in the financial sec- Dimitrova KR, Hoffman DM, Geller CM, Ko W, Lucido DJ,
tor by taking out insurance policies. Dincheva GR, Tranbaugh RF (2012) Radial artery graft-
ing in women improves 15-year survival. J Thorac
Despite all efforts to regulate the handling of
Cardiovasc Surg. doi:pii: S0022–5223(12)01244–
risks, one will not be able to completely avoid the 5.10.1016/j.jtcvs.2012.10.004. [Epub ahead of print]
occurrence of errors in the hospital. Duthie E, Favreau B, Ruperto A, Mannion J, Flink E, Leslie R
(2005) Quantitative and qualitative analysis of medica-
tion errors: the New York experience. In: Henriksen K,
Battles JB, Marks ES, Lewin DI (eds) Advances in patient
3.7.9 Risk Controlling safety: from research to implementation, vol 1,
Research findings. Agency for Healthcare Research
Regarding risk controlling, it is important not to and Quality (US), Rockville
destroy the trust afforded to the staff by control Emanuel L, Berwick D, Conway J, Combes J, Hatlie M, Leape
measures being perceived as excessive and absurd. L, Reason J, Schyve P, Vincent C, Walton M (2008) What
exactly is patient safety? In: Henriksen K, Battles JB,
Rather, risk controlling must serve to check
Keyes MA, Grady ML (eds) Advances in patient safety:
whether the joint efforts show truly measureable new directions and alternative approaches, vol 1,
successes toward risk reduction. To do this, it is, Assessment. Agency for Healthcare Research and
on the one hand, important that risk management Quality, Rockville
is understood as being a constant process that Ennker J, Zerkowski HR (eds) (2006) Risiko und Qualität in
der Herzchirurgie. Steinkopff, Darmstadt
cannot already be terminated a few months after
Ennker J, Debong B, Beller CJ (2004) Herzchirurgie und
its introduction. On the other hand, it is necessary Recht. Steinkopff, Darmstadt
that the measures employed to identify risks are Ennker J, Pietrowski D, Kleine P (2006) Risikomanagement
repeated and analyzed at regular intervals or pref- in der operativen Medizin. Steinkopff, Darmstadt
erably established from the beginning on a con- Fehr R, Hurley F, Mekel OC, Mackenbach JP (2012) Quantitative
health impact assessment: taking stock and moving for-
tinuous basis, as is possible with the help of an
ward. J Epidemiol Community Health 66(12):1088–1091.
incidence reporting system. If concrete measures doi:10.1136/jech-2011-200835, Epub 2012 Jul 7
emerge from the strategies for dealing with risks, Flameng W, Herijgers P, Bogaerts K (2003) Recurrence of
it is necessary to communicate the measures mitral valve regurgitation after mitral valve repair in
adopted and the resulting improvements not only degenerative valve disease. Circulation 107:1609–1613
Gausmann P, Schmitz R-M (1998) Incident Reporting,
to all members of staff but also to external part-
Zwischenfallerfassung zur Bewusstmachung,
ners and the patients to increase the level of trust Steuerung und Reduzierung klinischer Risiken. Führen
in the enterprise—a fact that in the light of Wirtschaften 6:533–537
84 J. Ennker and T. Walker
Head SJ, Osnabrugge RL, Howell NJ, Freemantle N, Nashef SA, Roques F, Michel P, Gauducheau E, Lemeshow S,
Bridgewater B, Pagano D, Kappetein AP (2013) A sys- Salamon R (1999) European system for cardiac opera-
tematic review of risk prediction in adult cardiac sur- tive risk evaluation (EuroSCORE). Eur J Cardiothorac
gery: considerations for future model development. Surg 16:9–13
Eur J Cardiothorac Surg 43(5):e121–e129 [Epub ahead O’Brien SM, Shahian DM, Filardo G, Ferraris VA, Haan CK,
of print] Rich JB, Normand SL, DeLong ER, Shewan CM,
Heinrich HW (1941) Industrial accident prevention: a scien- Dokholyan RS, Peterson ED, Edwards FH, Anderson RP,
3 tific approach, 2nd edn. McGraw-Hill, New York Society of Thoracic Surgeons Quality Measurement
Institute of Medicine (ed) (2000) To err is human: building Task Force (2009) The Society of Thoracic Surgeons
a safer health system. Institute of Medicine, 2008 cardiac surgery risk models: part 2—isolated
Washington, DC valve surgery. Ann Thorac Surg 88(1 Suppl):S23–S42.
Institute of Medicine (ed) (2001) Crossing the quality doi:10.1016/j.athoracsur.2009.05.056
chasm: a new health system for the 21 century. Osswald BR, Blackstone EH, Tochtermann U, Thomas G,
Institute of Medicine, Washington, DC Vahl CF, Hagl S (1999) The meaning of early mortality
Jones RH, Hannan EL, Hammermeister KE et al. (1996) after CABG. Eur J Cardiothorac Surg 15:401–407
Identification of preoperative variables needed for risk Pagnamenta A, Rabito G, Arosio A, Perren A, Malacrida R,
adjustment of short-term mortality after coronary Barazzoni F, Domenighetti G (2012) Adverse event
artery bypass graft surgery. The Working Group Panel reporting in adult intensive care units and the impact
on the Cooperative CABG Database Project. J Am Coll of a multifaceted intervention on drug-related adverse
Cardiol 28:1478–1487 events. Ann Intensive Care 2(1):47. doi:10.1186/2110-
Kara I, Cakalagaoglu C, Ay Y, Al Salehi S, Yanartas M, Anasiz 5820-2-47
H, Koksal C (2013) Reoperative coronary artery bypass Reason J (1995) Understanding adverse events: human
surgery: the role of on-pump and off-pump tech- factors. Qual Health Care 4:80–89
niques on factors affecting hospital mortality and Reason J (1997) Managing the risks of organizational acci-
morbidity. Ann Thorac Cardiovasc Surg 19(6):435–440 dents. Ashgate Publishing Company, Burlington
[Epub ahead of print] Reason J (2000) Human error: models and management.
Kastenholz H, Geraedts M, Selbmann HK (2011) BMJ 320:768–770
Benchmarking in health care: an instrument for qual- Reason J (2012) James Reason: patient safety, human
ity improvement prevails. Z Evid Fortbild Qual error, and Swiss cheese. Interview by Karolina
Gesundhwes 105(5):329–330 Peltomaa and Duncan Neuhauser. Qual Manag Health
Korenstein D, Falk R, Howell EA, Bishop T, Keyhani S (2012) Care 21(1):59–63
Overuse of health care services in the United States: Rosser JC, Rosser LE, Savalgi RS (1997) Skill acquisition and
an understudied problem. Arch Intern Med 172(2): assessment for laparoscopic surgery. Arch Surg
171–178 132:200–204
Lack N, Gerhardinger U (2010) An analysis of the effective- Seghieri C, Mimmi S, Lenzi J, Fantini MP (2012) 30-day in-
ness of external quality assurance programmes using hospital mortality after acute myocardial infarction in
changes in quality indicators of individual hospitals. Z Tuscany (Italy): an observational study using hospital
Evid Fortbild Qual Gesundhwes 104(6):503–511. discharge data. BMC Med Res Methodol 2:170.
doi:10.1016/j.zefq.2010.07.009 doi:10.1186/1471-2288-12-170
Lack N, Schneider KT (2005) Quality measurement Shahian DM, O’Brien SM, Filardo G, Ferraris VA, Haan CK,
between illusion and reality—is the quality of medical Rich JB, Normand SL, DeLong ER, Shewan CM,
care measurable? Z Geburtshilfe Neonatol 209(1):1–7, Dokholyan RS, Peterson ED, Edwards FH, Anderson RP,
Review Society of Thoracic Surgeons Quality Measurement
Leape LL (2002) Reporting of adverse events. N Engl J Med Task Force (2009) The Society of Thoracic Surgeons
347:1633–1638 2008 cardiac surgery risk models: part 1—coronary
Leape LL, Berwick DM (2005) Five years after To Err Is artery bypass grafting surgery. Ann Thorac Surg 88(1
Human: what have we learned? JAMA 293((19):2384– Suppl):S2–S22. doi:10.1016/j.athoracsur.2009.05.053
2390 Stauss B, Seidel W (2002) Beschwerdemanagement,
Leape LL, Bates DW, Cullen DJ et al. (1995) Systems analysis Kundenbeziehungen erfolgreich managen durch
of adverse drug events. JAMA 274:35–43 Customer Care, 3rd edn. Carl Hanser, Munich
MacKenzie TA, Brown JR, Likosky DS, Wu Y, Grunkemeier GL Tranbaugh RF, Dimitrova KR, Friedmann P, Geller CM, Harris
(2012) Review of case-mix corrected survival curves. LJ, Stelzer P, Cohen BM, Ko W, DeCastro H, Lucido D,
Ann Thorac Surg 93(5):1416–1425 Hoffman DM (2012) Coronary artery bypass grafting
Merten M (2005) Patienten-Befragung: Gefühlte using the radial artery: clinical outcomes, patency, and
Unzufriedenheit. Dtsch Ärztebl 102:A3144 need for reintervention. Circulation 126(11 Suppl
Metzler B, Winkler B (2012) SYNTAX, STS and EuroSCORE— 1):S170–S175
how good are they for risk estimation in atherosclerotic Ulrich V (2012) Fostering of health economics in Germany.
heart disease? Thromb Haemost 108(6):1065–1071 Bundesgesundheitsblatt Gesundheitsforschung
Moreland RL (1999) Transactive memory: learning who Gesundheitsschutz 55(5):604–613
knows what in work groups and organizations. In: WHO (2005) WHO draft guidelines for adverse event
Thompson L, Levine J, Messick D (eds) Shared cogni- reporting and learning systems. WHO EIP/SPO/
tion in organizations: the management of knowledge. QPS/05.3. WHO, Geneva
Erlbaum, Mahwah, pp 3–31 7 www.iso.org/iso/iso_9000
85 4
4.1 Introduction – 86
4.3 Conclusion – 93
References – 93
4.1 Introduction cardiac surgery for many years. These models are
accepted by the cardiac surgery community and
In recent years, quality control, outcome mea- their representative societies.
surement, and reporting of performance criteria With the interest growing in control of cost in
have become central parts of daily activities for health care, the general belief has developed that
cardiac surgeons in the United States (Loebe et al. quality measures are one of the strongest vehicles
2009). Several layers of outcome reporting and to reduce expenditures. A good outcome leads to
quality control have been introduced by a number decreased resource utilization at all levels, shorter
4 of players in the health-care industry. Hospitals, length of stay (LOS), and less cost. In an environ-
practitioners, and entities such as insurance com- ment where reimbursement is based on diagnosis-
panies, provider networks, and state health-care related group (DRG) methodology, this is a
agencies are all subject to public reporting and necessary element in maintaining financial viabil-
have to compare their performance with national ity of both nonprofit and for-profit organizations
and regional benchmarks. and individuals. Therefore, as health care has
While public reporting is expected to generate moved from a type of free-market economy to a
awareness and influence decision-making by con- more regulated and controlled field, payors and
sumers, insurance companies as well as govern- governments have adapted. In the United States,
ment agencies use outcome reports for the Federal Government has become the largest
credentialing, re-credentialing, and regulation of provider of health care; it will be unavoidable that
health-care providers. Payments and financing mechanisms of central governance will be more
are now modified based on the outcomes reported. and more influential in how health care is pro-
To position oneself in the competitive market of vided. Again, cardiac procedures are in the fore-
health-care providers in the United States, institu- front as they are both very common and quite
tions have expanded the requirements on internal expensive, but other areas such as vascular surgery
reporting. Frequent reviews of individual practi- and general surgery are soon to follow. There is
tioners as well as service lines outcomes, internal little doubt that in the near future, all health-care
reporting, and internal benchmarking are increas- providers will be increasingly subject to intense
ingly applied to improve performance of pro- outcome monitoring and quality reporting.
grams and institutions and increase their standing About 60 % of cardiac patients in the United
in public reporting and contractual negotiations. States are covered by Medicare, a federal govern-
Furthermore, institutions are investing in ancil- ment agency. Patients covered through Medicaid
lary staff to maximize coding on each patient so (state government entities) add another 10 % of
that they can benefit from the risk adjustment of government-sponsored health care. Veterans are
outcomes and improve reimbursement. The entitled to receive care in a separate health-care
majority of the work on additional documenta- system, fully funded by the Federal Government
tion now falls on the clinical staff at each level of and with a unique quality control system, as all
patient care. information is centrally collected by the
There is no doubt that cardiac surgery lends Department of Veterans Affairs (VA) (Takaro
itself particularly well to such efforts of intense et al. 1986). VA funding is separate from Medicare.
outcome monitoring and public reporting due to The number of private-paying patients, either
the relative homogenicity of cases treated by car- through private insurance or directly, has con-
diac surgeons and the length of time that has been stantly declined, and today is less than 6 %. That
spent recording and developing the databases. leaves about 20 % of patients who have insurance
Comparably few procedures make up the vast through commercial carriers nearly always
majority of surgeries performed in the field (Guru bought and provided by the employer. These com-
et al. 2009). The key procedures, such as coronary mercial insurance companies and networks have
artery bypass grafting (CABG) or aortic valve their own benchmarks and quality standards.
replacement, have been extensively studied, and Performance data are used within the United
there is fairly good evidence that can be used as a States in different ways: some basic criteria are
basis for performance measurement and outcome adopted from the Medicare system, but additional
reporting. In addition, risk models have been performance requirements may be established by
developed and extensively tested in the field of the commercial payors to restrict the number of
Chapter 4 · Quality Control in Cardiac Surgery in the United States
87 4
certified providers, particularly in specialty pro- cardiovascular procedures play a primary role in
grams such as solid organ transplant. Different the quality assessment through the Federal
levels of partnership (provider network, center of Government. Hospitals and regions are measured
excellence, etc.) have been implemented to direct by their utilization of and performance in cardiac
patients to specific institutions. Quality parame- procedures. Consequently, great attention is
ters are the most important factors in such grad- directed to outcomes in this field by the adminis-
ing, but they are not the only ones: center volume, trative leadership of hospitals (Klein and Nashef
location, spectrum of other services, and pricing 2008).
do play a substantial role in becoming a center of
excellence.
Recently, the Federal Government, through 4.2 Levels of Quality Control
Medicare, has started to link reimbursement with
quality parameters in that certain complications 4.2.1 Local/Institutional Level
are not paid for at all (such as hospitalization for
line sepsis and decubitus ulcers) and, in general, Several levels of quality assurance in cardiac sur-
reimbursement is reduced if the hospital is not in gery have been established (. Table 4.1). One level
compliance with pre-specified benchmarks. For is the measurement of care performance on the
example, if performance criteria in discharge edu- institutional level. This would include documenta-
cation and discharge medication for heart failure tion and reporting of medication errors, sentinel
patients are not fulfilled, the total Medicare reim- events (e.g., retained sponges), patient falls, and
bursement for all services to the hospital will be patient satisfaction. Medication errors are docu-
reduced by 3 %. These are substantial punish- mented and reported by the pharmacy service of
ments for health-care providers and require hos- the hospital, and these reports are based on com-
pitals to increase their supervision of practitioners puterized records. Ongoing monitoring of patient
to change the way they deliver health care and satisfaction by interviews and sending out ques-
may force hospitals to remove physicians from the tionnaires after hospital discharge is mandated and
staff in order to control noncompliant personnel. can be performed by commercial service providers.
The Federal Government has long focused on The quality of physician performance is moni-
quality reporting in health care. Under the Clinton tored and reported internally. In most institu-
administration and continued under the second tions, this is based on the data derived from the
Bush administration, the Centers for Medicare Society of Thoracic Surgeons (STS) registry.
and Medicaid Services (CMS) through the Agency Individual outcomes are made available to the
for Healthcare Research and Quality (AHRQ) surgeon and to his peers. Usually, this includes
developed the Healthcare Cost and Utilization number of cases, mortality, time on ventilator, use
Project (HCUP) to identify Quality Indicators of blood products, and LOS. Furthermore, cases
(QIs) in response to the increasing demand for are identified and reviewed by peers based on cer-
information regarding the quality of health care. tain triggers, such as mortality, unplanned return
These measures, based on discharge data obtained to the operating room (OR), or complaints by
through Medicare, were intended to flag potential nonphysicians. These reviews are protected as
quality problems in hospitals or regions. Out of confidential and should not be confused with
200 possible indicators, 45 were selected, with 25 educational events such as mortality and morbid-
indicators evaluating quality of care at the hospi- ity conferences, which are open to residents and
tal level. These included seven volume indicators, fellows and not part of quality assurance.
and among these were five cardiovascular indica-
tors: abdominal aortic aneurysm (AAA) repair, 4.2.1.1 Morbidity and Mortality
carotid endarterectomy (CEA), CABG, percuta- Conferences
neous transluminal coronary angioplasty (PTCA), Morbidity and mortality conferences are depart-
and pediatric heart surgery. In addition to AAA mental events that are not protected under the
repair, pediatric heart surgery and CABG in- laws regulating quality control and reporting for
house mortality appear as indicators. CABG and the hospital. They are, of course, protected by
PTCA per capita are listed as indicators for confidentiality rules governing health care in gen-
regional quality in health-care delivery. Obviously, eral. These conferences cannot substitute for the
88 M. Loebe and M.G. Davies
force health-care providers to dedicate significant STS match. They found that the unmatched cases
resources to quality management and outcome were more likely to be minority patients and/or
reporting (Lindenauer et al. 2007). emergency operations. They also discovered that
the cases excluded from the STS database were
4.2.3.3 ociety of Thoracic Surgeons
S more likely to be associated with mortality. Some
National Database of the explanations for these discrepancies could
The STS National Database was established in 1989 include potential selective underreporting of
as an initiative for quality improvement and patient patients with poor outcomes.
4 safety among cardiothoracic surgeons. The STS
database now has over 1,000 participating surgeons 4.2.3.4 University Hospital
and data on almost four million operations (Brown Consortium
et al. 2010). There are three components to the STS University Hospital Consortium (UHC), Chicago,
National Database, each focusing on a different Illinois, formed in 1984, is an alliance of 116 aca-
area of cardiothoracic surgery: adult cardiac, gen- demic medical centers and 276 of their affiliated
eral thoracic, and congenital heart surgery, with hospitals representing approximately 90 % of the
the availability of anesthesiology participation nation’s nonprofit academic medical centers. The
within the Congenital Heart Surgery Database. Consortium’s membership includes academic
The component databases provide opportunities health systems from across the United States.
for quality improvement to their participants. The These systems came together to develop the
STS has developed quality performance measures Quality and Accountability Study, so they could
in all three subspecialties of surgery. have a truly objective, data-driven measure for
By collecting outcomes data for submission to comparing their systems. UHC offers an array of
the STS National Database, surgeons are commit- performance improvement products and services.
ting to improve the quality of care that their car- Extensive databases provide comparative data in
diothoracic surgery patients receive. STS continues clinical, operational, faculty practice manage-
to develop and maintain quality performance mea- ment, financial, patient safety, and supply chain
sures in the areas of adult cardiac, general thoracic, areas. Programs such as UHC’s Imperatives for
and congenital heart surgery. Many of these mea- Quality and the UHC/AACN Nurse Residency
sures are included in CMS’s Physician Quality Program™ offer opportunities for knowledge shar-
Reporting System. In the interests of transparency, ing and education. List servers allow members
STS has also established STS Public Reporting from across the Unites States to share information
Online: the publishing on sts.org of CABG com- on outcomes and performance.
posite quality ratings from STS Adult Cardiac
Surgery Database participants who have volun- 4.2.3.5 Hospital Quality Alliance
teered to participate. STS initially received consent In December 2002, the American Hospital
from 226 database participants to report their Association, the Federation of American
information through STS Public Reporting Online. Hospitals, and the Association of American
With over 1.5 million patients, the STS data- Medical Colleges launched the Hospital Quality
base provides unmatched outcome comparisons. Alliance (HQA), a national public–private col-
The STS National Database is about 97 % com- laboration to encourage hospitals to collect and
plete and ready to support longitudinal research report data regarding the quality of care on a vol-
on the outcomes of cardiothoracic surgery, untary basis. The HQA was designed to provide
according to results of a study that matched STS information about the quality of hospital care to
database records to records kept by CMS. A study the public and to «invigorate efforts to improve
by the Duke Clinical Research Institute matched quality.» Financial incentives were suggested and
records in the STS database and CMS database for have been since implemented.
CABG hospitalizations of Medicare patients over
age 65 from 2000 to 2007 (Guru et al. 2008). The 4.2.3.6 American College
researchers evaluated the representativeness of of Cardiology
the STS database by comparing data from the Medical outcome research has traditionally used
CMS records that were not matched to STS three different sources of data: randomized clini-
records with the CMS records that did have an cal trial data, administrative data, and registry
Chapter 4 · Quality Control in Cardiac Surgery in the United States
91 4
data. All forms of data have their respective and information solutions that support the areas
advantages and shortcomings. In recent years, of cardiovascular care where quality can be mea-
government agencies such as the FDA or CMS sured, benchmarked, and improved.
have increasingly requested registries for certain
procedures or devices. These post-marketing reg- 4.2.3.7 Vascular Quality Initiative
istries allow for evaluation of therapies in daily In 2011, the Society for Vascular Surgery (SVS)
practice. However, the quality of data reported launched the Vascular Quality Initiative (VQI) to
and provided to the agencies determines the value improve the quality, safety, effectiveness, and cost
of such registries. of vascular health care by collecting and exchang-
To improve the quality of cardiovascular ing information. VQI combines the concepts of
patient care by providing information, knowledge, regional quality improvement developed in New
and tools; implementing quality initiatives; and England with the intention of becoming a robust
supporting research that improves patient care and national vascular database and leader in quality
outcomes, the American College of Cardiology improvement in limb salvage. The New England
(ACC) launched the National Cardiovascular Data registry is a validated regional data registry within
Registry (NCDR) as a result of its exploration of a quality improvement initiative that has been
various strategies for collecting and implementing associated with improved preoperative medica-
clinical data in order to improve cardiovascular tion usage and has helped define real-world prac-
care (Messenger et al. 2012). As part of a data qual- tice and potential practice improvements.
ity assurance program, annual audits are per- VQI provides a potential vehicle for future
formed to assure the completeness and correctness public and pay-for-performance reporting and
of the information transferred into the registries has the potential to improve patient outcomes. It
by the sides. Educational tools to improve perfor- uses the structure of a Patient Safety Organization
mance of documentation and reporting are pro- (PSO) to ensure a focus on quality improvement
vided to the participating centers. Through this and to provide the necessary safeguards for data
effort, it is guaranteed that stakeholders using the confidentiality. The SVS PSO was listed by AHRQ
information derived from the registries can base in February 2011. The SVS PSO collects data
their decisions on high-quality data. related to commonly performed major vascular
The outgrowth of the NCDR effort has focused procedures. These include carotid endarterec-
on quality patient care through standardized tomy (CEA), carotid artery stenting (CAS),
measurement of clinical practice and patient out- infrainguinal bypass (INFRA), suprainguinal
comes. The first registry, encompassing cardiac bypass (SUPRA), open infrarenal abdominal aor-
catheterization and percutaneous coronary inter- tic aneurysm (oAAA) repair, endovascular infra-
vention (PCI) activities, was designed to help renal AAA repair (EVAR), thoracic endovascular
health-care provider groups and institutions aortic repair (TEVAR) including branch/fenes-
respond to increasing requirements to document trated AAA repair, peripheral vascular interven-
their processes and outcomes of care. Today, the tion (PVI) of aortoiliac and lower extremity
NCDR maintains several registries in the field of arterial disease, and hemodialysis access
cardiovascular medicine. Among them are the (ACCESS). Participants in VQI must commit to
ICD registry, collecting information about defi- entering data for all consecutive procedures per-
brillator therapy; the CARE registry, collecting formed and must submit billing data periodically
information about carotid interventions; and the to allow an audit that confirms complete entry of
TVT registry which has recently been started in all procedures. Follow-up information is collected
conjunction with STS to monitor percutaneous at 1 year for all procedures, an element unique to
aortic valve interventions. VQI, and at additional time points for selected
Today, more than 2,200 hospitals nationwide procedures that may require interval or longer
participate in the NCDR. As the preeminent US follow-up.
cardiovascular data repository, the NCDR pro- Analysis of aggregated data within the SVS PSO
vides evidence-based quality improvement solu- is directed by the Quality Committee and per-
tions for cardiologists and other medical formed by PSO staff. This includes the develop-
professionals. As a patient-centered resource, the ment of risk-adjustment algorithms to allow fair
NCDR has developed clinical modules, programs, comparisons of hospitals or physicians, adjusted
92 M. Loebe and M.G. Davies
for potentially important differences in patient on quality and performance measures. Other
characteristics. Based on these analyses, the Quality companies provide advice to health-care providers
Committee develops and disseminates informa- how to improve their performance. Finally, some
tion to improve patient safety, such as recommen- media attract advertisement by providing «best
dations, protocols, or information regarding best of» lists (best doctor, best hospital, etc.). Discussed
practices. The Quality Committee also coordinates here are some of the most important entities in the
and facilitates quality efforts of regional quality field of commercial quality review and reporting.
groups, as discussed above (Sect. 4.2.2). The SVS In addition to these entities, there are several other
4 PSO is permitted to publish data that are non-iden- smaller enterprises providing ratings, all with very
tifiable in terms of patient, physician, or hospital. different procedures to establish their ranking. As
This allows regional quality groups to obtain non- a consequence, at any given time, one can find sev-
identifiable data for their centers for specific eral hospitals advertising their services by claim-
regional quality analyses or research projects. It is ing to be «the best» in cardiac care in the region.
also possible for multiple regional groups or cen-
ters to pool non-identifiable data for analysis, and 4.2.4.1 The Commonwealth Fund
such projects are approved and coordinated by the The Commonwealth Fund is a private US founda-
SVS PSO Quality Committee. tion whose stated purpose is to «promote a high
performing health-care system that achieves bet-
4.2.3.8 Registries in Other Nations ter access, improved quality, and greater effi-
Proponents have long recognized the potential ciency, particularly for society’s most vulnerable»
value of collaborative registries to answer clinical and the elderly. This foundation covers many
questions and provide benchmarking for quality areas of national and international health-care
improvement or assurance. While their value is policy. One of these many fields of activity is the
significant, so are the logistical issues associated gathering and dissemination of evidence on excel-
with data collection, auditing data accuracy, lence in health care from across the country and
ensuring confidentiality, obtaining patient con- the world. National and international compari-
sent, and funding such efforts. In vascular sur- sons should help to show what is possible to
gery, one of the earliest registries that has been achieve and stimulate health-care providers, poli-
maintained is the Swedvasc effort, which has been cymakers, and stakeholders to take action to
in place since 1987. Subsequently, several other improve performance. Since 2006, The
European vascular registries were organized, and Commonwealth Fund and its Commission on a
more recently integrated as Vascunet, under the High Performance Health System have tracked
auspices of the European Society for Vascular the performance of US health care through a
Surgery. The United Kingdom (UK) Audit and series of national, state, and regional scorecards.
Australasian Vascular Audit (Australia and New
Zealand) have been organized in the UK and in 4.2.4.2 Leapfrog
Australia and New Zealand to function as quality Leapfrog is a for-profit organization advising
assurance registries. employers on what health insurance coverage to
offer to their employees. Its goals are to inform
Americans about hospital safety and quality, pro-
4.2.4 ommercial Quality Review
C mote full public disclosure of hospital perfor-
and Reporting mance information, and help employers provide
the best health-care benefits to their employees.
Several companies have entered the market in Leapfrog sets and publishes certain standards on
reviewing and reporting health-care outcomes. which to base health-care plan purchasing deci-
Again, cardiovascular medicine is the most docu- sions. For the most part, these standards are taken
mented and watched area in this regard. As the US from local or national databases, but they also
health-care system is a quasi-free market, these include recommendations on such organizational
companies exercise their influence to stir decision- and structural issues as participation in databases,
making towards certain health-care providers. intensive care unit staffing with board-certified
Some companies advise insurance companies in intensivists, door-to-needle time for acute myo-
contracting with hospitals and physicians based cardial infarction, and technical equipment. As
Chapter 4 · Quality Control in Cardiac Surgery in the United States
93 4
Leapfrog acts on behalf of purchasers and employ- qualification. The Scientific Registry of Transplant
ers across the country, it represents a substantial Recipients (SRTR) is the basis of outcome evalua-
market force in health care. Through its guide- tion. Here, every 6 months, detailed reports on the
lines and criteria, the group has substantial influ-performance of transplant centers are made avail-
ence on how health care is delivered. able to the public. This includes survival, graft
function, wait time, and demographics of recipi-
4.2.4.3 Healthgrades ents. Centers that do not meet expected perfor-
Healthgrades Inc. is a US company that develops mance levels get flagged and risk the loss of their
and markets quality and safety ratings of health- certification if they continue to underperform.
care providers, including hospitals, nursing Both CMS and UNOS visit transplant centers
homes, physicians, and dentists. Quality ratings unannounced to perform chart reviews. This has
are devised from publicly available patient safety led transplant centers to dedicate substantial
data and analyzed with proprietary technology amounts of time and personnel to documentation
developed by Healthgrades. In addition to these and reporting. It has also urged numerous small
ratings, Healthgrades offers consulting services to transplant programs to close down as the demand
health-care providers to improve on safety mea- in resources and outcome quality was deemed to
sures and how to enhance marketing and public be too economically challenging.
relations. As a for-profit organization, it has a
strong presence on the internet, where it provides
gradings for hospitals and physicians. 4.3 Conclusion
4.2.4.4 US News & World Report Quality control in cardiac surgery in the United
US News & World Report is a former print maga- States has grown rapidly in recent years. Efforts in
zine that now offers its ratings online. A multitude documentation and reporting of outcome data
of reports are available (schools, universities, hos- have become a substantial part of the cardiac sur-
pitals, etc.). The report on hospital rankings is one gery practice. Through their administrations, hos-
of the most prominent and offers a ranking of hos- pitals have taken a leading role in implementing
pitals by specialty. If a hospital is ranked in multi- benchmarks, monitoring outcome, and reporting
ple specialties among the top performers, it is these numbers to regional and national databases.
included in an Honor Roll. Recently, US News & Reimbursement has now been tied to perfor-
World Report started to offer regional comparisons mance criteria. Multiple layers of documenting
as well. Its findings are hugely used for promotion and reporting have been implemented. To remain
and advertisement of hospitals in the United competitive in this environment, institutions have
States. In their reporting, cardiac surgery is ranked no choice but implement processes of quality con-
together with cardiology. Factors influencing the trol and quality improvement. Constant internal
rank are volume, outcome, technical equipment, reporting and rapid intervention on data suggest-
reputation score, and number of nurses. In the ing a process or practitioner underperforming are
upper segment, the ranking is largely driven by a necessary to maintain effective health-care deliv-
reputational score, which is obtained through a ery. The hope is that these efforts will result in
questionnaire sent out to peers in the field. improved outcomes for patients and a more effi-
cient use of resources in cardiac surgery.
Guru V, Naylor CD, Fremes SE, Teoh K, Tu JV (2009) Publicly Loebe M, Tewani S, Bruckner BA, Disbot M (2009) Quality
reported provider outcomes: the concerns of car- management in cardiac surgery in the USA. Dtsch Med
diac surgeons in a single-payer system. Can J Cardiol Wochenschr 134(Suppl 6):S234–S236, German
25(1):33–38 Messenger JC, Ho KK, Young CH et al. (2012) The National
Klein AA, Nashef SA (2008) Perception and reporting of Cardiovascular Data Registry (NCDR) data quality
cardiac surgical performance. Semin Cardiothorac brief: the NCDR data quality program in 2012. J Am
Vasc Anesth 12(3):184–190 Coll Cardiol 60(16):1484–1488
Lindenauer PK, Remus D, Roman S et al. (2007) Public Takaro T, Ankeney JL, Laning RC, Peduzzi PN (1986)
reporting and pay for performance in hospital quality Quality control for cardiac surgery in the Veterans
improvement. N Engl J Med 356(5):486–496 Administration. Ann Thorac Surg 42(1):37–44
4
95 5
Databases in Cardiac
Surgery
Wolfgang Schiller and Jan F. Gummert
5.1 Introduction – 96
References – 107
Counts Coronary Artery Bupass Surgery Valve Surgery Congenital Heart Disease Others
120.000
100.000
80.000
60.000
40.000
20.000
0
94 95 96 97 98 99 00 01 02 03 04 05 06 07 08 09 10 11 12
Year
..Fig. 5.1 DGTHG performance statistics: annually performed main cardiac surgical procedures since 1994 (Copy-
right: German Society for Thoracic and Cardiovascular Surgery)
In Germany, every diagnostic or surgical proce- bility to preserve the comparability of data even
dure has to be coded for reimbursement reasons. over decades.
Coding is based on a German Operation and The database registers procedures, not patients.
Procedures System (OPS) which was derived in 1994 Thus, the number of procedures is higher than the
from the «International Classification of Procedures number of treated patients. As a result, mortality is
in Medicine» (ICPM), and since that time, new meth- declared on a slightly lower level than an effective
ods have been adopted on an annual basis. Therefore, one; however, analyzing electronically submitted
it represents not only the state-of-the-art medical data showed that this difference is neglectably small.
therapies but also new procedures. For example, Future development of this database will pro-
transcatheter aortic valve implantation (TAVI) could vide a risk-adjusted presentation of all cardiac
be coded as early as 2006 with the code «5–35a.0 surgical procedures performed in Germany.
endovascular aortic valve implantation» which was Besides the publication in the Journal of
later subdivided in «5–35a.00 endovascular implanta- Thoracic and Cardiovascular Surgery, selected
tion» and «5–35a.01 transapical implantation.» data are published by the «Deutsche Herzstiftung»
Software that processes administrative data (German Heart Foundation) in «Deutscher
with OPS codes is free to every institution. Each Herzbericht» (German Heart Report) along with
operation is assigned to defined classes according data concerning interventional therapies in
to an open and accessible specification. Germany (7 www.herzstiftung.de).
Since 2004, data can be submitted electroni- For further information, visit 7 www.dgthg.de.
cally, and for each procedure, risk variables can
also be transmitted at the discretion of the institu-
tion. Thus, a risk-adjusted presentation of the 5.2.2 ational Mandatory Quality
N
results is possible and a more detailed sub-analysis Assurance in Germany
can be performed. Currently, not all departments
are using this opportunity; thus, risk adjustment Since 2001, results of isolated aortic valve proce-
is possible only for a subset of data. dures, isolated coronary artery bypass grafting
The top priority of this database is the com- (CABG) and combined aortic valve and coronary
pleteness of annual data collection. For compara- surgery have been reported to the German Head
bility reasons, the basic structure of cardiac Office for Quality Assurance (AQUA-Institute,
operations classification was maintained. It was Göttingen, until 2008; BQS-Institute, Düsseldorf).
subdivided or enlarged when more information For economic reasons, this mandatory quality
had to be reported or new methods have been assurance is restricted to a numerically essential
introduced, always in mind a backward compati- part of the entire cardiac surgery activity. This
98 W. Schiller and J.F. Gummert
type of external quality assurance is a legally German health care including physicians, hos-
required process. pitals, and health insurances. In Germany, these
Historically, this quality assurance initiative self-governing partners have the order to trans-
goes back to the QUADRA study performed in pose legislation into daily practice.
Germany beginning in 1989 (Struck et al. 1990). . Table 5.2 is derived and translated from the
In 2000, the data set was adopted to the defini- Quality Report 2012, AQUA-Institute (AQUA-
tions for EuroSCORE variables as risk-adjusted Institut für angewandte Qualitätsförderung und
outcome measurements had to be performed by Forschung im Gesundheitswesen GmbH 2013). It
means of EuroScore. As observed, mortality rates gives procedural and outcome results for isolated
turned out to be only half of the predicted ones; coronary artery bypass grafting in Germany.
5 new scores for isolated CABG (KCH-Score) and For further information, visit 7 www.sqg.de.
isolated AVR (AKL-Score, published as German
AV-Score) (Kötting et al. 2013) and the combina-
tion of both (KBA-Score) have been developed. 5.2.3 erman Quality Assurance
G
Data analysis is performed on an annual basis, for Congenital Heart Diseases
and reporting to hospitals is accompanied by an
audit of departments with suspicious quality indi- In 2006, the German Society for Thoracic and
cators. In the case of serious irregularities, the Cardiovascular Surgery (DGTHG) together with
clinic will be de-anonymized for the assessment the German Society for Pediatric Cardiology
panel. Thus, an inspection or a professional audit established a task force to develop national quality
will be carried out to bring about quality improve- assurance for congenital heart disease. On the
ment by defining common objectives. If no basis of the EACTS Congenital Database, a data
improvement can be achieved with these arrange- set was defined not only for surgical therapies but
ments, the professional group can recommend a also for interventional treatment. Follow-up data
de-anonymization to the federal commission. of the patients is requested 3 months and 1 year
Data validation is implemented via statistical after the initial operation or intervention.
control measures as well as on-site in randomly After a successful pilot study, the database was
selected hospitals. launched in October 2011. The vast majority of
Today, quality assurance in Germany is man- German institutions performing interventional or
datory for 13 different organs or regions of the surgical treatment for congenital heart disease is
body comprising 31 different types of treatment. participating on a voluntary basis. Hospitals have
Of these, 14 components refer to the cardiovascu- to pay for participation, and the database is spon-
lar system: revascularization of the carotid artery, sored by «Deutsche Herzstiftung» (German Heart
heart transplantation, lung and heart-lung trans- Foundation).
plantation, implantation of a cardiac pacemaker, With this longitudinal database, estimations
exchange of a pacemaker, revision of a pacemaker, of outcome regarding multistage procedures will
implantation of an implantable cardioverter defi- be possible.
brillator, exchange of an implantable cardioverter For further information, visit 7 www.
defibrillator, revision of an implantable cardio- nationale-qs-ahf.de.
verter defibrillator, percutaneous coronary inter-
vention (PCI), coronary artery bypass grafting
(CABG), isolated conventional aortic valve 5.3 he Society of Thoracic Surgeons
T
implantation, isolated transcatheter aortic valve National Database (STS-NDB)
implantation (TAVI), combined coronary artery
bypass grafting and conventional aortic valve After the publication of unadjusted outcome data
implantation, and heart transplantation. on the basis of administrative data by the US
The regulatory authority and contracting Health Care Financing Administration (HCFA)
entity is the «Gemeinsamer Bundesausschuss» in the late 1980s, several databases and risk mod-
(G-BA; Common Federal Committee) which els for risk-adjusted outcome measurements had
is the body of the self-governing partners in been developed. One of these databases is the
..Table 5.2 Isolated coronary bypass grafting in Germany, from Quality Report 2012
2011 2012
332 Utilization of the left internal thoracic artery 93.7 % 94.1 % 32,401 34,417 →
Chapter 5 · Databases in Cardiac Surgery
2256 Postoperative mediastinitis after elective/urgent operation 0.5 % 0.4 % 128 34,398 →
2257 Postoperative mediastinitis in patients with risk class 0 or 1 according to NNIS 0.4 % 0.4 % 114 31,373 →
2259 Neurological complications after elective/urgent operation 0.9 % 0.8 % 277 32,800 →
349 In-hospital mortality after elective/urgent operation 1.9 % 2.0 % 684 34,429 →
11617 Ratio of observed to expected mortality (O/E) 1.00 % 1.04 % 1,177 1,128 39,282 →
3.00 % 2.87 %
353 Status at the 30th postoperative day is known 78.3 % 79.2 % 31,798 40,160 ↗
..Fig. 5.2 The Society of Thoracic Surgeons National Database: screenshot and example of public reporting with
respect to hospitals and surgeon groups on a voluntary basis for isolated CABG; names are erased by black bars.
7 www.sts.org/report-search-group-results-2013 (used with permission)
102 W. Schiller and J.F. Gummert
..Fig. 5.3 Example of bubble chart reporting from the EACTS Congenital Database: mortality score vs hospital mor-
tality for neonates, all procedures. The graphs show the results of one unit (red) over the mean values of the other units
split by hospitals. Each bubble represents one hospital; X-axis, mortality-score, Y-axis, 30d mortality; the bubble sizes are
correlating to the relative case numbers of each institution (Courtesy of B. Maruszewski)
5.4.2 EACTS Adult Cardiac Database tion). The second report was published in
September 2005 with additional data sets from
EACTS Adult Cardiac Database aims at collecting 2003. It included 350,000 data sets of 18 countries,
data on procedures from all European countries and for the first time, Germany participated in the
and to publish them as a comprehensive report. In report. The third report was published in
the future, structure and quality standards that September 2007 using additional data sets from
align closely with those of the STS-NDB will be 2004 to 2005. This report was based on 627,000
implemented. data sets and was the first to analyze total lethality
The first report was based on 220,000 data sets when sufficient data was available. In July 2010,
of procedures from 2001 to 2002 including 12 the Fourth European Association for Cardio-
countries (at that time without German participa- Thoracic Surgery Adult Cardiac Surgical Database
Chapter 5 · Databases in Cardiac Surgery
103 5
Report was published. The report contains infor- tries. NICOR is funded by the Healthcare Quality
mation about over one million patients Improvement Partnership (HQIP), which is a
undergoing adult cardiac surgery in the period up charity contracted by the Department of Health
to the end of 2008. The submissions are from 366 in England to deliver outcome-focused quality
hospitals in 29 countries across Europe and China improvement programs structured around collec-
(Bridgewater et al. 2010). tion of clinical data.
Currently, the major problem of this data- Participation in the National Adult Cardiac
base system lies in its heterogeneous data quality Surgery Audit is mandatory for all National
due to markedly different local structures and Health Service (NHS) hospitals in England and
historically evolved data collecting cultures in Wales as part of the National Clinical Audit
the participating countries (Bridgewater et al. Patient Outcomes Programme (NCAPOP). Data
2010). from the National Adult Cardiac Surgery Audit
Moreover, in some countries, there are legal are used to publish mortality rates for all NHS
considerations concerning data protection, which hospitals in the United Kingdom (and of some
hamper hospitals participation in this registry voluntarily participating private hospitals), which
(Hickey et al. 2013). carry out major adult cardiac surgery. Moreover,
since 2005, individual consultant adult cardiac
surgeon’s mortality rates have been reported to the
public (. Fig. 5.4). Over 90 % of adult cardiac sur-
5.5 The Society of Cardiothoracic geons chose to «opt in» to having their individual
Surgery in Great Britain data published for the most recent publication of
and Ireland’s National Adult these data on the SCTS website (7 www.scts.org/
Cardiac Surgery Audit patients).
and the British Congenital NICOR tracks long-term mortality and re-
Cardiac Association’s interventions centrally by using a unique patient
Congenital Heart Disease identifier (the NHS number), along with other
Audit data set fields such as date of birth.
The Congenital Heart Disease Audit is man-
In the United Kingdom, monitoring of survival aged by NICOR, with professional leadership pro-
rates after cardiac surgery began in 1977 with vol- vided by the British Congenital Cardiac Association
untary submission of data to the Society of (BCCA) and the SCTS. All pediatric cardiac cen-
Cardiothoracic Surgery in Great Britain and ters in the United Kingdom participate in the
Ireland (SCTS). A more detailed National Adult Congenital Heart Disease Audit. The first report
Cardiac Surgery Audit data set was first released was published based on 2000/2001 data from
in 1996 and was later updated in 2003 and 2010. CCAD together with BCCA and SCTS. It contains
Comprehensive reports with analysis of the hospital mortality rates for specific operations or
National Adult Cardiac Surgery Audit data at catheter interventions. Today, mortality rates for
national level were published in the so-called Blue congenital heart procedures are published online
Books. The most recent Blue Book (Demonstrating (7 https://nicor4.nicor.org.uk/CHD/an_paeds.nsf/
Quality) was published in 2009 (Bridgewater et al. vwContent/home?Opendocument) at hospital
2009). «Blue Book» analysis is now available level only, as low procedure rates per surgeon and
online and updated on a regular basis (7 www. per type of procedure mean that mortality report-
bluebook.scts.org). ing on individual surgeon basis would risk making
In 2003, the SCTS agreed to join the Central analysis patient identifiable (. Fig. 5.5).
Cardiac Audit Database (CCAD), which was For further information, visit the following:
established in 1996 in order to coordinate differ- NICOR 7 www.ucl.ac.uk/nicor/audits/
ent data collection activities. CCAD has now SCTS Data set 7 www.ucl.ac.uk/nicor/audits/
become part of the National Institute for Adultcardiacsurgery/data sets
Cardiovascular Outcomes Research (NICOR), SCTS Outcomes data 7 www.scts.org/patients/
based at University College London (UCL), which The Congenital Heart Disease Audit 7 www.ucl.
manages seven cardiovascular audits and regis- ac.uk/nicor/audits/congenitalheartdisease
104 W. Schiller and J.F. Gummert
..Fig. 5.4 Example of public presented outcome data for an individual surgeon (names are not included) (Derived
from 7 www.scts.org/patients/) (used with permission)
Chapter 5 · Databases in Cardiac Surgery
105 5
National average
95 % 2 98 % Control Limit
4
6
90 % 21
99.95 % Control Limit
8 13
9
85 %
Survival at 30 days
10
80 % 18
75 %
70 %
65 %
60 %
55 %
50 %
0 20 40 60 80 100 120
Cases
..Fig. 5.5 Example of public presented outcome data for Norwood stage I procedures (each number represents one
hospital) (Derived from 7 https://nicor4.nicor.org.uk/CHD/an_paeds.nsf/vwContent/home?Opendocument)
5.6.1.2 STS/ACC TVT Registry™ and the need for «intervention for paravalvular
The Society of Thoracic Surgeons (STS) and the regurgitation». All units performing TAVI proce-
American College of Cardiology (ACC) developed dures in the United Kingdom submit data for every
a registry in order to investigate practice patterns consecutive patient where TAVI was attempted. The
and patient outcomes regarding the TAVI p rocedure first long-term results derived out of this database
within an observational period of 30 days and 1 year have been published in 2011 (Moat et al. 2011).
post implant. The sophisticated data set (version 1.2) For further information, visit 7 www.ucl.
of this registry contains all commonly known pre-, ac.uk/nicor/audits/tavi/.
intra-, and postprocedural parameters with cath
and lab findings and a long list of possible compli-
5 cations. Moreover, it comprises preoperative indi- 5.6.2 ational Pediatric Cardiology
N
cation parameters like «Hostile chest,» «Porcelain Quality Improvement
aorta,» or «EuroScore II» as well as the «Kansas City Collaborative (NPC-QIC)
Cardiomyopathy Questionnaire» in its short form
KCCQ-12 and the «Five-Meter Walk Test» preoper- The database of the NPC-QIC is primarily not
atively and in the two follow-ups. As many of these based on cardiac surgery initiatives, and it is not
parameters are not obligatory, the validity of derived a database for quality measurement of cardiac
analyses will depend on the collaboration of partici- surgery procedures. But it is very closely linked
pants. Participation is voluntary and bound to fees to cardiac surgery, as the underlying aim is for it
that have to be paid by each facility. to improve the outcomes of care for children with
For further information, visit 7 www.ncdr. congenital heart disease, and it is the first project
com/TVT. «to improve survival and quality of life in infants
with a single ventricle during the interstage
5.6.1.3 he United Kingdom
T period between discharge after neonatal cardiac
Transcatheter Aortic Valve surgery and admission for bidirectional Glenn.»
Implantation Registry The NPC-QIC is an initiative of the Joint
In the United Kingdom, the British Cardiovascular Council on Congenital Heart Disease (JCCHD),
Intervention Society (BCIS) and the Society of which consists of delegates from four key organi-
Cardiothoracic Surgeons in Great Britain and zations related to pediatric cardiology and liaison
Ireland (SCTS) have developed a data set for TAVI, representatives from different other organizations
as the National Institute for Health and Clinical including the Society of Thoracic Surgery (STS).
Excellence (NICE) suggested that all TAVI proce- The first achievement of this voluntary
dures should be recorded in a national database. multicenter project with the involvement of
Similar to the SCTS National Adult Cardiac parents is the development of clinical and
Surgical Database, this TAVI registry is imple- monitoring processes together with an inter-
mented and managed by the National Institute for stage feeding protocol that promises to
Cardiovascular Outcomes Research (NICOR). improve interstage growth. The project started
This data set contains typical pre-, intra-, and in 2007 with pilot funding obtained from the
postprocedural parameters as well as variables Cincinnati Children’s Heart Association. One
concerning the indication for TAVI like «extensive of the first tasks was to develop the longitudi-
calcification of ascending aorta,» «poor mobility,» nal NPC-QIC database as the basis for the
the Canadian Study of Health and Aging (CSHA) whole project. In contrast to other databases
Clinical Frailty Scale score, or the Katz Index of presented in this chapter, which are used to
Independence in Activities of Daily Living. measure quality of care and deriving conclu-
Follow-up data after 1 and 3 years contain the sions, the NPC-QIC database is primarily a
CCS angina class and the NYHA heart failure class of research tool to improve quality by developing
each patient. Late valve deterioration together with and validating methods.
«valve failure mode» or late paravalvular leakage can For further information, visit 7 www.jcchdqi.org.
be documented together with the date of occurrence For a list of more databases, see . Table 5.3.
Chapter 5 · Databases in Cardiac Surgery
107 5
USA (NH, ME, VT) The Northern New England Cardiovascular 7 www.nnecdsg.org/
Disease Study Group (NNECDSG) database
(voluntary participation)
Rankin JS, He X, O’Brien SM et al. (2013) The society of thoracic Shahian DM, O’Brien SM, Filardo G et al. (2009b) Society
surgeons risk model for operative mortality after multiple of thoracic surgeons quality measurement task
valve surgery. Ann Thorac Surg 95(4):1484–1490 force. The society of thoracic surgeons 2008 cardiac
Shahian DM, Edwards FH (2009) The society of thoracic surgery risk models: part 3—valve plus coronary
surgeons 2008 cardiac surgery risk models: introduc- artery bypass grafting surgery. Ann Thorac Surg
tion. Ann Thorac Surg 88(1):S1 88(1):S43–S62
Shahian DM, O’Brien SM, Filardo G et al. (2009a) Society of Struck E, De Vivie ER, Hehrlein F et al. (1990) Multicentric
thoracic surgeons quality measurement task force. quality assurance in cardiac surgery. QUADRA study of
The society of thoracic surgeons 2008 cardiac surgery the German Society for Thoracic and Cardiovascular
risk models: part 1—coronary artery bypass grafting Surgery (QUADRA: quality data retrospective analysis).
surgery. Ann Thorac Surg 88(1):S2–S22 Thorac Cardiovasc Surg 38(2):123–134
5
109 II
Technical
Prerequisites
Contents
Extracorporeal Circulation
and Myocardial Protection
in Adult Cardiac Surgery
Christof Schmid
References – 150
Chapter 6 · Extracorporeal Circulation and Myocardial Protection in Adult Cardiac Surgery
113 6
6.1 Conduct of Cardiac Surgery ..Table 6.1 Necessary pump flows related to the
with Extracorporeal body temperature
Circulation
Body temperature Pump flow L/min/m2
Adult cardiac surgery can be performed in different Normothermia 2.2–2.6
ways. For the majority of cardiac surgical proce-
dures, extracorporeal circulation is employed, i.e., a Mild hypothermia (32–35 °C) 2.0
standard heart-lung machine is connected, whereas Moderate hypothermia 1.5
some operations such as off-pump coronary artery (26–31 °C)
bypass surgery or the transcatheter aortic valve
replacement (TAVR) can be carried out without it
(see also 7 Chapter «Minimally Invasive Surgery», (SVR). Under normal perfusion conditions, the
Sect. 26.3.4). Minimized heart-lung machines basi- central venous saturation (SvO2) should be >65 %,
cally consisting of a combined pump-oxygenator and the serum lactate should be normal. A
provide another tool, which is predominantly used decreasing SvO2 during extracorporeal circula-
for coronary artery bypass surgery and in a few tion indicates insufficient circulatory support.
centers for aortic valve replacement too. Cerebral autoregulation keeps cerebral perfu-
sion constant for an arterial blood pressure range
of 50–150 mmHg. As brain metabolism decreases
6.1.1 Physiology to as low as 40 % of normal during anesthesia, a
During Extracorporeal perfusion pressure of 40–60 mmHg at full flow suf-
Circulation fices during normothermia or mild hypothermia.
In elderly hypertensive patients and those with sig-
The purpose of extracorporeal circulation is the nificant carotid artery stenosis, however, one is
maintenance of peripheral perfusion and gas inclined to maintain the perfusion pressure some-
exchange during open-heart surgery and in car- what higher to prevent ischemic complications.
diopulmonary failure. Therefore, extracorporeal The oxygenator of the heart-lung machine
circulation has a profound influence on the hemo- usually contains a heat exchanger, which allows
dynamic status and the oxygenation/acid-base cooling and rewarming of a patient with a hypo-/
status of the patient. The hemodynamic status is hyperthermia system. Between 22 and 37 °C,
optimized via the pump flow, whereas the gas cerebral perfusion remains constant, again due to
exchange is adjusted with the oxygenator. The autoregulation, and drops below 22 °C down to
blood flow over artificial surfaces necessitates 15 % of normal. For that reason, the pump flow of
anticoagulation and compromises end-organ the heart-lung machine can be lowered to 1.5 L/
function with time. A prolonged cardiac arrest min/m2 at a temperature of about 28 °C and even
requires cardioplegic myocardial protection. further with more pronounced hypothermia.
The benefit or harm of total body hyperperfu-
6.1.1.1 Hemodynamics sion is still unclear. In vasoplegic or septic patients,
The main task of circulatory support is the main- who normally present with high cardiac output
tenance or reestablishment of a physiological sys- and high central venous saturation, respectively, it
temic perfusion. As the required cardiac output is probably advantageous to offer a maximized
(CO) is related to the patient’s body size and pump flow. So far, high pump flows have been
shape, the necessary pump flow has to be normal- successfully offered only in patients with a total
ized to the body surface (. Table 6.1). artificial heart, but this phenomenon has not been
The pump flow aimed at for standard adult analyzed in proper studies (Copeland et al. 2003).
open-heart surgery employing extracorporeal cir-
culation is commonly defined to 2.4 L/min/m2. In 6.1.1.2 Pulsatility
miniaturized systems, less hemodilution allows The significance of pulsatility during extracorpo-
for a lower pump flow. The regulation of the mean real circulation was a matter of research for
arterial pressure is achieved by adjusting the many years. Frequently, there is an erroneous
pump output and the systemic vascular resistance assumption that a roller pump generates only a
114 C. Schmid
ECMO, the arterial saturation would not raise flow correlates with cerebral oxygen consumption
higher than 95 % and would be typically even during hypothermia as the cerebral auto regula-
lower, PaO2 would be only around 40 mmHg, and tion remains intact. It is the temperature regula-
the patients would be cyanotic and hypoxic tion type in poikilothermic animals, where the
(Bartlett 2005). However, normally, cardiac output degree of ionization of several important enzymes
in these patients is compensatorily increased and remains intact. Accordingly, the α-stat manage-
the systemic oxygen supply sufficient. An improve- ment seems to be more physiologic and is mostly
ment of lung function increases arterial oxygen- preferred in adult perfusion. The advantage of the
ation, so that recovery of native pulmonary pH-stat approach, which is found in hibernating
function can be monitored by the difference of animals, is the better cerebral perfusion due to the
arterial and venous saturation. carbon dioxide-related vasodilatation. This tech-
As in the native lung, carbon dioxide elimina- nique is partially favored in pediatric surgery (see
tion in the oxygenator is much more efficient than also 7 Chapter «Advances in Cardiopulmonary
6 oxygen uptake. Carbon dioxide elimination dur- Bypass for the Neonate and Infant», Sect. 7.3.2.2).
ing extracorporeal circulation is (as in the native
lung) mainly determined by the properties of the
6.1.2 Anticoagulation During
oxygenator and hardly by the pump flow. An
Extracorporeal Circulation
increase of the membrane surface or the gas flow
improves carbon dioxide elimination, but not the
oxygen uptake. Intact vessel endothelium has active and passive
In both venoarterial and venovenous ECMO, antithrombogenic properties such as the release
carbon dioxide (but not oxygen) levels can be of prostacyclin and endothelium relaxation factor,
adjusted to any desired level by choosing the which inhibit platelet aggregation. It also activates
respective membrane surface and the appropriate the physiologic anticoagulant protein C and inac-
gas flow. In clinical practice, the oxygenator is tivates thrombin.
usually oversized, and increased carbon dioxide The artificial surfaces inside the oxygenator,
elimination with a respiratory alkalosis can the reservoir, the pump, and tubing (PVC, poly-
develop if the gas flow is not reduced. This overdi- urethane, silicone) have no endothelial coverage
mension can be useful for long-term use when the and are thrombogenic. There is immediate plate-
gas exchange surface gradually declines. let adhesion and consecutive thrombus forma-
tion. An activation of the coagulation cascade, the
6.1.1.5 pH Management: α[Alpha] kinin-kallikrein system, the fibrinolytic system,
Stat and pH Stat and the complement system follows. The intrinsic
The gas exchange by the oxygenator does not only coagulation pathway is initiated by the factor XII
allow sufficient oxygenation and elimination of high-molecular-weight kininogen-prekallikrein
carbon dioxide, it also has a profound impact on complex, whereas the extrinsic cascade is acti-
the acid-base balance and the blood pH. As vated with the release of tissue phospholipids. The
changes in the acid-base balance and blood pH median sternotomy also releases tissue thrombo-
have significant consequences in the physiology plastin, which activates platelets as well. The
of the circulation, close monitoring and an ade- hemodilution during extracorporeal circulation
quate management are crucial. further reduces the concentration of
There are two possibilities for the pH and anticoagulation factors, platelets, and physiologic
pCO2 management. With lower body tempera- anticoagulants protein C, protein S, and anti-
tures, pCO2 drops and the pH increases with thrombin III.
0.017 °C, i.e., at 25 °C the pH is 7.6. Employing the Due to the activation of the coagulation sys-
α-stat management, the pH changes are not coun- tems by the surgical procedure and the use of the
terbalanced; a relative alkalosis is the conse- heart-lung machine, a strict anticoagulation is nec-
quence. With the pH-stat approach, the essary. This is generally accomplished with heparin
temperature related pH changes are counterbal- at a dose of 300–400 IE/kg. This dose is termed
anced with an increased carbon dioxide applica- «full heparinization.» During surgery, anticoagula-
tion to maintain pH as in respiratory acidosis. In tion is monitored with the activated clotting time
case of the α-stat management, the cerebral blood (ACT). An ACT of 350–450 s is c onsidered ade-
Chapter 6 · Extracorporeal Circulation and Myocardial Protection in Adult Cardiac Surgery
117 6
quate. In case of heparinized minimized extracor- 6.1.2.2 Heparin-Induced
poreal systems, an ACT of >250 s seems sufficient. Thrombocytopenia (HIT)
After termination of cardiopulmonary bypass, a and Heparin Analogues
1:1 antagonization of heparin with protamine nor- During treatment with heparin, thrombocytope-
malizes the ACT to a level of about 120 s. nia can develop, where two different types can be
distinguished:
6.1.2.1 Heparin 55 Heparin-induced thrombocytopenia type 1
Heparin was discovered at the Johns-Hopkins (HIT 1): 2–4 days after the start of heparin
University in Baltimore in 1916 and was intro- treatment, the count of platelets mildly
duced into clinical practice in the 1930s (McLean decreases due to direct activation. Within a
1916). In 1939, it became evident that a plasmatic few days, the number of platelets spontane-
factor is necessary for the anticoagulant action of ously normalizes without therapeutic means.
heparin. The identification of this factor, termed The exact incidence is unknown. In the liter-
antithrombin III, succeeded not before the 1970s ature, reported incidences vary from 1 to
(Brinkhous et al. 1939). 25 % with unfractionated heparin, while
Standard heparin (unfractionated heparin, LMWH heparins are told to have a lower
UFH, chain length ≥18 saccharides) is a mix- risk.
ture of polysaccharides (negative loaded sulfated 55 Heparin-induced thrombocytopenia type 2
glycosaminoglycans) and is mainly obtained (HIT 2): The HIT 2 occurs less often and
from porcine small bowel and bovine lungs. manifests after 4–14 days with a much more
Fractionated low-molecular-weight heparin dramatic drop of platelet numbers. The
(LMWH, chain length 5–17 saccharides) has a underlying pathophysiology bases on an anti-
shorter chain length and differs with regard to the body formation against heparin bound to
various coagulation factors. antithrombin III. In about 75 % of cases, the
Both types of heparin bind antithrombins heparin-platelet factor 4 (H-PF 4) complex is
(AT), predominantly AT III, by which a complex the causative antigen. The antibody, mostly
is formed, which accelerates the activity of AT III IgG, recognizes H-PF 4 and activates platelets
a thousand times. UFH acts faster as LMWH, via the Fc receptor leading to platelet aggrega-
since it inactivates not only the prothrombinase tion. Despite the thrombocytopenia, bleeding
complex (consisting of the activated factor X, acti- complications are infrequent, whereas throm-
vated factor V, calcium ions, and phospholipids) boses with so-called white clots are reported
as does LMWH but also thrombin. Further mech- to form in arterial and venous vessels in
anisms of action include the inactivation of the 50–70 % of cases (Greinacher et al. 2003). HIT
factors IX, XI, XII and kallikrein, and the binding 2 is a life-threatening complication with a
of calcium ions, which augments the anticoagu- mortality rate of more than 20 %.
lant properties. Because of the different effective-
ness, the heparin dosage is standardized in Patients with a HIT 2 syndrome must not be
international units (IU) and not in milligram treated with heparin, i.e., heparin therapy has to
(mg). One unit of heparin prevents coagulation of be stopped immediately. Anticoagulation can
1 mL citrate plasma after addition of calcium chlo- only be performed with alternative drugs, of
ride at 37 °C for an hour. AT III is substituted at which a few are available (Magnani 1993;
levels <50 % of normal because of its importance. Warkentin and Greinacher 2003) (. Table 6.2):
It is noteworthy to recognize, that thrombin is 55 Argatroban (Argatra™): Argatroban is a syn-
not totally inactivated during extracorporeal cir- thetic direct thrombin inhibitor (arginine
culation despite high dosages of heparin. As a analogue), which is only used in patients with
consequence, thrombus formation may occur in HIT 2. The dosage is adjusted with the aPTT,
the clinical setting despite full heparinization. which is 1.5–3 times augmented. The metabo-
Apart from the anticoagulation properties, lism is mostly hepatic and elimination over
heparin exhibits various other traits. It increases the feces. Therefore, renal failure is not prob-
fibrinolysis by release of tissue plasminogen acti- lematic. As half-life is only 50 min, argatroban
vator (tPA) and has an anti-inflammatory effect by is the mostly used alternative drug to replace
hindering granulocyte migration into the tissue. heparin, also in ECMO therapy.
6
118
C. Schmid
Anticoagulant drug Mechanism of action Neutralization Administration and Control parameter and Elimination and half-life (HL)
dosage therapeutic range
Heparin, UFH Inhibitor of thrombin and Protamine 1:1 I.v. bolus: 300–400 U/kg ACT >400 s (40 %) Binds to endothelium,
prothrombinase complex macrophages, and plasma
proteins −5–15 min, thereafter
renal elimination
HL: 60–90 min
Argatroban (Argatra) Synthetic direct thrombin Not possible I.v. bolus: 0.3–1 mg/kg ACT >400 s Liver, HL: 52 min
inhibitor (arginine analogue)
Infusion: 2–40 μg/kg/min
Bivalirudin (Angiomax) Synthetic direct thrombin Not possible I.v. bolus: 1 mg/kg Kaolin-ACT >400 s, ecarin Proteolysis, kidney c. 20 %
inhibitor (arginine analogue) clotting time (ECT):
Infusion: 2.5 mg/kg/h 400–500 s HL: 25–34 min
Danaparoid (Orgaran) Anti-factor Xa Not possible Factor Xa: 1.5 ± 0.3 U/mL Kidney: 40–50 %
HL (anti-Xa): 19–24 h
Lepirudin (Refludan) Direct thrombin inhibitor Not possible I.v. bolus: 0.25 mg/kg Ecarin clotting time (ECT) Catabolic hydrolysis kidney
c.50–60 %
Drug Mechanism of platelet inhibition Reversibility Administration Elimination half-life Recovery of platelet
function
Aspirin COX-1 inhibitor Irreversible Oral (1× daily) Production of new platelets
Thienopyridine ADP P2Y12 receptor antagonist Irreversible Oral (1× daily) Production of new
platelets
Ticlopidine
Clopidogrel
Prasugrel
Ticagrelor ADP P2Y12 receptor antagonist Reversible noncompetitive Oral (2× daily) 7 h 80 % recovery by 72 h
Abciximab Fibrinogen receptor antagonist (GP IIb/IIIa) Monoclonal antibody Intravenous ≥50 % recovery by 48 h
Eptifibatide Fibrinogen receptor antagonist (GP IIb/IIIa) Reversible Intravenous 2,5 h 50 % recovery by 4 h
Tirofiban Fibrinogen receptor antagonist (GP IIb/IIIa) Reversible Intravenous 2 h 4–8 h
surgical procedures with a heart-lung machine a barrier by an uptake of water, allowing the blood
have been described. proteins to move along without adherence and
denaturation. Platelet adhesion is inhibited too.
6.1.2.4 Surface Coating
The aim of surface coating (. Table 6.4) is to aug-
ment biocompatibility of foreign material, i.e., to 6.1.3 Myocardial Protection
lower the risk of thrombus formation. Since many
years, several different techniques of surface coat- The aim of myocardial protection is to create con-
ing, mainly various types of heparin coating, are ditions which allow a surgical procedure on the
tested experimentally and in clinical practice. heart while myocardial integrity and function are
Distinct advantages and disadvantages of the preserved. The optimal conditions are not the
coating techniques are not evident, so far. same for all operations but depend on the pathol-
However, it seems important to achieve complete ogy of the heart and the surgical requirements to
coverage of the whole extracorporeal circuit from repair it. Finally, there is always an individual
«tip to tip» as only that allows abdication of full compromise between optimal surgical conditions
heparinization. As many adverse events manifest and ideal myocardial protection (Guyton 1995).
only after longer-term extracorporeal circulation,
the value of surface coating in the daily routine of 6.1.3.1 Concepts of Myocardial
extracorporeal circulation is controversially dis- Protection
cussed. Several concepts have been devised for myocar-
For heparin coating, heparin is covalently dial protection, which can all be employed con-
bound to the artificial surface in order to mimic secutively (Guyton 1995):
the antithrombogenic properties of heparan sul-
fate on the natural endothelium. The exact mech- kMyocardial Protection Prior to Cardiac
anism of action of the bound heparin is still Arrest
unclear, but a diminished activation of comple- The induction of anesthesia in patients with
ment, of proinflammatory cytokines, and of plate- impaired left-ventricular pump function or coro-
lets has been widely demonstrated. nary heart disease is more dangerous than in
Polypeptides are attached to the artificial sur- healthy patients. Several reports have shown that
face with electrostatic forces and van der Waals an injury of myocytes starts already before initia-
forces. As a consequence, the surface is hydrophi- tion of extracorporeal circulation in 18 % of
lized and more rapidly moistened, which hinders patients (Delva et al. 1978). As many patients
the adhesion of plasma proteins. reach the operation room in a suboptimal status
The polymer coverage X-coating (PMEA = (agitation, tachycardia, hypoglycemia, hypovole-
poly(2-methoxyethylacrylate)) consists of two lay- mia) and are thus opposed to an increased risk, a
ers. A hydrophobic layer binds to the foreign sur- careful hemodynamic and pharmacological opti-
face; the hydrophilic layer is at the luminal side and mization should be performed prior to surgery in
in contact to the blood. The hydrophilic layer forms elective cases.
122 C. Schmid
The decline of mean arterial pressure during management at the membrane oxygenator allows
extracorporeal circulation below 60 mmHg can faster cooling, as it is our practice for years. Yet,
lead to subendocardial ischemia even in healthy the temperature difference between heat
patients. Accordingly, special care has to be taken exchanger and blood in the oxygenator should
to maintain sufficient perfusion pressure in not exceed 10 °C, even if scientific evidence is
patients with coronary artery disease. If neces- lacking. If deep hypothermia below 20 °C is aimed
sary, perfusion pressure should be augmented at, e.g., for aortic arch surgery, administration of
with α-adrenergic drugs like norepinephrine, an α-blocker can be helpful as it enables a more
despite the risk of significant side effects. evenly and faster temperature decline. During
Furthermore, the heart-lung machine implies deep hypothermia, a circulatory arrest is relatively
hemodilution, which also may lower subendothe- well tolerated for up to 45 min.
lial perfusion (Kleinman et al. 1978). Hypothermia Historically, rewarming was even more care-
can impair autoregulation of local blood flow and fully performed than cooling, since the high pO2
6 translate into local hypoperfusion. In hypertro- in bubble oxygenators could lead to gas embolism.
phied hearts and those with coronary artery ste- Since introduction of membrane oxygenators and
nosis, ventricular fibrillation can also favor a precise pO2 management to achieve 150 mmHg
subendothelial ischemia. The same is true for at the outlet, the formation of microbubbles is
overdistension of the ventricle. Therefore, an much less, and therefore a faster rewarming is pos-
immediate vent placement should follow if a dis- sible. Nevertheless, blood temperature in the oxy-
tended ventricle cannot be rapidly arrested by genator should not exceed 38 °C.
clamping the aorta. The underlying pathophysiology during hypo-
thermia and rewarming are only partially known.
kLowering the metabolic rate during cardiac With cooling, the metabolism responds rather
arrest inconsistent, which also leads to a significant
After optimal preparation for the ischemic arrest, alteration of myocardial homeostasis. This means
the latter should be withstood in the best way pos- that the supply of the hypothermic heart with
sible. For the myocardium, rapid surgery with oxygen and substrates does not maintain normal
brief ischemia is best—this, however, is rarely cellular function (Cameron and Gardner 1988). A
possible. Therefore, the heart is protected with further disadvantage, especially for deep hypo-
hypothermia and cardioplegia. thermia, is a significant postoperative coagulopa-
It is long known that the heart can be pro- thy and cardiac edema formation.
tected with hypothermia. In the nineteenth cen- Cardioplegia is more effective in protecting
tury, Van’t Hoff demonstrated a decline of the heart than hypothermia alone. The oxygen
myocardial metabolism of 50 % for every 10 °C consumption of an arrested heat is only about one
drop in cardiac temperature. Thus, the use of fifth of a normal beating heart at 37 °C. The com-
hypothermia during cardiac surgery was sug- bination of hypothermia and cardioplegia poten-
gested early and initially applied to the whole tiates cardiac protection. Studies have shown that
body. Later, surface cooling with irrigation of cold even rather cold solutions (2 °C) do not harm the
saline was introduced. myocardium, but the cooling of heart with infu-
Simple cardiac surgical cases are operated upon sion of cold solutions is quite heterogeneous,
with adequate cardioplegia in normothermia. In especially in patients with coronary artery dis-
more difficult cases with extended ischemic times ease. A topical cooling may help to create a more
exceeding 1.5–2 h, hypothermia around 30 °C is uniform hypothermia. The optimal myocardial
mostly favored. Only if a long ischemic time temperature is still discussed controversially.
beyond 2 h is anticipated, hypothermia below While crystalloid cardioplegic solutions are
28 °C is recommended. Nowadays, even rather applied with 4 °C, the assumed temperature opti-
complex operations with ischemic times of 3 h can mum for blood cardioplegia is higher (15–20 °C).
be performed safely if myocardial protection is Even normothermic blood cardioplegia protocols
adequate. have been established in some institutions (Abah
Many earlier publications emphasized a cool- et al. 2012). Due to the higher temperature range,
ing rate not to exceed 1 °C/min for the patient. blood cardioplegia requires reinfusion about
This is no longer true as an improved gas exchange every 20 min.
Chapter 6 · Extracorporeal Circulation and Myocardial Protection in Adult Cardiac Surgery
123 6
kA Favorable Metabolic Milieu Increases reperfusion («hot shot») was to limit reperfusion
Safety During Cardiac Arrest damage (1977). The rationale for this cardiopro-
During cardioplegic arrest, cardiac metabolism is tective action is that amino acids like glutamate
altered. Accordingly, it is important to maintain and aspartate play an important role in myocar-
an adequate metabolic milieu during cardioplegic dial intermediary metabolism and their relative
arrest. This task is in part accomplished with car- importance is further enhanced during and after
dioplegia in various ways: ischemia (Caputo et al. 1998). Before the release
1. By minimizing interstitial and intracellular of the aortic cross-clamp, a normothermic
edema formation substrate-enriched blood cardioplegia is applied,
2. By hindering loss of cellular metabolites mostly via the coronary sinus to actively resusci-
3. By supplying sufficient substrates for the tate the ischemically damaged, substrate-depleted
metabolism myocardium by maximizing the kinetics of repair.
4. By preserving an equated acid-base balance Thereafter, reperfusion with normothermic blood
follows until cardiac activity resumes (Ascione
Frequently, the metabolism is not properly et al. 2008). Considering the induction of cardio-
kept up, as coronary artery stenosis and myocar- plegia as first phase of reperfusion, warm cardio-
dial hypertrophy lead to regional underperfusion plegic induction was extensively studied in 1983
and hyperperfusion. Moreover, cardioplegia can to prove the «active resuscitation» of the heart
be washed out in part by collateral flow. In this (Buckberg 1995) (. Table 6.5).
regard, blood cardioplegia seems advantageous as The techniques of delivery include single and
blood is the natural perfusion media with all multidose, continuous, antegrade, and retrograde
nutritive elements and excellent buffering proper- (either, both alternatively or simultaneously). For
ties. In the same way, reconvalescence of ischemic single-dose cardioplegia application, low- and
myocardium with blood cardioplegia is probably high-volume concepts exist, which means that
superior (see below). some centers administer less than 1000 mL and
others more than 2000 mL. The rationale for mul-
kControlled Reperfusion Lowers Structural tidose cardioplegia derives from the occurrence
and Functional Myocardial Damage of noncoronary collateral flow, which replaces the
The problem of reperfusion injury caused by oxy- carefully formulated cardioplegic solution with
gen free radicals and a massive increase of endo- systemic blood at the temperatures prevailing in
thelial permeability with cellular edema the surrounding systemic arteries. Additional
formation is well known. The increasing tissue benefits of multidose cardioplegia are formula-
pressure hinders reperfusion, and the tissue tions that include buffering and hypocalcemia
edema leads to an increased diffusion distance which may limit reperfusion damage during sub-
through the tissue. Both worsens metabolism. sequent doses (Buckberg 1995).
Several concepts for controlled reperfusion with Microplegia is a more recent concept. It is
warm and cold, low calcium, and substrate- marketed as a cost-effective strategy utilizing
enriched solution have been designed and pres- undiluted blood with targeted amounts of car-
ent advantages in experimental and clinical dioprotective additives, adjustable to meet the
studies. However, there is still no defined opti- changing requirements of each patient during
mum and no concept for daily routine established cardioplegia procedures (Menasché 1996, 1997).
for controlled reperfusion after cardiac ischemia As it delivers blood from the circuit and additives
(Mohan Rao and Simha 2011). for cardioplegia with minimal crystalloid, it is
sometimes called blood cardioplegia without the
6.1.3.2 Cardioplegia Strategies crystalloid. Standard blood cardioplegia contains
Over the years, a wide spectrum of cardioprotec- crystalloid in a 4:1 or 8:1 dilution. The constitu-
tive (protecting the myocardium) strategies has ents of microplegia total 1.5 mL of crystalloid for
evolved. Multidose crystalloid cardioplegia was each 100 mL of circuit blood, equalling a ratio of
introduced in 1976. Cold blood cardioplegia fol- 66.6:1.
lowed in 1978 with the idea to add myocardial Regardless the delivery technique, no superi-
nourishment to the substrate-depleted heart ority of either could be proven. As a consequence,
(Buckberg 1995). The idea of warm cardioplegic cardioplegia administration is far from being
124 C. Schmid
Mannitol 30.0 mmol/L
Tryptophan 2.0 mmol/L
6
St. Thomas solution Sodium chloride 110.0 mmol/L
Raffinose 30.0 mmol/L
Adenosine 5.0 mmol/L
Glutathione 3.0 mmol/L
Allopurinol 1.0 mmol/L
Heparin 1000 U/L
Lactobionate 80.0 mmol/L
Mannitol 60.0 mmol/L
Histidine 30.0 mmol/L
Glutamate 20.0 mmol/L
Glutathione 3.0 mmol/L
Arterial shunt
sensor Arterial
filter Bubble
Bubble detector
cardiopiegia
Continuous Cardioplegia
From
blood parameter delivery system
oxygenator
monitor
Hemoconcentrator
Oxygenator
Cooler/heater
Leukocyte
reduction filter
Centrifugal pump
(or roller pump)
Arterial suction vent Cardioplegia
Temperature control and Heart-lung machine
monitoring system
..Fig. 6.1 Configuration and setup of a standard heart-lung machine (Courtesy of Terumo)
128 C. Schmid
a b
..Fig. 6.4 Oxygenator with semi-permeable silicone membrane a and microporous membrane b (Courtesy of Maquet,
Sorin and Medtronic)
..Fig. 6.5 Venous reservoir: hard shell (left) and soft bag (right) (Courtesy of Maquet)
132 C. Schmid
Most reservoirs have an integrated blood fil- trap, which accumulates the bubbles in the mid-
ter. It is composed of Dacron wool or polyure- line stream and eliminates them from there.
thane foam and catches particles from 20 to Bubble traps and arterial filters are available as
100 um, depending on the filter size. An incorpo- single components as well as an integrated part of
rated bubble trap allows elimination of air bub- a complete system, e.g., cardioplegia sets. The
bles. Furthermore, the venous reservoir offers sizes have to be adapted because of priming vol-
access for drug application and blood sample umes and flow limitations in very small patients
withdrawal. only. The efficacy of bubble traps and arterial fil-
Technically, reservoirs can be constructed as a ters is judged rather inconsistently. However,
rigid container or a soft bag. The rigid systems are most studies demonstrate a reduction of air
advantageous as they are larger, and volume esti- bubbles and microembolism in transcranial
mation and air elimination are simpler. Likewise, Doppler sonography measurements.
priming and vacuum-assisted drainage are easier.
6 6.2.1.5 Gas Blender
6.2.1.4 Bubble Trap and Arterial Filter With only few exceptions, oxygen and carbon
Bubble traps are incorporated as first element into dioxide transfer is determined by a gas blender.
the venous line, i.e., prior to the pump and oxy- Gas flow can be altered from 0 to 16 L/min, and air
genator. Blood and air bubbles are split up by can be supplemented so that an oxygen tension of
gravity and centrifugal forces, and the blood addi- 21–100 % can be offered to the patient. A high
tionally passes a membranous sieve with a poros- oxygen concentration leads to a higher pO2 and
ity of 40–200 um. The priming volume is about consecutive enhanced oxygen transfer. In contrast,
150 cm3, which is not problematic in a normal carbon dioxide transfer is governed by the gas
heart-lung machine but is of significance in a flow. A higher gas flow accelerates carbon dioxide
mini-ECC setting. Special air removal devices washout by better maintaining the pCO2 pressure
consist of combined bubble detection and bubble difference. Another way to regulate carbon diox-
elimination, which functions as an automatic pro- ide exchange is to admix it to the sweep gas.
cess in a rather effective manner.
Arterial filters are integrated into the arterial 6.2.1.6 Cannulas and Tubing
line prior to the blood returning into the patient. Various cannulas (. Fig. 6.6) are available to con-
They also have a sieve with a pore size of 20–40 um, nect an extracorporeal circuit to a patient.
trapping air bubbles and small solid particles. A Standard cannulas are manufactured by a trans-
peculiar variant is the so-called dynamic bubble lucent flexible synthetic material. The cannula
a b
..Fig. 6.7 Cannulation of ascending aorta. A purse-string suture is placed and the cannula inserted after a stab
incision a or with the aid of a small Satinsky clamp in children b
a b
..Fig. 6.8 Exposition of the superior caval vein a, the inferior caval vein is provided with a tourniquet b
Suspicion for any LSVC should arise when no much higher than 15 mmHg, simple clamping
or only a diminutive brachiocephalic vein or an may be allowed during ECC. Otherwise, clamp-
unusually large/dilated coronary sinus is visible. ing of the vessel could lead to venous stasis and
In presence of a brachiocephalic vein, it may finally to a cerebral impairment. In these cases,
sufficiently drain the LSVC when temporarily or the left superior caval vein should be cannulated,
even definitely occluded. If probatory clamping of either directly close to the left atrial appendage or
a LSVC without visible connection to the right via the coronary sinus. A retrograde cardioplegia
side does not result in a left-sided caval pressure has to be adapted to the anatomical findings.
Chapter 6 · Extracorporeal Circulation and Myocardial Protection in Adult Cardiac Surgery
135 6
a b
..Fig. 6.9 Cannulation of the superior caval vein via the atrial appendage a; both caval veins are placed b
Absent or dilated coronary sinus may not allow Peripheral cannulation is an alternative to
for administration of retrograde cardioplegia. central cannulation. In standard surgical proce-
The size of the aortic cannula is kept small to dures with median sternotomy, it is no longer
avoid extensive laceration of the aortic wall. used, except for emergency situations including
However, the tip of the cannula is the narrowest severe bleeding, cardiac arrest, acute aortic dis-
part, where pressure drops and jets and section, reoperation, and minimally invasive
turbulences develop which can injure the aortic procedures. During recent years, the cannula-
wall from inside. Venous cannulas are chosen as tion of the subclavian artery has proven very
large as possible to guarantee optimal drainage advantageous in patients with acute aortic dis-
from the heart. According to Hagen-Poiseuille’s section, since this vessel is only rarely involved
law (at laminar flow), the resistance correlates in the disease, giving unrestricted flow access to
with the 4 power of the radius of the cannula. the aortic arch. Mostly, the vessel is not directly
Therefore, a short cannula with a large diameter cannulated. Instead, a Dacron graft (size
allows for maximal pump flow. 6–8 mm) is anastomosed to it in an end-to-side
At the arterial side, blood is propelled by the manner, and a straight aortic cannula is tied into
pump, i.e., flow resistance in cannula and tubing it. Lacerations of the subclavian vein and the
have to be overcome. The pressure in the arterial brachial plexus may occur but are infrequent.
line rises in relation to the pump flow. Usually the Apart from the subclavian vessels, iliac and fem-
flow is set, and the resultant pressure is measured. oral arteries as well as the jugular vein may serve
Line pressures up to 300 mmHg are tolerated. as access for extracorporeal circulation. Arterial
Systemic relationships of flow and pressure in cannulas can be rather short; venous cannulas
cannulas and tubing are expressed with the help should be long enough to reach the right atrium
of the so-called M-figure. Small cannulas have a in order to prevent collapse of the inferior caval
high shear stress which destroys blood cells. The vein resulting in poor drainage.
critical limit of shear stress dependent hemolysis In contrast to intrathoracic cannulation, a
is unknown; in the literature, values from 80 to peripheral access with a single venous cannula
400 N/m2 have been reported. The limit for plate- usually does not permit total bypass, i.e., complete
lets is significantly lower with 10–35 N/m2. The mechanical support. Only 80 % of blood volume
venous blood is mainly drained by gravity, where can be drained via peripheral cannulas, whereas
the central venous pressure and the hydrostatic 20 % of blood volume passes the lung. This means
pressure (difference in height between patient and also that the maximal pump flow is lower as com-
reservoir) are the driving forces. With a known pared to central cannulation (3.5–4 L/min). With
blood viscosity (c. 3 cP), the necessary minimal a femoral perfusion and a still ejecting heart, the
cannula size can be chosen. arterial ECC blood hardly reaches the aortic arch.
136 C. Schmid
a b
..Fig. 6.10 Cardioplegia cannula with sidearm for venting a, coronary sinus catheter b
Venous drainage can be improved with suction by measurement of the aortic pressure during car-
integrating a roller pump or centrifugal pump dioplegia delivery. In case of retrograde cardio-
into the venous line or by connecting the hard plegia administration, a balloon catheter is placed
shell reservoir to a vacuum. Venous suction for ventrally to the venous cannula with a purse-
optimizing drainage has several advantages: (1) string suture and into the coronary sinus. There
unloading of the right atrium and ventricle is are self-inflating balloons and those to be manu-
improved; (2) the cannula size can be reduced by ally inflated with the aid of a syringe. Catheter
25 %, which is very usefully in peripheral cannula- placement is simple and can be achieved even if
tion; (3) the extracorporeal circulation is less sus- only the right atrium and ventricle are prepared in
ceptible to an air block after lacerating of the right redo procedures. Its highest value of retrograde
atrium; and (4) procedures at the pulmonary catheters is in aortic root surgery as well as in
artery can be performed with a two-stage cannula. coronary reoperations. Correct positioning can
The term «total bypass» is used when on full be judged best by digital palpation of the balloon
ECC flow, all systemic venous flow is forced into just below the left atrial appendage. Failure of
the venous cannulas by closing the tourniquets catheter placement is rare and then caused by a
around the individual caval cannulas. This term Chiari net (remnant of the embryologic large
may not be applicable for two-stage venous can- valve of the sinus venosus) or an extraordinary
nulation, although it may come close with excel- small coronary sinus. After initiation of extracor-
lent drainage. poreal circulation, a constant flow from the cath-
eter must remain evident, despite proper venous
6.2.1.7 Cardioplegia Administration drainage and low central venous pressure. If the
For the application of cardioplegia, a large bore heart is lifted up during retrograde cardioplegia,
sharp needle is sufficient. Alternatively, special swollen veins including the posterior descending
catheters can be inserted and fixed with a suture. vein (V. cordis media) should be visible
Most of the latter have a sidearm, which allows (. Fig. 6.10).
Chapter 6 · Extracorporeal Circulation and Myocardial Protection in Adult Cardiac Surgery
137 6
6.2.1.8 Vent Placement 6.2.1.10 Open and Closed Systems
With institution of extracorporeal circulation, In open systems, the venous reservoir can be de-
pulsatility decreases and with good drainage may aired during operation. Mostly, the venous reser-
finally cease on full flow and/or total cardiopul- voir is built with a rigid housing and an integrated
monary bypass and an empty heart. However, as defoamer. The latter allows removal of air in a
bronchial and Thebesian veins drain into the left simple manner, but if the blood levels drop below
atrium and ventricle even with total bypass, the a critical level, air embolism may occur. Therefore,
left ventricle will fill and distend, or blood will a blood level sensor is necessary, which not only
spill into the operative field if the heart is entered monitors the level and alarms in time but also
and not vented after cross-clamping. stops the heart-lung machine automatically.
A vent catheter usually has a mandrel or a Closed venous reservoirs are flexible bags
reinforced but flexible tip, which facilitates its which can crumple. Air is trapped on top of it and
placement. Main indication is a left-sided valve can be removed via a valve. If the blood levels
replacement or LV aneurysm resection. Thus, the decrease, the walls of the bag collapse and prevent
operative field is kept bloodless, and de-airing the dislodgement of critical amounts of air to the
heart at the end of the procedure is easier. patient.
Standard approach is the right superior pulmo- Reliable data suggesting preference for one
nary vein. Cannulation through the interatrial system over the other are not available; both sys-
groove just anterior to the pulmonary vein may tems are on the market with the rigid housing/
prevent pulmonary vein obstruction in the rare open system taking the larger share.
event of tears in the vein requiring extensive
sutures/repair. Alternatively, especially in emer-
gency situations, vent placement can occur via the 6.2.2 Miniaturized Extracorporeal
left ventricular apex after a stab incision. Insertion Systems
of the vent into the pulmonary artery trunk is also
possible. For coronary artery bypass surgery, a After more than 30 years of clinical success with
vent is placed into the aortic root, often as a com- standard ECC, about two decades ago the side
bined cannula for antegrade cardioplegia delivery. effects of extracorporeal circulation became the
focus of intensive discussion. Off-pump coronary
6.2.1.9 Tubing artery bypass surgery (OPCAB) emerged, but the
The tubing system connects the various compo- technical challenge of this new surgical technique
nents of the ECC with the patient. In many insti- could not be met by all surgeons. Even when
tutions, the ECC components are individually OPCAB surgery could not convince the cardio-
selected and preassembled in a custom pack, surgical community with clearly superior results,
which eliminates the infectious risk almost com- the desire for less traumatic tools and techniques
pletely. The length of the tubing must be appropri- remained and lead to the development of minia-
ately sized to provide sufficient distance to the turized extracorporeal systems. They combine an
non-sterile heart-lung machine. In general, the adequate safety standard with reduced side effects
diameter should be large enough to keep flow claimed (Remadi et al. 2004; Wiesenack et al.
resistance and shear stress which impair blood 2004).
components as low as possible. On the other A miniaturized heart-lung machine is not
hand, tubing should be shortest possible und only a reduced size system but also a new con-
small in diameter to allow minimal priming and cept of extracorporeal circulation. Its goal is a
low gradients along the tubing. Most commonly, constant volume perfusion with minimal blood
for adult patients, the arterial line is 3/8″ in diam- trauma, reduced hemodilution, less postopera-
eter and the venous lines 1/2″ in diameter. tive systemic inflammatory response syndrome
Cannulas and tubing are transparent to facili- (SIRS), lower transfusion requirements, and
tate the evaluation of the blood with regard to finally faster and better reconvalescence. The
color, air bubbles, and clots. Most of the tubes are first mini- systems were slimmed standard
available with heparin coating, which is supposed heart-lung systems, i.e., suckers and the venous
to reduce the heparin need and thus lessens the reservoir were left off and the tubing was short-
risk of bleeding complications. ened. The pump consisted (and still does) of a
138 C. Schmid
a b
centrifugal pump and a low flow oxygenator assembled, or the system can be purchased as a
with a small priming volume. Some systems are complete set. Due to the different technical fea-
heparin coated including all tubing. A separate tures, the mode of operation of the systems varies.
heating device is unnecessary as a heat exchanger An optimum cannot be defined, yet.
is an obligate and integral part of each oxygen-
ator. Bubble traps are optional. Cardioplegia is 6.2.2.1 MECC System (Maquet®)
administered with low-volume techniques too. The MECC (miniaturized extracorporeal circula-
Most often, the Calafiore technique is employed, tion) system (. Fig. 6.11) was developed in the
where arterial blood is deviated from the main late 1990s and was the first mini-system which
line, enriched with potassium, and afterward was commercially available. It is completely
reintegrated into the venous return (Calafiore assembled from Maquet products (Maquet
et al. 1995). A systemic blood sucker does not Cardiovascular, Rastatt, Germany; Wayne, NJ,
exist as spilled blood is not immediately fed to USA):A Rotaflow® centrifugal pump, a Quadrox
the venous line to keep the circuit closed. D® oxygenator with a diffusion membrane, and an
Instead, it is collected and washed with a cell optional venous bubble trap to eliminate circulat-
saver and returned to the patient thereafter. ing air bubbles. All components are heparin
All these individual procedures are not new, but coated (Bioline®), allowing a short-term run of
their combination delineates a completely new the device without full heparinization. This is
principle of extracorporeal circulation. The main truly advantageous for patients with a high bleed-
indication for miniaturized extracorporeal systems ing risk. The arterial Quart® filter may be added
is coronary artery bypass (CABG) surgery where as well but also increases the priming as does the
the cavities of the heart are not entered. An aortic bubble trap by 25 %. The preparation of the sys-
valve replacement and a resection of a left ventricu- tems requires only about 1–2 min.
lar aneurysm may also be achieved with a mini- The Rotaflow® pump can propagate 4.0–
system, but the surgical procedure is more complex 4.5 L/min and has a priming volume of
and needs further refinements of the system, and 32 mL. The Quadrox D® oxygenator offers a
the basic idea to prevent the blood air contact is par- membrane surface of 2.4 m2 and requires a
tially undermined. It is self- evident that before priming volume of 250 mL. The great advantage
starting valve surgery with a mini-bypass system, of the MECC systems is that the bubble traps
significant experience with coronary bypass surgery and the arterial filter can be easily left out which
employing this system should have been obtained. lowers the basic priming volume to 500 mL and
There are several mini-systems commercially hemodilution is minimal. No other system pro-
available. Their components can be individually vides this opportunity.
Chapter 6 · Extracorporeal Circulation and Myocardial Protection in Adult Cardiac Surgery
139 6
6.2.2.2 esting Heart System
R propel 1–6 L/min. The oxygenator has a membrane
(Medtronic) surface of 2.5 m2 and a priming volume of
The Resting Heart® system (. Fig. 6.12) is a com- 250 mL. An interesting feature is the Affinity
plete set based on Medtronic products (Medtronic, Venous Air Removal Device (VARD®). With the
Minneapolis, MN, USA): the centrifugal Bio-Pump help of two ultrasound detectors, air bubbles are
Plus® and their Affinity NT® membrane oxygen- recognized, an audiovisual signal is turned on, and
ator. The whole system is heparin coated with the the bubbles are automatically removed. A filter with
Carmeda® technology. The Bio-Pump Plus® can a pore size of 38 um is integrated in the VARD®.
The advantages of this system are its high safety
level and the perfect bubble elimination. The large
priming volume of 1400 mL is a disadvantage as it
equals or even exceeds that of a normal heart-lung
machine ECC circuit. Thus, hemodilution and
inflammation are not substantially influenced.
Since April 2013, the complete ECC set is no
longer available in the United States, but all compo-
nents are still obtainable to assemble the system.
one device body. The maximal pump flow is bles; an arterial filter eliminates microbubbles. In
reported as 8 L/min. The compact construction case of air entering the circuit, an ultrasound
renders the priming volume with 680 mL in an detector reduces pump speed and finally blocks
acceptable range, but the tubing system has to be the venous line in an automated process. The
added. An air purge system for automatized air inner surface of the whole system is equipped
bubble elimination can be well added to the system. with the biocompatible X-coating.
The Synergy system differs from its competi- The safety feature with the venous bubble trap
tors by its compact construction. Another advan- and the arterial filter is opposed by a large prim-
tage is the possibility to convert the closed system ing volume which may lead to a considerable
in an open system with a reservoir with a few steps. hemodilution. A retrograde autologous filling can
lower the priming volume.
6.2.2.4 ROC-Safe System (Terumo®)
The ROC-Safe® system (Terumo Corp., Hatagaya, 6.2.2.5 Modifications for Aortic
6 Japan), R, O, and C (. Fig. 6.14) being the initials Valve Surgery
of the perfusionist who invented the system, is a Aortic valve replacement with mini-systems has
closed perfusion system, which was developed for been performed with several systems. Cannulation
coronary bypass surgery. With an additional of the aorta and right atrium is as usual; blood
module, other surgical procedures can be accom- cardioplegia is administered ante- or retrogradely.
plished as well. Its centrifugal pump offers up to The main problem is to keep the aortic root dry.
6 L/min, whereas the polypropylene oxygenator As a standard vent in the left atrium would aspi-
has a surface of 1.8 m2 and a heat exchanger inte- rate large amounts of air and cause air blockade, it
grated. A venous bubble trap removes larger bub- cannot be used when the aorta is opened. Instead,
Air Bubble
Add Detector Centrifugal
Volume Pump
Air removal
Volume
Buffer
Venous
Bubble
Trap
Electronic
Venous Line
Occluder
Remove
Volume
Arterial
Filter Oxygenrator
retention. However, a clear advantage for the use at the steering console to maintain adequate cardio-
of colloidal solutions has not been demonstrated, pulmonary support. Mean arterial pressure and
yet. The priming is recirculated sufficiently long pump flow depend on the degree of hypothermia;
to remove all air from the circuit. As the filling the latter and the amount of cardioplegia are related
volume of the extracorporeal circuit equals about to the type of surgery planned. The important
one third of the patient’s blood volume, the hema- surveillance parameters are noted in the perfusion
tocrit drops to about two thirds with the initiation protocol. The interaction between perfusionist, anes-
of cardiopulmonary bypass. In case of hypother- thesiologist, and cardiac surgeon and the strict sur-
mic perfusion, the hematocrit is lowered even veillance of the perfusion parameters allow the
further. So far, an optimum for the hematocrit on optimal conduct of cardiopulmonary bypass, as well
extracorporeal circulation in adult patients has as the prevention or early detection of complications.
not been defined. After the priming, the heart-
lung machine should be used within 8 h. 6.2.3.3 Termination of
6 Prior to the start of extracorporeal circulation, Extracorporeal Circulation
the patient has to be sufficiently, so-called «fully» After the surgical repair of the heart or great ves-
heparinized. For a standard heart-lung machine sels has been completed, the patient can be weaned
extracorporeal circuit in open-heart surgery, 300– from cardiopulmonary bypass. At this point, the
400 IU/kg of heparin is considered a full heparin- patient has to be rewarmed to at least 34 °C, and
ization. It is administered intravenously (see the intravascular compartment and the heart have
above). In (closed) heparinized (surface-coated) to be refilled and de-aired by reducing the venous
systems, one third to 50 % of this dose are usually unloading. Myocardial contractility is judged
sufficient for anticoagulation. Additionally, 2500– visually be the cardiac surgeon and optionally by
10,000 IU is added to the priming to make up for transesophageal echocardiography (TEE) also. If
the additional volume and to prevent thrombotic myocardial contractility is insufficient, it has to be
complications within the circuit prior to go on augmented by appropriate drugs, mainly by cate-
bypass in case of blood priming. Two to 5 min cholamines and phosphodiesterase inhibitors.
after heparin administration and prior to the ini- Atrial fibrillation can be converted to sinus rhythm
tiation of cardiopulmonary bypass, coagulation is to improve cardiac performance; likewise, brady-
controlled by the activated clotting time (ACT). cardia can be counteracted by various pacemaker
Over the whole perfusion period, ACT should be stimulation modes (A00, AAI, DVI, DDD, VVI,
kept between 350 and 400 and is therefore con- see 7 Chapter «Device Therapy of Rhythm Disor-
trolled regularly, i.e., at a 30 min interval. ders», Sect. 30.2.1, Table 30.2). In case of severely
compromised cardiac pump function, biventricu-
6.2.3.2 Monitoring During lar stimulation and/or an intra-aortic balloon
Extracorporeal Circulation pump is favored, as is nitric oxide (NO) (up to
During extracorporeal circulation, cardiovascular 30 ppm) or prostaglandin (iloprost) inhalation in
function, gas exchange, and acid-base balance have situations with right heart failure due to elevated
to be surveyed not only by the anesthesiologist but pulmonary resistance/pulmonary hypertension.
also by the certified clinical perfusionist. The perfu- When a normotensive pulsatile blood pres-
sionist has to keep an eye on the monitor to read the sure is reached, the flow of the heart-lung machine
electrocardiogram (ECG), the arterial blood pres- is stepwise reduced and finally stopped. After ter-
sure, the central venous pressure, the arterial satura- mination of cardiopulmonary bypass, venous
tion, and the body temperature(s). During complex decannulation follows. When hemodynamics are
surgical procedures, further parameters such as the stabilized, heparin is antagonized with protamine,
left atrial pressure can be important too. mostly 1:1. ACT rapidly normalizes to a level
Furthermore, an adequate urine production of <130 s. In contrast to heparin, protamine is not
0.5–1 mg/kg/h should be present. If the latter is administered as a bolus but as a brief infusion
insufficient, augmentation of the pump flow should over about 5 min or more. A rapid protamine
follow prior to the use of diuretics. During deep application can cause a so-called protamine reac-
hypothermia, urine output decreases and may cease. tion, which is a life-threatening complication with
The perfusionist controls pump flow and line pulmonary vasoconstriction and pulmonary
pressures as well as the filling of the venous reservoir edema. The blood, which has been collected in the
Chapter 6 · Extracorporeal Circulation and Myocardial Protection in Adult Cardiac Surgery
143 6
heart-lung machine, can be retransfused via the 6.2.3.6 Problems and Complications
arterial cannula, with the help of vasodilative During Extracorporeal
drugs, if necessary. The use of the pump suckers Circulation
has to be stopped when 50 % of the protamine is Problems and complications may arise from can-
given. If there is further bleeding, the blood is col- nulas and tubing, from insufficient hepariniza-
lected and retransfused by the anesthesiologist tion, and from unexpected cardiac findings.
after being washed in a cell saver.
kAortic Cannulation
6.2.3.4 Anesthesia Aortic cannulation should be a technically simple
During Extracorporeal procedure. As a consequence of malpositioning, the
Circulation tip of the cannula can be within or close to the vessel
During extracorporeal circulation, volatile anes- wall. It also may be inadvertently directed toward the
thetic drugs can be administered at the heart-lung aortic valve. A thin vessel wall can tear, and in the
machine directly into the extracorporeal circuit, worst case aortic dissection may occur. The latter can
when the gas exchange membrane of the oxygen- often be immediately recognized by a bluish color-
ator is permeable for it (the diffusion membrane ation of the aortic wall and verified with transesoph-
of polymethylpentene is not suitable!). Most of the ageal echocardiography. Then, an immediate aortic
volatile anesthetic drugs (sevoflurane, isoflurane, replacement with an interposition graft is necessary,
desflurane) act rapidly and allow for a reliable nar- but mortality is high. In case of atherosclerotic aortic
cosis without hemodynamic compromise. They vessel walls (high correlation with carotid stenosis
also cease their action fast. As the conductance of and peripheral occlusive arterial disease), debris can
anesthesia is excellent and safe, volatile narcotics loosen and embolize. Therefore, some institutions
are used for many years in cardiac surgery. The favor an intraoperative epiaortic ultrasound in
use of a vaporizor mounted on a heart- lung patients with severe atherosclerotic disease. For cor-
machine, however, is somewhat problematic since onary surgery, off-pump surgery may serve as alter-
this combination is not commercially available native. Air embolism can be prevented with careful
and self-assembled systems have to get approval de-airing of the aortic cannula and arterial tubing.
by the respective authorities. In contrast to the
legally optimal intravenous anesthesia, volatile kVenous Cannulation
anesthetics seem to increase end-organ tolerance The placement of the venous cannula(s) in
against adverse influences. On the heart, they have patients with fragile atrial and/or caval walls may
a cardioprotective effect by an anesthesia induced lead to considerable bleeding complications and
preconditioning, and they lessen the reduction of also to air embolism. If a large amount of air is
the cerebral perfusion. A recent meta-analysis of aspirated into the venous line, an air block will
the literature shows that volatile anesthetics develop, i.e., the venous blood flow and finally
increase cardiac performance and reduce the need also the heart-lung machine stop. A rapid de-
of inotropic support and length of postoperative airing procedure is necessary with head down
ventilation (Symons and Myles 2006). positioning, compression of the liver, and initia-
tion of venous suction. Sutures for repairing tis-
6.2.3.5 Hemofiltration During sue tears as well as normal cannulation sutures
Extracorporeal Circulation can trap venous monitoring lines and catheters.
The number of patients with impaired renal func- They may also obstruct caval veins. Malpositioning
tion or even with terminal renal failure who of a venous cannula can render venous drainage
undergo open-heart surgery is steadily rising. inadequate and lead to venous congestion, which
Sufficient turnover and elimination of the fluids by in case of temporary hepatic vein obstruction can
the patient are not possible, neither during nor lead to early postoperative (reversible) liver fail-
after the procedure. To prevent a fluid overload, a ure. Further causes of inadequate venous return
hemofiltration system can be integrated into the are small or obturated cannulas and hypovolemia.
extracorporeal circuit. The hemofilter is connected
between the arterial and venous line distal to the kPeripheral Cannulation
pump. It generates an ultrafiltrate over a highly Peripheral cannulation, especially via femoral ves-
permeable membrane as does a native glomerulus. sels, is prone to multiple complications including
144 C. Schmid
not possible, mainly due to the limiting capacity of sen, with a short drainage cannula and a long can-
the peripheral venous drainage system. As a rule, nula for arterial reinfusion. As the drainage of a
about 80 % of blood is directed toward the ECMO, short cannula is worse and the inferior caval vein
whereas 20 % still find its way into the pulmonary may collapse with increased suction, these tech-
vascular bed and protect the latter. niques are less effective. Another alternative pro-
Patients with immediate postcardiotomy fail- vide the double-lumen cannulas, which are
ure, who are still centrally cannulated at the ascend- inserted in the right jugular vein. As they drain
ing aorta and right atrial appendage, are usually mainly from the inferior caval vein and the out-
connected to the ECMO system employing these flow should be directed toward the tricuspid valve
cannulas in place. In this ECMO circuit, the blood orifice, its placement is much more demanding.
is drained from the right atrium and reinfused into More effective than for oxygenation in adult
the ascending aorta, as with the routine intraopera- applications is the use for venovenous CO2
tive extracorporeal circulation (. Fig. 6.15). removal (see below: ECCO2R) (. Fig. 6.16).
6
6.3.3 Venovenous ECMO 6.3.4 Indication for ECMO
The indication for venovenous ECMO (vv- There are no clear-cut indications for ECMO ther-
ECMO) is respiratory failure without significant apy. The large societies for cardiopulmonary medi-
ventricular dysfunction. As in va-ECMO, periph- cine/surgery have not established respective
eral cannulation is favored to lower bleeding com- guidelines. There are only guidelines from the
plications. In the standard setup, venous blood is Extracorporeal Life Support Organization (ELSO)
drained from the femoral vein and the arterialized which recommend ECMO placement when the risk
blood returned into the (right) jugular vein. It is to die without would be 50 %. Immediate ECMO is
important to have the tips of both cannulas at a to be performed if the mortality risk approaches
distance large enough to prevent a short circuit, 80 %. In most institutions, the indication is deter-
i.e., pump recirculation. If a jugular access is mined by the institutional experience. A large expe-
impossible, a femorofemoral configuration is cho- rience with ECMO leads to a more liberal indication
for its application and ultimately to better results high skill level, vv-ECMO in adults is not wide-
(Muller et al. 2009; Schmid et al. 2009). spread. As for va-ECMO, criteria when to install
In myocardial pump failure, a cardiac index of vv-ECMO are not well defined and differ among
2.0–2.3 L/min/m2 or lower defines today’s indica- centers. In a prospective study in 1979, two
tion for mechanical circulatory support with a indications for pulmonary support with ECMO
ventricular assist device. Accordingly, refractory were proposed (Zapol et al. 1979):
medical treatment for cardiac failure is an accepted 1. A pO2 < 50 mmHg at an FiO2 of 1.0 requires
indication for va-ECMO therapy. Postcardiotomy immediate ECMO placement.
circulatory failure offers more therapeutic options. 2. Whereas a pO2 < 50 mmHg at an FiO2 > 0.6
In case of myocardial contractile failure, the usual and a PEEP > 5 cmH2O for more than 12 h
primary attempts for cardiovascular stabilization was defined as slow entry criterion.
include augmented catecholamine administration
followed by an intra-aortic balloon pump place- Nowadays, emergency ECMO placement for
ment. Only after failure of both combined and an pulmonary support is indicated when the pre-
expected recovery of myocardial pump function, dicted risk for conventional respirator manage-
va-ECMO is indicated. If there is no recovery ment is high. Such a high risk is seen with an
potential, assist devices for long-term support are oxygenation index [(mean airway pressure × FiO2
the better choice (see 7 Chapter «Cardiac Assist × 100)/pO2)] >40 and with a pO2/FiO2 < 60–80. In
Devices and Total Artificial Heart», Sect. 38.4). this regard there is no difference between venove-
While va-ECMO is indicated for emergency nous and venoarterial ECMO. However, prior to
treatment of primary myocardial failure, r eplacing an ECMO installation, all other treatment options
the function of the lungs also, vv-ECMO is to should be considered, and a lung protective venti-
replace the impaired gas exchange in primary pul- lation aspired (tidal volume 6 mL/kg, PIP < 35
monary failure (endpoints: recovery, transplanta- cmH2O, FiO2 < 0.6, cmH2O and pO2 goal: arterial
tion, or death), thereby preventing secondary pO2 > 60 mmHg) (Schmid 2009).
cardiac failure also. Since these therapies require a A special type of ECMO is ECCO2R (extracor-
high logistic effort, considerable resources, and a poreal carbon dioxide removal). Here, carbon
148 C. Schmid
..Table 6.7 Indications for va-ECMO and vv-ECMO (Schmid et al. 2009)
Emergency Resuscitation with insufficient cardiac PaO2/FiO2 <60 mmHg; (FiO2 = 1.0; PEEP = 20 cmH2O),
function severe respiratory acidosis
dioxide is eliminated with an ECMO pump flow approaches: on the right side into or between the
of 1–2 L/min, whereas oxygenation is achieved by pulmonary veins and on the left side through the
6 the native lung by endotracheal/bronchial oxygen left atrial appendage. The LV apex may be cannu-
insufflation without significant ventilation. lated for drainage also. A transition from periph-
There are only few absolute contraindications eral to central cannulation can be helpful too. An
for ECMO. These are mainly situations, where alkalotic hyperoxygenation by lowering pH/pCO2
recovery of end-organ function (late tumor stage, and pulmonary vascular resistance is a further
significant cerebral disease/damage) cannot be means to benefit the right ventricle.
expected. All other contraindications are relative, A recovery of myocardial pump function with
i.e., in every case benefit and risk have to be persistent respiratory impairment can cause prob-
weighed such as peripheral vascular disease and lems during femorofemoral venoarterial perfusion.
abdominal aortic aneurysm with thrombus for- The lower half of the body is well perfused with
mation. This is also true for the bleeding risk in arterialized blood, whereas the upper body includ-
polytraumatized patients, when an ECMO with ing the brain and coronary arteries is provided with
heparin coating is used (Arlt et al. 2010). Yet, hypoxemic blood from the native (malfunctioning)
maintenance of ECMO without heparin adminis- lung only. Diagnosis can be established by percuta-
tration is only possible for a few days. The carbon neously measuring oxygen saturation at a right-
dioxide-lowering properties can also be beneficial sided finger or directly with a radial artery blood
for neurosurgical patients (Bein et al. 2002). A gas drawn. Therapeutic options consist of a transi-
septic patient can be supported with ECMO too, tion to central cannulation or of a relocation of the
but loss of peripheral resistance with a consecu- arterial cannula into the right subclavian artery.
tive increase of cardiac output may not be fully Major complications are bleeding and throm-
mastered with peripheral ECMO systems, if at all bosis at the cannula sites, followed by ischemic
(. Table 6.7). complications at the respective extremities.
Therefore, it is advisable to anastomose a (6–8 mm)
Dacron graft for cannulation to the arterial vessel
6.3.5 Physiological Consequences if the latter is small in size. Most institutions follow
and Problems that strategy always when accessing the subclavian
artery due to the risk of deleterious complications.
The main goal of mechanical support with ECMO At the femoral artery, a separate small cannula
is to allow for recovery of heart and/or lung func- placement for distal perfusion is another option.
tion. This works well as long as there is no total Further problems arise during long-term sup-
loss of cardiopulmonary function, i.e., if the port. Patient mobilization and physiotherapy are
ECMO supports but not replaces heart and lung, difficult. The patients are usually bedridden and
although this may be initially the case. A total loss tethered to the bed by the tubing. A successful
of cardiac pump function can be compensated extubation to benefit lung function and to prevent
with a peripheral ECMO in small patients with respiratory infections hardly facilitates mobiliza-
large vessels only. Otherwise, the left heart is not tion. The use of a solitary double-lumen cannula
sufficiently unloaded and distends. Consecutively, for venovenous ECMO inserted into the jugular
an additional vent may become necessary, which vein increases the mobility but may limit pump
can be inserted into the left atrium with several output (Chimot et al. 2013).
Chapter 6 · Extracorporeal Circulation and Myocardial Protection in Adult Cardiac Surgery
149 6
6.3.6 Long-Distance Transport or the distal abdominal aorta; the tip of the
on ECMO venous cannula was placed in the inferior vena
cava. A so-called distal perfusion can be estab-
Critically ill patients with cardiac and/or pulmonary lished but is frequently omitted. Central cannu-
failure may not have access to all therapeutic means lation is not performed. In cases of vv-ECMO,
in a primary care center; they have to be referred to outflow was achieved via the femoral vein, and
a tertiary care center or a specialized heart and lung inflow was gained by cannulation of the right
failure unit. However, the clinical state of patients internal jugular vein and thereafter into the
being on high-dose inotropes or being maximally superior vena cava. Before vessel cannulation
ventilated is rather unstable, and conventional 5000 IU heparin is administrated intravenously,
patient transport is associated with a high risk. except when the partial thromboplastin time
With the availability of the miniaturized ECMO (PTT) value is ≥1.5 times above normal range.
systems (see above 7 Sect. 6.2.2), patient transport With a heparin-coated circuit, no further antico-
became safe, both in a venovenous and venoarterial agulation is needed during transport. After start-
configuration. Prerequisites are a hub and a radio ing the ECMO, circulatory state and respirator
system for the participating hospitals as well as an on settings are optimized in a brief period. A dra-
call system in the hub hospital/unit which can be matic reduction of catecholamine requirement is
organized in various ways. An incoming request for common; a mean systemic blood pressure of
ECMO transportation demands immediate 50–70 mmHg is sufficient. The patient is trans-
response: (1) Verifying that the patient is an appro- ferred to the helicopter or ambulance car, and
priate candidate for ECMO treatment with further monitoring is established in the vehicle.
definitive therapeutic options to be offered. The Monitoring includes continuous electrocardio-
cause of heart failure, reversibility of cardiac dysfunc- graphic surveillance, invasive blood pressure
tion, end-organ failure, neurology, tumor, and other measurement, pulse oximetry, and capnography.
terminal diseases should be asked for to judge Tissue perfusion and oxygen delivery are
whether the patient may be weaned from ECMO, assessed by pulse oximetry and estimation of
can undergo conventional open-heart surgery, or arterial blood gas exchange and mixed venous
will need long-term mechanical support. (2) Avail- oxygen saturation if available. Minimal require-
ability of staff and equipment. ECMO transport ments are electrocardiogram, blood pressure,
teams usually include an anesthesiologist or an inten- pump flow (3–5 L/min), and right radial arterial
sivist, a cardiac surgeon, a perfusionist, and a para- saturation. During transport, low flow situations
medic. Depending on the organizational structure are treated with crystalloid infusions and
and individual skill level, the team can be reduced to impaired oxygenation with airway toilet and
two staff members, as the space in most helicopters increased oxygen supply. An ischemic leg cannot
and ambulance cars is limited. (3) Availability of ICU be treated, but will not become a clinical prob-
capacity. In Europe, adult ECMO transportation is a lem for the few hours in the air/on the road.
new mission, predominantly created by cardiac sur- Upon arrival, the patient is immediately referred
geons. Therefore, not only the staff but also ICU to the cardiac ICU, and further treatment options
capacity is shared. Shortness of ICU beds is common. are discussed and applied (Arlt et al. 2008).
After arrival at the distant hospital, the
patient’s clinical condition is reviewed again. If
the patient unexpectedly is no longer a candidate 6.3.7 ECMO Resuscitation
for ECMO according to (1), the mission is
aborted. If the patient, now with specialists sup- Acute cardiovascular collapse is life threatening
port available, can be stabilized for normal and mandates immediate action. In most
patient transfer, ECMO is not installed either. instances, an appropriate drug therapy is suffi-
For ECMO placement, the size of the femoral cient to stabilize the patient; some, however, may
vessels can be measured by ultrasound, and require chest compressions/external heart mas-
appropriate cannulas are chosen to minimize the sage and artificial ventilation. Further treatment
risk of limb ischemia (arterial 15–17 Fr, venous options are usually not available except in institu-
17–23 Fr). For va-ECMO, the tip of the arterial tions offering ECMO placement during resuscita-
cannula is positioned in the common iliac artery tion. Systems for ECMO as a resuscitation tool
150 C. Schmid
include mobile larger devices and light-weight the same opinion with regard to maintenance or
portable systems. withdrawal of therapy, ethical debates are diffi-
For ECMO resuscitation, femoral artery and cult. (3) If ECMO resuscitation was successful
vein—preferably on opposite sides—are punc- and the patient denies consecutive necessary
tured percutaneously during brief periods of halt- treatment such as VAD therapy, mechanical sup-
ing external cardiac massage. With Seldinger’s port must be terminated.
technique, a borderline small arterial cannula
(e.g., 15 Fr) and a long venous cannula (21 Fr or
23 Fr) are inserted and connected to the ECMO
References
circuit after careful de-airing. When ECMO is Abah U, Garfjeld Roberts P, Ishaq M, De Silva R (2012) Is
started, both cannulas are fixed to the patient, and cold or warm blood cardioplegia superior for myocar-
echocardiography is performed to assess cardiac dial protection? Interact Cardiovasc Thorac Surg
function and filling/unloading. Ventricular fibril- 14:848–855
6 lation is terminated with defibrillation. Inotropic Almond CS, Harrington J, Thiagarajan R et al. (2006)
Successful use of bivalirudin for cardiac transplanta-
drug support and volume administration are opti- tion in a child with heparin-induced thrombocytope-
mized, and the pH is normalized. The afterload of nia. J Heart Lung Transplant 25:1376–1379
the heart is lowered to prevent cardiac distension, Andersen LW, Landow L, Baek L, Jansen E, Baker S (1993)
and the respirator is set to lung protective ventila- Association between gastric intramucosal pH and
tion, if possible. The time period needed to estab- splanchnic endotoxin, antibody to endotoxin, and
tumor necrosis factor-alpha concentrations in patients
lish mechanical support is about 15–20 min. undergoing cardiopulmonary bypass. Crit Care Med
Recent studies showed that longer pre- 21:210–217
ECMO resuscitation duration was associated Arlt M, Philipp A, Zimmermann M et al. (2008) First experi-
with poor prognosis. The same was true for ences with a new miniaturised life support system for
mobile percutaneous cardiopulmonary bypass.
patients with pulseless electrical activity or asys-
Resuscitation 77:345–350
tole as compared to ventricular tachycardia or Arlt M, Philipp A, Voelkel S et al. (2010) Extracorporeal
ventricular fibrillation as the initial rhythm. membrane oxygenation in severe trauma patients
These finding suggests that the main factors with bleeding shock. Resuscitation 81:804–809
associated with outcome are baseline condition, Ascione R, Suleiman SM, Angelini GD (2008) Retrograde
hot-shot cardioplegia in patients with left ventricular
underlying cause, and the rapid response of the
hypertrophy undergoing aortic valve replacement.
resuscitation team. Overall, a survival of about Ann Thorac Surg 85:454–458
30 % with resuscitation periods up to 1 h has Baer DM, Osborn JJ (1960) The postperfusion pulmonary
been reported (Chen et al. 2008). It has been sug- congestion syndrome. Am J Clin Pathol 34:442–445
gested that applying mild hypothermia (34 °C) Banbury MK, Kouchoukos NT, Allen KB et al. (2003) Emboli
capture using the Embol-X intraaortic filter in cardiac
for 24 or 48 h after ECMO initiation may add a
surgery: a multicentered randomized trial of 1,289
benefit to the patient, as therapeutic hypother- patients. Ann Thorac Surg 76:508–515; discussion 515
mia is currently recommended after cardiac Bannister CF, Finalyson DC (1995) The endocrine system:
arrest. Survival among the elderly supported on effects of cardiopulmonary bypass. In: Mora CT (ed)
ECMO is lower than that for younger adult Cardiopulmonary bypass. Springer, New York, p 191
Bartlett R (2005) Physiology of ECLS. In: Van Meurs K, Lally KP,
patients; however, age should not be a principle
Peek GJ, Zwischenberger JB (eds) ECMO extracorporeal
contraindication for the use of ECMO in older cardiopulmonary support in critical care. Extracorporeal
patients (Mendiratta et al. 2013). Life Support Organization, Ann Arbor, pp 5–27
Several important issues have to be considered Bein T, Kuhr LP, Metz C et al. (2002) ARDS and severe brain
when performing ECMO resuscitation. (1) The injury. Therapeutic strategies in conflict. Anaesthesist
51:552–556
complication rates from femoral vessel cannula-
Berrizbeitia LD, Tessler S, Jacobowitz IJ et al. (1989) Effect
tion are high including limb ischemia, severe ves- of sternotomy and coronary bypass surgery on post-
sel laceration, retroperitoneal and thigh bleeding, operative pulmonary mechanics. Comparison of inter-
and placement of a cannula in a wrong vessel. nal mammary and saphenous vein bypass grafts.
Once the patient is stabilized, these problems Chest 96:873–876
Bhat JG, Gluck MC, Lowenstein J, Baldwin DS (1976) Renal
have to be fixed. (2) As the effectiveness of resus-
failure after open heart surgery. Ann Intern Med
citation is never known, patients may end up 84:677–682
being invalidated by severe cerebral injury. If the Biancari F, Rimpilainen R (2009) Meta-analysis of
family and the treating physicians do not share randomised trials comparing the effectiveness of
Chapter 6 · Extracorporeal Circulation and Myocardial Protection in Adult Cardiac Surgery
151 6
miniaturised versus conventional cardiopulmonary Holland FW 2nd, Brown PS Jr, Weintraub BD, Clark RE
bypass in adult cardiac surgery. Heart 95:964–969 (1991) Cardiopulmonary bypass and thyroid function:
Bolotin G, Domany Y, de Perini L et al. (2005) Use of intra- a “euthyroid sick syndrome”. Ann Thorac Surg 52:46–50
operative epiaortic ultrasonography to delineate aor- Kleinman LH, Yarbrough JW, Symmonds JB, Wechsler AS
tic atheroma. Chest 127:60–65 (1978) Pressure-flow characteristics of the coronary
Brinkhous KM, Smith HP, Warner ED et al. (1939) The inhibition collateral circulation during cardiopulmonary bypass.
of blood clotting: an unidentified substance which acts Effects of hemodilution. J Thorac Cardiovasc Surg
in conjunction with heparin to prevent the conversion of 75:17–27
prothrombin into thrombin. Am J Physiol 125:683–687 Klotz S, Vestring T, Rotker J et al. (2001) Diagnosis and
Buckberg GD (1995) Update on current techniques of treatment of nonocclusive mesenteric ischemia after
myocardial protection. Ann Thorac Surg 60:805–814 open heart surgery. Ann Thorac Surg 72:1583–1586
Calafiore AM, Teodori G, Mezzetti A et al. (1995) Intermittent Koster A, Weng Y, Bottcher W et al. (2007) Successful use of
antegrade warm blood cardioplegia. Ann Thorac Surg bivalirudin as anticoagulant for ECMO in a patient
59:398–402 with acute HIT. Ann Thorac Surg 83:1865–1867
Cameron DE, Gardner TJ (1988) Principles of hypothermia. Kuitunen A, Hynynen M, Salmenpera M et al. (1993)
In: Chitwoods WR (ed) State of the art reviews. Anaesthesia affects plasma concentrations of vasopres-
Myocardial preservation. Clinical applications. Hanley sin, von Willebrand factor and coagulation factor VIII in
& Belfus, Philadelphia, pp xii–xxv cardiac surgical patients. Br J Anaesth 70:173–180
Caputo M, Dihmis WC, Bryan AJ, Suleiman MS, Angelini GD Kuntschen FR, Galletti PM, Hahn C et al. (1985) Alterations
(1998) Warm blood hyperkalaemic reperfusion (‘hot of insulin and glucose metabolism during cardiopul-
shot’) prevents myocardial substrate derangement in monary bypass under normothermia. J Thorac
patients undergoing coronary artery bypass surgery. Cardiovasc Surg 89:97–106
Eur J Cardiothorac Surg 13:559–564 Longstreth WT Jr, Inui TS (1984) High blood glucose level
Chen YS, Lin JW, Yu HY et al. (2008) Cardiopulmonary resus- on hospital admission and poor neurological recovery
citation with assisted extracorporeal life-support ver- after cardiac arrest. Ann Neurol 15:59–63
sus conventional cardiopulmonary resuscitation in Lundberg S (1967) Renal function during anaesthesia and
adults with in-hospital cardiac arrest: an observational open-heart surgery in man. Acta Anaesthesiol Scand
study and propensity analysis. Lancet 372:554–561 Suppl 27:1–81
Chimot L, Marque S, Gros A et al. (2013) Avalon(c) bicaval Magnani HN (1993) Heparin-induced thrombocytopenia
dual-lumen cannula for venovenous extracorporeal (HIT): an overview of 230 patients treated with orga-
membrane oxygenation: survey of cannula use in ran (Org 10172). Thromb Haemost 70:554–561
France. ASAIO J 59:157–161 Mannucci PM (2000) Desmopressin (DDAVP). Haemophilia
Copeland JG, Arabia FA, Tsau PH et al. (2003) Total artificial 6(suppl 1):60–67
hearts: bridge to transplantation. Cardiol Clin 21:101–113 McLean J (1916) The thromboblastic action of cephalin.
Crock PA, Ley CJ, Martin IK, Alford FP, Best JD (1988) Am J Physiol 41:250–257
Hormonal and metabolic changes during hypother- Menasché P (1996) Blood cardioplegia: do we still need to
mic coronary artery bypass surgery in diabetic and dilute? Ann Thorac Surg 62:957–960
non-diabetic subjects. Diabet Med 5:47–52 Menasché P (1997) New strategies in myocardial preserva-
Delva E, Maille JG, Solymoss BC et al. (1978) Evaluation of tion. Curr Opin Cardiol 12(6):504–14
myocardial damage during coronary artery grafting Mendiratta P, Wei JY, Gomez A et al. (2013) Cardiopulmonary
with serial determinations of serum CPK MB isoen- resuscitation requiring extracorporeal membrane oxy-
zyme. J Thorac Cardiovasc Surg 75:467–475 genation in the elderly: a review of the Extracorporeal
Ferraris VA, Saha SP, Oestreich JH et al. (2012) 2012 update Life Support Organization registry. ASAIO J 59:211–215
to the Society of Thoracic Surgeons guideline on use Mohan Rao PS, Simha PP (2011) Myocardial preservation:
of antiplatelet drugs in patients having cardiac and controlled reperfusion. Semin Thorac Cardiovasc Surg
noncardiac operations. Ann Thorac Surg 94: 23:318–321
1761–1781 Muller T, Philipp A, Luchner A et al. (2009) A new miniatur-
Filsoufi F, Rahmanian PB, Castillo JG et al. (2007) Predictors ized system for extracorporeal membrane oxygen-
and outcome of gastrointestinal complications in ation in adult respiratory failure. Crit Care 13:R205
patients undergoing cardiac surgery. Ann Surg 246: Peters RM, Wellons HA Jr, Htwe TM (1969) Total compliance
323–329 and work of breathing after thoracotomy. J Thorac
Greinacher A, Lubenow N, Hinz P, Ekkernkamp A (2003) Cardiovasc Surg 57:348–355
Heparin-induzierte thrombozytopenie. Dtsch Ärzteblatt Philipp A, Arlt M, Amann M et al. (2011) First experience
100:A2220–A2222 with the ultra compact mobile extracorporeal mem-
Guyton RA (1995) The myocardium: physiology and pro- brane oxygenation system cardiohelp in interhospital
tection during cardiac surgery and cardiopulmonary transport. Interact Cardiovasc Thorac Surg 12:978–981
bypass. In: Mora CT (ed) Cardiopulmonary bypass. Porter GA, Kloster FE, Herr RJ et al. (1966) Relationship
Springer, New York, pp 21–39 between alterations in renal hemodynamics during
Hilberman M, Myers BD, Carrie BJ et al. (1979) Acute renal cardiopulmonary bypass and postoperative renal
failure following cardiac surgery. J Thorac Cardiovasc function. Circulation 34:1005–1021
Surg 77:880–888
152 C. Schmid
Ranmsey JG (1995) The respiratory, renal, and hepatic sys- bypass surgery: a meta-analysis. Br J Anaesth 97:
tems: effects of cardiac surgery and cardiopulmonary 127–136
bypass. In: Mora CT (ed) Cardiopulmonary bypass. Vargas FS, Cukier A, Terra-Filho M et al. (1992) Relationship
Springer, New York, p 159 between pleural changes after myocardial revasculariza-
Remadi JP, Rakotoarivello Z, Marticho P et al. (2004) Aortic tion and pulmonary mechanics. Chest 102:1333–1336
valve replacement with the minimal extracorporeal Warkentin TE, Greinacher A (2003) Heparin-induced
circulation (Jostra MECC System) versus standard car- thrombocytopenia and cardiac surgery. Ann Thorac
diopulmonary bypass: a randomized prospective trial. Surg 76:2121–2131
J Thorac Cardiovasc Surg 128:436–441 Wiesenack C, Liebold A, Philipp A et al. (2004) Four years’
Schmid C (2014) Leitfaden Erwachsenenherzchirurgie, 3rd experience with a miniaturized extracorporeal circula-
edn. Springer, Heidelberg, pp 185–186 tion system and its influence on clinical outcome. Artif
Schmid C, Philipp A, Mueller T, Hilker M (2009) Organs 28:1082–1088
Extracorporeal life support—systems, indications, and Wilde MI, Markham A (1997) Danaparoid. A review of its
limitations. Thorac Cardiovasc Surg 57:449–454 pharmacology and clinical use in the management of
Stafford-Smith M, Patel UD, Phillips-Bute BG, Shaw AD, heparin-induced thrombocytopenia. Drugs 54:903–924
6 Swaminathan M (2008) Acute kidney injury and
chronic kidney disease after cardiac surgery. Adv
Wood P (1956) Disesase of heart and circulation, 2nd edn.
JB Lippincott, Philadelphia
Chronic Kidney Dis 15:257–277 Yeboah ED, Petrie A, Pead JL (1972) Acute renal failure and
Stock MC, Downs JB, Weaver D et al. (1986) Effect of pleu- open heart surgery. Br Med J 1:415–418
rotomy on pulmonary function after median sternot- Zapol WM, Snider MT, Hill JD et al. (1979) Extracorporeal
omy. Ann Thorac Surg 42:441–444 membrane oxygenation in severe acute respiratory
Symons JA, Myles PS (2006) Myocardial protection with failure: a randomized prospective study. Am Med
volatile anaesthetic agents during coronary artery Assoc 242:2193–2199
153 7
Advances
in Cardiopulmonary
Bypass for the Neonate
and Infant
Richard A. Jonas
References – 168
Chapter 7 · Advances in Cardiopulmonary Bypass for the Neonate and Infant
155 7
7.1 Introduction 7.2 Improvements in the Hardware
of Cardiopulmonary Bypass
The results of surgery for congenital heart for Neonates and Infants
anomalies in neonates and infants have
improved dramatically over the last two decades. 7.2.1 Oxygenator
Not only is mortality less but in addition dura-
tion of time in the intensive care unit and over- The earliest oxygenators such as the Gibbon oxy-
all morbidity have continued to decline. There is genator relied on a thin film of blood adhering to a
no single explanation for this progress. disk or mesh that rotated through a trough of
. Table 7.1 suggests that the development of blood. Not only did this system require a huge
cohesive multidisciplinary teams managing priming volume, but in addition it generated a large
neonates and infants with congenital heart dis- number of emboli. The many deleterious effects of
ease has been one of the most significant fac- cardiopulmonary bypass (. Table 7.2) include
tors. However, the second factor listed is also emboli, hemolysis, and the systemic inflammatory
extremely important and that has been improve- response to cardiopulmonary bypass (Edmunds
ments in both the hardware and techniques of 1998). In addition for the neonate and infant, expo-
cardiopulmonary bypass. sure to a massive volume of homologous blood
In the early years of cardiopulmonary bypass (bank blood) was equivalent to many exchange
in the 1950s, 1960s, and 1970s, both the hardware transfusions. Even in the adult world, the «homolo-
and techniques of bypass that were used for gous blood syndrome» had been recognized in the
infants and neonates were no more than slight early years of cardiopulmonary bypass (Gadboys
modifications of the hardware and techniques et al. 1962). This response to massive transfusion
used for adults undergoing cardiopulmonary was definitely exaggerated in the neonate and
bypass. It wasn’t until the late 1980s that develop- infant and was responsible for much of the morbid-
ments in cardiopulmonary bypass were focused ity of cardiopulmonary bypass in its early years.
specifically on neonatal and infant cardiopulmo- When bubble oxygenators came into commer-
nary bypass. Continuing developments in the cial use in the 1970s, there was an even greater
1990s and in the new millennium have been exposure to gaseous microemboli than had been
accompanied by further improvements in out- the case with disk and mesh oxygenators (Dewall
comes. et al. 1956). Arterial line filters at this time
required a large priming volume of their own, and
they were generally ineffective. They were likely to
generate as many emboli as they removed because
..Table 7.1 Reasons for improved results of platelets and fibrin adhered to the downstream
surgery for congenital heart anomalies in neonates side of the filter mesh. Early attempts to introduce
and infants membrane oxygenators in the 1970s required an
extremely large priming volume. The first widely
Cohesive multidisciplinary teams
used membrane oxygenator for pediatric use did design of the modern heart-lung pump, it has
not come into existence until the late 1980s. This been possible to reduce total priming volume to
was the Cobe variable prime flat sheet membrane approximately 200–250 cc in most centers. Some
oxygenator or «VPCML» (Cobe Cardiovasular centers are using custom-developed microcircuits
Inc., Arvada, CO, USA) (Nagatsu et al. 1987). This with even smaller priming volumes.
early membrane oxygenator was used, for exam-
ple, in the Boston circulatory arrest study of the
late 1980s without an arterial line filter (Newburger 7.2.2 Pump Design
et al. 1993). It was not until the early 1990s that an
oxygenator specifically designed for neonates, the Like oxygenators, early heart-lung pumps used
Lilliput hollow fiber membrane oxygenator, was for pediatric bypass were essentially the same as
available (Sorin S.P.A, Milan, Italy). Subsequent those used for adult cardiopulmonary bypass.
improvements in oxygenator design have resulted Thus, long lengths of tubing were required to
in an ever decreasing priming volume. Today reach to the sucker heads as well as to the main
neonatal and infant oxygenators are likely to have pump head. With the development of specific
7 an integrated arterial filter within the oxygenator pediatric heart-lung machines, it is now possible
itself thereby avoiding the additional priming vol- to bring the principal pump head extremely close
ume, for example, the Terumo FX oxygenator to the patient so that the tubing length is very
(Terumo Corp., Hatagaya, Japan) (. Fig. 7.1). The short (Ando et al. 2004) (. Fig. 7.2). Furthermore
priming volume required for the heat exchanger both the oxygenators and the pumps accommo-
component of oxygenators has also become date 3/16 diameter arterial line tubing as well as
remarkably small. Taken in conjunction with the ¼ in. or even 3/16 in. venous line tubing. The
sucker pump heads also can be brought much
closer to the patient thereby further decreasing
the priming volume. The advantages and disad-
vantages of centrifugal versus roller head pumps
continue to be debated as well as pulsatile versus
nonpulsatile systems. The majority of centers con-
tinue to favor nonpulsatile roller head pumps
because of their safety and simplicity as well as
cost considerations.
7.2.3 Cannulas
..Fig. 7.3 Venous cannulas used for neonatal and infant cardiopulmonary bypass. a DLP Single Stage cannula features the
multiport tip that makes insertion easier for single atrial cannulation. b The right-angled Terumo Tender Flow provides a
unique step-down design, which maximizes flow performance and minimizes pressure drop, available in a range of sizes
from 8Fr to 24Fr
0.2
IVS
0.0
0 10 20 30 40 50 60 70 80
Circulatory arrest (minutes)
less these studies did allow a better understanding of flow index of approximately 0.75 L/min/m2
the consequences of different bypass techniques (Newburger et al. 1993) (. Fig. 7.4). Although in
leading to subsequent improvements (Zeltser et al. the early neurodevelopmental follow-up studies,
2008; Limperopoulos et al. 2010). The first major there tended to be a worse outcome in patients
prospective randomized trial of bypass technique who underwent hypothermic circulatory arrest,
for neonates and infants was the Boston Circulatory by 8 years of age, and in subsequent follow-up
Arrest Study (Newburger et al. 1993). studies there were minimal differences(Bellinger
et al. 2003). For example, in academic achieve-
ment at 8 years of age, there were no differences
7.3.1 oston Circulatory Arrest
B between circulatory arrest and low flow patients
Study in reading score, math score, any WIAT (Wechsler
Individual Achievement Test) subscale score, or
The Boston Circulatory Arrest Study was a ran- the competence scales of teacher reports.
domized prospective trial of neonates and infants However, both groups scored lower than popula-
less than 3 months of age undergoing an arterial tion norms. While today those reduced scores for
switch procedure for d-transposition between both groups might be solely attributed to the pre-
1988 and 1992. As noted above, the hardware natal effects of a hypoxic cerebral circulation, at
used for that study was primitive by today’s stan- the time of the study, they were attributed solely
dards and included a flat sheet membrane oxy- to the deleterious effects of cardiopulmonary
genator with a priming volume of 750 cc. Also no bypass. This resulted in the generation of a new
arterial line filter was employed. The periopera- hypothesis, namely, that the technique of bypass
tive results of that study included a higher inci- that was employed in the late 1980s resulted in
dence of seizures in babies undergoing circulatory limited oxygen delivery secondary to the interac-
arrest relative to those undergoing continuous tion of several points of bypass technique. The
low flow bypass at deep hypothermia with a flow most important factors that interacted were
index of 50 cc/kg/min which is equivalent to a thought to be reduced overall flow rate relative to
Chapter 7 · Advances in Cardiopulmonary Bypass for the Neonate and Infant
159 7
normal, the alkaline pH of hypothermic bypass gaseous microemboli. A high perfusion flow rate
using the alpha-stat strategy and the impact of also can result in the reservoir level dropping
hemodilution. abruptly if there is any interference with systemic
venous return. Thus, the safety margin for the
perfusionist becomes much narrower, and there is
7.3.2 ubtle Brain Injury Secondary
S a risk of pumping air into the patient if there is
to Hypoxia inattention for just a few seconds and if modern
safety mechanisms such as level sensors fail (De
The concept that a subtle degree of brain injury Somer 2007; Murphy et al. 2009).
could occur during heart surgery in the absence
of gross neurological findings such as a stroke was kpH Strategy
a new one in the field of cardiac surgery in the The cardiac surgeon and perfusionist must have a
1980s. However in other fields, the concept of very good understanding of the oxyhemoglobin
subtle cumulative cognitive injury has become dissociation curve (. Fig. 7.5) (Hamilton et al.
increasingly appreciated. In the field of sports 2004). A shift of the oxyhemoglobin dissociation
medicine, for example, the cumulative effect of curve to the left means that oxygen is bound
multiple concussions in American football has more tightly to hemoglobin. Thus, tissues that
received increasing attention from the lay press as require oxygen for ongoing metabolism may be
the science of this field becomes more focused. In unable to obtain an adequate amount. This will
the sport of high-altitude climbing, there are sev- result in the venous oxygen saturation being
eral reports, some including MRI studies, that falsely elevated. This is extremely important
have documented the injury that can occur from because venous oxygen saturation is the principal
hypoxia related to high altitude. Cognitive studies means of monitoring the «safety» of cardiopul-
of mountain climbers who have climbed without monary bypass. It is a fundamental premise that
supplementary oxygen have documented persis- if the venous oxygen saturation is greater than
tent memory impairment (Cavaletti et al. 1990). 70 %, then oxygen delivery is being adequately
maintained (Swan et al. 1990).
7.3.2.1 actors That Potentially Limit
F
Oxygen Delivery During kFactors that Shift Oxyhemoglobin
Cardiopulmonary Bypass Dissociation Leftward
Several factors in the neonate and young
kFixed Flow Rate infant undergoing hypothermic cardiopulmo
A normal cardiac index is approximately 3.5–4 L/ nary bypass can shift oxyhemoglobin dissocia-
min/m2. If there is acute hemodilution, an indi- tion leftward. These include the presence of fetal
vidual can increase cardiac output to at least hemoglobin as well as reduced 2,3-diphospho-
10–15 L/min/m2 in order to maintain oxygen glycerate. Reduced disphosphoglycerate in red
delivery. However, the individual who is placed blood cells occurs in bank blood which is used in
on cardiopulmonary bypass has a fixed cardiac greater relative volume in the small neonate. This
output that is determined by the perfusionists and was particularly a problem in the early years of
not by oxygen and substrate needs. The usual cardiopulmonary bypass when large prime vol-
maximal perfusion flow rate is 2.4 L/min/m2. umes were required. Other important factors that
shift the oxyhemoglobin dissociation curve left-
kWhy Is Maximal Perfusion Flow Rate ward are hypothermia and alkalinity. Hypocarbia
Limited to Only 2.4 L/min/m2? also has the same effect. Hypothermia also has the
The flow rate that is used during cardiopulmonary effect of shifting the pH of neutrality in an alka-
bypass is a trade-off between achieving safe oxy- line direction. It is for this reason that in the early
gen delivery and avoiding an excessive priming years of perfusion, carbon dioxide was routinely
volume and excessive delivery of microemboli. added to the sweep gas passing through the oxy-
Particularly in the early years of cardiopulmonary genator in order to counteract that alkaline shift.
bypass when there was no arterial line filter and Today, however, there is controversy regarding
bubble oxygenators were used, it was well known whether carbon dioxide should be added for
that a high flow rate resulted in greater delivery of pediatric bypass.
160 R.A. Jonas
% Hemoglobin saturation
hypothermic bypass. It may
fail to indicate that an 60 N Shift to the right:
intracellular oxygen debt is Hyperthermia
being incurred because of Hypercarbia
inadequate flow 50 Acidosis
2,3–DPG
40
7 30
20
10
0
0 10 20 30 40 50 60 70 80 90 100
6.9 6.9
pk
Phosphate
6.7 6.7
Bicarbonate
6.3 6.3
6.1 6.1
0 10 20 30 40
Temperature (˚C)
will reduce the number of cerebral emboli by 9 months of age were randomized to either the
reducing cerebral blood flow (Henriksen 1986). pH-stat or alpha-stat strategy. The study sup-
However, there are few studies that have directly ported the hypothesis that had been proposed,
demonstrated this fact or that have demonstrated namely, that the pH-stat strategy is safer for neo-
an improved clinical outcome, particularly with natal and infant bypass. All perioperative compli-
respect to neurological factors (Murkin et al. 1995). cations were more common with the alpha-stat
strategy. Even mortality approached a significant
kProspective Randomized Study p value of 0.05. In addition the hospital course of
of pH Strategy patients with transposition was significantly
A prospective randomized trial of pH strategy improved with the pH-stat strategy including a
was undertaken at Children’s Hospital Boston significantly shorter duration of intubation and a
between 1992 and 1996 (du Plessis et al. 1997). shorter stay in the intensive care unit. Subsequent
One hundred eighty-two patients less than neurodevelopmental studies in these patients
162 R.A. Jonas
Development indexes
positive correlation
between arterial PCO2 dur-
ing cooling and develop-
mental score, that is, 100
children undergoing the
alpha-stat strategy had a
worse developmental out-
come (From Jonas et al. 80
1993; used with permis-
sion)
60
30 40 50 60 70 80 90
7 PCO2 (torr) at circulatory arrest
using the Bayley scale of infant development particularly susceptible to choreoathetosis were
demonstrated a consistent trend toward an
patients with multiple aortopulmonary collateral
improved psychomotor development index in vessels. Particularly for these patients, there is a
patients randomized to the pH-stat strategy risk that alkalinity will result in cerebral vaso-
(Bellinger et al. 2001). However, this was a diverse constriction and pulmonary vasodilation exac-
group of patients (in contrast to the circulatory erbating the steal of blood from the cerebral
arrest trial that enrolled only patients with trans- circulation into the lungs. The findings of these
position), and it also predated the era of genetic retrospective studies resulted in an enhanced
testing. Therefore, overall and perhaps not sur- appreciation of the dangers of alkalinity for pedi-
prisingly, there was not a statistically significantly atric patients undergoing hypothermic bypass.
advantage for the pH-stat strategy with respect to By shifting back to the pH-stat strategy, choreo-
developmental outcome though once again the athetosis was essentially eliminated as a neuro-
overall trend was definitely in this direction. logical complication of hypothermic bypass.
Furthermore the perioperative results alone con-
vincingly documented that the pH-stat strategy is kWhy Is the pH-Stat Strategy Not Used
preferable to the alpha-stat strategy for hypother- Routinely for Pediatric Bypass?
mic bypass. Adding carbon dioxide to the oxygenator sweep
gas increases the complexity of managing perfu-
kRetrospective Studies of Development sion for the perfusion technician. For perfusionists
A small retrospective study of development who mainly undertake adult perfusion, it can be
looked at patients who underwent the Senning intimidating to have to manage not only a smaller
operation under circulatory arrest during the and modified circuit for pediatric perfusion but in
period when the pH strategy was being changed addition having to manage blood gases in a differ-
from a more acidotic (pH stat) to more alkaline ent fashion. Furthermore there has been less use of
(alpha-stat) strategy (Jonas et al. 1993). Despite hypothermia and particularly deep hypothermia
the fact that only 16 patients were studied, there than in the early years of pediatric bypass. At a
was a remarkably powerful influence of pH strat- mild degree of hypothermia such as 30–32 °C,
egy on developmental outcome with a p value of there is very little difference between the pH-stat
0.002 (. Fig. 7.7). This finding was also consis- strategy and alpha-stat strategy. In fact some cen-
tent with the fact that an epidemic of choreoath- ters have eliminated the use of hypothermia alto-
etosis occurred at Children’s Hospital Boston gether and undertake normothermic bypass
beginning in the mid 1980s and coincident with (Corno 2007). So far there are no randomized pro-
the introduction of the alkaline alpha-stat strat- spective trials that have documented that there is
egy (Wong et al. 1992). Patients who appeared any advantage in applying normothermic bypass.
Chapter 7 · Advances in Cardiopulmonary Bypass for the Neonate and Infant
163 7
150
37°C
Oxygen consumption (ml.min–1.m–2)
100
30°C
50 25°C
20°C
15°C
0
0.0 0.5 1.0 1.5 2.0 2.5
Perfusion flow rate (1.min–1.m–2)
..Fig. 7.8 Even a mild degree of hypothermia markedly reduces cerebral oxygen consumption. Thus, a lower flow
rate safely supplies adequate substrate. Lower flow generates fewer emboli, reduces the inflammatory effects of bypass,
and allows a lower reservoir level and hence less addition of blood products
7.3.3 Risks of Normothermic Bypass it had been recognized by this time that the
«homologous blood syndrome» was an important
Normothermic bypass requires a much higher source of morbidity after cardiopulmonary bypass
flow rate than mildly hypothermic bypass (Gadboys et al. 1962). During the 1980s with the
(. Fig. 7.8). There is a steep decline in the meta- discovery of HIV as well as hepatitis C and there-
bolic rate of the brain with even a mild degree of fore increasing cost for testing and storing bank
hypothermia. Thus, the safety margin with hypo- blood, there was increasing pressure from hospital
thermia is substantially increased, for example, in blood banks for cardiac surgeons to reduce blood
the event that a technical problem with the bypass usage during cardiopulmonary bypass.
circuit requires a temporary stop of cardiopulmo-
nary bypass. The greater flow rate required for 7.3.4.1 Safe Limits of Hemodilution
normothermia also results in increased delivery of One of the most influential studies regarding the
microemboli and increased use of the cardiotomy question of the safe limit of hemodilution was pub-
suction system. This is where most of the blood lished by Kawashima (Kawashima et al. 1974). They
injury occurs such as hemolysis and complement used a noncardiopulmonary bypass dog model and
activation. Hypothermia per se reduces the determined that systemic oxygen consumption was
inflammatory effects of cardiopulmonary bypass. maintained until the hematocrit fell to less than
20 %. It is important to recognize that using a non-
bypass model fails to take account of the fixed upper
7.3.4 Hemodilution limit of perfusion flow rate that exists in the setting
of cardiopulmonary bypass. In the nonbypass
In the earliest years of cardiopulmonary bypass in model, there is a compensatory increase in cardiac
the 1950s, pure blood was used to prime the bypass output that maintains oxygen delivery. Further
circuit. No doubt this reflected the inefficiency of studies were undertaken by Laver et al. (1975). They
early disk and mesh oxygenators. A high hemato- determined that in the setting of profound hypo-
crit was required to achieve adequate oxygen thermia and circulatory arrest, even extreme hemo-
delivery. However, by the 1960s it was possible to dilution to hematocrits below 15 % appeared to be
hemodilute the perfusate and maintain reasonable clinically well tolerated. Ott and Cooley extended
oxygen delivery (Neptune et al. 1960). Furthermore the limits of hemodilution among Jehovah’s Witness
164 R.A. Jonas
† #
80 †
60 †
#
#
40 #; p, 0.05 I vs. II Group III
# *; p, 0.05 II vs. III Group II
20 † #
# * †; p, 0.05 III vs. I Group I
0 # #
0 50 100 150 200 250 300 350
Time (minutes)
7 ..Fig. 7.9 Extreme hemodilution to a hematocrit of 10 % (group I) was associated with a significant decline in
cerebral phosphocreatine as measured by magnetic resonance spectroscopy in piglets undergoing cooling to deep
hypothermia. Piglets with a hematocrit of 20 % (group II) and 30 % (group III) demonstrated a slight increase in
phosphocreatine during cooling (From Shin’oka et al. 1996; used with permission)
patients. They reported in 1977 that they had per- five different groups. Two of the groups had a
formed 542 operations without blood transfusion relatively low hematocrit of 20 %, but the colloid
(Ott and Cooley 1977). Work undertaken in the oncotic pressure was boosted by addition of
1990s at the Mayo Clinic using a dog model with either hetastarch or pentafraction. In another
cardiopulmonary bypass also suggested that group modified ultrafiltration was undertaken at
extreme hemodilution to a hematocrit of 12–15 % the conclusion of bypass. The conclusions of this
was «safe» (Cook et al. 1997). The reduced perfu- study were that both higher hematocrit and
sion pressure resulting from hemodilution was higher colloid oncotic pressure with pentafrac-
compensated for in this study by an increase in tion improved cerebral recovery after deep hypo-
pump flow rate to double the normal flow. thermic circulatory arrest. Higher hematocrit
improved cerebral oxygen delivery but did not
7.3.4.2 Laboratory Studies reduce total body edema. Modified ultrafiltration
of Hemodilution at Children’s after cardiopulmonary bypass was less effective
Hospital Boston than having a higher initial prime hematocrit or
A piglet model of cardiopulmonary bypass was colloid oncotic pressure.
developed that employed simultaneous magnetic
resonance spectroscopy and near-infrared spectros- 7.3.4.3 Microcirculation During Deep
copy as well as survival of the animals after 1 h of Hypothermia
deep hypothermic circulatory arrest. An initial The concept that deep hypothermia was associ-
study compared hematocrits of 10, 20, and 30 % ated with disturbances of the microcirculation
(Shin’oka et al. 1996). It was found that there was a became increasingly entrenched in cardiac surgi-
significantly more rapid recovery of high-energy cal texts from the 1970s onward (Messmer et al.
phosphates after circulatory arrest with the highest 1972). It was felt that increased red cell rigidity
hematocrit (. Fig. 7.9). The cerebral oxyhemoglo- and increased plasma viscosity resulted in occlu-
bin level measured by magnetic resonance spectros- sion of capillaries. Hemodilution was proposed as
copy was maintained at a significantly higher level a method to counteract this microcirculatory dis-
with a higher hematocrit. The neurological deficit turbance.
score was significantly improved in the highest
hematocrit group relative to the other two groups. 7.3.4.4 Potential Disadvantages
In an extension of the initial laboratory study of Hemodilution
of hemodilution, the differential effects of oncotic The most important risk of hemodilution is that is
pressure and oxygen delivery were analyzed reduces oxygen carrying capacity. Below a hemato-
(Shin’oka et al. 1998). Piglets were separated into crit of 20 %, the oxygen transport capacity of blood
Chapter 7 · Advances in Cardiopulmonary Bypass for the Neonate and Infant
165 7
declines quite rapidly. However, this is not the only ..Table 7.4 Randomized clinical trial of hematocrit
risk of hemodilution. Hemodilution also results in a
marked decrease in viscosity and therefore a reduc- Eligible Excluded
tion in perfusion pressure. While this is not of great
significance for the pediatric patient, it is very impor- Planned two Weight <2.3 kg
ventricle repair
tant for the adult patient who may have a carotid or using CPB
cerebrovascular stenosis. Furthermore cerebral
blood flow must increase in order to maintain cere- <9 months old Genetic syndrome
bral oxygen delivery. Thus, there is potential for Major extracardiac anomalies
greater delivery of both particulate and gaseous cere-
Previous cardiac surgery
bral microemboli when hemodilution is employed.
Arch reconstruction
7.3.4.5 Study of Cerebral
A
Microcirculation During Deep Children’s Hospital Boston December 1996–Decem-
ber 2000
Hypothermia
In order to study the effects of hypothermia and
hemodilution on the cerebral microcirculation, a neurological function with the hematocrit of 30 %
new model using intravital microscopy in the pig relative to the more hemodilute animals.
was developed (Duebener et al. 2001). A burr hole Furthermore the histological score was signifi-
was placed in the skull of a pig. The dura and arach- cantly worse with a p value of 0.02 in animals that
noid were removed from the surface of the brain. were subjected to hemodilution to 10 %.
Using a scanning microscope, the microcirculation
on the surface of the brain can be viewed. By admin-
istering fluorescent dyes, it is possible to label white 7.3.5 Clinical Studies of Hematocrit
cells and to label the plasma (FITC, fluorescein iso-
thiocyanate) thereby allowing visualization of red An initial prospective randomized clinical trial of
cells. An initial study that was undertaken compared hemodilution was undertaken at Children’s Hospital
hemodilution to 10, 20, and 30 % during cooling to Boston between 1996 and 2000 (Jonas et al. 2003).
deep hypothermia as well as before and after hypo- The eligibility criteria are shown in . Table 7.4. All
thermic circulatory arrest. It was found that the patients were less than 9 months of age at the time of
functional capillary density, a measure of capillaries surgery and were undergoing biventricular repair
perfused within a given area, was well maintained using cardiopulmonary bypass. Most patients did
with an undiluted hematocrit of 30 % at deep hypo- not undergo circulatory arrest. Patients were ran-
thermia and with full flow cardiopulmonary bypass. domized to a hematocrit of either 20 or 30 %.
Hemodilution resulted in an increased flow rate rela- However, as shown in . Fig. 7.10, the hematocrits
tive to baseline confirming the risk of greater emboli achieved were 27.8 ± 3.2 for the higher hematocrit
delivery with hemodilution (Duebener et al. 2001). group and 21.5 ± 2.9 for the lower hematocrit group.
Following deep hypothermic circulatory arrest, Thus, the spread of average hematocrit was only 6 %.
reperfusion occurred more rapidly and more Seventy-three patients were randomized to the
evenly with a hematocrit of 30 % relative to a hema- higher hematocrit group and 74 to the lower hema-
tocrit of 10 %. Furthermore there was greater acti- tocrit group. The use of blood products was essen-
vation of white cells in the hemodilute group of tially identical between the two groups. This was
10 %. These findings suggest that there is greater achieved through conventional ultrafiltration and
risk of endothelial injury during deep hypothermic reduced prime volume circuits. There was 1 death
and circulatory arrest if a severe degree of hemodi- among 147 patients for an overall mortality of 0.7 %.
lution is employed. It is possible that this is hypoxic The lactate level 1 h after bypass was significantly
endothelial injury that may result in a reduced abil- higher with the lower hematocrit. Whole-body
ity to secrete nitric oxide. The endothelial activa- edema measured by bioimpedance was significantly
tion also results in a greater number of rolling white higher with the lower hematocrit. Interestingly the
cells that adhere to the activated endothelium. lowest cardiac index measured by thermodilution
The animals that were studied were tested for for the first 24 h postoperatively was significantly
functional recovery after circulatory arrest. There lower with the lower hematocrit. This was true for
was a significantly more rapid return to normal all subgroups including the transposition subgroup
166 R.A. Jonas
7
.15
who had the lowest cardiac index postoperatively as of less than 20 % are now strenuously avoided
well as in the tetralogy/truncus subgroup which had although they used to be standard of care for deep
the intermediate cardiac output and the VSD/com- hypothermia. In order to maintain a reasonable
plete AV canal group which had the highest cardiac safety margin from the cut point of 23.5 %, most
output (. Fig. 7.11). centers use a hematocrit of at least 25 % and during
Developmental testing was undertaken at rewarming will use conventional ultrafiltration to
1 year of age. There was a significantly lower psy- hemoconcentrate up to a hematocrit of 30–35 %. In
chomotor development index, which assesses patients who will be cyanotic after weaning from
motor skills, among the lower hematocrit patients. bypass, for example, patients with hypoplastic left
A significantly higher percentage of patients in the heart syndrome undergoing the Norwood proce-
lower hematocrit group scored below two stan- dure, it is now common practice to hemoconcen-
dard deviations of normal (p = 0.01). This resulted trate up to a hematocrit of 40 % for weaning from
in the data and safety monitoring board of the bypass.
NIH discontinuing the trial before its planned
completion date. However, the trial was subse-
quently extended to compare a hematocrit of 25 % 7.3.6 Interaction of Flow Rate, pH,
vs. hematocrit of 35 % (Newburger et al. 2008). Hematocrit, Blood
The extension trial did not show any additional Temperature, and Brain
advantage in increasing the hematocrit to 35 %. Temperature
The data analysis of the combined trials suggested
that there was a cut point at approximately 23.5 % The cardiac surgeon should be acutely aware of
below which there was a higher probability of a the patient’s brain temperature during cardiopul-
low psychomotor development index score (Wypij monary bypass. Nasopharyngeal temperature and
et al. 2008). tympanic membrane temperature will give
approximations as to the patient’s brain tempera-
7.3.5.1 Modifications to Clinical ture. When the brain is at normothermia, it is par-
Practice as a Result ticularly important to avoid a «perfect storm» of
of the Hematocrit Trial limited oxygen delivery. The most typical perfect
Most centers have modified their hemodilution storm occurs when flow to the brain is reduced,
practice in response to the prospective randomized for example, there is a fixed upper bypass flow rate
trials of hematocrit described above. Hematocrits (even at «full flow») and collaterals are stealing
Chapter 7 · Advances in Cardiopulmonary Bypass for the Neonate and Infant
167 7
* p < 0.05
4.5
120
*
4.0
Score
* * 100
3.5
80
3.0
60
2.5
0 3 6 9 12 15 18 21 24 Lower Higher Lower Higher
Time after cross-clamp removal (hours) hematocrit hematocrit
Psychomotor development Mental development
c
index (n 5 109) index (n 5 112)
Psychomotor development index
120
p < 0.02
100
80
60
15 20 25 30 35
Hematocrit at onset of low flow (%)
..Fig. 7.11 Results of a randomized prospective clinical trial of lower hematocrit (21.5 %) versus higher hematocrit
(27.8 %) at Children’s Hospital Boston. a The use of a higher hematocrit (closed circles) was associated with a significantly
higher cardiac index at 6 and 9 h after cross-clamp removal compared with lower hematocrit (open circles). b
Developmental assessment at 1 year of age demonstrated a significantly higher psychomotor development index (a
measure of motor skills) in patients managed with a higher hematocrit (p = 0.008). c Analysis using hematocrit as a con-
tinuous variable demonstrated a significant association between high Psychomotor Development Index at 1 year of age
and higher hematocrit during cardiopulmonary bypass
from the systemic circulation (open shunt, ability has been increased at a time when the brain
collaterals, etc.) and the oxyhemoglobin dissocia- metabolic rate is still low.
tion curve is left shifted because of cold blood and
alkalinity. Hemodilution will further exacerbate 7.3.6.1 The Importance of Brain
the limitation of oxygen delivery (. Table 7.5). Temperature
Thus, the early phase of cooling when the brain is The most critically important point for the car-
warm (high metabolic rate) and the blood is cold diac surgeon and perfusionist to remember is that
and dilute (low oxygen availability) is the highest the brain metabolic rate is essentially exponential
risk situation (Sakamoto et al. 2002). Monitoring with respect to the effect of temperature. The
venous oxygen saturation can be quite misleading hyperthermic brain that is above 37 °C is at
because during the early cooling phase, venous exceedingly high risk of brain injury because of a
oxygen saturation may be falsely elevated due to markedly increased metabolic rate. On the other
the hypothermic blood and leftward shift of oxy- hand, a very mild degree of hypothermia, for
hemoglobin. example, to 32 or 34 °C, substantially decreases
The rewarming phase is generally a less dan- brain metabolic rate. It is also important to appre-
gerous time because the brain is cool and the ciate that temperature gradients exist within the
blood is warmer so that oxyhemoglobin dissocia- brain. The cortex of the brain tends to be at a
tion has been shifted rightward and oxygen avail- lower temperature than the deep brain structures
168 R.A. Jonas
..Table 7.5 The probability of histologic injury after circulatory arrest increases with higher temperature,
lower hematocrit, and more alkaline pH
Multivariate predictor
Temperature pH strategy Hematocrit (%) HCA (min) Predicted total histologic score
15 pH 30 60 0.0
15 pH 20 60 0.5
15 Alpha 30 60 1.0
15 pH 30 80 or 100 1.5
15 Alpha 20 60 2.0
25 pH 30 60 2.5
25 pH 20 60 3.5
25 Alpha 30 60 4.0
25 pH 30 80 or 100 4.5
25 Alpha 20 60 5.0
25 pH 20 80 or 100 6.0
such as the thalamus and basal ganglia. This tem- 7.4 Conclusion
perature gradient can be magnified by the use of
surface cooling techniques, for example, packing Continuing refinement in the hardware and tech-
the head in ice. niques of cardiopulmonary bypass specifically
designed for neonates and infants will result in
further reduction in the morbidity of
7.3.7 Adjunctive Techniques cardiopulmonary bypass. However, it is critically
for Brain Protection important to remember at all times that
cardiopulmonary bypass is not a physiological
Many innovative methods for maintaining brain state so that expeditious and yet accurate surgery
perfusion have been developed including retrograde including minimization of the use of blood prod-
cerebral perfusion and antegrade cerebral perfusion. ucts will result in an optimal outcome.
Studies of retrograde cerebral perfusion both in the
lab and clinically have led to its complete discontin- References
uation. There is still enthusiasm for various methods
of antegrade cerebral perfusion though today the Ando M, Takahashi Y, Suzuki N (2004) Open heart surgery
only clinical studies that have been undertaken have for small children without homologous blood transfu-
sion by using remote pump head system. Ann Thorac
been disappointing and have if anything suggested a
Surg 78:1717–1722
higher level of brain injury, particularly embolic Aoki M, Nomura F, Stromski ME et al. (1993) Effects of pH
injury, with this technique relative to deep hypother- on brain energetics after hypothermic circulatory
mic circulatory arrest (Goldberg et al. 2007). arrest. Ann Thorac Surg 55:1093–1103
Chapter 7 · Advances in Cardiopulmonary Bypass for the Neonate and Infant
169 7
Bellinger DC, Wypij D, du Plessis AJ et al. (2001) Kawashima et al. (1974) Safe limits of hemodilution in car-
Developmental and neurologic effects of alpha-stat diopulmonary bypass. Surgery 76:391
versus pH—stat strategies for deep hypothermic car- Laver MB, Buckley MJ, Austen WG (1975) Extreme hemodi-
diopulmonary bypass in infants. J Thorac Cardiovasc lution with profound hypothermia and circulatory
Surg 121:374–383 arrest. Bibl Haematol 41:225–238
Bellinger DC, Wypij D, duPlessis AJ, Rappaport LA, Jonas Limperopoulos C, Tworetzky W, McElhinney DB, Newburger
RA, Wernovsky G, Newburger JW (2003) JW, Brown DW, Robertson RL Jr, Guizard N, McGrath E,
Neurodevelopmental status at eight years in children Geva J, Annese D, Dunbar-Masterson C, Trainor B,
with dextro-transposition of the great arteries: the Laussen PC, du Plessis AJ (2010) Brain volume and
Boston Circulatory Arrest Trial. J Thorac Cardiovasc metabolism in fetuses with congenital heart disease:
Surg 126:1385–1396 evaluation with quantitative magnetic resonance
Cavaletti G, Garavaglia P, Arrigoni G, Tredici G (1990) imaging and spectroscopy. Circulation 121:26–33
Persistent memory impairment after high altitude Messmer K, Sunder-Plassman L, Klovekorn WP, Holper K
climbing. Int J Sports Med 11:176 (1972) Circulatory significance of haemodilution : rhe-
Cook DJ, Orszulak TA, Daly RC, MacVeigh I (1997) Minimum ological changes and limitations. Adv Microcirc 4:1–77
hematocrit for normothermic cardiopulmonary Murkin JM, Martzke JS, Buchan AM, Bentley C, Wong CJ
bypass in dogs. Circulation 96(suppl II):200–204 (1995) A randomized study of the influence of perfu-
Corno AF (2007) Normal temperature and flow: are the sion technique and pH management strategy in 316
‘physiological’ values so scary? Eur J Cardiothorac Surg patients undergoing coronary artery bypass surgery.
31:756 I. Mortality and cardiovascular morbidity. J Thorac
De Somer F (2007) What is optimal flow and how to vali- Cardiovasc Surg 110:340–348
date this. J Extra Corpor Technol 39:278–280 Murphy GS, Hessel EA 2nd, Groom RC (2009) Optimal per-
Dewall RA, Gott VL, Lillehei CW, Read RC, Varco RL, Warden fusion during cardiopulmonary bypass: an evidence-
HE (1956) Total body perfusion for open cardiotomy based approach. Anesth Analg 108:1394–1417
utilizing the bubble oxygenator; physiologic Nagatsu M, Sawatari K, Imai Y, Takanashi Y, Kurosawa H,
responses in man. J Thorac Surg 32:591–603 Nakae S, Hoshino S, Soejima K, Sugiyama Y, Yoshikai M
du Plessis AJ, Jonas RA, Wypij D et al. (1997) Perioperative et al. (1987) Clinical experience with a membrane oxy-
effects of alpha stat versus pH stat strategies for deep genator, COBE VPCML, for pediatric open heart sur-
hypothermic cardiopulmonary bypass in infants. gery—comparison with the bubble oxygenator. Kyobu
J Thorac Cardiovasc Surg 114:991–1001 Geka 40:118–122
Duebener LF, Sakamoto T, Hatsuoka S et al. (2001) Effects Neptune et al. (1960) Open heart surgery without the
of hematocrit on cerebral microcirculation and tissue need for donor blood priming in the oxygenator. New
oxygenation during deep hypothermic bypass. Engl J Med 263:111
Circulation 104:1260–1264 Newburger JW, Jonas RA, Wernovsky G, Wypij D, Hickey
Edmunds LH Jr (1998) Inflammatory response to cardiopul- PR, Kuban KC, Farrell DM, Holmes GL, Helmers SL,
monary bypass. Ann Thorac Surg 66(5 Suppl):S12–S16 Constantinou J et al. (1993) A comparison of the
Gadboys HL, Slonim R, Litwak RS (1962) Homologous perioperative neurologic effects of hypothermic cir-
blood syndrome: I. Preliminary observations on its culatory arrest versus low-flow cardiopulmonary
relationship to clinical cardiopulmonary bypass. Ann bypass in infant heart surgery. N Engl J Med 329:
Surg 156:793–804 1057–1064
Goldberg CS, Bove EL, Devaney EJ, Mollen E, Schwartz E, Newburger JW, Jonas RA, Soul J et al. (2008) Randomized
Tindall S, Nowak C, Charpie J, Brown MB, Kulik TJ, Ohye trial of hematocrit 25% versus 35% during hypother-
RG (2007) A randomized clinical trial of regional cere- mic cardiopulmonary bypass in infant heart surgery.
bral perfusion versus deep hypothermic circulatory J Thorac Cardiovasc Surg 135:347–354
arrest: outcomes for infants with functional single Ott DA, Cooley DA (1977) Cardiovascular surgery in
ventricle. J Thorac Cardiovasc Surg 133:880–887 Jehovah’s Witnesses: report of 542 operations without
Hamilton C, Steinlechner B, Gruber E et al. (2004) The oxy- blood transfusion. JAMA 238:1256–1258
gen dissociation curve: quantifying the shift. Perfusion Rider AR, Ji B, Kunselman AR, Weiss WJ, Myers JL, Undar A
19:141–144 (2008) A performance evaluation of eight geometrically
Henriksen L (1986) Brain luxury perfusion during cardio- different 10 Fr pediatric arterial cannulae under pulsa-
pulmonary bypass in humans: a study of cerebral tile and nonpulsatile perfusion conditions in an infant
blood flow response to changes in CO2, O2 and blood cardiopulmonary bypass model. ASAIO J 54:306–315
pressure. J Cereb Blood Flow Metab 6:365–378 Sakamoto T, Zurakowski D, Duebener LF, Hatsuoka S, Lidov
Jonas RA, Bellinger DC, Rappaport LA et al. (1993) pH strat- HG, Holmes GL, Stock UA, Laussen PC, Jonas RA (2002)
egy and developmental outcome after hypothermic Combination of alpha-stat strategy and hemodilution
circulatory arrest. J Thorac Cardiovasc Surg 106:362–368 exacerbates neurologic injury in a survival piglet
Jonas RA, Wypij D, Roth SJ et al. (2003) The influence of model with deep hypothermic circulatory arrest. Ann
hemodilution on outcome after hypothermic cardio- Thorac Surg 73:180–189
pulmonary bypass: results of a randomized trial in Severinghaus JW (1966) Blood gas calculator. J Appl
infants. J Thorac Cardiovasc Surg 126:1765–1774 Physiol 21:1108–1116
170 R.A. Jonas
Shin’oka T, Shum-Tim D, Jonas RA, Lidov HG, Laussen PC, Wong PC, Barlow CF, Hickey PR et al. (1992) Factors associ-
Miura T, DuPlessis A (1996) Higher hematocrit improves ated with choreoathetosis after cardiopulmonary
cerebral outcome after deep hypothermic circulatory bypass in children with congenital heart disease.
arrest. J Thorac Cardiovasc Surg 112:1610–1620 Circulation 86(Supp II):118–126
Shin’oka T, Shum-Tim D, Laussen PC, Zinkovsky SM, Lidov Wypij D, Jonas RA, Bellinger DC et al. (2008) The effect of
HG, DuPlessis A, Jonas RA (1998) Effects of oncotic hematocrit during hypothermic cardiopulmonary
pressure and hematocrit on outcome after hypother- bypass in infant heart surgery: results from the com-
mic circulatory arrest. Ann Thorac Surg 65:155–164 bined Boston hematocrit trials. J Thorac Cardiovasc
Swan H, Sanchez M, Tyndall M, Koch C (1990) Quality con- Surg 135:355–360
trol of perfusion: monitoring venous blood oxygen Zeltser I, Jarvik GP, Bernbaum J, Wernovsky G, Nord AS,
tension to prevent hypoxic acidosis. J Thorac Gerdes M, Zackai E, Clancy R, Nicolson SC, Spray TL,
Cardiovasc Surg 99:868–872 Gaynor JW (2008) Genetic factors are important
Wilson E (1977) Theoretical analysis of the effects of two determinants of neurodevelopmental outcome after
pH regulation patterns on the temperature sensitivi- repair of tetralogy of Fallot. J Thorac Cardiovasc Surg
ties of biological systems in non homeothermic ani- 135:91–97
mals. Arch Biochem Biophys 181:409–419
7
171 8
Cardiovascular Tissue
Engineering
Mathias H. Wilhelmi and Axel Haverich
References – 188
End-stage organ failure and tissue loss represent Over the years, various methods and techniques to
the most devastating and costly problems in manufacture bioartificial tissues have been
modern medicine. Over the last years, many described. Although different in detail, almost all of
treatment options, i.e. tissue and organ trans- these approaches can be categorized according to
plantation as well as the development of one of the following three superordinate concepts:
advanced alloplastic prostheses and devices, 1. Guided Tissue Regeneration: (engineered) cell-
have significantly improved patient outcome. free matrices of various origins—biological,
However, due to a number of limiting factors, synthetic or combined—are implanted into a
both these substitutional and reconstructive recipient organism for in vivo cellular (re-)
options are still imperfect. The most important seeding, maturation and restoration. Example:
factors are: an in vitro decellularized biological blood vessel
1. The increasing lack of donor organs for trans- or heart valve of allogeneic or xenogeneic origin
plantation and associated second-line risks is implanted into a recipients organism and
due to lifelong immunosuppressive therapy, then regenerates under in vivo conditions in
which may induce the development of malig- corpore.
8 nant tumors 2. Selective Cellular Transfer: systemic or local
2. The qualitative and quantitative shortage of application of cell preparations (of autologous,
usable donor tissues allogeneic or xenogeneic origin) to elevate the
3. An increased risk for infectious and thrombo- concentration of a specific cell type and to
embolic complications, i.e. following the provide a defined cell-mix within a target tis-
implantation of mechanical devices and allo- sue area, respectively. Example: injection of a
plastic grafts stem cell suspension into areas of myocardial
4. The disability of alloplastic prostheses to infarction to induce regenerative processes and
dynamically adapt to altered surroundings, thus to improve tissue function
e.g. an apparent infection 3. Tissue Engineering (classical sense): in vitro
isolated, expanded and, sometimes, differen-
tiated cells (preferentially of autologous origin)
8.1.1 Aim of Discipline are seeded in vitro onto or into a matrix scaf-
fold to form a living tissue or solid organ.
Seeking for a method «designed and constructed to Example: endothelial cells isolated from an
meet the needs of each individual patient» (Vacanti individual patient are expanded and seeded
and Langer 1999) and to avoid or at least to reduce in vitro on the luminal surface of a tubular
the above-mentioned limitations of currently matrix scaffold to generate a bioartificial
used prostheses, the idea of (cardiovascular) «tis- vascular prosthesis.
sue engineering» was created in 1987 by members
of the American Science Foundation (NSF) in Many different (cardiovascular) tissue struc-
Washington, DC. It describes the interdisciplin- tures have already been generated and larger
ary approach to combine and apply principles and 3D-tissue structures, e.g. bioartificial myocar-
methods of engineering and life sciences towards dium grown in vitro are beginning to take shape
the development of biological substitutes that (Ferber 1999). However, many obstacles and
restore, maintain and/or improve diminished tis- challenges still remain. This chapter focuses on
sue functions (Ferber 1999; Vacanti 1988; Vacanti surgically implantable prostheses and thus pre-
and Langer 1999). The concept of Cardiovascular dominantly reports on selected examples and
Tissue Engineering follows the bioartificial genera- concepts of grafts generated by methods of guided
tion of solid cardiovascular tissue structures, such tissue regeneration and tissue engineering in the
as the myocardium, heart valves and blood vessels classical sense. Additionally, some historical
as well as the application of cellular preparations, background information as well as still existing
e.g. to treat arrhythmic disorders or myocardial limitations and prospective key activities in car-
infarction. diovascular tissue generation are provided.
Chapter 8 · Cardiovascular Tissue Engineering
173 8
8.2 Components Necessary acellular or decellularized matrices and build-up
for Bioartificial Tissue extracellular matrix components through their
Generation proliferative activity and differentiation and thus
regenerate, remodel and construct new tissues,
Regardless of the desired tissue type, the success- respectively. A cell source should be easily acces-
ful generation of bioartificial tissue constructs sible and ideally of autologous origin. The har-
relies on four superordinate components: vested cells should fast proliferate, be easily to
1. Tissue-specific cells which form, vitalize and differentiate into a desired cell type or—if already
establish the functionality of a desired tissue differentiated—should preserve their cellular
2. Matrix scaffolds, which provide niches to phenotype and function. They may not transmit
accommodate these cells and direct their growth pathogens and should be none antigenic.
within a definite three-dimensional architecture Fibroblasts, keratinocytes and endothelial cells
3. Stimulating signals (chemical or physical), are examples for such already differentiated and
which modulate cellular gene expressions and, highly proliferative cells and therefore are broadly
thus, cellular differentiation and synthetic applied in experimental as well as clinical settings
activity, e.g. extracellular matrix production of bioartificial skin, cartilage and cardiovascular
4. Cellular and humoral components of the graft generation/regeneration. However, other
recipient’s immune system, which adjudicate organ-specific cells, e.g. hepatocytes or cardio-
on tissue integration or graft deterioration myocytes, show no or only very limited and slow
and destruction proliferation (Teebken et al. 2005).
In all these cases, embryonic and adult stem
The ideal characteristics of a bioartificial cardio- cells are of special interest. Embryonic stem cells
vascular prosthesis are summarized in . Table 8.1. are able to differentiate into structures of all three
germinal sheets and thus are of high benefit—at
least experimentally. Due to legislation and ethi-
8.2.1 Cells cal concerns, its use is very limited or forbidden in
most countries. In contrast, adult stem cells are
Cells are key components in cardiovascular tissue only able to differentiate into a limited number of
generation and regeneration. They (re-)vitalize tissues, e.g. bone, cartilage, tendons and skeletal
muscle. However, due to its legal harmlessness
and good availability even in adult, old and multi-
..Table 8.1 Properties of an ideal (bioartificial) morbid patients, these cells are an attractive alter-
cardiovascular prosthesis native and object of intense research (Pittenger
et al. 1999).
Autologous origin
A third, relatively new approach is the emer-
Resistance against infection gence of so-called induced pluripotent stem cell
No transmission of pathogens
(iPS cell) technology, which base in ground-
breaking works of Yamanaka and colleagues
Non-carcinogenic (Takahashi and Yamanaka 2006) (. Fig. 8.1). They
Non-teratogenic demonstrated that the overexpression of four
genes («Yamanaka factors»= Oct4, Sox2, Klf4,
High hemo- and biocompatibility
c-Myc) allow for re-programming of already dif-
Lifelong durability ferentiated somatic cells into the embryonic state
Ability to grow and thus, at least in theory, the creation of a whole
organism from one single somatic cell. Over the
Ability to integrate, regenerate and dynamically
last years, many groups picked up this unique
adapt to changing conditions
concept and, i.e. performed studies to simulate
Good availability genetic cardiovascular disorders (Narsinh et al.
Passable costs 2011; Freund and Mummery 2009) or to develop
and test drugs (Inoue and Yamanaka 2011; Wu
Good surgical manageability
and Hochedlinger 2011). Some researchers
174 M.H. Wilhelmi and A. Haverich
a b
..Fig. 8.1 a, b iPS-cell culture. Cell culture of human umbilical vein blood derived iPS cells (HSC-iPS-clone2):
a original magnification 50×; b original magnification 200× (Courtesy of Prof. U. Martin and Dr. A. Haase, Leibniz Research
Laboratories for Biotechnology and Artificial Organs—LEBAO, Hannover Medical School, Hannover, Germany)
8
anticipate that this technique will close the gap However, apart from this anatomic advantage,
between the advantages of pluripotent cells and major disadvantages simultaneously lie in the
legislation. Although many efforts have been fixed shape and size of these matrices as well as in
undertaken to follow this concept and some its immunogenic/antigenic potential due to their
working groups already reported on successful allogeneic and xenogeneic origin.
applications in experimental settings (Schmidt Regarding the latter aspect, we recently ana-
2010; Rufaihah 2011), especially the development lyzed decellularized equine carotid arteries
of methods to assess the risk for tumor induction, (dEAC) with a low DNA content for residual cel-
e.g. teratomas is still open. lular proteins. A detergent-based decellulariza-
tion protocol including endonuclease treatment
resulted in dEAC with 0.6 ± 0.15 ng DNA/mg dry
8.2.2 Matrices weight representing 0.33 ± 0.14 % of native tissue
DNA content. In contrast, when matrices were
Another important prerequisite for the generation homogenized and extracted by high detergent
of tissues is a structural fundament—the matrix. concentrations Western blot analyses revealed
Matrices are of biological, synthetic or combined cytosolic and cytoskeleton proteins like GAPDH
(hybrid constructs) origin. They serve as struc- and smooth muscle actin which were depleted to
tural backbone and provide guidance and anchor- 4.1 ± 1.9 % and 13.8 ± 0.55 %, respectively. Also
age for seeding cells. However, in contrast to putative immunogenic MHC I complexes and the
construction materials in building industries, tis- alpha-Gal epitope were reduced to only
sue matrices are non-static but prone to dynamic 14.8 ± 1.2 % and 15.1 ± 2.05 %, respectively. Mass
tissue remodeling in the sense of situation-adapted spectrometry of matrix extracts identified 306
synthesis and degradation of its components and proteins belonging to cytosol, organelles, nucleus
thus possess varying biomechanical properties. and cell membrane. Moreover, aqueous matrix
An ideal matrix scaffold should exhibit the follow- extracts evoked a pronounced antibody forma-
ing characteristics: it should be bio- and hemo- tion when administered in mice and thus display
compatible non- antigenic and immunogenic high immunogenic potential. Our data indicated
supportive for cellular growth and (neo-) angio- that an established decellularization protocol
genesis to provide sufficient cell- and matrix which results in acellular matrices evaluated by
nutritionable to grow natural materials such as low DNA content reduces but not eliminates cel-
acellularized extracellular matrix components of lular components and thus may contribute to its
allogeneic or xenogeneic origin are mostly applied immunogenic potential in vivo (Böer et al. 2011).
for the generation of pre-defined anatomical Synthetic matrices are artificial constructs
structures, e.g. blood vessels or heart valves. often based on polymers, which are synthesized
Chapter 8 · Cardiovascular Tissue Engineering
175 8
out of biological, artificial or combined bioartifi- varies depending on the porosity of a certain
cial materials. Shape and size of the resulting con- matrix, tissues need vasa vasorum to ensure suf-
structs are highly variable and arbitrary and the ficient cell and matrix nutrition at lower dimen-
polymers applied in tissue engineering are often sions by diffusion. Therefore, pivotal questions
sprouts of polyhydroxy acid (PHA) of aliphatic regarding cell-cell interactions and the in vitro
polyesters, e.g. polyglycolic acid (PGA), polylactic induction of angiogenesis are as important as the
acid (PLA) or copolymers (PGLA) of these mate- matrix material itself. Referring to these aspects,
rials. Polyhydroxyalkanoates (PHAs) are a class of porosity of a scaffold and its internal pore orga-
natural occurring polymers and exhibit thermo- nization influences cell migration and plays a
plastic properties. They are biocompatible, reab- major role in its biodegradation dynamics, nutri-
sorbable and highly flexible and induce only slight ent diffusion and mechanical stability. Thus, in
inflammatory reactions after implantation into a order to control cell migration and cellular inter-
living organism. Because of these characteristics actions within the scaffold, novel technologies
and its high tensile strength, these polymers are capable to precisely produce predefined 3D
often applied for the bioartificial generation of structure designs are required as a first step. The
blood vessels and heart valves, too (. Table 8.2). two-photon polymerization (2PP) technique
Regardless of the matrix material used, the allows for the realization of those arbitrary 3D
most crucial and thus limiting factor of all cur- structures with submicron spatial resolution. We
rently undertaken approaches to generate living used this technique to generate highly porous
three-dimensional tissue structures remains 3D scaffolds made out of acrylated poly(ethylene
thickness. Beyond a critical thickness, which glycol) (PEG) and seeded the resulting matrix
Synthetic matrices
Biodegradable Derivatives of poly α-hydroxy acids of Suture material, vascular replacement therapy
aliphatic polyesters, e.g. polyglycolic
acid (PGA), polylactic acid (PLA) and
copolymers thereof (PLGA),
poly-epsilon-aminocaproic acid,
poly-4-hydroxybutyrate (P4HB),
polyglycolic acid (PGA), hyaff-11
(hyaluronic acid ester)
Biologic matrices
Secondary Native acellularized matrix scaffold of Decellularized heart valve, venous valve out
acellular small intestinal submucosa (sis), bone, of sis
heart valves or blood vessels
Non-acellularized Native matrix scaffolds, e.g. human Cryopreserved human allograft prostheses for
allograft/homograft prostheses heart valve-, vascular- or skin-replacement
therapy
a b
c d
..Fig. 8.2 Complex bioreactor system. Bioreactor system for in vitro generation of a vascular and b cardiac valve
grafts. The system shown in c allows for parallel generation of up to three vascular grafts and is housed in an incubator.
A computer system controls all relevant parameters, e.g. media flow velocities, pressure and shear stress d and media
flow is generated by e a centrifugal pump (Courtesy of Prof. M. Wilhelmi, Dept. of Cardiothoracic, Transplantation, and
Vascular Surgery, Hannover Medical School, Hannover, Germany)
to complete substitution by biological (= autolo- (Gross and Bill 1948; Gross et al. 1949). However,
gous, allogeneic or xenogeneic), synthetic (= allo- 3–5 years after implantation of these allogeneic
plastic) or hybrid vascular grafts. grafts, severe calcifications visible on X-rays
pointed to degenerative processes, which often
resulted in complete and initially inexplicable
8.3.1 eneration of Vascular Grafts
G graft loss (Dubost et al. 1952; Outdot and
Based on Biological Matrix Beaconsfield 1953; Outdot 1951).
Materials Addressing this observation, a first milestone
in the history of biological grafts was laid by
Autologous vessel grafts harvested from an indi- Rosenberg and co-workers, who tried to reduce
vidual patient to substitute or reconstruct his own the presumable underlying graft immunogenicity
diseased vessels are used since the early eigh- by impregnation. He used bovine carotid arteries
teenth century (Goyanes 1906; Murphy 1897; and tried to improve its in vivo performance by
Carrel 1902). The clinical application of alloge- impregnation with dialdehyde starch (Rosenberg
neic prostheses mainly harvested in the context of and Henderson 1956; Rosenberg 1976) and antic-
autopsies started in the 1940s and mainly based ipated that induced cross-linkings between extra-
on works of Charles Hufnagel and Robert Gross cellular matrix proteins of these vessels would
178 M.H. Wilhelmi and A. Haverich
weaken immunological responses of the recipient revealed endothelial monolayers on the luminal
while preserving graft structure/shape and pro- surface of these grafts, which stained positive for
longing its storage time. However, it emerged that von Willebrand factor. However, although histo-
dialdehyde starch was a bad choice. These grafts logical data were very promising, biomechanical
also demonstrated severe calcification and degra- tests revealed that these constructs had only very
dation. Subsequently, different chemical agent— limited structural stability so that Dacron nets
glutaraldehyde—was discovered and found to be had to be wrapped around the grafts to allow at
a much better choice. Many different tissues were least physiological pressure loads (Weinberg and
impregnated very successfully with this agent and Bell 1986). L’Heureux picked up the principle of
became readily available off the shelf. this method, changed some culture conditions
In 1972, Dardik evaluated glutaraldehyde pre- and noticed that it was possible to positively influ-
served human umbilical veins as an alternative ence graft stability and pressure tolerance/burst
biological bypass material, i.e. for lower extremity strength by modification of environmental condi-
bypasses (Dardik and Dardik 1973). However, as tions (Edelman 1999; L’Heureux et al. 1998).
already postulated by Kunlin in 1949, these grafts These two concepts rely on the principle of tis-
also failed in the long term, and the greater saphe- sue engineering in the classical sense. Campbell
nous vein was found to be the most suitable graft and co-workers also imitated this basic concept
8 in this anatomic area (Kunlin 1949). but utilized on in vivo situation for graft genera-
Reviewing the underlying mechanisms of tion (= guided tissue regeneration). They implanted
graft deterioration, two factors seem to play a par- silastic tubings into the peritoneal cavity of rats,
ticular role in this phenomenon: (i) immunologi- and after 2 weeks they observed that fibroblasts
cal reactions in the sense of subliminal tissue and mesothelial cells seeded on the outer surface
rejection (Wilhelmi et al. 2003a, b), which seem of these tubings. The resulting tubular tissue sheet
to be induced by the antigeneity of resident allo- was then dissected and everted so that the previ-
geneic cells and (ii) the method of tissue preserva- ously outer mesothelial cells subsequently built-
tion/fixation (glutaraldehyde) itself. As mentioned up the inner surface and thus mimicked the
before, initially this latter agent was used to reduce lamina interna. In animal models these bioartifi-
the immunogenicity of tissues via the cross- cial vessel grafts showed physiological reactivity
linking of collagen fibers to prolong its durability. towards vasoactive agents and were patent for up
However, over the years it became clear that to 4 months (Campbell et al. 1999). Another clas-
glutaraldehyde increases the risk for calcification, sical tissue engineering approach was used by a
potentially amplifies immunological reactions working group of Huynh. They used small intesti-
and, most importantly, inhibits processes of nal submucosa (SIS) and bovine type-I collagen to
in vivo regeneration (O’Brien et al. 1999). Today it generate a new kind of vascular prosthesis. After
is believed that histoincompatibilities are respon- removal of all cellular components via hypotonic
sible for the induction of immunological solutions, they tested autologous, allogeneic and
responses and resulting tissue rejection. We could xenogeneic grafts in large animal models and
indeed show that even established decellulariza- reported on excellent patency rates. In the histo-
tion protocols reduce but not eliminate all cellular logical analysis, they found these primary decel-
components and thus may still contribute to lularized implanted grafts to spontaneously
immunogenic reactions in vivo (Böer et al. 2011). re-seed in vivo and thus obviously underwent
The first vascular prosthesis completely gener- regenerative processes (Huynh et al. 1999).
ated on the basis of biological materials goes back However, in experiments using smaller animals
to Weinberg and Bell (Weinberg and Bell 1986). such as rats, these small calibre vessel grafts were
They seeded smooth muscle cells in vitro on a col- found to be occluded very early due to thrombus
lagen gel, which mimicked the lamina media, formation (Schmidt and Baier 2000).
added fibroblasts to the outer surface of this con- Apart from the use of conventional matrix
struct and thus generated a bioartificial adventi- materials, e.g. just decellularized preformed vas-
tia. Following 2 weeks of further in vitro culture, cular matrices of allogeneic or xenogeneic origin
endothelial cells were added to the luminal sur- or the de novo synthesis of collagen fibers as sug-
face to serve as an artificial lamina interna. gested by L’Heureux, another interesting concept
Scanning electron microscopic (SEM) evaluations was developed based on the use of fibrinogen and
Chapter 8 · Cardiovascular Tissue Engineering
179 8
fibrin. Fibrin is known to facilitate matrix synthe- able polylactide mesh structure (degradation time
sis by seeded cells compared to other reconsti- of 6–9 months), and again, after in vitro matura-
tuted proteins such as type I collagen. However, tion, the graft was ready for implantation
despite these promising attributes, fibrin gel alone (Tschoeke et al. 2009), also shown by others (Aper
possesses inadequate mechanical properties to et al. 2004) (. Fig. 8.3).
withstand implantation in the vascular system.
Therefore, Tschoeke et al. (2008) developed an
injection molding technique to completely inte- 8.3.2 eneration of Vascular Grafts
G
grate a non-biodegradable, high porous textile Based on Alloplastic Matrix
structure (pore size ~ 2 mm) into the vascular Materials
wall, which consists of an autologous fibrin gel
scaffold as cell carrier for the vascular smooth In 1952, Voorhees and co-workers identified an
muscle cells (Tschoeke et al. 2008). He hypothe- alloplastic material called Vinyon «N» (Voorhees
sized that the combination of a mesh and dynamic et al. 1952). Vascular conduits made out of this
culture conditions allows for the generation of material and tested in animal models revealed
mechanically stable and implantable vascular good initial functionality. However, soon it
grafts within a shorter cultivation period than tra- became clear that it was not possible to autoclave
ditional methods. He developed a two-step mold- this material without severe deformation and
ing technique to integrate a polyvinylidene pronounced shrinking. Even with these initially
fluoride (PVDF) mesh (pore size: 1–2 mm) in the somewhat disappointing data, many scientific
wall of a fibrin-based vascular graft (inner diam- groups began to think about alloplastic materials,
eter 5 mm) seeded with carotid myofibroblasts. and soon another material—polyester
The graft was cultured under increasing physio- (=Dacron)—was discovered and evaluated for its
logical flow conditions for 2 weeks. Cell growth applicability as a vascular prosthesis. A new era
and tissue development were excellent within the in vascular surgery was heralded and the clinical
fibrin gel matrix surrounding the PVDF fibers, introduction of these prostheses spread all over
and tissue structure demonstrated similarity to the world. More and above, a new medical
native tissue. The grafts were successfully sub- speciality—medical material engineering—came
jected to physiological flow rates and pressure up. Following the establishment of basic techni-
gradients from the outset, and mechanical prop- cal methods in the production of woven prosthe-
erties were enhanced by the mesh structure. Mean ses, it was tried to optimize material properties,
suture retention strength of the graft tissue was e.g. compliance, manageability. The necessity to
6.3 N and the burst strength was 236 mmHg, and do so based on the observation that the first gen-
thus small-calibre vascular grafts with good eration of these prostheses was very porous and
mechanical properties could be obtained within therefore had to be pre-clotted with blood prior
14 days. In a next step, the non-biodegradable to implantation. Furthermore, they were very
material was replaced by a midterm biodegrad- stiff and kinky and stenoses occurred when they
were implanted in angles. Therefore, Sauvage reveal much better patency rates. However, in
introduced a crimped prosthesis to avoid those contrast to the assumption that the greater saphe-
kinking-related stenoses (Sauvage et al. 1974). A nous vein may represent a universally applicable
further development in this sense was the appli- vessel graft, it should be noticed that due to prior
cation of external rings to prevent kinking and surgical interventions, varicoses or deep vein
outer velour coverage for better integration of the thrombosis, this vessel is not available in every
prostheses into the surrounding tissue. patient. Furthermore, it belongs to the venous and
In 1954 another new material—«nylon»—was thus low pressure part of the cardiovascular sys-
discovered (Shumacker and King 1954), and tem, predisposing ectatic and degenerative defor-
Edwards and Tapp constructed a new prosthesis mations when exposed to arterial blood pressure
out of this material (Edwards and Tapp 1955). load. Other autologous venous grafts, e.g. the
However, in 1958 Harris observed that 100 days femoral vein or those obtained from the upper
following implantation into the aorta, these pros- extremity, exhibit the same structural disadvan-
theses lost their structural stability, whereas tages and are reported to be even less qualified
Teflon and Dacron did not. Thus, the era of nylon than the greater saphenous vein. Based on these
was terminated before it really began. In the 1970s limitations, the search for tissue engineered grafts
another new material became available. It was a has intensified, especially for prostheses in the
8 variant of Teflon, named expanded polytetrafluo- venous system and for small-diameter arterial
roethylene (ePTFE). The microstructure of this grafts.
material initially discovered by Eiseman is char-
acterized by microscopic small knots and trans-
versal fibers running between them. Because of 8.3.3 eneration of Vascular Grafts
G
the resulting huge internodal spaces, up to 80 % Based on Hybrid Matrix
of the prosthesial wall are simply made of noth- Materials
ing. The practical meaning of this characteristic is
that these prostheses are primarily blood tight The basic idea for the generation of hybrid con-
and thus do no not require pre-clotting prior to structs combining biological and synthetic matrix
implantation. materials was to profit from positive attributes of
Deduced from further studies aiming to both these material groups. An early approach
increase the impermeability of vascular aiming to realize such a concept was the subcuta-
prostheses, collagen, albumin and gelatin impreg- neous implantation of polyethylene, polyvinyl or
nations were discovered as useful tools. silastic mandrains covered by a polyester net. The
Importantly, it was observed that these modifica- ratio behind this concept was that fibroblasts may
tions were not associated with loss of biomechan- spontaneously seed on these alloplastic nets to de
ical stability or other characteristics relevant for novo synthesize a tissue sheet within a few weeks
clinical application. Further refinements com- following the concept of guided tissue regenera-
prise the binding of various effectors, e.g. fibrino- tion. Indeed, Sparks and co-workers demonstrated
lytic or antibiotic substances, anticoagulants or that it was possible to generate such a tubular
other effectors to minimize damaging influences structure but also reported on degenerative
and to optimize bio- and hemocompatibility of changes in the sense of aneurysm formation and
these prostheses (Sagnella et al. 2003, 2005; resulting graft loss in the long term (Sparks 1973).
Murugesan et al. 2002; Lachapelle et al. 1994; More and above, the application of alloplastic
Ginalska et al. 2005a, b), a concept today termed vascular prostheses with an inner diameter
biofunctionalization. smaller than 5–6 mm was and still is often associ-
In the clinical setting, alloplastic prostheses, ated with early thrombotic occlusion and graft
i.e. those made out of Dacron and ePTFE, are failure. Thus, one of the most important clinical
primary used for reconstructive interventions of issues in cardiovascular surgery was and is to
large calibre vessels such as the aorta and its side identify materials, which allow for the generation
branches. The restriction of these implants to of vascular grafts ≤5 mm. In early experiments it
mainly large vessel areas (>6 mm) is explained by was tried to reduce the intrinsic thrombogeneity
the clinical observation that autologous vessel of alloplastic grafts by seeding autologous endo-
grafts such as the greater saphenous vein still thelial cells on their inner surface (Williams
Chapter 8 · Cardiovascular Tissue Engineering
181 8
1995). Miwa and Matsuda developed a prosthesis obtained from bovine aortas and cultivated under
on polyurethane basis with an artificial lamina pulsatile culture conditions in vitro. After 8 weeks,
basalis composed of collagen type-I and derma- endothelial cells were added at the luminal side
tan sulfate on which an endothelial monolayer and in the subsequent histological analysis up to
was transferred. Following in vitro culture, they 50 % of all extracellular matrix components were
implanted this construct in dogs and observed a identified as elastin and collagen proteins.
primary patency rate of 75 % without additional Furthermore, scanning electron-microscopic
anticoagulative therapy (Miwa and Matsuda analyses revealed homogeneous cellular mono-
1994). layers on the luminal surface, which stained posi-
Deutsch reported on a study in which endo- tive for CD31 (PECAM-1). In animal models,
thelial cells seeded ePTFE prostheses were these prostheses were patent for up to 8 weeks and
implanted as above the knee bypasses. He found a withstood a blood pressure load of more than
9-year patency rate of 65 % and thus comparable 2,000 mmHg. Presently, many working groups try
results to those obtained with greater saphenous to identify, synthesize and characterize further
vein bypass grafts. In contrast, non-seeded pros- biodegradable materials, which exhibit optimized
theses revealed only a patency rate of 16 % properties for the realization of such hybrid con-
(Deutsch et al. 1999). However, these good cepts (Nerem 1992).
patency rates of seeded grafts could not be If other prospective approaches, e.g. gene
achieved when used for coronary revasculariza- therapies aiming to modulate crucial tissue char-
tion (Laube et al. 2000). Thus, it was hypothesized acteristics, i.e. intrinsic thrombogeneity or fur-
that an increased intrinsic thrombogeneity might ther modified surgical methods and techniques
be responsible for this phenomenon. As a conse- will help to increase patency rates is uncertain
quence, polyurethane matrices seeded with endo- (Kuo et al. 1998; Hubbell et al. 1991). However, a
thelial cells were coincubated with heparin and few positive effects could already be observed
RGD groups to further increase endothelial cell (Flinn et al. 1984; Tyrrell and Wolfe 1991; Taylor
adherence. Indeed, a 75 % increase in endothelial et al. 1992; Siegman 1979; Miller et al. 1984;
surface coverage was observed. Linton and Darling 1962; DeLaurentis and
Another, potentially disadvantageous issue Friedmann 1972).
with regard to in vivo graft performance might be
the use of non-reabsorbable matrix materials for
that they may prevent remodeling and regenera- 8.4 Cardiac Tissue Engineering
tive processes (Seifalian et al. 2002). Alternative
may be reabsorbable materials, which degenerate 8.4.1 Heart Valves
over time and in parallel to the bioartificial in vivo
generation of a living graft. However, one essen- Today heart valve replacement therapy comprises
tial prerequisite in this regard is that the initially mechanical and bioprosthetic valves. Similar to
implanted biodegradable material has to be bio- small-calibre alloplastic vascular prostheses,
mechanically stable enough to withstand physio- mechanical valves incur high rates of thrombosis
logical hemodynamic stress load. Also, the and thromboembolic complications. Oral antico-
bioartificially generated tissue has to develop in agulants, however, are associated with an
parallel to material degradation with subsequent increased risk for bleeding complications, but
biomechanical properties similar to those of a they significantly reduce the risk for thromboem-
native vessel. bolic complications (Cannegieter et al. 1994). The
Over the last years, many polymers (degrad- valve itself is prone to an elevated risk of infec-
able and non-degradable) were evaluated, but tious complications but nevertheless is expected
most of them were found to be highly thrombo- to last a whole patient’s life. Bioprosthetic valves
genic, induced foreign body reactions, led to the fabricated from decellularized and preserved
formation of aneurysms and/or were reabsorbed xenogeneic valves or pericardial tissues are rela-
too fast. Niklason, therefore, tried to combine tively non-thrombogenic, and bleeding complica-
biodegradable polymers with living cells tions are rare due to the lack of lifelong
(Niklason et al. 1999). Polyglycolic acid (PGA) anticoagulation. However, these valves tend to
matrices were seeded with smooth muscle cells degenerate over time and have no or only limited
182 M.H. Wilhelmi and A. Haverich
remodeling capacities. Nonetheless, nowadays the pulmonary position of dogs, and, following
these valves last for up to 20 years (Rahimtoola 1 month, no hints to inflammatory reactions or
2003; Marchand et al. 2001). Taken together, the other immunological side effects could be
need for the generation of bioartificial heart valves observed—at least macroscopically.
bases on the following arguments: (i) ability to Other groups who used similar in vitro
remodel and grow (application in infants), (ii) decellularization protocols reported on compa-
reduced immunogenicity and thus reduced tissue rable results, so that the first commercially
deterioration/degradation (lifelong durability) available decellularized and cryopreserved
and (iii) avoidance of (oral) anticoagulants (i.e. in xenogeneic valvular prostheses came up. It
young, physical active and old, disabled patients). could be shown that decellularized valves
Finally, tissue-engineered heart valves may exhibit exhibit reduced immunogenicity in comparison
anti-infectious properties, if complete inner and to native control groups. However, it was warned
outer cellular coverage could be achieved. that even the application of these valves may
Historically, Ross and Barret-Boyes were the lead to accelerated destruction, especially when
first who used biological, allogeneic human heart used in infants (Simon et al. 2003). The pre-
valve prostheses in the clinical setting. Their pri- sumptive reason for this phenomenon was an
mary thought was to identify an alternative to elevated activity of the infant immune system in
8 mechanical prostheses, i.e. because of the lack of combination with a physiologically increased
distracting «click» noises and oral anticoagulation calcium metabolism at this age.
following implantation (Barratt-Boyes 1965; Ross As a logical consequence of the concept of
1967). However, initially, progressive degenera- exclusive decellularization, an additional step of
tive changes occurred 8–10 years following in vitro autologous endothelial re-seeding was
implantation, which finally led to complete added, and following promising initial data in
destruction and thus often necessitated redo animal models, results in human patients are
operations. The underlying mechanisms were available already (xenogeneic heart valves re-
thought to base especially on two major factors: seeded with autologous human endothelial cells)
(i) immunological reactions in the sense of sub- (Dohmen et al. 2002a, b). Another approach of
liminal tissue rejection (Wilhelmi et al. 2003a, b), autologous endothelial re-seeding of decellular-
which seem to be induced by the antigeneity of ized heart valve prostheses could be realized in
resident allogeneic cells, and (ii) the method of Hannover in close collaboration with the univer-
tissue preservation/fixation by glutaraldehyde. sity of Chisinau, Republic of Moldavia. Assuming
Initially, this latter agent was used to reduce the that decellularized, xenogeneic matrix scaffolds
immunogenicity of tissues via cross-link of colla- may still induce immunological reactions due to
gen fibers to prolong its durability. With time, interspecies differences, human allografts decel-
however, it became clear that actually the risk for lularized by using an elaborated protocol were re-
calcification increases, immunological reactions seeded by autologous endothelial cells obtained
are amplified and in vivo regeneration is ham- as mononuclear cells isolated from individual
pered by this agent (O’Brien et al. 1999). blood probes. Positive stains for the von
Trying to avoid the antigenic influence of resi- Willebrand factor, CD31 (PECAM-1) and flk-1 as
dent cells, Gulbins seeded cryopreserved human observed in monolayers of cells cultivated and
allografts with autologous endothelial cells differentiated on the luminal surface of the scaf-
in vitro and implanted these prostheses in ani- folds in a dynamic bioreactor system indicated
mals. However, the prostheses were not decellu- the endothelial nature of these cells. The re-
larized prior to the in vitro seeding, and thus the seeded valves were implanted in pulmonary posi-
success was only moderate (Gulbins et al. 2003). tion of two pediatric patients (age 13 and 11 years)
The group of Wilson picked up the basic idea to with congenital pulmonary valve failure.
use allogeneic cardiac valves but established a Postoperatively, a mild pulmonary regurgitation
multistep decellularization protocol, which based was documented in both children.
on the use of hypo- and hypertonic solutions, Based on regular echocardiographic investiga-
detergents and enzymes to remove all cellular tions, hemodynamic parameters and cardiac
components within the allogeneic valvular tissue morphology changed in 3.5 years as follows:
(Wilson et al. 1995). The valves were implanted in increase of the PV anulus diameter (18–22.5 mm
Chapter 8 · Cardiovascular Tissue Engineering
183 8
and 22–26 mm, respectively), decrease of valve valves showed improved freedom from explanta-
regurgitation (trivial/mild and trivial, respec- tion, provided low gradients in follow-up and
tively), one decrease (16–9 mmHg) and one exhibited adaptive growth (Cebotari et al. 2011)
increase (8–9,5 mmHg) of the mean transvalvular (. Fig. 8.4). This concept has now been evaluated
gradient, one remaining (26 mm) and one for experimental aortic valve replacement and is
decreasing (32–28 mm) right ventricular end- currently introduced in selected clinical cases
diastolic diameter. The body surface area (Baraki et al. 2009).
increased (1.07–1.42 m2 and 1.07–1.46 m2, An alternative approach basing on alloplastic
respectively), and no signs of valve degeneration matrix materials was followed especially by Mayer
were observed in both patients now 10 years after and Vacanti. They used synthetic matrix scaffolds
the procedure. Thus, it could be shown that the on polymer basis—polyglycolic acid (PGA) and
tissue engineering of heart valves using autolo- polylactide (PLA), respectively (Hoerstrup et al.
gous endothelial progenitor cells is a feasible and 1998). Following re-seeding of such matrices with
safe method at least for pulmonary valve replace- autologous arterial vessel wall-specific cells, pul-
ment. Furthermore, it was concluded that tissue monary valves and pulmonary artery segments
engineered valves have the potential to remodel were generated and tested in a sheep model
and grow according to the somatic growth of a (Shum-Tim et al. 1999; Shinoka et al. 1996, 1998).
child (Cebotari et al. 2002, 2006). However, the initially experienced disadvantage
Another interesting approach of this group of very high rigidity of polymer scaffolds led to
was the use of fresh decellularized pulmonary the development of new polymers and copoly-
homografts (DPH) for pulmonary valve replace- mers, e.g. polyhydroxyalkanoate (PHA), poly-4-
ment. Thirty-eight patients with DPH in pulmo- hydroxybutyrate (P4HB) and polyglycolic acid
nary position were consecutively evaluated during (PGA) (Sodian et al. 2000; Korecky et al. 1982;
the follow-up (up to 5 years) including medical Hoerstrup et al. 2000). In contrast to the above-
examination, echocardiography and MRI. Patients mentioned studies on DPH, till now none of these
were matched according to age and pathology and scaffold materials reached the clinical stage of
compared with glutaraldehyde-fixed bovine jugu- development.
lar vein (BJV) (n = 38) and cryopreserved homo-
graft (CH) (n = 38) recipients. In contrast to BJV
and CH groups, echocardiography revealed no
increase of transvalvular gradient, cusp thicken-
ing or aneurysmatic dilatation in DPH patients.
Over time, DPH valve anulus diameters converge
towards normal z-values. Five-year freedom from
explantation was 100 % for DPH and 86 ± 8 % and
88 ± 7 % for BJV and CH conduits, respectively.
Additionally, MRI investigations in 17 DPH
patients with follow-up time >2 years were com-
pared with MRI data of 20 BJV recipients. Both
patient groups (DPH and BJV) were at compara-
ble ages (mean, 12.7 ± 6.1 versus 13.0 ± 3.0 years)
and have comparable follow-up time (3.7 ± 1.0
versus 2.7 ± 0.9 years). In DPH patients, the mean
transvalvular gradient was significantly (P < 0.001)
lower (11 mmHg) compared with the BJV group
(23.2 mmHg). Regurgitation fraction was 14 ± 3 %
and 4 ± 5 % in DPH and BJV groups, respectively.
In three DPH recipients, moderate regurgitation
was documented after surgery and remained ..Fig. 8.4 Cardiac allograft valve. Decellularized pulmo-
nary homograft valve (DPH) (Courtesy of Prof. A. Haverich
unchanged in follow-up. and Dr. S. Cebotari, Dept. of Cardiothoracic, Transplanta-
Thus, in contrast to conventional homografts tion, and Vascular Surgery, Hannover Medical School, Han-
and xenografts, decellularized fresh allograft nover, Germany)
184 M.H. Wilhelmi and A. Haverich
8.4.2 Myocardial Tissue muscular cell types as well. The resulting data
revealed that a 3D matrix architecture led to
According to its typical three-layered architec- improved cellular differentiation of all kinds of
ture, the heart represents the largest «vascular» evaluated cells and tissue generation.
structure of the body. Thus, at the first glance, it Beside cellular composition and differentia-
may seem to be very simple to bioartificially tion, living tissues and, i.e. functional myocar-
construct a heart. Actually, the mimicry of this dium are characterized by the orientation of its
organ or even parts thereof still faces the scien- resident cells. Here, two factors seem to play a
tific world with one of the most challenging crucial role for the heart (i) mechanical stimuli
tasks. The first, probably accidental event to and (ii) the geometric orientation of matrix com-
develop a three-dimensional myocardial tissue ponents. Regarding the first point, electronically
dates back to the early 1950s, when Moscana and controlled bioreactors were developed, which
co-workers isolated embryonic chicken cardiac provide, e.g. stretching forces to muscle cell cul-
muscle cells and cultivated them under continu- tures originating from the musculature and the
ous rotation in an in vitro setting. After 18 h in heart. Using these devices, it could be shown that
culture, the cells spontaneously formed spher- mechanical stimuli exhibit positive effects on dif-
oid, three-dimensional and approximately 200 ferentiation and orientation of both types of mus-
8 cells comprising aggregates, which most inter- cular cells (Terracio et al. 1988; Chambard et al.
estingly exhibited spontaneous contractile activ- 1981). Under conditions of continuous cyclic
ity (Moscona 1959). Many other groups picked stress consisting of distension and relaxation,
up this promising model and observed that these skeletal muscle cell cultures formed longitudi-
cellular aggregates resembled functional native nally orientated 3D muscle fibers, which began to
cardiac tissue much more than any other two- form tendons as well (Vandenburgh et al. 1991).
dimensional cellular monolayer before In another concept, collagen sponges were
(McDonald et al. 1972). Thus, this early observa- combined with a suspension of newborn rat car-
tion demonstrated that embryonic cardiac cells diomyocytes in Matrigel and stimulated electri-
grow even under in vitro culture conditions and cally (Radisic et al. 2004). This concept led to the
tend to form spontaneous cellular aggregates. formation of cardiac muscle structures with
However, following longer times of in vitro cul- enhanced tissue morphology, contractile function
ture and ongoing cell proliferation, it could be and specific molecular marker expression. Thus,
observed that: (i) the spontaneous contraction electrical stimuli seem to have similar effects as
ceased, (ii) the whole tissue contracted and (iii) mechanical ones (Fink et al. 2000; Zimmermann
the cellular sheets loosened and started to float et al. 2002). Furthermore, it could be observed
in the culture medium. that other parameters, e.g. strong magnetic fields,
For a long time, insufficient culture conditions influence the orientation of fibroblasts and smooth
were accused to cause this phenomenon and many muscle cells as well (Guido and Tranquillo 1993;
groups skipped to follow this experiment. However, Torbet and Ronzière 1984; Tranquillo et al. 1996).
Shimizu and co-workers regarded this phenome- Regarding the second influencing factor—the
non as a chance to harvest and staple the floating geometric orientation of matrix components—it
monolayers and thus to generate a 3D-tissue struc- was observed that the matrix scaffold itself has
ture without the need for an additional matrix scaf- essential influence on tissue growth and cellular
fold (Shimizu et al. 2002). According to the differentiation. One important example, which
observation that free floating and thus mechanical underscores this observation, was that cardiomy-
nonloaded myocardial monolayers cease their con- ocytes obtained from newborn rats and seeded on
tractile activity over time, Vandenburgh and co- a rill-shaped collagen type-1 matrix grew along
workers covered cultured muscle cells with type-1 these structures (Simpson et al. 1994).
collagen to further mechanically load and stimu- Perhaps one of the most striking examples
late these cells (Vandenburgh et al. 1988). And demonstrating the influence and importance of
indeed, they observed that this method led to extracellular matrix components for the survival
improved differentiation of myocytes so that this of injected cells was a study in which a whole
basic principle was transferable to other, non- rat heart was decellularized and subsequently
Chapter 8 · Cardiovascular Tissue Engineering
185 8
repopulated with neonatal cardiomyocytes, (Matrigel) (Zimmermann et al. 2000; Souren et al.
resulting in a beating heart (Ott et al. 2008). 1992). However, the resulting contractile forces
However, and as already mentioned above, the could be tripled by applying cyclic distension
optimal matrix scaffold for the bioartificial gen- forces (Fink et al. 2000).
eration of cardiac tissue is still unknown. The Two other, totally different approaches based
reasons for this fact are manifold and may be on cell-matrix combinations consisting of polyg-
characterized as follows: lycolic acid (PGA) or a liquid gelatin matrix and
1. The necessity of high mechanical stability cells, e.g. fetal rat cardiomyocytes (Langer and
with coexistent high compliance Vacanti 1993; Li et al. 1999; Carrier et al. 1999).
2. The helical and interweaved fiber structure of Both these cell matrix composites exhibited
the cardiac muscle, which are mandatory for spontaneous contractile activities—in vitro and
optimal pump function in vivo. However, following implantation in
3. Adequate tissue niches to host/retain tissue- infracted myocardial tissue areas, only a very few
specific cells, (iv) the guarantee of a sufficient of these cardiomyocytes could be detected histo-
supply of these key cells with oxygen and logically. In contrast, following implantation of
nutrients alginate-based matrices seeded with fetal cardiac
4. The ability to completely integrate into the cells, Leor observed distinctive vascularization
surrounding myocardial tissue within these constructs. However, a real integra-
tion into the surrounding recipient’s myocar-
Today it is not clear, if it ever will be techni- dium could not be observed either (Leor et al.
cally possible to generate materials, which com- 2000).
pletely mimic the complex structure of a living Apart from all matrix-based techniques men-
heart. Even if it should be possible, e.g. on basis of tioned above, Shimizu and co-workers presented
modern nanotechnologies, it has to be guaran- a complete matrix-free approach. They used a cul-
teed that quantitatively enough nishes are present ture flask coated by a thermosensitive surface on
within those matrices, so that specific cells can be which cells of all kinds can be grown. However, in
nested within the scaffolds, connect with each contrast to other known culture flasks, this spe-
other and build up a fully functioning electrical cific setting allows cells to detach in reaction to
and mechanical syncytium. Whether strategies changes in temperature (Shimizu et al. 2002).
developed in Taylor’s laboratory to decellularize Thus, confluent monolayers of cardiac myocytes
and reseed an entire organ will proof successful can be grown and subsequently piled up to
has to be awaited (Badylak et al. 2011). At least, a 3D-connected cellular layers (up to 50–75 μm
matrix closest to native would be provided. thickness). The advantages of this technique are
Another approach to bioartificially generate the simplicity of the method and the indepen-
myocardial tissue, resulted from an in vitro model dence from the use of potentially immunogenic,
initially developed to evaluate embryonic fibro- pathogenic or even toxic matrix scaffolds. The
blasts (Kolodney and Elson 1993). Here, cardio- disadvantages comprise limitations with regard to
myocytes were cultured in a collagen-gel matrix geometric shapes, fragility and thus only limited
(Chambard et al. 1981; Hall et al. 1982). The par- mechanical load capacity. Meanwhile, various
ticular characteristic of an experimental setting variations of this method have been described
established by Eschenhagen and co-workers was (Kofidis et al. 2002; Akins et al. 1999).
that the cell containing matrices were positioned
between two rectangular positioned Velcro cov- 8.4.2.1 Vascularization and Cellular
ered glass rods. Spontaneous muscle activities and Composition
forces generated by the resulting cellular aggre- Over the last decade, various methods and tech-
gates adhering to these glass rods thus could be niques to bioartificially generate cardiovascular
identified and quantified (Eschenhagen et al. tissues have been described. However, as finds
1997). Cardiomyocytes obtained from newborn true for almost every other kind of 3D-tissue
rats and embedded in collagen type-1 gel exhib- structure as well, one of the most essential and
ited no growth and differentiation until the addi- still unsolved problems is the limitation of the
tion of further extracellular matrix components maximal achievable tissue thickness, which bases
186 M.H. Wilhelmi and A. Haverich
on limited diffusion capacities for nutrients and implantation seemed to be of only minor impor-
oxygen. Studies evaluating the process of angio- tance with regard to tissue fate, some essential
genesis in tumors have shown that in the absence questions could not be answered: (i) is the pre-
of capillary vessels and perfusion, the thickness of existing vascularization sufficient for long-term
living tissues is restricted to a maximum of survival, (ii) how many of the initially implanted
2–4 mm (Folkman 1971). One crucial parameter cardiomyocytes survive over time, (iii) do these
in this regard is the individual and tissue-specific implanted cells functionally and structurally
metabolic activity and the local concentration of integrate into the recipient myocardium and (iv)
cells, respectively. Contractile cardiac tissue con- does the implantation of those constructs really
tains a very number and high density of cellular lead to an augmented cardiac function? In con-
components and thus exhibit a very high meta- text of some in vitro studies, such as Langendorff
bolic activity. Depending on the stage of develop- models, it was observed that the cardiac function
ment, a human heart contains between 2,400 and actually improved, higher pressure values were
3,300 capillaries per mm2 area (Rakusan et al. obtained (Li et al. 1999) and the fractional short-
1992). ening was increased (Leor et al. 2000). However,
On the other side, it is known that embryonic these effects, overall, were not very pronounced
rat hearts and adult frog hearts are completely and could be obtained in a similar way by the
8 avascular and are fed only by diffusion. The rea- injection of different and, i.e. non-cardiomyo-
son for this phenomenon lays in the structure of cytic cells. In direct comparison of cellular sus-
these tissues, which are characterized by a wide pensions and 3D bioartificially generated tissue
spread trabecular system composed of muscular grafts, the advantage of latter is not clear at pres-
struts (Sys et al. 1997; Ratajska et al. 2003). ent.
Regarding strategies for the bioartificial genera- Another conceptional issue regarding the bio-
tion of tissues, this means that a physiological artificial generation of cardiac tissue refers to spe-
vascularization or an intensive trabecular sys- cific cellular components and sources to isolate or
tems with fibers not thicker than 50–75 μm has generate such cells, respectively. It is estimated
to be present. Some working groups tried to that an adult human heart comprises 5 × 109 car-
increase the transport capacity for oxygen and diomyocytes within the left ventricle alone
other nutrients by using specialized bioreactor (Beltrami et al. 1994). This means that approxi-
systems or oxygen carriers, e.g. peroxy fluoro- mately 40 million cardiomyocytes are present
carbon (Radisic et al. 2005; Carrier et al. 2002). within 1 g of cardiac tissue or in other words that
Actually it was possible to induce a positive it is nearly impossible to harvest a sufficient cell
effect on the cellular density as well as metabolic count to generate a complete bioartificial heart,
activity (Carrier et al. 2002); subsequently car- e.g. via cardiac biopsies. Scientific approaches
diac tissue with a clinical relevant tissue thick- with focus on adult stem cell research might rep-
ness of up to 500 μm could be generated (Radisic resent a possible solution for this problem. Today
et al. 2005). it is known that all cells necessary to generate
Other strategies based on data observed at autologous cardiac tissues can be differentiated
newborn rat hearts. Here it was tried to generate from (adult) stem cells, which can be harvested
single tissue struts, which than were woven to from, e.g. bone marrow aspirates (Makino et al.
each other so that the resulting network was 1999; Orlic et al. 2001), peripheral blood probes
completely perfused and allowed for diffusion (Asahara et al. 1997; Badorff et al. 2003), cord
through the whole tissue structure. Alternatively blood (Condorelli et al. 2001) or fatty tissue (Zuk
it was tried to integrate bioartificially generated et al. 2001).
cardiac muscle constructs into native vascular- Another potentially interesting alternative to
ized tissues (with or without additional pharma- generate higher quantities of suitable cells might
cological stimulation of angiogenic processes). be the induction of pluripotent stem cells (iPS
Although it was observed that all these artificially cells) from somatic cells of an individual patient.
generated heart muscle tissues exhibited a fast Currently, the most common method of generat-
(re-)vascularization following implantation and ing cardiomyocytes from iPS cells is the embryoid
that hypoxic periods during and early after body differentiation system which coaxes the iPS
Chapter 8 · Cardiovascular Tissue Engineering
187 8
cells to differentiate into the cardiac lineage 55 The development of dynamically (self-)adapt-
(Narsinh et al. 2011). However, although the ing systems (intelligent bioreactors) for the
resulting cells show many functional properties of (automated) in vitro generation of cardiovas-
normal human cardiomyocytes, some other cular tissues
aspects of these cells, including their morphology, 55 The identification and establishment of
remain relatively immature and «fetal-like». More micro- and nanotechnologies, which facilitate
and above, these pluripotent cells still run the risk tissue/matrix (neo-)angiogenesis and thus
to induce teratomas (Oh et al. 2012). Thus, all of allow for the generation of complex 3D-tissue
the above-mentioned techniques and cellular structures
sources have to be investigated intensely in the 55 The identification of suitable autologous cell
future. sources and techniques for the isolation
(adult stem cells) or generation (iPS cells) of
high quantities of suitable key cells for matrix
8.5 Outlook (re-)vitalization and functionalization
55 The evaluation of residual immunological dif-
Shortcomings of all known alloplastic implant ferences between donor tissue and the recipi-
materials, namely, infectious and thromboem- ent immune system
bolic complications, blazed a trail for biological 55 The development of strategies to avoid or
and bioartificial tissue substitutes in the cardio- minimize the immunological consequences
vascular system. However, although a huge vari- 55 The identification, formulation and imple-
ety of methods and techniques have been mentation of standard operating procedures
established to generate and evaluate bioartificial (SOP) for the bioartificial generation of car-
cardiovascular grafts, only two of these have diovascular grafts
found their way into clinical practice (. Table 8.3), 55 Formulation of nationally and internationally
i.e. the following issues still have to be addressed accepted uniform requirements for product
and further evaluated: licencing procedures
Acellular and re-seeded vessel grafts of human Clinical case reports (Zehr et al. 2005)
origin
Cardiac valve Seeding of biodegradable synthetic matrices Animal experiments (Teebken et al.
replacement with different kinds of cells 2005)
therapy: with
synthetic matrices
Valves (homografts)
Re-seeded human heart valves (homografts) Clinical study (Cebotari et al. 2011)
Constructs made out of cell sheets Animal studies (Shimizu et al. 2006)
Decellularized vein as a potential scaffold for vascular Sparks CH (1973) Silicone mandril method for growing
tissue engineering. J Vasc Surg 40:146–153 reinforced autogenous femoro-popliteal artery grafts
Schmidt CE, Baier JM (2000) Acellular vascular tissues: nat- in situ. Ann Surg 177:293–300
ural biomaterials for tissue repair and tissue engineer- Steinhoff G, Stock U, Karim N, Mertsching H, Timke A,
ing. Biomaterials 21:2215–2231 Meliss RR, Pethig K, Haverich A, Bader A (2000) Tissue
Schmidt D, Dijkman PE, Driessen-Mol A, Stenger R, Mariani C, engineering of pulmonary heart valves on allogenic
Puolakka A, Rissanen M, Deichmann T, Odermatt B et al. acellular matrix conduits: in vivo restoration of valve
(2010). Minimally-invasive implantation of living tissue tissue. Circulation 102:III50–III55
engineered heart valves: A comprehensive approach fro- Sys SU, Pellegrino D, Mazza R, Gattuso A, Andries LJ, Tota L
mautologousvascular cells tostem cells 56(6):510–520 (1997) Endocardial endothelium in the avascular heart
Seifalian AM, Tiwari A, Hamilton G, Salacinski HJ (2002) of the frog: morphology and role of nitric oxide. J Exp
Improving the clinical patency of prosthetic vascular Biol 200:3109–3118
and coronary bypass grafts: the role of seeding and Takahashi K, Yamanaka S (2006) Induction of pluripotent
tissue engineering. Artif Organs 26:307–320 stem cells from mouse embryonic and adult fibroblast
Shimizu T, Yamato M, Isoi Y, Akutsu T, Setomaru T, Abe K, cultures by defined factors. Cell 126:663–676
Kikuchi A, Umezu M, Okano T (2002) Fabrication of Taylor RS, Loh A, McFarland RJ, Cox M, Chester JF (1992)
pulsatile cardiac tissue grafts using a novel Improved technique for polytetrafluoroethylene
3-dimensional cell sheet manipulation technique and bypass grafting: long-term results using anastomotic
temperature-responsive cell culture surfaces. Circ Res vein patches. Br J Surg 79:348–354
90:e40 Teebken OE, Haverich A (2002) Tissue engineering of small
8 Shimizu T, Sekine H, Isoi Y, Yamato M, Kikuchi A, Okano T
(2006) Long-term survival and growth of pulsatile
diameter vascular grafts. Eur J Vasc Endovasc Surg
23:475–485
myocardial tissue grafts engineered by the layering of Teebken OE, Wilhelmi M, Haverich A (2005) Tissue
cardiomyocyte sheets. Tissue Eng 12:499–507 Engineering für Herzklappen und Gefässe. Chirurg
Shinoka T, Ma PX, Shum-Tim D, Breuer CK, Cusick RA, Zund 76:453–466
G, Langer R, Vacanti JP, Mayer JE (1996) Tissue- Terracio L, Miller B, Borg TK (1988) Effects of cyclic mechan-
engineered heart valves. Autologous valve leaflet ical stimulation of the cellular components of the
replacement study in a lamb model. Circulation heart: in vitro. In Vitro Cell Dev Biol 24:53–58
94:II164–II168 Torbet J, Ronzière MC (1984) Magnetic alignment of colla-
Shinoka T, Shum-Tim D, Ma PX, Tanel RE, Isogai N, Langer R, gen during self-assembly. Biochem J 219:1057–1059
Vacanti JP, Mayer JE (1998) Creation of viable pulmo- Tranquillo RT, Girton TS, Bromberek BA, Triebes TG,
nary artery autografts through tissue engineering. Mooradian DL (1996) Magnetically orientated
J Thorac Cardiovasc Surg 115:536–545; discussion tissue-equivalent tubes: application to a circumferen-
545–6 tially orientated media-equivalent. Biomaterials
Shumacker HB, King H (1954) The use of pliable plastic 17:349–357
tubes as aortic substitutes in man. Surg Gynecol Tschoeke B, Flanagan TC, Cornelissen A, Koch S, Roehl A,
Obstet 99:287–294 Sriharwoko M, Sachweh JS, Gries T, Schmitz-Rode T,
Shum-Tim D, Stock U, Hrkach J, Shinoka T, Lien J, Moses Jockenhoevel S (2008) Development of a composite
MA, Stamp A, Taylor G, Moran AM et al. (1999) Tissue degradable/nondegradable tissue-engineered vascu-
engineering of autologous aorta using a new biode- lar graft. Artif Organs 32:800–809
gradable polymer. Ann Thorac Surg 68:2298–2304; Tschoeke B, Flanagan TC, Koch S, Harwoko MS, Deichmann
discussion 2305 T, Ellå V, Sachweh JS, Kellomåki M, Gries T et al. (2009)
Siegman FA (1979) Use of the venous cuff for graft anasto- Tissue-engineered small-caliber vascular graft based
mosis. Surg Gynecol Obstet 148:930 on a novel biodegradable composite fibrin-polylactide
Simon P, Kasimir MT, Seebacher G, Weigel G, Ullrich R, scaffold. Tissue Eng A 15:1909–1918
Salzer-Muhar U, Rieder E, Wolner E (2003) Early failure Tucker OP, Syburra T, Augstburger M, van Melle G, Gebhard
of the tissue engineered porcine heart valve S, Bosman F, von Segesser LK (2002) Small intestine
SYNERGRAFT in pediatric patients. Eur J Cardiothorac without mucosa as a growing vascular conduit: a por-
Surg 23:1002–1006; discussion 1006 cine experimental study. J Thorac Cardiovasc Surg
Simpson DG, Terracio L, Terracio M, Price RL, Turner DC, 124:1165–1175
Borg TK (1994) Modulation of cardiac myocyte pheno- Tudorache I, Kostin S, Meyer T, Teebken O, Bara C, Hilfiker A,
type in vitro by the composition and orientation of the Haverich A, Cebotari S (2009) Viable vascularized autol-
extracellular matrix. J Cell Physiol 161:89–105 ogous patch for transmural myocardial reconstruction.
Sodian R, Hoerstrup SP, Sperling JS, Daebritz S, Martin DP, Eur J Cardiothorac Surg 36:306–311; discussion 311
Moran AM, Kim BS, Schoen FJ, Vacanti JP, Mayer JE Tyrrell MR, Wolfe JH (1991) New prosthetic venous collar
(2000) Early in vivo experience with tissue-engineered anastomotic technique: combining the best of other
trileaflet heart valves. Circulation 102:III22–III29 procedures. Br J Surg 78:1016–1017
Souren JE, Schneijdenberg C, Verkleij AJ, Van Wijk R (1992) Vacanti JP (1988) Beyond transplantation. Third annual
Factors controlling the rhythmic contraction of colla- Samuel Jason Mixter lecture. Arch Surg 123:545–549
gen gels by neonatal heart cells. In Vitro Cell Dev Biol Vacanti JP, Langer R (1999) Tissue engineering: the design
28A:199–204 and fabrication of living replacement devices for
Chapter 8 · Cardiovascular Tissue Engineering
193 8
surgical reconstruction and transplantation. Lancet emia after implantation of xenogeneic pulmonary
354(Suppl 1):SI32–SI34 valve conduits: histological evaluation after 6 to 12
Vandenburgh HH, Karlisch P, Farr L (1988) Maintenance of months in sheep. Int J Artif Organs 26:411–420
highly contractile tissue-cultured avian skeletal myo- Williams SK (1995) Endothelial cell transplantation. Cell
tubes in collagen gel. In Vitro Cell Dev Biol 24:166–174 Transplant 4:401–410
Vandenburgh HH, Swasdison S, Karlisch P (1991) Wilson GJ, Courtman DW, Klement P, Lee JM, Yeger H
Computer-aided mechanogenesis of skeletal muscle (1995) Acellular matrix: a biomaterials approach for
organs from single cells in vitro. FASEB J 5:2860–2867 coronary artery bypass and heart valve replacement.
Vara DS, Salacinski HJ, Kannan RY, Bordenave L, Hamilton Ann Thorac Surg 60:S353–S358
G, Seifalian AM (2005) Cardiovascular tissue engineer- Wu SM, Hochedlinger K (2011) Harnessing the potential of
ing: state of the art. Pathol Biol (Paris) 53:599–612 induced pluripotent stem cells for regenerative medi-
Vogt PR, Stallmach T, Niederhäuser U, Schneider J, Zünd cine. Nat Cell Biol 13:497–505
G, Lachat M, Künzli A, Turina MI (1999) Explanted Zehr KJ, Yagubyan M, Connolly HM, Nelson SM, Schaff HV
cryopreserved allografts: a morphological and immu- (2005) Aortic root replacement with a novel decellu-
nohistochemical comparison between arterial larized cryopreserved aortic homograft: postoperative
allografts and allograft heart valves from infants and immunoreactivity and early results. J Thorac
adults. Eur J Cardiothorac Surg 15:639–644; discus- Cardiovasc Surg 130:1010–1015
sion 644–645 Zimmermann WH, Fink C, Kralisch D, Remmers U, Weil J,
Voorhees AB, Jaretzki A, Blakemore AH (1952) The use of Eschenhagen T (2000) Three-dimensional engineered
tubes constructed from vinyon “N” cloth in bridging heart tissue from neonatal rat cardiac myocytes.
arterial defects. Ann Surg 135:332–336 Biotechnol Bioeng 68:106–114
Weinberg CB, Bell E (1986) A blood vessel model con- Zimmermann WH, Schneiderbanger K, Schubert P, Didié
structed from collagen and cultured vascular cells. M, Münzel F, Heubach JF, Kostin S, Neuhuber WL,
Science 231:397–400 Eschenhagen T (2002) Tissue engineering of a differ-
Wilhelmi MH, Mertsching H, Wilhelmi M, Leyh R, Haverich entiated cardiac muscle construct. Circ Res
A (2003a) Role of inflammation in allogeneic and 90:223–230
xenogeneic heart valve degeneration: immunohisto- Zuk PA, Zhu M, Mizuno H, Huang J, Futrell JW, Katz AJ,
chemical evaluation of inflammatory endothelial cell Benhaim P, Lorenz HP, Hedrick MH (2001) Multilineage
activation. J Heart Valve Dis 12:520–526 cells from human adipose tissue: implications for cell-
Wilhelmi MH, Rebe P, Leyh R, Wilhelmi M, Haverich A, based therapies. Tissue Eng 7:211–228
Mertsching H (2003b) Role of inflammation and isch-
195 9
Cardiac Surgical
Intensive Care
Andreas Markewitz, Axel Franke, René H. Bombien,
and Ali Khoynezhad
9.3.2.3 Neurological Status tial in early diagnosis along with prompt diagnostic
Glasgow Coma Scale (GCS) is a useful tool in quan- tools (e.g., cranial computed tomography) to
tifying and monitoring the progress of patient’s potentially minimize neurological deficit.
neurological status (. Table 9.2). The Glasgow
Coma Scale provides a score in the range of 3–15; 9.3.2.4 Volume Status,
patients with scores of 3–8 are usually said to be in Centralization, and Body
a coma. The total score is the sum of the scores in Temperature
three categories. However, the GCS has only a lim- The basic principles of postoperative cardiac sur-
ited use in sedated and intubated patient. Since gical intensive care are hemodynamic monitoring
cerebral ischemia and intracerebral hemorrhage and the differentiated management of intravascu-
occur in 1–5 % of patients undergoing cardiac sur- lar volume and vasoactive substances, inotropic
gery (Markewitz and Lante 2006), it is important to support, heart rate, and rhythm management.
monitor the pupillary response, the position of the Therefore early on CSICU admission, the inten-
eyeballs, and the motor response of the extremities sivist has to estimate patient’s intravascular vol-
on CSICU admission and closely in the postopera- ume status and the degree of centralization or
tive course. Frequent neurological exams are essen- peripheral vasoconstriction. The volume status
can be estimated by evaluating core body temper-
ature, central venous pressure (correlated to «pos-
..Table 9.2 Glasgow Coma Scale and Score itive end-expiratory pressure» on the ventilator),
and the undulation of the arterial pressure wave-
9 Reaction Action Score form, the heart rate, and blood pressure.
If there are no signs of volume overload
Eye Spontaneous—open with 4
opening blinking at baseline (hypervolemia) at the time of CSICU admission
response (pulmonary edema, central congestion on the
Opens to verbal command, 3
thoracic X-ray, right heart failure, insufficiency of
speech, or shout
the tricuspid valve), volume substitution should
Opens to pain, not applied 2 be considered to reduce hypotension due to
to face rewarming and the concomitant loss of peripheral
None 1 resistance.
Verbal Oriented 5
response
9.3.2.5 Heart Rhythm and Heart Rate
Confused conversation, but 4 To achieve an adequate cardiac output, a heart rate
able to answer questions
of 60–120 bpm with a cardiac output of >2.0 l/
Inappropriate responses, 3 min/m2 body surface has to be established. Regular
words discernible atrial and ventricular function in sinus rhythm or
Incomprehensible speech 2 under pacemaker sensing/pacing is helpful in
maintaining adequate cardiac output. The frequent
None 1
episodes of bradycardia or tachycardia after car-
Best Obeys commands for 6 diac surgery require the continuous heart rate
motor movement monitoring. Patient’s abnormal rhythm should be
response
Purposeful movement to 5 correlated to invasive arterial blood pressure mon-
painful stimulus itoring and the pulse oximetric curve. In case of
Withdraws from pain 4
discrepancies, and when the electronic measure-
ment is not reliable, the pulse may be palpated.
Abnormal (spastic) flexion, 3
decorticate posture 9.3.2.6 Secure the Pacemaker
Extensor (rigid) response, 2 Function
decerebrate posture Depending on the operation and the occurrence
None 1 of preoperative or intraoperative arrhythmias, the
temporary pacemaker wires are attached to the
According to Teasdale and Jennett (1974), Jennett right atrium and the right ventricle and, in special
et al. (1977) cases, also to the left ventricle. If heart rhythm
Chapter 9 · Cardiac Surgical Intensive Care
201 9
disturbances occur in the postoperative course, 9.3.3.2 I nvasive Blood Pressure
the cardiac output will be reduced, and this may Monitoring
require external electrical stimulation. Therefore The postoperative measurement of the arterial
the function of the pacemaker wires has to be blood pressure is an essential part of CSICU mon-
tested and documented at the time of CSICU itoring. The acquisition of the arterial pressure can
admission: proper capture and sensing is critical! be carried out in two ways: a noninvasive method
using a blood pressure cuff or an invasive method
requiring cannulation of the radial or femoral
9.3.3 Basic Monitoring artery. The noninvasive measurement is error
and Diagnostics prone and inadequate for a safe blood pressure
monitoring. The invasive blood pressure measure-
To assess all relevant parameters in cardiac sur- ment allows close pressure monitoring as well as
gery patients, various monitoring systems have to assessment of the volume status of the patient due
be intertwined. to respiratory undulation of the pressure curve.
Basic monitoring of CSICU patients includes Furthermore during mechanical ventilation and
(Carl et al. 2010): under inotropic and vasopressor administration,
55 Continuous ECG and 12-lead ECG arterial blood gas may be drawn from the arterial
55 Invasive and noninvasive blood pressure line for easy blood sample and laboratory analysis.
measurement Possible errors occur if the pressure trans-
55 Measurement of central venous pressure ducer is not correctly zeroed or if any air bubbles
55 Pulse oximetry remain in the pressure lines. The air leads to a dis-
55 Input and outputs (chest tube drainage, volume tortion of the pressure curve due to damping. The
input, and urine output) mean arterial pressure (MAP) is utilized routinely
55 Body core temperature measurement in CSICU. It can be calculated after measuring the
systolic (APsyst) and diastolic (APdiast) blood
If the postoperative course will be compli- pressure using the following formula:
cated, additional monitoring will be required.
After CSICU admission and review of the basic MAP [ mm Hg ] = AP diast
monitoring tools, the diagnostic tools may be + 1 / 3 ( AP syst − AP diast )
supplemented by:
55 Mixed venous and arterial blood gas analysis
55 Chest X-ray, a - p (lying and in inspiration) 9.3.3.3 Measurement of Central
55 Routine laboratory diagnostics Venous Pressure
The central venous pressure (CVP) is invasively
9.3.3.1 ECG and 12-Lead ECG measured in the upper caval vein in about 1–2 cm
To get a basic monitoring for arrhythmia and isch- distance the right atrium. CVP correlates—in
emia, continuous ECG monitoring is required on absence of an insufficiency of the tricuspid valve—
CSICU. In lead II the electrical axis is parallel to with the end-diastolic pressure in the right ven-
the sinus node and the AV node. Usually the P tricle. The CVP value depends on the intravascular
wave is clearly detectable in lead II, so supraven- volume, the peripheral vascular resistance, the
tricular and ventricular rhythm disturbances can right ventricular ejection fraction/compliance,
be differentiated. Lead V5 should be added on the the pulmonary artery resistance, and the intratho-
monitor in patients with coronary artery disease racic pressure (PEEP ventilation/intrinsic PEEP).
to recognize any ischemia at the anterior and lat- The CVP is low in relative volume-deficient
eral wall. An ST-segment analysis is recommended patients and elevated due to volume overload, right
for each ECG monitoring. In cardiac surgical heart failure, pulmonary embolism, pericardial tam-
patients, a 12-lead ECG should be performed rou- ponade, tension pneumothorax, and in ventilated
tinely for any patient at CSICU admission and patient with high PEEP (CVP real = CVP–PEEP).
daily on the first three postoperative days. If The diagnostic value of the CVP is limited due
patients stay longer on CSICU, indication for addi- to the high volume compliance of the venous
tional ECG depends on clinical status, especially if system. However, if followed as a trend, the CVP
any ST-changes occur as seen on the monitor. provides useful information about the volume
202 A. Markewitz et al.
status as well as the right ventricular preload and 9.3.3.6 Chest Tube Output
compliance. It is helpful to know the CVP value at On admission, the filling state of the pleura vacs
the time of weaning from extracorporeal bypass has to be documented. The chest tube output is an
and correlating cardiac function with correspond- important indicator for postoperative bleeding in
ing right ventricular preload help assist as guide- cardiac surgery. However, the chest tubes have to
line for postoperative volume management. be frequently checked by active manipulation
(gentle «milking» motion) for proper drainage.
9.3.3.4 Pulse Oximetry Breathing and pulse synchronous movements of
The percutaneous spectrophotometric determi- the liquid level are also an indication for patency.
nation of oxygen saturation is a very useful and In case of a pulmonary parenchymal injury, there
noninvasive and continuous approach to measure will be breath-dependent air bubble leaking from
the peripheral arterial oxygen saturation (SaO2). the first fluid chamber.
It is displayed as a pulse synchronous undulating The total chest tube output should ideally be
curve. The SaO2 is defined as percent of oxygen- less than 100 ml per hour. If the drainage is more
ated hemoglobin denominated by the sum of oxy- than 100 ml per hour, it may be necessary to check
genated and deoxygenated hemoglobin. It can be one or more of these blood coagulation parameters:
compared in its diagnostic relevance to the partial 55 Activated clotting time
pressure of oxygen (paO2). 55 Prothrombin time (PT)
The respiratory monitoring allows the assess- 55 Partial thromboplastin time (PTT)
ment of pulmonary oxygen uptake and allows, by 55 Level of fibrinogen
9 knowing the actual hemoglobin concentration, 55 Platelet count
the assessment of the arterial oxygen supply of the 55 Functional coagulation tests such as throm-
tissue. Additionally, the acquisition of the pulse boelastogram
curve shows the mechanical heart function.
Pulse oximetry cannot discriminate among If the patient is stable without evidence of
oxygenated hemoglobin, carboxyhemoglobin, pericardial tamponade, the abnormal coagulation
and methemoglobin. In these cases, the measured values should be normalized first. If a high chest
SaO2 concentration is actually lower than dis- tube is combined with a significant hemoglobin
played SaO2 given concomitant dyshemoglo- drop or a new pericardial effusion leading to atrial
binemia in the bloodstream. or ventricular collapse and circulatory instability,
Estimated oxygen saturations (SaO2) at the patient should be taken back for mediastinal
40 mmHg carbon dioxide, at a pH 7.4, and physio- exploration and control of hemorrhage (see also
logical body temperature are depicted in . Table 9.3. 7 Section 9.5.3 «Bleeding Complications and Pericar-
dial Tamponade»).
9.3.3.5 Fluid Balance
The postoperative fluid balance is close monitoring 9.3.3.7 Measurement of the Body
of fluid input and output (I&O) including urine out- Temperature
put and chest tube and nasogastric drainage. In the The body temperature should be measured con-
first 24 h after operation, the hourly documentation tinuously. Among others, it affects the values of
of I&O may be useful. However, after the first postop- the blood gas analysis. Furthermore, hypothermia
erative day, the interval can be extended to every 4 h. can exacerbate bleeding tendencies in the early
Separate documentation of crystalloid versus colloid postoperative course. For this reason, the temper-
volume resuscitation in the postoperative phase has ature should be collected at least to every arterial
not been clinically useful and has fallen out of favor. blood gas sample taking. Typically, a four-hourly
interval is recommended. The temperature can be
..Table 9.3 Reference values for estimated measured by using a Foley catheter with inte-
oxygen partial pressure (paO2) based on measured grated temperature sensor or transesophageal or
oxygen saturation (SaO2) by pulse oximetry (see text) intravascular by the Swan-Ganz catheter or by
infrared (middle ear temperature) approach.
Parameter Values
The interpretation of body temperature is
PaO2[mmHg] 26 35 40 60 90 150 always intertwined with the clinical picture: while
in the postoperative rewarming phase and
SaO2[%] 50 66 75 90 95 100
peripheral vasoconstriction, the body temperature
Chapter 9 · Cardiac Surgical Intensive Care
203 9
can arise to 102 °F (39 °C) in 2–3 h and decreases nary status. Additional chest X-rays are only
to normal values after vasodilatation; a tempera- ordered for specific questions arising from
ture rise combined with peripheral vasodilatation changes in the clinical status of the patient.
is rather a sign of prolonged systemic inflamma-
tory response (SIRS) or septic event. 9.3.3.10 Routine Laboratory
Investigations
9.3.3.8 Blood Sample Analysis Cardiac surgical operations change important
A blood gas analysis should be performed physiological processes: changes to the body tem-
immediately after CSICU admission. perature, full heparinization and reversal along with
Furthermore, blood gas sampling should be heparin rebound, intravascular fluid changes, elec-
repeated if cardiopulmonary instability occurs trolyte shifts, activation of inflammatory cascades,
or if the ventilation settings have been changed etc. During surgery only a few laboratory values are
(here with a time interval of 30 min after tested. Therefore, upon CSICU admission, checking
change). At an inspiratory oxygen fraction the following parameters may be necessary:
(FiO2) of >0.6, a blood gas analysis is recom- 55 Blood count
mended every 4–8 h intervals. 55 Activity of lactate dehydrogenase (LDH)
To maintain an «oxygen reserve» during the 55 Urea and creatinine
transport from the OR to the CSICU, the patient is 55 PT value
generally transferred with a FiO2 of 1.0. Further- 55 PTT
more, manipulations like disconnection from the 55 Antithrombin III levels
ventilator or other problems can be better managed, 55 Activity of creatine kinase (CK) and CK-MB
and the lung function can be estimated on CSICU 55 Troponin I levels
admission. If a patient under controlled ventilation 55 Activity of aspartate transaminase (AST), also
with an FiO2 of 1.0 for transport have a body tem- known as serum glutamic oxaloacetic trans-
perature-corrected PaO2 of <200 mmHg (Horovitz aminase (SGOT)
quotient of <200, normal value in pulmonary 55 Activity of alanine transaminase (ALT), also
healthy patients 350–450), a pulmonary problem known as serum glutamic-pyruvic transami-
can be expected (For Horovitz quotient see also nase (SGPT)
7 paragraph Respiratory Failure in Sect. 9.5.4 Lung and 55 Concentration of C-reactive protein (CRP)
Mechanical ventilation, p. 226). Then the need of spe- 55 Lactate
cial attention and close controls is necessary. In 55 Magnesium
patients with opened pleural cavities, an initial PEEP
of 8 cm H2O is recommended to reduce atelectasis In an uneventful course, a 12-h lab check
and may be weaned down prior to extubation. interval should be adequate. Additionally, daily
Point-of-care laboratory tests using special- laboratory test may include the function of the
ized blood gas-analyzing machines, prompt liver, the lactate, and the total protein and albu-
results can be obtained in the CSICU including min levels. The monitoring of blood glucose lev-
determination of the acid-base balance, concen- els and electrolyte values is additionally measured
trations of electrolytes (especially potassium), and with the periodic blood gas analysis. Other labo-
the hemoglobin and blood glucose values. ratory parameters are checked as indicated.
To recognize the optimal time point for adjust- 9.4.2 Sedation and Analgesia
ing the therapy, the objectives and therapeutic
goals and their progress require a close monitoring For the patient with an uncomplicated course, a
and documentation (usually once per shift). The differentiated analgesia and sedation is suggested,
following criteria have to be achieved during the aiming for antianxiety, analgesia, and vegetative
first 24 h of an uncomplicated postoperative course: protection. According to the current guidelines,
55 Awake patient (Ramsay score of 2) with no various combinations of drugs are available for
evidence of neurological deficit adequate analgesia and sedation. Preferably, the
55 Warm extremities without edema with even concept has to be adapted to the intraoperative
balance anesthetic management to avoid unnecessary
55 No abnormalities in the blood gas analysis change of medication, possibly causing adverse
compared to patient’s baseline interactions of various analgesics and sedatives
55 No evidence of bleeding or clotting disorder due to different pharmacokinetics. . Table 9.4
55 Hemoglobin concentration of >8 mg/dl provides a list of these medications.
55 SaO2 of >92 % (peripheral) To attenuate autonomic responses during
55 MAP of >65 mmHg weaning from mechanical ventilation and in asso-
55 Sinus rhythm with a heart rate of 60–90/min ciation with the extubation, sedation can be com-
55 Adequate left and right ventricular function plemented with clonidine (IV clonidine is not
on echocardiogram available in the USA):
55 CVP of 8–12 mmHg 55 Objective of clonidine use: weaning
9 55 Stable urine output >0.5 ml/kg/h 55 Drug (active ingredient): clonidine
55 Normal lactate levels 55 Drip dosage: 1.5 mg/50 ml
55 Concentration: 0.03 mg/ml
With increased age and patient’s comorbidi- 55 Infusion rate: 1–4 ml/h/70 kg body weight
ties, there is increased risk for adverse events
associated with complicated operative and post- A deep sedation in cardiac surgery should
operative course. The clinical experience shows only be used in special cases:
that errors in the early postoperative period may 55 Complicated long-term mechanical ventila-
lead to life-threatening complications, or at least a tion
complicated course, requiring more costly expan- 55 Abdominal positioning (usually for severe
sion of monitoring, therapeutic arrangements, hypoxemia)
and longer ICU stay. In the following, we will dis- 55 Inadequate oxygen delivery to peripheral
cuss various therapeutic goals and regimens, organs caused by sepsis or multi-organ dys-
some supported by published guidelines. function syndrome
hemostasis and the coagulation system. (age >70 years), (2) low RBC volume either from
Additionally, a high percentage of cardiac surgi- preoperative anemia or from small body size or
cal patients are on dual antiplatelet medications, from both, and (3) urgent or complex operations
altering the coagulation profile as well. Therefore, usually associated with prolonged CPB time and
many reference values for the coagulation profile non-CABG procedures.
are approximations. . Table 9.5 contains expected The transfusion of PRBC in patients with a
values and their development. hemoglobin level less than 7 g/dL is reasonable,
Even a significant abnormality in the coagulation but it is based on good evidence (class IIb, level of
profile may not require immediate therapeutic inter- evidence C), and the transfusion in patients with a
vention. Before any values are treated, the clinical pic- hemoglobin more than 10 g/dL, trying to improve
ture has to be taken into account for decision-making. the oxygen transport, is not recommended (class
Only the context of clinical presentation and labora- III, level of evidence C) (Ferraris et al. 2011).
tory extents should lead to a therapy—for example, In clinical practice the transfusion of PRBC
an elevated blood loss from the chest tubes has to be correlates to the patient’s condition. Patients in
treated—rather a deranged coagulation profile with- life-threatening situation should receive blood
out any clinical evidence of bleeding. The necessity of even if the hemoglobin level is not under 7 g/dL.
blood products has to be checked in any case. If a surgical problem (e.g., surgical site suture
bleeding) is suspected, a resternotomy has to be
9.4.5.1 acked Red Blood Cells
P performed promptly.
(PRBC) Of particular concern are the side effects of
9 The cutoff for transfusion of PRBC has been contro- blood and blood products:
versial. There is more evidence supporting the fact 55 Hemolytic transfusion reaction of immediate
that PRBC transfusion increases morbidity and mor- type
tality, along with increased local and systemic infec- 55 Hemolytic transfusion reaction of the delayed
tious complications (Engoren et al. 2002; Whitson type
et al. 2007). However, it is not clear whether the 55 Febrile, nonhemolytic transfusion reaction
transfusion itself or the comorbidities of the patients 55 Allergic transfusion reaction
requiring transfusions are the ultimate culprit. 55 Transfusion-related acute lung injury
STS guidelines from 2011 suggests three (TRALI)
important preoperative risk factors are linked to 55 Transfusion reactions due to bacterial con-
bleeding and blood transfusion: (1) advanced age tamination
55 Transfusion-associated infections
..Table 9.5 Reference values for the coagulation Transfusion-associated respiratory insufficiency
profile on admission on the CSICU and their may exacerbate the clinical picture in a multi-mor-
postoperative changes bid patient, complicating the postoperative differen-
tial diagnosis and therapeutic approach.
On Postoperative
Extent admission course
9.4.5.2 Platelet Concentrates
Activated Basic value No change The use of platelet concentrates in cardiac surgery
clotting time is necessary due to platelet dysfunction after
(ACT) CPB. A platelet transfusion is necessary for unsta-
Thrombocyte >50.000/μl Increase ble patients with active bleeding if the platelet
count count is <50/nl (<50.000/μ1). In hemodynamically
Prothrombin (PT) >50 % Increase
stable patients, even without bleeding, the thresh-
value old for transfusion is a platelet count of <10/nl
(<10.000/μ1). After transfusion of one unit of
Activated partial <40 s Decrease
thromboplastin
platelet concentrate, the expected raise of the plate-
time (aPTT) let count will be approximately 30/nl (30.000/μ1).
Furthermore, according to STS guidelines, the
Antithrombin III >60 % Increase
use of intraoperative platelet plasmapheresis is
level
reasonable to assist with blood conservation
Chapter 9 · Cardiac Surgical Intensive Care
207 9
strategies as part of a multimodality program in Recommended PCC dose is 25–30 IU/kg as
high-risk patients if adequate platelets yield can initial bolus.
be reliably obtained (Ferraris et al. 2011).
9.4.5.5 Recombinant Factor VIIa
9.4.5.3 Fresh Frozen Plasma (FFP) (rFVIIa)
FFP contains all coagulation factors and their The use of rFVIIa has a limited indication in high-
inactivators. Considered indications for transfu- risk patients as last resort. Recommended dose
sion of FFPs are the emergency treatment of a ranges vary from 11 to 200 μg/kg body weight. We
clinically relevant bleeding and an acute bleeding use 90 μg/kg body weight to be administered within
due to a complex coagulation disorder, expected 2–5 min, preferably immediately after FFP transfu-
after prolonged CPB, and the loss and/or a dilu- sion. If the bleeding does not stop, a second infusion
tional coagulopathy in patients with severe blood may be considered after 20–30 min. There are vary-
loss and extensive transfusions. In these cases and ing results in literature concerning thromboem-
as rule of thumb, a unit of FFP is transfused for bolic side effects from 5 % (Warren et al. 2007) to no
each four PRBC transfusions. increased risk (Kluger et al. 2007; Chapman et al.
FFPs should not be given to expand volume. 2011). According to STS guidelines, rFVIIa concen-
Here are further recommendations from STS guide- trate may be considered for the management of
lines for blood conservation (Ferraris et al. 2011): intractable nonsurgical bleeding that is unrespon-
1. Plasma transfusion is reasonable in patients sive to routine hemostatic therapy after cardiac pro-
with serious bleeding in context of multiple or cedures using CPB (class IIb, level of evidence B).
single coagulation factor deficiencies when
safer fractionated products are not available 9.4.5.6 Antithrombin III (AT III)
(class IIa, level of evidence B). Antithrombin III concentrates has a class I recom-
2. For urgent warfarin reversal, administration mendation according to STS guidelines for blood
of prothrombin complex concentrate is pre- conservation: AT III is indicated to reduce plasma
ferred, but plasma transfusion is reasonable transfusion in patients with antithrombin-mediated
when adequate levels of factor VII are not heparin resistance immediately before cardiopul-
present in prothrombin complex concentrate monary bypass (level of evidence A). In case of
(class IIa, level of evidence B). heparin resistance, and instead of FFP transfusion,
3. Transfusion of plasma may be considered as it is strongly suggested to administer AT III to
part of a massive transfusion algorithm in improve activated clotting time for the CPB.
bleeding patients requiring substantial Various dosing algorithm exists based on
amounts of red blood cells (class IIb, level of baseline antithrombin activity levels. If such levels
evidence B). are not available in the operating room, 1,000 IU
is given parenterally, and the activated clotting
9.4.5.4 Prothrombin Complex time is remeasured.
Concentrate
Prothrombin complex concentrates (PCCs) are
hemostatic blood products containing four vita- 9.4.6 Nutrition
min K-dependent clotting factors (II, VII, IX, and
X), as well as the anticoagulant inhibitor proteins Poor nutritional status can adversely affect thoraco-
C, S, and Z (Ansell et al. 2004). They are a useful, pulmonary function in spontaneously breathing
reliable, and fast alternative to fresh frozen plasma and mechanically ventilated patients with respira-
for the reversal of the effects of oral anticoagulant tory disease by impairment of respiratory muscle
treatments (vitamin K antagonists). The use of function, ventilatory drive, and pulmonary defense
PCC rather than FFP is recommended for mechanisms. The questions of whether, when, and
Coumadin reversal in patients with major bleed- how intensive care patients must be fed are essential
ing (Baglin et al. 2006; Baglin et al. 2007). The use in planning the nutrition plan at the CSICU. In
of PCC is not the first choice in bleeding compli- critically ill patients, including cardiac surgical
cations, because PCC stems from a large pool of patients, an underfeeding is related with a poor out-
donors and puts the recipients at increased risk of come (Preiser et al. 2015). It is recommended to
anaphylactic shock, viral transmission, etc. consider enteral feeding in patients who will not
208 A. Markewitz et al.
have oral intake within the next 5 days. An enteral It is common practice that the daily need for
nutrition is preferable, unless the patient is on high- ICU capacities deforms the clinical picture of the
dose inotropes, making adequate gut perfusion patient to a better one. Unfortunately this may
unlikely, or there is an enteral passage malfunction lead to higher readmission rate to the CSICU. If
due to injury or surgical operations. Enteral feeding the readmission rate exceeds 5 %, discharge crite-
is started after the position of jejunal nutrition tube ria should be revisited.
has been confirmed on X-ray and with elevation of
the upper part of the body. For patients with venti-
lator failure, it is recommended to avoid overfeed- 9.5 Special CSICU Arrangements
ing, causing nutritionally associated hypercapnia.
This may delay timely extubation. 9.5.1 Introduction
It is important to provide patients with an esti-
mated prolonged intubation time with additional
calories, to be started within the first 24 h. The 9.5.1.1 The Importance
required amount of calories is 20–25 kcal/kg body of Communication
weight/day (Kreymann et al. 2006). Controlling sub- Especially in critically ill patients, the importance of
sequent hyperglycemia, therefore reducing the risk communication with the patient himself and his fam-
of infection using insulin drip protocols, remains ily, but also with the medical and nonmedical staff on
critical aspect in ICU nutrition (van den Berghe the CSICU, is of great importance. The clinical situa-
et al. 2001; Bhamidipati et al. 2011; Haga et al. 2011). tion of the patient as well as the medical course for the
9 Optimal support would establish neutral or next 24 h has to be evaluated and discussed in this
positive nitrogen balance, depending on the need «family-and-CSICU-staff team.» It is important to
for protein repletion. In the critically ill patient create a mutual trust, especially if serious and final
with renal function impairment, this can be accom- decisions have to be made. Additionally clerical or
plished by giving 1–3 g of protein/kg daily. psychological support can be an important part of
Generally, this amounts to approximately 20 % of the family-and-staff team and should be consulted
total calories being administered as protein. While early, especially in a complicated course. The bulk of
recommendations for an appropriate substrate mix problems can be avoided using open communication
of carbohydrates and fats vary, generally 60–70 % and care, whereas poor communication skills and a
carbohydrates are given with 20–30 % fats. lack of understanding may lead to complaints from
patient, family members, or health-care providers.
..Table 9.6 Prevalence and mortality of multi-organ failure after cardiac surgery
Organ complications/failure
surgery. The standard therapy is the adminis- (. Fig. 9.1). Differences in Guidelines from the
tration of volume and norepinephrine (Carrel European Resuscitation Council and the
et al. 2000). In some patients there is a norepi- American Heart Association are featured in a
nephrine-resistant reduced peripheral vessel Critical Care Nurse article more recently (Ley
resistance (Levin et al. 2004). This is called a 2015).
vasoplegic syndrome. A possible correlation to The survival rate of these patients is better than
preoperatively administered ACE inhibitor expected: overall 33 % of patients in whom a reth-
therapy is discussed. Besides ruling out hypo- oracotomy due to cardiac and circulatory arrest on
adrenal syndrome, initiation of intravenous the CSICU was performed will survive (Mackay
vasopressin therapy or methylene blue is rec- et al. 2002), compared to 48 % survival rate if the
ommended (Egi et al. 2007). rethoracotomy was performed within the first
10 min after the arrest (Mackay et al. 2002).
kCardiac arrest
The worst case of a LCOS is the cardiac arrest that 9.5.2.5 Therapy of LCOS
appears in 1–2 % of cardiac surgery patients
(European Resuscitation Council, Nolana et al. kPharmacological circulatory support
2010). The most common causes of postoperative The therapy of LCOS is the continuous intrave-
cardiac arrest are (modified after European nous application of circulatory supportive medi-
Resuscitation Council, Truhlár et al. 2015): cation that will be described in the following.
55 Myocardial ischemia
9 55 Tension pneumothorax kCatecholamines
55 Pericardial tamponade Dopamine acts directly and indirectly on
55 Massive bleeding with hypovolemic shock α-adrenergic receptors as well as on β-adrenergic
55 Pacemaker dysfunction in patients with little receptors, in addition to its affinity to dopamine
or no intrinsic rhythm receptors. The affinity to receptors is dose depen-
55 Electrolyte disturbances, especially in low or dent: 0.5–3 μg/kg/min leads to a vasodilatation of
high levels of potassium renal and abdominal vessels via dopamine recep-
tors, 3–10 μg/kg/min raises the heart rate and the
The cardiac arrest can be directly monitored cardiac output with increasing the arterial and pul-
by the minimal or missing peaks in the blood monary artery pressure via stimulation of the
pressure curve. Ongoing ventricular fibrillation ß-receptors, and a dosage of more than 10 μg/kg/
or cardiac arrest is displayed by the ECG. Therapy min increases the peripheral vascular resistance
has to be started immediately. Obvious reasons due to stimulation of the α-adrenergic receptors
can be treated easily: If a tension pneumothorax is with additional release of norepinephrine. An
present, the situation can be handled by deploy- increase in mean pulmonary artery pressure and
ment of a thoracic drain. If the pacemaker is not the wedge pressure can be corrected using pulmo-
active or the connection is defective, it must be nary vasodilators. Important side effects are sup-
reconnected or replaced or a transvenous or pression of the pituitary gland hormone, ischemia
transcutaneous pacer be placed. If the electrolyte of gastrointestinal mucosa, and, as with all cate-
situation is unbalanced, it has to be corrected. cholamines, an increase of myocardial oxygen con-
A myocardial ischemia leading to ventricular sumption. Important to mention that low-dose
fibrillation can be treated using a biphasic electric dopamine has not been shown to prevent renal
defibrillation shock with maximum energy. failure (Lassnigg et al. 2000).
Additionally the ischemia has to be treated with a Epinephrine activates ß1-, ß2-, and
cardiopulmonary resuscitation. The resuscitation α-adrenergic receptors. The receptor response is
has to be performed according to ACLS protocol. dose dependent: 0.02–0.05 μg/kg/min increases
If classical ACLS protocol does not resolve the the inotropic effect predominantly through the
cardiac arrest in CSICU, immediate opening of ß1-receptors, 0.05–0.2 μg/kg/min increases the
the chest is recommended, as last resort. This will inotropic effect and the peripheral vascular resis-
alleviate tamponade and pneumothorax, will help tance by the ß- and the α-receptors, and a dosage
diagnose significant bleeding, and will allow >0.2 μg/kg/min will increase the peripheral
internal cardiac compression and defibrillation vascular resistance by increasing effects on the
Chapter 9 · Cardiac Surgical Intensive Care
213 9
Irresponsive
patient
No response
..Fig. 9.1 Therapeutic algorithm for cardio-circulatory arrest (Modified after European Resuscitation Council (2010)).
VT ventricular tachycardia, ECG electrocardiogram
α-receptors. Important side effects are tachycar- Norepinephrine is the first choice as vasocon-
dia, increase of the mean pulmonary artery pres- strictor and is recommended in two situations:
sure and the wedge pressure, ischemia of 55 In patients with low blood pressure due to
gastrointestinal mucosa, and an increase of myo- low systemic vascular resistance, if this can-
cardial oxygen consumption. not be effectively treated by administration of
Norepinephrine activates α-adrenergic volume or positive inotropic agents
receptors, but also ß1- and ß2-receptors. 55 To compensate the initial blood pressure drop
Although there is increase in contractility, the after starting a phosphodiesterase-III-
cardiac output overall is not increased due to inhibitor therapy such as milrinone
higher peripheral vascular resistance. The arte-
rial vasoconstriction improves the perfusion Dobutamine activates the ß1-receptors and
pressure to all organs. Important side effects are significantly ß2- and α-adrenergic receptors.
tachycardia (less than epinephrine) and increase Dobutamine is a positive inotropic and lusitropic
of mean pulmonary artery and pulmonary artery agent (improves myocardial relaxation in the
wedge pressure. diastole) and has also vasodilatory effect. This
214 A. Markewitz et al.
improves the cardiac output. Most common rele- Furthermore it can be used to reduce an elevated
vant side effects are tachycardia and increased pulmonary artery pressure and in right heart fail-
myocardial oxygen consumption. ure. Important side effect is a hemodynamically
Due to lack in increase of the mean pulmo- relevant tachycardia with hypotension. Further,
nary artery pressure and the wedge pressure and the inhibition of hypoxemia-triggered pulmonary
maintenance of perfusion of the visceral arteries, vasoconstriction and an increased intrapulmo-
in our practice the use of dobutamine is the first nary right-left shunt can lead to decreased oxy-
choice in increasing cardiac output. genation. Finally, a high dosage can result in
severe headaches, and prolonged applications will
kVasodilators cause tolerance that mitigates the effect. Details of
Phosphodiesterase-III inhibitors such as milri- pharmacotherapy are discussed under treatment
none: In contrast to catecholamines, milrinone is of arterial hypertension (7 Sect. 9.5.2.6).
an adrenoreceptor-independent inotrope with
vasodilatory characteristics. The administration kNitroprusside
leads to an increase of intracellular cAMP levels Sodium nitroprusside decreases the afterload and
due to a blockade of cAMP depletion. This trig- the preload with consequent increase of cardiac
gers the calcium influx into the cell and an output. Therefore, the myocardial oxygen con-
increased release of calcium from the sarcoplas- sumption is decreased.
mic reticulum. Smooth muscle cells with an In severe LCOS the use of sodium nitroprus-
increased level of cAMP dilate. In addition milri- side is recommended to reduce systemic vascular
9 none may increase the heart rate by affecting the resistance. Similar to nitroglycerine, the side
sinus node and increase the atrioventricular con- effects are decreased oxygenation, and in our Los
duction transmission. In contrast to catechol- Angeles experience, we do not recommend it for
amines, the myocardial oxygen consumption is aortic dissection, as there is evidence that nitro-
not significantly augmented due to a simultane- prusside may increase shear stress on the aortic
ous reduction of the pre- and afterload. In sum- wall. Some references, however, suggest to
mary, milrinone has a positive inotropic effect employ it combined with beta-blockers (Erbel
with an increased cardiac output along with a et al. 2001; Tsai et al. 2005). The most dangerous
drop of cardiac filling pressures and the pulmo- side effect is cyanide intoxication. In order to
nary vascular resistance. Side effects are long avoid this side effect, nitroprusside medication
half-
life time with difficult dose adjustments, should be accompanied by IV sodium thiosulfate
increased pulmonary shunting, and hypotension infusion.
requiring vasoconstrictors. In clinical practice, Details of pharmacotherapy of sodium nitro-
milrinone is used when inotropic effect of dobu- prusside are discussed under treatment of arterial
tamine is reduced due to ß-adrenergic receptor hypertension (7 Sect. 9.5.2.6).
overregulation.
kInhalative Vasodilators
kLevosimendan Inhaled vasodilators, such as nitric oxide (NO) are
Levosimendan is used to prevent and to treat often used to treat a pulmonary hypertension. The
LCOS and acts as a calcium sensitizer. It is increas- mode of NO action is the activation of guanylate
ingly being used in Germany. Levosimendan is cyclase (cGMP) with a specific dilation of pulmo-
currently not available in the USA for clinical use. nary vessels. The drop of the pulmonary resistance
For further information see 7 Chapter «Critical and the shift of blood flow through ventilated lung
Care in Pediatric Cardiac Surgery», Sect. 10.3.2.4. areas reduce the pulmonary pressure and improve
the arterial oxygenation. In contact with hemoglo-
kNitroglycerin bin, the NO is been deactivated. Therefore, the NO
The administration of nitroglycerin leads to a dil- does not enter the systemic circulation. Important
atation of vessels, especially the venous and the side effects are the methemoglobinemia due to
coronary vessels and, in smaller amount, the arte- prolonged use and an increased bleeding tendency,
rial system. The primary use is the prophylaxis but the incidence is low by using the recommended
and the therapy of a myocardial ischemia. dosage of <20 ppm.
Chapter 9 · Cardiac Surgical Intensive Care
215 9
An alternative for pulmonary vasodilatory kBasic rules in pharmacological circulatory
effect is the phosphodiesterase-V inhibitor silde- support
nafil. Sildenafil is available orally with the similar A LCOS has to be treated immediately according
effects such as NO. There is a potential side effect to the underlying reason. Therefore an algorithmic
of systemic hypotension with sildenafil. approach to pharmacological circulatory support
is critical. The results of Swan-Ganz catheter and
kVasopressors transthoracic echocardiography are frequently not
Vasopressors are primarily used to increase the available immediately, allowing for algorithmic
arterial perfusion pressure, in cases where the approach recommended in . Figs. 9.2 and 9.3.
optimized volume status and positive inotropic However, the basic circulatory monitoring
response is inadequate for peripheral perfusion. will provide important information, allowing for
Vasopressin as well as methylene blue belongs to following rules of thumb:
the vasopressors. 55 In a patient with MAP <60 mmHg and a cen-
tral venous pressure of >12 mmHg, treatment
kVasopressin with positive inotropic agents should be con-
Vasopressin activates the vasopressin-1 receptor sidered.
with an increase of the intracellular calcium level. 55 The worse the left ventricular function is in
Vasopressin is very effective especially in the the patient, the earlier should these drugs be
vasoplegic syndrome, unresponsive to maximal initiated.
norepinephrine dosage. The side effect of vaso- 55 The worse the left ventricular function is in
pressin even in low dose is significant decrease of the patient, the more sensitive the heart may
microcirculation. Therefore the use of vasopressin respond to volume administration, especially
should be considered as second or third choice in an already preoperatively increased left
and preferably should be used in low dosage. and right ventricular preload.
55 Initial drug of choice is dobutamine in com-
kMethylene blue bination with norepinephrine and/or epi-
The effects, side effects, and indications for use nephrine given the aforementioned
of methylene blue are similar to vasopressin. advantages and side effects. Dobutamine
Although there are promising reports, overall reduces systemic and pulmonary vascular
there is little data supporting routine use of resistance along with inotropic effect. If the
methylene blue. Further details are given in vasodilatory effects are too strong, norepi-
. Table 9.7. nephrine or epinephrine may be used.
Levosimendan [not available in the USA] 12 bis 24 μg/kgKGa 0.1 μg/kgKG/min (0.05 bis 0.2 μg/kgKG/min)
LVEDAI: <5 cm2 /m2 <7 cm2 /m2 >9 cm2 /m2 >11 cm2 /m2
Norepinehrine 2) Epinephrine
3) Epinephrine
Goals achieved?
IABP, VAD, OP
..Fig. 9.2 Therapeutic algorithm for left heart failure according to results of extended monitoring. IABP intra-aortic bal-
loon pump, ITBVI intrathoracic blood volume index, LVEDAI left ventricular end-diastolic area index, OP redo surgery, PAC
pulmonary artery catheter, PAWP pulmonary artery wedge pressure, PDE III phosphodiesterase III, TEE transesophageal
echocardiography, VAD ventricular assist device (Modified after Carl et al. (2010). This is an open-access article distributed
under the terms of the Creative Commons Attribution License (7http://creativecommons.org/licenses/by-nc-nd/3.0/
deed.en))
Yes ! No !
Goals achieved ?
IABP, VAD, OP
..Fig. 9.3 Therapeutic algorithm for right heart failure according to data from extended monitoring. IABP intra-aortic
balloon pump, OP redo surgery, PAC pulmonary artery catheter, PAWP pulmonary artery wedge pressure, PDE III phos-
phodiesterase III, TEE transesophageal echocardiography, VAD ventricular assist device. CVP central venous pressure, LV
left ventricular, MAP mean arterial pressure, NO nitric oxide, NTG nitroglycerin, PHT pulmonary hypertension, RV right
ventricular, SVR systemic vascular resistance
Tamponade?
Optimize preload
..Fig. 9.4 Staged approach to therapy of postoperative low cardiac output syndrome (LCOS), stage I: CVP central
venous pressure, LHF left heart failure, LRHF left and right heart failure, MAP mean arterial pressure, RHF right heart
failure, SaO2 oxygen saturation, ScvO2 central venous oxygen saturation
and a decrease of the left ventricular afterload by patients with an impaired left ventricular ejection
well-timed balloon deflation. This leads to an fraction, left main stem stenosis, unstable angina,
enhancement of coronary perfusion and a relief of or the need of a coronary reoperation to improve
cardiac work with the result of an improved oxy- outcomes (Dunning and Prendergast 2003).
gen supply-demand ratio and a somewhat The useful effects of the IABP use can be
increased cardiac output. immediately seen at the pressure curve after
The appropriate time of initiation of the IABP IABP is initiated: due to the reduced cardiac out-
is as soon as possible (Baskett et al. 2002; put, diminished first peak in the arterial pressure
Christenson et al. 2002; Ramnarine et al. 2005). It curve is frequently followed by a second higher
is frequently helpful for the treatment of LCOS and peak as function of the preload reduction.
especially helpful for coronary patients during the Recovery of the left ventricular function leads
weaning process from extracorporeal circulation. usually to a conversion of the peaks—the first
The exceed threshold of an epinephrine dose level one overgrow the second one. This is an indica-
of >0.2 μg/kg/min or a dobutamine dose level of tion for removal of IABP, especially in face of sig-
>10 μg/kg/min should be the indication for IABP nificantly reduced pharmacological circulatory
use. Preoperatively, IAPB will be very useful in support. The IABP should be weaned from a 1:1
Chapter 9 · Cardiac Surgical Intensive Care
219 9
Hemodynamic goals not achieved despite rhythm optimization and volume adjustment
Extended monitoring
LHF RHF
..Fig. 9.5 Staged approach to therapy of postoperative low cardiac output syndrome (LCOS), stage II: CVP central
venous pressure, IABP intra-aortic balloon pump, LHF left heart failure, LRHF left and right heart failure, LV left ventricu-
lar, MAP mean arterial pressure, RHF right heart failure, RV right ventricular, SaO2 arterial oxygen saturation, ScvO2 central
venous oxygen saturation
Hemodynamic goals not achieved despite rhythm optimization, volume adjustment, adding
positive inotropic medication, improved pre-and afterload and insertion of IABP:
Still two of the following 5 criteria combined define LCOS:
Extended monitoring
If not: Timely decision for further therapeutic options required, evaluate indication for
cardiac transplantation
..Fig. 9.6 Staged approach to therapy of postoperative low cardiac output syndrome (LCOS), stage III: BiVAD biven-
tricular assist device, IABP intra-aortic balloon pump, LHF left heart failure, LVAD left ventricular assist device, MAP
mean arterial pressure, RHF right heart failure, RVAD right ventricular assist device, SaO2 arterial oxygen saturation,
ScvO2 central venous oxygen saturation
..Table 9.8 Review of approach to patients with acute aortic syndrome presenting with hypertension to the CSICU
Propafenone 100–150 mg po
with lidocaine (100–150 mg) or amiodarone lines for tolerable chest tube outputs, which, how-
(300 mg) or with an overdrive pacing. ever, do not apply to all patients (. Table 9.11).
While . Table 9.10 gives an overview about The amount of chest tube output and its assumed
commonly used antiarrhythmic drugs, . Fig. 9.7 cause are important for therapeutic decision-
shows our treatment algorithm for tachycardias. making. If the diagnosis is unsecure, a hematocrit
According to their mode of action, the com- check of chest tube drainage will establish the
monly used antiarrhythmic drugs can be diagnosis.
divided into four classes and others. The four After cardiac surgery employing extracorpo-
classes are: real circulation, function of the coagulation sys-
1. Sodium channel blockers tem is severely impaired. Reasons are full
2. β-blockers heparinization, reduced platelet function (preop-
3. Potassium channel blockers erative antiplatelet therapy, perioperative hemo-
4. Calcium channel blockers dilution, hypothermia, and mechanical irritation),
and lack of fibrinogen in combination with a
hyperfibrinolysis (in close correlation to the dura-
9.5.3 leeding Complications and
B tion of the extracorporeal circulation). Initially
Pericardial Tamponade increased chest tube output is not unusual but has
to be watched carefully.
On ICU admission, the surgeon will report
9.5.3.1 I ncidence, Cause and any intraoperative bleeding complication and if
Diagnostic there may be a surgical reason for the bleeding. If
Bleeding complications occur after cardiac sur- the deranged coagulation system is the presumed
gery with an incidence of 1–3 % (Mehta et al. underlying cause, appropriate diagnostics should
2009). The following table indicates general guide- be run to guide therapeutics:
226 A. Markewitz et al.
No
SR ?
Yes
..Fig. 9.7 Our management algorithm for tachycardia. AF atrial fibrillation, SR sinus rhythm, SVT supraventricular
tachycardia, VT ventricular tachycardia
IPPV, intermittent positive pressure SIMV, synchronized intermittent BIPAP, biphasic positive airway
ventilation mandatory ventilation pressure
CPPV, continuous positive pressure MMV, mandatory minute volume
ventilation PSV, pressure support Ventilation
VCV, volume controlled ventilation ASB, assisted spontaneous breathing
PCV, pressure controlled ventilation CPAP, continuous positive airway
pressure
3. Postoperative risks
Reduction of FiO2
55 Serum albumin level <4 g/dl
55 Low cardiac output requiring catechol-
amines
Assisted ventilation 55 Resternotomy for bleeding
55 Reperfusion injury after long-term extra- 2007) and gained some interest more recently in
corporeal circulation cardiac patients, also (Guarracino and Ambrosino
55 Pulmonary parenchymal hemorrhage 2011; Cabrini et al. 2015), but it remains contro-
55 Secondary involvement due to multi-organ versial.
failure
kVentilation in respiratory failure
Based on the causes, the required diagnostics Since only a portion of alveoli are sufficiently ven-
are: tilated and perfused, the ventilation of patient
55 Bronchoscopy, combined with sampling with ARDS is challenging: The interstitial edema
for microbiological analysis decreases the compliance of the lungs. Therefore
55 Critical analysis of the pulmonary and the ventilation pressure is increased to maintain
perioperative history the effective minute volume, imposing additional
55 Chest X-ray stress to the alveoli and causing their further
55 CT scan destruction. To reduce these deleterious effects,
reduced tidal volume to 4–6 ml/kg has been
The pathophysiology of the acute respiratory shown to be associated with improved outcomes
failure is topic of many research endeavors and is (The Acute Respiratory Distress Syndrome
discussed below in brief: Network 2000). A possible side effect of this
In the beginning of acute respiratory failure, alveoli-protective strategy is an increased CO2
the capillary endothelium is damaged by toxin partial pressure that can be tolerated upon
9 that leads to hyperpermeability of the endothe- 80 mmHg (permissive hypercapnia), unless the
lium followed by an interstitial pulmonary edema. associated metabolic acidosis would be harming
This increases the gas diffusion distance and the patient.
causes collapse or narrowing of the alveoli. This In acute severe lung failure, high PEEP levels
process is enhanced by the loss of surfactant fac- have to be used. The PEEP setting on the ventila-
tor. Ventilation-perfusion mismatch occurs: the tor is usually 12–15 cm H2O but may be increased
not ventilated lung parts are well perfused, to maximal 25 cm H2O. To calculate the approxi-
whereas the ventilated lung parts are only insuffi- mate required PEEP, the formula PEEP ≤
ciently or not perfused. The thrombotic occlusion FiO2/0.05 can be useful. The exacter method is to
of pulmonary capillaries, hypoxemia-triggered adjust the PEEP to the pressure-volume curve,
vasoconstriction, the release of proinflammatory where the PEEP value has to be chosen right
mediators, and the ventilation-perfusion mis- above the lower inflection point. The lower inflec-
match worsens the pulmonary function. The tion point indicates the pressure limit that is nec-
ensuing hypoxemia deteriorates the function of essary to open the alveolus. A PEEP right above
other organ systems. Adequate oxygenation needs this point ensure open alveolus (Hemmila and
to be maintained, and the aggressive ventilation Napolitano 2006; Malhotra 2007; Wheeler and
mode (FiO2 1.0, high ventilation pressure) neces- Bernard 2007). Adequate PEEP will prevent alve-
sary for adequate oxygenation may enhance the oli trauma caused by collapse and reopening. The
structural damage of the alveoli and the lungs. effect of high PEEP to the circulatory system
occurs only in patients with hypovolemia, but
kTherapy higher risk of pneumothorax has to be considered
Treatment of acute respiratory failure consists of in all patients with sudden respiratory deteriora-
two basic principles: a causal and a symptomatic. tion. It is important to mention that there is no
The cause of ARDS must be treated promptly. data supporting improved outcome with high
The symptomatic treatment entails ensuring PEEP ventilation in respiratory failure. The strat-
proper oxygenation of the patient. This includes egy of a low tidal volume, permissive hypercap-
adequate mechanical ventilation and other sup- nia, and high PEEP is called the open lung
portive care, such as special positioning of the concept.
patient. Noninvasive ventilation in patients In addition to high PEEP ventilation, recruit-
developing acute respiratiory failure after pri- ment maneuver is helpful in maintaining alveoli
mary extubation has been advocated (Hill at al. open: this includes brief gradual increase of
Chapter 9 · Cardiac Surgical Intensive Care
231 9
Ventilatory settings:
Goals achieved (1) No
– tidal volume: ≤6 cc/kg
No
..Fig. 9.9 Our initial therapeutic approach in acute respiratory failure/acute pulmonary failure. FiO2 inspired oxygen
fraction, paO2 arterial oxygen partial pressure, PEEP positive end-expiratory pressure, CXR chest X-ray, SaO2 arterial
oxygen saturation
maximal ventilation pressure up to 40 cm H2O to hypoxemic patients, but usually requires para-
«recruit» (reopen) collapsed alveoli. After recruit- lyzing medication.
ment maneuver is finished, the maximal ventila- Yet another mode of ventilation that can be
tion pressure is reduced slowly to lowest possible useful in patients with acute respiratory failure
PEEP that keeps the alveolus open based on lower and maximal reduced lung compliance is the
inflection point. high-frequency oscillatory ventilation mode. A
If the open lung concept with a low tidal vol- high ventilation frequency between 300 and 600/
ume and a high PEEP do not sufficiently improve min reduces the tidal volume to 1–4 ml/kg and
oxygenation, inversed ratio ventilation may be subsequently reduces stress to the alveoli
tried: the inspiration-expiration ratio has to be (Hemmila and Napolitano 2006). The expiration
changed to a prolonged inspiration time (2:1 up is actively carried out by a vibrating membrane, in
to 4:1), and the FiO2 has to be maximally contrary to other forms of high-frequency venti-
increased to 1.0. This is an effective approach in lation (i.e., jet ventilation).
232 A. Markewitz et al.
9
Goals (1) achieved No
Increase FiO2
..Fig. 9.10 Our approach for therapeutic escalation in acute respiratory distress syndrome: FiO2 inspired oxygen
fraction, paO2 arterial oxygen partial pressure, PEEP positive end-expiratory pressure, SaO2 arterial oxygen saturation
De-escalation
of therapy
The 135° positioning and special rotational beds time and the CSICU stay. This entails daily
are less effective than the true 180° positioning brief disruption of the sedatives, until the
but certainly very helpful. To perform these patient is awake and more alert, before reiniti-
maneuvers, the patient’s hemodynamics has to be ating the sedation.
stable. This may not be the case early after open- The early initiation of enteral nutrition has
heart cases. shown a positive effect in reducing ventilator
The fluid management of the respiratory- period in patients with mechanical ventilation
impaired patient has to be restrictive to avoid more than 36 h, whereas the endotracheal surfac-
any volume overload that results in an increase tant therapy and the inhaled medications, such as
of the existing lung edema, worsened by cardio- prostacyclin or iloprost, have not proven to be
genic pulmonary edema. However, intravascular efficacious.
hypervolemia has to be avoided given the high Steroids improve blood pressure, the pulmo-
PEEP ventilation. nary oxygenation, and the duration of mechanical
Furthermore, «sedation holiday» is an ventilation in ARDS but do not reduce the 60- or
effective method in shortening ventilation 180-day mortality.
234 A. Markewitz et al.
If, despite maximal aforementioned efforts, intubated and ventilated starts by reducing the
adequate oxygenation is not secured, more inva- sedation so far that the patient is stress-free and
sive methods may be considered in appropriate hemodynamically stable under assisted
patient cohort, understanding the significant ventilation. Then the patient controls his breath
cost involved: there are pump-driven systems, rate, but the tidal volume is assisted by the ven-
such as extracorporeal membrane oxygenation tilator. Subsequently the ventilation pressure is
(ECMO) for heart and lung support, or pump- reduced stepwise, allowing the patient do most
less systems that work due to the artery-venous of the work of breathing. The blood gas analysis
pressure gradient. In both systems the blood and the clinical situation should be stable
oxygenation occurs with assistance of a gas within 30–60 min without evidence of respira-
exchange membrane oxygenator. The oxygen- tory fatigue.
ation is more effective using ECMO, but the Signs of respiratory fatigue are:
pumpless systems are more often used in hyper- 55 Agitation
capnic patients with hypoxemia responding to 55 Anxiety
conventional measures. 55 Sweating
Due to its pump drive, the ECMO is not 55 Tachycardia
dependent on the circulatory situation, whereas 55 Blood pressure drop or rising
the pumpless systems require a stable or stabi- 55 Centralization (peripheral vasoconstriction)
lized cardiocirculatory function. ECMO con- 55 Inadequate ventilation time volume
sumes significantly more CSICU resources 55 Inadequate tidal volume
9 (Bein et al. 2007). 55 Breath rate of >35 per minute
. Figures 9.9, 9.10, and 9.11 show treatment 55 Peripheral oxygen saturation of <92 %
algorithms and therapeutic escalation in respira- 55 Arterial oxygen partial pressure <60 mmHg
tory distress situations.
If the patient displays these symptoms, the
kTracheotomy extubation should be delayed for another 24 h,
Tracheotomy is a supportive measure provid- before a second extubation is attempted.
ing the following advantages: secured airway, If the patient is stable and calm, the sedation
easier oral care, more comfort for the patient, should be ended. Just before extubation the
and no larynx trauma. Weaning from mechani- patient should be totally awake, should push the
cal ventilation is faster and less complicated in hand on command, and should raise the head off
patients having tracheotomy (De Leyn et al. the pillow. If a gastric tube is in place, it should
2007). Reduced dead space ventilation by tra- be completely suctioned and removed before
cheotomy reduces the work of breathing for the extubation.
patient. The procedure can be performed sur- After extubation, the patients are positioned
gically or using percutaneous Seldinger in sitting or with upper body elevated 30–45° to
approach in CSICU-dilatational tracheotomy— facilitate the work of breathing and reduce aspi-
that can be done on bedside with bronchoscopy ration risk. The possibility of reintubation has
(Ciaglia et al. 1985). to be considered in elderly, frail, malnutri-
The optimal timing for a tracheotomy is tioned patients, or those with poor mental
unclear yet (Young at al. 2013), but the positive status and prolonged ventilator support.
effect of this treatment should encourage the . Figure 9.12 summarizes our approach to wean
intensivist to perform it earlier if prolonged patients from ventilation after a complicated
mechanical ventilation is expected. course.
Once pulmonary problem solved, check daily whether weaning off ventilation is possible
Wait for 2 to 3 h
– Pt meets general criteria for extubation: SaO2: >92 % at FiO2 of 0.5, respiratory rate <35/min;
– <20 % change in heart rate and blood pressure, no agitation, pt coughs on command.
– Pt meets special criteria for extubation: paO2: >60 mmHg, SaO2: >92 % at FiO2 ≤0.5 with
– PS≤8 cm H2O, VMV <15 L/min, art. pH: 7.35–7.45
Yes No
..Fig. 9.12 Our approach for weaning off mechanical ventilation. FiO2 inspired oxygen fraction, paO2 arterial oxygen
partial pressure, PEEP positive end-expiratory pressure, PS pressure support, SaO2 arterial oxygen saturation, VMV
ventilator minute volume
55 Decrease of renal blood flow with activation 55 Pulmonary trauma due to a high FiO
of the renin-angiotensin system 55 Damages of the alveoli due to high tidal vol-
55 Reduction of airway mucus clearance ume or a high airway pressure
55 Damage to the surfactant monolayer by posi- 55 Barotrauma of the lungs with the risk of a
tive airway pressure-mediated shear stress pneumothorax
55 Decubitus and thromboembolic complica-
The following complications should be men- tions due to immobilization
tioned:
While most side effects and complications
55 Trauma to or edema of larynx, trachea, and may be self-limiting, the most deleterious effect
bronchi on a patient with acute respiratory failure is from
55 Ventilator-associated pneumonia ventilator-associated pneumonia (VAP). VAP
236 A. Markewitz et al.
Estimated eGFR ml / minute / 1.73 m 2 = 186 × [serum creatinine ]−1.154 × [ age ]−0.203
: [ 0.742 if female ] × [1.210 if African − American ]
disease (CKD) is defined as a baseline eGFR 2002); this not being the case with N-acetylcysteine
≤60 ml/min/1.73 m2, while chronic renal failure or «renal-dose» dopamine. Avoiding any nephro-
(CRF) is defined as eGFR ≤30 ml/min/1.73 m2 toxic function, such as aminoglycosides, and all
(stage III versus IV and V according to the Kidney prostaglandin-synthesizing blockers is important
Disease Outcome Quality Initiative) (National but not always possible.
Kidney Foundation 2002).
In clinical practice, many cardiac surgical 9.5.5.3 herapy of Renal
T
patients can be classified into RIFLE criteria Complications
«risk,» and some may correspond to the level During the first hours after cardiac operation, the
«injury.» However, the recovery is the norm in urine production decreases in nearly all patients
majority of these patients. due to various causes. Once in a while, the urinary
Starting with the level «failure,» the treatment catheter may be clogged or kinked, or the mean
of the patients is going to be more complex and arterial pressure is too low. These problems can be
resource intensive. CSICU capacities are limited, solved easily, and they have to be considered early
and patients that require hemodialysis may in the evaluation of the patient. Generally diuret-
occupy them for a prolonged period of time. The ics, such as furosemide or torasemide or mannitol,
preoperative risk stratification of renal failure is are given to improve urine output, understanding
not easy to perform, but the Society of Thoracic that urine output per se does not imply extend of
Surgeons (STS) provides a risk score for postop- kidney function. Also, the maximum dose per day
erative renal failure (Online STS Risk Calculator) of diuretics has to be taken into account.
(. Table 9.17). . Figure 9.13 shows how the If urine output cannot be reactivated with the
results of the STS Risk Calculator translate into a aforementioned methods, the decision for renal
probability for renal replacement therapy. replacement therapy should be made within 24 h
or less of oliguria or anuria. Options for renal
9.5.5.2 revention of Renal
P replacement therapy such as intermittent or a
Complications continuous filtration mode does not influence the
Prevention is the best therapy of renal failure. It is patient’s outcome (Garwood 2004). Diuretic
important to ensure an adequate hydration, oxy- administration, however, should to be stopped
genation, and adequate mean arterial pressure. due to its contra-productive effect (Mehta et al.
The perioperative administration of aspirin has 2002), as up to 25 % of these patients will require
proven to have a positive effect (Mangano et al. long-term renal replacement therapy.
40
30
20
10
0
1 4 7 10 1316 19 22 25 28 31 34 37 40 43 46 49 52 55 58 61 64 67 70
total points in Tab. 9.17 (STS-Risk-Score)
238 A. Markewitz et al.
..Table 9.17 Parameters and corresponding points for risk assessment, if perioperative renal replacement
therapy will be required
Age <55 55–59 60–64 65–69 70–74 75–79 80–84 85–89 90–04 95–99 >100
Points 0 1 2 3 4 5 6 7 8 9 10
Points 5 10 15 20 25 30 35 40
Points 0 2 5 4 7
Myocardial No <3 weeks
infarction
Points 0 3
Points 0 2
9 Chronic
lung disease
No Yes
Points 0 3
Reoperation No Yes
am Herzen
Points 0 3
NYHA IV No Yes
Points 0 3
Cardiogenic No Yes
shock
Points 0 7
Sum
9.5.6 Gastrointestinal Tract ated with a mortality of 70–100 %, and high index
of suspicion is critical to promptly achieve the
Severe complications of the gastrointestinal tract, right diagnosis. The symptoms are usually unspe-
such as bleeding, ulcer, prolonged ileus, pancre- cific, causing late diagnosis. Furthermore, there is
atitis, intestinal ischemia, or mesenteric artery no specific monitoring available, but increasing
thrombosis occur in about 3 % of the patients lactate and phosphate levels and persisting ther-
undergoing open-heart surgery. apy refractory metabolic acidosis should increase
The outcome of patients with severe gastroin- index of suspicion, prompting diagnostic testing.
testinal complications is associated with high The metabolic acidosis is usually accompanied by
mortality (Markewitz and Lante 2006). Most paralytic ileus and leukocytosis. The next step has
common problems involved gastrointestinal to be taken very quickly to confirm the diagnosis:
bleeding, amenable to endoscopic treatment mesenteric arteriogram, but if there is no possi-
options, and associated with much better out- bility for angiography, immediate laparotomy is
come. Ischemic bowel complications are associ- requested to diagnose and possibly treat the issue
Chapter 9 · Cardiac Surgical Intensive Care
239 9
with superior mesenteric or celiac artery bypass it can significantly reduce quality of life longterm
operation. If a nonocclusive ischemia is present, and does increase early mortality. Neurological
the administration of local vasodilatory drugs complications are classified into the following
such as papaverine is possible. Even with rapid categories:
decision taking, the mortality remains at 50 %. 55 Ischemic stroke
When symptoms are present for more then 12 h 55 ICU psychosis
survival is improbable. 55 Critical illness polyneuropathy
A subtype of patients with acute type A or B 55 Other neurological complications such as
aortic dissection may have or develop malperfu- neurocognitive deficits, neuropsychological
sion to celiac or mesenteric artery during the complications, and peripheral nerve inju-
index hospitalization (complicated aortic dis- ries
section). The malperfusion is usually due to
dynamic (dissection flap occluding the orifice of 9.5.7.2 Ischemic Stroke
the aortic branch vessels) or anatomic (dissec- The incidence of an ischemic stroke after cardiac
tion flap and hematoma in the orifice for the surgery is 2–10 %, being between 1.4 % and 3.8 % in
branch vessels). These patients have a treatment patients after coronary artery bypass grafting and
paradigm that has significantly changed in up to 10 % with multiple valve operations or total
recent years, requiring further details here arch replacement (Selim 2007). The patient
(Khoynezhad et al. 2009). typically has hemiparesis and aphasia depending
Prompt diagnosis and anti-impulsive treat- on the troubled brain hemisphere. CT scan will be
ment of patients with suspected complicated able to show the infarcted area 2 h after stroke. The
aortic dissection (CAD) is essential for usual causes for an ischemic stroke are ruptured
improved outcome. Originally, the minor only atherosclerotic plaques from the aorta, or calcific
available option used to be surgical fenestration aortic valve, thrombotic material from the left
of the membrane separating true and false atrium or the left ventricle, acute occlusion of a
lumen in order to perfuse both true and false high-grade-stenosed internal carotid artery. Next
lumen and reduce the pressure gradient that is to CT and duplex carotid, transesophageal echo-
the cause for the dynamic malperfusion. For cardiography is ordered to exclude emboli source
anatomic obstructions, mesenteric bypass oper- from the heart.
ation would be added. The endovascular treat- Therapeutic approach to thromboembolic
ment is, however, now the new standard to ischemic stroke is parenteral lysis with recombi-
promptly restore intestinal blood flow. Diagno- nant tissue-type plasminogen activator. However,
sis is confirmed with intravascular ultrasound this therapeutics cannot be used for most cardiac
and/or angiogram in the hybrid suite (See also surgical patients given the recent operation. In cer-
7 Chapter «Endovascular Surgical Therapy of Tho- tain circumstances, super-selective intra-arterial
racic and Thoracoabdominal Disease of the Aorta», lysis may be performed; however this has to be
Sect. 28.7.2). performed within 6 h of symptoms. In most cases
the stroke occurs in patients under sedation,
making any therapeutic intervention within that
«window» impossible. Therefore the therapy of a
9.5.7 Central and Peripheral stroke in cardiac surgical patients is mostly symp-
Nervous System tomatic:
55 Increase of the blood pressure
(160–180 mmHg systolic) as autoregulation
9.5.7.1 Incidence of Neurological in the stroke area may not be present any
Complications more
Depending on definitions and the diagnosing 55 Adequate oxygenation
physician, incidence of neurological complica- 55 Blood glucose level <150 mg/dl
tions may vary. As minor as it may appear to be, 55 Body temperature less than 99.5 °F (37.5 °C)
240 A. Markewitz et al.
While systemic heparinization is not recom- volume resuscitation necessary. In the individ-
mended, administration of aspirin (50–325 mg) ual case, especially in violent patients, adminis-
daily as secondary prophylaxis is established. tration of 2–10 mg haloperidol or clonidine IV
The most feared complication is brain edema (clonidine as an IV drug is not available in the
with increase of intracranial pressure. Therefore USA) as well as the newer generation of atypi-
the monitoring has to be extended to check for cal antipsychotic drugs may be considered.
signs of increased intracranial pressure and Only in extreme situations, endangering the
decreased consciousness. Recommended posi- patient and the CSICU staff, the sedation with
tioning is 30° elevated upper body, diuresis with propofol is recommended.
mannitol, or administration of short-acting To further differentiate and quantify psycho-
barbiturates, such as thiopental. While steroids sis, the Richmond Agitation-Sedation Scale
are no longer recommended, the use of hyper- (Sessler et al. 2002), in conjunction with the
ventilation is controversial and may be used as Confusion Assessment Method, may be employed
short-term therapy until other measures are (Ely et al. 2003, 2004; Sessler et al. 2002). The
initiated. The last option in reducing intracra- physical fixation of the patient should only be per-
nial pressure is surgical decompression with a formed in extreme situations; on the one hand, it
craniotomy. is restricted by law and needs clear documenta-
Furthermore, treatment of accompanying tion, and on the other hand the symptoms can be
seizures may be necessary, along with all accom- exacerbated by physical fixation.
panying problems of immobilized and uncon- It is not uncommon that patients with ICU
9 scious patients, such as aspiration pneumonia, psychosis develop an instable sternum requiring
thromboembolic complications, and decubitus surgical reintervention.
ulcer.
The prognosis of survivors with stroke after 9.5.7.4 Critical Illness Polyneuropathy
cardiac surgery is rather grim; only 47 % survive The cause of this rare complication is not well
the first 5 years, half of them with permanent def- understood, allowing for no treatment or pro-
icits requiring external support for daily living phylaxis. This polyneuropathy affects mainly
activities (Salazar et al. 2001). Early rehabilitation the motor axons, occurring most commonly
will help to restore the muscular coordination and after sepsis and polytrauma. A correlation with
improve functional outcome. systemic inflammatory reaction syndrome
(SIRS), multi-organ failure, and various drugs
9.5.7.3 ICU Psychosis is highly suggested. The main problem is to
ICU psychosis is a disorder in which hospitalized wean the patient from ventilator, as breathing
patients, especially in ICU, may experience anxi- muscles are commonly affected by critical ill-
ety, paranoia, hallucinations, disorientation, agita- ness polyneuropathy. After repeatedly failed
tion, and even violence against nurses and doctors. extubation attempts, the critical illness poly-
The condition has been formally defined as «acute neuropathy has to be taken into account. Early
brain syndrome involving impaired intellectual mortality is about 30 %, another 50 % of the
functioning which occurs in ICU patients.» ICU patients recover between 6 weeks and a year,
psychosis is a form of delirium or acute brain fail- while 20 % will have residual neuropathies
ure, and some organic factors including dehydra- (Kane and Dasta 2002).
tion, hypoxia, low cardiac output, infections, and
drugs may contribute to its development. The 9.5.7.5 Other Neurological
postoperative delirium occurs relatively often in Complications
elderly and remains clinically challenging for Other complications, such as neurocognitive defi-
many physicians, nursing staff, and the patient’s cits, neuropsychological complications, and
family. peripheral nerve lesions, are not specific to car-
Mild form of delirium does not require diac surgical patients. Peripheral nerve injuries
therapy, especially in elderly, or in case of sig- are potentially preventable complications during
nificant fluid loss (fever, sweating, diarrhea) or anesthesia, requiring extra attention while posi-
high ambient temperatures, making adequate tioning the patient.
Chapter 9 · Cardiac Surgical Intensive Care
241 9
9.5.8 cid-Base Balance and
A Negative base excess × 0.1
Electrolytes × body weight ( kg ) = ml tris buffer solution
The laboratory values and the common causes for A metabolic alkalosis is often the result of an
disorders of the acid-base balance are displayed in overtreated metabolic acidosis. A metabolic alka-
. Tables 9.18 and 9.19. losis can also cause a reduction of myocardial
Metabolic acidosis is the most relevant acid-base contractility and heart rhythm disturbances and
disorder in the CSICU, as the underlying causes can will worsen the oxygen tissue supply by shifting
be potentially fatal disorders. An insufficient oxygen the oxygen dissociation curve.
supply, caused by LCOS or mesenteric ischemia, ini- Electrolyte abnormalities will influence the
tiates a change from aerobe to anaerobe metabolism, heart function, especially the potassium level have
releasing more acids in the bloodstream. Main goal is the most clinical effect. The typical potassium
to treat the underlying cause of the acidosis, as sole level in the blood serum is 3.5–5 mmol/L. A low
treatment of the metabolic acidosis may not be level of potassium associated with metabolic alka-
enough. Clinical implications are reduced myocar- losis is accompanied by extrasystoles, tachycardia,
dial contractility and decreased effectiveness of cate- and ST interval decrease or a prolonged QT inter-
cholamines, clinically relevant in patients with pH val and will be treated by parenteral administra-
below 7.2. The amount of NaHCO3 to balance the tion of KCl. If hyperkalemia is present, high peaks
acidosis can be calculated with the following formula: of the T waves can be seen as well as enlargement
of QRS complex, branch blocks, arrhythmias, and
Negative base excess × 0.3
cardiac arrest. To lower the serum potassium
× body weight ( kg ) = mval NaHCO3 ( 8.4% ) level, the administration of diuretics, NaHCO3,
calcium, or glucose with insulin can be helpful.
Administrating large amounts of NaHCO3 results Diuretics will eliminate the potassium, but the
in iatrogenic hypernatremia. If more bicarbonate other attempts only provide a potassium shift
buffer is needed in case of a sodium level more from extracellular to intracellular. Another effec-
than 145 mmol/L, Tris (hydroxymethyl) amino- tive method to reduce potassium levels is Sodium
methane (THAM) may be used:
Metabolic acidosis Anaerobe metabolism Bicarbonate and correct the underlying cause
Metabolic alkalosis Overcorrected acidosis Acetazolamide and correct the underlying cause
242 A. Markewitz et al.
..Fig. 9.14 Differential
diagnosis and grading of
severity of inflammatory Presence of 2 or more of the following findings:
reactions without and with
infectious cause – Temperature: ≥38 C or ≤36 C (rectal, or urine bladder)
– Heart rate ≥90/min
– Tachypnea (breath rate > 20/min) or hyperventilation
(PaCO2 von <33 mmHg)
– Leukocytosis (>12,000/µl) or leukopenia (<4000/µl)
or >10 % immature precursor cells
SIRS
+ Infection
Sepsis
+ Organ dysfunction
9 Severe Sepsis
+ Hypotension
Septic Schock
Patient’s
24 hr observation, Yes No Suspicious for
hemodynamics
re-evaluation Sepsis
stable?
55 Renal dysfunction, diuresis <0.5 cc/kg/h for Since postoperative cardiac surgical infections
more than 2 h despite adequate volume load are associated with significantly increased mortal-
or serum creatinine climbed to twice nor- ity and cost, any signs of infection have to be eval-
mal uated carefully and promptly. If an infection is
55 Metabolic acidosis, negative base excess present, an appropriate and immediate treatment
exceeds −5 mmol/L or lactate increased more is recommended. This is especially true for
than 1.5 normal patients with high pretest probability of infection
55 Hypotension with systolic arterial pressure such as immune suppressed, HIV-infected, or
<90 mmHg or mean arterial pressure poorly controlled diabetic and obese patients
<70 mmHg for at least 1 h despite volume The search for infection after cardiac surgery
replacement and IV vasopressor therapy should focus on:
55 Pneumonia
kPractical steps 55 Urinary catheter infections
Fever is defined as a body temperature with more 55 Central vein catheter and peripheral line
than 100 °F (in Europe 38.3 °C = 100.9 °F). Initially infections
hypovolemia should be corrected that may cause 55 Surgical site infections
fever by itself. If the temperature is not dropping or
another fever is coming up, a high index of suspi- The first three types of infection occur usually
cion for brewing infection should be maintained. after the fifth postoperative day, whereas surgical
The next step is the search for the infection focus. site infections appear mostly after more than
244 A. Markewitz et al.
1–2 weeks depending on involved pathogen. These also, the more general term to describe these tis-
wound infections should be treated according to sue damages would be pressure sore. This com-
general surgical principles and accompanied by sys- pression of the tissue reduces the blood supply,
temic antibiotics. and the ensuing hypoxia may damage the nerves
If bedside investigations, such as chest X-ray, in this area within a few hours. If the patient can-
urine, and respiratory cultures, remain negative not change the position, a permanent skin dam-
for an infection, an antibiotic therapy should age will occur.
be started even without knowing the infectious Besides the hypoxia, compromised venous
organism. The typical organism described in the drainage is also important in accumulation of acid
literature may be helpful, but it is more useful to metabolism products in the skin. Furthermore,
know the hospital common organisms and their the acidic environment initiates vasodilatation
resistances. Furthermore, the initial antibiotic with hyperemia, with subsequent exudate and
should be wide spectrum, reducing to more nar- transudate with possible tissue edema and blisters,
row antibiotics once sensitivities are back. If an and microthrombotic vascular changes. This acid
infection focus is identified, removal of the focus environment stimulates usually the ambulatory
with or without surgical excision should be per- human to move a little bit, preventing thereby any
formed immediately. tissue damage in healthy individuals.
. Figure 9.15 illustrates our algorithm to eval- Frequently the decubitus is infected, prolong-
uate postoperative fever. ing the healing process. Body parts without a
If the infection progresses to a full-blown sep- proper muscle or fat tissue are most vulnerable to
9 sis, the prognosis will be significantly worse. decubitus.
These four strategies have shown to improve The decubitus can be categorized in different
severe sepsis or septic shock outcomes: depths and sizes and is commonly classified as
55 Early support for normalized hemodynamic follows:
function (Rivers et al. 2001) Stage I is the most superficial and recoverable,
55 Intensified insulin therapy (van den Berghe characterized by nonblanchable redness that
et al. 2001) does not subside after finger pressure is
55 Administration of human recombinant acti- relieved. This stage is visually similar to reac-
vated protein C (APC) (Bernard et al. 2001). tive hyperemia seen in the skin after prolonged
55 Widening of antibiotic therapy including anti- application of pressure. Stage I pressure ulcers
fungal or antiviral in specific patient cohort can be distinguished from reactive hyperemia
in two ways: (a) reactive hyperemia resolves
In the meantime, target levels for tight glyce- itself within three-fourth of the time pressure
mic control have been changed (Haga et al. 2011). was applied and (b) reactive hyperemia
Activated protein C (APC) has been removed from blanches when pressure is applied, whereas a
the market due to questionable benefit (Marti- Stage I pressure ulcer does not. The skin may
Carvajal et al. 2012). This topic is rather compre- be hotter or cooler than normal, have an odd
hensive and complex and cannot be sufficiently texture, or be painful. Although easy to iden-
handled in this chapter. Therefore, further detailed tify on a light-skinned patient, ulcers on
reading is suggested (Dellinger et al. 2013). darker-skinned individuals may show up as
shades of purple or blue in comparison to
lighter skin tone.
9.5.10 Decubitus Stage II is damage to the epidermis extending
into, but no deeper than, the dermis. In this
A decubitus is tissue damage caused by prolonged stage, the ulcer may be referred to as a blister
compression (Maklebust 2005; Thompson 2005). or abrasion that can be infected.
In clinical practice, decubitus is the short form for Stage III involves the full-thickness damage of the
the historical term decubitus ulcer. As the word skin and may extend into the subcutaneous tis-
decubitus in Latin means lying down, although sue layer. This layer has a relatively poor blood
these damages can be caused in other positions supply and can be more difficult to heal. At this
Chapter 9 · Cardiac Surgical Intensive Care
245 9
stage, there may be undermining tissue dam- HIT is a prothrombotic disease triggered by
age that makes the decubitus much larger than an immune reaction against the platelet factor 4
it may seem on the skin level. heparin complex. The clinical signs are drop of
Stage IV is the deepest, extending into the muscle, the platelet count more than 50 % and clinical
tendon, or even bone. thrombosis. Actual HIT tests are sensitive but less
The decubitus prophylaxis is the best therapy. The specific. Nearly half of cardiac surgery patients
treatment of a fulminant decubitus is very com- develop antibodies to heparin, whereas only
plex and requires plenty of resources. For prophy- 1–2 % will have a clinical relevant HIT. Functional
laxis, the following aspects are very important: HIT tests, such as serotonin-released assays, are
55 Tissue-protective patient exercise, position- more accurate by measuring the platelet activa-
ing, and transfer tion caused by heparin antibodies.
55 Routine use of softgel or air-filled mattresses The prompt therapy is to stop any exposure to
55 Continuous performance of prophylactic heparin and to change the anticoagulation to
maneuvers alternatives, such as lepirudin. Additionally, plate-
let infusions may have to be administered to
Local therapy of decubitus contains: remove the existing antibodies. . Figure 9.16
55 Debridement summarizes the approach to HIT.
55 Treatment of infection
55 Moist wound treatment
55 Wound conditioning 9.5.12 Endocrine System
55 Hyperbaric oxygen therapy and VAC wound
treatment After intensified insulin therapy to achieve blood
glucose levels less than 110 mg/dl had shown to
The causal wound therapy is: decrease mortality and the incidence of postopera-
55 Complete decompression of the damaged tis- tive infections, intensified insulin therapy was on
sue protocols of most CSICUs (van den Berghe et al.
55 Improvement of nutrition 2001). Meanwhile, it could be demonstrated that
55 Adequate pain therapy moderate glycemic control with blood glucose lev-
55 Improving the general functional condition els less than 180 mg/dl is more advantageous in
patients after cardiac surgery (Haga et al. 2011;
Stage II decubitus ulcer or higher is a sign of Bhamidipati et al. 2011). With both protocols, the
poor nursing and patient care. It should be inves- incidence of hypoglycemia is a concern, being
tigated if there was omission or human error asso- increased up to sixfold. Furthermore, low potassium
ciated with it. The incidence for decubitus on any level can occur, contributing to dysrhythmias. A
ICU should be less than 1 % per year. close monitoring of blood glucose level and potas-
sium should be ensured through close monitoring.
In critically sick patients, especially those on high-
9.5.11 Disturbances dose catecholamines, adequate blood glucose levels
of the Coagulation may be much harder to achieve.
System T3 levels in patients after open-heart surgery
are reduced. However, T3 supplement has failed
Most common problems with coagulation sys- to demonstrate any improved outcome (Ronald
tem after cardiac surgery have been discussed and Dunning 2006). Same principle applies to
(paragraph 7 Sect. 9.5.3). In this section, postoperative cortisol levels, making supplemen-
heparin-induced thrombocytopenia (HIT) will tal steroid administration useless and possibly
be briefly described (Napolitano et al. 2006; deleterious due to effects on postoperative wound
Selleng et al. 2007). The incidence of HIT com- healing. In patients requiring perioperative ste-
plications is 1–2 % after cardiac surgery, being roids, such as adrenal insufficiency, vitamin A
associated with an increased mortality (Kerendi administration may counteract steroid effects on
et al. 2007). wound healing.
246 A. Markewitz et al.
No Yes
HIT possible
9 No
HIT not probable
No
Functional HIT test
Continue heparin therapy Positive ?
Yes
..Fig. 9.16 Diagnostic algorithm for heparin-induced thrombocytopenia (HIT). ELISA enzyme-linked immunosorbent
assay (Modified according to Selleng et al. (2007))
9.6 Scores and Quality Assurance have been developed, also. Further scores are
available in the internet (Société Française
Both topics are subject for numerous publica- d’Anesthésie et de Réanimation 2008). In Ger-
tions of all kind. Their significance for clinical many, currently a combination of a modified
intensive care treatment in cardiac surgery has SAPS II score and TISS 28 score is employed to
yet to be shown. There are, however, in various evaluate the expenditure of an individual treat-
versions a vast number of intensive care medi- ment course to be used for reimbursement—an
cine scores available (see also 7 Chap. 2, «Risk example of how a potentially useful clinical
Scores in Cardiac Surgery»), which all have been instrument is used for purposes other than orig-
developed for different purposes, for example, inally intended.
APACHE (Acute Physiology and Chronic Quality assurance in intensive care medicine
Health Evaluation), SAPS (Simplified Acute currently is only done on a voluntary basis.
Physiology Score), or SOFA (Sequential Organ However, any intensive care unit should check its
Failure Assessment). To assess the economic own quality of treatment at least by a minimal
aspects of ICU treatment, scores like TISS 28 data set. An example for some parameters is given
(Therapeutic Intervention Scoring System 28) in . Table 9.20.
Chapter 9 · Cardiac Surgical Intensive Care
247 9
Bellomo R, Ronco C, Kellum JA, Mehta RL, Palevsky P (2004)
..Table 9.20 General guidelines for quality Acute renal failure—definition, outcome measures,
improvement in ICU with goals and thresholds, animal models, fluid therapy and information technol-
which should not be surpassed ogy needs: the Second International Consensus
Conference of the Acute Dialysis Quality Initiative
Parameter Goal Threshold (ADQI) Group. Crit Care 8:R204–R212
Bernard GR, Vincent JL, Laterre PF et al. (2001) Efficacy and
% of patients requiring early <3 5
safety of recombinant human activated protein C for
readmission to ICU
severe sepsis. N Engl J Med 344:699–709
% of patients with de novo <1 2 Bhamidipati CM, LaPar DJ, Stukenborg GJ, Morrison CC,
decubitus (stage II and Kern JA, Kron IL, Ailawadi G (2011) Superiority of mod-
higher) erate control of hyperglycemia to tight control in
patients undergoing coronary artery bypass grafting.
% post-op extubation >75 50 J Thorac Cardiovasc Surg 141:543–551
within 6 h Buendgens L, Bruensing J, Matthes M et al. (2014)
Administration of proton pump inhibitors in critically
% reintubation <3 5
ill medical patients is associated with increased risk of
developing Clostridium difficile-associated diarrhea.
J Crit Care 29:696e11–696e15
Cabrini L, Zangrillo A, Landoni G (2015) Preventive and
References therapeutic noninvasive ventilation in cardiovascular
surgery. Curr Opin Anaesthesiol 28:67–72
Alhazzani W, Alenezi F, Jaeschke RZ et al. (2013) Proton Carl M, Alms A, Braun J, Dongas A, Erb J, Goetz A, Goepfert
pump inhibitors versus histamine 2 receptor antago- M, Gogarten W, Grosse J, Heller AR, Heringlake M,
nists for stress ulcer prophylaxis in critically ill patients: Kastrup M, Kroener A, Loer SA, Marggraf G, Markewitz
a systematic review and meta-analysis. Crit Care Med A, Reuter D, Schmitt DV, Schirmer U, Wiesenack C,
41:693–705 Zwissler B, Spies C. (2010) S3 guidelines for intensive
Ansell J, Hirsh J, Poller L, Bussey H, Jacobson A, Hylek E care in cardiac surgery patients: hemodynamic moni-
(2004) The pharmacology and management of the toring and cardiocirculatory system. Ger Med Sci. 8:
vitamin K antagonists: the seventh ACCP conference Doc12. doi:10.3205/000101. Online: 7 http://www.
on antithrombotic and thrombolytic therapy. Chest egms.de/static/en/journals/gms/2010–8/000101.
126(3 Suppl):204S–233S shtml. Last access 2014–09–18
Antman EM, Anbe DT, Armstrong PW et al. (2004) ACC/ Carrel T, Englberger L, Mohacsi P, Neidhart P, Schmidli J
AHA guidelines for the management of patients with (2000) Low systemic vascular resistance after cardio-
ST-elevation myocardial infarction – executive sum- pulmonary bypass: incidence, etiology, and clinical
mary: a report of the American College of Cardiology/ importance. J Card Surg 15:347–353
American Heart Association Task Force on Practice Chapman SA, Irwin ED, Beal AL, Kulinski NM, Hutson KE,
Guidelines (Writing Committee to Revise the 1999 Thorson MA (2011) Prothrombin complex concentrate ver-
Guidelines for the Management of Patients With Acute sus standard therapies for INR reversal in trauma patients
Myocardial Infarction). Circulation 110: 588–636 receiving warfarin. Ann Pharmacother 45:869–875
ARDS Definition Task Force, Ranieri VM, Rubenfeld GD, Christenson JT, Cohen M, Ferguson JJ et al. (2002) Trends in
Thompson BT et al. (2012) Acute respiratory distress intraaortic balloon counterpulsation complications
syndrome: the Berlin definition. JAMA 307:2526–2533 and outcomes in cardiac surgery. Ann Thorac Surg
Aseeri M, Schroeder T, Kramer J, Zackula R (2008) Gastric acid 74:1086–1091
suppression by proton pump inhibitors as a risk factor Ciaglia P, Firsching R, Syniec C. Elective percutaneous dila-
for clostridium difficile-associated diarrhea in hospital- tational tracheostomy: a new simple bedsideproce-
ized patients. Am J Gastroenterol 103:2308–2313 dure; preliminary report. Chest. 1985;87(6):715–719
Baglin TP, Keeling DM, Watson HG; for British Committee De Leyn P, Bedert L, Delcroix M et al. (2007) Tracheotomy:
for Standards in Haematology (2006) Guidelines on clinical review and guidelines. Eur J Cardiothorac Surg
oral anticoagulation (warfarin): 3rd edition – 2005 32:412–421
update. Br J Haematol 132:277–285 Dellinger RP, Levy MM, Rhodes A et al. (2013) Surviving
Baglin TP, Cousins D, Keeling DM, Perry DG, Watson HG Sepsis Campaign Guidelines Committee including the
(2007) Recommendations from the British Committee Pediatric Subgroup. Surviving sepsis campaign: inter-
for Standards in Haematology and National Patient national guidelines for management of severe sepsis
Safety Agency. Br J Haematol 136:26–29. Erratum. Br J and septic shock: 2012. Crit Care Med 41:580–637
Haematol 136:681–681 Dunning J, Prendergast B (2003) Which patients would
Baskett RJF, Ghali WA, Maitland A, Hirsch GM (2002) The benefit from an intra-aortic balloon pump prior to
intraaortic balloon pump in cardiac surgery. Ann cardiac surgery? Interact Cardiovasc Thorac Surg
Thorac Surg 74:1276–1287 2:416–419
Bein T, Philipp A, Zimmermann M, Mueller T, Schmid FX Dunning J, Treasure T, Versteegh M, Nashef SAM on behalf
(2007) Extrakorporale Lungenunterstutzung. Dtsch of the EACTS Audit and Guidelines Committee (2006)
Med Wochenschr 132:488–491 Guidelines on the prevention and management of de
248 A. Markewitz et al.
novo atrial fibrillation after cardiac and thoracic sur- Jin R, Grunkemeier GL, Brown JR, Furnary AP (2008)
gery. Eur J Cardiothorac Surg 30: 852–872 Estimated glomerular filtration rate and renal func-
Edwards FH, Engelman RM, Houck P, Shahian DM, Bridges tion. Ann Thorac Surg 86(1):1–3
CR (2006) The Society of Thoracic Surgeons practice Kane SL, Dasta JF (2002) Clinical outcomes of critical illness
guideline series: antibiotic prophylaxis in cardiac sur- polyneuropathy. Pharmacotherapy 22:373–379
gery, part I: duration. Ann Thorac Surg 81:397–404 Kerendi F, Thourani VH, Puskas JD et al. (2007) Impact of
Egi M, Bellomo R, Langenberg C et al. (2007) Selecting a heparin-induced thrombocytopenia on postoperative
vasopressor drug for vasoplegic shock after adult car- outcomes after cardiac surgery. Ann Thorac Surg
diac surgery: a systematic literature review. Ann 84:1548–1553
Thorac Surg 83:715–723 Khoynezhad A (2007) Antihypertensive therapy in a
Ely EW, Truman B, Shintani A et al. (2003) Monitoring seda- patient with chronic type B and de novo type A aortic
tion status over time in ICU patients: the reliability and dissection: case study. Crit Care Clin 23:29–38
validity of the Richmond Agitation Sedation Scale Khoynezhad A, Plestis KA (2006) Managing emergency
(RASS). JAMA 289:2983–2991 hypertension in aortic dissection and aortic aneurysm
Ely EW, Shintani A, Truman B et al. (2004) Delirium as a pre- surgery. J Card Surg 21:S3–S7
dictor of mortality in mechanically ventilated patients Khoynezhad A, Donayre CE, Kopchok G, Walot I, Omari BO,
in the intensive care unit. JAMA 291:1753–1762 White RA (2009) Mid-term results of endovascular
Engoren MC, Habib RH, Zacharias A et al. (2002) Effect of treatment of complicated acute type B aortic dissec-
blood transfusion on long-term survival after cardiac tion. J Thorac Cardiovasc Surg 138:625–629; discus-
operation. Ann Thorac Surg 74:1180–1186 sion: 629–31
Erbel R, Alfonso F, Boileau C, Dirsch O et al. (2001) Diagnosis Kluger Y, Riou B, Rossaint R et al. (2007) Safety of rFVIIa in
and management of aortic dissection. Eur Heart hemodynamically unstable polytrauma patients with
J 22:1642–1681 traumatic brain injury: post hoc analysis of 30
Ferraris VA, Brown JR, Despotis GJ et al. (2011) 2011 update patients from a prospective, randomized, placebo-
9 to the Society of Thoracic Surgeons and the Society of controlled, double-blind clinical trial. Crit Care
Cardiovascular Anesthesiologists blood conservation 11(4):R85
clinical practice guidelines. Ann Thorac Surg 91:944– Kreymann KG, Berger MM, Deutz NEP et al. (2006) ESPEN
982 guidelines on enteral nutrition: intensive care. Clin
Flynn MJ, McComb JM, Darh JH (2005) Temporary left ven- Nutr 25:210–223
tricular pacing improves haemodynamic performance in Kuitunen A, Vento A, Suojaranta-Ylinen R, Pettila V (2006)
patients requiring epicardial pacing post cardiac surgery. Acute renal failure after cardiac surgery: evaluation
Eur J Cardiothorac Surg 28:250–253 of the RIFLE classification. Ann Thorac Surg 81:542–
Garwood S (2004) Renal insufficiency after cardiac surgery. 546
Semin Cardiothorac Vasc Anesth 8:227–241 Laheij RJ, Sturkenboom MC, Hassing RJ et al. (2004) Risk of
Giakoumidakis K, Baltopoulos GI, Charitos C, Patelarou E, community-acquired pneumonia and use of gastric
Galanis P, Brokalaki H (2011) Risk factors for prolonged acid-suppressive drugs. JAMA 292:1955–1960
stay in cardiac surgery intensive care units. Nurs Crit Care Lassnigg A, Donner E, Grubhofer G, Presterl E, Druml W,
16:243–251 Hiesmayr M (2000) Lack of renoprotective effects of
Guarracino F, Ambrosino N (2011) Non invasive ventilation in dopamine and furosemide during cardiac surgery. J
cardio-surgical patients. Minerva Anestesiol 77:734–741 Am Soc Nephrol 11:97–104
Haga KK, McClymont KL, Clarke S, Grounds RS, Ng KY, Leonard CE, Freeman CP, Newcomb CW et al. (2012) Proton
Glyde DW, Loveless RJ, Carter GH, Alston RP (2011) pump inhibitors and traditional nonsteroidal anti-
The effect of tight glycaemic control, during and inflammatory drugs and the risk of acute interstitial
after cardiac surgery, on patient mortality and mor- nephritis and acute kidney injury. Pharmacoepidemiol
bidity: a systematic review and meta-analysis. J Drug Saf 21:1155–1172
Cardiothorac Surg 6:3 Levin RL, Degrange MA, Bruno GF et al. (2004) Methylene
Hemmila MR, Napolitano LM (2006) Severe respiratory failure: blue reduces mortality and morbidity in vasoplegic
advanced treatment options. Crit Care Med patients after cardiac surgery. Ann Thorac Surg 77:496–
34(Suppl):S278–S290 499
Hill NS, Brennan J, Garpestad E, Nava S (2007) Noninvasive Ley SJ (2015) Standards for resuscitation after cardiac sur-
ventilation in acute respiratory failure. Crit Care Med gery. Crit Care Nurse 35(2):30–37. doi:10.4037/
35:2402–2407 ccn2015652; quiz 38
Hoste EAJ, Clermont G, Kersten A et al. (2006) RIFLE criteria for Mackay JH, Powell SJ, Osgathorp J, Rozario CJ (2002) Six-
acute kidney injury is associated with hospital mortality year prospective audit of chest reopening after car-
in critically ill patients: a cohort analysis. Crit Care 10:R73– diac arrest. Eur J Cardiothorac Surg 22:421–425
R82 MacLaren R, Campbell J (2014) Cost-effectiveness of hista-
Jennett B, Teasdale G, Galbraith S, Pickard J, Grant H, mine receptor-2 antagonist versus proton pump
Braakman R, Avezaat C, Maas A, Minderhoud J, Vecht CJ, inhibitor for stress ulcer prophylaxis in critically Ill
Heiden J, Small R, Caton W, Kurze T (1977) Severe head patients. Crit Care Med 42:809–815
injuries in three countries. J Neurol Neurosurg Psychiatry Maklebust J (2005) Pressure ulcers: the great insult. Nurs
40:291–298 Clin North Am 40:365–389
Chapter 9 · Cardiac Surgical Intensive Care
249 9
Malhotra A (2007) Low-tidal-volume ventilation in the Ramnarine IR, Grayson AD, Dihmis WC, Mediratta NK, Fabri
acute respiratory distress syndrome. N Engl J Med BM, Chalmers JAC (2005) Timing of intra-aortic bal-
357:1113–1120 loon pump support and 1-year survival. Eur J
Mangano DT; for the Multicenter Study of Perioperative Cardiothorac Surg 27:887–892
Ischemia Research Group (2002) Aspirin and mortality Ramsay MA, Savege TM, Simpson BR, Goodwin R (1974)
from coronary bypass surgery. N Engl J Med 347:1309– Controlled sedation with alphaxalone-alphadolone.
1317 Br Med J 2(5920):656–659
Markewitz A, Lante W (2006) Organ failure in cardiosurgi- Rivers E, Nguyen B, Havstad S et al. (2001) Early goal-
cal intensive care medicine. Dtsch Med Wschr directed therapy in the treatment of severe sepsis and
131:2485–2488 septic shock. N Engl J Med 345:1368–1377
Martí-Carvajal AJ, Solà I, Lathyris D, Cardona AF (2012) Robicsek F, Hollemann JH, Roush TS, Skipper ER, Robicsek
Human recombinant activated protein C for severe SA, Lyons M (2003) Peri-operative intraaortic balloon
sepsis. Cochrane Database Syst Rev (3):CD004388 assist, decreasing complications to the minimum.
McKeown PP, Gutterman D (2005) American College of Thorac Cardiovasc Surg 51:115–125
Chest Physician guidelines for the prevention and Ronald A, Dunning J (2006) Does perioperative thyroxine
management of postoperative atrial fibrillation after have a role during adult cardiac surgery? Interact
cardiac surgery. Chest 128:1S–5S Cardiovasc Thorac Surg 5:166–178
Mehta RL, Pascual MT, Soroko S, Chertow GM (2002) Salazar JD, Wityk RJ, Grega MA et al. (2001) Stroke after car-
Diuretics, mortality, and nonrecovery of renal function diac surgery: short- and long-term out-comes. Ann
in acute renal failure. JAMA 288:2547–2553 Thorac Surg 72:1195–1202
Mehta RH, Grab JD, O’Brien SM, for the Society of Thoracic Sarkar M, Hennessy S, Yang YX (2008) Proton-pump inhibi-
Surgeons National Cardiac Surgery Database tor use and the risk for community-acquired pneumo-
Investigators et al. (2006) Bedside tool for predicting nia. Ann Intern Med 149:391–398
the risk of postoperative dialysis in patients undergo- Sealy AJE, Christou NV (2000) Multiple organ dysfunction
ing cardiac surgery. Circulation 114:2208–2216 syndrome: exploring the paradigm of complex nonlin-
Mehta RL, Kellum JA, Shah SV, Molitoris BA, Ronco C, ear systems. Crit Care Med 28:2193–2200
Warnock DG, Levin A, on behalf of the participants Selim M (2007) Perioperative stroke. N Engl J Med 356:706–
(2007) Acute Kidney Injury Network (AKIN): report of 713
an initiative to improve outcomes in acute kidney Selleng K, Warketin TE, Greinacher A (2007) Heparin-
injury. Crit Care 11:R31 induced thrombocytopenia in intensive care patients.
Mehta RH, Sheng S, O’Brien SM et al. (2009) Reoperation Crit Care Med 35:1165–1176
for bleeding in patients undergoing coronary artery Sessler CN, Gosnell M, Grap MJ et al. (2002) The Richmond
bypass surgery: incidence, risk factors, time trends, Agitation-Sedation scale: validity and reliability in
and outcomes. Circ Cardiovasc Qual Outcomes adult intensive care patients. Am J Respir Crit Care
2:583–590 Med 166:1338–1344
Murthy SC, Arroliga AC, Walts PA et al. (2007) Ventilatory Société Française d’Anesthésie et de Réanimation (2008)
dependency after cardiovascular surgery. J Thorac Scoring systems for ICU and surgical patients. 7 http://
Cardiovasc Surg 134: 484–490 sfar.org/t/spip.php?article60
Napolitano LM, Warketin TE, AlMahameed A, Nasraway SA Spotnitz HM (2005) Optimizing temporary perioperative
(2006) Heparin-induced thrombocytopenia in the criti- cardiac pacing. J Thorac Cardiovasc Surg 129:5–8
cal care setting: Diagnosis and management. Crit Care St. André AC, DelRossi A (2005) Hemodynamic manage-
Med 34: 2898–2911 ment of patients in the first 24 hours after cardiac sur-
National Kidney Foundation (2002) K/DOQI clinical prac- gery. Crit Care Med 33:2082–2093
tice guidelines for chronic kidney disease: evaluation, Teasdale G, Jennett B (1974) Assessment of coma and
classification, and stratification. Am J Kidney Dis 39(2 impaired consciousness. A practical scale. Lancet
Suppl 1):S1–S266, Online STS Risk Calculator 2:81–84
Nolana JP, Soarb J, Zidemanc DA, on behalf of the ERC The Acute Respiratory Distress Syndrome Network (2000)
Guidelines Writing Group et al. (2010) European resus- Ventilation with lower tidal volumes as compared with
citation council guidelines for resuscitation 2010. traditional tidal volumes for acute lung injury and the
Resuscitation 81:1219–1276 acute respiratory distress syndrome. N Engl J Med 342:
Polonen P, Ruokonen E, Hippelainen M, Poyhonen M, 1301–1308
Takala J (2000) A prospective, randomized study of Thompson D (2005) A critical review of the literature on
goal-oriented hemodynamic therapy in cardiac surgi- pressure ulcer aetiology. J Wound Care 14: 87–90
cal patients. Anesth Analg 90:1052–1059 Tran P, Khoynezhad A (2009) Current medical manage-
Preiser JC, van Zanten AR, Berger MM, Biolo G, Casaer MP, ment of type B aortic dissection. Vasc Health Risk
Doig GS, Griffiths RD, Heyland DK, Hiesmayr M, Manag 5(1):53–63
Iapichino G, Laviano A, Pichard C, Singer P, Van den Truhlár A, Deakin CD, Soard J, et al; on behalf of the Cardiac
Berghe G, Wernerman J, Wischmeyer P, Vincent JL Arrest in Special Circumstances Section Collaborators
(2015) Metabolic and nutritional support of critically (2015) European Resuscitation Council Guidelines for
ill patients: consensus and controversies. Crit Care Resuscitation 2015 Section 4. Cardiac arrest in special
19:35. doi:10.1186/s13054-015-0737-8 circumstances. Resuscitation 95:148–201
250 A. Markewitz et al.
Tsai TT, Nienaber CA, Eagle KA (2005) Acute aortic syn- Whitson BA, Huddleston SJ, Savik K, Shumway SJ (2007)
dromes. Circulation 112:3802–3813 Bloodless cardiac surgery is associated with
van den Berghe G, Wouters P, Weekers F et al. (2001) decreased morbidity and mortality. J Card Surg
Intensive insulin therapy in critically ill patients. N Engl 22:373–378
J Med 345: 1359–1367 Yang YX, Lewis JD, Epstein S, Metz DC (2006) Long-term
Vargas-Hein O, Birnbaum J, Wernecke K, England M, proton pump inhibitor therapy and risk of hip fracture.
Konertz W, Spies C (2006) Prolonged intensive care JAMA 296:2947–2953
unit stay in cardiac surgery: risk factors and long-term- Young D, Harrison DA, Cuthbertson BH, Rowan K, TracMan
survival. Ann Thorac Surg 81:880–885 Collaborators (2013) Effect of early vs late tracheos-
Warren O, Mandal K, Hadjianastassiou V et al. (2007) tomy placement on survival in patients receiving
Recombinant activated factor VII in cardiac surgery: a mechanical ventilation: the TracMan randomized
systematic review. Ann Thorac Surg 83:707–714 trial. JAMA 309(20):2121–2129. doi:10.1001/
Wheeler AP, Bernard GB (2007) Acute lung injury and the jama.2013.5154
acute respiratory distress syndrome: a clinical review.
Lancet 369:1553–1564
9
251 10
10.10 R
espiratory Dysfunction and Mechanical
Ventilation – 269
10.10.1 I ndication and Ventilation Nodes – 269
10.10.2 Weaning and Extubation Readiness – 271
10.12 K
idney Injury and Renal
Replacement Therapy – 274
10.12.1 D efinition, Risk Factors, and Staging
of Acute Kidney Injury (AKI) – 274
10.12.2 Management of AKI – 274
10.14 P
erioperative Neurologic Injury
and Neurologic Monitoring – 276
10.14.1 P reoperative Neurological Abnormalities
and Risk Factors – 277
10.14.2 Peri-, Intra-, and Postoperative Neurologic Injury – 277
10.14.3 Neuromonitoring Techniques – 278
References – 281
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
253 10
10.1 Introduction 55 Treatment of pulmonary hypertension
55 Respiratory modalities such as high-fre-
Advances in outcomes for pediatric and congenital quency oscillatory ventilation
cardiac surgery cannot be attributed to advances in 55 Postoperative cardiac catheterization tech-
cardiac surgery alone; they rather have to be also niques with the option of using catheter
attributed to advances in pediatric cardiology, pedi- interventional procedures
atric cardiac anesthesia, and last but not least, postop- 55 Hybrid techniques which combine catheter
erative pediatric cardiac intensive care management. intervention and surgery already as a primary
Intraoperative care including myocardial protection, treatment option (hybrid operating room or
deep hypothermic cardiac arrest, ultrafiltration, and surgery in the cath lab).
technical advances has contributed to an increasing 55 Treatment for cardiac dysrhythmia and pac-
postoperative survival rate especially for infants with ing including epicardial/transvenous leads,
complex congenital heart defects. The overall surgi- resynchronization therapy, and intracardiac
cal 30-day mortality in childhood decreased to 2–4 % defibrillator ICD
and, today, nearly 90 % of the children will reach 55 Management strategies for patients with func-
adulthood. With even further reduction of the tionally univentricular hearts such as hypo-
immediate postoperative surgical mortality, the focus plastic left heart syndrome and balancing
is more directed on postoperative morbidity, sequelae systemic and pulmonary vascular resistance
of treatment, and residual defects that might com-
promise quality of life. These management strategies are supported by
While primary corrective surgery for most complex perioperative monitoring with the avail-
biventricular congenital heart defects and neonatal ability of intraoperative transesophageal echocar-
palliation for complex multistage definitive surgery diography even in neonates and routine sometimes
for univentricular lesions has been established, frequent transthoracic echocardiography in the
with the development of combined surgical/cathe- PCICU. Hemodynamics and tissue oxygenation
ter interventional procedures, new therapeutic are closely assessed in real time via intravascular
fields opened. Treatment now starts on one end of catheters, sometimes with direct measurements of
the spectrum with low and very low birthweight cardiac output. Thereby, significant complications
neonates and widens to an increasing number of (pleural and pericardial effusions, impaired ven-
adult patients with congenital heart defects palli- tricular function) and/or residual lesions can be
ated or operated upon during childhood requiring picked up even before they lead to deleterious
further surgical or catheter interventional therapy. consequences. Neuromonitoring includes near-
Therefore, management concepts for patients infrared spectroscopy, electroencephalogram, and
with congenital heart defects require interdisciplinary transcranial Doppler ultrasound whenever indi-
care including pediatric and congenital cardiac sur- cated with the additional option of cranial mag-
gery, cardiac anesthesia, pediatric cardiac intensive netic resonance imaging.
care, pediatric and adult cardiology, and neonatology. Postoperative morbidity and mortality have a
Ideally, management starts with fetal echocardiogra- close relation to postoperative complications.
phy for prenatal counseling. Other subspecialties such Therapeutic strategies should be developed to
as pulmonology, neurology, hematology, and/or hep- anticipate or to avoid adverse events. The early
atology are important to manage interacting prob- identification of low cardiac output syndrome is
lems with other organ systems. one of the most important goals in the care for
Over the last years, pediatric cardiac intensive children after cardiac surgery. An essential ele-
care has developed new management strategies for: ment of successful pediatric intensive care is mul-
55 Treatment of heart failure—including new tidisciplinary collaboration with standard
pharmacologic agents, mechanical cardiopul- treatment concepts, however, with decisions indi-
monary support with extracorporeal mem- vidualized based on close clinical assessment.
brane oxygenation and ventricular assist Continuum of service and a gradual transition in
devices, and heart and heart-lung transplan- small steps during recovery and weaning are
tation mandatory (Chang 2005).
254 R. Kaulitz et al.
Oxygen consumption VO 2
Qs =
systemic arterial oxygen content − mixed venous oxygen content
Oxygen consumption VO 2
Qp =
pulmonary venous oxygen content − pulmonary arterial oxygen content
Intracardiac shunt calculation results from the The calculation of the vascular resistance R is
ratio Qp/Qs. made from the relation of mean pressure changes to
blood flow across systemic or pulmonary circuit.
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
255 10
mean aortic pressure − mean right atrial pressure
Rs =
Qs
mean pulmonary artery pressure − mean left atrial pressure
Rp =
Qp
..Table 10.1 Causes for changes of right and/or left atrial and pulmonary artery pressures
Bleeding
Polyuria
Tamponade
Dysrhythmia
Hypoventilation/respiratory acidosis
Tamponade
Dysrhythmia
Fontan procedure. Vasoactive drugs are admin- are most often administered, sometimes in combi-
istered to reduce preload by increasing vascular nation with epinephrine. Increased pulmonary
capacity. vascular resistance requires sufficient mechanical
Elevation of the systemic and pulmonary vas- ventilation, mild hyperventilation, and temporar-
cular resistance can be observed in patients with ily inhaled nitric oxide; sometimes prostacyclin
acidosis, hypoxemia, and inadequate analgesia and derivates are added, also. In patients with LCOS
sedation. In patients with LCOS in the presence of and low systemic vascular resistance, catechol-
elevated systemic vascular resistance, inodilators amine support with dopamine or epinephrine
258 R. Kaulitz et al.
Dobutamine 0–1 3 1 0
Epinephrine 3 3 3 0
Norepinephrine 3 3 1 0
decreases required vasoactive support, and Suominen et al. 2011; Momeni et al. 2011).
improves renal perfusion and fluid balance Levosimendan is at least as efficacious as milri-
(Mackie et al. 2005). none and was administered in combination with
other vasoactive drugs as catecholamines, vaso-
10.3.2.4 New Class of Agents pressin, or inhaled nitric oxide (Follath et al.
As stated above, ideal strategies to treat LCOS 2005; Stocker et al. 2007; Suominen et al. 2011).
include: Levosimendan is currently not available in the
55 Systemic venous and arterial vasodilation USA for clinical use.
55 Maintenance or decrease of myocardial oxy-
gen consumption
55 Control of heart rate, avoiding arrhythmia 10.4 Systemic Inflammatory
55 Control of neurohumoral activation Response Syndrome (SIRS)
55 Improving renal perfusion and fluid balance
10.4.1 Clinical Manifestations of SIRS
Basic treatment strategies with inotropic sup-
port (i.e., dobutamine and milrinone) may induce Both cardiopulmonary bypass and deep hypother-
or sustain elevated myocardial oxygen consump- mia with or without circulatory arrest may induce
tion and are potentially arrhythmogenic (Moffett an inflammatory process mediated by cellular acti-
and Chang 2006). vation and activation of pro- and anti-inflammatory
Nesiritide, a recombinant form of B-type cytokines and mediators. The inflammatory cas-
natriuretic peptide, has a vasodilating effect on cade and activation of leucocytes, endothelial cells,
the venous, arterial, and coronary artery vascular myocytes, and hepatocytes lead to the systemic
10 system reducing pre- and afterload. Nesiritide can inflammatory response syndrome (SIRS). The
improve neurohumoral markers of heart failure; it incidence for this potentially life-threatening com-
decreases the autonomic stimulation by inhibit- plication is reported in 4–37 % of patients on extra-
ing the renin-angiotensin-aldosterone system corporeal circulation during the operation.
(Behara et al. 2009; Jefferies et al. 2007; Simsic Clinical manifestations in the first (three to five)
et al. 2006). The pharmacologic profile with a postoperative days begin with fever, fluid retention
short half-life makes this drug attractive for the due to capillary leak syndrome, myocardial dys-
early postoperative period although more pediat- function with low cardiac output syndrome
ric studies are necessary before routine use. The (LCOS), the need for initiation or increases in ino-
dosage used starts with a bolus of 1–2 μg/kg fol- tropic support, acute pulmonary dysfunction
lowed by a continuous infusion of 0.01–0.02 μg/ (acute respiratory distress syndrome, ARDS), renal
kg/min for 24 h (Simsic et al. 2006). dysfunction, neurologic symptoms, or rarely, mul-
Levosimendan—used to prevent and to treat tiorgan failure (Carvalho et al. 2001; Chang 2003).
LCOS—acts as a calcium sensitizer. Increasing The activation of an immunologic response can be
the sensitivity of the contractile myofilaments to attributed to the surgical trauma, ischemia, reper-
calcium results in inotropic support; via an fusion, or cellular activation by the artificial sur-
ADP-associated process, potassium channels of face of extracorporeal circulation. Fever as the
vascular smooth muscles are opened resulting in main symptom may be due to bacterial infection
decreased systemic and pulmonary vascular or a noninfectious origin such as SIRS; procalcito-
resistance and coronary dilatation. As the vaso- nin and C-reactive protein (CRP) kinetics can be
dilating effect precedes the inotropic effect, useful markers in differentiating these conditions
some continued catecholamine support should (Nahum et al. 2012; Arkader et al. 2004).
be considered. The myocardial oxygen con-
sumption remains unchanged. Levosimendan
acts via active metabolites, which explains the 10.4.2 linical Criteria for SIRS
C
long duration of drug action. Levosimendan (Cavadas et al. 2011)
infusion starts usually after a loading dose of
12–24 μg/kg over 10 min followed by continu- 55 Fever
ous infusion of 0.1–0.2 μg/kg/min for 24 h and 55 Myocardial dysfunction, hemodynamic insta-
in some patients up to 48 h (Osthaus et al. 2009; bility, and need for inotropic support
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
261 10
55 Pulmonary dysfunction with increased Fi02 10.4.3 Treatment Options
and respiratory distress syndrome
55 Renal dysfunction Various strategies have been considered to affect
55 Endothelial dysfunction and fluid retention and ameliorate the inflammatory cascade in
with weight gain SIRS. These include the use of heparin-coated
bypass circuits, ultrafiltration during cardiopulmo-
A few studies describe the molecular mecha- nary bypass procedures, and administration of ste-
nism of the inflammatory cascade («postcardio- roids or intravenous immunoglobulin or
pulmonary bypass inflammation,» «post pump leukocyte-endothelial blocking agents (Berdat et al.
syndrome») which seems to be age related and 2004; Bronicki et al. 2000; Chaney 2002; Checchia
more severe in very young patients. In relation to et al. 2003; Grossi et al. 2000; Lindberg et al. 2003;
their bodyweight and body surface area, neonates Thompson et al. 2001). Heparin-bonded bypass
with complex cardiac surgery are exposed to the circuits seem to have some clinical benefit, but data
largest artificial surface area of cardiopulmonary on the relation to cytokine response is limited.
bypass circuits. The most important mediators are Ultrafiltration techniques, either before, during,
interleukin (IL)-6, IL-8, and IL-10, tumor necro- and/or after cardiopulmonary bypass, have become
sis factor (TNFα(alpha)), and adhesion molecules a standard method to remove surplus body water
like integrin and selectin; there is an increase in administered with the circuit prime volume during
circulating complement fractions (Bronicki et al. cardiopulmonary bypass procedures (Wang et al.
2000; Carvalho et al. 2001; Neuhof et al. 2003; 2012). In addition, long duration of bypass, deep
Tarnok et al. 1999). The induced inflammatory hypothermia, and low weight increase the risk of
processes are responsible for the capillary leak water accumulation during bypass in infancy.
syndrome with extracardiac fluid retention, Excess water removal will induce hemoconcentra-
edema, and compromised organ function or mul- tion, reduce myocardial edema, improve contrac-
tiorgan failure requiring delayed chest wall clo- tility, improve pulmonary gas exchange, and
sure, longer period of mechanical ventilation and improve pulmonary ventilation. Low molecular
catecholamine support, higher risk of infection, weight substances such as inflammatory cytokines
and longer ICU stay (Casey 1993; Dickerson and and complements will be removed during ultrafil-
Chang 2005). An isolated organ dysfunction can tration; benefits for the early postoperative period
be observed as myocardial dysfunction and and recovery are obvious (Huang et al. 2003;
decrease in cardiac output (about 16 %), pulmo- Mahmoud et al. 2005). Monitoring of the inflam-
nary dysfunction (about 23 %), or renal dysfunc- matory response during cardiopulmonary bypass
tion (about 23 %) (Cavadas et al. 2011). was considered; this would allow monitoring of the
Different factors including preoperative pul- intensity of the inflammatory reaction. Combining
monary hypertension, cardiac failure, or cyanosis conventional (during CPB) and modified ultrafil-
have been assumed to increase the risk and clini- tration (after CPB) seems to be the most effective
cal manifestation of SIRS. In addition genetic strategy, especially in neonates and infants who
polymorphisms predisposing to systemic inflam- undergo complex cardiac surgery with long dura-
matory response are discussed (Chang 2003; Mou tion of extracorporeal circulation (Berdat et al.
et al. 2002). The pronounced complement activa- 2004; Gaynor 2003; Thompson et al. 2001).
tion in patients with lack of complement compo- The criteria for the use of steroids vary depend-
nent C4 might trigger the inflammatory reaction ing on the age of the patient or type and length of the
and capillary leak syndrome (Zhang et al. 2005). cardiac surgery. There is a lack of standardization
Younger age, lower weight, longer cardiopulmo- with regard to the type of corticosteroid, timing of
nary bypass time, and longer duration of mechan- administration (before, during, or after cardiopul-
ical ventilation were identified as predisposing monary bypass or combined), and dosing regimens
factors; about 22 % of pediatric patients 3 years (Checchia et al. 2005; Dickerson and Chang 2005).
and younger presented with SIRS (Cavadas et al. The probable mechanisms of steroids by which
2011). SIRS and organ injury after pediatric open- they may improve the hemodynamic situation
heart surgery were not influenced by bypass tem- in inotropic- and volume-resistant h ypotension
perature when moderate or mild hypothermia include (Bronicki et al. 2000; Chaney 2002; Seri
was employed (Stocker et al. 2011). et al. 2001; Shore et al. 2001):
262 R. Kaulitz et al.
55 Limiting the capillary permeability and fluid with artificial surfaces leads to activation of coag-
retention ulation with thrombin generation, fibrinolysis,
55 Improving pulmonary compliance and oxy- and an inflammatory reaction on humoral and
gen delivery cellular level. Coagulation and inflammatory
55 Preserving myocardial performance system have multiple interactions in association
55 Reduction of inotropic requirements with with thrombin generation and proinflammatory
adrenergic receptor induction state. Surgical trauma per se causes tissue factor
activation, also followed by thrombin generation.
Stress doses of hydrocortisone given intra- The aim of anticoagulation is the suppression of
and postoperatively can attenuate the SIRS by an thrombin formation and consumption of coagu-
immunomodulating effect with reduction of pro- lation factors.
inflammatory cytokines (e.g., IL-6 and IL-8), Bleeding complications after cardiac surgery
increases in anti-inflammatory cytokines (e.g., are related to platelet dysfunction, thrombopenia,
IL-10), and modulation of the IL-6-to-IL-10 ratio, activation of the complement system, hemodilu-
which is associated with improved outcome after tion, activation of the hemostasis, and fibrinolysis
major cardiac surgery (Weis et al. 2009). as well as consumption of coagulation factors
The combined pre- and intraoperative steroid (Chang 2005; Pychynska-Pokorska et al. 2004).
administration seems to have the best anti- During the neonatal period and infancy, additional
inflammatory effect. The peak effect of methylpred- aspects include the immaturity of the coagulation
nisolone occurs 1–4 h after application (Checchia system; impaired heparin clearance; more signifi-
et al. 2005; Lindberg et al. 2003). The recommenda- cant hemodilution with reduction of clotting fac-
tions for dosage vary from a dose equivalent of tors and thrombopenia; deep hypothermia and
10 1–30 mg/kg of methylprednisolone. Dexamethasone cardiac arrest with more profound inflammatory
dosage is usually 1 mg/kg. There are no data on the reaction associated with increased vascular perme-
effect of steroids during the period of reperfusion or ability, platelet activation, and fibrinolysis; platelet
early postoperative period. Steroids given postoper- dysfunction induced by preceding prostaglandin
atively in hemodynamically compromised neonates infusion or in association with asplenia or poly-
with low cardiac output unresponsive to high doses splenia; and immaturity or impairment of kidney
of inotropic agents and fluid resuscitation turned out and liver function. In cyanotic congenital heart dis-
to be highly effective in improving the hemody- ease with stimulation of erythropoiesis, platelet
namic parameters and decreasing the inotropic regeneration and survival time are often reduced.
requirements. In this «rescue protocol» hydrocorti- In children with preoperative hepatic congestion or
sone was administered using two dosing regimens right heart dysfunction, coagulation factor abnor-
(100 mg/m2/day for 2 days, 50 mg/m2/day for 2 days, malities for fibrinogen, antithrombin, factors II, V,
and 25 mg/m2/day for 1 day or 100 mg/m2/day for VII, IX, and X, as well as proteins C and S have to
1 days, 50 mg/m2/day for 2 days, and 25 mg/m2/day be assumed (Bulutcu et al. 2005; Despotis et al.
for 2 day; Suominen et al. 2005). Adverse effects 2001; Williams et al. 1999).
associated with steroid administration included Technical developments improving the CPB
hyperglycemia requiring insulin administration, circuit are aimed at minimizing the activation of
infectious complications, or wound infections coagulation, inflammatory, and fibrinolytic sys-
(Checchia et al. 2005; Heyn et al. 2011). tems. Reducing the size of the perfusion equip-
ment and circuit volume seems to be beneficial.
To enhance the biocompatibility of the CPB cir-
10.5 Postoperative Bleeding cuits, biopassive coatings have been developed
Complications and some promising pediatric studies could be
and Anticoagulation published (Eaton et al. 2011).
cardiopulmonary bypass time longer than on one end to the critically ill neonate after
185 min, aortic clamping time above 98 min, cen- complex cardiac surgery in low cardiac output
tral venous saturation below 51 %, and age at state with delayed sternal closure, with or without
operation less than 7 days (Samir et al. 2002). The mechanical circulatory assist device on the other
incidence of TCWPP in neonates came up to end. For the future the adult with congenital heart
30 %. After primary sternal closure, atypical tam- disease may become an additional important
ponade or the tight mediastinal syndrome with challenge with special requirements for neuro-
low cardiac output, hypoxemia, acidosis, or protection and adequate management of pain and
increased systemic venous pressure can be signs sedation (Beke et al. 2005; Chang 2005; Diaz 2006;
to indicate a need for reopening. This was Donald et al. 2007; Huber and Kretz 2005; Wolf
described in 6.7 % of patients after primary chest and Jackman 2011).
closure (Samir et al. 2002). In this group the post- Several tools are now available to assess pain
operative mortality rate was significantly higher and sedation from neonate to adult. Behavioral
(Ziemer et al. 1992). and self-reporting scales have been recom-
The risk of infection during TCWPP turned mended, but difficulties still exist for neonates
out to be negligible as a routine antibiotic prophy- and infants, preverbal children, and developmen-
laxis is given using a cephalosporin or vancomy- tally delayed children. To reduce the risk of neu-
cin in combination with an aminoglycoside rologic sequelae, multimodal neuromonitoring
(Christenson et al. 1996; Tabbutt et al. 1997). was developed including bispectral index (BIS)
After 2–4 days with reduction of edema and using data from the electroencephalogram, near-
hemodynamic stabilization, definite chest closure infrared spectroscopy to monitor regional cere-
can be planned. Central venous pressure should bral oxygen index, or transcranial Doppler
10 be less than 10 mmHg, the coagulation parame- evaluation.
ters normalized, a negative total fluid balance «Fast tracking» and early postoperative extu-
documented, and hemodynamics should have bation need appropriate modification of the anes-
been stable during the last 24 h. Close monitoring thetic technique using shorter-acting drugs;
of hemodynamic parameters is necessary includ- increasing the acceptance of regional anesthesia
ing heart rate, central venous (and left atrial) pres- supports the goal of «fast tracking,» if desired.
sure, systemic arterial pressure, central venous Long-term sedation and analgesia after complex
saturation, and arterial blood gases. Delayed ster- cardiac repair can promote the development of
nal closure is possible if after sternal wiring the tolerance and withdrawal phenomena. During
central venous pressure increases less than the weaning process, a continuing balance of ade-
2 mmHg and the mean arterial blood pressure quate analgesia without deep excessive sedation
does not decrease more than 5 mmHg, compara- while maintaining sufficient respiratory function
ble to the situation in the OR (Ziemer et al. 1992). is required.
To avoid hemodynamic instability by transferring
the patient to the OR, we rather maintain the
hemodynamic stability with the patient staying in 10.7.1 Analgesia
his ICU bed and receiving definite chest closure
almost always on the intensive care unit.
10.7.1.1 Intravenous Application
with cardiac failure, pulmonary hypertension, Analgesia and sedation for many days can be fol-
and lung or airway disease, additional sedation is lowed by withdrawal symptoms such as sweating,
recommended. tachycardia, hypertension agitation, vomiting, or
Benzodiazepines (midazolam, bolus 0.05– diarrhea; this needs a special oral weaning regimen
0.1 mg/kg; infusion 0.05–0.2 mg/kg/h) have an over 7–14 days using clonidine, an opioid, and/or
amnestic effect with a reduction in consciousness benzodiazepine or haloperidol in older children.
and help with seizure control but have the risk of
withdrawal with prolonged use. Some drugs
reduce consciousness and pain such as ketamine 10.7.3 Muscle Relaxation/Paralytics
or clonidine.
Ketamine in addition to its analgesic proper- Muscle relaxation has to be considered in special
ties has a sedative effect, also (see 7 Sect. 10.7.1.1 situations during the early postoperative period
«Intravenous Application»). It can cause hemody- when low cardiac output is present. Muscle relax-
namic compromise by increasing afterload in ants reduce oxygen demand, and by inducing
catecholamine-dependent patients. It has, how- chest wall relaxation, they have an indirect influ-
ever, on the other side, a catecholamine-sensitizing ence on pulmonary vascular resistance when
effect. remaining elevated in a hemodynamic unstable
Chloral hydrate (oral/rectal 25–50 mg/kg) may situation. For the same reasons they are applied in
be administrated in addition to morphine/mid- children with delayed chest closure or those who
azolam during weaning process and is well tolerated. require hypothermia because of severe dysrhyth-
Haloperidol is used for delirium in agitated mia (Playfor et al. 2007).
patients. It has the risk of extrapyramidal side Pancuronium (0.1 mg/kg bolus) is a nondepo-
10 effects. IV doses of 0.025–0.05 mg/kg/day divided larizing neuromuscular blocking agent, which is
in three to four doses have been used. long acting with vagolytic effects, resulting in
Propofol (normal infusion dose 50–200 ug/kg/ tachycardia and hypertension.
min, max. 4 mg/kg/h) is used for short-term pro- Vecuronium (0.1 mg/kg bolus) has less unwanted
cedures; has a risk of hypotension and decrease in side effects, but can accumulate in hepatic failure.
systemic vascular resistance, dysrhythmias, rising Rocuronium (0.6–1.2 mg/kg bolus) has a rapid
lactate, or acidosis; and may cause propofol infu- onset of action, is useful for tracheal intubation,
sion syndrome: acidosis, bradyarrhythmia, and and turned out to have only minimal cardiovas-
rhabdomyolysis. cular side effects (Reich et al. 2004).
Dexmedetomidine (loading dose, 0.5 ug/kg Cisatracurium (0.1–0.2 mg/kg bolus IV) is an
over 15–30 min)—a selective α(alpha) 2 agonist— intermediate-acting neuromuscular blocking agent.
has a sympatholytic effect resulting in hypoten- It has no significant cardiovascular adverse events.
sion and bradycardia and a decrease in systemic
vascular resistance without suppressing respira-
tory drive. These effects are thought to be benefi- 10.8 Perioperative Prophylaxis
cial in cardiac patients, especially for attenuating for Infectious Complications
the hemodynamic and endocrinal response to
cardiopulmonary bypass with an intraoperative The perioperative administration of antimicrobial
infusion (Munoz and Berry 2005). agents is routinely used in pediatric cardiac surgery.
Methadone is a synthetic opioid with potent While the onset of routine antimicrobial treatment
analgesic activity and a long half-life. Because of is rather uniform, about 1 h to immediately before
its half-life and good oral bioavailability, it has surgery, the duration of prophylaxis varies. Usually
been used successfully to treat iatrogenic narcotic the antimicrobials are administered up to 24 or 48 h
dependency or chronic pain. postoperatively. Longer durations may depend on
Clonidine, an α(alpha)2 agonist, has less anal- the presence of central venous lines and chest tube
gesic potency and can be used for longer sedation drains that are handled following more local inten-
and withdrawal from opioids (oral, 2–5 ug/kg sive care guidelines. Thus, antimicrobials for
every 4 h; also available in transdermal applica- 6–9 days postoperatively seem to be common.
tion; IV preparation not available in the USA, Nosocomial infections (NI) were found to occur in
dose 0.5–3 ug/kg/h). up to 15 % with the consequence of longer intensive
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
267 10
care stay and increased mortality. The predominant first 48 h after intracardiac surgery employing
organism was coagulase- negative Staphylococcus extracorporeal circulation. The reported incidence
from skin flora, but also Staphylococcus aureus, gram- varies between 15 and 25 %. They occur most
negative pathogens, and enterococci were found. Risk often as sustained or non-sustained tachyarrhyth-
factors for NI include younger age/infancy, complex mia (supraventricular tachycardia [SVT], atrial
cardiac surgery, or open-chest procedure; NI sites are flutter [AF], junctional ectopic tachycardia [JET],
mainly bloodstream or lower respiratory tract or the ventricular tachycardia [VT]), or bradyarrhyth-
urinary tract (Grisaru-Soen et al. 2009). An increased mia (higher-degree AV block, sinus bradycardia).
risk might also be present in pediatric patients with Predisposing risk factors are prolonged cardio-
intracardiac right-to-left shunt (cerebral emboli, brain pulmonary bypass time, cardiac arrest and deep
abscess) and after implantation of prosthetic material hypothermia, younger age, myocardial ischemia,
(valve prosthesis, conduits, patch material). atrial or ventricular cardiotomy and placement of
The antimicrobial agent used for prophylaxis suture lines, hemodynamic impairment, electro-
should be effective against staphylococci like lyte imbalance, or arrhythmogenic agents as cate-
cephalosporins or penicillins combined with a cholamines. Preoperative arrhythmia may recur
ß-lactamase inhibitor; limiting the duration of postoperatively or persist (Batra et al. 2006; Beke
prophylactic administration will reduce the risk et al. 2005; Craig et al. 2001; Hoffman et al. 2003;
of the development of resistant pathogens. Ravinshankar et al. 2003).
Glycopeptides such as vancomycin or teicoplanin Mechanisms of postoperative arrhythmia are
were used in patients with high risk for methicillin- abnormal automaticity as assumed for JET and
resistant Staphylococcus aureus (MRSA), espe- VT; they are influenced by the autonomic nervous
cially in the neonatal period. system and show a gradual onset and termination.
Kato et al. defined three potential groups of Reentry mechanisms by anatomic or functional
patients being at highest risk for postoperative boundaries can develop at the atrial, AV nodal,
MRSA infection: atrioventricular, or ventricular level; they occur
1. Preoperative colonization with MRSA with rapid onset and sudden termination and may
screened by sampling cause AF and VT. Triggered automaticity can
2. Young children (<3 months) who had been in develop in the presence of premature beats and is
the hospital since birth a reason for torsades de pointes or other polymor-
3. Infants and young children who had complex phic tachycardias.
cardiac surgery Hemodynamic consequences depend on the
heart defect and potential anatomic residua and
The risk of resistant pathogens such as are mainly characterized by loss of atrioventricu-
vancomycin-resistant enterococci (VRE) has also lar synchrony; the cardiac output decreases by
to be considered (Kato et al. 2007; Finkelstein nearly 10–20 %. In addition, tachycardias reduce
et al. 2002; Maher et al. 2002). the cardiac output by shortening the diastolic fill-
In patients with temporary chest wall patch ing phase, leading to a decreased stoke volume.
plasty and secondary chest closure, the risk of infec- Bradycardia can be compensated to a certain
tion or mediastinitis was low as a routine antibiotic degree by increasing stroke volume, but the filling
prophylaxis is given using a cephalosporin or vanco- pressure may increase also, with an increase of
mycin in combination with an aminoglycoside ventricular volume.
(Christenson et al. 1996; Tabbutt et al. 1997). This
also is our personal experience in over 25 years.
10.9.2 Diagnostic Procedures
10.9 Arrhythmia Monitoring heart rate variability is one of the diag-
nostic tools used to detect inadequate heart rate
10.9.1 Causes and Hemodynamic acceleration or arrhythmia; in addition, invasive
Sequelae monitoring by arterial and systemic venous lines is
routinely available. An intracardiac ECG via the
Arrhythmias are quite frequent during the early temporary epicardial pacemaker wires allows
postoperative period, predominantly during the detailed rhythm analysis, identifying atrial and
268 R. Kaulitz et al.
ventricular spikes. Esophageal electrogram can most often there is a 2:1 or 3:1 conduction. If the
identify atrial activity; as the catheter electrodes patient is hemodynamically compromised, atrial
have sensing and pacing modes, atrial stimulation overdrive pacing with 120 % of the flutter fre-
or overdrive pacing is possible in infants; the first quency or cardioversion is indicated. Pharma
choice epicardial wires allow for atrial stimulation cologic therapy is initiated to prevent recurrence
or overdrive pacing in any age group. Cardiac (e.g., digoxin and ß-blocker or class III agent
catheterization should be considered if the sotalol or amiodarone).
arrhythmia may be related to surgical technique Atrial fibrillation with an atrial rate of 400–
compromising coronary artery perfusion. 700/min is less common, and it causes loss of
mechanical atrial activity; the ventricular rate is
irregular (absolute arrhythmia). It is treated along
10.9.3 Management of Postoperative the decision lines and treatment options for atrial
Arrhythmia flutter; however, atrial overpacing is not possible.
Ectopic atrial tachycardia is rarely documented
First of all, before specific and eventually complex early postoperatively and may occur transiently
treatment modalities are embarked on, distur- triggered by abnormal automaticity. Treatment
bances of the electrolyte status or acid-base status strategies include digoxin, reduction of catechol-
have to be excluded. In addition, myocardial dys- amine infusion, ß-blocker (esmolol), and amiod-
function or ischemia with acute increase in atrial arone to control ventricular rate.
or ventricular volume or pressures can initiate Junctional ectopic tachycardia has become the
arrhythmia. Modification of the inotropic support most frequent postoperative tachyarrhythmia
by catecholamines has to be considered, also. (incidence 6–11 %). This arrhythmia can occur
10 Management of acute arrhythmia depends on the after any cardiac surgery but may be more com-
hemodynamic status and mechanism; treatment monly seen after surgery for tetralogy of Fallot,
may be pharmacologic or electrical or a combina- ventricular septal defect, atrioventricular septal
tion of both (Payne et al. 2011). defect, atrial switch operation, or Norwood pro-
cedure (Batra et al. 2006; Hoffman et al. 2003;
10.9.3.1 Treatment of Tachyarrhythmia Perry and Walsh 1998). Predisposing factors may
Supraventricular tachycardias result most often be younger age at surgery (<6 months), prolonged
from intra-atrial reentry mechanisms. Depending bypass times, and catecholamine support; it is not
on heart rate and hemodynamic situation, strate- necessarily surgery near the His bundle or hypo-
gies comprise vagal maneuvers, overdrive pacing, magnesemia (Batra et al. 2006; Dodge-Khatami
or application of IV adenosine (initial 0.05–0.1 mg/ et al. 2002; Hoffman et al. 2003). JET starts often
kg IV, max. 0.3 mg/kg IV). Adenosine induces a during the first 24–48 h postoperatively and may
short AV block terminating SVT which runs be acutely life-threatening by loss of AV syn-
across the AV node. The short half-life of 7–10 s chrony and rapid ventricular rates, but is often
allows repeated applications. In situations of atrial self-limiting after 2–8 days. Diagnosis is made
flutter, adenosine will unmask the atrial activity from the surface or intracardiac ECG showing AV
during short AV block. Overdrive pacing choosing dissociation with an atrial rate lower than the ven-
a heat rate 10–40 beats higher than SVT may inter- tricular (tachycardia with small QRS at a rate of
rupt the reentry tachycardia. If the patient is 170–230/min). This causes a dramatic decrease in
hemodynamically compromised or even in shock, cardiac output and hypotension. Managing JET
synchronized cardioversion (0.25–1.0 J/kg) is used today includes a combination of hypothermia
(Beke et al. 2005; Perry and Walsh 1998). (surface cooling; 32–34 °C) and amiodarone (IV
Atrial flutter (intra-atrial reentry mechanism) bolus 5 mg/kg, continuous infusion 10–15 mg/kg/
can be diagnosed in the classic form by a saw- day) to control the ventricular rate or restore sinus
tooth p-wave pattern in the surface ECG with a rhythm. Temporary atrial/DDD pacing with a
p-wave rate of 300–600/min. More often, the heart rate just above the ectopic tachycardia
p-wave morphology is irregular and the fre- induces AV synchrony (Janousek et al. 2000; Laird
quency varies, related to various locations of et al. 2003). Adequate analgesia, sedation, and
potentially surgically induced foci. Depending on paralysis are needed to tolerate surface cooling
the AV conduction, the ventricular rate varies; (Hoffman et al. 2003; Laird et al. 2003).
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
269 10
Ventricular tachycardias (VT) are uncom- Fallot, or l-transposition of the great arteries. The
mon in early childhood. They are potentially incidence of advanced second-degree or third-
related to electrolyte or acid-base status, myo- degree AV block requiring pacemaker implanta-
cardial ischemia, myocarditis, or a low cardiac tion is now less than 1 %. All patients with a
output state (LCOS). Patients with a damaged or persistent higher- degree conduction block for
scarred myocardium after surgery for tetralogy more than 10–14 days should undergo perma-
of Fallot, aortic valve procedures, or Ross proce- nent cardiac pacemaker implantation (VVI-
dure may develop ventricular tachycardia with VVIR or DDD system) (Gregorates et al. 2002;
widened (>0.09 s) QRS complexes of a single Lin et al. 2010).
dominant morphology. AV dissociation, fusion Less often, sinus node dysfunction may be the
beats, and intermittent sinus beats are other cause of sinus bradycardia. It may follow atrial
observed features. surgery like correction of sinus venosus defect,
The other types of VT show varying QRS Mustard or Senning surgery (atrial switch) for
morphology, twisting around the isoelectric transposition of the great arteries, or Fontan-type
baseline (torsades de pointes). This type of VT is surgery. Permanent cardiac pacemaker implanta-
at high risk for ventricular fibrillation (and in tion (AAI, AAIR, or DDD mode) is indicated
need of defibrillation with 2–4 J/kg). Treatment when bradyarrhythmia causes symptoms or even
should be initiated immediately with synchro- reported syncope.
nized cardioversion (1–2 J/kg). More hemody-
namically stable patients can be treated
pharmacologically with magnesium (25–50 mg/ 10.10 Respiratory Dysfunction
kg in 5–15 min), lidocaine (1 mg/kg; infusion and Mechanical Ventilation
20–50 ug/kg/min), or amiodarone (Craig et al.
2001; Hanisch 2001). Detailed evaluation of the 10.10.1 Indication and Ventilation
hemodynamic situation and electrophysiologic Nodes
examination are usually initiated. For chronic
prevention of symptomatic VT, medical treat- Prolonged respiratory dysfunction after pediatric
ment or implantation of an «automatic implant- cardiac surgery may have various reasons. The
able cardioverter/defibrillator» is recommended respiratory mechanics may be impaired by upper
(Kleinman et al. 2010). airway obstruction (subglottic stenosis, injury to
the recurrent laryngeal nerve), interstitial edema
10.9.3.2 Treatment of Bradyarrhythmia from left-to-right shunt or inflammatory response
Sinus bradycardia with slowing of the heart rate after cardiopulmonary bypass (SIRS), parenchy-
below the age-related normal rates postopera- mal compromise by atelectasis or pneumonia,
tively mainly results from various degrees of AV pleural effusion, diaphragmatic paralysis, chest
block. First-degree AV block becomes hemody- wall edema, or abdominal compression by ascites.
namically relevant only with extremely prolonged Insufficient ventilation can occur in association
PQ duration. This can occur after complex atrial with deep sedation/analgesia, neuromuscular
surgery or surgery around the AV node. Second- insufficiency after prolonged mechanical ventila-
degree AV block with intermittently missing QRS tion, or with advanced malnutrition. Pulmonary
complexes (Mobitz type II) can result in slow gas exchange is also impaired in situations with
heart rates. Here, sequential pacing can be indi- low cardiac output, ventilation-perfusion mis-
cated or infusion of isoproterenol (continuous match, or intrapulmonary shunts with the conse-
infusion 0.05–0.1 ug/kg/min) or atropine bolus quence of hypercapnia.
(0.02 mg/kg). Third-degree AV block after rou- The indication for prolonged endotracheal
tine surgery may be transient early postopera- intubation (or reintubation) and mechanical ven-
tively with return of conduction within a few tilation is given in situations with acute respira-
days; however, there is an increased risk of tory insufficiency and inadequate gas exchange
delayed AV block. Surgeries with a risk of at least with retention of carbon dioxide (pCO2
temporary postoperative AV block include cor- >50 mmHg), hypoxemia (pO2 <70 mmHg) and
rection of ventricular septal defect, atrioventricu- peripheral oxygen saturation <93 %, and/or respi-
lar septal defect, subaortic stenosis, tetralogy of ratory acidosis. These limits have to be adapted to
270 R. Kaulitz et al.
the individual situation of the patient. Patients xygenation with a low mean airway pressure.
o
with right-to-left shunt are used to having lower Here, the aim is more than in any other patient to
oxygen saturations, the same as in patients with extubate as early as possible in order to use the
chronic hypercapnia. negative intrathoracic pressure under spontane-
In spontaneous respiration there is a synchro- ous respiration for improvement of passive pul-
nous active contraction of the diaphragm and monary perfusion.
intercostal muscles, inducing a negative intratho- Modifications of mechanical ventilation have
racic pressure followed by a passive expiration. been introduced to reduce the ventilator-related
Invasive ventilation offers a positive pressure at lung injury, to improve the postoperative hemo-
inspiration; the pressure decrease at expiration dynamic situation, and to facilitate gas exchange.
can be modified by using positive end expiratory High-frequency oscillatory ventilation (HFOV)
pressure (PEEP). This can be associated with a uses the combination of very small tidal volumes
decrease in venous return, increase in pulmonary (1–3 ml/kg) and high-frequency oscillatory ven-
artery pressure and pulmonary vascular resis- tilation (2–15 Hz, 120–900/min). This allows for
tance, and reduction in pulmonary blood flow or lung-protective ventilation with a reduced risk of
cardiac output with the need for volume supple- barotrauma and atelectasis (Kneyber et al. 2012).
mentation. These effects are «compensated» by It generates a continuous positive airway pres-
improvement of oxygenation and consecutive sure system with small oscillations. It is mainly
reduction of the pulmonary vascular resistance. used as a rescue modality when hypoxemia and
In patients with Fontan circulation, however, the acidosis occur on conventional mechanical ven-
passive pulmonary artery flow can be significantly tilation despite increasing alveolar ventilation.
compromised by inadequate positive pressure Oxygenation depends on FiO2 and mean airway
10 ventilation and PEEP. pressure, ventilation on frequency, and amplitude
The initial, conventional mode of mechanical of oscillation. Early use of HFOV in pediatric
ventilation is often pressure-regulated volume cardiac surgery may be beneficial, and it can be
controlled; with the beginning of spontaneous used when there is evidence of pulmonary hyper-
breathing, the mode is switched to synchronized tension and right ventricular failure (Bojan et al.
intermittent mandatory ventilation (SIMV) with 2011). High-frequency jet ventilation (HFJV) uses
pressure control. After complex cardiac surgery rates of 60–600 per minute with tidal volumes of
with prolonged cardiopulmonary bypass time, 2–5 ml/kg; this results in a lower mean airway
most patients remain under controlled ventilation pressure. High-frequency positive pressure ventila-
for at least 24 h, especially in neonates and infants. tion (HFPPV) combines tidal volumes of 3–4 ml/
Principal ventilatory parameters for infants/ kg with rates of 60–150 per minute.
children include a ventilator rate of 15–40/8–20 Neurally adjusted ventilatory assist (NAVA) is
per minute, a tidal volume of 8–10–12 ml/kg, and the newest mode of mechanical ventilation that
an I:E ratio of 1:1.5–1:3. Elevation of mean airway uses the electrical activity of the diaphragm to
pressure (by increase of PEEP, inspiratory time or trigger the ventilator breath and to adjust the ven-
flow) and inspired oxygen concentration will tilatory assist to the patient’s neural drive. First
increase the oxygenation. Hypercapnia needs an clinical data demonstrated a superior patient-
increase ventilatory minute volume. ventilator synchrony, lung-protective mechanical
International guidelines for the variety of ven- ventilation mode, and efficient unloading of the
tilation modes are still lacking; modes included respiratory muscles and suggested potential
pressure control, synchronized intermittent man- advantages for the weaning process after pro-
datory ventilation, pressure support, and pressure- longed mechanical ventilation (Verbrugghe and
regulated volume control. Jorens 2011).
The ventilation mode should take the underly- Noninvasive positive pressure ventilation
ing disease into account. Patients with pulmonary (NPPV) provides mechanical respiratory support
hyperperfusion may require pressure-controlled without the need for endotracheal intubation. It
ventilation with increased PEEP and a lower ven- refers to continuous positive airway pressure
tilator rate. After univentricular palliation with (CPAP) or bilevel respiratory support including
Glenn- or Fontan-type surgery, mechanical venti- expiratory or inspiratory positive airway pressure
lation is focused on optimal ventilation and or biphasic positive airway pressure (BIPAP).
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
271 10
It can be used in selected patients including cir- patient arriving on the intensive care unit. He
cumstances with postoperative or postextubation may, however, have already been extubated in the
respiratory failure, atelectasis, pneumonia, or OR.
ARDS. NPPV can facilitate weaning from The weaning process begins with the onset of
mechanical ventilation reducing the risk of rein- spontaneous breathing accompanied by a decrease
tubation as it improves gas exchange and reduces in ventilator support. There is no standard strat-
work of breathing. Predictive factors for failure of egy for the weaning process. Factors influencing
NPPV are higher FiO2 and hypercapnia on weaning are level of sedation, fluid balance of the
admission (Najaf-Zadeh and Leclerc 2011). Care patient (lung compliance decreases with intersti-
has to be taken that the face mask used can be tol- tial edema, chest wall edema), pulmonary hyper-
erated comfortably and does not cause pain. Tight tension (with the need for oxygen supplementation
masks leading to face hematomas have to be and special inhalation regimen), and function of
avoided. the diaphragm. Steroids started 6–24 h before
In neonates and infants, some units prefer to planned extubation after prolonged ventilation
apply high flow (4–8 l/min) air or oxygen via nasal may be useful in reducing tracheal inflammation
prongs after extubation, as a substitute/alternative and subglottic edema (Newth et al. 2009).
for CPAP. Care has to be taken to avoid skin break- Using indices to predict success of weaning
down from prolonged prong placement. and extubation is probably less reliable than clini-
Following repair of tetralogy of Fallot, low car- cal judgment and decision-making. A spontane-
diac output state secondary to restrictive right ven- ous breathing test or extubation readiness test
tricular physiology, refractory to conventional consisting of a period of spontaneous breathing
measures, may be improved by using negative pres- with FiO2 <0.5 failed to indicate extubation suc-
sure ventilation (NPV). NPV can augment pulmo- cess (Newth et al. 2009).
nary blood flow and cardiac output in patients with The weaning process in infants and children
restrictive right ventricular physiology and dia- employs SIMV (synchronized intermittent manda-
stolic dysfunction (Shekerdemian et al. 2000). The tory ventilation) or IMV mode with pressure sup-
use of negative pressure ventilation may also be port and gradual reduction of the ventilator
beneficial in patients after Fontan operation with support, i.e., ventilator rate. Comparing the effect
improvement of pulmonary blood flow and stroke of SIMV and BIPAP ventilation, the last mode
volume (Shekerdemian et al. 1997). allows more synchronization with the respirator.
The administration of heliox, a mixture of Criteria for extubation (extubation readiness)
helium and oxygen, during mechanical ventila- include spontaneous ventilation, stable hemody-
tion decreases the work of breathing and dimin- namic parameters, and optimal cardiac output,
ishes respiratory distress. It facilitates gas exchange normal cardiac rhythm/adequate pacing, intact
by improving oxygenation and eliminating car- airway reflexes with vigorous coughing, manage-
bon dioxide, making it a rescue strategy in patients able airway secretion, adequate oxygenation on
with severe airway obstruction and respiratory fraction of inspired oxygen <0.4, maintenance of a
failure (asthma, bronchiolitis, upper airway pH >7.35 and PaCO2 <45 mmHG, adequate hemo-
obstruction, cystic fibrosis, and congenital tra- stasis, and sufficient consciousness. Extubation
cheal stenosis, postoperatively after tracheal sur- failure (need for reintubation within 24–72 h) is
gery). Early experience made ventilation with most often related to upper airway obstruction and
heliox a promising strategy for children with vari- is expected in nearly 6 % of patients. The require-
ous complex respiratory diseases (Gupta and ment to reintubate increases morbidity. Residual
Cheifetz 2005; Martinón-Torres 2012). anatomic lesions have to be excluded as well as pul-
monary causes of respiratory failure (i.e., pleural
effusion, diaphragm paralysis, pneumothorax).
10.10.2 Weaning and Extubation These all, however, should have been excluded
Readiness before extubation. Neurologic problems such as
oversedation or neuromuscular blockade may lead
Weaning off mechanical ventilation is a priority to reintubation, also.
once postoperative hemodynamic stabilization is Prolonged mechanical ventilation (>72 h) fol-
achieved. This may be already the case with the lowing cardiac surgery in neonates and infants
272 R. Kaulitz et al.
correlated with postoperative morbidity includ- PVR. These are predominantly total anomalous
ing nosocomial infections and pneumonia, low pulmonary venous connection, truncus arterio-
cardiac output syndrome, need for inotropic sup- sus communis, atrioventricular septal defects,
port and more fluid retention, delayed sternal clo- nonrestrictive ventricular septal defect, transposi-
sure, and extubation failure. Longer stay in the tion of the great arteries with ventricular septal
ICU and increased mortality may be the conse- defect or aortopulmonary window, and during
quence. Risk factors associated with prolonged the early postoperative period after cardiac trans-
mechanical ventilation were younger age, more plantation.
complex form of congenital heart disease, or pre- Monitoring shows an increase of the systemic
existing comorbidities such as chromosomal venous pressure accompanied by a decrease of
abnormalities, dystrophy/malnutrition, heart fail- cardiac output as well as arterial pressure and oxy-
ure, pulmonary hypertension, and pulmonary gen saturation. A pulmonary hypertensive crisis
disease (Szekely et al. 2006). was defined as a pulmonary-to-systemic pressure
Early extubation after pediatric cardiac sur- ratio of more than 0.75, potentially leading to
gery (<8 h postoperatively) offers some benefits as acute right heart failure. Postoperative PH is the
reduction of pulmonary and nosocomial infec- result of endothelial damage, suppression of
tions and lower use of sedation. An additional endogenous NO production, inflammatory pro-
aspect is a shorter duration of intensive care stay cesses, ischemic lesions, or reperfusion syndrome
with reduced financial costs (Davis et al. 2004). after cardiopulmonary bypass leading to an
Fast-track pediatric anesthesia and cardiac sur- increased reactivity of the pulmonary vasculature.
gery is a concept suitable for selected patients The pulmonary vascular endothelium produces
with uncomplicated cardiac defects (as ASD, endogenous vasoconstrictors (as endothelin-1)
10 VSD, partial AVSD, subaortic stenosis, a replace- and vasodilators (as prostacyclin). Additional cir-
ment of RV-PA conduits) and absence of relevant cumstances can promote a postoperative increase
comorbidities such as pulmonary hypertension or of PVR requiring a longer period of mechanical
heart failure. Effective pain management is one of ventilation and stay on intensive care unit. These
the most important aspects of fast-track process circumstances include pulmonary complications
(Howard et al. 2010; Iodice et al. 2011). (atelectasis, pneumothorax, pleural effusion,
bronchospasm), pulmonary vascular microem-
boli, pulmonary edema, inadequate ventilation
10.11 Postoperative Pulmonary with carbon dioxide retention, and inadequate
Hypertension high PEEP (Brown et al. 2003; Checchia and
Bronicke 2012; Huddleston and Knoderer 2009;
10.11.1 Diagnosis and Specific Schulze-Neick et al. 2001).
Causes In postoperative situations with systemic-to-
pulmonary connections as after aortopulmonary
During the last years, the incidence and severity shunt procedures, especially in preoperative duct-
of postoperative pulmonary hypertension (PH) dependent cardiac lesions as hypoplastic left heart
have been reduced related to early surgical correc- syndrome, the relation of PVR to systemic vascular
tion of congenital heart disease, associated with resistance may be rather low, as a result of severe
modification of cardiopulmonary bypass like pulmonary hypercirculation. Balancing the sys-
using modified ultrafiltration, and changes in temic and pulmonary circulation can be achieved
treatment of critically ill patients. PH is due to by measures which either increase the PVR, like
persistent increased pulmonary blood flow (L/R reduction of the inspired oxygen fraction, hypoven-
shunt) or elevated pulmonary vascular resistance tilation, and elevation of the mean airway pressure
(PVR). It is defined as a mean pulmonary artery (e.g., with higher PEEP), or by lowering the sys-
pressure >25 mmHg at rest. An increased vascular temic vascular resistance with specific agents as
reactivity and the development of hypertensive sodium nitroprusside or phentolamine.
crisis postoperatively have to be anticipated in In biventricular circulation, an abnormal
some cardiac lesions with long-standing preoper- increase of the right ventricular pressure—calcu-
ative increased pulmonary blood flow or pulmo- lated by the trans-tricuspid Doppler velocity—
nary venous obstructive lesions with elevated can also be caused by residual anatomic lesions.
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
273 10
These may be right ventricular outflow tract of endothelial NO, can complicate the weaning
obstructions, pulmonary valve stenosis, left heart process (Atz et al. 1996; Miller et al. 2000). The
obstructive lesions, or pulmonary venous obstruc- recommended starting dose of NO is 20 ppm, but
tion. Right ventricular pressure overload shifts it may vary between 5 and 40 ppm (Journois et al.
the interventricular septum toward the left ven- 2005; Stocker et al. 2003).
tricle, causing severe tricuspid regurgitation, Sildenafil, a specific phosphodiestarese-5
arrhythmias, and left ventricular dysfunction, inhibitor, allows for a more selective pulmonary
each of these sequelae leading to low output states. vasodilation and is used for acute and chronic
Primary treatment options beside the manage- treatment of PH with lower risk of systemic arte-
ment of the PH are fluid management for ade- rial hypotension. The special effects in children
quate preload, control of arrhythmias, and after congenital heart surgery need further evalu-
inotropic support. ation (Atz et al. 2002; Schulze-Neick et al. 2002).
However, data suggest that oral sildenafil can
facilitate the weaning of iNO (Huddleston and
10.11.2 Treatment Options: Knoderer 2009). As enteral absorption may be
Prevention First! compromised during the early postoperative
period, intravenous sildenafil is advantageous and
Management strategies in patients at risk for post- was reported to reduce pulmonary artery pressure
operative PH are mainly focused on preventing (Fraisse et al. 2011). Preoperative sildenafil
potential trigger mechanisms and by providing reduced postoperative pulmonary artery pressure
deep sedation, moderate hyperventilation (i.e., and shortened length of stay in the intensive care
pCO2 30–35 mmHg), an increase of inspiratory unit (Palm et al. 2011).
oxygen fraction, and adequate PEEP ventilation. Chronic treatment options include bosentan—
The application of pulmonary vasodilators needs an endothelin receptor antagonist; it was shown
a strong indication because of the potential side to be safe and improves functional capacity.
effects. Milrinone, a selective phsphodiesterase-3 Potential side effects described were (reversible)
inhibitor with inotropic and vasodilator effects, abnormalities in hepatic function tests (Galie
works as an unspecific pulmonary vasodilator et al. 2003). In combination with sildenafil, both a
with pronounced lowering of the systemic blood decrease of sildenafil and increase of bosentan
pressure and therefore has only limited indica- concentration have to be considered; at present,
tions in PH. Intravenous prostacyclin (epopros- no dose recommendations are available for this
tenol, started with 1–2 ng/kg/min, titrated to combined therapy. Dosing for bosentan mono-
10–20 ng/kg/min) is an alternative option; how- therapy is weight related: for 5≤10 kg, 15.6 mg
ever, dose-dependent systemic side effects such as daily for 4 weeks and thereafter 15.6 mg twice
systemic hypotension, headache, nausea, and daily; for weight 10–20 kg, 31.25 mg daily for
vomiting may limit its use. Abrupt withdrawal 4 weeks and thereafter 31.25 mg twice a day; for
may induce rebound pulmonary hypertension. 20–40 kg, 31.25 mg twice a day for 4 weeks and
Inhaled prostacyclin and the prostacyclin analog, thereafter 62.5 mg twice a day; and for body-
iloprost, seem to be as effective as inhaled nitric weight >40 kg, 62.5 mg twice a day and thereafter
oxide (Mulligan and Beghetti 2012; Rimensberger 125 mg twice a day.
et al. 2001); the most common side effects are The degree of right ventricular failure must be
cough, headache, or flushing. Inhaled iloprost is determined, and treatment is aimed to reduce
often used in combination with other vasodilator pulmonary vascular resistance, increase cardiac
drugs (sildenafil, bosentan). output, and resolve systemic hypotension.
Inhaled nitric oxide (iNO) is a potent vasodi- Dobutamine in doses up to 5 ug/kg/min signifi-
lator and now a standard of care for postoperative cantly decreased pulmonary vascular resistance
pulmonary hypertension; however, prophylactic and increased cardiac output especially when
administration in patients at risk for pulmonary combined with iNO. Individualized therapy also
hypertensive crisis is not recommended. may include the administration of norepineph-
Development of methemoglobinemia may limit rine and dopamine. In these patients modification
its use. After abrupt reduction of NO, «rebound» of mechanical ventilation has to be considered as
pulmonary hypertension, because of downregulation hyperinflation or inadequate PEEP ventilation
274 R. Kaulitz et al.
can fatally reduce cardiac output. In refractory 55 Renal dysfunction has a multifactorial
right ventricular failure, atrial septectomy/septos- origin, sometimes determined by preoperative
tomy or the use of mechanical right ventricular events:
assist devices has to be discussed. 55 Prerenal (hypovolemia, hypotension, LCOS,
sepsis, peripheral vasoconstriction, increased
central venous pressure, mechanical ventila-
10.12 Kidney Injury and Renal tion, intra-abdominal pressure elevation,
Replacement Therapy ascites, postoperative residual lesions)
55 Renal (renal failure due to hypoxemia, acido-
10.12.1 efinition, Risk Factors,
D sis, nephrotoxic agents, hemolysis with
and Staging of Acute Kidney hemoglobinuria)
Injury (AKI) 55 Neurohumoral abnormalities (i.e., inadequate
ADH secretion, increase of plasma-renin or
Acute kidney injury (AKI) in infants and children aldosterone)
after heart surgery is a serious complication 55 Postrenal (preexisting/congenital renal anom-
encountered in 11–30 % (Sethi et al. 2011, alies or uropathy)
Chiravuri et al. 2011). AKI requiring dialysis
occurs in up to 5 % of these patients. The mortal- The diagnosis and classification of AKI can be
ity rate under these conditions might approach made using the gold standard of serial creatinine
40 %. However, even after a period of dialysis, measurements; biomarkers such as neutrophil
chronic renal impairment rarely develops gelatinase-associated lipocalin (NGAL), N-acetyl-
(Skippen and Krahn 2005). ß-(D)-glucosaminidase (NAG), interleukin-18,
10 AKI can be defined as an abrupt (within 48 h) kidney injury molecule-1, and cystatin C, how-
reduction in renal function with increase in ever, may allow for early rapid diagnosis of AKI as
serum creatinine of >0.3 mg/dl or a total increase they precede the increase in serum creatinine by
in serum creatinine to >1.5-fold from baseline or several hours. This might enable prevention and
reduction in urine output (<0.5 ml/kg/h for more early modification of clinical management of AKI
than 6 h) (Sehti et al. 2011). Using the modified after cardiopulmonary bypass (Krawczeski et al.
RIFLE (Risk, Injury, Failure, Loss of kidney func- 2010, 2011; Parikh et al. 2011).
tion, and End-stage kidney disease.) criteria, a
decrease of estimated creatinine clearance by 50 %
(resp. 75 %) and reduction of urinary output 10.12.2 Management of AKI
<0.5 ml/kg/h for 16 h (resp. <0.3 ml/kg/h for 24 h)
might indicate kidney injury (resp. failure) Postoperatively compromised cardiac function
(Akcan-Arikan et al. 2007). Uniform standards and hemodynamics lead to decreased kidney per-
for defining and staging of AKI were established fusion. The clinical features of a low cardiac out-
in 2008 by the AKI Network (Mehta et al. 2008). put syndrome include tachycardia, poor systemic
The risk for renal dysfunction is increased in: perfusion and inadequate mean arterial pressure,
55 Children younger than 12 months increase in serum lactate, and decreased urinary
55 Weight <10 kg output. Therapeutic options include inotropes,
55 Duration of cardiopulmonary bypass time vasopressors, vasodilators, and diuretics to opti-
>90 min mize cardiac function, renal and systemic perfu-
55 Complex heart defect and cyanosis sion, as well as pulmonary blood flow for oxygen
55 Postoperative LCOS and prolonged inotropic supply. To balance systemic and pulmonary blood
support flow, ensuring adequate systemic organ perfusion,
55 Systemic inflammatory response syndrome as well as sufficient oxygenation, medication, and
with intraoperative activation of the drug dosing, has to be titrated continuously,
immune system despite changes in CPB accompanied by ventilatory adjustments.
practice (hemofiltration, modified ultrafil-
tration) (Sethi et al. 2011; Picca et al. 2008; kDopamine
Beke et al. 2005; Chang 2003; Ravinshankar Dosages of 3–5 ug/kg/min improve renal perfusion
et al. 2003). and urinary output (dopamine receptor, also present
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
275 10
in the mesenteric vascular system), whereas dosages acid (IV 0.5–1 mg/kg/day, max. 2 mg/kg/day)
from 5 to 10 ug/kg/min increase inotropy and chro- may be administered early postoperatively. A
notropy (ß1-stimulation) and from 10 to 20 ug/kg/ more expensive alternative to furosemide is
min increase systemic vascular resistance (α(alpha)- bumetanide in single doses of 0.05 mg/kg every
receptor stimulation). High doses of dopamine for 6–8–12 h or in a continuous infusion (0.2–0.4–
treatment of low cardiac output syndrome might 0.5 mg/kg/day).
induce peripheral and splanchnic vasoconstriction
(Gajarski et al. 2003; Prins et al. 2001). kRenal replacement therapy (RRT)
Renal replacement therapy may be required to be
kFenoldopam required in nearly up to 10 % of children undergo-
It is a selective dopamine-1 receptor agonist which ing heart surgery. Acute peritoneal dialysis is
combines the effect of decreasing systemic vascu- most frequently used, as hemofiltration has the
lar resistance while increasing renal blood flow, disadvantage of vessel access and systemic antico-
tubular natrium extraction, and urinary output. In agulation.
contrast to dopamine, dopamine-2 and adrenergic Indications for peritoneal dialysis (PD) are
receptors are not activated, and chronotropic and oliguria (<0.5 ml/kg/h for more than 4 h) or
inotropic effects are not observed (Bove et al. 2005; anuria (despite adjusted inotropic, vasodilator,
Costello et al. 2006; Goldstein and Chang 2006). and diuretic support), fluid overload, creatinine
Pediatric experience is still limited. level >1.2 mg/dl, hyperkalemia or electrolyte
imbalances, or acidosis. Fluid overload may be
kEpinephrine an indication without acute renal failure (Chan
Treatment of low cardiac output syndrome often et al. 2003; Ravinshankar et al. 2003). As PD is
includes epinephrine at a dose >0.05 ug/kg/min easy and safe, it does not need any anticoagula-
for maintenance of adequate mean arterial pres- tion or vessel access and is well tolerated in the
sure. hemodynamically instable infant, and early
application is now recommended to avoid
kMilrinone increasing fluid overload. Prophylactic place-
It is a phosphodiesterase III inhibitor. Without the ment of peritoneal catheters intraoperatively for
increase of myocardial oxygen consumption, it routine early postoperative dialysis is expected
combines the effect of moderate inotropy after to reduce the effects of systemic inflammatory
end diastolic relaxation with vasodilation of pul- response by removing inflammatory mediators
monary and systemic circulation (Stocker and and allows early fluid balance (Baskin et al. 2010;
Shekerdemian 2006). Dittrich et al. 2004).
kDiuretics
Prevention of fluid overload, pulmonary edema, 10.13 Postoperative Nutrition
and elevated cardiac afterload is an important clin-
ical goal in treatment of critically ill children after Early nutrition assessment in the postoperative
cardiac surgery. To support a net negative fluid bal- period is necessary to avoid underfeeding or
ance from the start, diuretic therapy is introduced overfeeding; outcome and length of stay on the
almost immediately postoperatively. Furosemide, a ICU can be affected. The energy requirements are
loop diuretic, is most often used either as a bolus difficult to estimate and will change during the
(1 mg/kg every 6–8–12 h) or in a continuous infu- postoperative course; nutritional needs may be
sion (4–8–10 mg/kg/day) as recommended in the calculated using preoperative anthropometric
hemodynamically unstable patient. Usually treat- measurements or indirect calorimetry. Reasons
ment is started with higher doses and adjusted for inadequate nutrition therapy can be based on
based on urinary output (Ravinshankar et al. 2003; fluid restriction, ceasing of enteral nutrition for
Van der Vorst et al. 2001). In addition to furose- diagnostic procedure, weaning process and extu-
mide, aminophylline (single bolus infusion bation, or surgery, gastrointestinal intolerance
5–10 mg/kg) is an effective adjuvant for diuresis as with vomiting, abdominal distension, and dis-
is chlorothiazide (10–20 mg/kg/day, divided q6 h, turbed motility. Overfeeding also has adverse
or later 20 mg/kg/day p.o). Additionally ethacrynic metabolic effects with hepatic dysfunction.
276 R. Kaulitz et al.
are lacking. A long-term follow-up study in chil- and circulatory arrest and in infants with coexist-
dren with transposition of the great arteries under- ing abnormalities of the central nervous system
going neonatal arterial switch surgery revealed (Gaynor et al. 2006; Clancy et al. 2003).
worse results in motor and speech functioning for Thromboembolic events may occur intraoper-
infants after predominantly deep hypothermic cir- atively (contact of blood with artificial surfaces of
culatory arrest; however, after low-flow cardiopul- extracorporeal circulation, air embolism) or early
monary bypass (initiated to reduce the risk of postoperatively (venous stasis, clotting factor
ischemic cerebral injury), a higher proportion of imbalance, residual lesions, artificial patches, and
patients with behavioral difficulties was described. tubes). Due to postoperative sedation and pharma-
The entire study group, regardless of bypass strat- cologic paralysis, this injury may be recognized
egy, presented with difficulties in sustained atten- only days later. Screening for coagulopathies (pri-
tion, speech, and learning abilities or developmental mary prophylaxis) and appropriate treatment
delay (Bellinger et al. 1999; Skaryak et al. 1996). reduce the risk at the original surgery. Secondary
Animal studies could demonstrate that low-flow prophylaxis means to prevent recurrence.
cardiopulmonary bypass may induce a greater Postoperative dyskinesias, most often
inflammatory response with nonneurologic mor- described as choreoathetosis, have an incidence of
bidity; the intermittent perfusion during deep 0.5 %; they usually become evident clinically after
hypothermic circulatory arrest seems to have a several days. They are assumed to be secondary to
neuroprotective effect resulting in normal cerebral hypoxic-ischemic injuries related to a special vul-
metabolic and ultrastructural recovery (Langley nerability of the basal ganglions during extracor-
et al. 1999). poreal circulation. They occur more often after
Postoperative risk factors for cerebral injury surgery for cyanotic lesions, especially with high
10 include invasive monitoring, mechanical ventila- pulmonary and consecutively less cerebral flow
tion, pharmacologic management in a hemody- during extracorporeal circulation. There is recov-
namically unstable patient, risk of cerebral emboli, ery; however, in most cases, it is not complete.
post-bypass cerebral edema related to inflamma-
tion, reperfusion injury or microemboli, and
hyperthermia with increase cerebral oxygen 10.14.3 Neuromonitoring Techniques
requirements. The duration of postoperative
intensive care unit length of stay in the newborn A standard for continuous intraoperative and
period was associated with worse developmental early postoperative cerebral monitoring that
outcome (Newberger et al. 2003). During the allows for immediate detection of potential brain
early postoperative period, the first 24–48 h after damage has not been defined. Maintaining cere-
cardiopulmonary bypass, the systemic blood flow bral oxygenation and cerebral autoregulation is
and oxygen delivery are decreased, and the most important in preventing brain injury during
cerebral pressure autoregulation is impaired,
cardiopulmonary bypass and when employing
implying the high risk for localized ischemic or deep hypothermic circulatory arrest. Noninvasive
global hypoxic injury of the central nervous sys- monitoring techniques include cranial near-
tem (Bassan et al. 2005; Wernovsky 2006). infrared spectroscopy (NIRS), electroencephalo-
The occurrence of seizures in the immediate gram (EEG), transcranial Doppler ultrasound
postoperative period after repair or palliation of (TCD), and bispectral index monitoring (BIS).
congenital heart defects was defined as a marker NIRS uses near-infrared light signals to moni-
of early central nervous system injury. Continuous tor nonpulsatile signal component reflecting tis-
electroencephalographic monitoring demon- sue (arteriolar (25 %), capillary (5 %), venous
strated seizures in 10–20 % of neonates and (70 %)) circulation and measures oxygenation
infants and was thought to be a surrogate marker regionally in the frontal cerebral cortex. Tissue
for long-term neurologic outcomes in early stud- oxygenation measured by NIRS showed a good
ies, while more recent data could not prove the correlation to mixed venous saturation used as an
negative predictive value (Gaynor et al. 2006; indicator for cardiac output (Nagdyman et al.
Clancy et al. 2003; Rappaport et al. 1998). 2005; Tortoriello et al. 2005). This correlation,
Postoperative seizures were more often identified however, is best for children weighing less than
after prolonged duration of deep hypothermia 10 kg. As studies revealed that it may not be
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
279 10
possible to predict absolute mixed venous satura- form of ECLS in pediatrics, the largest experience
tion, NIRS can be used for indicating trends is now available for ECMO.
(Mittnacht 2010). In cyanotic patients, NIRS
baseline values may vary between 40 and 60 %; in
acyanotic patients they reach approximately 70 %. 10.15.1 Indications
Trends during cardiopulmonary bypass are simi-
lar in infants and children, but they showed a The indication for ECLS connected with cardiac
wide inter-patient variability and an absence of surgery may exist for:
normal values. 55 Any unstable postoperative state after complex
Postoperative EEG monitoring allows detec- congenital heart surgery, predominantly con-
tion of (subclinical) seizures in patients under nected with Bland-White-Garland (Anomalous
sedation und neuromuscular blockade. Left Coronary Artery from the Pulmonary
TCD measures the blood flow velocity in the Artery (ALCAPA)) syndrome, transposition of
anterior and/or middle cerebral arteries; patient’s the great arteries, total anomalous pulmonary
baseline measurement can be compared with the venous drainage, and hypoplastic left heart
actual intra- or postoperative measurements. syndrome
Age-specific normal values can be provided and 55 Inability to wean from cardiopulmonary
allows the detection of decreased Doppler veloci- bypass; postoperative myocardial dysfunc-
ties related to hemodynamic instability, general tion (LCOS)
anesthesia, or low temperature (Zimmermann 55 Occasional preoperative stabilization (i.e.,
et al. 1997; Andropoulous et al. 2003). cardiogenic shock, after clinical deterioration
BIS as a simplified processed EEG can corre- or even resuscitation, pulmonary hyperten-
late brain wave activity with awareness or depth of sion refractory to medical therapy; in rare
sedation; it was found to indicate changes in cere- exceptions for patients with obstructive total
bral activity in the presence of acute hypotension anomalous pulmonary venous drainage or
causing cerebral hypoperfusion. The BIS monitor Ebstein’s anomaly)
may indicate by burst suppression pattern the 55 Severe hypercapnia after tracheal resection in
adequate cooling before initiating deep hypother- pulmonary artery sling surgery not amenable
mic circulatory arrest (Hayashida et al. 2003). to Heliox treatment (see 7 Sect. 10.10)
55 Exclusion of relevant residual lesions and function, and inability to wean from cardiopul-
surgical bleeding monary bypass (Hintz et al. 2005; Ravinshankar
55 Neurological integrity et al. 2003). Some centers have recommended
55 Upper limit of pharmacologic treatment routine mechanical ventricular support to stabi-
lize the early postoperative period after Norwood
Clinical findings mirror a low cardiac output I procedure coining it NOMOVAD («no mem-
constellation with inadequate organ perfusion brane oxygenator ventricular assist device») (Shen
with low mean arterial blood pressure, reduced and Ungerleider 2004). The known difficulties in
ejection fraction (<25 %) and cardiac index (<2 l/ resuscitating patients after Glenn or Fontan pro-
min/m2), limited diuresis (<1 ml/kg/h), central cedure, with limitations to achieve effective pul-
venous pressure >15 mmHg or left atrial pressure monary blood flow and oxygenation to start with,
>18 mmHg, and increasing lactate level. The cri- continue with technical difficulties of cannulation
teria to start with ECLS are variable. Nearly 1–5 % for ECMO in this challenging patient group.
of pediatric patients need mechanical circulatory Experience is very limited in the literature, most
support after corrective surgery for complex con- probably due to individual poor outcome in these
genital heart defects (Duncan et al. 1998; Morris cases.
et al. 2004; Walker et al. 2003). The principal management includes monitor-
ing of the systemic perfusion (acid-base status,
mixed venous oxygen saturation, serum lactate,
10.15.2 Extracorporeal Membrane urine output), providing afterload reduction (i.e.,
Oxygenation (ECMO) vasodilators as nitroprusside or milrinone), mod-
and Ventricular Assist ifying ventilator settings, assessment of the neuro-
10 Devices (VAD) logic state (including daily cranial ultrasound
examination) and organ function, as well as exclu-
For pediatric patients two forms of mechanical sion of infections. Fluid retention and total body
life support are available: the ECMO system for edema formation can require hemofiltration.
critically ill children who require acute or short- Heparin is used for anticoagulation (400–
term circulatory support and the VAD for long- 1000 IU/kg/day) monitored by the «activated
term support in children with cardiac failure who clotting time» (ACT, target 180–220 s). When
need longer time for recovery or bridging to using heparinized tubings, the ACT can be kept
transplant. Obviously, VAD implantation requires lower, around 150 s for centrifugal pump VADs
a sufficient pulmonary function. The decision for and 180 s for centrifugal pump ECMOs. The
one or the other system also depends on anatomy hematocrit should be optimized to 40–50 %, the
(uni- or biventricular circulation), pathophysiol- platelet count >100,000/μl (Cooper et al. 2007,
ogy, cannulation, emergent resuscitation, and Coskun et al. 2011). Weaning from ECMO
local experience. requires inotropic support, monitoring of cardiac
For cardiac support ECMO cannulation usu- output and filling pressures, and stepwise reduc-
ally requires venoarterial access (transthoracic, tion of flow. Usually mechanical support is
right atrium-ascending aorta; cervical, jugular required for 2–3 days; if myocardial recovery is
vein-carotid artery). More than 70 % of venous limited or needs more than 3–5 days, VAD should
return can be passed to the membrane oxygenator be considered in cases of good pulmonary func-
bypassing the heart. Respiratory failure in isolated tion. Poor prognosis is associated with an ECMO
parenchymal lung disease with sufficient cardiac period of longer than 8–10 days (Coskun et al.
function can be managed by venovenous ECMO, 2011).
usually via a transjugular double lumen catheter. Bleeding is one of the major complications
With increasing experience, ECMO indica- during ECMO. It often is associated with the need
tion was extended to functionally univentricular for extensive replacement of blood products; sub-
circulation, requiring some modification in the sequent re-exploration of the mediastinum might
technical management (e.g., increased flow be indicated. Other complications comprise
because of the runoff by the systemic-to- thromboembolic events, hemolysis, air embolism,
pulmonary shunt). It can be lifesaving in acute infections, cerebral bleeding, and stroke. Finally,
shunt thrombosis, deterioration of ventricular multiorgan failure after repeat transfusions or
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
281 10
major technical problems can complicate the on VAD is supposed to be as high as 25 % (Cooper
ECMO period even more (Montgomery et al. et al. 2007). Neurological impairment has been
2000; Sorof et al. 1999). Thus, management of an described in up to 20 % after VAD and more than
ECMO patient requires an experienced interdisci- 60 % after ECMO, which, however, is more often
plinary team. used in critically ill infants with complex surgical
The advantages of ECMO include the option procedures.
of biventricular support as well as respiratory sup-
port. It can readily be applied in emergent resusci-
tation, it offers the possibility of peripheral References
cannulation, and it is suitable for newborn babies.
Disadvantages are patient immobilization and the Aharon AS, Drinkwater DC, Churchwell KB et al. (2001)
need for anticoagulation with the risk for bleeding Extracorporeal membrane oxygenation in children
complications. Pulmonary blood flow may be after repair of congenital cardiac lesions. Ann Thorac
Surg 72:2095–2101
critically diminished especially with nonpulsatile
Akcan-Arikan A, Zappitelli M, Loftis LL et al. (2007) Modified
flow dynamics in two ventricle hearts. RIFLE criteria in critically ill children with acute kidney
ECMO has become the routine system for injury. Kidney Int 71:1028–1035
short-term circulatory support. Bridging to Andropoulos DB, Stayer SA, McKenzie DE et al. (2003)
recovery or cardiac transplantation can be offered Novel cerebral physiologic monitoring to guide low-
flow cerebral perfusion during neonatal aortic arch
by VAD. This long-term mechanical support is
reconstruction. J Thorac Cardiovasc Surg 125:491–499
now also available for children weighing less than Arkader R, Troster EJ, Abellan DM et al. (2004) Procalcitonin
5 kg. For long-term use pulsatile devices are used and C-reactive kinetics in postoperative pediatric car-
for univentricular and biventricular support (e.g., diac surgical patients. J Cardiothorac Vasc Anesth
Berlin Heart VAD, MEDOS VAD; Reinhartz et al. 18:160–165
Atz AM, Adatia I, Wessel DL (1996) Rebound pulmonary
2002). Indications include myocarditis, dilated
hypertension after inhalation of nitric oxide. Ann
cardiomyopathy, and postsurgical complex con- Thorac Surg 62:1759–1764
genital heart disease. Atz AM, Lefler AK, Fairbrother DC (2002) Sildenafil aug-
Advantages of VAD include mobilization if no ments the effect of inhaled nitric oxide for postopera-
mechanical ventilation is required, lower-level tive pulmonary hypertensive crisis. J Thorac Cardiovasc
Surg 124:628–629
anticoagulation, less trauma and alteration of
Bailey JM, Miller BE, Lu W et al. (1999) The pharmacokinet-
blood cells, and lower risk for infections (Aharon ics of milrinone in pediatric patients after cardiac sur-
et al. 2001; Mehta et al. 2000; Cooper et al. 2007; gery. Anesthesiology 90:1012–1018
Fan et al. 2011; Morales et al. 2010). Baskin E, Gulleroglu KS, Saygili A et al. (2010) Peritoneal dialy-
As VAD provides only cardiac support, its sis requirements following open-heart surgery in chil-
dren with congenital heart disease. Ren Fail 32:784–787
effectiveness requires sufficient pulmonary func-
Bassan H, Gauvreau K, Newberger JW et al. (2005) Identification
tion, which may be listed as a disadvantage in of pressure passive cerebral perfusion and its mediators
comparison with ECMO. after infant cardiac surgery. Pediatr Res 57:35–41
The survival for children with complex con- Batra AS, Chun DS, Johnson TR et al. (2006) A prospective
genital heart defects after ECMO support varies analysis of the incidence and risk factors associated
with junctional ectopic tachycardia following surgery
from 30 % to 60 % and is determined also by the
for congenital heart disease. Pediatr Cardiol 27:51–55
timing of initiating ECLS (intraoperatively, after a Behera SK, Zuccaro JC, Wetzel GT et al. (2009) Nesiritide
period of being successfully weaned, emergent improves hemodynamics in children with dilated car-
resuscitation, or after resuscitation), age, and diomyopathy: a pilot study. Pediatr Cardiol 30:26–34
accompanying renal and hepatic dysfunction. Beke DM, Braudis NJ, Lincoln P (2005) Management of the
pediatric postoperative cardiac surgery patient. Crit
With increasing duration of ECMO, the prognosis
Care Nurs Clin N Am 17:405–416
will decrease as myocardial dysfunction persists, Bellinger DC, Wypij D, Kuban KC et al. (1999) Developmental
renal and hepatic dysfunction might develop, and and neurological status of children at 4 years of age after
the risk for additional complications of mechani- heart surgery with hypothermic circulatory arrest or low-
cal circulatory support increases (Hintz et al. flow cardiopulmonary bypass. Circulation 100:526–532
Bellinger DC, Wypij D, du Plessis AJ et al. (2001)
2005; Kolovos et al. 2003; Morris et al. 2004). The
Developmental and neurologic effects of alpha-stat
survival rate after VAD is different as the children versus pH-stat strategies for deep hypothermic cardio-
are usually older and nearly 50 % were subse- pulmonary bypass in infants. J Thorac Cardiovasc Surg
quently transplanted; mortality rate while being 121:374–383
282 R. Kaulitz et al.
Berdat PA, Eichenberger E, Ebell J et al. (2004) Elimination Chang AC, Atz AM, Wernovsky G et al. (1995) Milrinone:
of proinflammatory cytokines in pediatric cardiac sur- systemic and pulmonary hemodynamic effects in neo-
gery: analysis of ultrafiltration method and filter type. nates after cardiac surgery. Crit Care Med 23:1907–1914
J Thorac Cardiovasc Surg 127:1688–1696 Checchia PA, Bronicke RA (2012) Review of inhaled nitric
Berg RA, Donnerstein RL, Padbury JF (1993) Dobutamine oxide in the pediatric cardiac surgery setting. Pediatr
infusions in stable, critically ill children: pharmacoki- Cardiol 33:493, Epub ahead of print
netics and hemodynamic actions. Crit Care Med Checchia PA, Backer CL, Bronicki RA et al. (2003)
21:678–688 Dexamethasone reduces postoperative troponin lev-
Bhamidipati CM, LaPar DJ, Stukenborg GJ, Morrison CC, els in children undergoing cardiopulmonary bypass.
Kern JA, Kron IL, Ailawadi G (2011) Superiority of mod- Crit Care Med 31:1742–1746
erate control of hyperglycemia to tight control in Checchia PA, Bronicki RA, Costello JM et al. (2005) Steroid
patients undergoing coronary artery bypass grafting. use before pediatric cardiac operation using cardio-
J Thorac Cardiovasc Surg 141:543–551 pulmonary bypass: an international survey of 36 cen-
Biewer ES, Zürn C, Arnold R et al. (2010) Chylothorax after ters. Pediatr Crit Care Med 6:442–445
surgery on congenital heart disease in newborns and Cheung PY, Chui N, Joffe AR et al. (2005) Postoperative lac-
infants—risk factors and efficacy of MCT-diet. tate concentrations predict the outcome of infants
J Cardiothorac Surg 5:127–133 aged 6 weeks or less after intracardiac surgery: a
Bojan M, Gioanni S, Mauriat P et al. (2011) High-frequency oscil- cohort follow-up to 18 months. J Thorac Cardiovasc
latory ventilation and short-term outcome in neonates Surg 130:837–843
and infants undergoing cardiac surgery. Crit Care 15:R259 Chiravuri SD, Riegger LQ, Christensen R et al. (2011) Factors
Bove T, Landoni G, Calabro MG et al. (2005) Renoprotective associated with acute kidney injury or failure in chil-
action of fenoldopam in high-risk patients undergo- dren undergoing cardiopulmonary bypass: a case-
ing cardiac surgery: a prospective, double-blind, ran- controlled study. Pediatr Anesth 21:880–886
domized clinical trial. Circulation 111:3230–3235 Christenson JT, Maurice J, Simonet F, Velebit V, Schmuziger
Bronicki RA, Backer CL, Baden HP et al. (2000) M (1996) Open chest and delayed sternal closure after
Dexamethasone reduces the inflammatory response cardiac surgery. Eur J Cardiothorac Surg 10:305–311
to cardiopulmonary bypass in children. Ann Thorac Clancy RR, McGaurn SA, Wernovsky G et al. (2003) Risk of
10 Surg 69:1490–1495 seizures in survivors of newborn heart surgery using
Brown KL, Ridout DA, Goldman AP et al. (2003) Risk factors deep hypothermic circulatory arrest. Pediatrics 11:
for long intensive care unit stay after cardiopulmonary 592–601
bypass in children. Crit Care Med 31:28–33 Codispoti M, Mankad PS (2000) Management of anticoag-
Bulutcu FS, Ozbek U, Polat B et al. (2005) Which may be ulation and its reversal during pediatric cardiopulmo-
effective to reduce blood loss after cardiac operations nary bypass: a review of current UK practice. Perfusion
in cyanotic children: tranexamic acid, aprotinin or a 15:191–201
combination? Paediatr Anaesth 15:41–46 Cooper DS, Jacobs JP, Moore L et al. (2007) Cardiac extra-
Cai J, Su Z, Shi Z et al. (2008) Nitric oxide and milrinone: corporeal life support: state of the art in 2007. Cardiol
combined effect on pulmonary circulation after Young 17(Suppl 2):104–115
Fontan- type procedure: a prospective, randomized Coskun KO, Cokun ST, Popov AF et al. (2011) Extracorporeal
study. Ann Thorac Surg 86:882–888 life support in pediatric cardiac dysfunction.
Carvalho MV, Maluf MA, Catani R et al. (2001) Cytokines J Cardiothorac Surg 5:112–116
and pediatric open heart surgery with cardiopulmo- Costello JM, Thiagarajan RR, Dionne RE et al. (2006) Initial
nary bypass. Cardiol Young 11:36–43 experience with fenoldopam after cardiac surgery in
Casey LC (1993) Role of cytokines in the pathogenesis of neonates with an insufficient response to conven-
cardiopulmonary-induced multisystem organ failure. tional diuretics. Pediatr Crit Care Med 7:28–33
Ann Thorac Surg 36:S93–S96 Craig J, Fineman LD, Moynihan P et al. (2001) Cardiovascular
Cavadas da Costa Soares L, Ribas D, Spring R et al. (2011) critical care problems. In: Curley MAQ, Moloney-
Clinical profile of systemic inflammatory response Harmon P (eds) Critical care nursing of infants and
after pediatric cardiac surgery with cardiopulmonary children, 2nd edn. Saunders, Philadelphia, pp 579–654
bypass. Arq Bras Cardiol 94:119–124 Davis S, Worley S, Mee RB, Harrison AM (2004) Factors asso-
Chan K, Ip P, Chiu C, Cheung Y (2003) Peritoneal dialysis ciated with early extubation after cardiac surgery in
after surgery for congenital heart disease in infants young children. Pediatr Crit Care Med 5:63–68
and young children. Ann Thorac Surg 76:1443–1449 Dent CL, Spaeth JP, Jones BV et al. (2006) Brain magnetic
Chaney MA (2002) Corticosteroids and cardiopulmonary resonance imaging abnormalities after the Norwood
bypass: a review of clinical investigations. Chest procedure using regional cerebral perfusion. J Thorac
121:921–931 Cardiovasc Surg 131:190–197
Chang AC (2003) Inflammatory mediators in children Despotis GJ, Avidan MS, Hogue CW (2001) Mechanisms
undergoing cardiopulmonary bypass: is there a uni- and attenuation of hemostatic activation during
fied field theory amidst this biomolecular chaos? extracorporeal circulation. Ann Thorac Surg 72:1821–
Pediatr Crit Care Med 4:386–387 1831
Chang AC (2005) Common problems and their solutions in Diaz LK (2006) Anesthesia and postoperative analgesia in
pediatric cardiac intensive care. Cardiol Young pediatric patients undergoing cardiac surgery.
15(Suppl 1):169–173 Paediatr Drugs 8:223–233
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
283 10
Dickerson HA, Chang AC (2005) Steroids and low cardiac Gajarski RJ, Mosca RS, Ohye RG (2003) Use of extracorpo-
output syndrome after cardiac surgery in children. real life support as a bridge to pediatric cardiac trans-
Pediatr Crit Care Med 6:495–496 plantation. J Heart Lung Transplant 22:28–34
Dittrich S, Aktuerk D, Seitz S et al. (2004) Effects of ultra- Galal MO, El-Naggar WI, Sharfi MH (2005) Phentolamine
filtration and peritoneal dialysis on proinflammatory as a treatment for poor mixing in transposition of the
cytokines during cardiopulmonary bypass surgery great arteries with adequate intraatrial communica-
in newborns and infants. Eur J Cardiothorac Surg 25: tion. Pediatr Cardiol 26:444–445
935–940 Galie N, Hinderliter AL, Torbicki A et al. (2003) Effects of
Dodge-Khatami A, Miller OI, Anderson RH et al. (2002) the oral endothelin-receptor antagonist bosentan on
Surgical substrate of postoperative junctional ecto- echocardiographic and Doppler measures in patients
pic tachycardia in congenital heart defects. J Thorac with pulmonary artery hypertension. J Am Coll Cardiol
Cardiovasc Surg 123:624–630 16:1380–1386
Donald C, Duncan R, Blair L, Thakore S, Clark M (2007) Galli KK, Zimmerman RA, Jarvik GP et al. (2004)
Paediatric analgesia in the emergency department, Periventricular leukomalacia is common after neonatal
are we getting it right? Eur J Emerg Med 14:157–159 cardiac surgery. J Thorac Cardiovasc Surg 127:692–704
Douri M, Shafi T, Khudairi D (2000) Effect of the adminis- Gaynor JW (2003) The effect of modified ultrafiltration on
tration of recombinant activated factor VII in the man- the postoperative course in patients with congenital
agement of severe uncontrolled bleeding in patients heart disease. Semin Thorac Cardiovasc Surg Pediatr
undergoing heart valve replacement surgery. Blood Card Surg Annu 6:128–139
Coagul Fibrinolysis 11(Suppl 1):121–127 Gaynor JW, Jarvik GP, Bernbaum J et al. (2006) The rela-
Doyle RA, Dhir AK, Moors AH et al. (1995) Treatment of tionship of postoperative electrographic seizures
perioperative low cardiac output syndrome. Ann to neurodevelopmental outcome at 1 year of age
Thorac Surg 59:S3–S11 after neonatal and infant cardiac surgery. J Thorac
Duggal B, Pratap U, Slavik Z, Kaplanova J, Macrae D (2005) Cardiovasc Surg 131:181–189
Milrinone and low cardiac output following cardiac Goldstein SL, Chang AC (2006) Fluid balance in children
surgery in infants: is there a direct myocardial effect? after cardiac surgery: is fenoldopam an answer?
Pediatr Cardiol 26:642–645 Pediatr Crit Care Med 7:89–90
Duncan BW, Ibrahim AE, Hraska V et al. (1998) Use of rapid- Gregorates G, Abrams J, Epstein AE et al. (2002) ACC/
deployment extracorporeal membrane oxygenation AHA/NASPE 2002 guidelines update for implantation
for the resuscitation of pediatric patients with heart of cardiac pacemakers and antiarrhythmia devices.
disease after cardiac arrest. J Thorac Cardiovasc Surg Circulation 106:2145–2161
116:305–311 Grisaru-Soen G, Paret G, Yahav D et al. (2009) Nosocomial
Dunser MW, Mayr AJ, Stallinger A et al. (2003) Cardiac per- infections in pediatric cardiovascular surgery patients:
formance during vasopressin infusion in postcardiot- a 4-year survey. Pediatr Crit Care Med 10:202–206
omy shock. Intensive Care Med 28:746–751 Grossi EA, Kallenbach K, Chau S et al. (2000) Impact of heparin
Easton MP, Iannoli EM (2011) Coagulation considerations bonding on pediatric cardiopulmonary bypass: a pro-
for infants and children undergoing cardiopulmonary spective randomised study. Ann Thorac Surg 70:191–196
bypass. Pediatr Anesth 21:31–42 Gupta VK, Cheifetz IM (2005) Heliox administration in
Fakler U, Pauli C, Balling G et al. (2007) Cardiac index moni- the pediatric intensive care unit: an evidence-based
toring by pulse contour analysis and thermodilution review. Pediatr Crit Care 6:204–211
after pediatric cardiac surgery. J Thorac Cardiovasc Haga KK, McClymont KL, Clarke S, Grounds RS, Ng KY, Glyde
Surg 133:224–228 DW, Loveless RJ, Carter GH, Alston RP (2011) The effect
Fan Y, Weng YG, Xiao YB, Huebler M et al. (2011) Outcomes of tight glycaemic control, during and after cardiac
of ventricular assist device support in young patients surgery, on patient mortality and morbidity: a system-
with small body surface area. Eur J Cardiothorac Surg atic review and meta-analysis. J Cardiothorac Surg 6:3
39:699–704 Hanisch D (2001) Pediatric arrhythmias. J Pediatr Nurs
Finkelstein R, Rabino G, Mashiah T et al. (2002) Vancomycin 16:351–362
versus cefazolin prophylaxis for cardiac surgery in the Hausdorf G (2000) Intensivtherapie angeborener
setting of a high prevalence of methicillin-resistant Herzfehler. Steinkopff, Darmstadt
staphylococcal infections. J Thorac Cardiovasc Surg Hayashida M, Chinzei M, Komatsu K et al. (2003) Detection
123:326–332 of cerebral hypoperfusion with bispectral index dur-
Follath F, Franco F, Cardoso JS (2005) European experience ing cardiac surgery. Br J Anaesth 90:694–698
on the practical use of levosimendan in patients with Heyn J, Beiras-Fernandez A, Luchtung B et al. (2011)
acute heart failure syndromes. Am J Cardiol 96:80G–85G Inflammatory reactions and hydrocortisone in the set-
Forbess JM, Visconti KJ, Bellinger DC et al. (2002) ting of cardiac surgery. Cardiovasc Hematol Agents
Neurodevelopmental outcomes after biventricular Med Chem 9:56–61
repair of congenital heart defects. J Thorac Cardiovasc Hintz SR, Benitz WE, Colby CE et al. (2005) Utilization and
Surg 123:631–639 outcomes of neonatal cardiac extracorporeal life sup-
Fraisse A, Butrous G, Taylor MB, Oakes M, Dilleen M, Wessel port 1996–2000. Pediatr Crit Care Med 6:33–38
DL (2011) Intravenous sildenafil for postoperative Hoffman TM, Wernovsky G, Wieand TS et al. (2003) The inci-
pulmonary hypertension in children with congenital dence of arrhythmias in a pediatric cardiac intensive
heart disease. Intensive Care Med 37:502–509 care unit. Pediatr Cardiol 23:598–604
284 R. Kaulitz et al.
Hoffmann TM, Wernovsky G, Atz AM et al. (2002) support after cardiac surgery. Ann Thorac Surg
Prophylactic intravenous use of milrinone after car- 76:1435–1441
diac operation in pediatrics (PRIMACORP). Am Heart Krawczeski CD, Vandevoorde RG, Kathman T et al. (2010)
143:15–21 Serum Cystatin C is an early biomarker of acute kidney
Hoffmann TM, Wernovsky G, Atz AM et al. (2003) Efficacy injury after pediatric cardiopulmonary bypass. Clin
and safety of milrinone in preventing low cardiac out- J Am Soc Nephrol 5:1552–1557
put syndrome in infants and children after corrective Krawczeski CD, Goldstein SL, Woo JG et al. (2011) Temporal
surgery for congenital heart disease. Circulation relationship and predictive value of urinary acute kid-
107:996–1002 ney injury biomarkers after pediatric cardiopulmonary
Howard F, Brown K, Garside V et al. (2010) Fast-track paedi- bypass. J Am Coll Cardiol 58:2301–2309
atric cardiac surgery: the feasibility and benefits of a Laird WP, Snyder CS, Kertesz NJ et al. (2003) Use of amioda-
protocol for uncomplicated cases. Eur J Cardiothorac rone for postoperative junctional ectopic tachycardia
Surg 37:193–197 in children. Pediatr Cardiol 24:133–137
Huang H, Yao T, Wang W et al. (2003) Continuous ultrafiltra- Langley SM, Chai PJ, Miller JR et al. (1999) Intermittent per-
tion attenuates the pulmonary injury that follows fusion protects the brain during deep hypothermic
open heart surgery with cardiopulmonary bypass. circulatory arrest. Ann Thorac Surg 68:4–12
Ann Thorac Surg 76:136–140 Lechner E, Hofer A, Mair R et al. (2007) Arginine-vasopressin
Huber D, Kretz FJ (2005) Efficacy of clonidine in paediatric in neonates with vasodilatory shock after cardiopul-
anaesthesia. Anästhesiol Intensivmed 40:567–575 monary bypass. Eur J Pediatr 166:1221–1227
Huddleston AJ, Knoderer ES (2009) Sildenafil for the treat- Lee C, Mason LJ (2001) Pediatric cardiac emergencies.
ment of pulmonary hypertension. Pediatr Cardiol Anesthesiol Clin N Am 19:287–308
30:871–882 Leonhard SR, Nikaidoh H, Copeland MM et al. (1997)
Hulst JM, Jossten KF, Tibboel D et al. (2006) Causes and con- Cardiothoracic surgery. In: Levin DL, Morris FC (eds)
sequences of inadequate substrate supply to pediatric Essentials of pediatric intensive care, 2nd edn.
ICU patients. Curr Opin Clin Nutr Metab Care 9:297–303 Churchill Livingstone, New York, pp 611–623
Iodice FG, Thomas M, Walker I et al. (2011) Analgesia in Li JS, Bengur AR, Ungerleider RM et al. (1998) Abnormal
fast-
track paediatric cardiac patients. Eur J Cardio left ventricular filling after neonatal repair of congeni-
10 Thorac Surg 40:610–613 tal heart disease: association with increased mortality
Janousek J, Vojtovic V, Chaloupecky V et al. (2000) and morbidity. Am Heart J 136:1075–1080
Hemodynamically optimized temporary cardiac pac- Limperopoulos C, Majnemer A, Shevell MI et al. (2000)
ing after surgery for congenital heart defects. Pacing Neurodevelopmental status of newborns and infants
Clin Electrophysiol 23:1250–1259 with congenital heart defects before and after open
Jefferies JL, Price JF, Denfield SW et al. (2007) Safety and heart surgery. J Pediatr 137:638–645
efficacy of nesiritide in pediatric heart failure. J Card Lin A, Mahle WT, Frias PA et al. (2010) Early and delayed
Fail 13:541–548 atrioventricular conduction block after routine sur-
Journois D, Baufreton C, Mauriat P et al. (2005) Effects of gery for congenital heart disease. J Thorac Cardiovasc
inhaled nitric oxide administration on early postoper- Surg 140:158–160
ative mortality in patients operated for correction of Lindberg L, Forsell C, Jögi P, Olsson AK (2003) Effects of
atrioventricular canal defects. Chest 128:3537–3544 dexamethasone on clinical course, C-reactive protein,
Kaltman JR, DI H, Tian Z et al. (2005) Impact of congenital S100B protein and von Willebrand factor antigen after
heart disease on cerebrovascular blood flow dynamics paediatric cardiac surgery. Br J Anaesth 90:728–732
in the fetus. Ultrasound Obstet Gynaecol 25:32–36 Mackie AS, Booth T, Newburger JW et al. (2005) A ran-
Kato Y, Shime N, Hashimoto S et al. (2007) Effects of con- domised, double-blind, placebo-controlled pilot trial
trolled perioperative antimicrobial prophylaxis on of triiodothyronine in neonatal heart surgery. J Thorac
infectious outcome in pediatric cardiac surgery. Crit Cardiovasc Surg 130:810–816
Care Med 35:1763–1768 Maher KO, Van Der Elzen K, Mosca RS, Chenoweth CE, Kulik
Kenet G, Strauss T, Kaplinsky C et al. (2008) Hemostasis and TJ (2002) A retrospective review of three antibiotic
thrombosis in critically ill children. Semin Thromb prophylaxis regimes for pediatric cardiac surgical
Hemost 34:451–458 patients. Ann Thorac Surg 74:1195–1200
Kirklin JW, Barratt-Boyes BG (1993) Postoperative care. In: Mahle WT, Tavani F, Zimmerman R et al. (2002) An MRI
Terry D (ed) Cardiac surgery. Churchill Livingstone, study of neurological injury before and after congeni-
New York, pp 222–225 tal heart surgery. Circulation 106:109–114
Kleinman ME, Chameides L, Schexnayder SM et al. (2010) Mahmoud AS, Burhani MS, Hannef AA et al. (2005) Effect of
American Heart Association guidelines for cardiopul- modified ultrafiltration on pulmonary function after
monary resuscitation and emergency cardiovascular cardiopulmonary bypass. Chest 128:3447–3453
care. Circulation 122(Suppl):S876–S908 Martin K, Gertler R, Liermann H et al. (2011) Switch from
Kneyber MCJ, van Heerde M, Markhorst DG (2012) aprotinin to ε-aminocaproic acid: impact on blood
Reflections on pediatric high-frequency oscillatory loss, transfusion, and clinical outcome in neonates
ventilation from a physiologic perspective. Respir Care undergoing cardiac surgery. Br J Anaesth 107:
57:1496, Epub 934–939
Kolovos NS, Bratton SL, Moler FW et al. (2003) Outcome of Martinón-Torres F (2012) Noninvasive ventilation with
pediatric patients treated with extracorporeal life helium-oxygen in children. J Crit Care 27:220–229
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
285 10
Matok I, Vardi A, Augarten A et al. (2007) Beneficial effects Motta P, Mossad E, Toscana D et al. (2005) Comparison of
of terlipressin in prolonged pediatric cardiopulmo- phenoxybenzamine to sodium nitroprusside in infants
nary resuscitation: a case series. Crit Care Med undergoing surgery. J Cardiothorac Vasc Anesth
35:1161–1164 19:54–59
Matok I, Rubinstein M, Levy A et al. (2009) Terlipressin for Mou SS, Haudek SB, Lequier L et al. (2002) Myocardial
children with extremely low cardiac output after open inflammatory activation in children with congenital
heart surgery. Ann Pharmacother 43:423–429 heart disease. Crit Care Med 30:827–832
Maund E, McDaid C, Rice S et al. (2011) Paracetamol and Mulligan C, Beghetti M (2012) Inhaled iloprost for the con-
selective and non-selective non-steroidal anti- trol of acute pulmonary hypertension in children: a
inflammatory drugs for the reduction in morphine- systematic review. Pediatr Crit Care Med 13:472, Epub
related side effects after major surgery: a systemic ahead of print
review. Br J Anaesth 106:292–297 Munoz R, Berry D (2005) Dexmedetomidine: promising drug
Mehta U, Laks H, Sadeghi A et al. (2000) Extracorporeal for pediatric sedation? Pediatr Crit Care Med 6:493–494
membrane oxygenation for cardiac support in pediat- Murzi B, Lervasi G, Masini S et al. (1995) Thyroid hormones
ric patients. Am Surg 66:879–886 hemostasis in patients during and after cardiopulmo-
Mehta RL, Kellum JA, Shah SV et al. (2008) Acute kidney nary bypass. Ann Thorac Surg 59:481–485
injury network: report of an initiative to improve out- Nagashima M, Imai Y, Seo K et al. (2000) Effect of hemofil-
comes in acute kidney injury. Crit Care 11:R31 trated whole blood pump priming on hemodynamics
Mehta NM, Compher C, ASEPN Board of Directors (2009) and respiratory function after arterial switch opera-
ASPEN clinical guidelines: nutrition support of the tion in neonates. Ann Thorac Surg 70:1901–1906
critically ill child. J Parenter Nutr 33:260–276 Nagdyman N, Fleck T, Schubert S et al. (2005) Comparison
Miller OI, Tang SF, Keech A et al. (2000) Inhaled nitric oxide between cerebral tissue oxygenation index measured
and prevention of pulmonary hypertension after con- by near-infrared spectroscopy and venous jugular bulb
genital heart surgery: a randomised double-blind saturation in children. Intensive Care Med 31:846–850
study. Lancet 356:1464–1469 Nahum E, Livni G, Schiller O et al. (2012) Role of C-reactive
Mittnacht A (2010) Near infrared spectroscopy in children protein velocity in the diagnosis of early bacterial
at high risk of low perfusion. Curr Opin Anesthesiol infections in children after cardiac surgery. J Intensive
23:342–347 Care Med 27:191–196
Moffett BS, Chang AC (2006) Future pharmacologic agents Najaf-Zadeh A, Leclerc F (2011) Noninvasive positive pres-
for treatment of heart failure in children. Pediatr sure ventilation for acute respiratory failure in chil-
Cardiol 27:533–551 dren: a concise review. Ann Int Care 1:15–25
Moga MA, Manlhiot C, Marwali EM et al. (2011) Neuhof C, Walter O, Dapper F et al. (2003) Bradykinin and
Hyperglycemia after pediatric cardiac surgery: impact histamine generation with generalized enhancement
of age and residual lesions. Crit Care Med 39:266–272 of microvascular permeability in neonates, infants,
Moganasundram S, Hunt BJ, Sykes K et al. (2010) The rela- and children undergoing cardiopulmonary bypass
tionship among thromboelastography, hemostatic surgery. Pediatr Crit Care 4:299–304
variables, and bleeding after cardiopulmonary bypass Newburger JW, Wypij D, Bellinger DC et al. (2003) Length
surgery in children. Anesth Analg 110:995–1002 of stay after infant heart surgery is related to cognitive
Momeni M, Rubay J, Matta A et al. (2011) Levosimendan in outcome at age 8 years. J Pediatr 143:67–73
congenital cardiac surgery: a randomized, double-blind Newth CLJ, Venkataraman S, Willson DF et al. (2009)
clinical trial. J Cardiothorac Vasc Anesth 25:419–424 Weaning and extubation readiness in pediatric
Monroe DM, Hoffman M, Oliver JA (1997) Platelet activity patients. Pediatr Crit Care 10:1–24
of high-dose factor VIIa is independent of tissue factor. Osthaus WA, Boethig D, Winterhalter M et al. (2009) First
Br J Haematol 99:542–547 experiences with intraoperative Levosimendan in
Montgomery VL, Strotman JM, Ross MP (2000) Impact of pediatric cardiac surgery. Eur J Pediatr 168:735–740
multiple organ system dysfunction and nosocomial Palma G, Giordano R, Russolillo V, Cioffi S, Palumbo S,
infections on survival of children treated with extra- Mucerino M, Poli V, Vosa C (2011) Sildenafil therapy for
corporeal membrane oxygenation after heart surgery. pulmonary hypertension before and after pediatric
Crit Care Med 28:526–531 congenital heart surgery. Tex Heart Inst J 38:238–242
Morales DL, Zafar F, Rossano JW et al. (2010) Use of ven- Parikh CR, Devarajan P, Zappitelli M et al. (2011)
tricular assist devices in children across the United Postoperative biomarkers predict acute kidney injury
States: analysis of 7.5 million pediatric hospitaliza- and poor outcome after pediatric cardiac surgery.
tions. Ann Thorac Surg 90:1313–1318 J Am Soc Nephrol 22:1737–1747
Morris MC, Ittenbach RF, Godinez RI et al. (2004) Risk fac- Payne L, Zeigler VL, Gilette PG (2011) Acute cardiac arrhyth-
tors for mortality in 137 pediatric cardiac intensive mias following surgery for congenital heart disease.
care unit patients managed with extracorporeal mem- Crit Care Nurs Clin N Am 23:255–272
brane oxygenation. Crit Care Med 32:1061–1069 Penny DJ, Sano T, Smolich JJ (2001) Increased systemic
Mossinger H, Dietich W, Braun SL (2003) High-dose apro- oxygen consumption offsets improved oxygen deliv-
tinin reduces activation of hemostasis, allogeneic ery during dobutamine infusion in newborn lambs.
requirements and duration of postoperative ventila- Intensive Care Med 27:1518–1525
tion in pediatric cardiac surgery. Ann Thorac Surg Perry JC, Walsh EP (1998) Diagnosis and management of
75:430–437 cardiac arrhythmias. In: Chang AC, Hanley FL,
286 R. Kaulitz et al.
Wernovsky G et al. (eds) Pediatric cardiac intensive Schulze-Neick I, Dietz P, Stiller B et al. (2002) Sildenafil is a
care. Lippincott, Williams and Wilkins, Philadelphia, potent pulmonary vasodilator in children after heart
pp 461–480 surgery. Circulation 19(Suppl):396
Picca S, Ricci Z, Picardo S (2008) Acute kidney injury in an Seri I, Tan R, Evans J (2001) Cardiovascular effects of hydro-
infant after cardiopulmonary bypass. Semin Nephrol cortisone in preterm infants with pressor-resistant
28:470–476 hypotension. Pediatrics 107:1070–1074
Playfor S, Jenkins I, Boyles C et al. (2007) Consensus guide- Sethi SK, Goyal D, Yadav DK et al. (2011) Predictors of acute
lines for sustained neuromuscular blockade in criti- kidney injury post-cardiopulmonary bypass in chil-
cally ill children. Pediatr Anesth 17:881–887 dren. Clin Exp Nephrol 15:529–534
Prins I, Plötz FB, Uiterwaal C, van Vught HJ (2001) Low-dose Shekerdemian LS, Bush A, Shore DF et al. (1997)
dopamine in neonatal and pediatric intensive care: a Cardiopulmonary interactions after Fontan opera-
systematic review. Int Care Med 27:206–210 tions: augmentation of cardiac output using negative
Proulx F, Lemson J, Choker G et al. (2011) Hemodynamic pressure ventilation. Circulation 13:211–212
monitoring by transpulmonary thermodilution and Shekerdemian LS, Schulze-Neick, Redington AN et al.
pulse contour analysis in critically ill children. Pediatr (2000) Negative pressure ventilation as hemodynamic
Crit Care Med 12:459–466 rescue following surgery for congenital heart disease.
Pychynska-Pokorska M, Moll JJ, Krajewski W, Jarosik P Int Care Med 26:93–96
(2004) The use of recombinant coagulation factor VIIa Shen I, Ungerleider RM (2004) Routine use of mechanical
in uncontrolled postoperative bleeding in children ventricular assist following the Norwood procedure.
undergoing cardiac surgery with cardiopulmonary Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu
bypass. Pediatr Crit Care Med 5:246–250 7:16–21
Rappaport LA, Wypij D, Bellinger DC et al. (1998) Relation Shillingford AJ, Marino BS, Ittenbach RF et al. (2005)
of seizures after cardiac surgery in early infancy to Microcephaly is common in neonates with hypoplas-
neurodevelopmental outcome. Boston Circulatory tic left heart syndrome. Pediatr Cardiol 25:581
Arrest Study Group. Circulation 97:773–779 Shore S, Nelson DP, Pearl JM et al. (2001) Usefulness of cor-
Ravinshankar C, Tabbutt S, Wernovsky G (2003) Critical care in ticosteroid therapy in decreasing epinephrine require-
cardiovascular medicine. Curr Opin Pediatr 15:443–453 ments in critically ill infants with congenital heart
10 Reich DL, Hollinger I, Harrington DJ et al. (2004) disease. Am J Cardiol 88:591–594
Comparison of cisatracurium and vecuronium by infu- Simsic J, Scheurer M, Tobias JD et al. (2006) Perioperative
sion in neonates and small infants after congenital effects and safety of nesiritide following cardiac sur-
heart surgery. Anesthesiology 101:1122–1127 gery in children. J Intensive Care Med 21:22–26
Reinhartz O, Stiller B, Eilers R et al. (2002) Current clinical Skaryak LA, Lodge AJ, Kirshbom PM et al. (1996) Low-flow
status of pulsatile pediatric circulatory support. ASAIO cardiopulmonary bypass produces greater pulmonary
J 48:455–459 dysfunction than circulatory arrest. Ann Thorac Surg
Riess FC, Poetzsch B, Madlener K et al. (2007) Recombinant 62:1284–1288
hirudin for cardiopulmonary bypass anticoagulation: Skillman HE, Mehta NM (2012) Nutrition therapy in the
a randomized, prospective and heparin-controlled critically ill child. Curr Opin Crit Care 18:192–198
pilot study. Thorac Cardiovasc Surg 55:233–238 Skippen PW, Krahn GE (2005) Acute renal failure in children
Rimensberger PC, Spahr-Schopfer I, Berner M et al. (2001) undergoing cardiopulmonary bypass. Crit Care Resusc
Inhaled nitric oxide versus aerosolized iloprost in sec- 7:286–291
ondary pulmonary hypertension in children with con- Sorof JM, Stromberg D, Brewer ED et al. (1999) Early initia-
genital heart disease. Circulation 103:544–548 tion of peritoneal dialysis after surgical repair of con-
Ririe DG, James RL, O’Brien JJ (2002) The pharmacokinetics genital heart disease. Pediatr Nephrol 13:641–645
of epsilon-aminocaproic acid in children undergoing Stocker C, Penny DJ, Brizard CP et al. (2003) Intravenous
surgical repair of congenital heart disease. Anesth sildenafil and inhaled nitric oxide: a randomised trial
Analg 94:44–49 in infants after cardiac surgery. Intensive Care Med
Roediger L, Larbuisson R, Lamy M (2006) New approaches 29:1996–2003
and old controversies to postoperative pain control fol- Stocker CF, Shekerdemian LS (2006) Recent develop-
lowing cardiac surgery. Eur J Anaesthesiol 23:539–550 ments in the perioperative management of the pae-
Samir K, Riberi A, Ghez O et al. (2002) Delayed sternal clo- diatric cardiac patient. Curr Opin Anaesthesiol
sure: a life-saving measure in neonatal open heart 19:375–381
surgery; could it be predictable? Eur J Cardiothorac Stocker CF, Shekerdemian LS, Norgaard M et al. (2007)
Surg 21:787–793 Mechanism of a reduced cardiac output and the effects
Schears G, Zaitseva T, Schultz S (2006) Brain oxygenation of milrinone and levosimendan in a model of infant
and metabolism during selective cerebral perfusion in cardiopulmonary bypass. Pediatr Crit Care 35:252–259
neonates. Eur J Cardiothorac Surg 29:168–174 Stocker CF, Shekerdemian LS, Horton SB et al. (2011) The
Schulze-Neick I, Li J, Penny DJ, Reddington AN (2001) influence of bypass temperature on the systemic
Pulmonary vascular resistance after cardiopulmonary inflammatory response and organ injury after pediat-
bypass in infants: effect on postoperative recovery. ric open surgery: a randomized trial. J Thorac
J Thorac Cardiovasc Surg 121:1033–1039 Cardiovasc Surg 142:174–180
Chapter 10 · Critical Care in Pediatric Cardiac Surgery
287 10
Suominen PK, Dickerson HA, Moffett BS et al. (2005) Vogt W, Läer S (2011) Prevention for pediatric low cardiac
Hemodynamic effects of rescue protocol hydrocorti- output syndrome: results from the European survey
sone in neonates with low cardiac output syndrome EuLoCOS-Paed. Paediatr Anaesth 21:1176–1184
after cardiac surgery. Pediatr Crit Care Med 6:655–659 Walker GM, McLeod K, Brown KL et al. (2003) Extracorporeal
Suominen PK (2011) Single-center experience with levosi- life support as a treatment of supraventricular tachy-
mendan in children undergoing cardiac surgery and cardia in infants. Pediatr Crit Care Med 4:52–54
in children with decompensated heart failure. BMC Wang S, Palanzo D, Ündar A (2012) Current ultrafiltration
Anesthesiol 5:1 techniques before, during and after pediatric cardio-
Szekely A, Sapi E, Kiraly L et al. (2006) Intraoperative and pulmonary bypass procedures. Perfusion 27:438,
postoperative risk factors for prolonged mechanical Epub ahead of print
ventilation after pediatric cardiac surgery. Pediatr Weiss F, Beiras-Fernandez A, Schelling G (2009) Stress
Anesth 16:1166–1175 doses of hydrocortisone in high-risk patients under-
Tabbutt S, Duncan BW, McLaughlin D et al. (1997) Delayed going cardiac surgery: effects on interleukin-6 to
sternal closure after cardiac operations in a pediatric interleukin-10 ratio and early outcome. Crit Care Med
population. J Thorac Cardiovasc Surg 113:886–893 37:1685–1690
Takami Y, Ina H (2002) Significance of the initial arterial lac- Wernosky G (2006) Current insights regarding neurologi-
tate level and transpulmonary arteriovenous lactate dif- cal and developmental abnormalities in children and
ference after open-heart surgery. Surg Today 32:207–212 young adults with complex congenital cardiac dis-
Tarnok A, Hambsch J, Emmrich F et al. (1999) Complement ease. Cardiol Young 16:92–104
activation, cytokines, and adhesion molecules in chil- Wernovsky G, Hoffman TM (2001) Pediatric heart failure:
dren undergoing cardiac surgery with or without car- solving the puzzle. Crit Care Med 29:S212–S213
diopulmonary bypass. Pediatr Cardiol 20:113–125 Wernovsky G, Wypij D, Jonas RA et al. (1995) Postoperative
Thammasitboon S, Rosen DA, Lutfi R et al. (2010) An insti- course and hemodynamic profile after the arterial
tutional experience with epidural analgesia in chil- switch operation in neonates and infants—a compari-
dren and young adults undergoing cardiac surgery. son of low-flow cardiopulmonary bypass and circula-
Pediatr Anesth 20:720–726 tory arrest. Circulation 92:2226–2235
Thompson LD, McElhinney DB, Findlay P et al. (2001) A pro- Wessel DL (2001) Managing low cardiac output syndrome
spective randomised study comparing volume- after congenital heart surgery. Crit Care Med 29:S220–
standardized modified and conventional ultrafiltration S230
in pediatric cardiac surgery. J Thorac Cardiovasc Surg Williams GD, Bratton SL, Riley EC et al. (1999) Coagulation
122:220–228 tests during cardiopulmonary bypass correlate with
Tobias JD, Berkenbosch JW, Russo P (2003) Recombinant blood loss in children undergoing cardiac surgery.
factor VIIa to treat bleeding after cardiac surgery in an J Cardiothorac Vasc Anesth 13:398–404
infant. Pediatr Crit Care Med 4:49–51 Wolf AR, Jackman L (2011) Analgesia and sedation after
Tortoriello TA, Stayer SA, Mott AR et al. (2005) A noninva- pediatric cardiac surgery. Pediatr Anesth 21:567–576
sive estimation of mixed venous oxygen saturation Wypij D, Newberger JW, Rappaport LA et al. (2003) The
using near-infrared spectroscopy by cerebral oximetry effect of duration of deep hypothermic circulatory
in pediatric cardiac surgery patients. Pediatr Anesth arrest in infants on late neurodevelopment: the
15:495–503 Boston Circulatory Arrest Trial. J Thorac Cardiovasc
Tweddell JS, Hoffman GM, Fedderly RT (1999) Surg 126:1397–1403
Phenoxybenzamine improves systemic oxygen deliv- Zhang S, Wang S, Li Q et al. (2005) Capillary leak syndrome
ery after the Norwood procedure. Ann Thorac Surg in children with C4A-deficiency undergoing cardiac
67:161–167 surgery with cardiopulmonary bypass: a double-blind,
Van den Berghe G, Wouters P, Weekers F et al. (2001) randomised controlled study. Lancet 366:556–562
Intensive insulin therapy in critically ill patients. N Engl Ziemer G, Karck M, Müller H et al. (1992) Staged chest clo-
J Med 345:1359–1367 sure in pediatric cardiac surgery preventing typical
Van der Vorst MM, Ruys-Dudok van Heel I, Kist-van Holthe and atypical cardiac tamponade. Eur J Cardiothorac
JE et al. (2001) Continuous intravenous furosemide in Surg 6:91–95
haemodynamically unstable children after cardiac Zimmermann AA, Burrows FA, Jonas RA et al. (1997) The
surgery. Int Care Med 27:711–715 limits of detectable cerebral perfusion by transcranial
Verbrugghe W, Jorens PG (2011) Neurally adjusted ventila- Doppler sonography in neonates undergoing deep
tory assist: a ventilation tool or a ventilation toy? hypothermic low-flow cardiopulmonary bypass.
Respir Care 56:327–335 J Thorac Cardiovasc Surg 114:594–600
289 III
Congenital
Anomalies of the
Heart and Great
Vessels
Contents
Surgery for Congenital
Heart Defects: A Historical
Perspective
Aldo R. Castañeda
References – 296
provided through the various research and Ph.D. 4. The emotional burden placed on child and
opportunities, the tools to pursue new knowl- parents living with the threat of another
edge. The cardiac surgical leadership at that time operation.
also included Dr. Richard L. Varco, a very erudite 5. The increased cost of two operations.
individual, intellectually versatile with a deduc-
tive intelligence, a synthesizer of knowledge, and a In the late 1960s and early 1970s, Horiuchi et al.
masterful surgeon. C. Walton Lillehei was a vision- (1963) and Hikasa et al. (1967) in Japan and Barrat-
ary surgeon, innovative, an unrelenting pioneer, Boyes et al. in New Zealand (1972) started to obtain
emotionally hardy, an iconoclast, and outwardly good results with primary repair in infants. In 1972,
flamboyant. Clearly the controlled cross-circula- at Boston Children’s Hospital, we followed this lead
tory idea carried his imaginative seal. John Kirklin and accumulated as well a large and satisfactory
accompanied by Mr. Richard Jones, an engineer experience with open heart operations in infancy
also from the Mayo Clinic, paid a visit to Gibbon’s (Castañeda et al. 1974). However, review of our
laboratory to study in detail his heart–lung data from the New England Infant Cardiac Program
machine. Kirklin and Jones succeeded in improv- (Report of the New England Regional Infant
ing and simplifying Gibbon’s screen oxygenator, Cardiac Program 1980) showed that deaths from
called from then on the Mayo–Gibbon pump oxy- complex congenital cardiac defects occurred
genator. Kirklin using this machine in early 1955 mostly during the first few weeks of life.
inaugurated the outstanding accomplishments in Based on this information and also after
cardiac surgery of the Mayo Clinic Group (Kirklin reviewing our midterm and late results with the
et al. 1955). atrial switch operations (Senning and Mustard) for
The first patient was a child with a ventricular transposition of the great arteries (TGA), we
septal defect. In the meantime Richard de Wall at became convinced that complex cardiac lesions
the University of Minnesota developed a very should, by preference, be repaired during the neo-
simple, easy to assemble and use, bubble oxygen- natal period in order to reduce early deaths and to
11 ator (DeWall 1956). For nearly 2 years, all open minimize secondary organ damage, including the
heart operations in the world were carried out in heart, lungs, and CNS. This early approach would
Minnesota in two institutions located 70 miles also allow normal postnatal development, such as
apart. Subsequently, and in great measure thanks physiologic myocardial hyperplasia/hypertrophy,
to the simplicity and low cost of the de Wall bub- coronary angiogenesis, and pulmonary angio- and
ble oxygenator, open heart centers spread rapidly, alveologenesis. Also preliminary laboratory exper-
first in the United States and then throughout the iments in 2-kg puppies subjected to 2 h of cardio-
world. Note also that at the beginning all open pulmonary bypass convinced us that the effects of
heart operations were indicated for repair of con- this 2-h period of extracorporeal bypass on both
genital cardiac lesions. During the 1950s and the formed elements of blood and the lungs (air
1960s, the prevailing impression was that open and fluid static volume–pressure measurements as
heart operations were poorly tolerated by the very well as alveolar bubble stabilizations) revealed only
young, as demonstrated by the cross circulation minor, transitory, and rapidly reversible changes
experience and other sporadic attempts at open that were well tolerated by these very young ani-
heart repair in infancy. Therefore, symptomatic mals (Visudh-Arom et al. 1970).
neonates and infants were first subjected to pallia- Consequently, on January 2, 1983, we per-
tive procedures, while intracardiac repair was formed our first arterial switch operation (ASO-
delayed until age 5–7 years. Jatene) in an 11-day-old neonate with TGA and an
This therapeutic policy presented serious dis- intact ventricular septum (Castañeda et al. 1984).
advantages, including: The operation consists of first transecting both
1. The need for two operations. great arteries that arise anomalously, the aorta from
2. The palliative operation did not always the right ventricle and the pulmonary artery from
accomplish its purpose. the left ventricle. Then, both left and right coronary
3. Iatrogenic damage was not uncomely arteries are explanted from the proximal aorta,
produced by the palliative procedure itself. now the neopulmonary artery, and are reimplanted
Chapter 11 · Aldo R. Castañeda
295 11
into the proximal neoaorta. The operation is com- Postoperatively these patients were kept in the
pleted by anastomosing the proximal neoaorta, pediatric cardiac care unit for 7–8 days in order to
with its implanted coronaries, to the distal ascend- undergo the second-stage operation that included
ing aorta and the proximal neopulmonary artery to removal of the pulmonary artery band, closure of
the distal main pulmonary artery. The interatrial the shunt, and an arterial switch operation.
communication, mostly enlarged by a balloon During this week-long interval, serial two-
atrial septostomy (Rashkind procedure) and that dimensional echocardiography showed that LV
permitted early postnatal survival and also serves mass increased similar to the rat by a mean of over
to decompress the pulmonary circuit, is closed as is 50 %, and cardiac catheterization revealed a mean
a ventricular septal defect, if present. left ventricular–right ventricular pressure ratio
The results both early and late of the ASO in increase from 0.5 ± 0.08 before to 1.04 ± 0.29
the neonate have been very satisfactory (Castañeda 7 days after the first stage. Of the first 49 patients
et al. 1994) (. Table 11.2). We had established to subjected to this two-stage approach, there was
our satisfaction that optimal management of dex- one death after the first stage and one more death
tro-transposition of the great arteries with an after the second stage (Jonas et al. 1989).
intact ventricular septum was achieved by an The obvious advantages of this rapid two-stage
ASO, performed ideally within the first 2 weeks of approach are the following:
life. However, a substantial number of patients 1. That a larger group of patients with dextro-
were referred to our institution for an ASO beyond transposition and an intact ventricular
the first month of life for various reasons, includ- septum can be offered this option beyond the
ing sickness or late referral by parents or physi- first month of life.
cians. Experimental work related to left ventricular 2. Both stages can be performed at one hospital-
hypertrophy had shown that LV muscle mass ization and offer also important psychologi-
increased very rapidly following experimental cal, logistic, and financial advantages.
coarctation of the aorta in rats. In fact within
7 days, the LV muscle mass increased by more Clearly, there are many other important areas
than 40 %. Beyond that time there was no appre- of development within pediatric cardiac surgery
ciable continued increase in hypertrophy. This over these last 66 years, which within the limita-
rapid response of the myocytes proved to be due tions of this chapter cannot all be mentioned, but
to a fivefold increase in c-fos and c-myc myosin they will in part be dealt with in the specific chap-
heavy chain mRNA and HSP 70 within 1–3 h after ters of this book.
the applied pressure stimulus (Izumo et al. 1988). In . Table 11.3 these important areas are enu-
Based on these experimental data, we initiated merated. Finally, outlined in . Table 11.4 are the
a clinical series in which the preparatory first-
stage operation consisted of pulmonary artery
banding and a modified Blalock–Taussig shunt. ..Table 11.3 Additional areas of important
developments in pediatric cardiac surgery
..Table 11.2 Transposition of the great arteries: The dispensable right ventricle (Fontan)
arterial switch operation 1983–1997 Conduits: valved and nonvalved
Anomalous Pulmonary
Venous Connections and
Congenital Defects of the
Atria, the Atrioventricular
Septum, and the
Atrioventricular Valves
Vladimir Alexi-Meskhishvili, Axel Unbehaun, and Roland Hetzer
References – 409
304 V. Alexi-Meskhishvili et al.
His-bundel Insertion of
septal tricuspd
valve leaflet (lower
Septum atrioventriculare
border of Koch’s triangle)
Pars membranacea
AV-node
Tendon of Todaro
(upper border of Coronary sinus
Koch’s triangle) (Base of Koch’s triangle)
Valvula Thebesii
V. cava superior
Ostium, Valvula
V. cavae inferioris
(Eustachii)
Koch’s triangle
of the limbus of the fossa ovalis, which surrounds 12.1.1.5 Forms of Atrial Septal Defect
the valve of the fossa ovalis (septum primum) Atrial septal defects occur at several sites within the
superiorly, anteriorly, and posteriorly; the broad- atrial septum, but only atrial communications occur-
based atrial appendage; the Eustachian valve at ring in the region of the fossa ovalis or so-called
the orifice of the inferior vena cava; the Thebesian secundum defects—as related to the embryonic
valve at the orifice of the coronary sinus; the mus- ostium secundum—are true ASDs because the inter-
cle band known as the crista terminalis, which atrial septum is only slightly larger than the fossa
separates the trabeculated from the nontrabecu- ovalis itself (Silverman 1997; Wilcox et al. 2004).
lated portion of the atrium; and the ostium of the Many classifications for atrial septal defects
tricuspid valve. have been suggested. In the classification of
It has been suggested that there are three Edwards, the following types of ASD are recog-
internodal tracts in the right atrium connecting nized (Edwards 1953):
the sinus node and the atrioventricular node: 55 Secundum type within the fossa ovalis (60 %
an anterior, middle, and posterior bundle of cases)
(James 1963; Sealy et al. 1969). This is a contro- 55 Sinus venosus type, often associated with
versial issue. According to Anderson and anomalous return of the right pulmonary
Becker (1980), there are parallel and closely vein(s) (10 %)
packed bundles of working myocardium in the 55 Ostium primum defects below the fossa ova-
crista terminalis and in the anterior limb of the lis (today part of AV septal defects)
fossa ovalis, which could be the morphological 55 Raghib-type ASD (with absent coronary sinus
counterpart of the preferential pathways dem- in the presence of a persistent left superior vena
onstrated by the electrophysiologists. However, cava, thereby connecting to the left atrium)
it has not been possible to identify any such 55 Multiple coalescent defects, essentially form-
bundles in the muscular bands (Wilcox et al. ing a common atrium
2004). Experimentally it was shown that dam-
age to the crista terminalis can be a substrate A more recent classification of ASD suggested
for the development of atrial arrhythmias by the Congenital Heart Surgery Nomenclature
12 (Gandhi et al. 1996). and Database Project (. Fig. 12.2) identifies the
1° CS
CS CS
FO 2° FO
SCV IVC SCV IVC SCV IVC
RUPV RLPV
RAA TV RAA TV
CS
FO Common
SCV SV SV IVC SCV Atrium IVC
RUPV RLPV
..Fig. 12.2 Common types of atrial septal defects according to the Congenital Heart Surgery Nomenclature and
Database Project. CS coronary sinus, FO fossa ovalis, IVC inferior vena cava, RAA right atrial appendage, RLPV right lower
pulmonary vein, RUPV right upper pulmonary vein, SV sinus venosus, SVC superior vena cava, TV tricuspid valve
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
307 12
following main types of atrial septal defects The exact nature of the development of sinus
(Jacobs et al. 2000b): venosus defects remains controversial.
55 Common atrium (single atrium): complete
absence of the intra-atrial septum. The term This type of ASD would more properly be
«single atrium» is applied to defects with termed a «sinus venosus defect» because the lesion
completely normal atrioventricular valves represents an intra-atrial communication rather
and «common atrium» to defects accompa- than a defect of atrial septal tissue. Anderson and
nied by malformation of the atrioventricular colleagues suggested that the superior sinus veno-
valves. sus defect results from a failure of infolding of the
55 Coronary sinus ASD (synonym: «unroofed atrial walls between the superior vena cava and the
coronary sinus») defines a defect of the wall right pulmonary vein (Al Zaghal and Anderson
separating the left atrium from the coronary 1998; Anderson et al. 1995). Van Praagh et al.,
sinus, often allowing blood to drain from the however, postulated that sinus venosus defects
left atrium via the coronary sinus and the result from a deficiency in the common muscular
coronary sinus ostium to the right atrium. wall that normally separates the right pulmonary
Coronary sinus defects make up a small sub- veins from the superior vena cava, resulting in
set of ASDs and are often associated with a «unroofing» of the right pulmonary veins allowing
persistent left superior vena cava draining them to drain into the superior vena cava or the
anatomically into the coronary sinus, here right atrium (Van Praagh et al. 1994).
functionally into the left atrium, leading to
cyanosis if ASD is closed. 12.1.1.6 Surgical Indications
55 ASD, patent foramen ovale: a small interatrial Although the presence of an ASD does not represent
communication in the region of the foramen a life-threatening condition, there is a danger of
ovale characterized by no absence of septal tis- complications and chronic sequelae developing with
sue of the septum primum or septum secun- time, and it is generally accepted that all ASDs should
dum. be closed either surgically or by an interventional
55 ASD, primum: a usually crescent-shaped procedure before the patients reach school age.
ASD in the inferior portion of the atrial sep- Today candidates for surgical closure are the
tum directly above the AV valve level. patients with large secundum ASDs deemed not
55 ASD, secundum: an ASD confined to the area amenable to transcatheter closure, patients with
of the fossa ovalis. It is not necessarily to be sinus venosus and coronary sinus defects (LSVC),
distinguished from a wide-open foramen and those with primum-type ASD as well as com-
ovale («stretched foramen»). The most com- mon atrium.
mon etiology is a defect of the septum pri- This recommendation of closure of ASD before
mum, but an absence of tissue of the septum school age includes asymptomatic ASD. Early ASD
secundum may also contribute. An ostium closure is indicated to avoid atrial fibrillation, pulmo-
secundum defect may be associated with an nary hypertension, chronic right ventricular over-
aneurysm of the interatrial septum. This is load, mitral valve incompetence, and the risk of
thought to be due to excess tissue of the fossa paradox systemic embolization (Cheng et al. 1994;
ovalis valve. It may be accompanied by mitral Mas et al. 2001). Cardiac catheterization is rarely nec-
valve prolapse or atrial arrhythmias. essary. Echocardiographic evidence of right atrial and
55 ASD, sinus venosus: an interatrial communi- in particular right ventricular enlargement is usually
cation in the region of the right horn of the considered sufficient evidence of a clinically signifi-
sinus venosus, usually in the area of the cra- cant left-to-right shunt representing an indication for
nial sinoatrial junction close to the superior surgical closure of the ASD. Surgery is ideally per-
vena cava orifice, but more rarely posterior to formed in children aged 2–4 years, when the surgical
the fossa ovalis and without a connection to a risk is very low. However, if symptoms of congestive
cava orifice or in the region of the caudal heart failure occur and cannot be effectively con-
sinoatrial junction near the inferior vena cava trolled, the operation may be indicated even in early
orifice. The superior sinus venosus defect is infancy. In adult patients closure of ASDs results in
usually associated with anomalous drainage clear clinical improvement because of the reduction
of the right upper and occasionally also of in the volume load of the chambers (Thilén and
other pulmonary veins into the right atrium. Persson 2006). For example, it has shown that mitral
308 V. Alexi-Meskhishvili et al.
valve prolapse was reduced or eliminated in 80 % of (Barnard and Speake 2004; Olinger 1995; Svenarud
patients because of normalization of the left ventricu- et al. 2003). More recently, however, in a double-
lar geometry (Schreiber et al. 1989). In adult patients blind, randomized clinical trial of neurocognitive
with an ASD and atrial fibrillation, closure of the ASD effects, a benefit of carbon dioxide insufflation in
should be combined with antiarrhythmic surgery open-chamber cardiac surgery could not be shown
(atrial ablation). in an adult population (Chaudhuri et al. 2012).
a b
..Fig. 12.5 a, b Continuous double-suture technique for the closure of small defects
310 V. Alexi-Meskhishvili et al.
a b
..Fig. 12.7 a, b Closure with untreated autologous pericardium and nonabsorbable sutures
a b
malaise. In young children, symptoms may be defects are, strictly speaking, not a form of ASD
nonspecific and include irritability and decreased because they occur outside the boundaries of the
appetite. atrial septum and are thus interatrial communica-
tions but not true ASD. Operating on a sinus
kInterventional closure of atrial septal venosus defect with anomalous pulmonary
defects venous drainage obliges the surgeon to reform the
The transcatheter approach to ASD closure has entire superior cavoatrial junction, taking the
gained acceptance for both pediatric and adult utmost care to avoid damage to the sinus node
patients (Berger et al. 1999). Benefits of the trans- and thus postoperative arrhythmic sequelae
catheter approach include its minimal invasive- (Anderson et al. 1983).
ness, the avoidance of both thoracotomy and
cardiopulmonary bypass, and the relatively short 12.1.2.2 I solated Partial Anomalous
recovery time. Potential drawbacks are residual Pulmonary Venous Drainage
shunting around the device and displacement of The superior vena cava cannula must be placed
the device requiring surgical intervention (Bohm cranial to the anomalous connection of the right
et al. 1997). Interventional closure is not suitable superior pulmonary vein. After atriotomy, the
when the rim of the defect is not large enough to atrial septum, namely, the cranial part of the sep-
properly seat the device. Further, long-term safety tum primum in the fossa ovalis, is resected. The
concerns have been raised, and there is a need for edges of the created ASD caudally and the cranial
specific technical expertise and equipment. circumference of the abnormal right upper pul-
monary vein must be incorporated into the patch
closing suture so that, during defect closure with a
Secundum ASDs are currently the only pericardial patch, correction of the anomaly and
subtype of ASD that are amenable to this redirection of the right superior pulmonary vein
type of treatment, which can be performed in into the left atrium are achieved (. Fig. 12.9).
the catheterization laboratory without It is often assumed that nonendothelialized
fluoroscopy and with echocardiographic edges of the created ASD can lead to thrombus
12 monitoring alone (Ewert and Berger 2003). formation and systemic embolization. However,
experience from the field of heart transplantation
contradicts this assumption.
12.1.2 Partial Anomalous
Pulmonary Venous Drainage 12.1.2.3 Partial Anomalous
Pulmonary Venous Drainage
12.1.2.1 General Comments and Sinus Venosus Defect
Partial anomalous pulmonary venous drainage On rare occasions the sinus venosus defect is so
(PAPVD) may occur as an isolated anomaly in small that there will not be free drainage of the
patients with intact atrial septum or in patients superior pulmonary vein after closure of the defect.
with ASD. In patients with upper sinus venosus In such cases the defect is enlarged by an incision
defects, the pulmonary vein is often connected into the atrial septum toward the fossa ovalis, and
high on the superior vena cava. Sinus venosus then a patch is placed. There are different methods
a b c
..Fig. 12.9 a–c Pericardial patch closure of the atrial septal defect created and redirection of right upper pulmonary
vein in isolated partial anomalous pulmonary venous drainage
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
313 12
a b c
..Fig. 12.10 a–c Redirection of the pulmonary veins in large sinus venosus defect
of correcting a sinus venosus defect and partial cavoatrial junction or even both the caval and pul-
anomalous pulmonary venous drainage: monary venous pathways. In such cases, after tran-
55 Simple patch closure of the defect (primary section and reimplantation of the superior vena
or enlarged defect) cava, a cavoatrial anastomosis can be created by
55 Patch closure of the defect and second patch patching the sinus venosus defect and the orifice of
for augmentation of the superior vena cava— the SVC. This represents a very useful technique
right atrial junction (Gustafson and Warden 1995; DiBardino et al.
55 Patch closure of the defect incorporating the 2004; Agarwall et al. 2011; Warden et al. 1984)
SVC orifice with distal superior vena cava and was originally proposed by John Lewis in 1958
reimplantation into the right atrium (Warden (Benvenuto and Lewis 1959; Ehrenhaft et al. 1958;
technique) Groves 1967). The superior vena cava is mobilized
55 Transcaval closure to make room for the anastomosis to the right
atrial appendage and is transected above the inser-
The choice of the method depends on the tion of the uppermost pulmonary vein; its cardiac
individual anatomy of the patient. There is little end is then closed with a continuous suture. The
data on the long-term functional outcomes fol- orifices of azygos vein and abnormal pulmonary
lowing repair of these defects. veins have clearly to be distinguished, the azygos
If the superior vena cava is large, the right vein usually closed and divided for better mobili-
atrium is opened in the usual manner. The open- zation. Prior to completing this anastomosis, it is
ing of the pulmonary veins into the superior vena critically important to identify and resect any
cava is visualized, and the veins are completely potentially obstructive trabeculae carneae of the
redirected into the left atrium by roofing the pul- pectinate muscles within the right atrial append-
monary vein, namely, closing the interatrial com- age (DiBardino et al. 2004). The sinus venosus
munication using an autologous pericardial patch defect is closed with a pericardial patch in such a
(. Fig. 12.10). way that the stump of the superior vena cava and
Incision through the cavoatrial junction must the pulmonary veins can drain into the left atrium
be avoided because the sinus node and its arterial in an unobstructed manner (. Figs. 12.11 and
supply may be compromised during repair 12.12). Caval division techniques (Said et al. 2011),
(Anderson et al. 1979a, b, 1983; Buz et al. 2008). If a transcaval technique (Nassar et al. 2012), and a
for the atriotomy an incision in the cranial area is pulmonary vein reimplantation technique
selected that runs parallel to the course of the (Aramendi et al. 2011) were also reported.
superior vena cava directly anterior to the anoma-
lously connected pulmonary veins, no sinus node 12.1.2.4 Scimitar Syndrome
arteries can be damaged (R. Van Praagh personal Complete or partial drainage of the right pulmo-
communication). nary veins into the inferior vena cava (scimitar
In the case of a smaller superior vena cava and syndrome) is a rare anomaly, first described in
rather distant anomalous pulmonary venous 1836 (Cooper 1836). Scimitar syndrome is often
drainage into the SVC, placement of the patch to associated with aplasia of parts or the whole of
divert the pulmonary venous blood into the left the right lung. In scimitar syndrome drainage, the
atrium may result in significant stenosis of the right pulmonary veins can also drain into the
314 V. Alexi-Meskhishvili et al.
a b
..Fig. 12.11 a–c Warden’s technique for sinus venosus defect repair. a Anomalous pulmonary venous connection to
12 the superior vena cava (SVC) well above the cavoatrial junction. An associated high sinus venosus defect is shown by
the dashed lines. b First stage of repair: a superficial purse-string suture is placed around the cephalad SVC, and partial
cardiopulmonary bypass is instituted with venous return from the inferior vena cava (IVC) only. The SVC is mobilized, the
azygos vein transected. c Total cardiopulmonary bypass is established by introducing a cannula through the lateral wall
of the right atrium and the tip of the right atrial appendage into the SVC. The SVC is transected, the caudal stump is
oversewn, and anastomosis of the cephalad end of the SVC to the right atrial appendage is begun. It often requires
patch enlargement
..Fig. 12.12 a–c Warden’s technique for sinus venosus defect repair (continued). a The cavoatrial anastomosis is com-
plete. The right atriotomy exposes the sinus venosus defect. b The inferior margin of the defect is sutured to the anterior
and lateral margins of the intracardiac SVC orifice. c Completed repair. Anomalous pulmonary venous flow has been
directed through the proximal SVC and across the septal defect into the left atrium. Flow from the SVC into the right
atrium is reconstructed by the cavoatrial anastomosis (Warden et al. 1984)
The infantile scimitar syndrome, in addition 55 A milder form presenting in late childhood
to its high incidence of ASD, is associated with a or adulthood that is frequently asymptomatic,
large number of further cardiovascular anomalies with diagnosis often being made incidentally
(Brown et al. 2003): because of radiographic abnormalities
55 Ventricular septal defect
55 Patent ductus arteriosus Botalli Surgery for scimitar syndrome was first per-
55 Hypoplastic aortic arch formed in 1950 by Drake and Lynch (1950). In a
55 Aortic isthmus stenosis patient with recurrent right-sided pneumonia
55 Tetralogy of Fallot and a scimitar vein draining the bronchiectatic
55 Anomalous origin of the left coronary artery lower right lung, they resected the right lower
55 Truncus arteriosus communis lung lobe, with good results.
The first corrective surgical procedure in a
There are two forms of scimitar syndrome that patient with scimitar syndrome and an ASD was
are clearly distinguishable in terms of clinical pre- performed in 1956 by Kirklin using cardiopul-
sentation (Gudjonsson and Brown 2006): monary bypass (Kirklin et al. 1956). The scimitar
55 An infantile syndrome associated with signif- vein was anastomosed to the right atrium in
icant mortality proximity to the ASD, and this portion of the
316 V. Alexi-Meskhishvili et al.
a b
..Fig. 12.13 a, b Correction of scimitar syndrome. a Correction by reimplantation of the scimitar vein into the right
atrium and redirection by creation of a tunnel by suturing of a short baffle. b Correction by creation of a tunnel from the
inferior vena cava to the ASD by suturing of a long baffle
..Fig. 12.15 a Isolated anomalous drainage of the left pulmonary veins into the innominate vein. b Anastomosis of
the anomalously draining left pulmonary veins with the left atrial appendage
pulmonary sequestrations (persistent infections) or entrance into the innominate vein to create a long
if the attempt at correction has failed (Huddleston tunnel for the anastomosis to the left atrium. The
et al. 1999; Thibault et al. 1995; Torres and Ca 1993). pulmonary venous stem is opened longitudinally
to receive an anastomosis with large lumen. The
12.1.2.5 Anomalous Drainage left atrial appendage is amputated, and the anasto-
of the Left Pulmonary Veins mosis is carried out with a continuous 7/0 or 6/0
into the Innominate Vein monofilament suture (see . Fig. 12.15). In some
In its isolated form, this anomaly is very rare cases patch enlargement of this anastomosis with
(see . Fig. 12.15a). Partial left drainage of a small autologous pericardium is necessary.
upper lobe vein into the innominate vein may be
left uncorrected, but if all the left pulmonary
veins drain abnormally and create similar hemo- 12.1.3 Anomalous Drainage
dynamic consequences to those of an ASD, surgi- of the Caval Veins
cal correction is indicated. In asymptomatic
infants and small children, the operation can be 12.1.3.1 Definition and General
postponed to grow into a size with less postopera- Comments
tive risk of stenosis of the anastomosis between Anomalies of the systemic venous return that
the left pulmonary veins and the left atrial drains to the heart may occur in isolation or in
appendage. association with other congenital heart defects
The operation is conducted using cardiopul- (DeLeval et al. 1975; Gandy and Hanley 2006).
monary bypass and cardioplegic cardiac arrest. Many of the common defects are clinically of minor
The phase of cardioplegia is important to obtain a significance when found in isolation. There are
bloodless operative field, which facilitates the cre- many rare anomalies that may or may not become
ation of a wide, nonconstrictive anastomosis clinically significant. However, systemic venous
between the left pulmonary venous stem and the anomalies are of great importance in patients with
left atrial appendage. the different forms of a univentricular heart and
The persisting left upper caval vein should be may complicate the creation of cavopulmonary
doubly ligated and may be transected at its connections as well as the later Fontan completion.
318 V. Alexi-Meskhishvili et al.
The presence of a systemic venous anomaly may In situs solitus a left superior vena cava is con-
necessitate modifications of the implantation tech- nected to the coronary sinus, and, in the absence
niques for heart transplantation or combined heart of other cardiac defects, this has no functional
and lung transplantation. The classification of these impact. Its importance for surgery is that it must
anomalies is primarily descriptive (Gaynor et al. be taken into consideration in the technique of
2000; . Figs. 12.16, 12.17, and 12.18). venous cannulation or intermittent occlusion and
causes difficulties if minimally invasive surgical
12.1.3.2 Anatomy approaches are used.
Isolated anomalies of the caval veins are overall The most common malformation of clinical sig-
rare, but they are frequently associated with other nificance is drainage of the left superior vena cava
congenital heart defects, especially in the pres- into the left atrium. In the Raghib-type ASD (Raghib
ence of heterotaxy and isomerism. et al. 1965), the coronary sinus roof is completely or
12
..Fig. 12.16 Anomalies of the superior vena cava. Top row: right superior vena cava to right atrium (left); right supe-
rior vena cava through the coronary sinus to left atrium (center); right-sided superior vena cava to left atrium (right).
Middle row: Left superior vena cava to left-sided atrium (completely unroofed coronary sinus; left); left superior vena
cava through the coronary sinus to right atrium (center); left vena cava to right atrium (right). Bottom row: bilateral supe-
rior vena cava: right superior vena cava to right atrium, left superior vena cava to left atrium (completely unroofed coro-
nary sinus; left), bilateral superior vena cava: right upper vena cava to right atrium, left superior vena cava through the
coronary sinus to right atrium (center), bilateral superior vena cava: right upper vena cava through the coronary sinus to
left atrium, left superior vena cava to left atrium (right)
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
319 12
This can, if not recognized, lead to inadequate total
cavopulmonary connection during performance of
the Fontan operation or ASD closure, with resulting
residual or de novo right-to-left shunt.
12
..Fig. 12.18 Anomalies of the hepatic veins. Upper row: right lower vena cava and separate entry of hepatic veins into
left atrium (left); right inferior vena cava and separate entry of hepatic veins into right atrium (center); right inferior vena
cava and separate entry of hepatic veins into coronary sinus (right). Middle row: left inferior vena cava into the left
atrium and separate entry of hepatic veins into right atrium (left); left inferior vena cava and separate entry of hepatic
veins into left atrium (center); left inferior vena cava and separate entry of hepatic veins into coronary sinus both into
the left atrium (right). Bottom row: interrupted right lower vena cava with azygos continuation into right upper vena
cava and separate entry of hepatic veins into right atrium (left); interrupted right inferior vena cava with azygos continu-
ation into right upper vena cava and separate entry of hepatic veins into left atrium (second from left); interrupted left
inferior vena cava with hemiazygos continuation into left superior and separate entry of hepatic veins into left atrium
(second from right); interrupted left inferior vena cava with hemiazygos continuation into left superior vena cava and
separate entry of hepatic veins into right atrium (right)
venous pressure does not exceed 15 mmHg and et al. 1998a, b, c); in some cases spontaneous
the coronary sinus and the pulmonary venous enlargement, actually caused by elevated LSVC
drainage are not compromised (Laks 1994). In pressures after ligation, may be expected.
cases of hypoplastic innominate vein, enlarge- Reimplantation of the left superior vena
ment of the innominate vein between the right cava into the right atrium or the pulmonary artery
brachiocephalic vein and the left jugular vein with is practiced successfully (Gandy and Hanley 2006;
a pericardial patch and ligation of the left superior Liang et al. 1996; Quarti et al. 2005). There are,
vena cava can be successfully performed (Van Son however, concerns about the potential early
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
321 12
a b
..Fig. 12.19 a, b Procedure for intra-atrial redirection of anomalous LSVC to left atrial connection
s tenosis of the anastomosis and thrombus forma- superior vena cava, so as to redirect the blood
tion and the possibility of reverse flow in the case from the anomalously draining left superior
of elevated pulmonary arterial pressure at rest or vena cava to the front side of the patch. The
during physical exertion in the later course. suture line then runs further to the roof of the
Intra-atrial redirection of the left superior left atrium and reaches the rim of the atrial
vena cava is the preferred option. There are two septum laterally, thus completely separating
possible techniques (Gandy and Hanley 2006; the systemic venous return (. Fig. 12.19).
Quaegebeur et al. 1979):
55 Roofing procedure. This operation can be rec-
ommended if the roof of the coronary sinus is kAnomalous drainage of the inferior vena
absent. After the establishment of cardiopul- cava into the left atrium
monary bypass and cardioplegic cardiac As an isolated anomaly, this defect occurs very
arrest, the right atrial incision is performed. rarely (Zenker et al. 1965) and usually presents in
A separate cannula is introduced into the patients with heterotaxy syndrome and atrial
innominate vein orifice through the coronary isomerism (Rubino et al. 1995; Yun et al. 2006a).
sinus orifice. If there is no additional ASD, To connect the heart–lung machine, separate can-
the interatrial septum is incised. The repair is nulation of the anomalous vein is necessary. For
started at the point of entrance of the left the Fontan operation, these veins can be con-
superior vena cava into the left atrium. A nected to total cavopulmonary connection to pre-
pericardial patch is used to cover the defect of vent the development of pulmonary arteriovenous
the coronary sinus roof. With a separate peri- fistulas (Shah et al. 1997). If an extracardiac tun-
cardial patch, the ASD is then closed. nel is created during the Fontan operation, the
55 Intra-atrial baffle procedure. Because of the risk hepatic veins can be incorporated into the cavo-
of obstruction of the left pulmonary veins and pulmonary connection using the atrial flap tech-
the mitral valve and also the risk of creating nique (Shah et al. 1997) or separately connected
too small a tunnel, the preferred method is the to the extracardiac conduit using an additional
intra-atrial redirection, which is carried out in tube graft (Lee et al. 2002). If an intracardiac lat-
such a fashion that the anomalous vein drains eral tunnel operation is performed, these veins
into the right atrium. If there is a secundum can be incorporated in the created lateral canal
ASD, the patch is inserted between the two (Gandy and Hanley 2006; Shah et al. 1997).
atrioventricular valves on the rim of the atrial In isolated cases repair consists of redirecting
septum or, with an ostium premium defect, the direct hepatic veins or the inferior vena cava into
along the anulus of one of the atrioventricular the right atrium by creating an intracardiac tunnel.
valves. The suture line is then carried down If there is an ASD, the pericardial patch must be
toward the posteroinferior corner of the left inserted in such way that the blood flow leads into
atrium and around the entrance of the left the right atrium (Derra et al. 1965; . Fig. 12.20).
322 V. Alexi-Meskhishvili et al.
a b c
..Fig. 12.20 a–c Redirection of the blood flow into the right atrium for anomalous IVC or hepatic veins to left atrial
connections
life. They stressed the importance of taking From the surgical point of view, we do not
enough tissue from the tricuspid valve to create consider the question of whether the cleft found
an adequate left AV valve. with complete AVSD is a normal commissure to
The most controversial issue in the surgical be of great importance. It is far more important to
treatment of the complete AVSD was whether it note that a partially or completely open cleft is the
is necessary to close a so-called mitral cleft. The main cause of subsequent valve incompetence
issue remains controversial for patients with and that cleft closure represents the only means
parachute «mitral valve» (Sharma et al. 2013). for achieving long-term valve competence in
This so-called mitral cleft (better referred to as such cases. The incidence of late operation for late
a cleft of the left AV valve) is referred to by severe left AV valve incompetence in patients
some authors as the «zone of apposition» with nonclosed cleft was 14–26 % (McGrath and
(Wetter et al. 2000). The opinion that the cleft Gonzales-Lavin 1987; Pozzi et al. 1991: Ross et al.
should be left intact is not new and was pro- 1991). Nowadays surgeons must be advised
posed for patients with incomplete AVSD many always to close a left AV valve cleft to approxi-
years ago (Dubost and Blondeau 1959; Van mate to the age-related normal diameter of the
Mierop and Alley 1966). In the late 1970s, the mitral valve (Crawford 2007; Litwin et al. 2007;
concept of the tri-leaflet mitral valve was intro- Nunn 2007; Wetter et al. 2000). It should be
duced by Carpentier, Becker and Anderson remembered that closure of the zone of apposi-
(Becker and Anderson 1982; Carpentier 1978). tion (cleft) does not create a leaflet comparable to
Carpentier believed that the left AV valve func- the aortic leaflet of the normal mitral valve
tions best when reconstructed as a tri-leaflet (Wilcox et al. 2004).
valve (Carpentier 1987). This led some sur- From the end of the 1960s and the beginning
geons to leave the cleft open during total repair of the 1970s, the surgical indication for AVSD
of the complete AVSD (McGrath and Gonzales- repair was extended to include children in the first
Lavin 1987; Ross et al. 1991). year of life (Bailey et al. 1976; Barratt-Boyes 1973).
At the same time, some proponents of the tri- Since then the age at which complete AVSD is
leaflet mitral valve concept maintained that electively corrected has dropped. In many centers
12 although it was impossible to produce a bileaflet the mean age of patients at the time of complete
valve which structurally approximated the mitral AVSD repair is around 4 months (Backer et al.
valve of a normal heart, «closure of the ‘cleft’ in 2007; Singh et al. 2006). More recent recommen-
some cases may be the only way to produce a dations are based on operating before pneumonia
competent atrioventricular valve» (Becker and occurs and before the development of irreversible
Anderson 1982). pulmonary hypertension and the complications
of congestive heart failure and AV valve insuffi-
ciency. The ability to perform safe surgery in early
The left AV valve reconstructed during repair infancy has been facilitated by improvements in
of AVSD is not a mitral valve. anesthesia, cardiopulmonary bypass, and postop-
erative management. In most centers the preop-
erative diagnosis is made on the basis of
The main goal of left AV valve reconstruc- transthoracic echocardiography only. Cardiac
tion in patients with complete AVSD is to pro- catheterization is rarely used. The ideal age for the
duce a competent and nonstenotic systemic correction of complete AVSD appears to be
atrioventricular valve. Criticism of the tri-leaf- between 3 and 6 months (Backer et al. 2007). The
let concept has increased over the past 25 years ideal age for correction of an incomplete, non-
as surgeons have noted that the high rate of complicated AVSD is about 2 years.
reoperations necessitated by late AV valve If patients with a partial AVSD present with
insufficiency after total correction of complete symptoms very early in life, complex anatomy
AVSD could be mainly attributed to nonclosure should be suspected, including multilevel left-
or incomplete closure of the cleft or avulsion of sided hypoplasias, which may pose a significant
the suture used to close the cleft (Alexi- challenge to management and suggest a poor
Meskishvili et al. 1996, 1997; Ten Harkel et al. prognosis (Giamberti et al. 1996; Manning
2005; Wetter et al. 2000). 2007).
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
325 12
12.1.5.3 Surgical Anatomy common posterior leaflet, the Rastelli classification is
Knowledge of the main anatomic characteristics applied to the behavior of the superior leaflets only: if
of AV septal defects is essential not only to define they are divided from each other, type A is present; if
the diagnosis but also for the surgical treatment. there is a so-called common superior leaflet without
These characteristics are (Anderson and Ho 1989; attachments to the interventricular septum, the type
Piccoli et al. 1979; Wilcox et al. 2004): is C. With type B, which is extremely rare, tendinous
55 The absence of the atrioventricular septum threads attached to the left superior leaflet lead to an
55 The absence of the normal «wedging» of the anomalous papillary muscle in the right ventricle.
aorta between the mitral valve and the tricuspid In line with modern concepts (Anderson et al.
valve, which is partially responsible for the 1985; Wilcox et al. 2004), the common anterior
presence of a common atrioventricular valve (or superior) and posterior (or inferior) leaflets
55 Abnormal relationship between the left ven- are to be seen as «bridging leaflets» that override
tricular outflow tract (LVOT) and the the ventricular septal defect to a greater (type C)
«unwedged» aortic valve or lesser (type A) extent. The greater variance is in
55 Abnormal relationship between the inflow the relationship of the anterior (superior) leaflet
and outflow tract of the left ventricle in favor to the bridged septum, so that the classification is
of the outflow tract, which results from the largely made in this respect.
above features The size of the interventricular communica-
55 Posterior displacement of the conduction sys- tion, the ventricular septal defect, is also very vari-
tem due to the absence of the atrioventricular able. Sometimes there is only a small gap between
septum the left superior and inferior leaflet. Frequently,
To successfully reach the goals of surgery, however, the defect reaches over the whole extent
secure defect closure without AV block, and com- of the superior leaflet, up to the aortic valve, just
petent AV valves, the pathomorphology of the AV as it can extend below the inferior leaflet.
valves and the anatomical course of the conduc- In addition to the variants described so far, we
tion system are of utmost importance (. Fig. 12.21). should also mention those that result from the rela-
There are significant differences between the AV tive position of the common AV valve in relation to
valves of a normal heart and of a heart with partial or the ventricular septum. As well as a balanced form
total AVSD (see . Fig. 12.21a–c). As in the normal (similar size right and left ventricle with the com-
heart (see . Fig. 12.21a), the heart with a partial mon AV valve lying centrally above them), a right-
AVSD has two distinct AV valve orifices, while the dominant form is found, which is defined by an
individual leaflets of these AV valves differ: both the enlarged right ventricle, small left, and displace-
tricuspid valve and the mitral valve are not to be ment of the AV valve to the right. By analogy the
regarded as such in the presence of an AVSD but left-dominant form has the opposite features.
rather as right and left AV valves. In principle these Owing to the absence of the atrioventricular
are double-leaflet valves with the corresponding com- septum, hearts with AVSD show displacement of
missures (Anderson and Ho 1989). The resulting six the conduction system in comparison with nor-
leaflets are named according to their position: for the mal hearts. This concerns first and foremost the
right AV valve, right superior (RS), right lateral (RL), AV node, which is normally situated in the apex
and right inferior (RIS), and for the left AV valve, left of the Koch triangle but in the heart with an AVSD
superior (LS), left lateral (LL), and left inferior (LIS) is displaced in posterior direction and is found
(see . Fig. 12.21b). When a complete AVSD is pres- between the insertion line of the posterior (infe-
ent, there is a single common AV valve ostium (see rior) common leaflet and the anterior area of the
. Fig. 12.21c). This common AV valve usually con- coronary sinus (. Fig. 12.22).
sists of five leaflets, rarely of six, and their nomencla- In comparison with the normal heart (see
ture corresponds to that used with a partial AVSD. . Fig. 12.22a), the crest of the ventricular septum
It was recognized that the classification of total and also the entire axis of the conduction system
AVSD into types A, B, and C by Rastelli et al. (1966) is, like the AV node, displaced in posterior direc-
did not exactly reflect the actual anatomic features, tion (see . Fig. 12.22b; Mitchell et al. 2007).
but for surgical purposes the detailed description of Surgical view of conduction system is presented
the individual morphology in terms of this classifica- on . Fig. 12.22c, d.
tion has proven its worth. Assuming that the inferior AVSDs occur not only in isolation but also in
portions of the left and right AV valve usually form a combination with other heart defects, e.g., with
326 V. Alexi-Meskhishvili et al.
tetralogy of Fallot, transposition of the great ate hypothermia and with cardioplegic heart
arteries, and total anomalous pulmonary venous arrest to protect the myocardium (Crawford 2007;
drainage. The surgical procedure must be tailored Litwin et al. 2007; Manning 2007). For operations
to addressing both malformations. In such com- on infants, deep hypothermic cardiac arrest with
binations the interventricular communication is or without cardioplegia is an option.
particularly large, so that the patch used for clo-
sure must be large enough and must be cut to cor- kAccess and general surgical technique
respond to the particular form. The standard access is by median longitudinal
sternotomy. In large patients the right-sided
kSpecifics of extracorporeal circulation anterolateral thoracotomy in the fourth or fifth
Today intracardiac correction procedures are per- intercostal space may be selected. Today the lim-
formed using cardiopulmonary bypass in moder- ited lower sternotomy and the posterolateral
a b
Valva tricuspidalis
RLL
RSL
Cuspis anterior
Cuspis posterior
RIL
Cuspis septalis
LSL
Cuspis anterior
12 LIL
Valva mitralis
LLL
Cuspis posterior
d e
c
RLL
RSL
RIL
LIL
LSL
LLL
..Fig. 12.21 a–e Morphology of the AV valves and course of the conduction system. LIL left inferior leaflet, LLL left
lateral leaflet, LSL left superior leaflet, RIL right inferior leaflet, RLL right lateral leaflet, RSL right superior leaflet. a Normal
heart with tricuspid and mitral valve. b Partial AVSD, c total AVSD (Rastelli type A), d total AVSD (Rastelli type B), and
e total AVSD (Rastelli type C)
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
327 12
a b
«Risky» area
Penetrating bundle
Line of tricuspid
valve attachment
AV-node
Coronary sinus
Tendon of Todaro
c d
Atrial Long,
connections Left bundle branch non-branching Posterior
Axis of septal bundle displacement
AV-node defect
Right atrium Right bundle branch Diminished of septal crest
Right ventricle atrial
connections
..Fig. 12.22 a–d Displacement of the conduction system. a Diagram of normal configuration, b diagram of posterior
displacement, and c, d diagrammatic view of surgical situs
12.1.5.4 S
urgery for Partial
Atrioventricular Septal
Defect
After access has been gained, a patch of autolo-
gous pericardium is harvested, keeping an ade-
quate distance from the phrenic nerve. The patch
can be pretreated with glutaraldehyde (0.1–0.6 %)
according to the surgeon’s preference. Aortic and
..Fig. 12.23 Right atriotomy
bicaval cannulation is performed. If there is a per-
sistent left superior vena cava, this is cannulated
separately or temporarily closed with a tourni- incised up to the atrial wall (Sondergaard’s groove,
quet. interatrial groove) to prevent extreme pull-up and
We usually perform the correction on high- deformation of the mitral valve and to ensure
flow cardiopulmonary bypass with hemodilution optimal suspension and mobility of the recon-
and normothermia or moderate hypothermia to structed valve (Alexi-Meskishvili et al. 1996).
(28–32 °C). Myocardial protection is provided by Sometimes there is, in addition to the ostium pri-
antegrade application of cold crystalloid or warm mum defect, an atrial septal defect of secundum
blood cardioplegic solution. type, which is divided from the primum defect by
The right atriotomy is made from the base of a muscle bridge. A small sucker is placed through
the right atrial appendage to just above the infe- the atrial septal defect into the left atrium to keep
rior vena cava (. Fig. 12.23). the operative field bloodless. We prefer to contin-
After right atriotomy has been performed, the uously direct carbon dioxide into the operative
remains of the septum secundum and primum are field with flow of 3–6 l/min.
328 V. Alexi-Meskhishvili et al.
a b
the cleft. Asymmetric apposition of the leaflets close to their native and systole position and so
should be avoided because this can lead to post- that they are separated from the left ventricular
operative incompetence of the left AV valve. The outflow tract by the short anterior end of the
deepest part of the cleft at the valve leaflet free patch (Van Arsdel et al. 1995). The stitches are
margin is identified by bringing together the pulled tight so that the right side of the patch is
bridging leafs at the point of the first chordal firmly fixed to the ventricular septum.
attachments to the free margins. The extent of the After the VSD patch is secured, 6-0 or 5-0
cleft closure is indicated by marking sutures monofilament nonabsorbable sutures are passed
placed along the facing margins of the anterior through the upper rim of the VSD patch and then
and posterior bridging leaflet (. Fig. 12.26). through the adjacent AV valve leaflets using the
When the leaflets are undivided, an attempt is previously placed marking sutures as a guide.
made to place more leaflet tissue on the side of the Thus, the separation is achieved, enabling a left
left AV valve. If leaflets are naturally divided, this and right AV valve to be formed (. Fig. 12.27c, d).
usually determines the size of the new anterior left The cleft in the new anterior left AV valve leaflet is
AV valve leaflet (. Fig. 12.27a). closed completely up to the insertion point of the
When the leaflet anatomy has been identified primary chordae at the leaflets edges, using inter-
and marking sutures have been placed on the base rupted fine monofilament nonabsorbable sutures.
point of the AV valve cleft, the ventricular septal The valve size is measured with a Hegar dilator
defect is closed with multiple interrupted felted and compared with the age-dependent normal
mattress sutures or with continuous sutures diameter to avoid AV valve stenosis (Rowlatt et al.
placed on the right ventricular side of the 1963). If the newly created orifice is too small, a
VSD. Usually six to eight single sutures are neces- few of the sutures at the free margin of the valve
sary (. Fig. 12.27b). These sutures should be that were placed for cleft closure can be removed,
placed further from the crest at the posterior end although we consider a diameter of 1–2 mm
of the VSD to avoid injury to the conduction tis- smaller than the normal age-related orifice diam-
sue. A scalloped Dacron patch (or alternatively a eter to be acceptable (Rowlatt et al. 1963). Valve
pericardial or Gore-Tex patch) is trimmed to competence is tested by filling the left ventricle
12 make it slightly smaller than the VSD. This with saline. If there is residual insufficiency, addi-
reduces the size of the dilated anulus and there- tional anuloplasty sutures are necessary, with the
fore may help to reduce the incidence of AV valve position of the anuloplasty determined by the
incompetence newly occurring after the repair direction of the insufficiency jet (Spray 2004). It
(Suzuki and Fukuda 2002). may be necessary to tolerate slight residual insuf-
When fashioning the VSD patch, the height ficiency. Although incomplete cleft closure has to
should be chosen to maintain the valve leaflets be tolerated in cases of small orifices with com-
plete closure, these patients are at a higher risk for
reoperation for left AV valve regurgitation in
medium-term follow-up (Alexi-Meskishvili et al.
1997).
Next, the ASD is closed according to the tech-
nique described in the section on partial AVSDs
(. Fig. 12.27e). The right atrium is then closed
and after de-airing of the heart the aortic clamp is
released.
Only in patients with a risk of developing a
critical elevation of pulmonary vascular resis-
tance postoperatively do we place a left atrial pres-
sure measurement catheter through the sulcus
interatrialis at the level of the right upper pulmo-
nary vein and a pulmonary arterial pressure mea-
surement catheter through the right ventricular
..Fig. 12.26 Closure of a total atrioventricular septal outflow tract. Others routinely place left atrial
defect using a double-patch technique catheters postoperatively in any AVSD patient.
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
331 12
Transesophageal contrast echocardiography and meticulous technical repair, it is, in our
is performed to evaluate cardiac function, experience, rarely necessary to reestablish
residual shunt flow, and atrioventricular valve extracorporeal circulation to perform a further
function. Using careful intracardiac evaluation correction.
..Fig. 12.27 Closure of a total atrioventricular septal defect using a double-patch technique
332 V. Alexi-Meskhishvili et al.
kRepair of complete AVSD with single-patch the left ventricle with a small catheter. Because the
technique superior and inferior bridging leaflets of the
The original single-patch technique for correc- common AV valve must be completely divided in
tion of the total AVSD was described by the the single-patch technique, it is very important that
group from Mayo Clinic (Rastelli et al. 1968a). the point of apposition of these leaflets be identi-
This technique was favored by Aldo Castaneda fied and marked with fine stay sutures. Once the
also and found again more recent appreciation line of division has been planned, the superior and
(Crawford 2007; Reddy and McElhinney 1997). It inferior bridging leaflets are divided, therefore
is described shortly below (. Fig. 12.28). becoming part of the left and right AV valves. After
A single pericardial patch, harvested at the division of the leaflets, the underlying ventricular
beginning of surgery and fixed in glutaraldehyde septal defect can be seen. All chordae tendineae
(0.1–0.6 %), is used to repair the entire septal attaching to the free edge of the leaflets should be
defect. After aortic cross-clamping and cardiople- preserved as far as possible. The interventricular
gia administration, the right atriotomy is per- component of the defect is first closed with inter-
formed as described above. The valve leaflets are rupted pledget-supported 5-0 nonabsorbable
inspected after infusion of 20-mm cold saline into sutures or continuous 5-0 polypropylene sutures.
a b
12
d
c
..Fig. 12.28 a–d Closure of an atrioventricular septal defect using single-patch technique
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
333 12
When the level of the AV valve orifice is reached, 12.1.5.7 Important Technical
the divided valve leaflets are sewn to the patch with Comments for AVSD repair
interrupted nonabsorbable doubly pledget-sup- To avoid injury to the conduction system, some
ported sutures. The level of reattachment of the surgeons used alternative techniques for ASD clo-
leaflets to the patch should be chosen rather lower sure. The suture line can be placed outside the
than too high. Closure of the cleft in the newly cre- coronary sinus so that this opens under the patch
ated left AV valve is performed with interrupted into the left atrium leading to a mandatory right-
5-0, 6-0, or 7-0 polypropylene sutures, which are to-left shunt. If a left upper vena cava leading into
placed up to the first-order chordae tendineae the coronary sinus is present, this technique can-
when closure is complete. The cleft can be also not be used. If the coronary sinus is unroofed, the
closed before reattachment of the leaflets to the defect should be closed through the left atrium. If
patch. It can even be closed before the superior there is a prominent left upper vena cava leading
leaflet is divided for this technique. In circum- into the coronary sinus, patch closure of the ASD
stances in which complete closure would result in a by the technique described above means that the
restrictive valve orifice, fewer sutures are placed coronary sinus and the left upper vena cava lead
(partial closure). The newly created left AV valve is into the right atrium. A left upper vena cava is
tested by injecting saline into the left ventricle. If treated in the manner described in the section on
necessary, anuloplasty is performed. After resus- anomalies of the caval veins (12.1.3).
pension and adjustment of the leaflets, the pericar- In about 10 % of partial, intermediate, or total
dial patch is used to close the atrial component of AVSD, additional AV valve abnormalities may
the septal defect (including the secundum defect, complicate the surgical procedure. Most often a
when present) with a running 5-0 polypropylene double orifice of the left AV valve («double-orifice
suture so that the coronary sinus orifice comes to mitral valve») and a potential parachute valve,
lie in the right atrium. The right atrium is closed which is caused by fusion of the papillary muscles
and the operation is completed in the usual manner. to form a single papillary muscle, are found
(Ilbawi et al. 1983). In addition to a left open cleft
kRepair of complete AVSD with modified or a reopening cleft, these additional abnormali-
single-patch technique ties lead to postoperative incompetence of the AV
When the modified single-patch technique is valve and result in the need for reoperation fol-
used (Bender et al. 1982; Nicholson et al. 1999; lowing AVSD correction (Alexi-Meskishvili et al.
Wilcox et al. 2004), the VSD is closed directly 1996, 1997; Nakano et al. 2002). Sometimes there
using multiple interrupted mattress sutures. is more than one additional anomaly of a valve
Teflon-buttressed 5-0 sutures are placed from the (Bano-Rodriges et al. 1988). If there is a double-
right side of the interventricular septal crest and orifice valve, the main orifice is often associated
passed through the bridging leaflet of the com- with a cleft formation supported by both papillary
mon AV valve, through one edge of the autolo- muscles, while the second orifice, which lies in the
gous pericardial patch—to close the atrial septal posteromedial portion of the left AV valve, is
defect—and finally through a thin strip of Dacron usually supported by only one papillary muscle.
or pericardium. These sutures are then tied down This prevents the normal excursion of the leaflet
to close the ventricular septal defect. The length of during the diastole (Manning 2007). Separation
Dacron or pericardial strip must be shorter than of the bridging leaflet tissue must be avoided to
the septal crest, so that suture tying results in a prevent floating and prolapsing of the central seg-
central anuloplasty of the common orifice and ment of the valve, which would lead to severe
ensures that adequate leaflet tissue is available for postcorrection valve insufficiency (Lee et al. 1985;
coaptation of the leaflets. If the central leaflets are Manning 2007). If there is a cleft in the true ori-
naturally divided rather than bridging, the sutures fice, it should be closed up to the point on the
are passed from the septal crest through the edge edge of the leaflet where the chordae tendineae
of the leaflets on each side of the septum (creating support the leaflet. In patients with a narrow
a bridging leaflet) and then through pericardium mural valve portion and when complete cleft clo-
or Dacron as described above (Nunn 2007). The sure would significantly reduce the true opening
remainder of the AVSD repair is conducted as area, closure of the cleft must remain partial.
described for single-patch repair. In such situations some surgeons accept a true
334 V. Alexi-Meskhishvili et al.
opening area of a minimum of 80 % of the normal was hypothesized that right ventricle volume
value (Nakano et al. 2002; Rowlatt et al. 1963). overload results in right-to-left septal bowing
When an accessory mitral valve orifice is well sup- and contributes to the presence of a hypoplastic
ported by chordae and does not cause incompe- left ventricle, which can actually accommodate a
tence, it may be left as it is (Lee et al. 1985; Nakano greater potential volume (Phoon and Silverman
et al. 2002; Schaff 1982). In these cases, the cleft of 1997). Hence, surgical decision-making depends
the true opening may be completely closed to not only on the AVVI but also on the absolute
achieve complete valve competence, even if now left ventricular volume and the potentially toler-
the true orifice becomes stenotic. The second, ated total volume. However, the definition of
double orifice would compensate for this. ventricular hypoplasia to be determined preop-
When there is only a single left ventricular eratively remains unclear, since the main criteria
papillary muscle, this potentially leads to a stenotic used so far apply to small left ventricles in other
parachute valve, which represents a considerable clinical conditions (Phoon and Silverman 1997;
challenge for the AVSD correction procedure. This Van Son et al. 1997).
situation should be distinguished from patholo- At our institution we have developed a guide-
gies in which one of the papillary muscles is hyper- line for the choice of surgical strategy based on
plastic. In single papillary muscle, anatomical the relationship between the long axis of the left
complete closure of the cleft carries the risk of pro- ventricle and the long axis of the right ventricle
ducing a significant mitral valve stenosis. In such (LV/RV long axis ratio) (Delmo Walter et al.
situations the division of a single papillary muscle 2008). A small left ventricle is defined on the basis
and partial cleft closure, without creating a steno- of angiographic measurements when the LV/RV
sis, represent an acceptable compromise (David et long axis ratio is smaller than 1.1. The ventricular
al. 1982; Litwin et al. 2007; Manning 2007). axis lengths were measured in the plane in which
If tetralogy of Fallot is present, the anterior the left and right AV valve reach a position in one
edge of the patch used to close the VSD must be plane with their respective apices during ventric-
much wider and reach far enough to the right to ulography (. Fig. 12.29).
completely subsume the overriding aortic root In our experience, 16 patients with an LV/RV
12 and prevent narrowing of the subaortic area value of >0.65 (mean 0.80 ± 11) underwent suc-
(Ilbawi et al. 1990). When applying a single-patch cessful biventricular correction of the defect. In
technique for complete AVSD repair in TOF, the two out of three patients with severe left ventricu-
superior bridging leaflet has to be divided more lar hypoplasia (LV/RV value of 0.45, 0.60, and
anteriorly to prevent subaortic stenosis and/or 0.62, respectively), weaning from cardiopulmo-
increased risk for patch dehiscence. nary bypass was not possible. Two of them (LV/
RV value of 0.45 and 0.60) died on the 8th and
12.1.5.8 Patients with AVSD and 11th postoperative day, respectively, despite
Small Left Ventricle mechanical circulatory support. The third patient
Surgical correction of a partial or complete (LV/RV of 0.62) needed postoperative extracor-
AVSD in patients with a preoperatively identi- poreal membrane oxygenation due to myocardial
fied small left ventricle poses a challenge for car- failure and successfully underwent heart trans-
diac surgeons, in particular in terms of the plantation on the 21st postoperative day.
choice of surgical strategy (Apitz et al. 2009). Our experience demonstrates that the size
There are as yet no proven guidelines for decid- of the left ventricle in total AVSD should not be
ing between biventricular correction and uni- considered absolute and that the left ventricle
ventricular palliation (Cohen and Stevenson has the potential to increase to a size sufficient
2007). These authors reported on a large group to support the systemic circulation. In this regard
of patients with unbalanced AVSD, in whom it was surprising that preoperatively the volume-
they used echocardiographic measurement of overloaded right ventricle can compress the
the atrioventricular valve index (AVVI). This already small left ventricle. Further, it was notable
parameter is the left valve area divided by the that after surgical correction of the total AVSD,
right valve area. The authors suggested that only primarily because of reversal of septal deviation,
patients with AVVI >0.67 (symmetrical) can the left ventricle is allowed to attain its full status
safely undergo biventricular repair. Further, it as a ventricle.
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
335 12
a b
LV/RV Long Axis Ratio (LAR)
Length LV
Length LV Length RV
Length RV (Normal >1,1)
LV length (long axis)
RV length (long axis)
..Fig. 12.29 a, b Measurements of LV-to-RV long axis ratio (LAR) where ventricular lengths were obtained by measur-
ing the distance in the plane in which the left AV valve and right AV valve are on the same level as their respective api-
ces. The relation of the left to the right side is determined (normal: >1.1)
The presence of a small left ventricle (with LV/ in patients with partial AVSD than in those with
RV value of as low as 0.66) did not preclude suc- complete AVSD. This phenomenon can partially
cessful biventricular repair. However, a value of be explained by the fact that a higher incidence
under 0.65 in the preoperative angiogram was of additional anomalies of the left AV valve mor-
clearly a predictor of myocardial failure. In such a phology is observed in patients with partial AVSD
case a univentricular surgical strategy should be than in patients with complete AVSD.
followed (Drinkwater and Laks 1997). Han and colleagues (1995) found that the over-
Although our findings cannot be compared all increase of valve incompetence in patients after
directly with those of Cohen and Rychik (1999), correction of partial AVSD correlated to an increase
we agree that the true LV size may be misleading. in mild regurgitation. There is a tendency toward an
Our series suggest that the size of the left ventricle increase in moderate regurgitation and no change
may be influenced by surgical manipulation. The in the group with severe regurgitation. The inci-
key parameter is the relationship between the dence of reoperations for significant left AV valve
long axes, since this value is independent of septal incompetence after correction of various types of
deviation. Further, this parameter is not only sim- AVSD ranges between 8 and 20 % and seems to be
ple to obtain but probably also far better than higher in patients with unsutured cleft or additional
volumetry with its complex measurements. valve anomalies (Alexi-Meskishvili et al. 1997;
Nakano et al. 2002; Hoohenkerk et al. 2012).
12.1.5.9 Results of AVSD Repair There is no single or ideal procedure for cor-
Complete correction of a complete AVSD remains recting residual AV valve incompetence if the
a challenging operation (Kaza et al. 2011). In cause is other than open cleft. Various surgical
recent years there have been many modifications techniques have been suggested, among others
in the surgical technique and different approaches cleft closure or valve perforation suturing with or
to the AV valve, and many centers have reported without different types of anuloplasty, triangular
low early and late postoperative mortality. or quadrangular leaflet resection, chordal short-
Nevertheless, left AV valve incompetence remains ening, or resection of secondary chords and plica-
the main cause of postoperative mortality and tion of redundant leaflets; the number of variations
morbidity and is the most frequent indication reflects the complexity of the problem.
for reoperations, which are necessary in 10 % Valve replacement during initial surgery for
of cases (Ando and Takahashi 2011; Patel et al. AVSD is rarely necessary, although the presence
2012; Permut and Mehta 1997). The incidence of of severe valve anomalies increases risk of recur-
severe preoperative left AV valve incompetence is rent valve incompetence and the subsequent need
rare. However, in some series a higher incidence for valve replacement. Mitral valve replacement
of preoperative left AV incompetence was noted can be performed safely in adult patients, but in
336 V. Alexi-Meskhishvili et al.
infants and small children it can be a challeng- The choice of procedure depends on the spe-
ing procedure. Drawbacks of prosthetic valve cific etiology of the obstruction. The most fre-
replacement in children include the requirement quent form of subaortic stenosis, which may
of long- term anticoagulation, the potential of occur many years after AVSD correction,
compromising annual growth and the necessity remains, however, the typical fibromuscular
of repeat valve replacement as the child grows. («membranous») form, which generally does
Proposed techniques of valve replacement in not cause more technical difficulties than any
small children include supra-anular implantation other fibromuscular SAS in the absence of
in cases of severe anular hypoplasia and enlarge- AVSD.
ment of the mitral valve anulus after incision of
the ventricular septal patch (Ando and Fraser 12.1.5.10 Atrium commune
2001). The term «common atrium» is used for malfor-
A further potential danger after AVSD repair mations with complete absence of the atrial sep-
is the development of an obstruction of the left tum accompanied by anomalies of the
ventricular outflow tract, which is observed in atrioventricular valves and can be considered a
2–10 % of patients operated upon (Crawford variant of the AVSD (Jacobs et al. 2000a). In these
2007; Nunn 2007). The causes of this complica- cases surgical treatment consists of complete cre-
tion are intrinsic abnormalities of the left ven- ation of the atrial septum with autologous peri-
tricular outflow tract. Elongation, anterior cardium or a synthetic patch. If coexisting
rightward displacement, and stenosis of the left anomalies of the caval or pulmonary veins are
ventricular outflow tract are present in most present, they are also treated appropriately. The
hearts with an AVSD and represent the anatomic term «single atrium» is reserved for atrial septal
cause of the «gooseneck» deformity. When these defects resulting virtually in one atrium; however,
features are pronounced, tubular stenosis may there are no anomalies of the atrioventricular
occur. Excrescences of fibrous tissue related to valves present.
the left AV valve and thickening of the fibrous
tissue in the membranous portion of the septum
12 may produce a discrete ridge within the left ven- 12.2 ongenital Heart Defects
C
tricular outflow tract. Other causes of obstruc- with Obstruction of the Left
tion include abnormal insertion of papillary Ventricular Inflow Tract
muscles or chordae tendineae into the infundib-
ular septum and dynamic obstruction by sys- 12.2.1 Overview
tolic anterior motion of the left AV valve into the
left ventricular outflow tract (Shiokawa and This group of congenital heart defects function-
Becker 1997). Significant left ventricular outflow ally has an obstruction of the blood flow from the
tract obstruction after AVSD correction is lung into the heart and embryologically shows
regarded as present when the pressure gradient anomalous drainage of the pulmonary veins into
at rest is >40 mmHg, which then is considered the true left atrium. These defects are classified
an indication for reoperation (Permut and Mehta according to their localization:
1997). 55 Stenosis and atresia of one or more pulmo-
Management of this complication must often nary veins
incorporate a number of techniques (Van Arsdel 55 Total anomalous pulmonary venous connec-
et al. 1995). There are several basic surgical tion to the systemic venous system with or
options to treat this complication (DeLeon et al. without obstruction
1991; Starr and Hovaguimian 1994; Van Son et al. 55 Cor triatriatum
1997):
55 Resection of fibromuscular tissue In addition to these developmentally related
55 Modified Konno procedure anomalies, the following also belong to the con-
55 Detachment and resuspension of the left AV genital heart defects with obstruction of the left
valve ventricular inflow tract:
55 Implantation of an apico-aortic valve-bearing 55 Congenital mitral valve defects
conduit 55 Supravalvular stenotic mitral ring
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
337 12
55 Left atrial cardiac tumor, which mainly of other congenital anomalies (Ito et al. 2001).
occurs as an atrial myxoma (see 7 Chapter Different surgical techniques have been used to
«Cardiac Tumors and Pericardial Diseases», correct congenital or acquired pulmonary vein
Sect. 31.1.2.1). stenosis in pediatric patients, but the success of the
treatment was limited (Sakamoto et al. 1995;
The clinical symptoms presented by these Spray and Bridges 1999). The problem with the
heart defects are extremely similar. Two- proposed techniques of patch repair in small chil-
dimensional echocardiography should be able to dren and infants is partially related to the fact that
establish the diagnosis. Cardiac catheterization minor geometric changes in small patients can
and cardio-angiography could help in determin- have critical effects on the low-pressure system, in
ing the actual hemodynamics if desired. which the flow is high, resulting in turbulence,
In the interests of completeness and less to aid intimal hyperplasia, or kinking of the patch repair
differential diagnosis, congenital diverticulum of causing additional venous obstruction. Sutureless
the left atrium is included in this section. techniques proposed in the late 1990s, on the
other hand, allow hemodynamically optimal cor-
rection (Azakie et al. 2011; Caldarone et al. 1998;
12.2.2 Congenital Pulmonary Lacour-Gayet et al. 1996, 1999; Yanagawa et al.
Vein Stenosis 2011). The patients with involvement of all pulmo-
nary veins have a uniformly poor prognosis due to
12.2.2.1 Introduction progressive pulmonary vascular disease and are
Congenital pulmonary vein (PV) stenosis is a rare candidates for heart–lung transplantation
anomaly, accounting for only 0.4–0.6 % of all con- (Mendelhof et al. 1995; Spray and Bridges 1999).
genital cardiac anomalies (Edwards 1960; Park Clearly no single-treatment algorithm can be
et al. 1974; Aleksi-Meskhishvili 1977; Latson and applied in all cases. Congenital pulmonary venous
Prieto 2007). It may involve any or all pulmonary stenosis is often an isolated lesion, but it may
veins. The term pulmonary vein stenosis describes coexist with total anomalous pulmonary vein
a variety of entities with variable clinical courses. connection, complete transposition of the great
The basic pathological process is fibrous intimal arteries, a ventricular septal defect, or other
thickening, which can gradually cause obliteration anomalies (Van Son et al. 1995a).
of the lumen of the pulmonary veins at the atrial
junction. In its most severe form, congenital pul- 12.2.2.2 Embryology
monary vein stenosis is a progressive disease with Congenital pulmonary venous stenosis has an
rapid development of pulmonary hypertension embryologic relation with total anomalous pulmo-
and rare survival beyond the first year of life. It nary vein connection and cor triatriatum. Despite
may not be found immediately after birth but this embryologic relation, co-occurrence of con-
develops within the first 6 months of life to clinical genital pulmonary venous stenosis and cor triatri-
significance. Surgical intervention has not been atum is extremely rare (Sade et al. 1974). In the
successful in this group. Other forms of pulmo- 3-mm large embryo, a diverticulum has formed
nary vein stenosis, including unilateral types asso- that in the future will develop into the left atrial
ciated with further congenital heart defects, may portion of the sinoatrial region. The diverticulum,
be managed by various surgical procedures or in or common pulmonary vein, grows toward the
some cases by pneumonectomy. The pulmonary developing lungs and ultimately connects with the
venous stenosis that can develop following radio- pulmonary veins, which have already formed.
frequency ablation to correct atrial fibrillation is When this drainage pathway is established, the
an iatrogenic complication (Saad et al. 2003). early connection between the splanchnic plexus
Although multiple direct surgical techniques and the umbilicovitelline and cardinal venous sys-
have been described for the repair of congenital tems becomes of secondary importance and is
pulmonary vein stenosis, the «sutureless» tech- largely lost. As the left atrium rapidly grows, the
niques are also important (Caldarone et al. 1998; common pulmonary vein is absorbed into the pos-
Lacour-Gayet et al. 1996). terior atrial wall, and the pulmonary veins enter
Often coexisting congenital pulmonary venous the left atrium individually. A variety of obstruc-
stenosis is an incidental finding during correction tive lesions of the pulmonary venous drainage may
338 V. Alexi-Meskhishvili et al.
occur, depending upon the stage at which normal described in a report on pulmonary vein steno-
embryogenesis is interrupted. If atresia of the com- sis after TAPVD repair, can be treated effectively
mon pulmonary vein occurs very early, a major by excision of the obstruction (Lacour-Gayet
drainage route can be established through either 2006). Initially, increase of the vein caliber can
the umbilical or cardinal venous system and lead be effectively achieved by patch angioplasty.
to total anomalous pulmonary vein connection of Whenever possible the use of artificial or
the infracardiac or supracardiac type. If the steno- allograft patches should be avoided, as they may
sis occurs at a later stage, the result may be stenosis induce neointimal formation with increased
of the common pulmonary vein (cor triatriatum). need for reoperation (Van Son et al. 1995a, b).
If stenosis occurs at a very late stage, after absorp- When segmental stenosis or atresia is present,
tion of the common pulmonary vein into the pos- the technique described by Pacifico for the
terior left atrial wall, the result will be stenosis or removal of pulmonary venous obstructions
atresia of an individual pulmonary vein. Further, using either atrial wall or atrial septal tissue may
pulmonary vein stenosis may occur on a physio- be useful (Pacifico et al. 1985).
logical basis in patients with cor triatriatum due to The surgical method of treatment for
restriction of flow from the chamber containing congenital and acquired pulmonary venous
the pulmonary veins to the chamber containing stenosis is described in detail in the sec-
the mitral valve. tion dedicated to total anomalous pulmonary
venous drainage.
12.2.2.3 Clinical Presentation Histologically, the membrane that may be
The clinical presentation of congenital pulmonary removed from the orifices of the pulmonary veins
venous stenosis resembles that seen with all the shows intimal fibrous thickening without cell
other lesions that produce pulmonary venous infiltration and therefore no evidence of active
hypertension. The prognosis is poor, particularly if inflammation (Reye 1951).
all pulmonary veins are affected. Although pro-
gression of symptoms may be less rapid if only one
or two pulmonary veins are involved, progression
12 to bilateral pulmonary vascular disease leading to 12.2.3 Cor Triatriatum Sinistrum
death is usually the rule, even in initially less severe
cases (Edwards 1960; Lacour-Gayet 2006; Sade 12.2.3.1 Introduction
et al. 1974; Spray and Bridges 1999). Two- Cor triatriatum is a rare congenital cardiac anom-
dimensional echocardiography with pulse Doppler aly and represents 0.1 % of all congenital cardiac
examination and color-coded flow imaging pro- malformations. It is associated with other cardiac
vides a reliable method for identifying and local- defects in up to 50 % of cases, for example, with
izing the pulmonary venous obstruction and (Löffler 1949; Marin-Garcia et al. 1975; Cooley and
determining its degree (Van Son et al. 1995a, b). Murphy 1990; Masayaoshi et al. 2001; Oelert et al.
1973; Oglietti et al. 1983; Pirc et al. 1996; Richardson
12.2.2.4 History et al. 1981; Rodefeld et al. 1990; Sethia et al. 1988):
The first surgical correction of congenital pulmo- 55 Transposition of the great arteries
nary stenosis was reported by Kawashima in 1971 55 Tetralogy of Fallot
(Kawashima et al. 1971). Correction later in the 55 Atrioventricular canal defects
life may be successful in removing a localized 55 Anomalous pulmonary venous drainage
venous obstruction (Binet et al. 1972; Kawashima 55 Tricuspid atresia
et al. 1971). In subsequent studies, high early and 55 Aortic isthmus stenosis
late mortality rates have been reported (Binet 55 Ebstein’s anomaly
et al. 1972; Pacifico et al. 1985). 55 Persistent ductus arteriosus Botalli
a b c
d e f
..Fig. 12.30 a–f Types of cor triatriatum sinistrum with the different connections between the atria: intracardial a–e
and extracardial f
340 V. Alexi-Meskhishvili et al.
a Tricuspid valve b
Coronary sinus
c d
..Fig. 12.31 a–d Cor triatriatum: operation through the right atrium and the atrial septum. a Incision of the atrial
septum along the upper edge of the foramen ovale in the direction of the right upper pulmonary vein. b After visual
inspection of both atrial portions, membrane excision is begun along the upper and posterior attachment margin. c
Once the membrane is excised, the mitral valve becomes visible and is inspected to make sure it is intact. d Closure of
the remaining communication between the atria with a synthetic or pericardial patch
342 V. Alexi-Meskhishvili et al.
a b
12
..Fig. 12.32 a–c Cor triatriatum sinistrum: operation through the left atrium. a Excision of the membrane dividing
the left atrium starting from the central perforation and proceeding along the attachment margin. b After complete
membrane excision, the two parts of the left atrium are connected, and blood flow to the mitral valve is unhindered.
c Direct closure of the left atrium by continuous suture
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
343 12
after de-airing of the atrium by inflation of the depending on the diameter of the ring. Usually
lung, is knotted with the lower row of sutures. the valve itself is abnormal, and frequently it is
Release of the aortic cross-clamp after de-airing of stenotic or hypoplastic. In many cases this
the heart and weaning of the patient from the lesion is associated with other stenotic lesions
heart–lung machine in normothermia are then such as:
routine procedures. 55 Parachute valve
55 Hammock valve
kOperation for complete division 55 Papillary muscle fusion
of the left atrium 55 Double-orifice mitral valve
If the accessory left atrium is completely divided
from the actual left atrium by an unperforated Supravalvar, or subvalvar where applicable
membrane (see . Fig. 12.30d) but has a connec- mitral ring must be differentiated from cor tria-
tion to the right atrium, the correction is best per- triatum. In both cor triatriatum and supravalvar
formed by transatrioseptal access through the mitral ring, the left atrium is divided into two
right atrium. Here the membrane is also excised, compartments. In cor triatriatum, the distal, pos-
beginning at its caudal edge, which is attached to terior compartment contains the pulmonary
the inferior rim of the atrial septal defect. To enable veins, while the anterior compartment contains
visual inspection, the atrial septal defect is first the left atrial appendage and the mitral valve ori-
enlarged in caudal direction (see . Fig. 12.31a). At fice. In supravalvular mitral ring, the posterior
the same time, this also opens the actual left atrium compartment contains the pulmonary veins and
and allows the surgeon to assess the extent of the the left atrial appendage, while the anterior com-
membrane. This is then entirely excised (see partment contains only the mitral valve orifice.
. Fig. 12.31b, c), and finally the remaining large
atrial septal defect is closed with a pericardial or 12.2.4.2 History
synthetic patch (see . Fig. 12.31d). The procedure The first case was described by Fisher (Cassano
is completed by direct closure of the right atrial 1964; Fisher 1902). Shone and Edwards described
incision with a continuous monofilament suture. a developmental complex of left-sided obstruc-
tions, consisting of a supravalvar mitral ring and a
12.2.3.6 Complications and Their parachute mitral valve in addition to subaortic
Avoidance stenosis, ventricular septal defect, and coarctation
The most frequent complication is injury to the of the aorta (Shone et al. 1963). Combinations
posterior left atrial wall between the mitral valve with other congenital heart defects, mostly with
and the entry of the left lower pulmonary vein. coarctation of the aorta and a ventricular septal
Such injury cannot always be avoided during com- defect, have been reported (Srinvasan et al. 1980;
plete excision of the triatrial membrane and in Sullivan et al. 1986; Watraida et al. 1997). A para-
principle is not dangerous as long as it is recog- chute mitral valve is often associated with a supra-
nized and the damage is repaired by direct suturing. valvular mitral ring (Banerjee et al. 1995). The
When operated upon timely, surgical results first successful surgical correction was reported by
are very good. Persistent pulmonary hyperten- Lynch and colleagues in 1962 in a 10-year-old girl
sion—especially in the early postoperative with a coexisting ventricular septal defect (Lynch
period—can negatively affect, especially prolong, et al. 1962).
the postoperative course.
12.2.4.3 Surgical Procedure
Resection of the fibrous tissue taking care not to
12.2.4 Supravalvular Stenotic damage the anterior mitral valve leaflet is the pro-
Mitral Ring cedure of choice. The ring is usually easily
separated from the valve with the resection begun
12.2.4.1 Introduction posteriorly and extended anteriorly. Only rarely is
This defect is formed by a circular ridge of mitral valve replacement necessary. The operation
endocardial tissue that is attached to the ante- is performed through a median sternotomy with
rior leaflet of the mitral valve below its insertion standard extracorporeal circulation and cardiac
on the anulus. Varying degrees of stenosis exist, arrest. The obstructive membrane/ring can be
344 V. Alexi-Meskhishvili et al.
a b c
..Fig. 12.33 a–c Supramitral ring. a Horseshoe-shaped supravalvular mitral valve ring. Only in the area of the anterior
commissure the stenotic effect of the membrane is less evident. b Excision of the supravalvular stenotic mitral ring
along its margin of attachment to the mitral anulus. c After total removal of the stenotic ring, the (usually intact) mitral
valve is freely visible
resected through the left or right atrium after inci- (TAPVC) can be associated with other heart
sion of the atrial septum. defects, especially in children with polysplenia,
asplenia, or heterotaxy syndrome, we will mainly
12.2.4.4 Surgical Techniques address isolated total anomalous pulmonary
The operation always requires cardiopulmonary venous connection and its combination with
bypass and consists of complete resection of the other minor anomalies, such as patent ductus
supramitral ring, regardless of whether it con- arteriosus Botalli or atrial septal defect.
sists of a ridge, a ring, or a membrane. Access is
gained either through the right atrium and the 12.2.5.2 Surgical Anatomy
atrial septum (especially when further intracar- Knowledge of the normal development of the pul-
12 diac anomalies are present) or directly through monary veins facilitates an understanding of how
the enlarged left atrium. Resection begins with the various types of anomalous pulmonary venous
incision of the superfluous anterosuperior tissue, drainage occur. Failure of the common pulmonary
which can then be relatively easily separated vein to connect with the pulmonary venous plexus
from the mitral anulus (. Fig. 12.33). It should leads to persistence of one or more venous connec-
be possible to avoid injury to the mitral valve. tions to the right superior vena cava, to the left ver-
Coexisting heart defects must be corrected at the tical vein/innominate vein, or to the umbilicovitelline
same time. If the defect arose from acquired vein/portal vein. Failure of the septum primum to
endocardial fibrosis and the mitral valve is there- form normally or abnormal septation of the sinus
fore insufficient, the valve will need to be recon- venosus can lead to direct connection of the pulmo-
structed so that the insufficiency does not nary veins to the right atrium. Late obstruction of
increase postoperatively. the common pulmonary vein after earlier venous
channels have disappeared can lead to isolated pul-
monary vein atresia, a rare and u sually fatal condi-
12.2.5 otal Anomalous Pulmonary
T tion. If incorporation of the common pulmonary
Venous Connection vein does not take place, left atrial division may
occur, or a cor triatriatum membrane may form,
12.2.5.1 Introduction leading to stenosis of the common pulmonary vein.
The diagnosis of total anomalous pulmonary TAPVC is divided into four broad categories
venous connection is made when all four pulmo- defined by the site of entry of the anomalous con-
nary veins drain anomalously to the right atrium nection to the systemic venous circulation
or to a tributary of the caval veins. This malfor- (Herlong et al. 2000).
mation is present in between 1 and 1.5 % of all Darling proposed the most commonly used
children with congenital heart disease. Although classification system for TAPVC (which he called
total anomalous pulmonary venous connection TAPVD: total anomalous pulmonary venous
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
345 12
a b c
d e
..Fig. 12.34 a–e Anatomic forms of total anomalous pulmonary venous connection: a type I supracardiac connection of
all pulmonary veins to a left superior vena cava; b type I supracardiac connection of all pulmonary veins to the right superior
vena cava; c type II (cardiac connection) with drainage of the pulmonary veins into the right atrium; d type II (cardiac con-
nection) with drainage of all pulmonary veins into the coronary sinus; and e type III (infracardial/infradiaphragmatic) with
drainage of all pulmonary veins into the portal venous system or directly into the inferior vena cava
drainage) based on the site of pulmonary venous 55 Type III or infracardiac connection is found
drainage (Darling et al. 1957; . Fig. 12.34): in 14 % of cases (. Fig. 12.34e). Here the
55 Type I or supracardiac connection common pulmonary vein travels down ante-
(. Fig. 12.34a, b) is the most common form and rior to the esophagus through the diaphragm
is seen in 51 % of cases. The four pulmonary to connect to the portal vein. Infradiaphrag-
veins drain via a common vein into the right matic connections have to be considered in
superior vena cava, left superior vena cava, or almost every case: either because they drain
their tributaries. In supracardiac connections, a through a still wide-open ductus venosus
stenosis may occur at the origin of the ascend- into the portal vein which acts stenotic due
ing vertical vein or at its attachment to the to its inherent elevated pressure or severe
innominate vein, or the vertical vein may be stenoses of the common pulmonary vein
stenosed where it crosses the left pulmonary may almost inhibit the pulmonary venous
artery and the left bronchus. flow when the ductus venosus constricts
55 Type II or cardiac connection accounts for shortly after birth.
28 % of cases (. Fig. 12.34c, d). The pulmo- 55 Type IV or mixed connection is rare and
nary veins connect directly to the right side accounts for only 7 % of cases. The right and left
of the heart, for example, to the coronary pulmonary veins drain to different sites (e.g.,
sinus or directly to the right atrium. In this the left pulmonary vein into the left vertical
anomalous connection, obstructions are rare vein and this in turn into the left innominate
but may occur at the junction between the vein, with separate anomalous drainage of the
common vein and the coronary sinus. right pulmonary vein) (Berdat et al. 2001).
346 V. Alexi-Meskhishvili et al.
patent foramen ovale and completely unroofing and then the foramen ovale is enlarged to enable
the coronary sinus until all four pulmonary veins a better view into the left atrium. Next, a curved
are adequately visualized. The enlarged atrial sep- instrument is introduced into the enlarged coro-
tal defect is then closed with a patch of pericar- nary sinus, and its anterior wall, which at the
dium or other material incorporating and same time is the posterior wall of the left atrium,
redirecting the pulmonary veins to the left atrium is levered upward. The taut membrane thus
(. Figs. 12.35 and 12.36). Care must be taken to brought into view is incised and cut out so that a
avoid the conduction pathways. defect with a diameter of at least 1.5–2 cm results.
The mere unroofing of the large coronary The creation of such a large opening far below
sinus when all pulmonary veins are draining into the opening of the coronary sinus largely excludes
it was described by Van Praagh (Van Praagh et al. injury to the atrial conduction pathways and the
1972). The right atrium is opened longitudinally, AV node. The opening of the coronary sinus into
the right atrium is closed with individual sutures
or a continuous suture placed 4–5 mm inside the
coronary sinus. In these cases the interatrial
communication, which had to be enlarged for
visualization of the roof of the coronary sinus,
can now also be closed by direct suture or with a
patch.
Many techniques have been described for
the correction of the supracardiac form of
TAPVC. Commonly, once the patient is on
extracorporeal circulation, the pulmonary veins
are exposed in the posterior pericardial space by
dividing the pericardial fold between the right
atrium and the pulmonary venous confluence
(Kirklin 1973). An incision is made in the com-
12 ..Fig. 12.35 Correction of anomalous pulmonary mon pulmonary veins—the confluence, which
venous connection to the coronary sinus (cardiac type) by
is typically oriented in a transverse direction. In
opening the residual atrial septum between patent fora-
men ovale and coronary sinus and further resecting the cardioplegic arrest, with the aorta crass-clamped,
anterior wall of the coronary sinus («unroofing») followed an appropriately large incision is made in the
by closure of the atrial communication posterior wall of the left atrium, corresponding
a b
..Fig. 12.36 a The wall dividing the coronary sinus from the left atrium is deeply incised and excised as much as pos-
sible. b The pericardial or synthetic patch closing the atrial septal defect is anchored into the anterior wall of the coro-
nary sinus so that the coronary sinus itself drains into the left atrium together with the previously anomalously draining
pulmonary veins
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
349 12
to the course of the incision into the confluens between the pulmonary venous confluence and
before. The incision is started near the atrial sep- the back wall of the left atrium. This pedicled flap
tum and then directed toward the base of the left of autologous vein wall serves to strengthen the
atrial appendage, making a transverse incision in anastomosis and to increase the effective volume
the left atrial back wall that corresponds to the of the left atrium. The vertical vein is usually
incision in the pulmonary venous confluence. ligated/clipped or transected (Kumar et al. 2001;
Next, a direct anastomosis, which should be Spray 2001).
as large as possible, is made between these two An alternative technique for the correction of
incisions using a continuous 7-0 or 6-0 absorb- supracardiac TAPVC is to use a biatrial trans-
able suture (. Fig. 12.37). The anastomosis can verse incision (Shumacker and King 1961). This
be augmented by dividing the left-sided verti- consists of making a transverse right atrial inci-
cal vein as far superiorly as possible at its entry sion across the crista terminalis and then
into the innominate vein. An incision is made through the posterior wall of the left atrium.
on the right medial aspect of the vertical vein Under direct vision, the posterior wall of the left
down to where it enters the pulmonary venous atrium can then be directly anastomosed to the
confluence. This vein is then flapped down medi- pulmonary venous confluence. For the last por-
ally on to the superior aspect of the anastomosis tion of the anastomosis, an interrupted suture
a b
..Fig. 12.37 a Horizontal incision of the left atrium in line with the pulmonary venous confluence (dashed line); b
anastomosis of the pulmonary venous confluence and the left atrium with a running absorbable suture; c schematic
representation of the completed correction with broad anastomosis between pulmonary venous confluence and left
atrium; and transection of the left ascending superior vena cava venous confluence drainage between two ligatures
350 V. Alexi-Meskhishvili et al.
technique can be used. The atrial septal defect is which is then closed with a pericardial patch. The
typically closed with a piece of pericardium or authors postulate that this technique eliminates
other patch material. The incision in the right the possibility of distortion of the anastomosis
atrium is closed directly or with a separate patch because it is performed with the heart in the ana-
if necessary. This technique has the advantage of tomically correct position.
enhanced exposure of the pulmonary veins. Surgical correction for the infracardiac type of
However, it requires more suture lines on the TAPVC is very similar to that described for supra-
atrium, which potentially increases the risk of cardiac TAPVC. Often, however, the pulmonary
postoperative atrial arrhythmias (. Figs. 12.38 venous confluence is oriented vertically in the
and 12.39). common pulmonary vein so that the incision in
Cobanoglu and Menashe described a tech- the common pulmonary vein is more hockey-
nique of repair in supracardiac or infracardiac stick shaped, running vertically and then on to
TAPVC performing the anastomosis from the left the left upper pulmonary vein to correspond with
side of the operating table with the apex of the a similar incision on the back wall of the left
heart tipped up and to the right (Cobanoglu and atrium from the medial to the inferior side of the
Menashe 1993). The anastomosis starts at the left atrial appendage (. Fig. 12.42).
right side, and the surgeon works toward himself It is not necessary to ligate the common pul-
or herself to the base of the left atrial appendage, monary vein below the diaphragm, and, in fact,
connecting the common pulmonary vein to the some authors have advocated routinely leaving
left atrium close to the left upper pulmonary vein this vein open in patients who show symptoms of
(. Fig. 12.40). an obstruction (Cope et al. 1997). The advantage
The persisting foramen ovale is then closed of leaving the vertical vein unligated is that, theo-
directly or, if there is concern about impingement retically at least, it can allow decompression of the
on the pulmonary vein anastomosis, with a patch. pulmonary venous channel through the sinus
Then the vertical vein is typically ligated venosus in the early postoperative phase, particu-
(. Fig. 12.41). The heart is closed and the child is larly when the left atrium is small and poorly
rewarmed and weaned from the cardiopulmo- compliant (Cheung et al. 2005; Ishino et al. 1997).
12 nary bypass. On the other hand, if the left atrium is quite small,
it can be helpful to divide the vertical vein and use
the remnant as an onlay patch to augment to the
If there is concern about postoperative size of the left atrium. This technique involves
pulmonary artery hypertension and wide mobilization of the pulmonary veins and
subsequent right ventricular failure, a pulmonary confluence. The correction is com-
life-saving technique can be to leave the pleted with the apex of the heart tipped up and to
persisting foramen ovale partially open to the right. After the vertical vein has been divided
allow decompression of the right side at the diaphragm, it is divided longitudinally. A
through the atrial septum with right-to-left parallel incision is made in the back of the left
shunting, at the expense of some systemic atrium extending into the left atrial appendage.
arterial desaturation. The venoatrial anastomosis begins at the upper
end of the pulmonary venous confluence and the
base of the left atriotomy. The anastomosis pro-
Hawkins et al. described a direct approach to ceeds along both sides until the inferior side of the
the anastomosis between the left atrium and the confluence (the divided vertical vein) is anasto-
common pulmonary vein in which the fossa ova- mosed to the tip of the left atrial appendage
lis is first excised through the right atrium (. Fig. 12.42c) The surgical management of mixed
(Hawkins et al. 1983). A generous transverse inci- forms of TAPVC varies according to the particu-
sion is made in the back wall of the left atrium, lar anatomy of each patient (Imoto et al. 1998). A
and a corresponding incision is made in the single anomalous pulmonary vein can be occa-
pulmonary venous confluence. The common pul- sionally left uncorrected if the other three veins
monary vein is now anastomosed to the back wall are satisfactorily reconstructed and correctly con-
of the left atrium directly through the fossa ovalis, nected. However, close observation is required
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
351 12
a b
c d e
..Fig. 12.38 a Right atrium, atrial septum, and left atrium have been opened horizontally. The opposite pulmonary vein con-
fluence is incised in parallel. b Anastomosis between pulmonary vein confluence and posterior wall of the left atrium is per-
formed with continuous absorbable suture. c Anastomosis between left atrium and pulmonary vein confluence is complete. d
To enlarge the left atrium and enable unobstructed drainage from the pulmonary veins into the left ventricle, the atrial septal
defect is closed not directly but with a synthetic patch. e The patch used to close the atrial septal defect is anchored with a con-
tinuous suture. Closure of the right atrial incision begins with a continuous suture at the remaining anterior incision edge. f Clo-
sure of the right atrial incision is performed with a continuous resorbable over-and-over suture, if necessary by sliding
technique. At times an additional patch may be required to enlarge the posterior lateral wall of the right atrium also
352 V. Alexi-Meskhishvili et al.
a b
..Fig. 12.39 a In some cases of infradiaphragmatic TAPVD, the anastomosis between the left atrium and the pulmo-
nary vein confluence is created from the left, the heart being held out of the thorax to the right. The incision runs to the
right, horizontally along the posterior wall of the left atrium and, in parallel, along the anterior wall of the pulmonary
vein confluence. b The anastomosis between the opened pulmonary vein confluence and the left atrium is performed
with a continuous over-and-over resorbable suture. The foramen ovale/ASD II has already been closed by direct sutur-
ing from the right atrium. The infradiaphragmatic connection is divided
a b
12
during the postoperative follow-up so that a sig- may have been misdiagnosed as having persistent
nificant left-to-right shunt or the onset of pulmo- fetal circulation with pulmonary artery hyper-
nary vascular hypertension can be identified early. tension and may need continuing extracorporeal
membrane oxygenation (ECMO) support. If the
12.2.5.7 Postoperative Complications pulmonary artery pressure has risen following the
and Their Avoidance correction, continuous measurement of the pul-
Postoperatively, these children can present pulmo- monary artery pressure may be helpful to guide
nary artery hypertension, particularly those who appropriate management (Imoto et al. 1998).
had suffered from the symptoms of pulmonary Occasionally, children with persistent pulmo-
vein obstruction. In fact, as noted above, some nary artery hypertension will require nitric oxide
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
353 12
a b
..Fig. 12.41 a Access from the right side in infracardiac total anomalous pulmonary venous connection. The incision
is sometimes made vertically along the course of the draining vein in caudal direction, an orientation the confluens in
infradiaphragmatic TAPVC often reveals. The corresponding incision in the left atrium runs parallel to it. b The incision
openings are connected by a continuous suture. c Halfway across the opening, the suture ends are pulled tight. d The
anastomosis between the pulmonary vein confluence and the left atrium is completed, and the drainage vein is tran-
sected between the two ligatures
ventricular compliance and at the same time reduce TAPVC surgery shows fibrous intimal hyper-
the pulmonary vascular resistance may be helpful. plasia associated with some media hypertrophy.
Volume overload has to be avoided. In these very There is increasing severity in the spectrum of
critical patients, temporary chest wall patch plasty lesions from anastomotic strictures to ostial and
with secondary sternal closure is a further expedi- diffuse pulmonary vein stenoses. In the case of
ent strategy (Alexi-Meskishvili et al. 1995). an anastomotic lesion, revision of the left atrial
A particularly challenging group of patients anastomosis with patch enlargement plasty brings
with total anomalous pulmonary venous about good results. On the other hand, conven-
connection are those with single left ventricle tional techniques for treating an ostial pulmonary
morphology. In these children the maintenance vein stenosis are of no use and have shown very
of adequate pulmonary blood flow is difficult poor results. The sutureless correction technique
since they develop pulmonary artery hyperten- introduced in 1996 provides better midterm
sion early on, particularly if they need either a results than any other technique and improved
systemic to pulmonary artery shunt or pulmo- the freedom from mortality and recurrence of
nary artery banding. What appears to be adequate the stenosis from 65 to 90 % (Lacour-Gayet 2006;
pulmonary blood flow in the operating room can Lacour-Gayet et al. 1966).
later mean excessive blood flow through the pul- When pulmonary venous stenosis occurs
monary vessels when the pulmonary vascular immediately after surgery or during the first post-
resistance drops and becomes normal. It may be operative days, it is the result of a primary restric-
necessary to readjust the source of the pulmonary tive anastomosis, a technical problem. More
blood flow to these changes. Even in the best of commonly, however, the stenosis occurs after a
hands, these children are problematical. A report delay varying from two to several months, fre-
from the Children’s Hospital of Philadelphia pub- quently after a large anastomosis repair and
lished in 1999 showed a 1-year survival in this uneventful postoperative course.
group of children with TAPVC and single-ventri- Complex forms of TAPVR are associated with
cle physiology of only 37 % (Gaynor et al. 1999). a significantly higher risk of pulmonary vein ste-
The early mortality was 58 % if the TAPVC was nosis. A 20–50 % incidence of postoperative pul-
12 corrected during the first operation. More than monary venous stenosis has been reported after
half of the survivors later developed a pulmonary repair of TAPVR associated with single-ventricle
vein stenosis. Hashmi and associates from the and right atrial isomerism. The mixed type of
Hospital for Sick Children in Toronto reported drainage, aberrant pulmonary venous drainage,
a 95 % early mortality in 20 children with right association with scimitar syndrome, presence of
atrial isomerism in whom TAPVC correction genetic syndromes, and boy weight of less than
was performed either alone or in combination 2.5 kg are all associated with increased occur-
with other procedures (Hashmi et al. 1998). rence of a secondary pulmonary vein stenosis
(Lacour-Gayet 2006). The common denominator
12.2.5.8 Pulmonary Vein Stenosis of most complex TAPVR is hypoplasia of the pul-
After Correction of Total monary venous confluence. The Toronto group
Anomalous Pulmonary introduced sutureless repair as the initial proce-
Venous Connection dure in patients with hypoplasia of the pulmo-
The major complication and the main cause nary venous confluens (Yun et al. 2005). The
of reoperation following surgery for TAPVC critical element of this technique is to perform
is the occurrence of pulmonary vein stenosis. the atrial anastomosis directly to the pericardium
Pulmonary venous stenotic disease is a poorly around the incised common pulmonary vein,
understood phenomenon associated with con- which should not have been further dissected out
genital pulmonary vein hypoplasia and carries of its connective tissue surroundings.
a very poor prognosis (Ando et al. 2004). The The diagnosis of ostial pulmonary vein steno-
causal mechanism is not well understood but sis is based on Doppler echocardiography showing
may be related to endothelial trauma in the pul- continuous flow with a velocity of >1.5 mm/s at
monary vein during the initial operation or to the pulmonary vein ostium.
pericardial adhesions. Pathological examination Magnetic resonance imaging is also, at least
of the pulmonary venous obstruction following partially, helpful here. It is the only imaging
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
355 12
procedure able to visualize intact portions of tomy of scar tissue or patch venoplasty (using
the pulmonary veins in the case of ostial atre- either pericardium, living atrial tissue, or PTFE)
sia. A pulmonary artery pressure of >50 mmHg has been associated with a high risk of recurrence
is an indication for surgery (Lacour-Gayet (Ricci et al. 2003).
2006). In these cases, which present usually much
Growing severity of pulmonary venous later than the cases of isolated anastomotic steno-
obstruction after repair of TAPVC can be evalu- sis, the anterior aspect of the previous anastomo-
ated according to the score proposed by Lacour- sis between the common pulmonary venous
Gayet (2006): trunk and the left atrial wall is incised to start
1. Anastomotic stenosis between the pulmonary with, also. Exposure is usually achieved via a right
vein confluence and the left atrium without atriotomy and transseptal approach
individual ostial stenosis (. Fig. 12.43a–c). The stenotic communication
2. Right pulmonary vein ostial stenosis involving between the left atrium and the pulmonary vein
the upper and/or the lower vein (a single confluens can be relieved by making a series of
pulmonary vein stenosis is usually well five or six radial incisions (. Fig. 12.43d, e).
tolerated) Resection of a portion of the stenotic rim is
3. Left pulmonary vein ostial stenosis involving sometimes necessary to further enlarge the pul-
the upper and/or lower vein monary vein orifice (Devaney et al. 2006a, b).
4. Bilateral pulmonary vein stenosis involving Patch enlargement of a left pulmonary venous
two, three, or four individual pulmonary stenosis using left atrial appendage tissue is an
venous ostia alternative technique (Pacifico et al. 1985).
5. Diffuse hypoplasia of one or several
pulmonary veins 12.2.5.10 Sutureless Correction
6. Total atresia of several pulmonary vein ostia of Postoperative Pulmonary
Vein Stenoses
Yun and associates proposed another score for with Pericardium
the evaluation of pulmonary vein stenosis: This technique was described by Lacour-Gayet
0—no stenosis in 1996 (Lacour-Gayet et al. 1996). The proce-
1—mild stenosis dure is based on complete resection of the ste-
2—severe stenosis notic scar tissue of the pulmonary vein, creating
3—occluded pulmonary vein an opening in the left atrial wall that is left open.
For the operation it is necessary to leave intact
The higher the score, the greater is the risk of the adhesions between the posterior left atrial
the need for reoperation or death (Yun et al. wall, the venae cavae, and the pericardium.
2005). Posteriorly a minimal dissection is performed.
While the use of total circulatory arrest is sim-
12.2.5.9 Standard Techniques pler, bicaval cannulation with full-flow CPB is
For each patient, the operative technique is our preferred technique. The superior vena cava
selected on the basis of the specific anatomy of the is directly cannulated and snared in as high a
stenosis. position as possible. The inferior vena cava can-
In patients with isolated anastomotic stenosis, nula is cannulated in low position and is not
who in most cases present in the first days after snared, which allows drainage of most of the
original repair, revision of the left atrial anasto- return flow through the inferior vena cava. The
mosis is performed by patch enlargement using a left atrium is approached through a transseptal
transseptal approach. The anterior aspect of the incision. The stenotic pulmonary venous ostia
previous anastomosis between the common pul- are identified. They can be reduced to the size of
monary venous trunk and the left atrial wall is pinheads and are therefore often difficult to
incised. A polytetrafluorethylene (PTFE) patch is identify. The stenotic tissue of the right pulmo-
used to enlarge the anastomosis. nary vein is totally resected from inside the left
Ostial stenosis is sometimes associated with atrium and also from the outside below the
this lesion. Conventional repair of an individual interatrial groove. This resection creates a large
pulmonary vein stenosis by either endarterec- opening in the left atrial wall that is left open.
356 V. Alexi-Meskhishvili et al.
a b
12
..Fig. 12.43 Correction of anastomotic pulmonary vein stenosis via a right atriotomy a and transseptal incision
b which exposes the narrowed anastomosis between left atrium and pulmonary vein confluens c. A series of five or six
radial incisions can be made d resulting in a wide-open communication e
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
357 12
The stenotic pulmonary vein scar tissue is The interatrial septum is closed, as is the right
excised to the level of the pericardial reflection, atrial wall. The fenestration between the left
until normal pulmonary vein tissue is reached atrium and the pericardium is left wide open. The
(. Figs. 12.44 and 12.45). further course of the operation using this tech-
nique differs for the right and left side.
For right-sided pulmonary vein lesions, the
right pericardial tissue is anastomosed around
and in some distance to the atrial wall, above the
left atrial fenestration, creating a left neo-atrial
pouch made by the pericardial sac. The pericar-
dial sac is dorsally kept closed by the pericardial
adhesions from the previous operation. The pat-
ent right pulmonary vein leads freely into the
pouch, and the blood drains through the opening
in the left atrial wall (. Figs. 12.45 and 12.46).
For left-sided pulmonary vein lesions, the cor-
rection can be conducted through the left atrial
cavity (. Figs. 12.47, 12.48, and 12.49).
The portion of the left atrial tissue surround-
ing the stenotic scar tissue is excised. This creates
a large opening in the left atrial wall. The left pul-
..Fig. 12.44 Resection and sutureless correction (right monary veins are dissected out to the left pericar-
side). This technique is based on total resection of the ste- dium and transected once normal pulmonary
notic scar tissue in the pulmonary vein. A small incision is vein tissue is reached beyond the stenotic seg-
required posteriorly. The left atrium is approached through ment. In the presence of sufficient pericardial
a transseptal incision. The stenotic tissue of the right pul-
monary vein is totally resected. This resection creates a
adhesions left in place, no suturing is necessary
large opening in the left atrial wall that is left open (. Fig. 12.48).
The blood from the left pulmonary veins is
allowed to drain passively into the left atrium
through the posterior pericardial cavity, which is
12
..Fig. 12.48 Resection and sutureless correction (left maintained as a closed space by the pericardial
side, from inside). The left pulmonary veins have been dis- adhesions. In the absence of sufficient pericardial
sected and transected and longitudinally incised across the adhesions or in cases of congenital pulmonary
stenotic segment into normal pulmonary vein tissue. In the vein stenosis without pericardial adhesions, it is
presence of sufficient pericardial adhesions, no suturing is
necessary to perform an atrio-pericardial anasto-
necessary. Blood from the left pulmonary veins is allowed
to drain passively into the left atrium through the posterior mosis either from inside the left atrium or from
pericardial cavity maintained as a closed space by the peri- the outside by elevating the heart (see . Figs. 12.48,
cardial adhesions 12.49, and 12.50). The suture line is placed on the
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
359 12
pericardium, distant to the pulmonary venous 12.2.5.11 Pulmonary Vein Stenting
ostia. The left phrenic nerve is located anterior to and Balloon Angioplasty
the pulmonary veins and outside the anastomotic The results of stenting for pulmonary vein steno-
suture line (. Fig. 12.51). sis have been disappointing. The tendency of the
In 1998, Caldarone and associates described a pulmonary veins to develop significant intimal
similar technique in which the stenotic tissue is proliferation makes the stenting procedure haz-
left in place, but the obstructed pulmonary venous ardous. Nevertheless, this therapy option may be
lesions are incised longitudinally (Caldarone and seen as an ultima ratio solution if stenosis has
Behrendt 2000; Caldarone et al. 1998; . Figs. 12.52 recurred again after several operations. Leaving
and 12.53; Najm et al. 1998). an atrial fenestration to allow angioplasty is
preferable. New generations of stent systems, such
as the drug-eluting stents used for coronary artery
interventions, may be more effective in the future.
Knowing the tendency of the pulmonary venous
endothelium to restenose after any manipulation,
it is unlikely that the pulmonary veins will stay
open for more than a few months (Bingler et al.
2012).
a b c
..Fig. 12.52 a–c Right-sided marsupialization is performed for right pulmonary vein stenosis by making a left atrial
incision a that is extended into each stenotic vein and across the area of narrowing. A posteriorly based flap of pericar-
dium is mobilized b and reflected onto itself and the left atrium c, which avoids the need to directly suture the veins.
The right phrenic nerve has to be watched out for
360 V. Alexi-Meskhishvili et al.
a b c
..Fig. 12.53 a–c Left-sided marsupialization is accomplished in an analogous fashion to the right. Here elevation
of the apex of the heart is necessary to facilitate exposure. A left atrial incision is made a and extended into each ste-
notic vein. A pericardial flap is cut out b and the marsupialization is completed c. The left phrenic nerve has to
be watched out for
opening incision to the pulmonary vein conflu- 55 There is no evidence of a pericardial defect.
ence, and staying well away from the individual 55 Distortion of the left ventricular free wall by
pulmonary vein ostia (Kanter 2006). A further the aneurysm is present.
precaution is to use resorbable sutures of
polydioxanone for the anastomosis (Lacour- Very rare are multiple small aneurysms of
Gayet 2006). both atria; these often become symptomatic
through atrial tachycardia (Bokeria et al. 1989;
Miyamura et al. 1990; Varghese 1969).
12.2.6 ongenital Diverticula and
C
12 Aneurysms of the Left Atrium 12.2.6.3 Clinical Picture
In asymptomatic patients, the diagnosis can be
12.2.6.1 Historical Background suspected if the heart silhouette in the chest X-ray
Congenital aneurysms of the left atrium belong to is enlarged.
the very rare congenital anomalies. They were first Despite its congenital etiology, manifestations
described by Semans and Taussig (1938). Two usually do not arise until about the second decade
variants of this anomaly are recognized: those of life, with a mean age of first presentation of
with intact pericardium and those with partial 26 years. Atrial fibrillation is observed in 40 % of
absence of the pericardium. In the latter case, it is these patients, and stroke occurs in 18 % (Gold
possible that the left atrial appendage protrudes et al. 1996). Strokes have been reported even in
through the pericardial defect rather than there infants (Sands et al. 2003). Typical chest pain can
being an aneurysm in the strict sense (Bukharin be caused by compression of the left coronary
and Aleksi-Meskhishvili 1969). Gold collected artery (Pomerantzeff et al. 2002). Congestive
reports of 48 patients from the international lit- heart failure in the neonate may be related to an
erature (Gold et al. 1996). Since then, many new anatomic obstruction of the pulmonary venous
cases have been reported (Kiaii et al. 2004; Pome drainage (Stone et al. 1990) or to displacement of
et al. 2000; Tanoue et al. 2004). the mediastinum or airway obstruction (Morales
et al. 2001). Cardiac tamponade due to restricted
12.2.6.2 Surgical Anatomy diastolic expansion of the left ventricle has been
A true left atrial aneurysm must fulfill the follow- described (Dimond et al. 1960). In the presence of
ing criteria (Foale et al. 1982; Huang et al. 1993; multiple biatrial aneurysms, atrial tachycardia
Stone et al. 1990): can occur in infants and small children (Bokeria
55 There is a normal atrium. et al. 1989; Miyamura et al. 1990). Diagnosis is
55 There is direct continuity of the blood flow easily established with echocardiography and
through the atrium itself. magnetic resonance imaging.
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
361 12
a b c
..Fig. 12.54 a–c Left atrial aneurysm as a sacculation of the left atrium along the posterior wall of the left ventricle. a
View from anterior direction; b view from posterior direction; c closure of the left atrium with a direct, continuous over-
and-over suture following resection of the aneurysm
Because of the risk of life-threatening compli- fails (Morales et al. 2001). The outcome is usually
cations, including tamponade, tachyarrhythmia, good, and the symptoms disappear (Gold et al.
systemic embolization, heart failure, and poten- 1996; Pomerantzeff et al. 2002).
tially, at least, of heart rupture, surgery is recom-
mended even in asymptomatic patients of any age
(Fontain-Dommer et al. 2000; Gold et al. 1996; 12.3 Congenital Mitral Valve
Morales et al. 2001; Pomerantzeff et al. 2002). Anomalies
12.2.6.4 Surgical Techniques 12.3.1 Introduction
Surgery to correct this anomaly may be performed
through a left thoracotomy with or without car- Pediatric mitral valve anomalies present complex
diopulmonary bypass (McGuinness et al. 2007; management challenges to the surgeon, who has
Vagefi et al. 2007; Victor and Nayak 2001), using to choose between valve reconstruction and
stapling devices (Burke et al. 1992) or even with a replacement. Frequently not only the mitral valve
minimally invasive, endoscopic approach (Kiaii must be addressed but there are other associated
et al. 2004). Median sternotomy, however, is the congenital malformations also requiring correc-
favored approach because, particularly in the case tion. Mitral valve reconstruction is the preferred
of large structures, adequate exposure through a technique for any kind of mitral valve malforma-
lateral thoracotomy is not possible. Using cardio- tion in newborns, infants, and small children as
pulmonary bypass also provides a motionless well as in older children and adolescents. Valve
operative field, which helps to avoid embolization reconstruction allows for valve growth with
caused by manipulation (Gold et al. 1996; Tanoue increasing age, does not necessitate anticoagula-
et al. 2004; . Fig. 12.54). It is important to cor- tion, and carries very little risk of thrombus for-
rectly assess the relationship between the neck of mation. It also entirely avoids the difficulty that
the aneurysm and the posterior mitral commis- there is a complete lack of prostheses suitable for
sure of the mitral valve because if the aneurysm this age group, and especially for small children.
extends into the commissure, partial anuloplasty Even when the result of primary reconstruction is
may be necessary to reestablish mitral valve com- not optimal, the time until a repeat correction and
petence (Stone et al. 1990). Mitral valve replace- until the patient reaches adulthood and/or can
ment has also been reported to be necessary if the receive an adult-size prosthesis can be bridged.
attempt to reconstruct the deformed mitral valve The best results of valve reconstruction are
362 V. Alexi-Meskhishvili et al.
achieved when the whole variety of reconstruc- flow tract. Both ends of the fibrous continuity of
tional techniques are available, the technique is the aortic and mitral valve are anchored in the
selected on an individual basis, and the use of any short axis of the left ventricle. Toward the ven-
kind of prosthetic material is avoided. Valve tricular musculature, the fibrous tissue is thick-
replacement must be reserved only for patients ened to form right and left fibrous trigones
with definitively irreparable mitral valves. (Wilcox et al. 2004). The scalloped posterior
(mural) leaflet is narrower and its attachments
occupy 210° of the anulus. Controversy remains
12.3.2 Historical Comments regarding the most accurate terminology for the
mitral valve leaflets. While some authors favor
Mitral valve surgery has a long tradition (Böttcher the terms «aortic leaflet» and «mural leaflet»
and Hübler 2006; Murray et al. 1938). The first (Ho and Anderson 1988; Wilcox et al. 2004), usu-
closed mitral commissurotomy in a child was ally the leaflets are defined as an anterior and
reported in 1952 by Mannheimer (Mannheimer posterior mitral leaflet (Carpentier and Brizard
et al. 1952). Young and Robinson (1964) reported 2006; Zias et al. 1998). The two mitral valve leaf-
the first successful mitral valve replacement in an lets are separated by the anterolateral and pos-
infant with congenital mitral valve stenosis. teromedial commissures. Close to the
During the past three decades, mitral valve sur- commissures lie two corresponding papillary
gery in infants and children has become an muscles, which are extensions of the subendo-
important part of pediatric cardiac surgery cardial myocardium. Chordae tendineae of the
(Carpentier et al. 1976; Dunn 1998; Lamberti and papillary muscles insert on both sides of the
Kriett 2007; Wood et al. 2004). opposite commissure, so that each valve leaflet
receives chordae from both papillary muscles.
The chordae support the leaflets and connect
12.3.3 Incidence of Congenital them either to the free and rough side of the
Mitral Valve Anomalies papillary muscles or directly to the ventricular
wall, as so-called basal cords (Wilcox et al. 2004).
12 A congenitally abnormal mitral valve affects less The mitral valve leaflets are segmented in six
than 1 % of all infants born with a normal-sized components (scallops): three anterior (A1–A3)
left ventricle. Patients born with the different types and three posterior (P1–P3) scallops (Carpentier
of atrioventricular canal defects, single ventricle, and Brizard 2006; Kumar et al. 1995; . Fig. 12.55).
and congenitally corrected transposition do not The relationship between the mitral valve
have a mitral valve in the anatomic sense of a sys- ring and the coronary arteries depends on the
temic atrioventricular valve. In up to 60 % of cases, dominance type of the coronary artery supply.
congenital anomalies of the mitral valve occur in When the left coronary artery is dominant,
association with other cardiac lesions, and often the entire attachment of the mural leaflet is
more than one component of the mitral apparatus
is involved (Wilcox and Anderson 1985).
2 1
A1
12.3.4 Normal Mitral Valve Anatomy
A2
The mitral valve consists of the anulus, leaflets,
3 P1 A3
chordae tendineae, and papillary muscles. The
mitral anulus is an integral part of the fibrous
skeleton of the heart. Normally the mitral valve
P2
has two leaflets, anterior and posterior. The 4
P3 6
larger, anterior (septal or aortic) leaflet attaches 5
to 150° of the anulus and is squat and trapezoid
in shape. As a consequence of being in fibrous
continuity with the aortic valve, it forms the ..Fig. 12.55 Components (scallops) of the mitral valve
posterior boundary of the left ventricular out- (After Kumar et al. 1995). A anterior, P posterior
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
363 12
AV-node into anular dilatation, cleft leaflet, and par-
Right fibrous trigone tial leaflet agenesis (Chauvaud et al.
1997b).
55 Type II: Leaflet prolapse. The free edge of
one or both of the leaflets overrides the
plane of the valve orifice during systole.
These defects are subdivided into chordal
elongation, papillary muscle elongation, and
chordal agenesis.
55 Type III: Restricted leaflet motion. The motion
of one or both of the leaflets is limited, which
Sinus node usually results in mitral valve stenosis,
Left circumflex
Coronary although in some cases valve insufficiency is
sinus
coronary artery Dominant right observed. Stenosis results from commissural
coronary artery fusion, imperforation, and thickening or
shortening of the subvalvar apparatus.
..Fig. 12.56 Important surgical structures of the mitral
valve as viewed by the surgeon (After Wilcox et al. 2004)
There are two subtypes with further divisions:
55 Type III-1: restricted leaflet motion with nor-
mal papillary muscles:
intimately related to the coronary artery and its 1. With fused commissures of the papillary
branch to the atrioventricular node. In the case muscles
of dominant right coronary artery, the poste- 2. With shortened chordae
rior leaflet is encircled by the circumflex coro-
nary artery, with the branch running below and Also included in this last group are anomalies
to the left to the mitral ring as viewed by the with excessive leaflet tissue, the supravalvular
surgeon (. Fig. 12.56). mitral ring and anular hypoplasia.
55 Type III-2: restricted leaflet motion with
abnormal papillary muscles:
12.3.5 Classification of Congenital 1. Parachute mitral valve
Mitral Valve Anomalies 2. Hammock mitral valve
in Children 3. Papillary muscle hypoplasia
usually appropriate. Bioprosthetic valves and leaflet motion and to select the appropriate recon-
homografts tend to degenerate rapidly in infants struction technique (Chauvaud et al. 1997a, b).
and children. Experience with the Ross–Kabbani Visualization of the leaflet function by preopera-
operation (autologous transplantation of the pul- tive transthoracic two-dimensional echocardiog-
monary valve into mitral position) in infants is very raphy and by cardiac catheterization may differ
limited, and long-term results in children are not (Banerjee et al. 1995; Freedom and Smallhorn
available (Frigiolla et al. 2005; Mitchell et al. 2001). 1988). Preoperative cardiac catheterization helps
to evaluate the degree of pulmonary hypertension
12.3.5.6 Ischemic Mitral Valve and define the anatomy of associated intracardiac
Incompetence and extracardiac defects (Brown et al. 2005).
This type of mitral valve incompetence usually occurs
in patients with anomalous origin of the left coronary
artery from the pulmonary artery (Bland–White– 12.3.7 Surgical Indications
Garland syndrome, see 7 Chapter «Congenital
Anomalies of the Coronary Arteries and Coronary Often infants and children are referred for mitral
Diseases of Children and Adolescents», Sect. 20.2.1) valve operation in the absence of manifest symp-
and is the result of a combination of papillary mus- toms. Gross cardiomegaly and dilatation of the
cle ischemia, dyskinesia of the free left ventricular left atrium in a seemingly asymptomatic child
wall, and left ventricular dilatation (Michielon et may provide an adequate basis for operation if the
al. 2003; Noren et al. 1964). Mitral valve surgery in preoperative echocardiographic analysis suggests
such patients is controversial (Dodge-Khatami et al. that the valve is amenable to repair. In symptom-
2002). In patients with mild or moderate mitral valve atic children surgical intervention is considered
incompetence, it has been shown that correction of anyways, especially if there is intractable heart
the coronary system alone may lead to an improve- failure, severe pulmonary hypertension, or a com-
ment in mitral valve insufficiency. By normalizing bination of both or when symptoms become
the left ventricular end-diastolic diameter, the mitral severe or when exercise limitations become unac-
valve insufficiency gradually decreases, but will not ceptable (Mitruka and Lamberti 2000; Uva et al.
12 completely disappear. Although patients show an 1995). In patients with a markedly abnormal valve
improvement in global ventricular function, they or in those who have previously undergone recon-
may be not free of symptoms because of this residual struction, mitral valve replacement may be the
mitral valve incompetence (Huddleston et al. 2001; only remaining option.
Michielon et al. 2003).
A: Anulus
..Table 12.3 Heart valve diameters calculated in
1: Midvalvar ring relation to body surface area (BSA) according to
Rowlatt et al. (1963)
2: Hypoplasia
a b
..Fig. 12.57 Danger of coronary kinking in a lateral anuloplasty and b posterior Gerbode anuloplasty
The use of synthetic pledgets should be avoided, heart–lung machine with a small priming volume
especially in small children, as they can lead to is used, with aortic and bicaval cannulation. The
hemolysis. We do not use synthetic rings at all in superior vena cava is cannulated directly for bet-
children because they fix the anulus and eliminate ter mitral valve exposure. In most cases we oper-
the possibility of its growth (Aharon et al. 1994). ate in moderate hypothermia of 32 °C. The
As suture pledgets and reinforcing material for patient’s core body temperature can be further
anuloplasty, we prefer to use autologous nontreated lowered depending on the complexity of the pro-
pericardium which is excellently suited as a recon- cedure. Myocardial protection is achieved with
struction material, allows growth, and at the same antegrade crystalloid or blood cardioplegia, and
time remains stable. In patients with congenital topical cooling with slush ice may be added. We
mitral valve stenosis, aggressive commissurotomy repeat cardioplegia administration every 20 min.
may produce significant valve incompetence. When the intracardiac procedure has been fin-
12 An exemption for using a prosthetic mitral ished, the heart is de-aired by applying gentle
valve ring may be the rare congenital Marfan’s atrial and ventricular massage and aspiration
patient who may present already in early child- through the cardioplegia cannulas. We still believe
hood with a severely dilated mitral anulus, which in preventing air embolism by carbon dioxide
after reduction and improvement of valve insufflation into the operative field, although
function may still have (almost) normal adult more recently no advantage could be shown in an
dimensions. adult population (Chaudhuri et al. 2012).
Once the reconstruction is completed, the
heart is de-aired, and the results may be evaluated
with transesophageal echocardiography. Reinsti 12.3.10 reatment Options for
T
tution of cardiopulmonary bypass and revision of Congenital Mitral Valve
the reconstruction are necessary in some patients Anomalies
when the results of the reconstruction are not yet
satisfactory (Lamberti and Kriett 2007). This may Mitral valve surgery in infants and children is less
have been already suspected from hemodynamic well established and less standardized than in
data once having come off bypass. adults. Reasons for this are the greater complexity
of the defects, a broad variety of reconstruction
techniques, the prospect of multiple repeat opera-
12.3.9 Cardiopulmonary Bypass tions, and the lack of suitable prostheses (Lamberti
and Myocardial Protection and Kriett 2007). The limitations of replacing the
mitral valve with a mechanical valve in infants
The operation is performed through a full median and children are well recognized. Techniques for
sternotomy. For cosmetic reasons in female reconstruction of the mitral valve have become
patients with fully developed breasts, a right sub- increasingly sophisticated in recent years, so that
mammarian incision may be used. A conventional the interval before unavoidable valve replacement
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
369 12
has been considerably extended (Baird et al. 2012; tic mitral anulus in whom valve replacement
Hetzer et al. 2008; Mitruka and Lamberti 2000). would be problematical (Spevak et al. 1990).
The surgical techniques consist of dilatation, Application of this procedure is limited by the
reduction or plication of the anulus, the implanta- lack of decisive functional improvement in
tion of different types of ring, closure of clefts, response to balloon dilatation and the risk of it
sliding plasty, and many other alternatives precipitating acute and severe mitral valve insuf-
(Pritisanac et al. 2005). ficiency.
Valve replacement is the last option to be fol- Although balloon dilatation can significantly
lowed (Alsoufi et al. 2011; Brown et al. 2012; reduce the transmitral pressure gradient in the
Hetzer and Drews 1999; Sim et al. 2012). Although majority of patients, this effect persists in only
replacement of the mitral valve in children with about 40 % of cases (Moore et al. 1994). The best
cryopreserved mitral valve homografts provided result is observed in patients with pure stenosis
satisfactory early postoperative results (Plunkett and flexible leaflets. The worst results have been
et al. 1998), this technique must be considered seen in children who had anomalies of the papil-
investigational at the present time because the lary muscles in addition to the congenital mitral
homograft valves degenerate rapidly in children valve stenosis. Many patients need further proce-
(Revuelta 1998). dures to treat either recurrent mitral valve steno-
The Ross–Kabbani operation: Replacement of sis or mitral valve regurgitation resulting from
the mitral valve with the autologous pulmonary dilatation-related disruption of the mitral valve
valve (Kabbani et al. 1999, 2001) represents a fur- apparatus (McElhinney et al. 2005).
ther alternative option, although long-term
results in children are not yet available (Brown et
al. 2006). In individual case reports, this opera- 12.3.11 Surgical Treatment
tion was recommended for very small infants
when valve reconstruction is impossible or a pre- 12.3.11.1 Surgery for Stenotic
viously implanted mechanical prosthesis devel- Anomalies of the Mitral Valve
ops thrombosis despite adequate anticoagulatory kSupravalvar mitral ring
treatment (Frigiolla et al. 2005; Mitchell et al. Resection of the fibrous tissue taking care not to
2001). damage the anterior leaflet is the procedure of
More recently, on the AATS Mitral Conclave choice. While the ring has to be cut off the anulus,
in New York, NY, in May 2013, Quiñonez et al. it is usually bluntly separated from the valve leaf-
from the Boston Children’s Hospital presented his lets with the resection begun posteriorly and
early experience with catheter deployable valve extended anteriorly. Only rarely is mitral valve
for mitral valve replacement in seven neonates replacement necessary (Sullivan et al. 1986).
and infants with a mitral diameter of <12 mm
(range 8–12 mm). He modified an externally kSingle papillary muscle—parachute
stented bovine jugular vein graft (Melody™ mitral valve
valve) for open-heart surgical implantation in this Fenestration of the papillary muscle (split into
cohort. His group hypothesized that the valve can anterior and posterior) with fenestration of the
be expanded in the catheterization laboratory as interchordal leaflet spaces typically releases the
the child grows. There was one early death in a subvalvar stenosis. The most appropriate site
salvage patient. Two patients have undergone for leaflet-splitting incisions is on both sides of
subsequent transcatheter balloon expansion of the common papillary muscle toward the
the prosthesis (to 12 mm) at 3 and 4 months post- trigones (. Fig. 12.58a, b). These incisions are
operatively, both with ongoing mild regurgitation extended into the body of the papillary muscle
(Quiñonez et al. 2013). Experience with isolated which is split toward its base, ensuring suffi-
balloon dilatation for congenital mitral stenosis is cient thickness of both «new» papillary muscles
very limited, and there are only few reports about (. Fig. 12.58c–e). Valve incompetence can be
the long-term results of this procedure (Alday treated by leaflet suturing or anulus remodel-
et al. 1994). This procedure should be considered ing. As would be expected, the success rate of
before mitral valve replacement in young patients this procedure increases with the age and size
(less than 5 years old) and in those with hypoplas- of the patients.
370 V. Alexi-Meskhishvili et al.
a b
c d e
..Fig. 12.58 a–e Surgical procedure for single papillary muscle (parachute mitral valve). a, b Incisions to split the leaf-
lets; c–e extension of the incisions deep into the body of the papillary muscle
..Fig. 12.59 a–c Excision of a portion of the ventricular wall in hammock mitral valve correction
a b
a b c
Mitral valve anulus
sutures are very slippery and the knots may slide often render the valve incompetent, necessitating
when tied. In adults David (2004) recommends extensive reconstruction or valve replacement.
using the lateral commissure as a reference for Up to one half of the posterior leaflet is amenable
correct chordal length estimation. In children it to resection, but only a small wedge of the ante-
is much more difficult to estimate the correct rior leaflet can be safely resected. Larger areas of
length. The zone of apposition along the length of the unsupported anterior leaflet can be treated by
the healthy, nonelongated native chordae chordal shortening, transfer, or replacement. Due
(attached to the nonprolapsing part of the valve) to the consequential disparity between the mitral
may serve as a reference (Boon et al. 2007; valve opening area and the leaflet size after leaflet
Minami et al. 2005). resection, concomitant anuloplasty is usually nec-
essary. Occasionally valve replacement is unavoid-
kLeaflet hypoplasia or agenesis able.
Treatment for leaflet agenesis consists of a rectan-
gular resection with suturing of the free edges of kChordae tendineae agenesis and rupture
the leaflet remnants after a sliding valvuloplasty of The absence or rupture of the primary chordae of
the remnants has been performed. A defect in the the central posterior scallop is most effectively
anterior leaflet may be treated by direct suturing dealt with by rectangular leaflet resection and
or by application of an autologous pericardial direct suturing. Modified Gerbode plication
patch depending on its size (Aharon et al. 1994; plasty is the technique we use for ruptured chor-
Chauvaud et al. 1997a). dae of the central scallop of the posterior leaflet
(Gerbode et al. 1962) (. Fig. 12.62a). The flail leaf-
kIsolated leaflet cleft let segment is plicated toward the ventricle with a
The optimal treatment consists of closure of the V-shaped suture line of polypropylene inter-
leaflet cleft (Zias et al. 1998). Anterior leaflet rupted mattress sutures pledgeted with untreated
clefts are repaired by direct suture technique. autologous pericardium (. Fig. 12.62b, c). When
Care must be taken to avoid extending the valve competence is assured, the posterior anulus
suture line beyond the site of primary chordal is stabilized with a strip of untreated autologous
12 insertion, as this would limit the valve opening. pericardium which is anchored to both trigones
Posterior leaflet clefts may be treated by quad- with separate pledgeted mattress sutures
rangular resection or simple closure. In the case (. Fig. 12.62d, e).
of a three-leaflet mitral valve, commissuroplas- The use of artificial chordae of expanded
ties at the anterolateral and posteromedial com- polytetrafluoroethylene sutures in neonates and
missures are indicated. Suturing of the free children has been reported (Anagnostopoulos
edges of a cleft in the posterior leaflet and plica- et al. 2007; Kawahira et al. 1999; Matsumoto
tion of the anulus also offer good palliation. If et al. 1999). Some authors suppose that, when
there is associated anular dilatation, an anulo- this technique is combined with other con-
plasty is performed concurrently (Perier and servative methods of mitral valve reconstruc-
Clausnizer 1995). In rare cases the mitral cleft is tion in children, it delays and possibly entirely
a part of an anomaly of the ventriculo-arterial avoids the need for implantation of a mechani-
connection and is addressed during and after cal prosthesis (Murakami et al. 1998). Valvular
the complex corrective procedure (Fraisse et al. restriction by artificial chordae has so far not
2002; Menahem and Anderson 2004; Photiadis been observed in the midterm follow-up in
et al. 1995). growing children (Boon et al. 2007; Kawahira
et al. 1999; Minami et al. 2005). However, the
kDouble-orifice mitral valve long-term results could not yet be observed
There are two strategies in treating the mitral (Matsumoto et al. 1999). The latest report with
valve with two orifices: the accessory orifice can a mean follow-up of 8.3 years during a 17-year
be left intact if it is competent (Carpentier and experience showed satisfactory results with
Brizard 2006) or it can be oversewn and obliter- mitral valve repair with artificial chordae in
ated if it is incompetent and the main orifice is of infants and children (Oda et al. 2013). Defects
adequate diameter (Baño-Rodrigo et al. 1988). If of the secondary chordal defects are addressed
the valve is stenotic, the treatment is more com- by suturing the free edges of the leaflet to the
plicated, as transection of the bridging tissue will secondary chordae.
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
373 12
a b c
d e
..Fig. 12.62 a–e Procedure followed when chordae tendineae are ruptured or absent: modified Gerbode plasty
a b
c
d
..Fig. 12.63 a Anterior leaflet retention plasty for systolic anterior motion (SAM) in hypertrophic obstructive cardio-
myopathy (Hetzer technique); b completed repair (view from the atrium); c depiction of mitral valve insufficiency in
hypertrophic and obstructive cardiomyopathy with SAM, longitudinal cross-sectional view; d the mitral valve regurgi-
tant jet is diverted into the LVOT after septal myectomy and the retention plasty of the anterior leaflet; site of septal
12 myectomy as viewed through the aortic valve. Opposite the anterior mitral valve leaflet (the broken line marks the inci-
sion in the myocardial septum; Hetzer technique) (Delmo Walter et al. 2010; Hetzer and Delmo Walter 2013) ph
Intraoperative measurement of the mitral valve between the right and the left coronary sinuses
opening area uses Hegar dilators. It is important (Hetzer et al. 2008; . Fig. 12.63e). The incisions
to ensure that the age-related minimal normal should be continued apically beyond the point of
valve diameter is reached, to avoid mitral valve mitral–septal contact, which is usually marked by
stenosis. In addition to the retention plasty of the a fibrous band. This wide incision beneath the
anterior mitral valve leaflet, Morrow-type subaor- aortic valve improves exposure of the important
tic myectomy through transaortic access should area toward the apex.
always be performed. Left ventricular and aortic After septal myectomy and anterior leaflet
pressure is measured directly and simultaneously. retention plasty, the aortic and mitral valves must
As the gradient across the left ventricular outflow be inspected to ensure that they have not been
tract may be low (30 mmHg) because of anesthe- injured. Pressures in the left ventricle and aorta
sia, isoproterenol is administered, or premature must be remeasured, and the transesophageal
ventricular contractions are induced to determine echocardiographic evaluation is repeated after
the maximal pressure gradient. weaning from the cardiopulmonary bypass.
An oblique aortotomy is made, leading right- Myectomy has been successful when there is little
ward into the noncoronary sinus in the direction or no residual LVOT gradient, absence of SAM,
of the aortic anulus. The aortic valve is inspected and no mitral valve stenosis.
and the subvalvular region exposed. We make
parallel incisions in the septum directly opposite kMitral valve reconstruction for endocarditis
the anterior mitral leaflet and resect long blocks of The vital principle consists of adequate debride-
septal myocardium between the two incisions, ment of the infected tissue and meticulous flush-
which start directly below the aortic anulus at the ing of all affected areas with povidone–iodine
right coronary sinus and at the commissure solution, regardless of whether purulence or
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
375 12
v egetations were present or not. Further, meticu- In patients with hypoplastic left or right ven-
lous removal of vegetations, when present, and tricle and hypoplastic, insufficient corresponding
anatomic reconstruction using sutures reinforced valves not amenable to reconstruction, the insuf-
with untreated autologous pericardial pledgets or ficient valve van be closed with a pericardial
pericardial strips are necessary. patch, thus converting heart failure to mitral valve
Mitral valve reconstruction is performed or tricuspid valve atresia. The creation or enlarge-
using either anterior commissuroplasty (our ment of an atrial septal defect is important in
modified Kay–Whooler technique; see these circumstances to prevent atrial congestion.
. Fig. 12.60a, b), posterior commissuroplasty with
leaflet resection (see . Fig. 12.61a), or posterior 12.3.11.4 Mitral Valve Replacement
commissuroplasty with pericardial strip rein- At present there is no ideal valve prosthesis avail-
forcement as in our modification (see . Fig. 12.61b, able for mitral valve replacement in children, par-
c). Chordal rupture can be corrected by chordal ticularly in early childhood and infancy (Alexiou
reimplantation or replacement. et al. 2001). In children every possible effort
should be made to preserve the native valve. It is
12.3.11.3 Atrioventricular Valve our policy to attempt mitral valve reconstruction
Reconstruction in Patients in every child, even when less than optimal results
with Single Ventricle of the reconstruction must be accepted and reop-
Significant atrioventricular (AV) valve insuffi- erations after some time must be planned. The
ciency has been reported in up to one third of philosophy behind this concept is to bring every
patients with single ventricle who undergo pal- child—if necessary by repeated operations—up to
liation (Imai et al. 1999; Sallehuddin et al. 2004). an age when a prosthesis that will last for a life-
This is considered a significant preoperative risk time can be implanted (Hetzer and Drews 1999;
factor as well as a reason for the worsening in Hetzer et al. 2008). If reconstruction for mitral
late results after bidirectional cavopulmonary valve stenosis is attempted, mitral valve insuffi-
shunt (BCPS) and after Fontan operation ciency often remains, partly due to defects of the
(Scheurer et al. 2008). We therefore prefer to cor- subvalvar apparatus (such as parachute and ham-
rect significant AV valve insufficiency when per- mock deformities). In this situation valve replace-
forming BCPS. This provides a better ment is often the only remaining option (Beierlein
background, avoids the need for additional et al. 2007; Günter et al. 2000; Kadoba et al. 1999).
intracardiac repair when the Fontan operation However, in small children whenever it is possible
follows, and improves the results. Common rea- we prefer to implant a mechanical or biological
sons for AV valve insufficiency in patients with aortic valve prosthesis in upside-down position.
single ventricle are anular dilatation, elongation Most mechanical mitral valves are only available
of the chordae, and the presence of clefts. To in sizes 25 and larger. Therefore, for smaller sizes,
achieve valve competence, it is often necessary mechanical aortic valves, implanted in upside-
to combine valve reconstruction with anulo- down position, have to be used. The risk that
plasty. Although there are techniques that arises after implantation of a mitral valve prosthe-
improve AV valve function, such as anuloplasty, sis that is too large is far greater than if elective
bi-valvation of the common atrioventricular second mitral valve replacement is performed
valve, cleft closure, chordal elongation or short- after the child has grown (Caldarone et al. 2001).
ening, and the suturing of pericardial patches Small prosthetic valves can lead to patient–
(Lamberti and Kriett 2007; Oku et al. 1994), there prosthesis mismatch in the course of somatic
are still a number of patients who require AV growth of the patient (Vohra et al. 2006). The
valve replacement due to progressive valve supra-anular implantation technique allows the
insufficiency that is not amenable to reconstruc- mitral valve to be replaced with a prosthesis which
tion. To maintain ventricular function, it is is larger than the natural mitral valve ring (Kanter
important to preserve the chordae and the papil- et al. 2011). After 15 years, all children with pros-
lary muscles. Chordal attachments must be thesis of <23 mm have outgrown their valves, but
divided only if they obstruct the ventricular out- mitral valve re-replacement with a larger size
flow tract (Mahle et al. 2001). The development prosthesis is always possible, and the operative
of postoperative total AV block is common. risk is low (Beierlein et al. 2007). In a large
376 V. Alexi-Meskhishvili et al.
multicenter study, the risk of developing total AV growth of the anulus (Raghuveer et al. 2003).
block was found to be between 8 and 30 % Children with mechanical valve prostheses must
(Caldarone et al. 2001; Kanter et al. 2005). be placed on an anticoagulation regimen to main-
With the wide array of techniques currently tain an international normalized ratio (INR) of
available, satisfactory repair of congenitally anom- between 2.5 and 3.5 (Günter et al. 2000). Long-
alous mitral valves is possible in 80 % of cases term anticoagulation is usually well tolerated
(Baird et al. 2012). Preservation of the chordal (Beierlein et al. 2007; Caldarone et al. 2001;
attachments is important in preserving ventricu- Sachweh et al. 2007).
lar function but is often not possible in infants
and small children. When the anulus is too small 12.3.11.5 Implantation of the
to place the prosthesis in orthotopic position, the Extracardiac Valved Conduit
valve prosthesis may be implanted in supra-anu- for Mitral Valve Stenosis
lar position in the left atrium (Tierney et al. 2008; In cases when the stenotic mitral valve is irrepa-
Kanter et al. 2013; Rocafort et al. 2013). The most rable and valve replacement is not feasible due to
appropriate prosthesis for mitral valve replace- anular hypoplasia in the setting of a normal-sized
ment in older children is a low-profile mechanical left ventricle, an extracardiac conduit with a por-
bileaflet prosthesis (Alexiou et al. 2001; Alsoufi cine valve may be implanted (Amodeo et al.
et al. 2011; Günter et al. 2000; Sim et al. 2012). In 1990). The operation is performed through a left
the not so distant past, the perioperative mortality thoracotomy or a median sternotomy (Laks et al.
for valve replacement in infants and children was 1980). The prosthesis is attached to the left atrium
30–50 % (Kadoba et al. 1999; Zweng et al. 1989). by making an incision between the base of the left
However, with improved myocardial preservation atrial appendage and the pulmonary veins. The
techniques, better valve prostheses, tighter control distal end of the prosthesis is sutured to the apex
of postoperative anticoagulation, and improved of the left ventricle. Rapid calcification of the bio-
postoperative intensive care unit management, prosthesis in children is the main concern
the operative mortality for mitral valve replace- (Mazzera et al. 1989; Serraf et al. 2000). The more
ment in children decreased to 3.5–11 % (Alexiou recently published innovative surgical technique
12 et al. 2001; Günter et al. 2000; Brown et al. 2012), of implanting an individually modified transcath-
although valve replacement in the smallest chil- eter valve under direct vision with the option of
dren continues to carry a high early mortality of stepwise dilatation during further follow-up
up to 20 % (Beierlein et al. 2007; Serraf et al. 2000; (Quiñonez et al. 2013) may be applicable in these
Vohra et al. 2006). Repeat valve replacement cases where earlier an atrioventricular conduit
is necessary in half of the cases within 3 years was thought of.
(Kanter et al. 2005). Long-term survival in small
children remains poor, with an actuarial survival
rate after 10–20 years of approximately 50–61 % 12.3.12 Results of Repairing
(Alexiou et al. 1999; Günter et al. 2000; Kadoba Congenital Mitral Valve
et al. 1999). Anomalies
In a large multicenter study, the main reasons
for second mitral valve replacement in 102 chil- Results of mitral valve repair in pediatric patients
dren operated on at age of less than 5 years were have significantly improved in recent years (Hetzer
prosthetic valve stenosis and prosthetic endocar- et al. 2008; Delmo Walter et al. 2010). The r easons
ditis. In detail the following reasons for second for this trend lie in better understanding of valve
mitral valve replacement were given: pathology, improved surgical techniques, changes
55 Stenosis of the original valve prosthesis in myocardial protection and extracorporeal circu-
(24 children, 83 %) lation, and intraoperative use of transesophageal
55 Thrombosis of the original valve prosthesis echocardiography. Mortality rates reported in dif-
(4 children, 14 %) ferent studies are difficult to compare because there
55 Prosthetic valve endocarditis (1 child, 3 %) are differences in the disease complexity, ages of the
patients at operation, description of the mitral
The second implanted prosthesis was always valve anomalies, and surgical methods used
larger, which underlines the factor of continuing (Pritisanac et al. 2005; Seccombe 1999). Late results
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
377 12
are dependent on the age at operation, clinical pre- placement and the resultant tricuspid valve regur-
sentation, presence or absence of valve insuffi- gitation. In the case of mild displacement and
ciency or stenosis, and other associated cardiac mild valve regurgitation, the patient may remain
anomalies (Aharon et al. 1994). In general, higher asymptomatic for many years. If, however, leaflet
mortality with reconstruction is to be expected displacement and tricuspid valve regurgitation
when the mitral valve anomaly is associated with are severe, the pulmonary blood flow is decreased,
other additional heart defects (Wood et al. 2004). the right atrium becomes dilated, and the blood is
Best midterm and long-term results are achieved in shunted from right to left through an atrial septal
patients with isolated mitral valve insufficiency defect or more often a patent foramen ovale. The
because of anular dilatation or with the presence of patient becomes cyanotic. The signs of heart fail-
a cleft (Zias et al. 1998). Results are suboptimal in ure may also develop secondary to a functional
patients with absent chordae (Sugita et al. 2001). In small right ventricle and decreased right ventricu-
patients with mitral valve stenosis, the results are lar compliance.
less satisfactory since there is greater complexity
and variability in the pathology (Lorier et al. 2001).
Many patients with mitral stenosis later need repeat 12.4.2 Mortality and Morbidity
operations and even mitral valve replacement (Uva
et al. 1995; Serraf et al. 2000; Alghamadi et al. Intrauterine mortality with this lesion is as high as
2011; Remenyi et al. 2012; Jiang et al. 2013). 85 %. Mortality after birth is related to the severity
of cyanosis, the tricuspid valve deformation, and
the lack of antegrade flow through the pulmonary
12.4 Ebstein’s Anomaly valve (McElhiney et al. 2005). Newborns with
cyanosis have a mortality rate of up to 70 % com-
12.4.1 Introduction pared to 15 % for newborns without cyanosis
(Arizmendi et al. 2004). Death is precipitated by
Ebstein’s anomaly is a rare, complex congenital heart failure or postoperative complications or
malformation of the tricuspid valve and the right occurs as sudden cardiac death. Actuarial survival
ventricle with very variable anatomy. The anomaly among live-born patients has been reported to be
involves abnormal attachments of the tricuspid 67 % for the first year and 59 % for the first 10 years
valve leaflets to the valve anulus. Marked variability (Giuliani et al. 1979).
exists in the degree of displacement of the septal Severity of the leaflet displacement and the
and posterior leaflets into the cavity of the right degree of associated right ventricular outflow
ventricle. This may result in a right ventricle that is tract obstruction determine the age at which the
divided into an atrialized portion of the right ven- patient develops symptoms. In large-scale investi-
tricle above the valve leaflets and a remaining, gations of Ebstein’s anomaly, 81 % of the patients
more or less, hypoplastic ventricular portion below were diagnosed during their first week of life, 6 %
the valve leaflets. The lesion was first described in when aged 1–4 weeks, 11 % at the age of
1866 by the German pathologist Wilhelm Ebstein 5–25 weeks, and 2 % when older than 25 weeks
(1866) and was first referred to as Ebstein’s disease (Arizmendi et al. 2004; Correa-Villasenor et al.
in 1927 (Arstein 1927). The first diagnosis of 1994; Kumar et al. 1971). After the first 6 months,
Ebstein’s anomaly in a living patient was made by the prognosis in infancy is approximately the
Tournaire and associates (Tournaire et al. 1949). same as that in childhood and adolescence
The incidence of Ebstein’s anomaly of the tri- (Arizmendi et al. 2004; Davidson et al. 1995;
cuspid valve is approximately 1:20,000 live births, Davies et al. 2013; Yu et al. 2013).
accounting for less than 1 % of all congenital heart
defects. Male and female children are affected
equally. In a large cardiac surgical center, Ebstein’s 12.4.3 History of Surgical Treatment
anomaly accounts for less than 1 % of all cardio-
vascular procedures for congenital heart disease The surgical treatment of Ebstein’s anomaly, the
(Hetzer and Pasic 2004). associated tricuspid valve incompetence, and the
Hemodynamic consequences of this lesion are significant sequela remained controversial for a
directly related to the severity of the leaflet dis- long time with regard to the surgical indication,
378 V. Alexi-Meskhishvili et al.
the most suitable operative technique (valve In 2004 da Silva described a new technique for
replacement or repair), the appropriate type of repair of Ebstein’s anomaly (da Silva et al. 2004)
repair, and the optimal timing of the procedure. which uses some principles of the Carpentier
Reconstruction of the tricuspid valve in Ebstein’s technique, but reconstructs the tricuspid valve in
anomaly was not generally accepted, firstly a significantly different way (da Silva et al. 2004,
because of the immense variety of pathological 2007; Dearani et al. 2008). According to da Silva
manifestations of the anomaly that makes the (da Silva et al. 2007; da Silva and da Fonseca
development of a standardized reconstruction 2012), the surgical goal of the technique is to cre-
concept difficult. Further, few surgeons have the ate a cone-like structure from the available tricus-
opportunity to gain sufficient experience in the pid valve tissue, covering the right atrioventricular
management of this defect since it so rarely orifice and allowing leaflet-to-leaflet coaptation.
occurs. Many surgeons favored tricuspid valve This cone reconstruction technique was used suc-
replacement, which is technically the most cessfully for primary correction of all forms of
straightforward procedure, particularly after the Ebstein’s malformation (da Silva et al. 2007; da
first successful replacement was reported in 1963 Silva and da Fonseca 2012; Vogel et al. 2012; Liu
by Barnard and Schrire (1963). Although some et al. 2011; Dearani et al. 2013a) and for re-repair
patients do well for many years after tricuspid due to residual severe tricuspid valve incompe-
valve replacement, prosthetic valve replacement tence (Dearani et al. 2013b).
exposes the patient to the well-known potential In neonates and infants, two different surgical
problems of prosthetic valve dysfunction, throm- strategies can be applied: valve reconstruction
boembolism, endocarditis, and patient–prosthe- (Knott-Craig et al. 2000; Boston et al. 2011) or the
sis mismatch as a result of somatic growth of the univentricular approach known as the Starnes
patient. For these reasons a reparative plasty of procedure (Starnes et al. 1991).
the tricuspid valve should always be preferred to
valve replacement, whenever it is possible. In
cases where the tricuspid valve is completely 12.4.4 Anatomy
absent so that no chordae or papillary muscles
12 are recognizable or where the free edges of the Ebstein’s malformation of the tricuspid valve is
leaflet are attached to the ventricular wall, recon- characterized by three features:
struction is not possible, and there is no alterna- 55 Adherence of the septal and posterior leaflets
tive to prosthetic replacement in an older child to the underlying myocardium with down-
or adult. ward (apical) displacement of the functional
Hunter and Lillehei described the concept of anulus and so-called atrialization of the
valve reconstruction for the first time in 1958 affected portion of the right ventricle
(Hunter and Lillehei 1958). This technique was 55 Redundancy and fenestration of the anterior
applied with clinical success by Hardy (Hardy leaflet
et al. 1964) and was further modified by Danielson 55 Dilatation of the right atrioventricular junc-
who has been credited with popularization of tion (i.e., the true tricuspid anulus)
valve repair in Ebstein’s anomaly in a large series
of patients (Danielson et al. 1979a, b, c). In addi- Because each of these features may be mani-
tion to reconstruction of the tricuspid valve, this fested to different extents, the morphological
technique includes transverse plication of the so- appearance of the tricuspid valve in Ebstein’s
called atrialized chamber. In 1988, Carpentier anomaly is also highly variable (Anderson and Lie
introduced a technique of valve reconstruction 1979; Chauvaud et al. 2003; Chen et al. 2004).
with longitudinal (instead of transverse) plica- Apical displacement always affects the septal
tion of the atrialized chamber (Carpentier et al. leaflet, but it may also involve the posterior leaf-
1988). This technique was further developed by let, and large parts of the affected leaflets are usu-
Quaegebeur (Quaegebeur and Sreeram 1991). In ally adhered firmly to the right ventricular wall
contrast to these techniques, Hetzer in 1998 (Becker 1995). The anterior leaflet is usually
introduced a concept of tricuspid valve recon- enlarged and resembles a billowed sail, but it is
struction in which the atrialized chamber can always attached to the anulus at the normal posi-
remain untouched (Hetzer et al. 1998). tion (Becker 1995). Although the anterior leaflet
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
379 12
is not usually displaced, it is almost always ana- 12.4.6 Classification
tomically abnormal. This leaflet may also have
multiple chordal attachments to the ventricular Carpentier’s surgical classification of Ebstein’s
wall. The displacement of the septal and posterior anomaly is valuable for making surgical decisions
valve components in the direction of the apex of as to the most appropriate procedure and for esti-
the right ventricular cavity divides the right ven- mating individual surgical risk (Carpentier et al.
tricle into a supravalvular portion that is known 1988; . Fig. 12.64): In type A (minimal disease),
as the atrialized chambers and a finally smaller the septal and posterior leaflet origins are only
subvalvular portion of the ventricle, the so-called moderately displaced into the right ventricle. The
true right ventricle. The true anulus of the tricus- atrialized chamber is small, and a relatively large
pid valve at the anatomic atrioventricular junc- trabecularized right ventricular cavity is pre-
tion, the right atrium, and the functional served. The anterior leaflet is large and mobile.
tricuspid valve anulus are always dilated, and the The volume of true right ventricle is adequate
tricuspid valve itself is usually incompetent. (. Fig. 12.64a). In type B (intermediate disease),
Dilatation of the right ventricle is associated not the relationship between the volume of the atrial-
only with thinning of the right ventricular wall ized chamber and true right ventricle is reversed:
but also with an absolute decrease in the myocar- a large atrialized chamber and a smaller contract-
dial fiber count in the right ventricular myocar- ing ventricle are both present. The anterior leaflet
dium (Anderson and Lie 1979). Significant is also large and mobile (. Fig. 12.64b). In type C
fibrosis of the left ventricular myocardium in (severe disease), the anterior leaflet is restricted
deceased neonates was also described (Celermajer in motion by its adherence to the endocardium of
et al. 1992b). the anterior right ventricular wall by a fibrous
band or abnormal chordae tendineae. This
restriction may cause significant stenosis of the
12.4.5 Associated Anomalies right ventricular outflow tract (. Fig. 12.64c). In
type D (lesion of the tricuspid valve sac), the
An atrial septal defect or persistent foramen ovale entire right ventricle is lined by broad adherent
is present in 90 % of the patients (Celermajer et al. fibrous leaflet structures and is almost completely
1992a; Watson 1974). In neonates with Ebstein’s atrialized, with the exception of a small infun-
anomaly, the incidence of important associated dibular portion. Thus, the entire right ventricle
cardiac anomalies is higher, 20–25 % (Arizmendi forms a so- called tricuspid valve sac
et al. 2004; Celermajer et al. 1992a, 1994; Kumar (. Fig. 12.64d).
et al. 1971):
55 Ventricular septal defects
55 Transposition of the great arteries 12.4.7 Preoperative Assessment
55 Tetralogy of Fallot and Preparation
55 Anomalous pulmonary venous drainage
55 Aortic isthmus stenosis 12.4.7.1 Preliminary Comments
55 Atrioventricular septal defects Patients with Ebstein’s anomaly become symp-
tomatic depending on the severity of the anom-
Abnormalities of the mitral valve in the form aly. Neonates who are highly symptomatic have
of fibrosis and prolapse were reported in 21 % of massive cardiac enlargement, severe cyanosis,
the patients (Cabin and Roberts 1981; Gerlis et al. and metabolic acidosis; they require urgent sur-
1993). Additional significant problems in these gery (Knott-Craig et al. 2002; Pflaumer et al. 2004;
patients include an paroxysmal supraventricular Reemtsen et al. 2006). In these patients, physio-
tachycardia, which occurs in 25–50 %. Between 5 logical pulmonary atresia is present due to
and 20 % of these patients have Wolff–Parkinson– inability of the right ventricle to produce suffi-
White syndrome (Arizmendi et al. 2004; Giuliani cient pressure to open the pulmonary valve. This
et al. 1979). Other important complications of situation causes a right-to-left shunt through an
Ebstein’s anomaly are cerebral abscesses and para- interatrial communication, which leads to sys-
doxical emboli (Genton and Blount 1967; temic arterial desaturation and cyanosis and, if
Mathews et al. 1983). not treated, to high mortality. The rate of survival
380 V. Alexi-Meskhishvili et al.
a b
right atrium
atrialized ventricle
residual, «true» right ventricle
c d
12
..Fig. 12.64 a–d Carpentier’s surgical classification of forms of the Ebstein’s anomaly
up to the age of 30 years is 65 % (Arizmendi et al. reached the age of 80 years have been reported
2004). Some adult patients may show symptoms (Lillehei et al. 1967; Seward et al. 1979). In these
of chronic heart insufficiency with progressive patients, the diagnosis is usually made by echo-
cyanosis, paradoxical embolization, cerebral cardiographic examination after cardiac enlarge-
abscess, and atrial or ventricular arrhythmias. ment has been detected.
Paroxysmal atrial tachycardias may cause pro- Patients with Ebstein’s anomaly and severe
gressive heart failure or worsening of cyanosis cardiac failure who are not candidates for surgery
and may even cause syncope. In the current era, are treated with standard heart failure therapy,
application of the right-sided or biatrial maze including administration of diuretics and digoxin
procedure is a routine part of our corrective (Attenhofer et al. 2007).
technique when atrial arrhythmias are present.
Some patients with the mild form of Ebstein’s 12.4.7.2 Typical Radiological Findings
anomaly may be oligosymptomatic or even The chest X-ray of the adult patient is character-
asymptomatic up to adulthood. Only rarely do ized by massive enlargement of the right atrium,
patients survive in the long term without an inter- resulting in a triangular heart silhouette with a
vention. However, unusual cases of patients who broad basis sitting symmetrically on the
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
381 12
diaphragm. The pulmonary arteries and the aorta
are usually narrow, and the pulmonary artery vas-
culature is rarefied.
A further indication is the need to clarify pre- In symptomatic neonates and small infants, a
operative arrhythmias by electrophysiological delay in surgical treatment usually results in very
evaluation. Electrophysiological mapping serves high mortality (Knott-Craig et al. 2002). In this
to locate and ablate accessory conduction path- group of patients, two different surgical strategies
ways (Hebe 2000). Intracardiac pressure record- have been applied: valve reconstruction (Knott-
ing and oximetric determination of intracardiac Craig et al. 2000, 2007) or the univentricular
shunts may be useful when it is necessary to approach and the Starnes technique (Reemtsen et al.
verify the echocardiographic findings. However, 2006; Starnes et al. 1991). Experience with both tech-
cardiac catheterization is not without risk. niques is limited, and further experience is necessary
Tachyarrhythmias associated with the procedure to establish which of these approaches is superior.
are relatively frequent (Davidson et al. 1995). In
the early 1960s noncontrollable, malignant supra- 12.4.7.6 Contraindications
ventricular arrhythmias in patients with Ebstein’s There are no specific contraindications to recon-
anomaly were reported (Watson 1974). structive surgical treatment in Ebstein’s anomaly
per se. The contraindications include general con-
12.4.7.5 Indications for Surgical traindications for open-heart surgery and the assess-
Treatment ment that a surgical intervention would not improve
The management of patients with Ebstein’s anom- the patient’s quality of life or life expectancy.
aly is aimed at preventing and treating complica- Relative contraindications to the cone operation
tions. In general medical management is include age above 50 years, moderately elevated
recommended only for patients with mild symp- pulmonary arterial pressure, reduced ejection frac-
toms. This approach concentrates on medication tion (less than 30 %), significant delamination of the
for heart failure, arrhythmias, and anticoagula- anterior leaflet and complete failure of delamina-
tion. The classic indication for operative treatment tion of the septal and inferior leaflets, severe right
is the presence of severe, chronic heart failure with ventricular dilatation, and severe dilatation of true
symptoms of f unctional New York Heart tricuspid anulus (Dearani et al. 2008).
Association (NYHA) class III or IV. However,
12 based on favorable results of surgical treatment,
the indications for operation have been consider- 12.4.8 Surgical Treatment Options
ably extended. Currently, most patients beyond
the early infancy can be operated upon with good 12.4.8.1 Introduction
results (Davies et al. 2013; Yu et al. 2013). The There is still controversy surrounding the best type
extended indications for surgical treatment of tricuspid valve repair; indications for valve
include the following (Hetzer and Pasic 2004): replacement, whether and how plication of the atri-
55 Less symptomatic patients with functional alized part of the right ventricle should be performed;
NYHA class II and progression of symptoms and the surgical strategies to follow in neonates
55 Increasing cyanosis (Boston et al. 2006; Chauvaud et al. 2003; Chen et al.
55 Deterioration of exercise tolerance 2004; da Silva et al. 2007; Dearani 2007; Friesen et al.
55 Retardation of growth curve in children 2004; Hetzer and Pasic 2004; Knott-Craig et al. 2007;
55 Paradoxical embolization and cerebral Reemtsen et al. 2006; Sano et al. 2002; Ullmann et al.
abscesses 2004; Yun et al. 2006a, b). It is of great importance to
55 Atrial and/or ventricular arrhythmias note that every patient with Ebstein’s anomaly is dif-
55 Increase in cardiac size ferent and that different reconstruction techniques
55 Worsening of the echocardiographic findings, need to be available and to be applied in each indi-
such as an increase in tricuspid valve regurgi- vidual situation (Dearani 2007).
tation Reconstruction of the tricuspid valve is preferable
55 Enlargement of the right atrium and right to valve replacement to avoid the well-known prob-
ventricle lems of valve prosthesis dysfunction and anticoagula-
55 Deterioration of right ventricular function tion and the need for repeat valve reconstruction
procedures in line with the growth of child patients.
These indications mostly apply for older chil- In view of the excellent results, it seems justifiable to
dren and adult patients. extend valve reconstruction to include patients who
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
383 12
would previously have been treated by valve replace- energy-saving effect on the right ventricle during
ment. In line with the hospital-specific guidelines, systole. The key point for the preoperative decision
valvuloplasty is possible in 40–98 % of patients, with to create an additional cavopulmonary shunt during
a reoperation rate of 3–15 %. Numerous surgical repair of Ebstein’s anomaly is the assessment of the
techniques and modifications have been suggested. right ventricular function. Unfortunately, in Ebstein’s
The five most important reconstruction techniques anomaly there is no reliable method to measure the
are those developed by the following authors: Hardy, right ventricular wall stress, ejection fraction, and
Danielson, Carpentier, Quaegebeur, Hetzer, and da shortening fraction in the deformed right ventricle.
Silva. Depending on whether and how plication of In order for the bidirectional Glenn shunt to
the atrialized chamber is performed, the techniques be considered a potential adjunct to the o peration,
can be regarded as falling into three groups: it is important that there should be good left ven-
55 Transverse plication (Danielson et al. 1992; tricular function and low left atrial pressure,
Hardy et al. 1964) (. Fig. 12.68) which is not always the case in advanced cases of
55 Longitudinal plication (Carpentier et al. 1988; Ebstein’s anomaly.
Quaegebeur and Sreeram 1991) (. Fig. 12.69,
. Fig. 12.70)
55 Without plication (Augustin and Schmidt- Because concomitant left ventricular
Habelmann 1997; Hetzer et al. 1998; Ullmann dysfunction may be present when the right
et al. 2004; daSilva et al. 2004, 2007) ventricle fails, it is important to document by
(. Fig. 12.71, . Fig. 12.72, . Fig. 12.74) direct pressure measurements that the left
atrial and pulmonary arterial pressures are
low; otherwise, the shunt will not be effective.
12.4.8.2 urgical Options for Very
S
Severe Anomalies (Types C
and D of Carpentier’s
Classification) In newborns and small infants, the Starnes tech-
In severely sick patients with the most unfavorable nique may be indicated. Here a fenestrated tricus-
anomalies, any of the techniques described above pid valve closure is combined with a central
carry a high operative risk. In adult patients and aortopulmonary anastomosis and an atrial
children, a combination of tricuspid valve recon- septectomy (Starnes et al. 1991; Van Son et al.
struction and a bidirectional cavopulmonary anas- 1998a, b, c). Alternatively primary orthotopic
tomosis can be applied (Carpentier et al. 1988; heart transplantation can be considered.
Chauvaud et al. 1998a, b). The bidirectional cavo-
pulmonary shunt should be used selectively when
the right ventricle is functioning poorly and there is
difficulty in weaning the patient from the cardio-
pulmonary bypass. Among 169 patients with
Ebstein’s anomaly, Quiñonez and associates used
additional bidirectional cavopulmonary shunt in b
14 patients with decreased right ventricular func-
tion (Quiñonez et al. 2007). In high-risk patients a
with Ebstein’s anomaly, an associated bidirectional c
cavopulmonary shunt seems to offer several distinct
advantages including decreased operative mortality
and better tolerance of the residual tricuspid valve
dysfunction. Bidirectional cavopulmonary shunt
may be considered as a planned procedure, as an
intraoperative salvage maneuver, or as an alterna-
tive to cardiac transplantation in selected patients ..Fig. 12.66 The right atrium can be opened in three
alternative ways: an arched incision a, an oblique inci-
(Chauvaud et al. 1998b; Quiñonez et al. 2007). sion from the right atrial appendage to the inferior vena
The mechanism of improvement of the bidirec- cava, b or a straight incision parallel to the interatrial
tional cavopulmonary shunt may be due to an groove c
384 V. Alexi-Meskhishvili et al.
..Fig. 12.67 a, b The intracardiac situs. In this complex anomaly, the pathological intracardiac anatomy differs greatly
between patients, and no one case is the same as others in all aspects. Certainly the characteristic findings of Ebstein’s
anomaly are easily identified in all patients. The anatomic anulus of the tricuspid valve is greatly dilated, and the origin
of the septal leaflet lies in the chamber of the right ventricle. The posterior and anterior leaflets show broad variations.
The attachment of the posterior leaflet to the anulus is also displaced in apical direction and leads to the formation of
the so-called atrialized chamber, the size of which can greatly vary (see 7 Sect. 12.4.6). In most cases the anterior leaflet
is the largest. This leaflet may be entirely mobile or may be partially or even completely fixed to the neighboring free
right ventricular wall by a varying number of fibrous bands. In this case the leaflet mobility is reduced. In some patients
the posterior leaflet is strong and mobile and of about the same size as the anterior leaflet or even larger. The anterior
leaflet can show a cleft or fenestration. Rarely one finds a fourth small accessory leaflet originating from the anterosep-
tal commissure
which provides total cardiopulmonary bypass, and (. Fig. 12.68). In this procedure the displaced
moderate hypothermia of 30 °C is selected. The septal and posterior leaflets are transposed to the
aorta is clamped and cardioplegic solution is given. normal anatomical anulus level of the valve. Thus,
the atrialized chamber is plicated and closed at
12.4.9.3 Myocardial Protection the transverse level, i.e., parallel to the true tri-
Myocardial protection is given in accordance with cuspid valve anulus. This leads the anterior leaflet
our internal cardiac surgery guidelines. At our body to the true tricuspid valve anulus and forms
institution cardioplegia is induced by infusion of the coaptation of the anterior leaflet with the atri-
crystalloid cardioplegia into the aortic root. The alized septum. Danielson modified this tech-
myocardial protection is maintained by repeated nique by performing an additional posterior
infusion of cold hydroxyethyl starch solution. anuloplasty to reduce the diameter of the tricus-
pid valve anulus.
12.4.9.4 Tricuspid Valve Some modifications of the original Danielson
Reconstruction reconstruction were reported by the Mayo Clinic
kTricuspid valve reconstruction with group (Attenhofer et al. 2007; Boston et al. 2006;
transverse plication (techniques of Hardy Dearani 2007). A patent foramen ovale is closed
and Danielson) by direct suture, and larger atrial septal defects are
Reconstruction of the tricuspid valve by this closed with an autologous pericardial patch. Right
technique (Danielson et al. 1979a, 1992; Hardy reduction atrioplasty is routinely performed for
et al. 1964) uses the mobility of the anterior leaf- an enlarged right atrium. The original technique
let as a closing mechanism for the valve of tricuspid valve reconstruction brought the
386 V. Alexi-Meskhishvili et al.
functional tricuspid anulus up toward the true and can thereby promote remodeling. Unlike the
anatomical tricuspid anulus; thus, it effectively Danielson technique, in which a monocusp valve
plicated the atrialized part of the right ventricle is created, this procedure creates a bileaflet valve
(. Fig. 12.68). At present valve reconstruction is (at least when a posterior leaflet, albeit morpho-
performed at the level of the functional tricuspid logically abnormal, is present). In general, we
anulus. In addition, contrary to early experience, do not consider atrial reduction a necessary part
plication of the atrialized portion is not per- of the procedure. Finally, anular reinforcing
formed unless the wall is severely thinned. A sutures or artificial polytetrafluoroethylene
right-sided maze procedure is performed in (Gore-Tex™) chords have been placed only in a
patients with a history of intermittent or chronic small number of cases (Chen et al. 2004).
atrial flutter or atrial fibrillation. Left-sided maze
procedure usually is not applied. kTricuspid valve repair without plication
(technique of Hetzer)
kTricuspid valve repair with longitudinal This technique as suggested by Roland Hetzer
plication (techniques of Carpentier and restores the valve mechanism on the plane of the
Quaegebeur) true tricuspid valve anulus by using the most
Carpentier’s method of reconstruction (Carpentier mobile leaflet for valve closure, without plicating
et al. 1988; . Fig. 12.69) consists of separating the the atrialized ventricle (. Figs. 12.71 and 12.72). It
anterior and/or posterior leaflet from their hinge provides sufficient reconstruction, even in cases
points at the anulus, transecting the connective where only the posterior leaflet or parts of the ante-
tissue bands between the leaflets and the right rior leaflet can be remodeled and used for valve
ventricular wall, longitudinal plication of the atri- closure (Hetzer and Pasic 2004; Hetzer et al. 1998).
alized chamber, and reinsertion of the leaflets into The technique introduced by us, making the
the true anulus above the plicated chamber. In atrialized chamber part of the right ventricle
addition the leaflets are displaced in clockwise without any plication, may be employed in two
direction toward the posterior part of the septal ways: (1) either doing a posterior plication of the
anulus. To reduce the tension on the new leaflet anulus, i.e., partial closure of the «true» ostium
12 attachment line, transection and reattachment of (see . Fig. 12.71), or (2) the creation of a «double-
the papillary muscle to the anterior leaflet may be orifice valve» by suturing of the anterior anulus to
necessary, particularly in the anterior portion. It is the «true» septal anulus (see . Fig. 12.72). The
highly recommended that this new valve anulus is decision in favor of posterior anulus plication is
stabilized with a prosthetic ring, as originally sug- made when the posterior leaflet of the posterior
gested by Carpentier’s group (Carpentier et al. portion of the anterior leaflet is not mobile and
1988; Chauvaud et al. 1996). not capable of coaptation. We prefer the double-
Quaegebeur modified the Carpentier tech- orifice valve when both the anterior and posterior
nique in two respects (. Fig. 12.70): instead of leaflet segments, or at least the anterior leaflet, are
suturing a ring to keep the anulus in a particular present, mobile, and capable of coapting with the
form and to reduce its size, he placed the reduc- newly created ostia. Division of the ostium by an
tion plasty along the posterior leaflet by using a anulus suture is carried out when the anterior
suture that additionally reduces the anulus size. leaflet has a deep fissure.
The second modification consisted of not apply- To stabilize the ostium division suture, we
ing separation and reimplantation of the papillary regularly add the so-called Sebening stitch. This
muscles of the anterior leaflet. stitch tacks a papillary muscle or a strong fibrous
Since Jan Quaegebeur’s original report of chorda out of center of the anterior leaflet to the
experience with his technique (Quaegebeur and septum, usually to the fibrous tissue of the rudi-
Sreeram 1991), various alterations have taken mentary, displaced septal leaflet (. Fig. 12.73).
place. We still believe that vertical plication of the
right ventricle restores the normal inlet configu- 12.4.9.5 Cone Reconstruction
ration of the right ventricle, reduces the tricuspid of Ebstein’s Anomaly
valve anulus, and supports the subvalvular appa- As described by da Silva (da Silva et al. 2004,
ratus. In addition, the plication itself likely helps 2007, da Silva and da Fonseca 2012), «After
to maintain a more normal ventricular geometry establishment of cardiopulmonary bypass and
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
387 12
a b
Functional anulus True anulus
Anterior
leaflet
AV-node
Coronary sinus
Patch closed ASD Atrialized ventricle
Untied anuloplasty suture
c
..Fig. 12.68 a–c Tricuspid valve reconstruction with plication of the atrialized chamber (Danielson technique).
a Apicobasal transverse ventricular plication sutures are placed. Teflon-pledgeted single-button sutures pierce the edge
of the atrialized chamber in ventricular direction and exit at the true tricuspid valve anulus. b Transverse plication of the
atrialized chamber is performed. The plication sutures are pulled tight, so that the posterior and the septal leaflet are
pulled in the direction of the true anulus and the atrialized chamber is closed in a transverse plane, parallel to the true tri-
cuspid valve anulus. c The valve anulus is reduced by a posterior anuloplasty, using Teflon-pledgeted polypropylene (3/0)
mattress sutures. The sutures of the posterior anuloplasty are placed in a plane with the sinus coronarius to avoid injury of
the cardiac conduction system. The tricuspid valve is tested for competence by instilling cold isotonic saline solution into
the right ventricle. After the correction the tricuspid valve functions as a monocuspid valve
cardioplegia, right atriotomy is performed» papillary muscles and other tissues between the
(. Fig. 12.74a). When creating a component tri- individual leaflets and the corresponding right
cuspid valve by the cone technique, it is very ventricular wall areas are divided, taking par-
important to perform extensive mobilization of ticular care to preserve the right ventricular
the displaced or tethered leaflets. The abnormal apex attachments.
388 V. Alexi-Meskhishvili et al.
a b
c d
12
e f
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
389 12
Division of fused papillary muscles, fenestra-
tion of obliterated interchordal spaces, and triangu-
lar resections on the leaflet distal third are required
to improve subsequent right ventricular inflow.
Afterward the septal edge of the a nterior leaflet is
carefully mobilized by freeing its connections to
the interventricular septum. Only the normal
attachment of the anterior leaflet to the tricuspid
anulus and proper subvalvular a pparatus
are left in place (. Fig. 12.74b). Then the free edge
of the posterior leaflet is rotated clockwise and
sutured to the anterior leaflet septal edge, forming
a new tricuspid valve resembling a cone. The new
valvular anulus is constructed at the anatomically
correct level by means of plication of the true tri-
cuspid anulus to match the proximal circumfer-
ence of the constructed anulus (. Fig. 12.74c). If
the septal leaflet it is too short to reach the true
..Fig. 12.70 Quaegebeur technique. A posterior anulo-
plasty of the tricuspid valve anulus is performed. In this tricuspid anulus, longitudinal elongation is
modified form of the Carpentier technique, no prosthetic achieved by plicating its proximal edge toward the
ring is used to reinforce the anulus. The anulus diameter is center. On completion of these preparatory steps,
reduced by a posterior anuloplasty with a 3/0 polypropyl- the anterior edge of the septal leaflet is sutured to
ene mattress suture reinforced with Teflon-felt pledgets.
the septal edge of the anterior leaflet. The atrial-
Unlike in the Carpentier technique, the newly inserted
leaflets are not subjected to excessive tension so that tran- ized RV is usually longitudinally plicated to
section and translocation of the papillary muscle are not exclude its thin part. Endocardial placement of
necessary. The posterior anuloplasty is performed close to this suture avoids damage to the coronary arter-
the coronary sinus, and it is important to avoid injury to ies. Atrial septal defect or open foramen ovale is
the conduction pathways
..Fig. 12.69 a–f Valve reconstruction with longitudinal plication of the atrialized chamber—Carpentier technique.
a, b The anterior and posterior leaflets are mobilized. The anterior leaflet and the adjacent part of the posterior leaflet are
separated from their anchorage along the anulus. The incision begins at the anterior commissure. The anchorage of the
anterior leaflet at the anteroseptal commissure should, however, remain untouched. The released leaflets are further
mobilized by transecting accessory trabeculae, fibrous bands, and adhesions to the ventricular wall. The interchordal
spaces are enlarged by fenestration if they are narrowed. The accompanying papillary muscle is also entirely mobilized
by transecting the muscle band that inserts at the lateral wall of the right ventricle. If the muscle is insufficiently mobi-
lized, the leaflet coaptation may be unsatisfactory when there is excessive tension on the leaflets. c, d Longitudinal plica-
tion of the atrialized chamber is performed. The chamber is closed by placing sutures vertically to the true tricuspid
valve anulus. For plication either Teflon-pledgeted single-button sutures or a continuous suture reinforced with several
single-button sutures is used. It is important to ensure that the ventricular wall is not perforated or the coronary arteries
injured. Plication of the atrialized chamber reduces the diameter of the tricuspid valve anulus. e The mobilized leaflets
are rotated and refixed. The anterior and posterior leaflets are turned in clockwise direction and then attached to the
new, narrower anulus with a continuous 5/0 polypropylene suture. The sutures should be placed at a distance from the
atrioventricular note to avoid atrioventricular block. The danger zone lies in the septal portion of the anulus and close to
the opening of the coronary sinus. For this reason the suture should end here. After the mobilized anterior leaflet has
been attached, it covers the whole valve opening area, and the valve functions as a monocuspid valve. In this manner
the tricuspid valve anulus is reduced by the size of the plicated atrialized chamber, and the anterior leaflet is swung in
clockwise direction to cover the whole anulus portion of the anterior and posterior leaflet. The valve is then tested for
competence by instilling cold isotonic saline solution into the right ventricle. After the correction the tricuspid valve
functions as a monocuspid valve by virtue of the anterior leaflet. If the leaflet coaptation is not competent due to insuffi-
cient mobilization of the papillary muscle, the muscle can be detached from the lateral ventricular wall and refixed at a
higher point on the ventricular septum. f The tricuspid valve anulus is reinforced. To stabilize the anulus, an anuloplasty
ring is implanted. This ring reduces the diameter of the dilated tricuspid valve anulus, thus enlarging the coaptation area
of the leaflet and improving the valve competence. The ring should not be used in children; in patients with form A
according to the Carpentier classification, it should not necessarily be applied
390
V. Alexi-Meskhishvili et al.
a b
right atrium
anterior leaflet
residual, true
ventricle
posterior leaflet
true anatomical anulus septal leaflet
atrialized ventricle
functional anulus
c d
12
..Fig. 12.71 a–h Hetzer technique: reconstruction of the valve without plication of the atrialized chamber—applied
in a patient with a large and intact anterior leaflet. a–c The leaflet is mobilized and the sutures placed. First the anterior
leaflet is assessed for mobility. If there are fibrous bands between the leaflet and the right ventricular wall, they are tran-
sected to improve leaflet mobility. The original valve opening is reduced in size by closing the posterior portion of the
valve opening with four to six individual mattress sutures of 3/0 polypropylene, reinforced with autologous pericardium
or Teflon pledgets. The diameter of the newly created tricuspid valve opening should be at least 2.5 cm. So that this size
is reached, the first suture is placed as a trial suture. The suture runs through the true anulus at the area of the anterior
leaflet and then through the opposite point on the septum. Thus, the suture divides the original valve opening into two
parts. The anterior portion becomes the new valve opening and the posterior portion is closed. The valve is then tested
by instilling cold isotonic saline solution into the chamber of the right ventricle to show that the anterior leaflet can
effectively close the new valve opening. The remaining sutures are placed consecutively, to close the posterior portion
of the tricuspid valve orifice. The suture line is placed in the plane of the true tricuspid valve anulus, leaving out the sep-
tal region. To avoid injury to the atrioventricular node in this area, the suture line is placed in the muscular part of the
atrialized chamber, just below the real anulus. The atrialized chamber is not plicated. d, e The posterior portion of the
anulus is closed. The pre-laid sutures are pulled tight, closing the posterior part of the original valve orifice. Thus, the
anterior anulus is brought closer to the septum. f, g Next, the valve is tested for competence by filling the right ventricle
with isotonic saline solution so that the anterior leaflet coapts with the atrialized septum. The atrialized chamber is
thereby included in the chamber of the contracting ventricle. h Rarely is it necessary to correct the valve opening by
additional narrowing of the anteroseptal commissure if residual valve insufficiency is present
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
391 12
e f
g h
..Fig. 12.71 (continued)
usually valve closed which permits the flow of 12.4.9.6 Tricuspid Valve
blood in one direction only (. Fig. 12.74d). Replacement
Because of suture dehiscence at the septal and In general TV repair is preferable to valve replace-
posterior part of new tricuspid anulus in some of ment whenever it is feasible. Depending on the
their patients, da Silva suggested the need to rein- experience of the groups concerned, the rate of
force the new tricuspid valve ring with additional valve replacement differs greatly, between 2 and
interrupted sutures in children or with an anulo- 50 %. A clear indication for tricuspid valve
plasty ring in adult patients (da Silva and da Silva replacement is given when the valve tissue is not
2012). present or in the case of a Type D lesion according
392 V. Alexi-Meskhishvili et al.
a b
c d
12
..Fig. 12.72 a–d Hetzer technique: reconstruction of the valve without plication of the atrialized chamber—when the
anterior leaflet is divided by a cleft or fissure. a The anterior leaflet is inspected and mobilized. Here it is divided by a cleft
and deep fissures. Several chordae adhesions to the right ventricular wall are transected. b The sutures are placed. Two
polypropylene mattress sutures reinforced with autologous pericardium or Teflon-felt pledgets pierce the middle section of
the anulus in the area of the cleft and the opposite part of the atrialized septum, just below the septal anulus. c A new valve
morphology is created. The pre-laid sutures are pulled tight and the anterior anulus is drawn toward the septum. Thus, the
valve receives two openings. The two individual parts of the anterior leaflet coapt with the opposite septum. d Competence
of the valve closure is tested by filling the right ventricle with isotonic saline. Both valve openings are closed by both parts
of the anterior leaflet. Thus, the atrialized chamber is incorporated into the contracting right ventricle
to the Carpentier classification («tricuspid valve This allows the coronary sinus to drain into the
sac»; Boston et al. 2006). right ventricle. However, complete AV block can-
The prosthesis can be implanted by the stan- not always be avoided, and some patients need
dard procedure with insertion of the prosthetic cardiac pacemaker implantation. The standard
suture ring into the true tricuspid anulus or in a procedure for prosthetic valve implantation may
slightly modified procedure, which was recom- or may not be accompanied by plication of the
mended by Barnard and Schrire (1963) atrialized chamber. If the atrialized chamber is
(. Figs. 12.75, 12.76, and 12.77). In their classic excessively dilated and the volume of the
work, they described how the valve prosthesis is functional, true right ventricle is normal, plication
placed at the atrial side of the coronary sinus, to of the atrialized chamber may be performed dur-
reduce the risk of complete atrioventricular block. ing the valve implantation. There is no consensus
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
393 12
a
Moderate or severe residual tricuspid
regurgitation early after reconstruction
surgery in Ebstein’s anomaly is a significant
risk factor for reoperation (Boston et al.
2006).
a b
c d
12
..Fig. 12.74 a–d Operative steps for Ebstein’s anomaly repair using cone reconstruction. a Opened right atrium
showing displacement of the tricuspid valve. TTA true tricuspid anulus, ASD atrial septal defect, CS coronary sinus.
b Detached part of the anterior and posterior leaflet forming a single piece. c Clockwise rotation of the posterior leaflet
edge to be sutured to the anterior leaflet septal edge and plication of the true tricuspid anulus. d Complete valve
attachment to the true tricuspid anulus and valve closure of the atrial septal defect (From da Silva et al. 2007;
used with permission)
a b
..Fig. 12.75 a–c Tricuspid valve replacement: standard implantation without plication of the atrialized chamber. The
leaflets and chordae attachments remain in situ, to support right ventricular function. When a right ventricular outflow
tract obstruction is caused by leaflet tissue, commissurotomy-like radial incisions are made in the leaflet tissue to open
the outflow tract. If necessary, single mattress sutures with Teflon-pledgeted reinforcement are placed so that they
pierce the tissue of the true tricuspid valve anulus and then the prosthetic ring. It is important to carefully avoid the His
bundle by placing the sutures in the septum, at least between the coronary sinus and the tricuspid valve anulus. A pros-
thesis, which should normally measure 31–33 mm, is positioned in the anulus. When the sutures are pulled tight, the
suture ring of the prosthesis comes to rest on the anatomic tricuspid valve anulus. The atrialized chamber has not been
plicated and its position is now below the prosthesis
atrial filling pressure, the patient is weaned from terpulsation. Nitric oxide can be added to the ven-
extracorporeal circulation. tilator air to reduce the pulmonary vascular
resistance if the pulmonary artery pressure is
kPerioperative and postoperative care elevated; the indication should be made gener-
For weaning from extracorporeal circulation, ino- ously to relieve the right ventricular workload.
tropic support may be necessary and, in rarer Otherwise, the general institutional principles
cases, mechanical circulatory support, for applied in patients after open-heart surgery are
example, in the form of intra-aortic balloon coun- followed.
396 V. Alexi-Meskhishvili et al.
..Fig. 12.76 Tricuspid valve replacement: standard ..Fig. 12.77 Tricuspid valve replacement: classic tech-
implantation with plication of the atrialized chamber. If nique as described by Barnard and Schrire in (1963). The
the atrialized chamber is extremely dilated and the vol- suture line is placed posterior to the AV node and around
ume of the functional right ventricle is normal, atrialized the coronary sinus, to avoid injuring the conduction sys-
chamber plication may be performed in addition to the tem. In this way the valve prosthesis lies on the atrial side
prosthesis implantation. Individual Teflon-reinforced mat- of the coronary sinus, which thus drains into the low-pres-
tress sutures are placed close together through the false sure right ventricle. The atrialized chamber is not plicated.
anulus, at the insertion line of the posterior and septal If, however, plication of the atrialized chamber is desired,
leaflet, through the true anulus and finally through the an additional suture line must be placed to avoid constric-
suture ring of the prosthesis. Once the sutures are pulled tion of the coronary sinus
tight, the atrialized chamber is put out of action. The tri-
cuspid valve leaflets are not excised
12
4mm
..Fig. 12.78 a, b Starnes’ procedure. a A glutaraldehyde-fixed autologous pericardial patch is sewn into the anatomic
tricuspid valve anulus. b A fenestration is created in the patch using a 4-mm coronary punching device. The coronary
sinus still drains on the right atrial side of the patch
and severe right ventricular outflow tract University of Oklahoma reported for the first time
obstruction, a systemic artery-to-pulmonary successful biventricular Ebstein’s anomaly correc-
artery shunt is created in addition to the inter- tion in three cyanotic neonates with severe tricus-
atrial communication being enlarged (Starnes pid valve regurgitation (Knott-Craig et al. 2000).
et al. 1991) (. Fig. 12.78). The surgical techniques included reconstruction
of the tricuspid valve as a monocuspid valve, right-
12.4.10.2 Cardiopulmonary Bypass sided ventricular plication, subtotal closure of the
and Right Atriotomy atrial septal defect, and correction of associated
After median sternotomy a piece of pericardium heart defects (. Fig. 12.79) (Knott-Craig and
is harvested as patch material. Cardiopulmonary Goldberg 2007). A large and mobile anterior leaf-
bypass is established using standard aortic and let was present in all three patients, and this was
bicaval cannulation, and under moderate hypo- used in the valve repair, which was very similar to
thermia (28 °C), the heart is arrested with that described by Danielson (Danielson et al.
cardioplegic solution. Through an oblique right 1992).
atriotomy, the tricuspid valve is inspected and If the tricuspid valve is not amenable to
evaluated for possible reconstruction. The rela- repair or the remaining functional portion of the
tion in size between the atrialized chamber and right ventricle is inadequate or there is a right
the trabeculated (functional) right ventricle is ventricular outflow tract obstruction that can-
determined, and then the leaflet tissue is not reasonably be corrected, then right ventricu-
addressed. It is very important to analyze the pos- lar exclusion—the univentricular strategy—is
sibility of mobilizing the anterior leaflet. This leaf- undertaken (the Starnes procedure . Fig. 12.78;
let must be able to cover the main part of the new Starnes et al. 1991). This is performed by patch-
tricuspid valve orifice. Therefore, the remnants of ing the tricuspid valve with a glutaraldehyde-
the small tethered septal and posterior leaflets are fixed autologous pericardial patch or Gore-Tex
also assessed for their ability to be integrated in patch sewn at the anatomic level of the tricuspid
the reconstruction plasty. The right ventricular anulus. A 4-mm fenestration of the patch is then
outflow tract is investigated. If the ventricle is tri- made with a coronary punch. The coronary sinus
partite and tricuspid valve tissue is adequate, is left on the right atrial side of the patch, con-
reconstruction of the valve is undertaken by the trary to the original Starnes technique (Reemtsen
Danielson or Hetzer method. et al. 2006; Starnes et al. 1991). The opening in
Experience with biventricular correction of the interatrial septal opening is enlarged. A
Ebstein’s anomaly in newborns and small infants is right atrial reduction plasty is carried out and,
very limited. Only one surgical team from the depending on the size of the atrialized portion,
398 V. Alexi-Meskhishvili et al.
a b
Caudal orifice
Principle
tricuspid
Fenestrated ASD patch valve orifice
c
Pericardial patch
12
Fenestrated original
anterior leaflet
Pericardial patch
..Fig. 12.79 a–c Knott-Craig’s technique for correction of Ebstein’s anomaly in neonates. a The atrial septal defect is
closed with a fenestrated patch. For the anuloplasty a suture is placed so that its one pledgeted end is in the coronary
sinus and the other pledgeted end comes to rest between the anterior and posterior leaflet. b The orifice of the tricus-
pid valve is divided into two openings by pulling tight the pre-placed suture. Insert b Once the valve has been shown to
be sufficiently competent, the caudal orifice is closed and the atrialized part of the right ventricle is thus plicated. c A
competent monocuspid valve is created by detaching the anterior leaflet from the anulus. The native leaflet is fenes-
trated and enlarged with a pericardial patch. Insert c Cross-sectional schematic view of extended anterior leaflet, fenes-
trated in its native component
right ventricular plication. Care must be taken to 1998a, b, c). Finally, pulmonary blood flow is pro-
avoid kinking of the right coronary artery (see vided by a modified Blalock–Taussig shunt
. Fig. 12.78). (5–4 mm). The atriotomy is closed. Usually the
Next, the right ventricular outflow tract is sternum is electively left open and closed 2–3 days
addressed. To correct pulmonary valve insuffi- after the operation (Reemtsen et al. 2006).
ciency, the pulmonary trunk is interrupted and At about 6 months of age, a bidirectional supe-
oversewn (Reemtsen et al. 2006; Van Son et al. rior vena cava-to-pulmonary artery anastomosis
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
399 12
tricular correction of Ebstein’s anomaly in 524 con-
secutive patients. Tricuspid valve reconstruction
was performed in 189 patients and valve replace-
ment in 335 patients, most of whom received a bio-
prosthesis. Antiarrhythmic surgery was carried out
in 108 patients. The authors replaced the valve only
when it was not possible to entirely detach the
anterior leaflet and when strong linear adhesions
or numerous thread-like connections were present
between the free end and the myocardium below
it. Since 1999 the early mortality has been 2.4 %.
The rate of freedom from reoperation due to tri-
cuspid valve regurgitation after 5, 10, and 15 years
was 91 %, 80 %, and 68 %, respectively. There were
no significant differences in probability of reopera-
tion between patients with valve reconstruction
Atrial septal defect and those with valve replacement. Moderate or
Coronary sinus severe tricuspid valve insufficiency on hospital dis-
Patch closed tricuspid valve charge was a significant risk factor for reoperation
only in the group of patients who received valve
..Fig. 12.80 Exclusion of the right ventricle by the reconstruction.
Sano technique Chauvaud and associates from France
(Chauvaud et al. 2003) reported on 191 patients
and shunt takedown are performed. Fontan com- who were operated on using the technique of
pletion (inferior vena cava-to-pulmonary artery mobilization of the anterior leaflet introduced in
anastomosis) is usually performed when the 1988 by Carpentier (Carpentier et al. 1988).
patient is aged 2–4 years. Conservative surgery was possible in 187 patients
There are diverse modifications of the original (98 %) and included mobilization of the anterior
Starnes procedure. Sano and colleagues devel- leaflet, longitudinal plication of the atrialized
oped a procedure for complete exclusion of the right ventricle, and prosthetic anuloplasty with a
right ventricle in which also a part of the right ven- prosthetic ring in adults. A bidirectional cavo-
tricle is resected to reduce its size (Sano et al. pulmonary shunt had to be performed in 60
2002; . Fig. 12.80). A further modification was patients. Four patients had valve replacement.
published by Yun’s group (Yun et al. 2006b). Hospital mortality occurred in 18 patients (9 %)
and in nine patients due to right ventricular fail-
ure. Actuarial survival during the mean follow-
12.4.11 Results of Surgery up of 6.4 years was 82 % at 20 years. Tricuspid
for Ebstein’s Anomaly valve insufficiency was of grade I or II in 80 % of
the cases. Reoperation was necessary in 8 % (16
Many early studies found a 25–30 % hospital mor- patients). The second procedure was successful
tality rate. Late results are influenced by arrhyth- in ten patients. Electron beam computerized
mias, problems with artificial valve prostheses, tomography (in 20 patients) demonstrated that
and intrinsic primary myocardial disease. left ventricular ejection fraction improved from
Recent reports show decreased mortality and 56 to 66 % (p < 0.05). The number of patients
morbidity, compared with the early reports, but with supraventricular tachycardia and preexcita-
there is still controversy surrounding the question tion syndromes was reduced from 23 to 5.
of whether to recommend early surgery in asymp- Chen from Jan Quaegebeur’s group at
tomatic patients. It has been shown that some of Columbia University reported on 25 patients
these patients remain symptom free or have only (19 children and 6 adults) who underwent valve
mild symptoms for many years. reconstruction using the Quaegebeur tech-
Stulak and colleagues from the Mayo Clinic nique, which involved vertical plication of the
(Stulak et al. 2007) published the results of biven- atrialized ventricle and reimplantation of the
400 V. Alexi-Meskhishvili et al.
valve leaflet after clockwise rotation (Chen et al. Heart Surgeons Association in 2006 investigat-
2004). The average age at the time of operation ing the surgical treatment of Ebstein’s anomaly
was 14.2 ± 15.9 years, and the average follow- in 150 patients. The surgical procedures
up was 4.1 ± 3.4 years. Three patients required (n = 179) included 60 valve replacements, 49
reoperation for right ventricular overload (one valve reconstructions, 46 one-and-a-half ven-
child) and progressive severe tricuspid regurgi- tricle reconstructions, 13 palliative shunts, and
tation (two adults). Both adults received tricus- 11 other complex procedures. There were 20
pid valve replacements, one at 4 years and the hospital deaths (perioperative mortality
other at 8 years postoperatively. Three patients 13.3 %). Perioperative mortality did not differ
had radiofrequency ablation procedures per- significantly among repair, replacement, and
formed intraoperatively. Ten patients (40 %) one-and-a-half ventricle repair but was higher
had moderate-to-severe tricuspid regurgitation in patients with severe forms of the disease who
perioperatively. However, 18 children (95 %) underwent palliative procedures early in life.
and 5 adults (83 %) demonstrated significant Young age seemed to be the only independent
improvement in exercise capacity late postoper- predictor of perioperative mortality (Sarris
atively. Two children died suddenly 11 months et al. 2006).
and 4 years after the corrective procedure. A conic reconstruction of the tricuspid
The group from the German Heart Center valve and longitudinal plication of the atrial-
in Munich reported results of a technique of ized part of the right ventricle was suggested by
Ebstein’s anomaly repair in 23 patients by which da Silva and associates (2007). They reported
a predominantly monocuspid valve is created, the results of repair of Ebstein’s anomaly in 40
with simultaneous ventricularization of the atri- patients. An atrial septal defect, if present, was
alized right ventricular chamber (Augustin and closed with a patch. There was 2.5 % mortality.
Schmidt-Habelmann 1997; Ullmann et al. 2004). Two patients later required tricuspid valve re-
Mortality was 4 % (one patient). Valvuloplasty reconstruction. No atrioventricular block
consisted of creating a predominantly mono- occurred nor was a tricuspid valve replacement
cuspid valve at the level of the anatomical necessary at any time during the mean follow-
12 atrioventricular junction resulting in ventricu- up time of 4 years.
larization of the atrialized chamber. Important Boston and associates from the Mayo Clinic
recurrent atrioventricular valve regurgitation published the results of their surgical treatment
developed in three patients (13 %). The cause of Ebstein’s anomaly in 186 children aged
was rupture of the fixation suture. Reoperation <13 years (Boston et al. 2006). A total of 117
was necessary: two patients underwent repeat patients received tricuspid valve replacement.
valvuloplasty and one patient tricuspid valve Tricuspid valve reconstruction and anuloplasty
replacement. One patient presenting with hypo- were carried out in 52 patients with Ebstein’s
plastic right ventricle and subsequent right heart anomaly of forms A and B according to the
failure underwent creation of a total cavopul- Carpentier classification (1988). The authors
monary connection. During a follow-up of 4.6 applied valve-preserving reconstruction tech-
(0.5–10.9) years, all patients were doing well and niques only in cases in which at least 50 % of the
showed significant improvement of right atrio- anterior leaflet could be detached and the ante-
ventricular valve regurgitation and favorable rior end was not fixed to the endocardium. The
development of the right ventricular geometry early mortality rate in the reconstruction group
and function, as documented by serial echocar- was 5.8 %; for the last 25 years, since 1981 (31
diographic examination. The authors concluded patients), there had been no deaths. The risk
that their technique of Ebstein’s anomaly repair factors were age <2.5 years and weight of
with ventricularization of the atrialized cham- <10.7 kg. Morbidity consisted of transient
ber provides excellent results regarding right arrhythmias of atrial and ventricular origin and
atrioventricular valve function and leads to the need for early reoperation (n = 3). No
favorable restoration of RV geometry and func- patient required a permanent pacemaker.
tion (Ullmann et al. 2004). Actuarial survival following tricuspid valve
George Sarris reported the results of a mul- reconstruction after 5, 10, and 15 years was
ticentric study by the European Congenital 92.3 %, 89.9 %, and 89.9 %, respectively. The
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
401 12
rate of freedom from reoperation at 5, 10, and Van Praagh et al. (1964). Rastelli and colleagues
15 years was 91.0 %, 76.9 %, and 61.4 %. (Rastelli et al. 1968a, b) identified the malalign-
Moderate-to-severe tricuspid valve regurgita- ment between the atrial and ventricular septum as
tion before hospital discharge was the only risk a characteristic feature of hearts with overriding
factor for reoperation. Tricuspid valve stenosis valves and described the straddling phenomenon
did not occur in any of the patients. In the long- of the tricuspid valve in cases of atrioventricular
term follow-up, 89 % of the patients were in septal defects.
NYHA classes I and II. Straddling and overriding are often observed
A recent survey of a total of 595 patients with in combination with other complex congenital
Ebstein’s anomaly showed an overall in-hospital heart defects and are always associated with the
mortality of 5.9 %. Mortality among the neona- presence of a ventricular septal defect or a com-
tal population was between 22.7 and 26.9 % mon ventricle (Huhta et al. 1982a, b; Khairy et al.
depending on need for multiple o perations. 2007; Liberthson et al. 1971; Reddy and
Mortality in infants was 4.1 % and in children McElhinney 1997; Serraff et al. 1996; Tabry et al.
and adults 0.7 % and 1.1 %, respectively (Davies 1979; Van Son et al. 1998).
et al. 2013). Fetal distress and pulmonary atre- Since the introduction of echocardiography,
sia/stenosis are significant predictors of mortal- this anomaly has been more frequently recog-
ity in neonates with Ebstein’s anomaly (Yu et al. nized and can be exactly described in patients
2013). with complex congenital heart disease. On the
In 2006 Starnes’ group reported having treated other hand, angiography plays only an inferior
14 patients with his procedure, with total mortal- role (Delius et al. 1996; Freedom and Van Arsdell
ity of 30 %. Survival was improved within that 1998; Freedom et al. 1978; Güeven et al. 2003;
series, after a 4-mm fenestration of the tricuspid Huhta et al. 1982b; Seward et al. 1975; Yoshida
valve patch was introduced to avoid right ventric- et al. 1977).
ular distention and after the coronary sinus was Knowledge of the straddling phenomenon
retained on the right atrial side of the tricuspid in one of the atrioventricular valves is particu-
valve patch (Reemtsen et al. 2006). larly important for the choice of surgical strat-
Knott-Craig described biventricular repair of egy (univentricular or biventricular correction),
Ebstein’s anomaly in a consecutive series of 20 which depends on the type of straddling and on
neonates and four young infants, with a survival whether correction is possible without produc-
rate of 70 %. Three patients needed tricuspid valve ing valve incompetence or total atrioventricular
replacement during the median follow-up period block. The presence of such an anomaly can, in
of 4.5 years (Knott-Craig et al. 2007). some patients with transposition of the great
vessels and a ventricular septal defect and other
complex heart defects, mitigate against a
12.5 verriding and Straddling
O Rastelli correction, particularly when the clo-
Atrioventricular Valves sure of the ventricular septal defect would lead
to AV valve dysfunction (Anderson and Ho
12.5.1 Introduction 1998; Freedom and van Arsdell 1998; Huhta et
al. 1982b; Khairy et al. 2007; Russo et al. 1988;
Overriding and straddling phenomena of the van Arsdell 2006).
atrioventricular valves are rare: their rate of
occurrence in relation to all congenital heart
defects is between 0.3 and 3 %. Then it is particu- 12.5.2 Definitions
larly the tricuspid valve and hearts with discor-
dant atrioventricular connection that are affected An atrioventricular (AV) valve consists of the
(Liberthson et al. 1971; Milo et al. 1979; Reddy anulus, leaflets, and subvalvular apparatus.
and McElhinney 1997; Jacobs and Mayer 2000; Normally each AV valve has a morphological rela-
Güeven et al. 2003; Kiraly et al. 2007). tionship to a corresponding ventricle. This means
The phenomena of straddlings were described that the valve anulus with its leaflets is entirely
probably for the first time by Lambert (1951) and above this ventricle, and the subvalvular appara-
later analyzed in detail in univentricular hearts by tus is anchored in this ventricle.
402 V. Alexi-Meskhishvili et al.
Straddling of an AV valve arises when the sub- ventricular connection (univentricular heart).
valvular apparatus originates from both sides of In hearts with less than 50 % overriding of an
the ventricular septum, so that the opening usu- AV valve, i.e., if the larger part of the valve sec-
ally comes to lie above both ventricular chambers. tion is related to the corresponding chamber
Straddling often occurs above a ventricular septal (the secondary chamber; Anderson et al. 1981),
defect of the inlet septum. Using this definition a it is known as a biventricular atrioventricular
common AV valve almost always shows strad- connection (biventricular heart). The different
dling. However, there is no consensus on whether forms of straddling depend on determination
in this case one should speak of a straddling phe- of the atrioventricular connection.
nomenon (Liberthson et al. 1971; Milo et al. 1979)
or whether the concept «straddling» should be
reserved for describing either the mitral or tricus- 12.5.3 Surgical Anatomy
pid valve when its papillary muscles are connected
to both sides of the septum. The phenomenon of overriding AV valves sug-
In overriding valves, the subvalvular appara- gests, in addition to the valve anomaly, abnormal-
tus originates entirely in the original chamber, but ities of the atrial and ventricular septum
the AV valve anulus «rides» above a ventricular (malalignment, ventricular septal defect), the
septal defect so that there is a communication ventricular conduction system, and the ventricu-
with both ventricles. lar morphology.
The origin of the overriding and straddling
phenomena lies in the malalignment between the 12.5.3.1 AV Valves
anterior edge of the atrial septum and the crest of Only AV valves can show the straddling phe-
the muscular septum (Anderson and Shirali 2009; nomenon, since arterial valves have no tensor
Rastelli et al. 1968a, b). apparatus (Anderson and Shirali 2009). Only one
The chamber in which the whole tensor appara- AV valve can override in a heart. There are four
tus of an AV valve and parts of the tensor apparatus possible forms of AV valve overriding: overriding
of the contralateral AV valve insert is known as the mitral or tricuspid valve each in atrioventricular
12 primary chamber (Anderson et al. 1981) or as the concordance or discordance (Anderson 1997;
dominant ventricle. The chamber in which only one Güeven et al. 2003; Ho 2003; Jacobs and Mayer
part of the tensor apparatus of the straddling AV 2000; Khairy et al. 2007). Independently of the
valve inserts is regarded as the secondary chamber type of atrioventricular connection and the
or «incomplete ventricle.» The latter chamber is extent of the overriding, an overriding tricuspid
typically much smaller or even hypoplastic. valve is practically always above the posterior
Overriding and straddling phenomena are portion of the interventricular septum. In the
found both with distinct right and left AV valves area of the overriding, there is malalignment
and with a common AV valve (AV septal defect between the atrial and ventricular septum, and
anomalies). Further, they have been observed to the ventricular septum is not developed as far as
be independent of the atrial site (solitus, inversus, the crux cordis in posterior direction. In the con-
or ambiguous) and from the type of atrioventric- trary case of the mitral valve, the anteromedial
ular connection (concordant, discordant, or uni- portions are usually affected by overriding and
ventricular) (Güeven et al. 2003). straddling so that the valve rides above the ante-
Overriding and straddling of an AV valve usu- rior interventricular septum. In this situation the
ally occur together. Very rarely they are found septum is developed up to the crux cordis
with two AV valves. However, both straddling and (Anderson 1997; Anderson et al. 1981; Becker
overriding can occur in isolation (. Fig. 12.81). et al. 1980; Ho 2003).
Both overriding and straddling of AV valves The straddling of parts of the subvalvar appa-
are observed in different forms. When the over- ratus of an AV valve can be slight, moderate, or
riding applies to more than 50 % of the cross severe. That is to say that the chordae tendineae
section of the valve, i.e., the larger part of the that cross the ventricular septal defect may insert
AV valve counts as part of the dominant at the contralateral side of the septum very close
ventricle, the phenomenon is known as «dou- to the ventricular septal defect or 2–3 cm away
ble-inlet» ventricle with a univentricular atrio- from it or crossing the cavum of the contralateral
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
403 12
a b
RA LA
RV
..Fig. 12.81 a, b Illustration of the concepts of overriding and straddling. a A straddling valve has its subvalvar appa-
ratus originating in both ventricles. Its valvular orifice overrides the septum in all cases. b Isolated overriding occurs
with a subvalvar apparatus originating entirely and exclusively in one ventricle
ventricle and inserting at its free wall. In accor- type of malalignment VSD with an overriding
dance with these variants, Seward et al. (1975) tricuspid valve from defect of the AV canal type
and Tabry and associates (Tabry et al. 1979) sug- (AV septal defect) (Anderson 1997). The
gested classification into types A, B, and C malalignment defect with overriding tricuspid
(. Fig. 12.82). The opening area of the abnormal valve is not an AV septum defect: the difference
AV valve is normally relatively large; the contra- is that in AV septum defects the septum, although
lateral valve is smaller or frequently even hypo- deficient, is developed up to the crux cordis
plastic (Milo et al. 1979). (Anderson 1997).
When straddling of the morphological tri- With an overriding/straddling mitral valve,
cuspid valve is present, creating a double-inlet the anteromedial valve portions are normally
connection, the dominant ventricle always involved in the overriding or straddling process,
shows the morphology of a left ventricle. The so that here there is often a VSD in posterior
rudimentary and incomplete right ventricle can position. The posterior parts of the septum are
be on the right or left side. In this form the strad- intact. The focus of the anomaly on the anterior
dling and overriding is extreme, and the tensor septum also explains the frequent combination
apparatus is almost always attached to the pari- with defects such as the double outlet right ven-
etal wall of the dominant left ventricle (Anderson tricle and the subpulmonary VSD (Taussig–Bing
and Shirali 2009). anomaly) or transposition of the great arteries
(Anderson et al. 1981; Freedom et al. 1978; Milo
12.5.3.2 Ventricular Septal Defect (VSD) et al. 1979; Niinami et al. 1995; Ostermeyer et al.
All opinions published so far confirm that a large 1980; Serraff et al. 1996).
ventricular septal defect (VSD) is a necessary part
of straddling anomalies of the AV valves. 12.5.3.3 Conduction System
In all cases of overriding/straddling tricuspid The position and the course of the specific con-
valve, there is malalignment between the atrium duction structures (AV node, «penetrating» His
and chamber inlet septum. The VSD is in poste- bundle, ventricular bundle branches) depend
rior position, and the overriding phenomenon on the type of atrioventricular connection (con-
concerns the posteroseptal valve portions. The cordant or discordant) and the anatomy of the
VSD extends to the crux cordis (marginal peri- posterior atrioventricular junction (alignment
membranous VSD, juxtacrux VSD; Milo et al. or malalignment of the septa). When there
1979) and «prevents» the (inlet) septum from is no malalignment between the atrial and
being formed. It is important to distinguish this ventricular septum, i.e. the ventricular septum
404 V. Alexi-Meskhishvili et al.
a b c
..Fig. 12.82 a–c Classification for AV-valve straddling according to the site of insertion of the papillary muscle in the
opposite ventricle by Tabry et al. (1979). a Type A: insertion at the other side of the ventricular septum or at the edge of
the ventricular septal defect; b type B: insertion at the opposite side of the ventricular septum and removal from the rim
of the ventricular septal defect; c type C: insertion at the opposite free wall of the ventricular chamber
is developed in posterior direction up to the Milo et al. 1979). The AV node is found lateral to
crux cordis and also atrioventricular concor- the atrial septum in a position directly below the
dance is present, the position and course of the point at which the ventricular inlet septum
conduction system can be expected to be nor- reaches the tricuspid anulus (Milo et al. 1979).
mal, with a regular AV node in the Koch trian- The course of the His bundle is in the area of the
gle and posterior course of the His bundle with posterior rim of the VSD. Although Serraf and
normal branching. These relations are found colleagues (1996) assumed that the conduction
only in hearts with atrioventricular concor- system in biventricular heart with a VSD was
dance. In those with overriding tricuspid valve similar to that in hearts with a simple VSD, this
(malalignment between the atrial and ventricu- thesis has not been supported by anatomical evi-
12 lar septum, juxtacrux VSD), an anomalous dence (Anderson 1997).
position of the AV valve and an abnormal
course of the His bundle are to be expected. 12.5.3.4 Ventricular Morphology
Milo and colleagues reported that the conduc- Apart from atypically inserted chordae tendineae
tion path tissue does not originate from the and papillary muscles, the ventricles frequently
regular AV node and that, in biventricular show differences in size. Almost always there is
hearts with normal AV junction, the crux is hypoplasia of the secondary chamber (incomplete
derived from the conduction path tissue» (Milo ventricle) and a relatively larger primary chamber,
et al. 1979). In the case of atrioventricular dis- which still communicates more or less with the
cordance (isolated ventricle inversion, cor- overriding contralateral valve in addition to the cor-
rected transposition of the great arteries), the responding AV valve (Anderson et al. 1981; Corno
ventricular conduction system originates from 2005; Hanley 1999; Milo et al. 1979; Muster et al.
an AV node in anterolateral position, indepen- 1979; Seward et al. 1975; Van Praagh et al. 1964).
dently of the VSD position and of which AV
valve is overriding, and takes its course in the
area of the anterior circumference of the VSD 12.5.4 Surgical Treatment
(Anderson 1997). Here the determining factor
of the position of the specific musculature is the The corrective procedure for atrioventricular
atrioventricular discordance. valves with the straddling phenomenon was first
In hearts with atrioventricular concordance described in 1979 (Pacifico et al. 1979; Tabry et al.
and overriding tricuspid valve, the criteria for the 1979). The surgical considerations arising from
formation of a conduction path system are also the overriding phenomenon of the AV valve have
not fulfilled due to the malalignment between the found attention in a number of publications in
atrial and ventricular septum and the VSD that recent years (Aeba et al. 2000; Anderson 1997;
extends to the crux cordis (Anderson et al. 1981; Danielson et al. 1979a, b; Niinami et al. 1995;
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
405 12
Planche et al. 1982; Russo et al. 1988; Serraff et al. components to strengthen the sutures in the areas
1996; Tabry et al. 1979; Van Son et al. 1998a, b, c). where the suture line crosses the AV conduction
These show that the operative strategy always path. The sutures are placed at a distance from the
depends on the hemodynamically most relevant septum edge but in right ventricular or atrial tissue;
anomaly and that the real surgical problem with the straddling components remain in the left ventri-
the straddling AV valve lies in the need to close cle (Planche et al. 1982). Damage to the penetrating
the always coexisting VSD without impairing the AV bundle can be avoided by placing the sutures at
AV valve function, without producing AV block the left side of the septum edge (Serraff et al. 1996).
due to the positional anomalies of the specific Bob Anderson is of the opinion that surgical
muscles, and without the secondary chamber injury to the conduction path system can be
being too small after VSD closure. avoided by having exact knowledge of the anat-
Overriding of the AV valves, as discussed omy of the valves with the straddling phenome-
above, represents a whole spectrum of anomalies, non and the associated ventricular connection
with broad variability in position and size of the (Anderson 1997). However, in many patients a
VSD and in the location and course of the specific high incidence of postoperative complete AV
conduction structures and the ventricular geome- block has been observed (Pacifico et al. 1979;
try. For these reasons it appears not to be useful to Reddy and McElhinney 1997; Tabry et al. 1979).
describe the surgical procedure in detail and, so to Different surgical techniques in patients with
speak, «suture by suture» for an arbitrarily selected straddling of the tricuspid and mitral valve who
straddling situation, which would probably not be underwent biventricular correction have been
feasible with a different variant on the spectrum of described.
anomalies. Instead we describe in the following
the principle options for a surgical procedure, tak- 12.5.4.1 Transection of Tendinous Fibers
ing into account the different types of anomaly. When straddling affects only individual chordae
The straddling phenomenon remains a great that play no part in the closure mechanism of the AV
challenge for the cardiologist and pediatric car- valve, these can be transected without risk. The VSD
diac surgeon (Delius et al. 1996; Kiraly et al. 2007; closure can then be performed in the usual manner.
Tabry et al. 1979). In patients with well-developed The possibility of proceeding in this way is definitely
ventricles, an attempt should always be made to given when the crossing chordae in slight or moder-
perform biventricular correction rather than uni- ate straddling (type A or B) insert at an isolated pap-
ventricular correction; in doing so it is important illary muscle in the contralateral ventricle.
to avoid traumatic AV block (Anderson 1997).
Biventricular correction in valves with straddling 12.5.4.2 litting of the VSD Patch
S
phenomena carries the potential risk of causing and Division of the VSD Patch
dysfunction of the mitral and tricuspid valve, Suture Line
since dividing the chambers or placing the suture In rare cases of type A and type B straddling, the
line for VSD closure can often mechanically dis- crossing tendinous fibers could be brought
tort or injure the tensor apparatus of the AV valves through a slit in the VSD patch. To avoid a resid-
directly below the VSD. In addition, displacement ual defect, the slit is sutured after VSD closure
of the suture line to the left or right in patents with (Aeba et al. 2000; Pacifico et al. 1979). This tech-
the straddling phenomenon may lead to obstruc- nique should only be applied when the crossing
tion of the left or right ventricular outflow tract chordae are not an important part of the mecha-
(Delius et al. 1996; Serraff et al. 1996). Variable nism of valve closure since the valve mobility
valve morphology means that the valves in would be reduced (Aeba et al. 2000; Danielson
patients with functionally single ventricle are sus- et al. 1979b; Tabry et al. 1979; . Fig. 12.83).
ceptible to developing insufficiency in the long-
term course (Kiraly et al. 2007). 12.5.4.3 Papillary Muscle
A further problem is the high risk of developing Re-implantation
total AV block (Anderson 1997; Anderson and Ho With slight or moderate straddling (type A or B),
1998; Serraff et al. 1996). To avoid this, Planche and if the crossing chordae insert at an isolated papil-
colleagues (1982) developed a technique that uses lary muscle in the contralateral ventricle and this
the leaflet tissue of the overriding tricuspid valve papillary muscle has no connections to the other
406 V. Alexi-Meskhishvili et al.
a b
..Fig. 12.83 a, b Illustration looking into the right ventricle in a patient with transposition of the great arteries and
ventricular septal defect. a At the papillary muscle of the mitral valve with straddling phenomenon, the suture line has
been interrupted. b After completion of the VSD patch insertion, the straddling papillary muscles of the subvalvular
apparatus of the mitral valve come to rest between the ventricular septum and the VSD patch. The cranial part of the
VSD patch has been flipped over, allowing a free view of the aortic valve (Following Aeba et al. 2000)
AV valve, this muscle can be transected at its base 12.5.4.5 yocardial Conal Flap
M
and later be reimplanted in the septum of the cor- Methods
12 responding ventricle during VSD closure (Niinami Trimming of a conal myocardial flap to be used to
et al. 1995; Reddy and McElhinney 1997; Van Son correct abnormal attachment of the tricuspid
et al. 1998a, b, c). Whether this method can pro- valve to the outflow tract septum and its fixation
duce undisturbed valve function remains ques- to the intracardiac tunnel in a patient with double
tionable. Technically the procedure can only be outlet ventricle are shown in . Fig. 12.85 (Niinami
performed when reimplantation is possible in the et al. 1995; Serraff et al. 1996).
ventricle through which the operation is per-
formed, usually only for a tricuspid valve in typical 12.5.4.6 Retraction of the Chordae or
trans-right-atrial VSD patch closure. the Papillary Muscle
. Fig. 12.85 illustrates retraction of the chordae or
12.5.4.4 Pseudoseptation the papillary muscle of a tricuspid or mitral valve with
The patch selected to close the VSD is clearly straddling into the left ventricle through the native
larger and is placed in the primary chamber aortic valve (. Fig. 12.85) or the pulmonary valve
around the base points of the crossing chordae so (. Fig. 12.86) in a patient with dextrotransposition of
that the entire subvalvar apparatus of the former the great vessels (Serraff et al. 1996).
straddling valve lies within the newly created ven-
tricular cavum—a procedure that is particularly 12.5.4.7 AV-Valve Replacement
to be considered with type A straddling. This is When an overriding or straddling AV valve is pri-
technically relatively simple when the chords marily incompetent, which is extremely rare, or
cross into the ventricle through which the s urgeon when valve-preserving measures are not possible
works, mainly with straddling of the left AV valve in type C straddling, valve replacement must be
into the right-position ventricle. However, it is in considered. However, the well-known disadvan-
principle also possible with straddling of the right tages of valve replacement in children give rise to
AV valve into the left-position ventricle (Pacifico serious concerns (Tabry et al. 1979). If a malalign-
et al. 1979; . Fig. 12.84). ment VSD (straddling tricuspid valve) is present,
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
407 12
Cuspis septalis
Coronary sinus
..Fig. 12.84 a–e Pseudoseptation of a perimembranous malalignment (juxtacrux) ventricular septal defect in
an overriding and straddling tricuspid valve in a patient with situs solitus and normal atrioventricular connections.
a Access through an oblique incision in the right atrium. b The chordae of the septal tricuspid leaflet cross the ventricu-
lar septal defect to insert at papillary muscles of the left ventricle. The tricuspid valve anulus overrides by 10–20 %.
c Closure of the ventricular septal defect is performed transatrially. To preserve the tricuspid valve integrity, the patch is
placed on the left side of the septum in such a manner that the whole subvalvar apparatus remains to the right of the
Dacron patch. d, e Completed closure of the ventricular septal defect (Modified from Pacifico et al. 1979)
408 V. Alexi-Meskhishvili et al.
kFontan operation
Patients with a giant papillary muscle and anoma-
lous insertion of the tricuspid valve cannot be
treated by biventricular correction, since this
..Fig. 12.85 Surgical technique for trimming a myocar-
would cause obstruction of the left ventricular
dial flap to the correct size to operate on a tricuspid valve outflow tract and AV valve dysfunction (Aeba
with anomalous attachment to the septum of the outflow et al. 2000). Curtain-like tricuspid valve chordae
tract and fixation to the intracardiac tunnel in a case of with anomalous origin remain a contraindication
double outlet ventricle (After Serraff et al. 1996) to biventricular correction in patients with double
a b
12
..Fig. 12.86 a Surgical technique for the intraoperative management of a tricuspid valve with straddling with transposi-
tion of the great arteries. The papillary muscle chordae are pulled toward the right ventricle using a hook that is entered
through the native aortic valve. The ventricular septal defect is closed with access either through the native aortic valve or
through the pulmonary artery in the normal fashion. Occasionally it is necessary to complete the procedure through the
right atrium. b Surgical technique for the intraoperative handling of a straddling mitral valve with transposition of the great
arteries. The chordae of the papillary muscles are pulled with a hook in the direction of the left ventricle through the pulmo-
nary artery. The ventricular septal defect is then closed through the native aortic valve or through a right ventriculotomy.
Sometimes the procedure must be completed through the right atrium (After Serraff et al. 1996)
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
409 12
outlet right ventricle, transposition of the great phenomenon. In four patients the switch to uni-
vessels, or severe right ventricular hypoplasia. ventricular correction was made. There were four
Occasionally anomalies of the tricuspid valve or deaths after biventricular correction (13.3 %), and
anomalies of the papillary muscle origin around six reoperations were necessary due to a subaortic
the rim of the VSD in patients with double outlet stenosis, AV valve insufficiency, and the need for
ventricle make the creation of a satisfactory intra- pacemaker implantation.
cardiac rerouting difficult or even impossible. Reddy and colleagues (1997) described five
As long as the general criteria for a modified patients with straddling of the AV valves in whom
Fontan operation are fulfilled, this could be the the technique of chordal translocation and rein-
method of choice for patients with type B or type C sertion was used during the biventricular correc-
straddling and clear hypoplasia of the secondary ven- tion. None of the patients died, but three
tricle. developed total AV block necessitating perma-
In such situation it is assumed that univentricu- nent pacemaker implantation.
lar correction in the form of an extracardiac or intra- For patients with type A straddling and with-
cardiac lateral tunnel as part of the Fontan operation out overriding, the results are clearly more favor-
is superior to biventricular correction (Anderson able (Aeba et al. 2000; Niinami et al. 1995).
and Ho 1998; Delius et al. 1996; Freedom and Van
Arsdell 1998; Hanley 1999; Marino 2002; Pacifico
et al. 1979; Russo et al. 1988; van Arsdell 2006). References
Therefore, exact preoperative diagnostic pro-
cedures are obligatory in valves with straddling Abbattista A, Marino B, Iorio F, Marcelleti C (1994) Com-
plete atrioventricular canal and total anomalous pul-
and all surgical options should be carefully con-
monary venous drainage: a rare association. J Thorac
sidered before the decision in favor of biventricu- Cardiovasc Surg 107:1536–1538
lar or univentricular correction is made. In Abbott M (1936) Atlas of congenital cardiac disease. Amer-
patients with slightly hypoplastic right ventricle, ican Heart Association, New York
the method of «one-and-a-half ventricle repair» Abdulla R, Blew G, Holterman M (2004) Cardiovascular
embryology. Pediatr Cardiol 25:191–200
may be applied (Hanley 1999; Serraff et al. 1996).
Aeba R, Katogi T, Takeuchi S, Kawada S (2000) Surgical
management of straddling mitral valve in biventricu-
kPalliation lar heart. Ann Thorac Surg 69:130–134
In infants and small children, especially when Agarwall V, Okonta KE, Abubakar, Gichuhi S (2011) Impact
preoperative examinations have detected type B of Warden’s procedure on the sinus rhythm: our expe-
rience. Heart Lung Circ 20:718–721
or type C straddling, one of the «classical» pallia-
Aharon A, Laks H, Drinkwater D et al. (1994) Early and late
tive operations (pulmonary artery banding, sys- results of mitral valve repair in children. J Thorac Car-
temic–pulmonary arterial shunt) can be diovasc Surg 107:1262–1271
considered to optimize pulmonary perfusion. Al Zaghal J, Anderson R (1998) The nature of the superior
sinus venosus defect. Clin Anat 11:349–352
kHeart transplantation Alday L, Juaneda E, Spillmann A, Ruiz E (1994) Early and
late results of balloon dilatation for congenital mitral
Heart transplantation should be reserved for stenosis. Cardiol Young 4:122–125
patients with very severe myocardial dysfunction. Aleksi-Meskhishvili V (1977) Congenital stenosis of pulmo-
For this reason it is never primarily indicated in nary veins. Grudnaja Khirurgia 1:102–104
cases of overriding or straddling AV valves. Alexi-Meskishvili V, Hetzer R (2001) Surgical intervention
for anomalous origin of the left coronary artery from
the pulmonary artery (letter to editor). J Thorac Car-
diovasc Surg 122:635–636
Alexi-Meskishvili V, Konstantinov I (2003) Surgery of atrial
12.5.5 Results septal defect: from the firs experiments to clinical
practice. Ann Thorac Surg 76:322–327
There are only few reports of midterm and long- Alexi-Meskishvili V, Hetzer R, Weng Y et al. (1994) Anoma-
term results of surgical treatment of straddling lous origin of the left coronary artery from the pulmo-
nary artery: early results with direct aortic
valves; moreover, the groups of patients reported reimplantation. J Thorac Cardiovasc Surg 108:354–362
on are very small. Alexi-Meskishvili V, Weng Y, Uhlemann F, Lange PE, Hetzer R
Serraff and colleagues (1996) reported their (1995) Prolonged open sternotomy and delayed sternal
experience with 34 patients with double outlet closure after pediatric open heart surgery: experience
right ventricle and AV valves with the straddling with 113 cases. Ann Thorac Surg 59:379–383
410 V. Alexi-Meskhishvili et al.
Alexi-Meskishvili V, Ishino K, Dähnert I et al. (1996) Correc- Anderson R, Ho S, Becker A et al. (1983) The surgical anat-
tion of complete atrioventricular septal defects with omy of conduction tissues. Thorax 38:408–420
the double-patch technique and cleft closure. Ann Anderson RH, Zuberbuhler JR, Penkoske PA, Neches WH
Thorac Surg 62:519–524 (1985) On clefts, commissures, and things. J Thorac
Alexi-Meskishvili V, Hetzer R, Dahnert I, Weng Y, Lange P Cardiovasc Surg 90:605–610
(1997) Results of left atrioventricular valve reconstruc- Anderson R, Ettedgui J, Devine W (1995) Sinus venosus
tion after previous correction of atrioventricular septal defect. Am Heart J 129:1229–1232
defects. Eur J Cardiothorac Surg 12:460–465 Ando M, Fraser C (2001) Prosthetic mitral valve replace-
Alexi-Meskishvili V, Ovroutski S, Dähnert I, Fisher T (2000) ment after atrioventricular septal defect repair: a tech-
Correction of cor triatriatum in a Jehovah’s witness nique for small children. Ann Thorac Surg 72:907–909
infant. Eur J Cardiothorac Surg 18:724–726 Ando M, Takahashi Y (2011) Long-term functional analy-
Alexiou C, Galogavrou M, Chen Q et al. (2001) Mitral valve sis of the atrioventricular valve in patients undergo-
replacement with mechanical prosthesis in children: ing single ventricle palliation. Ann Thorac Surg
improved operative risk and survival. Eur J Cardiotho- 92:1767–1773
rac Surg 20:105–113 Ando M, Takahashi Y, Kikuchi T (2004) Total anomalous pul-
Alghamadi AA, Yanagawa B, Singh SK, Horton A, Al-Radi monary venous connection with dysmorphic pulmo-
OO, Caldarone CA (2011) Balancing stenosis and nary vein: a risk for postoperative pulmonary venous
regurgitation during mitral valve surgery in pediatric obstruction. Interact Cardiovasc Thorac Surg 3:557–561
patients. Ann Thorac Surg 92:680–684 Apitz C, Kaulitz R, Ziemer G, Hofbeck M (2009) Severe left
Alphonso N, Norgaard M, Newcomb A, d’Udekem Y, Brizard ventricular hypoplasia in patients with unbalanced
C, Cochrane A (2005) Cor triatriatum: presentation, incomplete atrioventricular septal defect and pulmo-
diagnosis and long-term surgical results. Ann Thorac nary hypertension: feasibility of biventricular repair.
Surg 80:1666–1171 Pediatr Cardiol 30:70–73
Alsoufi B, Manlhiot C, Al-Ahmadi M, McCrindle BW, Kal- Aramendi J, Rodriguez M, Luis T, Voces R (2006) No patch
loghlian A, Siblini G, Al-Halees Z (2011) Outcomes and technique for complete atrioventricular canal repair.
associated risk factors for mitral valve replacement in Interact Cardiovasc Thorac Surg 5:349–352
children. Eur J Cardiothorac Surg 40:543–551 Aramendi JI, Rey E, Hamzeh G, Crespo A, Luis M, Voces R
Amodeo A, Di Donato M, Corno A et al. (1990) Systemic (2011) Partial anomalous pulmonary venous connection
atrioventricular conduit for extracardiac bypass of to the superior vena cava. Ann Thorac Surg 91:64–66
hypoplastic atrioventricular valve. Eur J Cardiothorac Arizmendi A, Pineda L, Jimenez C et al. (2004) The clinical
Surg 4:601–604 profile of Ebstein’s malformation as seen from the fetus
Anagnostopoulos P, Alphonso N, Nölke L et al. (2007) Neo- to the adult in 52 patients. Cardiol Young 14:55–63
12 natal mitral and tricuspid valve repair for in utero pap-
illary muscle rupture. Ann Thorac Surg 83:1458–1462
Arstein A (1927) Eine seltene Misbildung der Trikuspi-
dalklappe (Ebstenische Krankheit). Virch Arch Pathol
Anderson R (1995) Ebstein’s malformation. Cardiovasc Anat 266:247–254
Pathol 4:225–226 Attenhofer JC, Connolly H, Dearani J, Edwards W, Danielson
Anderson R (1996) The surgical treatment of Ebstein’s mal- G (2007) Ebstein’s anomaly. Circulation 115:277–285
formation. Cardiol Young 6:1–3 Augustin N, Schmidt-Habelmann P (1997) Results after
Anderson R (1997) Surgical repair of straddling and over- surgical repair of Ebstein’s anomaly. Ann Thorac Surg
riding tricuspid valve. Cardiol Young 7:122–128 63:1650–1656
Anderson R, Becker A (1980) Cardiac anatomy. Churchill Azakie A, Lavrsen MJ, Johnson NC, Sapru A (2011) Early
Livingstone, Edinburgh outcomes of primary sutureless repair of the pulmo-
Anderson R, Ho S (1989) The surgical anatomy of atrioven- nary veins. Ann Thorac Surg 92:666–672
tricular septal defect. In: Grupi G, Parenzan L, Ander- Backer C, Stewart R, Mavroudis C (2007) Overview: history,
son R (eds) Perspectives in pediatric cardiology. anatomy, timing, and results of complete atrioventric-
Futura, Mount Kisko, pp 81–85 ular canal. Semin Thorac Cardiovasc Surg Pediatr Card
Anderson R, Ho S (1998) Which hearts are unsuitable for Surg Ann 10:3–10
biventricular connection? Ann Thorac Surg 66:121–126 Bailey C, Takeuchi Y, Williams W, Trusler G, Mustard W
Anderson K, Lie J (1979) The right ventricular myocardium (1976) Surgical management of congenital cardiovas-
in Ebstein’s anomaly. Mayo Clin Proc 54:181–184 cular anomalies with use of profound hypothermia
Anderson R, Shirali G (2009) Sequential segmental analy- and circulatory arrest: analysis of 180 consecutive
sis. Ann Pediatr Cardiol 2:24–35 cases. J Thorac Cardiovasc Surg 71:485
Anderson K, Ho S, Anderson R (1979a) Location and vascu- Baird CW, Myers PO, Marx G, del Nido PJ (2012) Mitral valve
lar supply of sinus node in human heart. Br Heart J operations at a high-volume pediatric heart center:
41:28–32 evolving techniques and improved survival with
Anderson K, Zuberbuhler, Anderson R, Becker A, Lie J mitral valve repair versus replacement. Ann Pediatr
(1979b) Morphologic spectrum of Ebstein’s anomaly Cardiol 5:13–20
of the heart. A review. Mayo Clin Proc 54:174–180 Banerjee A, Kohl T, Silverman N (1995) Echocardiographic
Anderson RH, Milo S, Ho SY, Wilkinson JL, Becker AE (1981) evaluation of congenital mitral valve anomalies in
The anatomy of straddling atrioventricular valves. In: children. Am J Cardiol 76:1284–1291
Becker AE, Losekoot G, Marcelleti C, Anderson RH (eds) Banka P, Bacha E, Powell AJ, Benavidez OJ, Luis M, Voces R
Paediatric cardiology, vol 3. Churchill Livingstone, (2011) Outcomes of inferior sinus venosus defect
Edinburgh, pp 1122–1134 repair. J Thorac Cardiovasc Surg 142:517–522
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
411 12
Bano-Rodriges P, Van Praagh S, Trowitzsch E, Van Praagh R Bharati S, Lev M (1973) The spectrum of common atrioven-
(1988) Double-orifice mitral valve. A study of 27 post- tricular orifice (canal). Am Heart J 86:553–561
mortem cases with developmental, diagnostic and sur- Binet JP, Bouchard F, Langlos J (1972) Unilateral congenital
gical considerations. Am J Cardiol 61:151–160 stenosis of the pulmonary veins. A very rare cause of
Baño-Rodrigo A, Van Praagh S, Trowitzsch E, Van Praagh R pulmonary hypertension. J Thorac Cardiovasc Surg
(1988) Double-orifice mitral valve: a study of 27 post- 63:397–402
mortem cases with developmental, diagnostic and Bingler MA, Darst JR, Fagan TE (2012) Cryo-balloon angio-
surgical considerations. Am J Cardiol 61:152–160 plasty for pulmonary venous stenosis. Pediatr Cardiol
Barbero-Marcial M, Riso A, De Albuquerque A, Atik E, 33:109–114
Jatene A (1993) Left ventricular apical approach for Black M, Freedom R (1998) Minimally invasive repair of
surgical treatment of congenital mitral stenosis. J Tho- atrial septal defects. Ann Thorac Surg 65:1065–1066
rac Cardiovasc Surg 106:105–110 Bleiziffer S, Schreiber C, Burgkart R (2004) The influence of
Barbero-Marcial M, Tanamati C, Jatene M, Atik E, Jatene A right anterolateral thoracotomy in prepubescent
(1998) Transxiphoid approach without median ster- female patients on late breast development and on
notomy for the repair of atrial septal defects. Ann Tho- the incidence of scoliosis. J Thorac Cardiovasc Surg
rac Surg 65:771–774 127:1474–1480
Barnard C, Schrire V (1963) Surgical correction of Ebstein’s Bohm J, Bittigau K, Köhler F, Baumann G, Konertz W (1997)
malformation with prosthetic tricuspid valve. Surgery Surgical removal of atrial septal defect occlusion sys-
54:302–308 tem-devices. Eur J Cardiothorac Surg 12:869–872
Barnard J, Speake D (2004) In open heart surgery is there Bokeria L, Alexi-Meskhishvili V, Chernishov V (1989) One-
is a role for the use of carbon dioxide field flooding stage correction of congenital heart disease and
techniques to reduce the level of postoperative complex rhythm disorders. Eur J Cardiothorac Surg
gaseous emboli? Interact Cardiovasc Thorac Surg
3:85–86
3:599–602 Bolling S, Iannettoni M, Dick M 2nd, Rosenthal A, Bove EL
Barratt-Boyes B (1973) Complete correction of cardiovas- (1990) Shone’s anomaly: operative results and late
cular malformations in the first two years of life using outcome. Ann Thorac Surg 49:887–893
profound hypothermia. Livingstone, Edinburg Boon P, Hazekamp M, Hoohenkerk G et al. (2007) Artificial
Bauer M, Alexi-Meskishvili V, Hetzer R (2000a) Less invasive chordae for pediatric mitral and tricuspid valve repair.
correction of atrial septal defects with transthoracic Eur J Cardiothorac Surg 32:143–158
cannulation. Ann Thorac Surg 69:981–982 Borst M (H: [sic]) (1905) Ein Cor triatriatum. Zentralbl Allg
Bauer M, Alexi-Meskishvilli V, Nakic Z, Redzepagic S, Bauer Pathol 16:812
UYW, Hetzer R (2000b) The correction of congenital Boston M, Dearani J, O’Leary P, Driscoll D, Danielson G
heart defects with less invasive approaches. Thorac (2006) Tricuspid valve repair for Ebstein’s anomaly in
Cardiovasc Surg 48:67–71 young children: a 30-year experience. Ann Thorac Surg
Becker A (1995) Ebstein’s malformation—what’s in a 81:690–696
name? Cardiovasc Pathol 4:25–28 Boston US, Goldberg SP, Ward KE, Overholt ED, Spentzas
Becker A, Anderson R (1982) Atrioventricular septal T, Chin TK, Knott. Kraig CJ (2011) Complete repair of
defects: what’s in a name? J Thorac Cardiovasc Surg Ebstein anomaly in neonates and young infants: a
83:461–469 16-year follow-up. J Thorac Cardiovasc Surg
Becker A, Ho S, Caruso G, Milo S, Anderson R (1980) Strad- 141:1163–1169
dling right atrioventricular valves in atrioventricular Böttcher W, Hübler M (2006) Der Beginn der chirurgischen
discordance. Circulation 61:1133–1141 Terapie der Mitralklappenstenose. Z Herz Thorax
Beierlein W, Becker V, Yates R et al. (2007) Long-term fol- Gefäßchir 20:203–216
low–up after mitral valve replacement in childhood: Brauner R, Laks H, Drinkwater D, Scholl F, McCaffery S
poor event-free survival in the young child. Eur J Car- (1997) Multiple left heart obstructions (Shone’s anom-
diothorac Surg 31:860–865 aly) with mitral valve involvement: long-term out-
Bender HJ, Hammon JJ, Hubbard S, Muirhead J, Graham T come. Ann Thorac Surg 64:721–729
(1982) Repair of atrioventricular canal malformation Brown J, Hanish S (2003) Cor triatriatum sinister, atresia of
in the first year of life. J Thorac Cardiovasc Surg the common pulmonary vein, pulmonary vein steno-
84:515–522 sis, and cor triatriatum dexter. Mosby, Philadelphia
Benvenuto R, Lewis FG (1959) Anastomosis between the Brown J, Ruzmetov M, Minnich D et al. (2003) Surgical
superior vena cava and the right atrium: a new tech- management of scimitar syndrome: an alternative
nique and a new application. Surgery 45:173–180 approach. J Thorac Cardiovasc Surg 125:238–245
Berdat P, Pfamatter J, Genyk I, Carrel T (2001) Modified Brown J, Ruzmetov M, Vijai P et al. (2005) Operative results
repair of mixed total anomalous pulmonary venous and outcomes in children with Shone’s anomaly. Ann
drainage. Ann Thorac Surg 71:723–725 Thorac Surg 79:1358–1365
Berger F, Ewert P, Bjornstad P (1999) Transcatheter closure Brown J, Ruzmetov M, Rodefeld M, Turrentine M (2006)
as standard treatment for interatrial defects, experi- Mitral valve replacement with Ross II technique: initial
ence in 200 patients treated with Amplatzer septal experience. Ann Thorac Surg 81:502–508
occluder. Cardiol Young 9:468–473 Brown JW, Fiore AC, Ruzmetov M, Eltayeb O, Rodefeld MD,
Berguis J, Kirklin J, Edwards J, Titus J (1964) The surgical Turrentine MW (2012) Evolution of mitral valve
anatomy of isolated congenital mitral insufficiency. J replacement in children: a 40-year experience. Ann
Thorac Cardiovasc Surg 47:791–798 Thorac Surg 93:626–633
412 V. Alexi-Meskhishvili et al.
Bukharin V, Aleksi-Meskhishvili V (1969) Congenital aneu- Castenada A, Jonas R, Mayer J, Hanley F (1994) Cardiac sur-
rysmal distension of the left auricle of the left atrium. gery of the neonate and infant. WB Saunders Co., Phil-
Grudn Khir 11:93–95 adelphia
Burke R, Mark J, Collins JLHC (1992) Improved surgical Cavigelli-Brunner A, Bauersfeld U, Prètre R, Kretschmar O,
approach to left atrial appendage aneurysm. J Cardiac Oxenius A, Buechel ERV (2012) Outcome of biventricu-
Surg 7:104–107 lar repair in infants with multiple left heart obstructive
Burr L, Krayenbuhl C, Sutoon M, Paneth M (1977) The mitral lesions. Pediatr Cardiol 33:506–512
plication suture: a new technique of mitral valve Celermajer D, Cullen S, Sullivan I, Spiegelhalter D, Wyse R,
repair. J Thorac Cardiovasc Surg 73:589–595 Deanfield J (1992a) Outcome in neonates with
Burroughs JT, Kirklin JW (1956) Complete surgical correc- Ebstein’s anomaly. J Am Coll Cardiol 19:1041–1046
tion of total anomalous pulmonary venous connec- Celermajer D, Dood S, Greenwald S, Wyse R, Deanfield J
tion: report of three cases. Mayo Clin Proc 31:182 (1992b) Morbid anatomy in neonates with Ebstein’s
Buz S, Alexi-Meskishvili V, Villavicencio-Lorini F et al. (2008) anomaly of the tricuspid valve: pathophysiologic and
Analysis of arrhythmias after correction of partial clinical implications. J Am Coll Cardiol 19:1049–1053
anomalous pulmonary venous connection. Ann Tho- Celermajer D, Bull C, Till J (1994) Ebstein’s anomaly: presen-
rac Surg 87:580–583 tation and outcome from fetus to adult. J Am Coll Car-
Cabin H, Roberts W (1981) Ebstein’s anomaly of the tricus- diol 23:170–176
pid valve and prolapse of the mitral valve. Am Heart J Chaudhuri K, Storey E, Lee GA, Bailey M, Chan J, Rosenfeldt
101:177–180 FL, Pick A, Negri J, Gooi J, Zimmet A, Esmore D, Merry
Caldarone C, Behrendt D (2000) Total anomalous venous C, Rowland M, Lin E, Marasco SF (2012) Carbon dioxide
drainage associated with complex cardiac anatomy. insufflation in open-chamber cardiac surgery: a dou-
Pediatr Cardiac Surg Ann Semin Thorac Cardiovasc ble-blind, randomized clinical trial of neurocognitive
Surg 3:142–152 effects. J Thorac Cardiovasc Surg 144:646–653
Caldarone C, Najm H, Kadletz MJFS, Freedom R (1998) Chauvaud S, Mihaileanu S, Gaer J, Carpentier A (1996) Sur-
Relentless pulmonary vein stenosis after repair of total gical treatment of Ebstein’s malformation—the Hospi-
anomalous pulmonary venous drainage. Ann Thorac tal Broussais experience. Cardiol Young 6:4–11
Surg 66:1514–1520 Chauvaud S, Milhaileanu S, Gaer JAR, Carpentier AC
Caldarone C, Raghuveer C, Hills C et al. (2001) Long-term (1997a) Surgical treatment of congenital mitral valvar
survival after mitral valve replacement in children insufficiency. Cardiol Young 7:5–14
aged less than 5 years. A multi-institutional study. Cir- Chauvaud S, Milhaileanu S, Gaer JAR, Carpentier AC
culation 104(Suppl 1):143–147 (1997b) Surgical treatment of congenital mitral valvar
Capoua E, Laks H, Drinkwater D, Pearl J, Milgalter E (1992) stenosis. Cardiol Young 7:15–21
12 Management of left atrioventricular valve in the repair
of complete atrioventricular septal defect. J Thorac
Chauvaud S, Fuzellier J, Houel R (1998a) Reconstructive
surgery in congenital mitral valve insufficiency (Car-
Cardiovasc Surg 104:196–204 pentier’s techniques). J Thorac Cardiovasc Surg
Carell T, Wattenbach M, Triller J, Schüpbach P (1995) Total 115:84–93
anomalous pulmonary venous connection to the por- Chauvaud S, Fuzellier J, Berrebi A et al. (1998b) Bi-directional
tal and splenic vein associated with unilateral hypo- cavopulmonary shunt associated with ventriculo and
plasia of pulmonary veins. Eur J Cardiothorac Surg valvuloplasty in Ebstein’s anomaly: benefits in high risk
10:1141–1143 patients. Eur J Cardiothorac Surg 13:514–519
Carpentier A (1978) Surgical anatomy and management of Chauvaud S, Brancaccio G, Carpentier A (2001) Cardiac
the mitral component of atrioventricular canal defects. arrhythmia in patients undergoing surgical repair of
Churchill Livingstone, London Ebstein’s anomaly. Ann Thorac Surg 71:1547–1552
Carpentier A (1983) Congenital malformations of mitral Chauvaud S, Berrebi A, Mousseaux E, Hernigou A, Carpen-
valve. In: Stark J, de Leval M (eds) Surgery for congenital tier A (2003) Ebstein’s anomaly: repair based on func-
heart defects. Gerune & Stratton, London, pp 457–482 tional analysis. Eur J Cardiothoracic Surg 23:525–531
Carpentier A (1987) Discussion of Pn-Chih, Chen-Chun: Chen J, Mosca R, Altmann K, Printz B, Targoff K, Quaege-
The surgical treatment of common atrioventicular beur J (2004) Early and medium-term results for repair
canal. Ann Thorac Surg 43:150–154 of Ebstein anomaly. J Thorac Cardiovasc Surg 127:990–
Carpentier A, Brizard C (2006) Congenital malformations of 999
the mitral valve in children. In: Stark J, de Leval M, Cheng T, Wang X, Zheng L (1994) Three-dimensional trans-
Tsang V (eds) Surgery for congenital heart defects. esophageal echocardiography in the diagnosis of
Wiley, London, pp 573–590 mitral valve prolapse. Am Heart J 128:1218–1224
Carpentier A, Branchini B, Cour J et al. (1976) Congenital Cherian K, Rao S (1994) Surgical correction of the unroofed
malformations of the mitral valve in children. J Thorac coronary sinus syndrome. Indian Heart J 46:91–95
Cardiovasc Surg 72:854–866 Cheung Y, Lun K, Chau A, Chiu C (2005) Fate of the unli-
Carpentier A, Chauvaud S, Mace L et al. (1988) A new gated vertical vein after repair of supracardiac anoma-
reconstructive operation for Ebstein’s anomaly of the lous pulmonary venous connection. J Paediatr Child
tricuspid valve. J Thorac Cardiovasc Surg 96:92–101 Health 41:361–364
Cassano G (1964) Congenital anular stenosis of the left Church WS (1867/1868) Congenital malformation of the
atrioventricular canal. So-called supravalvular mitral heart: abnormal septum in left auricle. Trans Pathol
stenosis. Am J Cardiol 13:708–713 Soc Lond 19:188
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
413 12
Cobanoglu A, Menashe V (1993) Total anomalous pulmo- Darling R, Rothney W, Craig J (1957) Total pulmonary
nary venous connection in neonates and young infants: venous drainage into the right side of the heart: report
repair in the current era. Ann Thorac Surg 55:43–48 of 17 autopsied cases not associated with other major
Cohen MS, Rychik J (1999) The small left ventricle: how cardiovascular anomalies. Lab Invest 6:44–55
small for biventricular repair? Sem Thoracic Cardiovasc Davachi F, Moller J, Edwards J (1971) Diseases of mitral
Surg Pediatr Card Surg Ann 2:189–202 valve in infancy. An anatomic analysis of 55 cases. Cir-
Cohen G, Stevenson J (2007) Intraoperative echocardiog- culation 43:565–579
raphy for atrioventricular canal: decision-making for David T (2004) Artificial chordae. Semin Thorac Cardiovasc
surgeons. Semin Thorac Cardiovasc Surg Pediatr Card Surg 16:161–168
Surg Ann 10:47–50 David I, Castaneda A, Van Praagh R (1982) Potentially para-
Cooley D (1960) Result of surgical treatment of atrial septal chute mitral valve in common atrioventricular canal.
defects: particular consideration of low defects includ- Pathological anatomy and surgical importance. J Tho-
ing ostium primum and atrioventricular canal. Am J rac Cardiovasc Surg 84:178–186
Cardiol 6:605–615 Davidson D, Bando K, Helfaer M, Cameron D (1995)
Cooley D (1998) Minimally invasive valve surgery versus the Ebstein’s anomaly. In: Nichols D, Cameron D, Greeley W
conventional approach. Ann Thorac Surg 66:1101–1105 et al. (eds) Critical heart disease in infants and chil-
Cooley D, Murphy M (1990) Cor triatriatum and anomalous dren. Mosby, New York, pp 907–921
pulmonary venous return. Tex Heart Inst J 17:118–121 Davies RR, Pasquali SK, Jacobs ML, Jacobs JJ, Wallace AS,
Cooper G (1836) Case of malformation of the thoracic vis- Pizzaro C (2013) Current spectrum of surgical proce-
cera. Lond Med Gaz 18:600–601 dures for Ebstein’s malformation: an analysis of the
Cope J, Banks D, McDaniel N, Schokey K, Nolan S, Kron I society of thoracic surgeons congenital heart surgery
(1997) Is vertical vein ligation necessary in repair of database. Ann Thorac Surg 96:1703–1710
total anomalous pulmonary venous connection? Ann Dearani J (2007) Discussion on the paper of da Silva et al.
Thorac Surg 64:23–28 The cone reconstruction of the tricuspid valve in
Corno A (2005) Borderline left ventricle. Eur J Cardiothorac Ebstein’s anomaly. The operation: early and mid-term
Surg 27:67–73 results. J Thorac Cardiovasc Surg 133:215–223. J Tho-
Correa-Villasenor A, Ferencz C, Neill C (1994) Ebstein’s mal- rac Cardiovasc Surg 113:222–223
formation of the tricuspid valve: genetic and environ- Dearani J, Danielson G (2000) Congenital heart surgery
mental factors. The Baltimore-Washington Infant nomenclature and database project: Ebstein’s anom-
Study Group. Teratology 50:137–147 aly and tricuspid valve disease. Ann Thorac Surg
Crawford F (2007) Atrioventricular canal: single-patch 69(Suppl):S106–S117
technique. Semin Thorac Cardiovasc Surg Pediatr Card Dearani J, Bacha E, da Silva JP (2008) Cone reconstruction
Surg Ann 10:11–20 of the tricuspid valve for Ebstein’s anomaly: anatomic
Croti U, Braile D, de Godoy M, De Marchi C (2003) A rare repair. Op Tech Thorac Cardiovasc Surg 13:109–125
association of total anomalous pulmonary venous Dearani JA, Said SM M.D., O’Leary PW, Burkhart HM, Barnes
connection and cor triatriatum. Rev Bras Cir Cardio- RD, Cetta F (2013a) Anatomic repair of Ebstein’s mal-
vasc 18:273–276 formation: lessons learned with cone reconstruction.
da Silva JP, da Fonseca L (2012) Ebstein’s anomaly of the Ann Thorac Surg 95:220–228
tricuspid valve: the cone repair. Semin Thorac Cardio- Dearani JA, Said SM M.D., Burkhart HM, Pike RB, O’Leary
vasc Surg Pediatr Card Surg Ann 15:38–45 PW, Cetta F (2013b) Strategies for tricuspid re-repair in
da Silva JP, Baumgratz JF, da Fonseca L et al. (2004) Anoma- Ebstein malformation using the cone technique. Ann
lia de Ebstein Resultados com a reconstrução cônica Thorac Surg 96:202–210
da valva tricúspide. Arq Bras Cardiol 82:212–216 Del Nido P, Bichell D (1998) Minimal-access surgery for
da Silva J, Baumgratz J, de Fonsca J et al. (2007) The cone congenital heart defects. Semin Thorac Cardiovasc
reconstruction of the tricuspid valve in Ebstein’s Surg Pediatr Cardiac Surg Ann 1:75–80
anomaly. The operation: early and mid-term results. J DeLeon S, Ilbawi M, Wilson WJ et al. (1991) Surgical options
Thorac Cardiovasc Surg 133:215–223 in subaortic stenosis associated with endocardial
da Silva JP, da Fonseca L, Moreira LFP et al. (2011) Cone cushion defects. Ann Thorac Surg 52:1076–1083
reconstruction in Ebstein’s anomaly repair: early and DeLeval M, Ritter D, McGoon D, Danielson G (1975) Anom-
long-term results. Arq Bras Cardiol 97:199–208 alous systemic venous connection. Mayo Clin Proc
Danielson G, Maloney J, Devloo R (1979a) Surgical repair of 50:599–610
Ebstein’s anomaly. Mayo Clin Proc 54:185–192 Delius R, Rademecker M, de Leval M, Elliott M, Stark J
Danielson G, Tabry I, Ritter DG, Fulton RE (1979b) Surgical (1996) Is a high-risk biventricular repair always prefer-
repair of criss-cross heart with straddling atrioventric- able to conversion to a single ventricular repair? J Tho-
ular valve. J Thorac Cardiovasc Surg 77:847–851 rac Cardiovasc Surg 112:1561–1569
Danielson G, Tarby I, Fulton RE, Hagler JH, Ritter DG (1979c) Delmo Walter E, Ewert P, Hetzer R (2008) Biventricular
Successful repair of straddling atrioventricular valve repair in children with complete atrioventricular sep-
by technique used for septation of univentricular tal defect and a small left ventricle. Eur J Cardiothorac
heart. Ann Thorac Surg 28:554–560 Surg 33:40–47
Danielson G, Driscoll D, Mair D, Warnes C, Oliver WJ (1992) Delmo Walter EM, Siniawski H, Ovroutski S, Hetzer R (2010)
Operative treatment of Ebstein’s anomaly. J Thorac Mitral valve growth after posterior anular stabilization
Cardiovasc Surg 104:1195–1205 with untreated autologous pericardial strip in children
414 V. Alexi-Meskhishvili et al.
with mitral valve insufficiency. Ann Thorac Surg Foale R, Gibson T, Guyer D, Gillam L, King MAEW (1982) Con-
90:1577–15 genital aneurysms of the left atrium: recognition by cross-
Derra E, Bayer O, Grosse-Brockhoff G (1955) Der Vorhofsep- sectional echocardiography. Circulation 66:1065–1069
tumdefect und sein operativer Verschluß unter Sicht Fontain-Dommer R, Wiles H, Shuler C, Bradley S, Shirali G
des Auges in Unterkühlungsanästhesie. Dtsch Med (2000) Recognition of left atrial aneurysm by fetal
Wochenschr 36:1277–1281 echocardiography. Circulation 102:2282–2283
Derra E, Loogen F, Satter P (1965) Anomalien der unteren Fortuna R, Ashburn D, De Oliveira N et al. (2004) Atrioven-
Hohlvene. Dtsch Med Wschr 16:689–695 tricular septal defect: effect of bridging leaflet division
Devaney E, Chang A, Ohye R, Bove E (2006a) Management on early valve function. Ann Thorac Surg 77:895–902
of congenital and acquired pulmonary vein stenosis. Fraisse A, Massih T, Vouhé P et al. (2002) Management and
Ann Thorac Surg 81:992–996 outcome of patients with abnormal ventriculo-arterial
Devaney E, Ohye R, Bove E (2006b) Pulmonary venous ste- connections and mitral valve cleft. Ann Thorac Surg
nosis following repair of total anomalous pulmonary 74:7l86–7l791
venous connection. Semin Thorac Cardiovasc Surg Freedom R, Smallhorn J (1988) A consideration of the mor-
Pediatr Cardiac Surg Ann 9:51–55 phology and diagnostic features of certain congenital
DiBardino DJ, McKenzie ED, Heinle JS, Su JT, Fraser CD Jr abnormalities of the mitral valve. In: Dunn J (ed) Cardiac
(2004) The Warden procedure for partially anomalous valve disease in children. Elsevier, New York, pp 185–212
pulmonary venous connection to the superior caval Freedom R, Van Arsdell G (1998) Biventricular hearts not
vein. Cardiol Young 14:64–67 amenable to biventricular repair. Ann Thorac Surg
Dimond E, Kittle C, Voth D (1960) Extreme hypertrophy of 66:641–643
the left atrial appendage: the case of the giant dog ear. Freedom R, Bini R, Dische R, Rowe R (1978) The straddling
Am J Cardiol 5:122–125 mitral valve: morphological observations and clinical
Dodge-Khatami A, Mavroudis C, Backer C (2002) Anoma- implications. Eur J Cardiol 8:27–50
lous origin of the left coronary artery from the pulmo- Friesen C, Chen R, Howlett J, Ross D (2004) Posterior anular
nary artery: collective review of surgical therapy. Ann plication: tricuspid valve repair in Ebstein’s anomaly.
Thorac Surg 74:946–955 Ann Thorac Surg 77:2167–2171
Drake E, Lynch J (1950) Bronchiectasis associated with Friesen C, Zurakowski D, Thiagarajan RFJM, Del Nido P,
anomaly of the right pulmonary vein and right dia- Mayer J, Jonas R (2005) Total anomalous pulmonary
phragm. Report of a case. J Thorac Surg 19:433–437 venous connection: an analysis of current management
Drinkwater JD, Laks H (1997) Unbalanced atrioventricular strategies in a single institution. Ann Thorac Surg
septal defect. Semin Thorac Cardiovasc Surg 1:21–25 79:596–606
Dubost C, Blondeau BP (1959) Canal atrio-ventriculaire et Frigiolla A, Badia T, Pomè G et al. (2005) Pulmonary autograft
12 ostium primum. J Chir 78:241–258
Dunn J (1998) Cardiac valve disease in children. Elsevier,
for mitral valve replacement in infant: the Ross-Kabbani
operation. Ann Thorac Surg 79:2150–2151
New York Gandhi S, Bromberg B, Rodefeld M et al. (1996) Lateral tun-
Ebstein W (1866) Über eine sehr seltenen Fall von Insuffi- nel suture line variation reduces atrial flutter after Fon-
cienz der Valvula tricuspidalis, bedingt durch eine tan operation. Ann Thorac Surg 61:1299–1309
angeborene hochgradige Missbildung derselben. Gandy K, Hanley F (2006) Management of systemic venous
Arch von Riecert Bois-Reymond 2:238–234 anomalies in the pediatric cardiovascular surgical
Edward W (1993) Embryology and pathological features of patient. Semin Thorac Cardiovasc Surg Pediatr Card
Ebstein’s anomaly. Progr Pediatr Cardiol 2:5–15 Surg Ann 9:63–74
Edwards J (1953) Congenital malformations of the heart Gatzoulis M, An R, Sommerville J, Shore D (1996) Should
and great vessels. A. Malformations of the atrial septal atrial septal defects in adults be closed? Ann Thorac
complex. Charles C Thomas, Springfield Surg 61:657–659
Edwards J (1960) Congenital stenosis of pulmonary veins. Gatzoulis M, Freeman M, Siu S, Webb G, Harris L (1999)
Pathology and developmental considerations. Lab Atrial arrhythmia after surgical closure of atrial septal
Invest 9:46–66 defect in adults. N Engl J Med 340:839–846
Ehrenhaft J, Theilen E, Lawrence M (1958) The surgical Gaynor J (2006) Management of sinus venosus defects.
treatment of partial and total anomalous pulmonary Semin Thorac Cardiovasc Surg Pediatr Cardiac Surg
venous connections. Ann Surg 148:249–259 Ann 9:35–39
Eroglu S, Yildirir A, Simsek V (2004) Cor triatriatum dexter, Gaynor J, Collins M, Rychik J, Gaughan J, Spray T (1999)
atrial septal defect and Ebstein’s anomaly in an adult Long-term outcome in infants with single ventricle
given a diagnosis by transthoracic and transesopha- and total anomalous pulmonary venous drainage. J
geal echocardiography: a case report. J Am Soc Echo- Thorac Cardiovasc Surg 117:506–514
cardiogr 17:780–782 Gaynor J, Weinberg P, Spray T (2000) Congenital heart sur-
Ewert P, Berger F (2003) Transcatheter atrial septal defect gery nomenclature and database project: systemic
closure without fluoroscopy. In: Rao P, Kern M (eds) venous anomalies. Ann Thorac Surg 69:S70–S76
Catheter based devices for the treatment of non-coro- Genton E, Blount SJ (1967) The spectrum of Ebstein’s
nary cardiovascular disease in adults and children. Lip- anomaly. Am Heart J 73:395–425
pincot Williams & Wilkins, Philadelphia, pp 85–90 Gerbode F, Kerth W, Osborn J, Selzer A (1962) Correction of
Fisher T (1902) Two cases of congenital disease of the left mitral insufficiency by open operation. Ann Surg
side of the heart. BMJ 1:639 155:846–854
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
415 12
Gerlis L, Sweeney A, Ho S (1993) Mitral valve anomalies Hebe J (2000) Ebstein’s anomaly in adults: arrhythmias:
associated with Ebstein’s malformation of the tricus- diagnosis and therapeutic approach. Thorac Cardio-
pid valve. Am J Cardiovasc Pathol 4:294–301 vasc Surg 48:214–219
Giamberti A, Marino B, diCarlo D et al. (1996) Partial atrio- Herlong J, Jaggers J, Ungerleider R (2000) Congenital heart
ventricular canal with congestive heart failure in the surgery nomenclature and database project: pulmo-
first year of life: surgical options. Ann Thorac Surg nary venous anomalies. Ann Thorac Surg 69:S56–S59
62:151–154 Hetzer R, Delmo Walter EM (2013) No ring at all in mitral
Gibbon JH (1954) Application of a mechanical heart and lung valve repair: indications, techniques and long-term
apparatus to cardiac surgery. Minn Med 37:171–177 outcome. Eur J Cardiothorac Surg 1:1–11
Giroud J, Jacobs J (2006) Fontan’s operation: evolution Hetzer R, Drews T (1999) Mitral valve replacement. In:
from a procedure to process. Cardiol Young 16(Suppl Franco K, Verrier E (eds) Advanced therapy in cardiac
1):67–61 surgery. Decker, Hamilton, pp 232–244
Giuliani E, Fuster V, Brandenburg R, Mair D (1979) The clini- Hetzer R, Pasic M (2004) Ebstein’s anomaly. In: Gardner T,
cal features and natural history of Ebstein’s anomaly of Spray T (eds) Operative cardiac surgery. Arnold, Lon-
the tricuspid valve. Mayo Clin Proc 54:163–173 don, pp 651–671
Gold J, Afifi H, Ko W, Horner N, Hahn R (1996) Congenital Hetzer R, Nagdyman N, Ewert P et al. (1998) A modified
giant aneurysm of the left atrial appendage: diagnosis repair technique for tricuspid incompetence in Ebstein’s
and management. J Cardiac Surg 11:147–150 anomaly. J Thorac Cardiovasc Surg 115:857–868
Gross RE (1952) Surgical closure of defects of the interau- Hetzer R, Delmo Walter E, Hübler M, Alexi-Meskishvili V, Weng
ricular septum by use of an atrial well. N Engl J Med Y, Nagdyman N, Berger F (2008) Modified surgical tech-
247:455–460 niques and long-term outcome of mitral valve recon-
Groves L (1967) Correction of anomalous pulmonary struction in 111 children. Ann Thorac Surg 86:604–613
venous drainage into the superior vena cava. Ann Tho- Ho SY (2003) Cardiac morphology and nomenclature. In:
rac Surg 4:301–307 Gatzoulis M, Webb G, Daubeney P (eds) Diagnosis and
Gudjonsson U, Brown J (2006) Scimitar syndrome. Semin management of adult congenital heart disease.
Thorac Cardiovasc Surg Pediatr Cardiac Surg Annu Churchill Livingstone, Philadelphia, pp 7–18
9:56–52 Ho S, Anderson R (1988) The morphology of the mitral
Güeven M, Carvalho J, Ho S, Shinebourne E (2003) Sequen- valve with regards to congenital malformations. In:
tial segmental analysis of the heart: a malformation Dunn J (ed) Cardiac valve disease in children. Elsevier,
screening technique. Artemis 4:21–23 New York, pp 163–184
Gundry S, Shattuck O, Razzouk A, Del Rio M, Sardari F, Bai- Hoohenkerk GJF, Bruggemans EF, Koolbergen DR, Rijlaars-
ley L (1998) Facile minimally invasive cardiac surgery dam MEB, Hazekamp MG (2012) Long-term results of
via ministernotomy. Ann Thorac Surg 65:1100–1104 reoperation for left atrioventricular valve regurgita-
Günter T, Mazzitelli D, Schreiber C et al. (2000) Mitral-valve tion after correction of atrioventricular septal defects.
replacement in children under 6 years of age. Eur J Ann Thorac Surg 93:849–855
Cardiothorac Surg 17:426–430 Huang QH, Wang ND, Yang DY, Doty DB (1993) Aneurysm
Gustafson R, Warden HGFM (1995) Partial anomalous pul- of the left atrium. Cardiovasc Surg 3:303–304
monary venous connection to the superior vena cava. Huang T, Lee C, Lin C, Tseng C, Hsieh K (2002) Use of Inoue
Ann Thorac Surg 60:S1614–S1617 balloon dilatation method for treatment of cor triatria-
Hammon J, Bender H (1983) Major anomalies of thoracic tum stenosis in a child. Catheter Cardiovasc Interv
and systemic veins. In: Sabiston DJ, Spenser F (eds) 57:252–256
Gibbon’s surgery of the chest. Saunders, Philadelphia, Huddleston C, Exil V, Canter C, Mendeloff E (1999) Scimitar
p 1035 syndrome presenting in infancy. Ann Thorac Surg
Hammon JJ, Bender JJ, Graham T (1980) Total anomalous 67:154–159
pulmonary venous connection in infancy. Ten years’ Huddleston C, Balzer D, Mendeloff E (2001) Repair of
experience including studies of postoperative ven- anomalous left main coronary artery arising from the
tricular function. J Thorac Cardiovasc Surg 89:544–551 pulmonary artery in infants: long-term impact on the
Han L, Kang S, Park S, Ettedgui J, Nehes W (1995) Long- mitral valve. Ann Thorac Surg 71:1985–1988
term left atrioventricular valvular function following Huhta J, Edwards W, Danielson GK, Feldt RH (1982a) Abnor-
surgical repair of atrioventricular septal defects. Car- malities of tricuspid valve in complete transposition of
diol Young 1995:230–237 the great arteries with ventricular septal defect. J Tho-
Hanley F (1999) Editorial: the one and half ventricle rac Cardiovasc Surg 83:569–576
repair—we can do it, but we should we do it? J Thorac Huhta J, Smallhorn JF, de Leval MR, Macartney FJ (1982b)
Cardiovasc Surg 117:659–661 Tricuspid valve abnormalities in DORV with subpul-
Hardy K, May I, Webster C, Komball K (1964) Ebstein’s monic VSD. J Thorac Cardiovasc Surg 84:154–155
anomaly: a functional concept and successful defini- Hunter S, Lillehei C (1958) Ebstein’s malformation of the
tive repair. J Thorac Cardiovasc Surg 48:927–940 tricuspid valve with suggestions of a new form of sur-
Hashmi A, Bu-Sulaiman R, McCrindle B (1998) Manage- gical therapy. Dis Chest 33:297
ment and outcomes of right atrial isomerism: a 26-year Husain S, Brown J (2007) When reconstruction fails or is
experience. Am J Cardiol 31:1120–1126 not feasible: valve replacement options in the pediat-
Hawkins J, Clark E, Doty D (1983) Total anomalous pulmo- ric population. Semin Thorac Cardiovasc Surg Pediatr
nary venous connection. Ann Thorac Surg 36:548–560 Card Surg Ann 10:117–124
416 V. Alexi-Meskhishvili et al.
Ilbawi M, Idriss F, DeLeon S et al. (1983) Unusual mitral with a pulmonary autograft: the Ross-Kabbani opera-
abnormalities complicating surgical repair of endo- tion. Ann Thorac Surg 72:947–950
cardial cushion defects. J Thorac Cardiovasc Surg Kadoba K, Jonas R, Mayer J, Castaneda A (1999) Mitral
85:697–704 valve replacement in the first year of life. J Thorac Car-
Ilbawi M, Cua C, DeLeon S (1990) Repair of complete atrio- diovasc Surg 100:762–768
ventricular septal defect with tetralogy of Fallot. Ann Kanter K (2006) Surgical repair of total anomalous pulmo-
Thorac Surg 50:407–412 nary venous connection. Semin Thorac Cardiovasc
Imai Y, Seo K, Terada M et al. (1999) Valvular repair for atrio- Surg Pediatr Cardiac Surg Ann 9:40–44
ventricular regurgitation in complex anomalies in Kanter KR, Forbess JM, Kirshbom PM (2005) Redo mitral valve
modified Fontan procedure with reference to a single replacement in children. Ann Thorac Surg 80:642–646
ventricle associated with common atrioventricular Kanter KR, Kogon BE, Kirshbom PM (2011) Supra-anular
valve. Semin Thorac Cardiovasc Surg Pediatr Card Surg mitral valve replacement in children. Ann Thorac Surg
Ann 2:5–19 92:2221–2229
Imoto Y, Kado H, Asou T (1998) Mixed type of total anoma- Kawahira Y, Yagihara T, Uemura H, Ishizaka T, Yoshizumi K,
lous pulmonary venous connection. Ann Thorac Surg Kitamura S (1999) Use of expanded polytetrafluoreth-
66:1394–1397 ylene sutures as artificial tendinous cords in children
Ishino K, Alexi-Meskishvili V, Hetzer R (1997) Myocardial with congenital mitral regurgitation. Eur J Cardiotho-
recovery trough ECMO after repair of total anomalous rac Surg 15:289–293
pulmonary venous connection: the importance of left Kawashima Y, Ueda T, Naito Y, Morikawa E, Manabe H
heart unloading. Eur J Cardiothorac Surg 11:585–587 (1971) Stenosis of pulmonary veins: report of a patient
Ito M, Kikuchi S, Hachiro Y, Abe T (2001) Congenital pulmo- corrected surgically. Ann Thorac Surg 12:196–202
nary vein stenosis associated with cor triatriatum. Ann Kay JH, Magidson O, Meihaus JE, Lewis R, Egerton WS,
Thorac Surg 71:722–723 Zubiate P, Lefevre T (1961) Mitral insufficiency and
Jacobs M, Mayer J (2000) Congenital heart surgery nomen- mitral stenosis: surgical treatment using the heart-
clature and database project: single ventricle. Ann lung-machine. Calif Med 95:382–385
Thorac Surg 69:S197–S204 Kaza KK, Colan SD, Jaggers J, Lu M, Atz AM, Sleeper LA,
Jacobs J, Burke R, Quintessenza J, Mavroudis C (2000a) McCrindle BW, Lambert LM, Margossian R, Lacro RV,
Congenital heart surgery nomenclature and database Richmond ME, Natarjan S, Minich L (2011) Surgical
project: atrioventricular canal defect. Ann Thorac Surg interventions for atrioventricular septal defect sub-
69:S36–S43 types: the pediatric heart network experience. Ann
Jacobs J, Quintessenza J, Burke R, Mavroudis C (2000b) Con- Thorac Surg 92:1468–1475
genital heart surgery nomenclature and database proj- Keith J, Rowe R, Vlad P (1967) Heart disease in infants, chil-
12 ect: atrial septal defect. Ann Thorac Surg 69:S18–S24
James T (1963) The connecting pathways between the
dren and adolescents. MacMilian, New York
Khairy P, Porier N, Mercier L-A (2007) Univentricular heart.
sinus node and A-V node and between the right and Circulation 115:800–812
the left atrium in the human heart. Am Heart J Khositseth A, Danielson G, Dearani J, Munger T, Porter C
66:498–508 (2004) Supraventricular tachyarrhythmias in Ebstein
Jiang Z, Mei J, Ding F, Bao C, Zhu J, Tang M, Ma N, Huang J, anomaly: management and outcome. J Thorac Cardio-
Shen S (2013) The early and mid-term results of mitral vasc Surg 128:826–833
valve repair for mitral regurgitation in children. Surg Kiaii B, Doll N, Kuehl M, Mohr F (2004) Minimal invasive
Today (in press) endoscopic resection of a giant left atrial appendage.
Jorgensen C, Ferlic F, Varco R, Lillehei W, Eliot R (1967) Cor Ann Thorac Surg 77:1437–1438
triatriatum review of the surgical aspects with a fol- Kiraly L, Hubay M, Cook A, Ho S, Anderson R (2007) Morpho-
low-up report on the first patient successfully treated logic features of the unilateral but biventricular atrioven-
with surgery. Circulation 36:101–107 tricular connection. J Thorac Cardiovasc Surg 133:299–234
Julian O, Lopez-Belio M, Dye W, Javid H, Grove W (1957) Kirklin J (1973) Surgical treatment of total anomalous pul-
The median sternal incision in intracardiac surgery monary venous return. In: Barratt-Boyes B, Neutze J,
with extracorporeal circulation; a general evaluation Harris E (eds) Heart disease in infancy: diagnosis and
of its use in heart surgery. Surgery 42:753–761 surgical treatment. Churchill Livingstone, Edinburgh,
Jung E, Imai Y, Hoshino S, Nakata S, Shinoka T, Isomatus Y pp 89–96
(1995) Two cases of successful arterial switch opera- Kirklin J, Ellis FJ, Wood E (1956) Treatment of anomalous
tion for double outlet right ventricle associated with pulmonary venous connections in association with
straddling and cleft mitral valve. Nippon Kyobu Shik- interatrial communications. Surgery 39:389–398
kan Gakkai Zasshi 43:229–235 Kiziltan HT, Theodoro DA, Warnes CA, O’Leary PW, Ander-
Kabbani S, Ross D, Jamil H et al. (1999) Mitral valve replace- son BJ, Danielson GK (1998) Late results of biopros-
ment with a pulmonary autograft: initial experience. J thetic tricuspid valve replacement in Ebstein’s
Heart Valve Dis 8:359–367 anomaly. Ann Thorac Surg 66:1539–1545
Kabbani S, Jamil H, Hammoud A (2000) The mitral pulmo- Knott-Craig C, Goldberg S (2007) Management of neonatal
nary autograft: a follow–up cautionary report. J Heart Ebstein’s anomaly. Semin Thorac Cardiovasc Surg
Valve Dis 9:801–804 Pediatr Card Surg Ann 10:112–116
Kabbani S, Jamil H, Hammoud A, Nabhani F, Hariri R, Sab- Knott-Craig C, Overholt E, Ward K, Razook J (2000) Neona-
bagh N (2001) Technique for replacing mitral valve tal repair of Ebstein’s anomaly: indications, surgical
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
417 12
technique, and medium-term follow–up. Ann Thorac Lewis FG, Taufic M (1952) Closure of atrial septal defects
Surg 69:1505–1510 with the aid of hypothermia; experimental accom-
Knott-Craig CJ, Overholt ED, Ward KE, Ringewald JM, Baker plishments with the report of one successful case. Sur-
SS, Razook JD (2002) Repair of Ebstein’s anomaly in the gery 33:52–59
symptomatic neonate: an evolution of technique with Lewis FJ, Varco RL, Taufic M, Niazi SA (1956) Direct vision
7-year follow-up. Ann Thorac Surg 73:1786–1793 repair of triatrial heart and total anomalous pulmonary
Knott-Craig CJ, Ward KE, Overholt ED, Kirklin JK (2007) Repair venous drainage. Surg Gynec Obstet 102:713–720
of neonates and young infants with Ebstein’s anomaly Liang J, Liu W, Liu J (1996) Unroofed coronary sinus syn-
and related disorders. Ann Thorac Surg 84:592–593 drome. Zhonghua; Wai Ke Za Zhi 34:546–548
Kumar A, Fyler D, Miettinen O, Nadas A (1971) Ebstein’s Liberthson RR, Paul MH, Muster AJ, Arcilla RA, Eckner FAO,
anomaly. Clinical profile and natural history. Am J Car- Lev M (1971) Straddling and displaced atrioventricular
diol 28:84–96 orifices and valves with primitive ventricle. Circulation
Kumar N, Kumar M, Duran C (1995) A revised terminology 43:213–227
for recording surgical findings of the mitral valve. J Lillehei C, Cohen M, Warden H, Varco R (1955) The direct-
Heart Valve Dis 4:76–77 vision intracardiac correction of congenital anomalies
Kumar R, Dharmapuram A, Rao I et al. (2001) The fate of the by controlled cross-circulation: results in thirty-two
unligated vertical vein after surgical correction of total patients with ventricular septal defects, tetralogy of
anomalous pulmonary venous connection in early Fallot, and atrioventricular communis defects. Surgery
infancy. J Thorac Cardiovasc Surg 122:615–617 38:11–29
Lacour-Gayet F (2006) Surgery for pulmonary venous Lillehei C, Kalke R, Carlson R (1967) Evolution of corrective
obstruction after repair of total anomalous pulmonary surgery for Ebstein’s anomaly. Circulation 15–16(Suppl):
venous return. Semin Thorac Cardiovasc Surg Pediatr I–111–I–118
Cardiac Surg Ann 9:45–50 Litwin S, Tweddell J, Mitchell M, Mussato K (2007) The dou-
Lacour-Gayet F, Rey C, Planche C (1996) Description of ble patch repair for complete atrioventricular commu-
sutureless surgical procedure using the pericardium in nis. Semin Thorac Cardiovasc Surg Pediatr Card Surg
situ. Arch Mal Coeur Vaiss 89:633–636 Ann 10:21–27
Lacour-Gayet F, Zoghbi J, Serraf AE et al. (1999) Surgical Liu J, Qiu L, Zhu Z, Chen H, Hong H (2011) Cone reconstruc-
management of progressive pulmonary venous tion of the tricuspid valve in: Ebstein anomaly with or
obstruction after repair of total anomalous pulmo- without one and a half ventricle repair. J Thorac Car-
nary venous connection. J Thorac Cardiovasc Surg diovasc Surg 141:1178–1183
117:679–687 Löffler E (1949) Unusual malformation of left atrium: pul-
Laks H (1994) Invited commentary to: Teske DW, Davis T, monary sinus. Arch Pathol 48:371–376
Allen HG. Cavopulmonary anastomotic aneurysm: a Lorier G, Kalil R, Barcellos C et al. (2001) Valve repair in chil-
complication in pulsatile pulmonary arteries. Ann Tho- dren with congenital mitral lesions: late clinical results.
rac Surg 57: 1661–1664 Pediatr Cardiol 22:44–52
Laks H, Hellebrand W, Kleinman C, Talner N (1980) Left Lucas R, Woolfrey B, Anderson R, Lester R, Edwards J (1961)
atrial-left ventricular conduit for relief of congenital Atresia of the common pulmonary vein. Pediatrics
mitral stenosis in infancy. J Thorac Cardiovasc Surg 29:729–739
80:782–787 Lynch M, Ryan N, Williams G et al. (1962) Preoperative diag-
Lam T, Reemtsen B, Starnes V, Wells W (2007) A novel nosis and surgical correction of supravalvular mitral
approach to the surgical correction of scimitar syn- stenosis and ventricular septal defect. Circulation
drome. J Thorac Cardiovasc Surg 133:573–574 25:854–861
Lambert E (1951) Single ventricle with rudimentary outlet Mahle W, Gaynor J, Spray T (2001) Atrioventricular valve
chamber. Bull Johns Hopkins Hosp 88:231–238 replacement in patients with a single ventricle. Ann
Lamberti J, Kriett J (2007) Mitral/systemic atrioventricular Thorac Surg 72:182–186
valve repair in congenital heart disease. Semin Thorac Mair D, McGoon D (1977) Surgical correction of atrioven-
Cardiovasc Surg Pediatr Card Surg Ann 10:94–100 tricular canal defect during first year of life. Am J Car-
Lamberti J, Mainwaring R, George L, Oury J (1993) Man- diol 40:66–69
agement of systemic atrioventricular regurgitation in Maloney J, Marable S, Mulder D (1962) The surgical treat-
infant and children. J Card Surg 8:612–621 ment of common atrioventricular canal. J Thorac Car-
Latson L, Prieto L (2007) Congenital and acquired pulmo- diovasc Surg 43:84–96
nary vein stenosis. Circulation 115:103–108 Mannheimer E, Bengsstone E, Winberg J (1952) Pure con-
Lee C, Danielson G, Schaff H, Puga F, Mair D (1985) Surgical genital mitral stenosis due to fibro-elastosis. Cardiolo-
treatment of double-orifice mitral valve in atrioven- gia 21:574–582
tricular septal defects. Experience in 25 patients. J Tho- Manning P (2007) Partial atrioventricular canal: pitfalls in
rac Cardiovasc Surg 90:700–705 technique. Semin Thorac Cardiovasc Surg Pediatr Card
Lee J, Lee C, Chang J, Bae E, Noh C (2002) Modified extra- Surg Ann 10:42–46
cardiac Fontan operation in a patient with separate Mantovani V, Sala A, Relland J (2005) Mitral regurgitation:
hepatic venous drainage. Ann Thorac Surg 73:992–993 overview of current surgical techniques and future
Lev M (1958) The architecture of conduction system in developments. Expert Rev Cardiovasc Ther 3:271–284
congenital heart disease. I. Common atrioventricular Marin-Garcia J, Tandon R, Lucas RJ, Edwards J (1975) Cor
orifice. AMA Arch Pathol 65:174–191 triatriatum: study of 20 cases. Am J Cardiol 35:59–66
418 V. Alexi-Meskhishvili et al.
Marino B (2002) Outcomes after Fontan procedure. Curr Mitchell M, Maharajh G, Bielfeld M, DeGroff C, Clarke D
Opin Pediatr 14:620–626 (2001) Emergency pulmonary autograft mitral valve
Mas J-L, Arquizan C, Lamy C (2001) Recurrent cerebrovas- replacement in a child. Ann Thorac Surg 72:251–253
cular events associated with patent foramen ovale, Mitchell M, Litwin S, Tweddell J (2007) Complex atrioven-
and atrial septal aneurysm or both. N Engl J Med tricular canal. Semin Thorac Cardiovasc Surg Pediatr
345:1740–1746 Card Surg Ann 10:32–41
Masayaoshi I, Kikuchi S, Hachiro Y, Abe T (2001) Congenital Mitruka S, Lamberti J (2000) Congenital Heart Surgery
pulmonary vein stenosis associated with cor triatrium. Nomenclature and Database Project: mitral valve dis-
Ann Thorac Surg 71: 722–723 ease. Ann Thorac Surg 69:S132–S146
Mathews J, Pennigton W, Isobe J, Gaskin T, Dumas J, Khan Miyamura H, Nakagomi M, Eguchi S, Aizava Y (1990) Success-
D (1983) Paradoxical embolisation with Ebstein’s ful surgical treatment of incessant automatic atrial tachy-
anomaly. Arch Surg 118:1101 cardia with atrial isomerism. Ann Thorac Surg 50:476–478
Matsumoto T, Kado H, Masuda M et al. (1999) Clinical Moore P, Adatia I, Spevak P et al. (1994) Severe congenital
results of mitral valve repair by reconstruction artificial mitral stenosis in infants. Circulation 89:2099–2116
chordae tendineae in children. J Thorac Cardiovasc Morales J, Patel S, Jackson J, Duff J, Simpson J (2001) Left
Surg 118:94–98 atrial aneurysm. Ann Thorac Surg 71:719–722
Mavroudis C, Backer C, Stewart R, Heraty P (2005) The case Muller WJ (1951) The surgical treatment of transposition of
against minimally invasive cardiac surgery. Semin Thorac the pulmonary veins. Ann Surg 134:683–693
Cardiovasc Surg Pediatr Cardiac Surg Annu 8:193–197 Murakami T, Yagihara T, Ayamamoto F, Uemura H,
Mazzera E, Corno A, DiDonato R et al. (1989) Surgical Yamashita K, Ashizaka T (1998) Artificial chordae for
bypass of the systemic atrioventricular valve in chil- mitral valve reconstruction in children. Ann Thorac
dren by means of a valved conduit. J Thorac Cardio- Surg 65:1377–1380
vasc Surg 96:321–325 Murray G, Wilkinson F, MacKenzie R (1938) Reconstruction of
McElhiney D, Salvin J, Colan S et al. (2005) Improving out- the valves of the heart. Can Med Assoc J 38:317–319
comes in fetuses and neonates with congenital dis- Muster A, Bharati S, Aziz K, Idriss F, Paul M, Lev M (1979)
placement (Ebstein’s malformation) or dysplasia of Taussig-Bing anomaly with straddling mitral valve. J
tricuspid valve. Am J Cardiol 96:582–586 Thorac Cardiovasc Surg 77:832–842
McElhinney D, Sherwood M, Keane JPJDN, Almond C, Lock Najm H, Caldarone C, Coles J (1998) A sutureless technique for
J (2005) Current management of severe congenital the relief of pulmonary vein stenosis with the use of in the
mitral stenosis. Outcomes of transcatheter and surgi- situ pericardium. J Thorac Cardiovasc Surg 115:468–470
cal therapy in 108 infants and children. Circulation Nakano T, Kado H, Shiokawa Y, Fukae K (2002) Surgical
112:707–714 results of double-orifice left atrioventricular valve
12 McGoon D, DuShane J, Kirklin J (1959) The surgical treatment
of endocardial cushion defects. Surgery 46:185–196
associated with atrioventricular septal defects. Ann
Thorac Surg 73:69–75
McGrath L, Gonzales-Lavin G-L (1987) Actuarial survival, Nassar M, Fouilloux V, Macé L, Kreitmann B, Metras D (2012)
freedom from reoperation and other events after Transcaval correction of partial anomalous pulmonary
repair of atrioventricular septal defects. J Thorac Car- venous drainage into the superior vena cava. Ann Tho-
diovasc Surg 94:582–590 rac Surg 93:193–196
McGuinness J, Kindawi A, Tajri S, Walsh K, Nolke L, Wood A Nicholson I, Nunn G, Scholler G, Hawker R, Cooper S, Lau K
(2007) Surgical management of giant left atrial diver- (1999) Simplified single patch technique for the repair
ticulum. J Thorac Cardiovasc Surg 133:820–822 of atrioventricular septal defect. J Thorac Cardiovasc
Meadows J, Marshall A, Lock J, Scheurer M, Laussen P, Surg 118:642–647
Bacha E (2007) A hybrid approach to stabilization and Niinami H, Imai Y, Sawatari K, Hoshino S, Ishihara K, Aoki M
repair of obstructed total anomalous pulmonary (1995) Surgical management of tricuspid malinsertion
venous connection in a critically ill newborn infant. J in the Rastelli operation: conal flap method. Ann Tho-
Thorac Cardiovasc Surg 131:e2 rac Surg 59:1476–1480
Menahem S, Anderson R (2004) Cleft mitral valve in trans- Noren G, Raghib G, Moller J, Ampaltz K, Adams P, Edwards J
position with intact ventricular septum. Cardiol Young (1964) Anomalous origin of the left coronary artery from
14:550–552 the pulmonary trunk with special reference to the occur-
Mendelhof E, Spray T, Huddleston C, Bridges N, Canter C, rence of mitral insufficiency. Circulation 30:171–179
Mallory GJ (1995) Lung transplantation for congenital Nunn G (2007) Atrioventricular canal: modified single
pulmonary vein stenosis. Ann Thorac Surg 60:903–907 patch technique. Semin Thorac Cardiovasc Surg Pedi-
Michielon G, Di Carlo D, Branacaccio G et al. (2003) Anoma- atr Card Surg Ann 10:28–31
lous coronary artery origin from the pulmonary artery: Oda S, Nakano T, Tatewaki H, Hinokiyama K, Machida D,
correlation between surgical timing and left ventricu- Kado H (2013) A 17-year experience with mitral valve
lar function recovery. Ann Thorac Surg 76:581–588 repair with artificial chordae in infants and children.
Milo S, Ho S, MaCartney F et al. (1979) Straddling and over- Eur J Cardiothorac Surg 44:e40–e45
riding atrioventricular valves: morphology and classifi- Oelert H, Breckenridge I, Rosland G, Stark G (1973) Surgical
cation. Am J Cardiol 44:1122–1134 treatment of cor triatriatum in 4 1/2-month-old infant.
Minami K, Kado H, Sai S et al. (2005) Midterm results of Thorax 28:242–246
mitral valve repair with artificial chordae in children. J Oelert H, Bernsau U, Grosse H, Kalfelz H (1977) Diagnose und
Thorac Cardiovasc Surg 129:336–342 Operation eines Cor triatriatum mit Fehlmündung aller
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
419 12
Lungenvenen in die obere Hohlvene bei einem 4 1/2 Pirc B, Grersak B, Ruzic-Medvesek N (1996) Cor triatriatum
Monate alten Säugling. Thoraxchirurgie 25:101–104 sinistrum, aortic coarctation and bicuspid aortic steno-
Oglietti J, Cooley D, Izguierdo J et al. (1983) Cor triatriatum: oper- sis in an adult. Eur J Cardiothorac Surg 10:1139–1140
ative result in 25 patients. Ann Thorac Surg 35:415–420 Planche C, Fermont L, Bruniaux J, Sidi D, Kachaner J, Binet
Oh J, Holmes D, Hayes D, Porter C, Danielson G (1985) Car- JP (1982) Straddling de la valve tricuspide à travers
diac arrhythmias in patients with surgical repair of une communication interventriculaire isolée. A pro-
Ebstein’s anomaly. J Am Coll Cardiol 6:1351–1357 pos de 4 cas. Arch Mal Couer 8:905–914
Ohno H, Imai Y, Terada M, Hiramatsu T (1999) The long- Plunkett M, Schneider D, Shah J, Bash S, Bond L, Geiss D
term results of commissure plication for congenital (1998) Homograft replacement of mitral valve in chil-
mitral insufficiency. Ann Thorac Surg 68:537–541 dren. J Thorac Cardiovasc Surg 66:849–852
Okita Y, Miki S, Kusuhara K et al. (1988) Early and late results Pome G, Pelenghi S, Grassi M, Vignati G, Pellegrini A (2000)
of reconstructive operations for congenital mitral Congenital intrapericardial aneurysm of the left atrial
regurgitation in pediatric age group. J Thorac Cardio- appendage. Ann Thorac Surg 69:1569–1571
vasc Surg 96:294–298 Pomerantzeff P, Freyere H, Brandao C, Barreto A, Oliviera S
Oku H, Iemura L, Kitayama H, Saga T, Shirotani H (1994) (2002) Aneurysm of the left atrial appendage. Ann
Bivalvation with bridging for common atrioventricular Thorac Surg 73:1981–1983
valve regurgitation in right isomerism. Ann Thorac Pozzi M, Remig J, Flimmers R, Urban A (1991) Atrioventric-
Surg 57:1324–1346 ular septal defects. Analysis of short- and medium-
Olinger G (1995) Carbon dioxide displacement of left heart term results. J Thorac Cardiovasc Surg 101:138–142
chambers. J Thorac Cardiovasc Surg 109:187–188 Prifti E, Vanini VB, Frati M et al. (2002) Repair of congenital
Ostermeyer J, Körfer R, Frenzel H, Bircks W (1980) Strad- anomalies of the mitral valve: early and mid-term
dling atrioventricular valves in biventricular hearts. results. Ann Thorac Surg 73:614–621
Thorac Cardiovasc Surg 28:233–238 Pritisanac A, Schmidt M, Hannekum A, Gulbins H (2005) An
Pacifico A, Soto B, Bargeron LM Jr (1979) Surgical repair of image of mitral valve repair. Expert Rev Cardiovasc
straddling tricuspid valves. Circulation 60:655–664 Ther 3:1009–1016
Pacifico AD, Mandke NV, McGrath LB (1985) Repair of con- Quaegebeur J, Sreeram N (1991) Surgery for Ebstein’s
genital pulmonary venous stenosis with living autolo- anomaly: the clinical and echocardiographic evolution
gous atrial tissue. J Thorac Cardiovasc Surg 89:604–609 of new technique. J Am Coll Cardiol 17:722–728
Park SC, Nehes WH, Zuberbuhler JR, Siewers RD, Bahnson Quaegebeur J, Kirlin J, Pacifico A, Bargeron L (1979) Surgi-
HT (1974) Diagnosis and surgical management of cal experience of unroofed coronary sinus. Ann Thorac
bilateral pulmonary vein stenosis. J Thorac Cardiovasc Surg 27:418–425
Surg 67:755–761 Quarti A, Di Eusanio M, Pierri M, Di Eusanio G (2005) Left
Park J-J, Lee SC, Kim JB, Seo D-J, Song J-M, Yun S-C, Yuin T-J superior vena cava draining into the left atrium, asso-
(2011) Deterioration of mitral valve competence after ciated with partial anomalous pulmonary venous con-
the repair of atrial septal defect in adults. Ann Thorac nection: surgical correction. J Card Surg 20:353–355
Surg 92:162933 Quiñonez L, Dearani J, Puga F et al. (2007) Results of the
Patel SS, Burns TL, Kochilas L (2012) Early outcomes and 1.5-ventricle repair for Ebstein anomaly and the failing
prognostic factors for left atrioventricular valve reop- right ventricle. J Thorac Cardiovasc Surg 133:1303–1310
eration after primary atrioventricular septal defect Quiñonez LG, Breitbart R, Tworetsky W et al. (2013) Stented
repair. Pediatr Cardiol 33:129–140 bovine jugular vein graft (Melody valve) for surgical
Peacock T (1846) Malformation of the heart consisting in mitral valve replacement in infants and children. J Tho-
an imperfection of the auricular and ventricular septa. rac Cardiovasc Surg doi:10.1016/j.jtcvs.2013.10.059.
Trans Pathol Soc Lond 1:34 [Epub ahead of print]
Perier P, Clausnizer B (1995) Isolated cleft mitral valve: Raghib G, Ruttenberg H, Anderson R, Amplatz K, Adams P,
reconstruction techniques. Ann Thorac Surg 59:56–59 Edwards J (1965) Termination of left superior vena
Permut L, Mehta V (1997) Late results and reoperation after cava in left atrium, atrial septal defect, absence of cor-
repair of complete and partial atrioventricular canal onary sinus; a developmental complex. Circulation
defect. Semin Thorac Cardiovasc Surg Pediatr Card 31:906–918
Surg Ann 9:44–54 Raghuveer G, Caldarone C, Hills C, Atkins D, Belmont J,
Pflaumer A, Eicken A, Augustin N, Hess J (2004) Symptom- Moller J (2003) Predictors of prosthesis survival,
atic neonates with Ebstein’s anomaly. J Thorac Cardio- growth, and functional status following mechanical
vasc Surg 127:1208–1209 mitral valve replacement in children aged <5 years, a
Phoon C, Silverman N (1997) Conditions with right ven- multi-institutional study. Circulation 108(Suppl
tricular pressure and volume overload, an a small 1):174–179
hypoplastic left ventricle or simply a squashed ventri- Rastelli G, Kirklin J, Titus J (1966) Anatomic observations on
cle? J Am Coll Cardiol 30:1547–1553 complete form of persistent common atrioventricular
Photiadis J, Loebe M, Berger F et al. (1995) Erstmanifesta- canal with special reference to atrioventricular valves.
tion eines Mitralklappen-Cleft nach artellieller Switch- Mayo Clin Proc 41:296–308
Operation. Z Herz, Thorax, Gefäßchir 9:170–172 Rastelli GC, Ongley PA, Kirklin JW, McGoon DC (1968a) Sur-
Piccoli G, Wilkinson J, Macartney F, Gerlis L, Anderson R gical repair of complete form of persistent common
(1979) Morphology and classification of complete atrioventricular canal. L Thorac Cardiovasc Surg
atrioventricular defects. Br Heart J 42:633–639 55:299–308
420 V. Alexi-Meskhishvili et al.
Rastelli G, Ongley P, Titus JL (1968b) Ventricular septal Russo P, Danielson G, Puga F, McGoon D, Humes R (1988)
defect of atrioventricular canal type with straddling Modified Fontan procedure for biventricular hearts
right atrioventricular valve and mitral valve deformity. with complex forms of double-outlet right ventricle.
Circulation 37:816–825 Circulation 78(Suppl 3):III–20–III–25
Reddy V, McElhinney DFLH (1997) Correction of left supe- Saad E, Marrouche N, Saad C et al. (2003) Pulmonary vein
rior vena cava draining to the left atrium using extra- stenosis after catheter ablation of atrial fibrillation:
cardiac techniques. Ann Thorac Surg 63:1800–1802 emergence of new clinical diagnose. Ann Intern Med
Reddy V, Liddicoat J, McElhinney D, Brook M, van Son J, 138:634–638
Hanley F (1997) Biventricular repair of lesions with Sachweh J, Tiete A, Mühler E et al. (2007) Mechanical aortic
straddling tricuspid valves using technique of cordal and mitral valve replacement in infants and children. J
translocation and realignment. Cardiol Young 7: Thorac Cardiovasc Surg 55:156–152
147–152 Sade R, Freed M, Matthews E (1974) Stenosis of individual
Reemtsen B, Fagan B, Wells W, Starnes V (2006) Current sur- pulmonary veins. J Thorac Cardiovasc Surg 67:953–962
gical therapy for Ebstein’s anomaly in neonates. J Tho- Sadeghi A, Laks H, Pearl J (1997) Primum atrial septal
rac Cardiovasc Surg 132:1285–1290 defect. Semin Thorac Cardiovasc Surg Pediatr Card
Remenyi B, Webb R, Gentles T, Russell P, Finucane K, Lee M, Surg Ann 9:2–7
Wilson N (2012) improved long-term survival for rheu- Said SM, Bukhart HM, Dearani JA, Eidem B, Stensurd P, Phil-
matic mitral valve repair compared to replacement in the lips SD, Schaff HV (2011) Outcome of caval division
young. World J Pediatr Congenit Heart Surg 4:155–16 techniques for partial anomalous pulmonary venous
Revuelta J (1998) Invited commentary on article of Plun- connection to the superior vena cava. Ann Thorac
kett et al. Homograft replacement of mitral valve in Surg 92:980–985
children. J Thorac Cardiovasc Surg 66:849–852. J Tho- Sakamoto Y, Kurosawa H, Hashimoto H (1995) Surgical
rac Cardiovasc Surg 66:852 repair of unilateral pulmonary venous stenosis. Car-
Reye R (1951) Congenital stenosis of the pulmonary veins diol Young 5:184–186
in their extrapulmonary course. Med J Aust 1:801–802 Sallehuddin A, Bulbul Z, Otero F, Al Dhafiri K, Al-Halees Z
Ricci M, Elliot M, Cohen G (2003) Management of pulmo- (2004) Repair of atrioventricular valve regurgitation in
nary venous obstruction after correction of TAPVD. Eur the modified Fontan operation. Eur J Cardiothorac
J Cardiothorac Surg 24:28–36 Surg 26:54–59
Richardson J, Doty D, Siewers R, Zuberbuhler J (1981) Cor Sands A, Edis B, Cochrane A (2003) Giant left atrial aneu-
triatriatum (subdivided left atrium). J Thorac Cardio- rysm in a 15-month-old child. Circulation 108:
vasc Surg 81:232–238 2722–2723
Roberson DA, Silverman N (1989) Ebstein’s anomaly: echo- Sano SI, Ishino K, Kawada M et al. (2002) Total right ven-
12 cardiographic and clinical features in the fetus and
neonate. J Am Coll Cardiol 14:1300–1307
tricular exclusion procedure: an operation for isolated
congestive right ventricular failure. J Thorac Cardio-
Rocafort AG, Aroca Á, Polo L, Rey J, Vilagrá F (2013) Chim- vasc Surg 123:640–647
ney technique for mitral valve replacement in chil- Sarris G, Giannopoulos N, Tsoutsinos A et al. (2006) Results
dren. Ann Thorac Surg 96:1885–1887 of surgery for Ebstein anomaly: a multicenter study
Rodefeld M, Brown J, Heimansohn D et al. (1990) Cor tria- from the European Congenital Heart Surgeons Asso-
triatum: clinical presentation and surgical results in 12 ciation. J Thorac Cardiovasc Surg 132:50–57
patients. Ann Thorac Surg 50:562–568 Schaff H (1982) Discussion on paper of David et al. Poten-
Rogers H, Edwards J (1948) Incomplete division of the tially parachute mitral valve in common atrioventricu-
atrioventricular canal with patent intraatrial foramen lar canal. Pathological anatomy and surgical
primum (persistent common atrioventricular ostium). importance. J Thorac Cardiovasc Surg 84:178–176, 186
Report of five cases and review of the literature. Am Scheurer M, Hill E, Vasuki N et al. (2008) Survival after bidi-
Heart J 36:28 rectional cavopulmonary anastomosis: analysis of pre-
Rokitansky C (1875) Die Defekte der Scheidewände des operative risk factors. J Thorac Cardiovasc Surg
Herzens. Braumüller, Wien 134(82–89):89e1–89e2
Ross D, Nanton M, Gillis DDAM (1991) Atrioventricular Schreiber C (2013) Invited commentary on: Vida VL, Tessari
canal defects: results of repair in the current era. J Car- C, Fabozzo A, et al. (2013) The evolution of the right
diovasc Surg 6:367–372 anterolateral thoracotomy technique for correction of
Rowlatt U, Rimoldi H, Lev M (1963) The quantitative anat- atrial septal defects: cosmetic and functional results in
omy of normal child’s heart. Pediatr Clin N Am prepubescent patients. Ann Thorac Surg 95:242–248
10:499–588 Schreiber T, Feigenbaum H, Weyman A (1989) Effect of
Rubino M, Van Praagh S, Kadoba K, Pessotto R, Van Praagh atrial septal defect repair on left ventricular geometry
R (1995) Systemic and pulmonary venous connection and degree of mitral valve prolapse. Circulation
in visceral heterotaxy with asplenia: diagnostic and 61:888–896
surgical considerations based on seventy-two autop- Schumacker H, King H (1961) A modified procedure for
sied cases. J Thorac Cardiovasc Surg 110:641–650 complete repair of total anomalous pulmonary venous
Ruckman RN, Van Praagh R (1978) Anatomic types of con- drainage. Surg Gynec Obstet 112:763–765
genital mitral stenosis: report of 49 autopsy cases with Sealy W, Farmer J, Young WJ, Brown IJ (1969) Atrial dys-
consideration of diagnosis and surgical implications. rhythmia and atrial secundum defects. J Thorac Car-
Am J Cardiol 42:592–601 diovasc Surg 57:245–250
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
421 12
Seccombe JS (1999) Mitral valve repair: current techniques Spevak P, Bass J, Ben-Shacher G et al. (1990) Balloon angio-
and indications. In: Franco K, Verrier E (eds) Advanced plasty for congenital mitral stenosis. Am J Cardiol
therapy in cardiac surgery. Decker, Hamilton, pp 220–231 66:472–476
Sellers R, Levy M, Amplitz R (1964) Retrograde angiogra- Spray T (2001) Commentary. J Thorac Cardiovasc Surg 122:617
phy in acquired cardiac disease: technique, indica- Spray T (2004) Atrioventricular canal defects. In: Gardner T,
tions and interpretation in 700 cases. Am J Cardiol Spray T (eds) Operative cardiac surgery. Arnold, Lon-
14:437–447 don, pp 605–615
Semans J, Taussig H (1938) Congenital aneurysmal dilata- Spray T, Bridges N (1999) Surgical management of congeni-
tion of the left auricle. Bull Johns Hopkins Hosp tal and acquired pulmonary vein stenosis. Semin Thorac
63:404–414 Cardiovasc Surg Pediatr Cardiac Surg Ann 2:177–178
Serraf A, Bruniaux J, Lacour-Gayet F (1991) Obstructed Srinvasan V, Lewin A, Pieroni D, Levinsky L, Alicea J, Subra-
total anomalous pulmonary venous return. Toward manian S (1980) Supravalvular stenosing ring of the
neutralization of a major risk factor. J Thorac Cardio- left atrium: case report and review of the literature. Tex
vasc Surg 101:601–669 Heart J 7:149–158
Serraf A, Zoghbi J, Bell E et al. (2000) Congenital mitral ste- Starnes V, Pitlick P, Bernstein D, Griffin M, Choy M, Shum-
nosis with or without associated defects: an evolving way N (1991) Ebstein’s anomaly appearing in the neo-
surgical strategy. Circulation 102(19 Suppl 3):166–171 nate. A new surgical approach. J Thorac Cardiovasc
Serraff A, Nakamura T, Lacour-Gayet F (1996) Surgical Surg 101:1082–1087
approaches for double-outlet ventricle or transposi- Starr A, Hovaguimian H (1994) Surgical repair of subaortic
tion of the great arteries associated with straddling stenosis in atrioventricular canal defects. J Thorac Car-
atrioventricular valves. J Thorac Cardiovasc Surg diovasc Surg 108:373–376
111:527–535 Steen H, Merten C, Lehrk S, Lossnitzer D, Giannitsis E, Katus
Sethia B, Sullivan I, Elliot M, de Leval M, Stark J (1988) Con- HA (2007) Two rare cases of left and right atrial con-
genital left ventricular outflow obstruction: is the out- genital heart disease: cor triatriatum dexter and sinis-
come related to the site of obstruction? Eur J ter. Clin Res Cardiol 96:122–124
Cardiothorac Surg 2:312–317 Stellin G, Padalino M, Milanesi O et al. (2000) Repair of con-
Seward J, Tajik A, Ritter DG (1975) Echocardiographic fea- genital mitral valve dysplasia in infant and children: is
tures of straddling tricuspid valve. Mayo Clin Proc it always possible? Eur J Cardiothorac Surg 18:74–82
50:427–434 Stone K, Brown J, Canal D, Caldwell R, Hurwitz R, King H
Seward J, Tajik A, Fiest D, Smith H (1979) Ebstein’s anomaly (1990) Congenital aneurysm of the left atrial wall in
in a 85-year-old men. Mayo Clin Proc 54:193–196 infancy. Ann Thorac Surg 49:476–478
Shah M, Rychik J, Fogel M, Murphy J, Jacobs M (1997) Pul- Stulak J, Dearani J, Danielson G (2007) Surgical manage-
monary AV malformations after superior cavopulmo- ment of Ebstein’s anomaly. Semin Thorac Cardiovasc
nary connection: resolution after inclusion of hepatic Surg Pediatr Card Surg Ann 10:105–111
veins in the pulmonary circulation. Ann Thorac Surg Sugita T, Ueda Y, Matsumoto T, Ogino M, Nishizawa J-I, Mat-
63:960–963 suyama K (2001) Early and late results of partial plica-
Sharma V, Burkhart HM, Schaff HV, Cetta F, Cabalka A, Dea- tion annuloplasty for congenital mitral insufficiency. J
rani JA (2013) Management of zone of apposition in Thorac Cardiovasc Surg 122:229–233
parachute left atrioventricular valve in atrioventricular Sullivan ID, Robinson PJ, de Leval M, Graham TP Jr (1986)
septal defect. Ann Thorac Surg 95:1665–1169 Membranous supravalvular mitral stenosis: a treatable
Shiokawa Y, Becker A (1997) The left ventricular outflow form of congenital heart disease. J Am Coll Cardiol
tract in atrioventricular septal defects revisited: surgi- 8:159–164
cal considerations regarding preservation of aortic Suzuki T, Fukuda T (2002) Two-patch repair of complete
valve integrity in the perspective of anatomic observa- atrioventricular septal defect using small ventricular
tions. J Thorac Cardiovasc Surg 114:586–593 patch. Ann Thorac Surg 74:1718–1719
Shone J, Sellers R, Anderson R, Adams PJ, Lillhei C, Edwards Svenarud P, Persson M, Van Der Linden J (2003) Efficiency
J (1963) The developmental complex of parachute of a gas diffuser and influence of suction in carbon
mitral valve, supravalvular ring of left atrium, subaor- dioxide deairing of a cardiothoracic wound cavity
tic stenosis, and coarctation of aorta. Am J Cardiol model. J Thorac Cardiovasc Surg 125:1043–1049
11:714–725 Tabry I, McGoon D, Danielson G, Wallace R, Tajik A, Seward
Shumacker HB, King H (1961) A modified procedure for J (1979) Surgical management of straddling atrioven-
complete repair of total anomalous pulmonary venous tricular valve. J Thorac Cardiovasc Surg 77:191–201
drainage. Surg Gynecol Obstet 112:763–765 Tanoue Y, Kado H, Shiokawa Y, Sagawa K (2004) Left atrial
Silverman N (1997) The secundum atrial septal defect and appendage aneurysm in a child. Ann Thorac Surg
atrioventricular septal defects. Mosby, Philadelphia 77:721–723
Sim H-T, Lee S-C, Shin HJ, Park J-J, Yun T-J, Jhang W-K, Seo Ten Harkel A, Cromme-Dijkhuis A, Heinerman B, Hop W,
DM (2012) Mitral valve replacement using mechanical Bogers A (2005) Development of left atrioventricular
prosthesis in children: early and long-term outcomes. valve regurgitation after correction of atrioventricular
Pediatr Cardiol 33:639–645 septal defect. Ann Thorac Surg 79:607–612
Singh R, Warren P, Reece T, Ellman P, Peeler B, Kron I (2006) Thibault C, Perrault L, Delisle G, Cartier P, Cloutier A, Houde
Early repair of complete atrioventricular septal defect CJD (1995) Lobectomy in the treatment of the scimitar
is safe and effective. Ann Thorac Surg 82:1598–1602 syndrome. Ann Thorac Surg 59:220–221
422 V. Alexi-Meskhishvili et al.
Thilén U, Persson S (2006) Closure of atrial septal defect in anatomic and echocardiographic findings and surgi-
adult: cardiac remodeling is an early event. Int J Car- cal treatment. Am Heart J 128:365–379
diol 108:370–375 Van Son J, Danielson G, Puga F (1995a) Repair of congeni-
Thompson L, McElhinney D, Reddy V, Jue K, Hanley F (2000) tal and acquired pulmonary vein stenosis. Ann Thorac
Infradiaphragmatic totally anomalous pulmonary Surg 60:144–150
venous return with two separate descending veins in Van Son JA, Hovaguimian H, Rao IM et al. (1995b) Strate-
association with right atrial isomerism. Ann Thorac gies for repair of congenital heart defects in infants
Surg 70:1400–1402 without the use of blood. Ann Thorac Surg 59:384–388
Tierney ESS, Pigula FA, Berul CI, Lock JE, del Nido PJ, McEl- Van Son J, Schnider P, Falk V (1997) Repair of subaortic ste-
hinney DB (2008) Mitral valve replacement in infants nosis in atrioventricular canal with absent of restric-
and children 5 years of age and younger: evolution in tive intraventricular communication by patch
practice and outcome over three decades with focus augmentation of ventricular septum, resuspension of
on supra-anular prosthesis implantation. J Thorac Car- atrioventricular valves, and septal myectomy. Mayo
diovasc Surg 136:954–961 Clin Proc 72:220–224
Torres A, Ca D (1993) Surgical management of the scimitar Van Son J, Hambsch J, Mohr FW (1998a) Repair of complex
syndrome: an age-dependent spectrum. Cardiovasc unroofed coronary sinus by anastomosis of the left to
Surg 1:432–438 right superior vena cava. Ann Thorac Surg 65:280–281
Tournaire A, Deyrieux F, Tortulier M (1949) Maladie Van Son J, Falk V, Black M, Haas G, Mohr F (1998b) Conver-
d’Ebstein: essai de diagnostic clinique. Arch Mal Coeur sion of complex neonatal Ebstein’s anomaly into func-
42:1211–1216 tional tricuspid or pulmonary atresia. Eur J
Tsang VT, Mullaly RJ, Ragg PG, Karl TR, Mee RB (1994) Cardiothorac Surg 13:280–285
Bloodless open-heart surgery in infants and children. van Son J, Hambsch J, Mohr FW (1998c) Suspension of
Perfusion 9:257–263 straddling tricuspid valve chordae into appropriate
Ullmann M, Born S, Sebening C, Gorenflo M, Ulmer H, Hagl ventricle. Ann Thorac Surg 65:850–852
S (2004) Ventricularization of the atrialized chamber. A Vargas F, Mengo G, Granja M, Gentile J, Rannzini M,
concept of Ebstein’s anomaly repair. Ann Thorac Surg Vazquez J (1998) Tricuspid annuloplasty and ventricu-
78:918–924 lar plication for Ebstein’s malformation. Ann Thorac
Uva MS, Galletti L, Lacour-Gayet F (1995) Surgery for con- Surg 65:1755–1757
genital mitral valve disease in the first year of life. J Varghese P (1969) Multiple saccular congenital aneurysm
Thorac Cardiovasc Surg 109:164–176 of the atria causing persistent atrial tachyarrhythmia
Vagefi P, Choudrhy M, Hilgenberg A (2007) Excision of an in an infant. Pediatrics 44:429–433
aneurysm of the left atrial appendage. J Thorac Car- Victor S, Nayak V (2001) Aneurysm of the left atrial append-
12 diovasc Surg 133:822–923
Van Arsdel G, Williams W, Boutin C et al. (1995) Subaortic
age. Tex Heart J 28:111–118
Vineberg A, Gialoreto O (1956) Report of successful opera-
stenosis in the spectrum of atrioventricular septal tion for stenosis of common pulmonary veins (cor tria-
defects. Solutions may be complex and palliative. J triatum). Can Med Assoc J 74:719–723
Thorac Cardiovasc Surg 110:1534–1542 Vogel M, Kirchhoff A, Buhlmeyer K, Bourlon F (1995) Open
van Arsdell G (2006) Discussion on paper of Kohn et al. heart surgery without homologous blood transfu-
Intermediate results of double-switch operation for sion and subsequent use of recombinant erythropoi-
atrioventricular discordance. Ann Thorac Surg 86:671– etin to treat postoperative anemia. Cardiol Young
677. Ann Thorac Surg 81:677 5:118–121
van der Wal H, Hamilton D, Godman N, Lacquert L, van Oort Vogel M, Marx GP, Tworetzky W, Cecchin F, Mayer JE, Pigula
A (1992) Pulmonary venous obstruction following cor- FA, Bacha EA, Del Nido PJ (2012) Ebstein’s malforma-
rection for total anomalous pulmonary venous drain- tion of the tricuspid valve: short-term outcomes of the
age: a challenge. Eur J Cardiothorac Surg 6:545–549 “Cone Procedure” versus conventional surgery. Conge-
Van Mierop L, Alley R (1966) The management of the cleft nit Heart Dis 7:50–58
mitral valve in endocardial cushion defects. Ann Tho- Vohra H, Laker S, Stumper O et al. (2006) Predicting the per-
rac Surg 2:416–423 formance of mitral prostheses implanted in children
Van Praagh R, Corsini I (1969) Cor triatriatum: pathologic under 5 years of age. Eur J Cardiothorac Surg 29:688–692
anatomy and a consideration of morphogenesis based Wakai CS, Edwards JE (1956) Developmental and patho-
on 13 postmortem cases and a study of normal devel- logic considerations in persistent common atrioven-
opment of the pulmonary vein and atrial septum in 83 tricular canal. Proc Mayo Clin 31:487–500
human embryos. Am Heart J 78:379–405 Wakai CS, Edwards JE (1958) Pathology study of persis-
van Praagh R, Ongley P, Swan HJC (1964) Anatomic types tent common atrioventricular canal. Am Heart J
of single or common ventricle in man. Am J Cardiol 56:779–794
13:367–386 Wang F, Li M, Xu X, Yi S, Cheng Z, Deng C, Yi D (2011) Totally
Van Praagh R, Harken A, Delisle G, Ando M, Gross R (1972) thoracoscopic surgical closure of atrial septal defect in
Total anomalous pulmonary venous drainage to the small children. Ann Thorac Surg 92:200–203
coronary sinus. A revised procedure for its correction. Warden H, Gustafson R, Tarnay T (1984) An alternative
J Thorac Cardiovasc Surg 1:132–135 method for repair of partial anomalous pulmonary
Van Praagh S, Carrera M, Snaders S (1994) Sinus venosus venous connection to the superior vena cava. Ann
defects: unroofing of the right pulmonary veins— Thorac Surg 38:601–605
Chapter 12 · Congenital Defects of Veins, Atria, AV-Septum, and AV-Valves
423 12
Watraida S, Shiraishi S, Katsuyama K et al. (1997) Supraval- Yoshida Y, Fukuda M, Sasaki T, Hoshino H, Sato T (1977)
vular stenotic mitral ring with ventricular septal Echocardiographic futures of straddling mitral valve.
defect. J Card Surg 12:46–48 Tohoku J Exp Med 122:387–392
Watson H (1974) Natural history of Ebstein’s anomaly of Young D, Robinson G (1964) Successful valve replacement
tricuspid valve in childhood and adolescence: an in an infant with congenital mitral stenosis. N Engl J
international co-operative study of 505 cases. Br Heart Med 26:660–664
J 36:417–427 Yu JJ, Yun T-J, Won H-S, Im YM, Lee BS, Kang SY, Ko HK, Park
Wedemeyer A, Lucas RV, Castaneda A (1970) Surgical cor- CS, Park J-J, Gwak M, Kim EA-R, Kim Y-H (2013) Out-
rection in infancy of an unusual form of triatrial heart. come of neonates with Ebstein’s anomaly in current
J Thorac Cardiovasc Surg 59:685–690 era. Pediatr Cardiol 34:1590–1596
Wetter J, Sinzobahamvyaa N, Blaschczoka C et al. (2000) Yun T, Coles J, Konstantinov I et al. (2005) Conventional
Closure of the zone of apposition at correction of com- and sutureless techniques for management of the
plete atrioventricular septal defect improves outcome. pulmonary veins: evolution of indications from
Eur J Cardiothorac Surg 17:146–153 postrepair pulmonary vein stenosis to primary pul-
Whooler GH, Nixon PGF, Grimshaw VA, Watosn DA (1962) monary vein anomalies. J Thorac Cardiovasc Surg
Experience with the repair of the mitral valve in mitral 129:167–174
incompetence. Thorax 17:49–57 Yun T, Al-Radi O et al. (2006a) Contemporary management
Wilcox B, Anderson R (1985) Surgical anatomy of the heart. of right atrial isomerism: effect of evolving therapeutic
Raven Press, New York strategies. J Thorac Cardiovasc Surg 131:1108–1113
Wilcox B, Cook A, Anderson R (2004) Surgical anatomy of Yun T-J, Lee S-H, Ko J-K (2006b) Neonatal stenotic Ebstein’s
the heart. Cambridge University Press, Cambridge anomaly: a novel technique of right ventricular exclu-
Wilson J (1798) On very unusual formation of the human sion. J Thorac Cardiovasc Surg 131:469–471
heart. Phil Trans B 88:332 Zenker R, Klinner W, Meisner H, Schmidt-Habelmann P,
Wood A, Healey D, Nilke L, Duff D, Oslizlok P, Walsh K (2004) Sebening F (1965) Seltene angeborene Herzfehler und
Mitral valve reconstruction in pediatric population: ihre chirurgische Behandlung. Dtsch Med Wschr
late clinical results and predictors of long-term out- 6:696–703
come. J Thorac Cardiovasc Surg 130:66–73 Zias E, Mavroudis C, Backer C, Kohr L, Gotteiner N, Rocchini
Yanagawa B, Alghamdi AA, Dragulescu A, Viola N, Al-Radi A (1998) Surgical repair of the congenitally malformed
OO, Mertens LL, Coles JG, Caldarone CA, Van Arsdell mitral valve in infants and children. Ann Thorac Surg
GS (2011) Primary sutureless repair for “simple” total 1998:1551–1559
anomalous pulmonary venous connection: midterm Zweng T, Bluett M, Mosca R, Callow L, Bove E (1989) Mitral
results in a single institution. J Thorac Cardiovasc Surg valve replacement in the first five years of life. Ann
141:1346–1354 Thorac Surg 47:720–726
425 13
Definite Palliation
of Functional
Single Ventricle
Rüdiger Lange and Jürgen Hörer
References – 456
428 R. Lange and J. Hörer
vena cava, which is present in up to 15 % of these 55 Ib: restrictive bulboventricular foramen and/
hearts. The position of the sinus node is normal; or pulmonary stenosis
the AV node is on the right hand side of the cen- 55 Ic: unrestrictive bulboventricular foramen, no
tral fibrous skeleton. The course of the ventricular pulmonary stenosis
bundles depends on the position of the bulboven-
tricular foramen but are mostly positioned poste- Group II (ventriculoarterial discordance,
rior and inferior of the defect. 30–40 % of the cases) (Edwards and Burchell
Two further groups can be distinguished 1949; Annecchino et al. 1980):
based on the type of the ventriculoarterial con- 55 IIa: no bulboventricular foramen and
nection: ventriculoarterial concordance (normal pulmonary atresia
position of the great arteries) and ventriculoarte- 55 IIb: restrictive bulboventricular foramen and
rial discordance (transposition of the great arter- pulmonary stenosis
ies) (Rosenthal and Dick 1983). According to the 55 IIc: unrestrictive bulboventricular foramen,
size of the bulboventricular foramen and the no pulmonary stenosis
grade of the pulmonary stenosis, three more sub-
groups can be recognized: Double outlet from the right or left ventricle is rare
Group I (ventriculoarterial concordance, (Anderson et al. 1977), while other forms are even
60–70 % of the cases) more uncommon. Altogether, 80 % of the patients can
55 Ia: no bulboventricular foramen and be assigned to the aforementioned groups (Anderson
pulmonary atresia and Rigby 1987; Forrest et al. 1987). . Figure 13.1
illustrates the classification according to Edwards and
la lb lc
13
..Fig. 13.1 Most common forms of tricuspid atresia in a simplified categorization according to Rosenthal and Dick
(1983) (Description in text)
Chapter 13 · Definite Palliation of Functional Single Ventricle
431 13
Burchell (1949) in a simplified categorization accord- 13.3.3 earts with Common
H
ing to Rosenthal and Dick (1983). Atrioventricular Connection
Where Only One Ventricle Is
13.3.2.2 Mitral Atresia Normally Developed
In analogy to the absence of the right atrioventricu-
lar connection, the absence of the left atrioventric- Functional single ventricles with a malformation
ular connection can be accompanied with a based on a defect of endocardial cushion are
number of different heart defects. The classical included in this rare subgroup. Unlike the hypo-
example of the mitral atresia is a functional single plastic ventricles in other groups described in this
ventricle with a morphological right chamber and chapter, the chambers in this subgroup are not rudi-
an absence of the left atrioventricular connection. mentary since the inlet part of the ventricle is devel-
Just like in the cases of tricuspid atresia, mitral atre- oped. They are however hypoplastic since the blood
sia can be accompanied by an impermeable valve flow through the common atrioventricular valve is
on which chordae may still be identifiable. preferentially directed in the dominant ventricle.
However, a membrane consisting of connective The underlying pathology is the imbalance between
and fat tissue is found more often (Thiene et al. the components (right/left) of the atrioventricular
1981). When mitral and aortic atresia is present, valve and the two ventricles (right/left). The dimen-
the heart defect is classified as a part of the hypo- sion of this imbalance determines the degree of
plastic left heart syndrome (see 7 chapter «Surgery hypoplasia and the dominance of the ventricle.
for Aortic Atresia, Hypoplastic Left Heart Syndrome, In the right-dominant type, the right ventricle
and Hypoplastic Left Heart Complex», Sect. 17.1). This is large, the left ventricle is smaller than normal
is also the case in different combinations of mitral and the blood flow from the atria in the ventricles
atresia/stenosis and aortic atresia/stenosis. is preferentially directed into the right ventricle
Borderline cases are sometimes difficult to classify, due to displacement of the common atrioventric-
which is why the term hypoplastic left heart syn- ular valve towards the right. In left-dominant
drome should always be used when there is no pos- type, the left ventricle is large, the right ventricle is
sibility of biventricular correction and where a smaller than normal and the blood flow from the
definitive palliation leading to Fontan circulation atria into the ventricles is preferentially directed
must be undertaken (Kouchoukos et al. 2003a). For into the left ventricle due to displacement of the
these borderline cases, also the term hypoplastic common atrioventricular valve towards the left.
left heart complex is used (Tchervenkov et al. 1998) In the cases with left dominance, there is a notable
see also 7 chapter «Surgery for Aortic Atresia, hypoplasia of the right ventricle. However, it is
Hypoplastic Left Heart Syndrome, and Hypoplastic sometimes very difficult to separate the right-
Left Heart Complex», Sect. 17.6.8. dominant type with a real dominance of the right
Most of these hearts have normally posi- ventricle, and the hypoplasia of the left ventricle
tioned atria, d-loop of the ventricles, a dominant from the common cases of atrioventricular sep-
right ventricle which is connected to the right tum defect with balanced ventricles, where the
atrium through the tricuspid valve, and a hypo- right ventricle is larger due to volume overload
plastic left ventricle positioned left and posterior. (Jacobs 2003).
In the cases with ventriculoarterial concordance It is very important to determine the degree of
and an existent connection between the domi- hypoplasia of the smaller ventricle in order to
nant and the hypoplastic ventricle, the size of the decide on surgical strategy. The atrioventricular
aorta ascendens and the aortic arch is determined valve index, introduced by Cohen et al. (1996),
through the size of the bulboventricular foramen. has proven to be helpful in deciding which patient
When the foramen is restrictive, the ascending is suitable for biventricular repair and in which
aorta and the arch are hypoplastic. In some cases, univentricular palliation is necessary.
the aortic isthmus is also stenotic, and rarely, Hypoplasia of the aortic arch, or aortic isth-
there is an interrupted aortic arch (Jacobs 2003). mus stenosis, as well as a prominent pulmonary
Mitral atresia with ventriculoarterial discor- artery is relatively common in patients with right
dance is very rare. More frequent are the cases dominance of the common atrioventricular con-
with double outlet from the right ventricle. The nection. On the other hand , the aortic arch and
left ventricle is in these cases a blind pouch which the pulmonary artery are usually normally devel-
is connected only to the right ventricle. oped in the left-dominant cases (Jacobs 2003).
432 R. Lange and J. Hörer
PA
13.5.3 Cavopulmonary Anastomosis
PA RPA Neo-
AOA
LA
LA
RV
LV RA RV
LV
RA
VCS
operation techniques, which are used less fre-
quently, and which have special indications, as
well as more controversial treatment strategies,
Neo-AOA will be discussed in 7 Sects. 13.7.4 and 13.7.5.
RPA
13.5.5 Staged Therapy
for Completing the Fontan
Circulation
LA
Most of the patients with functional single ven-
tricle require a palliative operation, which enables
the control over the pulmonary blood flow within
LV
RA RV the first days of life. This often resulted in a stable
situation over the next couple of years and enabled
patients to maintain good ventricular function
and low pulmonary resistance until the Fontan
operation. Nevertheless, the postoperative course
after Fontan operation was commonly compli-
VCI cated through high central venous pressure, pleu-
ral effusions, ascites, and a low-output syndrome.
..Fig. 13.6 Extracardiac cavopulmonary connection.
RPA right pulmonary artery
It was noticed that the conversion of the ‘mixed’
circulation in a Fontan circulation results in an
immediate volume unloading of the ventricle.
However, due to a slower regression of the ven-
The next logical step was to avoid using the tricle mass, there is a systolic and a diastolic dys-
atrial tissue at all for the construction of the cavo- function of the single ventricle. Accordingly,
pulmonary tunnel. Danielson and Norwood Kirklin et al. (1986) identified an older age at the
achieved this by implantation of a conduit between time of Fontan circulation and ventricular hyper-
the inferior vena cava and the pulmonary artery trophy as risk factors for mortality. Francois et al.
13 (2005) showed that younger age at Fontan opera-
(Jacobs 2003). Based on the report from Marcelletti
et al. (1990) who used this conduit outside of the tion has beneficial influence on major complica-
heart, first extracardiac cavopulmonary connec- tions and outcome. Schreiber et al. (2007) showed
tion was introduced (. Fig. 13.6). Using the modi- that completion of the Fontan circulation is feasi-
fications of the intracardiac and extracardiac ble with very low mortality at an age of
cavopulmonary connection, it became possible to 16–48 months.
complete the Fontan circulation in almost all This lead to a hypothesis that dividing the
patients with functional single ventricle. Fontan operation in two operations would lead to
The Fontan operation is the endpoint for all earlier volume unloading and possibly reduce the
patients in whom a biventricular repair is hypertrophy of the ventricle. Norwood et al.
unachievable due to intracardiac anatomy. The (Norwood and Jacobs 1993) introduced a ‘hemi-
objective of all Fontan modifications is to pas- Fontan’ operation in 1989, as a middle stage on a
sively divert the blood from the vena cavas (in way of completing the Fontan circulation. (Norwood
some modifications—from the coronary sinus as and Jacobs 1993). Physiologically, this operation
well) into to lungs and to use the functional single was similar to the bidirectional Glenn. The differ-
ventricle as the only pumping chamber for the ence is that this operation is a part of the treatment
newly constructed serial systemic pulmonary cir- strategy towards completing the Fontan circulation.
culation. This also explains the term ‘definitive A connection between the superior vena cava and
palliation’. Different Fontan modifications, which the right pulmonary artery is established, just like in
are still being performed today, have both poten- the bidirectional Glenn anastomosis. The continuity
tial advantages and disadvantages and will there- between the superior vena cava and the right atrium
fore be presented in the following paragraphs. The is however left in place and closed with a patch. At
Chapter 13 · Definite Palliation of Functional Single Ventricle
437 13
the time of Fontan operation, this patch is removed 13.7 Surgical Techniques
to complete the intraatrial total cavopulmonary
connection. The introduction of this additional 13.7.1 tage I Palliation: Controlling
S
operation as the second stage in Fontan completion the Pulmonary Blood Flow
has significantly improved the probability of sur-
vival of the patients with functional single ventricle The purpose of neonatal palliation of patients
(Attanavanich et al. 2007). with all forms of functional single ventricle is bal-
Other associated heart defects as pulmonary ancing the systemic and pulmonary blood flow. In
artery stenosis, subaortic stenosis, and insufficiency addition, an unrestrictive blood flow from the
of an atrioventricular valve may be addressed at the systemic and pulmonary veins has to be secured
time of hemi-Fontan operation. The main pulmo- at the atrial level. All stenoses of the systemic out-
nary artery is divided from the heart at the level of flow tract of the functional single ventricle as well
the valve. This step simplifies the Fontan operation, as central pulmonary artery stenoses have to be
during which the patch between the superior vena addressed and corrected, also.
cava and the right atrium is removed in order to
establish the continuity between the superior and the 13.7.1.1 Systemic-to-Pulmonary
inferior vena cava, in terms of formation of the lateral Artery Shunt
tunnel. Some surgeons prefer the bidirectional Glenn Reduced pulmonary blood flow is typically found
anastomosis over the hemi-Fontan operation and in the presence of tricuspid atresia with ventriculo-
tend to complete the Fontan circulation through an arterial concordance. The cause for this may be
implantation of an extracardial conduit between the pulmonary stenosis, infundibulum stenosis or a
inferior vena cava and the right pulmonary artery. restrictive bulboventricular foramen. In some
cases, a ductus-dependent pulmonary circulation
is present. In all these patients, lung perfusion has
13.6 Indications for Definitive to be secured through creation of a shunt between
Palliation and Fontan the aorta and one of its branches to the pulmonary
Circulation artery. Another large group of patients with func-
tional single ventricle who need a shunt are the
Definitive palliation and construction of Fontan patients with an obstruction in the outflow tract
circulation has to be considered in all patients towards the systemic circulation and the aortic
with a functional single ventricle, which possesses arch. In order to correct these stenoses, the main
a sufficient size and function, in order to provide pulmonary artery is used to bypass the obstruction
a serial connection of the pulmonary and sys- (Stansel anastomosis (Damus 1975; Kaye 1975;
temic circulation. This is usually the case in Stansel 1975)) as in modified Norwood operation.
patients with an absent atrioventricular connec- As a result, there is no continuity between the
tion, double inlet in the dominant ventricle or an functional single ventricle and the pulmonary
unbalanced atrioventricular septal defect. artery, which prompts for a shunt implantation.
The Fontan operation also has to be performed Sternotomy is the preferred approach, since it
in some cases where a normally functioning but enables the surgeon to have a better exposition of
morphologically underdeveloped ventricle is con- all relevant structures, and it secures a quick con-
nected to the pulmonary circulation. This is the nection to the heart-lung machine in case of hemo-
case in extreme forms of Ebstein anomaly and in dynamic instability. The shunt can be placed more
the presence of pulmonary atresia with intact ven- centrally than through a thoracotomy, which can
tricular septum (Alboliras et al. 1987). Finally, reduce the incidence of upper lobe artery stenosis,
there are heart defects, in which two normally while a possible main pulmonary artery stenosis
developed chambers are present, but a biventricu- can easily be reconstructed using a patch. The duct
lar repair is very difficult due to complex ventricle can be safely divided, and an atrioseptectomy can
position, abnormal position of the great vessels or easily be performed if indicated. The only disad-
multiple ventricular septal defects. In some of vantage of this approach is the formation of adhe-
these complex cases, the Fontan circulation might sions in the area of ventricle and atria, the extent of
provide better early results as the biventricular which can be reduced by limiting the pericardial
correction (Russo et al. 1988; Hraska et al. 2005). incision to the part above the great arteries.
438 R. Lange and J. Hörer
A further advantage of avoiding lateral thora- the following operations. The brachiocephalic
cotomy in staged palliation of single ventricle cir- trunk is mobilized up to the bifurcation of the
culations is the impression that later development right carotid and subclavian artery. Heparin is
of rather significant aortopulmonary collateral given at a dose of 3 mg/kg and a vascular clamp is
perfusion of the lungs is clearly pronounced on placed on the brachiocephalic trunk proximal to
the side of previous thoracotomies. the carotid artery, so that the vessel is excluded. A
longitudinal incision is made in the vessel
kModified Blalock-Taussig shunt (. Fig. 13.7). Care has to be taken not to incise the
In the next step, at least the right side of the thy- back wall of the vessel, also. An elastic polytetra-
mus is subtotally resected. We prefer subtotal thy- fluorethylene tube with a diameter of 3.5 or 4 mm
mus resection at the first surgery as this facilitates is usually used for newborns and infants. The
A.Pulmonary artery
a b
Aorta
c
13
..Fig. 13.7 a–c Construction of a right-sided modified Blalock-Taussig shunt through a median sternotomy, without
cardiopulmonary bypass. The thymus is subtotally resected, and the truncus brachiocephalicus mobilized up to the bifurcation
of the right carotid and subclavian artery. Either the proximal or the distal anastomosis can be performed as a first step. In the
described case, the anastomosis with the pulmonary artery is completed first. The right pulmonary artery is partially occluded
with a vascular clamp and longitudinally incised. The anastomosis with polytetrafluorethylene tube is performed using a
non-resorbable monofilament suture 6/0 or 7/0. The posterior circumference of the anastomosis is performed at the beginning
a. After completion of the anastomosis, the vascular clamp is removed from the right pulmonary artery, and the shunt occluded.
In this way, the distal anastomosis is free from rotating forces and the correct length of the shunt can be easily determined. The
length of the shunt is of outmost importance, since a shunt which is too long as well as a shunt which is too short can lead to
torsion and stenosis of the pulmonary artery. After trimming of the shunt to the correct length, a vascular clamp is placed on the
truncus brachiocephalicus proximal to the right subclavian artery, and the vessel is longitudinally incised b. The anastomosis
with polytetrafluorethylene tube is performed using a non-resorbable monofilament suture 6/0 or 7/0. Special attention has to
be paid to the angle between the artery and the shunt. After completion of the anastomosis, the vascular clamp is removed. At
the end, the ductus arteriosus is encircled with a non-resorbable suture 5/0 and ligated c
Chapter 13 · Definite Palliation of Functional Single Ventricle
439 13
anastomosis is performed using a non-resorbable than the modified Blalock-Taussig shunts.
monofilament suture 6/0 or 7/0. Special attention Furthermore, the proximal diameter of the bra-
has to be paid to the angle between the artery and chiocephalic trunk in modified Blalock-Taussig
the shunt. After completion of the anastomosis, shunt adds to flow restriction or even is a major
the vascular clamp is removed, and the shunt cause for flow reduction. Therefore, the diameter
occluded. In this way, the proximal anastomosis is of the tube in aortopulmonary shunts should be
free from pulling and rotating forces and the cor- proportionally smaller. The smallest polytetrafluo-
rect length of the shunt can be easily determined. rethylene tube has a diameter of 3 mm. If this tube
The length of the shunt is of utmost importance, is too large for the patient, a surgeon can try to
since a shunt which is too long as well as a shunt place a longer shunt with proximal anastomosis in
which is too short can lead to a torsion, kinking the aortic arch. Flow reduction through partially
and stenosis of the shunt itself or of the pulmo- shunt clipping (at least when smaller tubes are
nary artery. After trimming of the shunt to the used) leads to premature cyanosis requiring treat-
correct length, the right pulmonary artery is ment. Interventional balloon dilatation may be
occluded with a vascular clamp and longitudi- used to widen a partially clipped shunt to the nor-
nally incised. The anastomosis is done with con- mal size. The potential risk of shunt tearing at the
tinuous 6/0 or 7/0 non-resorbable monofilament site of clip application when balloon dilated should
suture. The vascular clamp is then removed. be taken into account by the interventionalist.
Basically, either the proximal or the distal anasto-
mosis can be made first. We prefer to perform the 13.7.1.2 Pulmonary Artery Banding
proximal anastomosis first, since the access to the Increased pulmonary blood flow is typically
inominate artery is more difficult compared to the found in the presence of tricuspid atresia with dis-
access to the pulmonary artery. cordant ventriculoarterial connection. Since the
Before opening the shunt, the ductus arterio- aorta rises from a hypoplastic ventricle, it is usu-
sus is encircled with vessel loop and temporarily ally underdeveloped. Many patients also have an
occluded. The hemodynamic situation is moni- aortic isthmus stenosis with isthmus hypoplasia
tored after opening of the shunt. The saturation and a hypoplastic aortic arch. In these cases, pul-
should be between 75 and 85 %. If the saturation is monary artery banding is often done concomi-
lower than 75 %, the shunt might be too small or tantly with the resection of the aortic isthmus
one or both of the anastomoses may be stenotic. stenosis (Rodefeld et al. 2005). In cases where the
Oxygen saturations higher than 85 % and a dia- aortic valve and the ascending aorta are hypoplas-
stolic blood pressure lower than 30 mmHg can tic, a modified Norwood operation with place-
point to an oversized shunt. In all these cases, ment of a systemic-to-pulmonary shunt is
shunt revision should be considered. In the pres- performed.
ence of stable hemodynamics, the ductus now A sternotomy is the preferred approach for the
should be permanently occluded to avoid com- same reasons described in the previous paragraphs.
petitive flow if it would reopen. After resection of the left part or all of the thymus,
the pericardium is opened above the great arteries.
kCentral aortopulmonary shunt A midpoint between the sinotubular junction of
There are many possible positions for an aortopul- the pulmonary artery and the right pulmonary
monary shunt, both on the aortic and the pulmo- artery is noted. The tissue between aorta and the
nary side. The decision of the exact place for shunt main pulmonary artery is dissected in this limited
implantation depends on the individual anatomy. area, and the two arteries are separated from each
In patients with small pulmonary arteries, it might other (. Fig. 13.9). The band should not be too
be an advantage to place the shunt on the main narrow to avoid erosions of the pulmonary artery,
pulmonary artery (. Fig. 13.8). The proximal and the material should induce as few adhesions as
anastomosis is then usually placed in the ascend- possible. A 5–8 mm cut open segment of a PTFE
ing aorta. In some cases, the central pulmonary tube or a band cut out of Dacron net-reinforced
artery has to be augmented, and the shunt can be silastic sheeting provide ideal banding material.
placed in an incision in the patch. In these cases, After placing of the band around the pulmo-
the distal anastomosis should be performed first. nary artery, the two free ends of the tape are fixed.
Central aortopulmonary shunts are usually shorter The gradient over the banding is slowly adjusted,
440 R. Lange and J. Hörer
a b
c d
13
..Fig. 13.8 a–d Construction of a central aortopulmonary shunt through a median sternotomy, with
cardiopulmonary bypass. The thymus is subtotally resected, and the ascending aorta, pulmonary trunk and both pulmo-
nary arteries mobilized a. After cannulation of the ascending aorta (on the opposite side of truncus brachiocephalicus)
and the right atrium, the pulmonary arteries are occluded. The pulmonary trunk is longitudinally incised at the
bifurcation. The shunt can be first connected with a polytetrafluorethylene or homograft patch on which a central
round excision was performed. This patch is then used for widening the pulmonary trunk b. The anastomosis is
performed using a non-resorbable monofilament suture 7/0. After trimming of the shunt to the correct length, an
appropriate place on the ascending aorta is identified. The aorta is partially occluded and incised c. The anastomosis of
the shunt with aorta is performed using a non-resorbable monofilament suture 7/0. The shunt is correctly positioned
when it is not kinking and when the pulmonary artery is not pushed dorsally or pulled anteriorly d
under strict control of the pressure in pulmonary in order to avoid dislocation and possible stenosis
artery distal from the banding and the systemic of the pulmonary bifurcation. Alternatively, the
pressure. The banding tape is constricted in a step- ‘Trusler formula’ can be used: the length of the
wise fashion, using metal clips or stitches in order band is calculated for each patient. It should be
to increase the gradient over the banding. The arte- 2 cm + 1 mm/kg of body weight in patients with
rial saturation is monitored during this procedure. normally positioned great vessels, and 2,2 cm +
A gradient of 30–60 mmHg and arterial saturation 1 mm/kg of body weight in patients with transpo-
of 75–85 % are acceptable, as long as the systemic sition of the great arteries. The calculated length of
arterial pressure corresponds to the normal pres- the band may require adjustments according to the
sure in a spontaneously breathing newborn. When arterial saturation, as described before. Pulmonary
the banding is in the definitive position, the tape is artery pressure is usually not measured when
fixed to the proximal part of the pulmonary artery employing this formula.
Chapter 13 · Definite Palliation of Functional Single Ventricle
441 13
a b c
..Fig. 13.9 a–c Pulmonary artery banding. After resection of the left part of the thymus, the pericardium is opened
above the great arteries. A midpoint between the sinotubular junction of the pulmonary artery and the right
pulmonary artery is noted. The tissue between aorta and the main pulmonary artery is dissected in this limited area,
and the two arteries are separated from each other a. Further resection should be avoided in order to prevent the
dislocation of the banding tape. After placing of the banding tape around the pulmonary artery, the two free ends of
the tape are fixed b. The gradient over the banding is slowly adjusted, under strict control of the pressure in pulmonary
artery distal from the banding and the systemic pressure. The banding tape is constricted in a stepwise fashion using
metal clips or sutures, in order to increase the gradient over the banding. The arterial saturation is monitored during this
procedure. A gradient of 30–60 mmHg and arterial saturation of 75–85 % are acceptable, as long as the systemic arterial
pressure corresponds to the normal pressure in a spontaneously breathing newborn. When the banding is in the
definitive position, the tape is fixed to the proximal part of the pulmonary artery in order to avoid dislocation and
possible stenosis of the pulmonary bifurcation c
valve insufficiency. Addressing all of the above- flow, the shunt can be divided. In the presence of
mentioned problems can preserve good function an anterograde flow through the pulmonary valve,
of the functional single ventricle and make the one may decide to divide the pulmonary artery
patient a better candidate for a Fontan operation. after securing the vessel with two vascular clamps
Two surgical techniques are used as stage II pal- and placing hemostatic sutures. If there is a need
liation, both resulting in partial separation of the for additional pulmonary blood flow, the shunts
pulmonary and systemic circulation through redi- may be temporarily occluded. In these cases, the
recting the desaturated blood from the superior vena pulmonary artery banding is left in place. In case
cava to the pulmonary artery: bidirectional superior of division of the main pulmonary artery, it is rec-
cavopulmonary anastomosis (bidirectional Glenn ommended to close the proximal pulmonary
anastomosis) and the hemi-Fontan operation. stump at the levels of the pulmonary valve, avoid-
ing a blind pouch which could become source of
13.7.2.1 Bidirectional Superior systemic thromboemboli.
Cavopulmonary The azygos vein is divided in order to prevent
Anastomosis (Bidirectional blood flow from the superior to the inferior vena
Glenn Anastomosis) cava after the Glenn anastomosis is accomplished.
Superior cavopulmonary anastomosis may be per- If there is interruption of the inferior vena cava
formed with or without extracorporeal circula- with azygos continuation, the azygos vein must be
tion. The precondition for not using kept patent. This is often the case in patients with
cardiopulmonary bypass is the presence of a heterotaxy syndrome with left isomerism. The
source of pulmonary blood flow aside from the Glenn anastomosis in face of an azygos contin-
site of the planned Glenn anastomosis. This chap- uum is referred to as Kawashima operation lead-
ter will describe the operation using the heart-lung ing comparatively high oxygen saturations up to
machine (. Fig. 13.10). Cardiopulmonary bypass 90 % and more (Matsuda et al. 1986) (See also
is initiated after cannulation of the aorta, the right 7 13.7.5.2.).
atrium and the superior vena cava at the junction In simple Glenn anastomosis, a vascular clamp
of the left brachiocephalic vein. All systemic-to- is applied immediately above the cavoatrial junc-
pulmonary shunts have to be controlled at this tion. The position of the sinus node must be noted.
point. If there is no need for additional pulmonary The superior vena cava is transected, and the
13
a b
..Fig. 13.10 a, b Superior cavopulmonary anastomosis. The right pulmonary artery is clamped near the bifurcation,
on the right side of the aorta. Superior vena cava, the right pulmonary artery and the upper lobe artery are snared. The
azygos vein is ligated and divided. The superior vena cava is divided from the right atrium, and the cardiac end is
oversewn a. The right pulmonary artery is partially excluded, and an incision is made on the superior wall. The
anastomosis between the superior vena cava and the pulmonary artery is carried out with a running resorbable
monofilament suture 6/0 or 7/0. The anastomosis should extend until the right upper lobe artery b
Chapter 13 · Definite Palliation of Functional Single Ventricle
443 13
cardiac end is oversewn. The right pulmonary 13.7.2.2 Hemi-Fontan Operation
artery is clamped either proximally or distally or a The physiology following a hemi-Fontan operation
C-clamp is used. A long incision is made on the is equivalent to the state after bidirectional supe-
superior wall of the RPA. The cavopulmonary rior cavopulmonary anastomosis. There are how-
anastomosis is then carried out with a running ever basic differences in the operation technique.
resorbable monofilament suture. Cardiopulmonary The hemi-Fontan operation always is an open-
bypass is discontinued while carefully observing heart procedure and must therefore be performed
the pressure in the right atrium, in the superior using extracorporeal circulation (. Fig. 13.11).
vena cava, the systemic pressure and the arterial Following aortic and bicaval cannulation, and
saturation. A transpulmonary gradient of 8 mmHg initiation of the cardiopulmonary bypass, the
and a pressure of less than 18 mmHg in the supe- caval veins are snared, the aorta is cross-clamped
rior vena cava are acceptable. The arterial satura- and cardioplegic solution is infused in the aortic
tion should be approximately 80 %. root. The main pulmonary artery is then divided,
a b
c d
..Fig. 13.11 a–d Hemi-Fontan operation. The hemi-Fontan operation must be performed using the heart-lung
machine and requires cardioplegia and cross-clamping of the aorta. Following aortic and bicaval cannulation, and
initiation of the cardiopulmonary bypass, the caval veins are snared, the aorta is cross-clamped and cardioplegic
solution is infused in the aortic root. The main pulmonary artery is then divided, and both ends are oversewn. The
pulmonary arteries are fully mobilized and controlled with snares. An incision is made in the dome of the right atrium
and spirally extended towards the medial wall of superior vena cava. An incision is made in the right pulmonary artery
immediately adjacent to the incision line in the right atrium a. The dorsal walls of the incision are connected with a
running resorbable monofilament suture b. The anterior anastomosis is completed by patch augmentation c. The
junction of the superior vena cava and the right atrium is closed using a patch, through a separate incision in the right
atrium to direct the blood from the superior caval vein to the pulmonary artery d
444 R. Lange and J. Hörer
and both ends are oversewn, avoiding creation of total cavopulmonary connection’) and a cre-
a proximal blind pouch (see 7 13.7.2.1). The pul- ation of an intraatrial lateral tunnel (‘intracar-
monary arteries are fully mobilized and controlled diac total cavopulmonary connection’).
with snares. An incision is made in the dome of
the right atrium and spirally extended towards the 13.7.3.1 Extracardiac Total
medial wall of superior vena cava. An incision is Cavopulmonary Connection
made in the right pulmonary artery immediately The surgical technique performed in patients with
adjacent to the incision line in the right atrium. tricuspid atresia, in whom a bidirectional supe-
The dorsal walls of the incision are connected with rior cavopulmonary anastomosis was previously
a running resorbable monofilament suture. The performed, will be described in this paragraph
anterior anastomosis is completed by patch aug- (. Fig. 13.12). The technique described can basi-
mentation in order to achieve a wide connection cally be performed in all patients with functional
and an unobstructed blood flow between the single ventricle, including patients who were not
superior vena cava and the pulmonary arteries. operated previously. In some cases, as for example
The incised junction of the superior vena cava atrial isomerism, situs inversus or interruption of
and the right atrium is closed using a patch from the inferior vena cava, the operative technique has
inside the right atrium, employing a separate inci- to be modified according to the specific anatomy.
sion in the right atrium. Consequently, the blood In patients who were previously not operated, and
flow from the superior vena cava into the pulmo- in patients in whom only stage I palliation was
nary arteries is separated from the blood flow performed, the extracardiac total cavopulmonary
from the inferior vena cava and the coronary sinus anastomosis is preceded by bidirectional superior
into the ventricle. Cardiopulmonary bypass is dis- cavopulmonary anastomosis. After previous
continued in the same way as during the bidirec- hemi-Fontan surgery, completion of the Fontan
tional superior cavopulmonary anastomosis. circulation is preferably accomplished by an intra-
cardiac total cavopulmonary connection.
Following midline sternotomy, cardiopulmo-
13.7.3 Stage III Palliation: Completion nary bypass is initiated after cannulation of the
of Fontan Circulation aorta and the superior and the inferior vena cava.
Inferior vena cava should be cannulated as d istally
13 In the present time, most of the candidates for the as possible, immediately superior to the dia-
Fontan operation have undergone bidirectional phragm. It is advisable to use a right-angled can-
superior cavopulmonary anastomosis or hemi- nula for the venous drainage. The right pulmonary
Fontan operation. The purpose of the Fontan artery is dissected from the right upper lobe
operation in this stage III setting is to complete branch across the midline posterior to aorta.
the separation of pulmonary and systemic circula- Superior vena cava and the superior cavopulmo-
tion by diverting the blood from the inferior vena nary connection are dissected. The adhesions
cava into the pulmonary arteries. Therefore, the around the right atrium and the right pulmonary
term ‘completion Fontan’ is also applied in this veins have to be thoroughly dissected, in order to
situation. Following the operation, only the pul- achieve correct position of the conduit.
monary veins and the blood from the coronary The right pulmonary artery is cross-clamped
sinus drain in the common atrium and therefore immediately next to the right upper lobe artery,
in the functional single ventricle. Therefore, sepa- and the main pulmonary artery is cross-clamped
ration of the pulmonary and systemic circulation on either side of the ascending aorta. The superior
is almost complete through stage III palliation. vena cava is then snared and the pulmonary artery
The correction of any additional or residual is therefore completely isolated from blood flow.
heart defect, such as pulmonary artery stenosis, Alternatively, in order to avoid the dissection of
valve regurgitation or stenosis in systemic out- the posterior wall of the aorta, a suction vent may
flow tract, should be performed at this time. be placed in the right pulmonary artery towards
Two surgical techniques are used for comple- the left side of the body instead of using a cross-
tion of Fontan circulation: connection of the clamp. This might help reduce the risk of severe
inferior vena cava with the pulmonary arteries bleeding complications in patients with previous
using an extracardiac conduit (‘extracardiac aortic arch reconstruction.
Chapter 13 · Definite Palliation of Functional Single Ventricle
445 13
b
a
c d
e
f
..Fig. 13.12 a–h Extracardiac total cavopulmonary connection. Superior cavopulmonary anastomosis was
performed previously a. The right pulmonary artery is obliquely clamped on the medial side of the superior
cavopulmonary anastomosis, so that blood can still flow from the superior vena cava into the pulmonary artery. The
second clamp can be positioned on the left side from the ascending aorta. In this way, the central pulmonary artery is
isolated and can be opened on the inferior wall b. The anastomosis is carried out with a non-resorbable monofilament
suture 5/0 or 6/0 c. The conduit is then cross-clamped and the snares and clamps are removed from the pulmonary
arteries and the superior vena cava d. Inferior vena cava is cannulated as distally as possible, immediately superior to
the diaphragm. It is advisable to use a right-angled cannula for the venous drainage e. A vascular clamp is then applied
immediately above the cavoatrial junction and the inferior vena cava is snared. The inferior vena cava is divided from
the right atrium, and the cardiac end is oversewn using non-resorbable monofilament 4/0 suture f. After cutting the
conduit in the appropriate length, the anastomosis to the inferior vena cava is carried out with a running
non-resorbable monofilament 5/0 suture g, h
446 R. Lange and J. Hörer
g h
An incision is made on the inferior wall of the peutic procedures. It also provides a possibility of
pulmonary artery which corresponds to the length volume substitution and catecholamine therapy
of an obliquely cut conduit. The conduit should have without primary passage through the lungs.
a diameter of at least 18 mm. A conduit of this size
may be implanted in children presenting with a 13.7.3.2 Intracardiac Total
body weight of at least 10 kg. The anastomosis is per- Cavopulmonary Connection
formed with a running non-resorbable monofila- Intracardiac total cavopulmonary connection can
ment 5/0 or 6/0 suture. The conduit is then be planned after stage II bidirectional Glenn or
cross-clamped and the snares and clamps are preferably after hemi-Fontan surgery. Following
removed from the pulmonary arteries and the supe- midline sternotomy, cardiopulmonary bypass is
rior vena cava. A vascular clamp is then applied initiated after cannulation of the aorta and the
immediately above the cavoatrial junction and the superior and the inferior vena cava (. Fig. 13.13).
13 inferior vena cava is snared. The inferior vena cava is The aorta is then cross-clamped and cardioplegic
divided from the right atrium, and the cardiac end is solution is infused in the aortic root in moderate
oversewn using non-resorbable monofilament 4/0 systemic hypothermia. Alternatively, the operation
suture. After cutting the conduit in the appropriate is performed in hypothermic circulatory arrest.
length, the anastomosis to the inferior vena cava is All systemic-to-pulmonary shunts are divided
carried out with a running non-resorbable monofil- and the pulmonary arteries are dissected until the
ament 5/0 suture. Alternative techniques, described upper lobe arteries. If there is a connection
in 7 Sects. 7.4 and 7.5, can be used to perform this between the ventricle and the pulmonary artery, it
operation without cardiopulmonary bypass. should be closed at this point. In these cases, the
Cardiopulmonary bypass is discontinued while main pulmonary artery is then divided close to
measuring the pressure in the common atrium and the pulmonary valve, and both ends are oversewn
the superior and inferior venae cavae and determin- taking the pulmonary valve into the proximal
ing cardiac filling pressure, prepulmonary pressure suture line. An incision is made in the anterior
and the transpulmonary gradient. A transpulmo- wall of the atrium, leaving enough of atrial tissue
nary gradient of less than 8 mmHg and a pressure of laterally for the formation of the lateral tunnel.
16 mmHg in both vena cava are ideal. Pressures up The superior vena cava is then snared, and a
to 20 mmHg in the cava are acceptable. The blood suction vent is placed in the pulmonary artery
pressure should be the same in the superior and the through the right atrial appendage after the
inferior vena cava. Placement of a temporary trans- patch, which was placed between the superior
cutaneous line in the common atrium (functional vena cava and the atrium during the hemi-Fon-
‘left atrial’ line) should be considered to monitor tan operation is removed. Prior to the creation of
ventricular preload and transpulmonary gradient. the lateral tunnel, the lateral wall of the atrium is
This can be helpful for both diagnostic and thera- freed from any thick trabeculas. In cases where
Chapter 13 · Definite Palliation of Functional Single Ventricle
447 13
a b
..Fig. 13.13 Intracardiac total cavopulmonary connection. The hemi-Fontan operation was performed previously.
Following midline sternotomy, cardiopulmonary bypass is initiated after cannulation of the aorta and the superior and
the inferior vena cava. The aorta is then cross-clamped and cardioplegic solution is infused in the aortic root in
moderate systemic hypothermia. The pulmonary arteries are dissected until the upper lobe arteries. An incision is made
in the anterior wall of the atrium, leaving enough of atrial tissue laterally for the formation of the lateral tunnel a. The
superior vena cava is then snared, and a suction vent is placed in the pulmonary artery through the right atrial
appendage. The patch, which was placed between the superior vena cava and the atrium during hemi-Fontan
operation, is removed. Prior to the creation of the lateral tunnel, the lateral wall of the atrium is freed from any thick
trabeculas. A polytetrafluorethylene patch is cut into an appropriate shape and implanted. The continuous suture is
started between the coronary sinus and the junction of the inferior vena cava and extended medially along the atrial
septal defect towards the medial aspect of the junction of the superior vena cava. In the lateral direction, the suture is
placed anterior to the junction of the inferior vena cava and extended along the lateral atrial wall, ending superior and
anterior to the junction of the superior vena cava b
the inflow into the dominant ventricle mainly Cardiopulmonary bypass is discontinued in
flows through a right atrioventricular valve, it is the same way as during the extracardiac total
important to check for unobstructed pulmonary cavopulmonary anastomosis.
venous and coronary sinus blood flow. Coronary
sinus unroofing may be performed in order to
achieve this. A polytetrafluorethylene patch is 13.7.4 Special Surgical Techniques
cut into an appropriate shape and implanted. To
cut a patch out of a large PTFE, prosthesis may
be advantageous for its preshaped curvature. The 13.7.4.1 ther Techniques of
O
continuous suture is started between the coro- Systemic-to-Pulmonary
nary sinus and the junction of the inferior vena Artery Shunt
cava and extended medially along the atrial septal Nowadays, a direct anastomosis of the ascending
defect towards the medial aspect of the junction aorta and the right pulmonary artery (Waterson
of the superior vena cava. In the lateral direction, shunt) and a direct anastomosis of the descending
the suture is placed anterior to the junction of the aorta and the left pulmonary artery (Potts shunt) are
inferior vena cava and extended along the lateral obsolete. Both of them are characterized by uncon-
atrial wall, ending superior and anterior to the trollable shunt flow. In addition, closure of Waterson
junction of the superior vena cava. The atriotomy and Potts shunts during the next step operations is
is then closed using a running suture. When cho- more difficult and rather complex compared to clo-
sen appropriately, the atriotomy could be closed sure of a modified Blalock- Taussig or a central
with the anterior patch suture limiting the num- shunt. The classic Blalock-Taussig shunt, with end-
ber of atrial suture lines. to-end anastomosis of the subclavian artery and the
448 R. Lange and J. Hörer
ipsilateral pulmonary artery, is rarely performed, in artery to the ascending aorta. Using the following
order to preserve upper extremity circulation. technique might help avoid stenosis or pulmo-
However, it may still be an option in special circum- nary valve insufficiency: an incision is made in
stances in very small patients (less than 2 kg). the wall of the ascending aorta which is adjacent
to the main pulmonary artery. An end-to-side
13.7.4.2 Anastomosis of the Proximal anastomosis is then made between the main pul-
Pulmonary Artery and the monary artery and the incised part of the ascend-
Ascending Aorta, with Aortic ing aorta with a resorbable monofilament 7/0
Arch Reconstruction suture. The rest of the defect is closed with a patch
The pericardium is opened following midline with a non-resorbable monofilament 7/0 suture.
sternotomy and subtotal thymectomy. The aortic In the presence of a restrictive interatrial com-
cannula is placed as far distal as possible. In munication, an atrioseptectomy should be per-
cases of interrupted aortic arch, the arterial duct formed through an incision in the right atrium.
needs to be cannulated via the pulmonary artery As soon as the atriotomy is closed, the cardiopul-
as well. Once the cannula is introduced into the monary bypass may be restarted, and the patient
duct, the duct can be snared around it. A single may slowly be rewarmed.
venous cannula is inserted through the right Finally, the pulmonary circulation must be
atrial appendage. After institution of cardiopul- restored. In most cases, the distal part of the main
monary bypass, the patient’s core temperature is pulmonary artery has to be closed with a patch in
reduced to 18 °C. Temperatures should be mea- order to avoid stenosis. The distal anastomosis of
sured in the rectum or urinary bladder and in the modified Blalock-Taussig or central shunt
esophagus or nasopharynx. During this cooling may be positioned in the middle of this patch. In
phase, the ascending aorta, the aortic arch, the the literature, these techniques of dealing with a
supraaortic branches and the descending aorta subaortic stenosis are connected to Damus, Kaye,
distal to the isthmus are exposed. The main, Stansel and Lamberti (Damus 1975; Kaye 1975;
right and left pulmonary arteries and the arterial Stansel 1975; Lamberti et al. 1991).
duct are dissected as well. When the arterial can-
nula is placed in aorta, the arterial duct may be 13.7.4.3 Modified Norwood Operation
occluded. If it was only possible to cannulate the The anastomosis of the proximal pulmonary artery
13 main pulmonary artery in interrupted arch, the and the ascending aorta, with aortic arch recon-
right and the left pulmonary artery should be struction procedure (7 Sect. 13.7.4.2), follows the
temporarily occluded at this point. Obviously, in same principles as the Norwood operation for
this situation, the duct needs to be open for hypoplastic left heart syndrome. It can therefore be
initial perfusion. referred to as a modified Norwood operation.
When the desired temperature is reached, the However, the position and the size of the great arter-
aorta is then cross-clamped, and cardioplegic solu- ies may differ significantly from the typical patient
tion is infused in the aortic root. The circulation is presenting with hypoplastic left heart syndrome.
then arrested and the arterial cannula removed. The The modified Norwood operation is performed in
arterial duct is transected, and the pulmonary part cases of tricuspid atresia with ventriculoarterial dis-
is oversewn. All ductal tissue is resected together cordance or double-inlet left ventricle with ventric-
with the aortic isthmus completely. Alternatively, ular L-loop and ventriculoarterial discordance. The
all ductal tissue is resected from the aortic isthmus, variable morphology must be considered when
and the continuity of the aorta is left in place. An constructing the neoaortic arch. The dimension and
incision is made in the inner curvature of the aortic orientation of the patch, used for reconstruction of
arch towards the ascending aorta and if necessary the aortic arch, must be adjusted according to the
towards the descending aorta. When the continuity specific anatomy and position of the great arteries.
of the distal aorta was interrupted, the distal poste-
rior wall is connected with the descending aorta 13.7.4.4 Resection of Subaortic Stenosis
with a resorbable monofilament 7/0 suture. The technique to resect a subaortic stenosis (SAS)
The pulmonary artery is divided immediately in patients with univentricular circulation is simi-
above the sinotubular junction. Many techniques lar for very different anatomic settings, as the
are used for connecting the main pulmonary point of obstruction always is the interventricular
Chapter 13 · Definite Palliation of Functional Single Ventricle
449 13
connection rather than a typical SAS in otherwise anastomosis is performed (Lamberti et al. 1990).
normal biventricular hearts. This is true for tri- A unilateral left cavopulmonary anastomosis in
cuspid atresia, ventriculoarterial discordance and the cases of situs solitus frequently requires car-
a restrictive bulboventricular foramen. The same diopulmonary bypass since manipulation of the
technique can be applied in cases of functional heart for exposure leads to circulatory instability.
univentricular heart with univentricular atrioven-
tricular connection, in which the aorta rises from 13.7.4.6 Fenestration of Total
the hypoplastic chamber, in cases of double-inlet Cavopulmonary Connection
left ventricle with ventriculoarterial discordance The surgical technique is different in cases of prior
and in cases of mitral atresia with concordant intracardiac versus prior extracardiac total cavopul-
atrioventricular connection. monary connections. Fenestration may be indicated
The chest is entered through midline sternot- in cases of high central venous pressure and low fill-
omy, taking care of the aorta which lies directly ing pressure of the heart following total cavopulmo-
behind the sternum due to its anterior placement. nary connection. In these cases, the fenestration can
The cardiopulmonary bypass is initiated after aor- usually be performed without cardiopulmonary
tic and bicaval cannulation. The aorta is then bypass. One clamp is placed on the atrium and
cross-clamped, and cardioplegic solution is another on the adjacent part of the extracardiac
infused in the aortic root. The bulboventricular conduit. A defect is made in the conduit, a larger
foramen is exposed through an incision in the out- part of the atrium is excised and the two defects are
flow chamber (Cheung et al. 1990). Alternatively, a connected. In order to prevent stenosis, the suture
transatrial or transaortic approach may be consid- line must be placed at the ridge of the defect in the
ered, keeping in mind that the small size of the atrium, and with a distance away from the ridge of
aortic valve can compromise a good exposure the defect in the conduit, similar to the principle of
(Newfeld and Nikaidoh 1987; Smolinsky et al. ‘sutureless’ pulmonary vein repair.
1988). The conduction system is on the posterior- The simplest technique to fenestrate a lateral
inferior ridge of the defect. This is also true for the tunnel is to perforate the intraatrial patch. The
double-inlet left ventricle with ventriculoarterial size of the fenestration should be 4 mm in patients
discordance. Therefore, the defect can be enlarged weighting less than 12 kg, 5 mm in the patients
through an incision in the anterior superior ridge. between 12 and 30 kg and 6 mm in patients over
Subvalvular muscle bundles should be resected, 30 kg (Kopf et al. 1992). Alternative techniques
taking care of the aortic valve. The incision in the include performing a fenestration in a way that
hypoplastic chamber is then closed with a patch. the opening can be controlled and later transcuta-
neously closed (Laks et al. 1991).
13.7.4.5 Bidirectional Superior
Cavopulmonary Anastomosis 13.7.4.7 xtracardiac Total
E
Without Cardiopulmonary Cavopulmonary Anastomosis
Bypass Without Cardiopulmonary
Bidirectional superior cavopulmonary connec- Bypass, in the Presence
tion can be performed without cardiopulmonary of Superior Cavopulmonary
bypass in the presence of additional pulmonary Anastomosis
artery blood source. When only one superior It is not necessary to cannulate the superior vena
vena cava is present, the venous drainage of the cava when the extracardiac total cavopulmonary
upper body through a cannula is essential when anastomosis is performed without cardiopulmo-
the venous cross-clamp time is longer than nary bypass. The right pulmonary artery is
20 min. In the presence of bilateral superior vena obliquely cross-clamped medially from the supe-
cava, the venous drainage is usually not necessary. rior vena cava, so that the blood flow from the
In these cases, the anastomosis is first done on the superior vena cava in the right pulmonary artery
opposite side of the systemic-to-pulmonary artery remains unobstructed. The left pulmonary artery
shunt. During this time, the lung perfusion is can be cross-clamped on the left side of the ascend-
ensured over the shunt. Following the first anasto- ing aorta. The main pulmonary artery is com-
mosis, which provides a new path of lung perfu- pletely isolated, and its inferior wall can be incised
sion, the shunt may be resected, and the second for the anastomosis with the extracardiac conduit.
450 R. Lange and J. Hörer
Prior to completing the anastomosis between ‘One and a half ventricle correction’ is a third
the conduit and the inferior vena cava, the venous alternative to Fontan operation, and biventricular
blood from the lower body must be drained. The operation in patients with hypoplastic right ventri-
inferior vena cava is cannulated immediately above cle, whose volume is less than 30 % of the normal
the diaphragm, the second cannula is placed in the (Muster et al. 1993; Hanley 1999). There are how-
right atrium and the two cannulas are connected ever no clear criteria to decide between the different
making sure no air is trapped in the system (Uemura strategies on an individual patient basis (DeLeon
et al. 1998). In the presence of collateral flow et al. 1989). Some reports present good results of the
between the superior and the inferior vena cava biventricular repair (Ebert 1984); others report bet-
through the azygos vein, the anastomosis can be ter results following univentricular palliation (Delius
performed with cross-clamping of the inferior vena et al. 1996; Hraska et al. 2005). Long-term results are
cava, without connecting it to the atrium (Shiraishi still pending in both patient collectives, which, how-
et al. 2005; Shinkawa et al. 2011). It is important to ever, are always difficult to exactly compare.
avoid even temporary obstruction of the liver veins.
13.7.5.2 Pro and Contra: Additional
Pulmonary Blood Source
13.7.5 Controversial Treatment Following Bidirectional
Strategies Superior Cavopulmonary
Connection
Additional pulmonary blood flow besides the
13.7.5.1 Fontan Operation Versus superior cavopulmonary anastomosis, either in
Septation Operation the form of a pre-existing shunt or an open pul-
In some cases where the underlying anatomy may monary valve, has both advantages and disad-
make the biventricular correction feasible, it vantages. One advantage is improvement of the
might however be advisable to choose the univen- systemic oxygen saturation (Webber et al.
tricular palliation. This is the case when an 1995). In addition, it may promote growth of
extremely complex operation is necessary to the pulmonary arteries. This is very important
achieve biventricular correction and when it is not because growth of the pulmonary arteries is not
certain if the hypoplastic ventricle will be able to proportional to somatic growth in patients with
support the systemic circulation. In the following
13 circumstances, the univentricular palliation might
functional single ventricle (Mendelsohn et al.
1994; Slavik et al. 1995; Tatum et al. 2006).
be preferred over the biventricular correction: However, even additional pulmonary blood
55 Unbalanced atrioventricular septal defect flow following partial cavopulmonary connec-
55 Moderate hypoplasia of the right ventricle tion does not lead to normal development of
55 Moderate hypoplasia of the left ventricle the pulmonary arteries (Berdat et al. 2004;
55 Double-outlet right ventricle combined with Yoshida et al. 2005).
non-committed ventricular septal defect Another advantage is that the lungs will be
55 Tricuspid atresia with ventricular septal supplied with blood containing angiotensin
defect and moderate hypoplasia of the right inhibitor of the liver (Clement et al. 1999). The
ventricle formation of arteriovenous fistulas can be pro-
55 Pulmonary atresia with ventricular septal moted in patients without an additional pulmo-
defect, accompanied by moderate hypoplasia nary blood flow due to absence of endostatin and
of the right ventricle with a moderately angiostatin in the pulmonary vessels. The right-
reduced function to-left shunt through the fistulas leads to a
55 Ebstein anomaly with moderate hypoplasia decrease in the systemic saturation. Arteriovenous
and moderately reduced function of the right fistulas are found in the ipsilateral lung of patients
ventricle with unilateral Glenn anastomosis (McFaul et al.
55 Significant straddling of one of the 1977) and in both lungs of patients with bidirec-
atrioventricular valves in the presence of tional partial cavopulmonary anastomosis
ventriculoarterial concordance or (Srivastava et al. 1995). Even in patients with azy-
discordance, ventricular septal defect and gos continuity, the arteriovenous fistulas can be
pulmonary stenosis found following the bidirectional cavopulmonary
Chapter 13 · Definite Palliation of Functional Single Ventricle
451 13
connection—Kawashima operation (Matsuda the patient and the preference of the surgeon. The
et al. 1986). The formation of these fistulas may anastomosis can usually be performed without
be, however, reversible after completion of the cardiopulmonary bypass with or without place-
Fontan circulation (Knight and Mee 1995; ment of a shunt between the superior vena cava
Srivastava et al. 1995; Praus et al. 2008). and the right atrium (Murthy et al. 1999).
The disadvantage of the increased lung perfusion Cardiopulmonary bypass is necessary when the
in patients with an additional pulmonary blood flow pulmonary circulation is exclusively shunt
is volume overload of the functional single ventricle. dependent and the shunt has to be removed for
The aim of early staging by partial cavopulmonary the superior cavopulmonary anastomosis. This is
connection is normalization of the workload of the usually the case with a right superior vena cava
functional single ventricle (Allgood et al. 1994; and a right- sided modified Blalock-Taussig
Jacobs et al. 1996a, b) and protection of the cardiac shunt.
muscle in the long term (Schwartz et al. 1996). In Off pump surgery avoids the disadvantage of
addition, higher pressure in the pulmonary artery hemodilution. This might lead to lower early
can lead to an increase in vascular resistance in the mortality (Hussain et al. 2007). When the supe-
lungs. The increased pulmonary resistance was rec- rior cavopulmonary connection is performed
ognized as a risk factor for mortality after the Fontan using cardiopulmonary bypass, it is recom-
operation (Boruchow et al. 1970; Fontan et al. 1989; mended to perform modified ultrafiltration after
Bartmus et al. 1990; Driscoll et al. 1992; Knott-Craig discontinuing cardiopulmonary bypass. This pro-
et al. 1995). A comparative study of patients with and cedure will possibly reduce the creation of edema
without an additional pulmonary blood flow showed and pulmonary effusions.
better results for patients without an additional pul-
monary blood flow (Mainwaring et al. 1995). 13.7.5.4 Bidirectional Superior
The bidirectional partial cavopulmonary anas- Cavopulmonary Connection
tomosis with an additional pulmonary blood Versus Hemi-Fontan
source provides very poor results as a definitive Operation
palliation without Fontan completion (Gerelli The choice between a bidirectional superior cavo-
et al. 2012). These patients have a survival rate of pulmonary connection and a hemi-Fontan opera-
75 % in the first 5 years after the operation. A late tion depends on the technique of Fontan
Fontan operation due to cyanosis or functional completion. If the surgeon prefers to perform an
deterioration in these patients yields a periopera- extracardiac total cavopulmonary connection, the
tive mortality of more than 50 % (Yamada et al. bidirectional superior cavopulmonary connec-
2000). In most of these patients, elevated pulmo- tion should be performed as the second-stage
nary artery pressure is the reason for delayed operation. If the surgeon prefers to perform an
completion of the Fontan circulation and the intracardiac lateral tunnel, both the hemi-Fontan
related poor results. Gerelli et al. (2012) confirm operation and the bidirectional superior cavopul-
that bidirectional partial cavopulmonary anasto- monary connection are an adequate second-stage
mosis with an additional pulmonary blood source procedure.
fails as a strategy for definitive palliation. However, The largest disadvantage of hemi-Fontan
the authors observed that the additional blood operation is the fact that it is an intracardiac oper-
flow may delay the long-term deleterious conse- ation, which usually requires cardioplegia and
quences of Fontan circulation and that it does not cross-clamping of the aorta. Unlike the bidirec-
preclude a successful Fontan completion. tional superior cavopulmonary anastomosis, the
hemi-Fontan operation always requires cardio-
13.7.5.3 Pro and Contra: Bidirectional pulmonary bypass.
Superior Cavopulmonary
Connection 13.7.5.5 Pro and Contra: Fenestration
Without Cardiopulmonary of the Total Cavopulmonary
Bypass Connection
The decision to perform a bidirectional superior Performing an incomplete separation of the pul-
cavopulmonary connection with or without car- monary and the systemic circulation by creation
diopulmonary bypass is based on the anatomy of of a communication between the Fontan tunnel
452 R. Lange and J. Hörer
and the systemic atrium can have advantages in the extracardiac conduit than in the intracar-
certain situations. In the late 1980s, this concept diac tunnel (Lardo et al. 1999).
was used in the treatment of high-risk patients One of the disadvantages of an intracardiac
during conversion of a shunt-dependent tunnel is the inevitable cross-clamp time and
circulation into a Fontan circulation. In the fol- open-heart surgery. On the other hand, the extra-
lowing years, many publications suggested per- cardiac conduit can be placed on cardiopulmo-
forming fenestration in all patients undergoing nary bypass with beating heart, which prevents
Fontan operation (Cochrane et al. 1997; Gentles the potential deteriorating effect of cardioplegia
et al. 1997b; Bando et al. 2000; Gaynor et al. 2002; on the ventricular function (Schreiber et al. 2007).
Kumar et al. 2003; Gupta et al. 2004). However, However, a very low mortality rate after perform-
this topic is still part of controversial discussions. ing an intracardiac lateral tunnel in hypothermic
Fontan operation without routine Fenestration circulatory arrest was also reported (Jacobs et al.
can also be performed with low mortality and 2008).
morbidity (Thompson et al. 1999; Schreiber et al. The extracardiac total cavopulmonary con-
2007; Salazar et al. 2011). nection can be performed without cardiopulmo-
Fenestration of the total cavopulmonary anas- nary bypass (Uemura et al. 1998) avoiding the
tomosis should be considered in patients with temporary increase in pulmonary artery resis-
increased pulmonary resistance of 2–4 Wood tance through the effects of cardiopulmonary
units. The resulting right-to-left shunt in these bypass and hypothermia. Performing the opera-
circumstances provides the necessary preload of tion without cardiopulmonary bypass may result
the systemic ventricle and therefore prevents a in better postoperative hemodynamics, lower rate
postoperative low-output syndrome. In addition, of secondary fenestration and lower rate of throm-
it prevents increased blood pressure in the caval bosis (Petrossian et al. 2006). In addition, almost
veins and the pulmonary arteries. All this may no atrial manipulation is necessary during
result in fewer pulmonary effusions and a shorter implantation of the extracardiac conduit. This
hospital stay. However, it also leads to a reduced may result in lower rate of sinus node dysfunction
systemic oxygen saturation. Publications have (Petrossian et al. 2006; Lee et al. 2007), which
shown no significant differences in the early or could not principally be confirmed (Kumar et al.
late mortality. However, in the long term, fenes- 2003). The extracardiac total cavopulmonary con-
13 tration leads to a higher cardiac output and lower nection can also be performed in malpositioned
rate of arrhythmias (Ono et al. 2006). Atz et al. hearts with apicocaval juxtaposition (Sakurai
observed spontaneous closure of surgical fenes- et al. 2010).
trations in 40 % of the patients at 8 ± 3 years after Lack of growth potential is the main disadvan-
the Fontan operation (Atz et al. 2011). There is tage of the extracardiac conduit. This is however
evidence that subjects with a current fenestration not an argument against using the extracardiac
are taking more medications (Atz et al. 2011) and conduit or the reason to delay the completion of
have lower resting oxygen saturation (Atz et al. the Fontan circulation. An 18 mm conduit may be
2011; Imielski et al. 2013). Patients with fenestra- implanted in children with 10 kg of body weight
tion require platelet aggregation inhibitors or (Schreiber et al. 2007), without the need for
anticoagulants in order to prevent thromboem- replacement in the future. The growth potential of
bolic events. the conduit may be preserved, by using patient’s
atrium for the extracardiac completion of the
13.7.5.6 Pro and Contra: Intracardiac Fontan circulation (Lemler et al. 2006). However,
or Extracardiac Total there is no clear answer which of the two tech-
Cavopulmonary Connection niques of performing the total cavopulmonary
There are still no long-term data, which show connection is superior. Both techniques are char-
an advantage of either intracardiac or extracar- acterized by low mortality and complication rates
diac total cavopulmonary connection. Both (Meyer et al. 2006; Fiore et al. 2007; Schreiber
techniques provide a laminar flow in the tun- et al. 2007; Jacobs et al. 2008). Multicenter data
nel, which is superior to an atriopulmonary suggest that in current use, the lateral atrial tunnel
connection (Alphonso et al. 2005). Fontan may be associated with superior early out-
Theoretically, the flow dynamics are better in comes (Stewart et al. 2012).
Chapter 13 · Definite Palliation of Functional Single Ventricle
453 13
13.8 Results 13.8.1.2 Stage II Palliation
Early mortality following the bidirectional superior
13.8.1 Mortality cavopulmonary connection or the hemi- Fontan
operation ranges between 0 and 10 % (Lamberti
et al. 1990; Chang et al. 1993; Reddy et al. 1995;
13.8.1.1 Stage I Palliation Bradley et al. 1996; Hussain et al. 2007). Associated
Early mortality of patients with tricuspid atresia, anomalies and the type of concomitant procedures
following a systemic-to-pulmonary shunt opera- determine outcome. Risk factors are increased pul-
tion, is similar to the mortality of patients with monary artery pressure, dominant right ventricle,
tetralogy of Fallot (Kouchoukos et al. 2003a, b). total anomalous pulmonary venous return, hetero-
Early mortality of patients following a modified taxy syndrome, atrioventricular valve insufficiency
Blalock-Taussig shunt operation for any indica- and very young age (Alejos et al. 1995; Reddy et al.
tion declined during the last 60 years from 16 % 1995; Scheurer et al. 2007; Nakata et al. 2010).
in the first half of this time period to 9 % in the However, the bidirectional superior cavopulmonary
second half (Williams et al. 2007). This signifi- connection has also been performed in patients as
cant early mortality rate is accompanied by a young as 2 months of age, with early and late mor-
high interstage mortality, which ranges between tality equivalent to that seen in older patients
14 and 26 % (Fermanis et al. 1992; Fenton et al. (Petrucci et al. 2010). Avoiding cardiopulmonary
2003; Li et al. 2007). Shunt thrombosis is the bypass might have a positive influence on early sur-
main cause of death in the interstage period vival (Hussain et al. 2007). Bidirectional cavopul-
(Cholette et al. 2007). monary anastomosis with additional source of
In older publications, the early mortality of pulmonary blood flow, as a definitive palliation
patients after pulmonary artery banding ranges without a planned completion of Fontan circulation,
between 25 and 35 % (LeBlanc et al. 1987; has an unfavorable prognosis (Yamada et al. 2000).
Horowitz et al. 1989). Patients with non-restric-
tive pulmonary blood flow, who required pulmo- 13.8.1.3 Stage III Palliation
nary artery banding, often present with stenosis Hospital mortality decreased from >20 % in the
in the systemic outflow tract. The obstruction first Fontan operations to <5 % in the present time
may be localized at the level of the ventricle or in (Annecchino et al. 1988; Fontan et al. 1989, 1990;
the aortic arch. Obstruction at both localizations Bartmus et al. 1990; Myers et al. 1990; Stein et al.
increases mortality (Lee et al. 2003). Patients 1991; Driscoll et al. 1992; Mavroudis et al. 1992;
who required pulmonary artery banding, with- Mayer et al. 1992; Day et al. 1994; Jacobs et al.
out aortic arch enlargement, have a lower early 1995b; Knott-Craig et al. 1995; Cetta et al. 1996;
mortality (<5 %) (Cleveland et al. 1984; Stefanelli Laschinger et al. 1996; Cochrane et al. 1997;
et al. 1984). Iemura et al. 1997; Petrossian et al. 2006;
Early mortality of 35 % is reported for patients d’Udekem et al. 2007; Hosein et al. 2007; Ocello
after connection of the proximal pulmonary et al. 2007; Schreiber et al. 2007). In the recent
artery to ascending aorta and concomitant aortic years, early mortality is reported to be fewer than
arch reconstruction (Rychik et al. 1991). With 2 % (Meyer et al. 2006; Nakano et al. 2007;
increasing experience with the Norwood proce- Schreiber et al. 2007; Jacobs et al. 2008).
dure in patients with hypoplastic left heart syn- Multivariate analysis of potential risk factors
drome, a modified Norwood operation can be for early failure of Fontan circulation could iden-
performed in patients with tricuspid atresia and tify the following factors (Gentles et al. 1997b;
ventriculoarterial discordance with improved Wallace et al. 2011):
results. Due to heterogeneity of the underlying 55 Preoperative pulmonary artery pressure
anatomy, it is hard to estimate the results after >19 mmHg
resection of subaortic stenosis. Early mortality of 55 Younger age at the time of the operation
11 % was reported for a small collective (Cheung 55 Weight-for-age z-score less than -2
et al. 1990). It is sometimes difficult to assess 55 Heterotaxy syndrome
whether a widening of bulboventricular foramen 55 Tricuspid valve as the dominant
is sufficient or a modified Norwood operation has atrioventricular valve
to be performed. 55 Stenosis of the pulmonary arteries
454 R. Lange and J. Hörer
55 Fontan modifications with atriopulmonary with Fontan circulation is practically the same as
connection through an atrial appendage the one in healthy children (Peterson et al. 1984),
55 Total cavopulmonary connection without most studies showed that they have worse hemo-
fenestration. dynamic parameters (Annecchino et al. 1988;
Chin et al. 1993; Harrison et al. 1995). Only few
Increased pulmonary artery pressure and patients are capable of reaching expected values
heterotaxy syndrome were confirmed as risk in stress tests—their heart rate rises, but the stroke
factors by many authors (Kirklin et al. 1986; volume remains the same (Shachar et al. 1982).
Fontan et al. 1989; Bartmus et al. 1990; Myers NYHA classification status of the patients
et al. 1990; Driscoll et al. 1992; Pizarro et al. deteriorates in time. One year after the operation,
2006). Since the introduction of staged pallia- 90 % of the patients are in NYHA I class, while
tion, younger age and shorter time between 20 years afterwards only 56 % are in NYHA I class
stage II and stage III palliation are connected to (Fontan et al. 1990; Gentles et al. 1997a).
lower rate of complications and lower early mor- Children with functional single ventricle do not
tality (Kirklin et al. 1986; Francois et al. 2005; have normal body growth (Ono et al. 2007; Vogt
Schreiber et al. 2007). et al. 2007). Early volume unloading by partial cavo-
The long-term results show the palliative char- pulmonary anastomosis has a positive effect on body
acter of the Fontan circulation. Follow-up of growth (Ono et al. 2007; Vogt et al. 2007) and the
patients, who were operated in 70s and 80s show functional status of the patients (Mahle et al. 1999).
70 % survival after 5 years, 65 % after 10 years and Patients who have a fenestrated Fontan tunnel have a
50 % after 15 years (de Brux et al. 1983; Williams higher cardiac index than the patients in whom the
et al. 1984; Fontan et al. 1989; Tam et al. 1989; pulmonary circulation is completely separated from
Earing et al. 2005). the systemic circulation (Ono et al. 2006).
Risk factors for late mortality are still not clearly
identified. The results of older studies with long fol-
low-up cannot be applied to the current patients. 13.8.3 Neurological Status
Improvement of the operative technique and the
introduction of the staged approach, leading to an Neurological development of the children with
early unloading of the ventricle, are expected to functional single ventricle is the same as the
13 yield better long-term results. Survival of patients development of healthy children (Uzark et al.
after an ‘ideal’ Fontan operation was calculated 1998; Goldberg et al. 2000). Their school perfor-
through a mathematical model, eliminating the mance is also normal (Mitchell et al. 2006). The
‘avoidable’ risk factors (Fontan et al. 1990). The pre- incidence of apoplexy is 3–9 % in long-term fol-
dicted survival was calculated to be 73 % after low-up (du Plessis et al. 1995; Barker et al. 2005;
15 years, with low constant mortality risk afterward. Chowdhury et al. 2005). The risk of cerebrovascu-
Older age at the time of Fontan operation was the lar incidents is significantly reduced by acetylsali-
only recognized risk factor for late mortality. cylic acid therapy. The presence of the ligated
However, the modern concept of staged therapy was pulmonary artery stump may place patients at
not followed in this patient collective. Ten-year sur- risk for embolic stroke. The surgical approaches
vival rates of patients, who were treated according to should avoid leaving any pulmonary pouch when
the staged concept and received total cavopulmonary ligating or disconnecting the pulmonary artery
connection, are reported to be over 90 % (Driscoll (Koide et al. 1999; Oski et al. 1996).
2007; Lee et al. 2007; Nakano et al. 2007).
Physical endurance improves significantly after Thrombosis can occur in the right atrium, Fontan
the Fontan operation. The cyanosis is usually tunnel, extracardiac prosthesis, in the venae cavae or
resolved after the Fontan operation in patients in in the pulmonary arteries. Half of the cases of throm-
whom no fenestration was performed (Ovroutski bosis occur immediately after the operation, while
et al. 2003). Although the cardiac index of patients the other half occurs in the long-term follow-up
Chapter 13 · Definite Palliation of Functional Single Ventricle
455 13
(Dobell et al. 1986; Putnam et al. 1988). Embolization Petrossian et al. 2006; Lee et al. 2007). Surgical
of the thrombotic material can lead to serious com- treatment of rhythm disturbances include
plications and might result in death of the patient MAZE operation and its variations and a con-
(Cromme-Dijkhuis et al. 1990). The formation of version of an atriopulmonary Fontan modifica-
thrombi is multifactorial. The aggravating factors are tion into either intra- or extracardiac
(Jacobs and Pourmoghadam 2007): cavopulmonary connection (Deal et al. 2007).
55 Stasis in venous circulation
55 Synthetic material
55 Atrial arrhythmias 13.8.6 Protein-Losing Enteropathy
55 Prothrombotic state
Protein-losing enteropathy is characterized by
Prophylactic therapy with vitamin K antago- low serum albumin concentration, it results in
nists or antiplatelet aggregation therapy can pleural effusions and ascites. The underlying
reduce the incidence of thromboembolic events. causes are not completely understood. High dia-
Special care should be taken to treat the atrial stolic atrial pressure in the systemic atrium, path-
arrhythmias and a potential prothrombotic status. ological resistance in the mesenterial vessels and
Optimal flow dynamics in the venous pathway inflammatory reactions are discussed as possible
should be achieved (Kaulitz et al. 2005). causes (Hess et al. 1984; Ostrow et al. 2006). The
prevalence reaches 13 % at 10 years after Fontan
operation (Feldt et al. 1996; Mertens et al. 1998).
13.8.5 Rhythm Disturbances Only 50 % of patients are alive 5 years after this
complication was first diagnosed. In patients with
Tachycardic as well as bradycardic arrhythmias sinus node dysfunction, an improvement may be
may occur in patients presenting with functional achieved through pacemaker stimulation (Cohen
single ventricle after Fontan-type palliation. The et al. 2001). In certain cases, fenestration of the
following factors are potential causes for develop- Fontan tunnel might reduce pleural effusions and
ment of rhythm disturbances: ascites (Jacobs et al. 1996a; Warnes et al. 1996).
55 Iatrogenic injury of the sinus node or its Other surgical options include Fontan conversion
blood supply and heart transplantation. The results of these
55 Long incisions and suture lines in atrium operations are discussed in the following para-
55 Effect of high pressure on the atrial tissue graph.
55 Congenital anomalies of the conductive system
55 Consequence of a valve insufficiency
13.8.7 Reoperations
The incidence of arrhythmia in the long term is
reported to be more than 50 % (Deal et al. 2007). A stenoses in the venous pathway is the most
The following parameters were identified as risk common indication for reoperations following
factors for (Durongpisitkul et al. 1998; Alphonso Fontan palliation (Girod et al. 1987; Mavroudis
et al. 2005; Chowdhury et al. 2005; Ono et al. 2006): et al. 2005). Freedom of obstruction in venous
55 Heterotaxy syndrome pathways, 15 years following the operation, was
55 Poor ventricular function 50 % in a study with heterogeneous operation
55 Anomalies of the pulmonary veins techniques (Fernandez et al. 1989). The incidence
55 Glenn anastomosis of reoperations is 2 % after direct atriopulmonary
55 Insufficiency of an atrioventricular valve connection and 13 % after interpositions of a con-
55 Preoperative atrial fibrillation duit (Fontan et al. 1989). Hospital mortality fol-
55 Atriopulmonary Fontan modification lowing these reoperations is reported to be 24 %
55 Older age at the time of Fontan operation in older series (Fontan et al. 1989). The incidence
of stenoses in venous pathways might decrease
The influence of the type of total cavopulmo- after the introduction of the lateral tunnel and the
nary connection (intracardiac vs. extracardiac) extracardiac total cavopulmonary connection.
on the dysfunction of the sinus node is dis- However, there is limited data available beyond
cussed controversial (Kumar et al. 2003; 10 years of follow-up in these patients.
456 R. Lange and J. Hörer
The following complications following an atri- is 68 % after heart transplantation (Jayakumar
opulmonary Fontan operation may be indications et al. 2004). This survival is lower than the sur-
for Fontan conversion: vival of children with other congenital or acquired
55 Large right atrium with unfavorable flow heart defects (Bernstein et al. 2006). The func-
dynamics tional status of the patients improves significantly
55 Rhythm disturbances after the transplantation. The symptoms of
55 Thrombosis protein- losing enteropathy usually improve as
55 Stenoses in venous pathways well (Bernstein et al. 2006).
55 Protein-losing enteropathy
the single left ventricle after volume-unloading sur- Forrest P, Bini RM et al. (1987) Congenital absence of the pul-
gery. J Am Coll Cardiol 26(4):1008–1015 monic valve and tricuspid valve atresia with intact ven-
Doty DB, Schieken RM et al. (1979) Septation of the univen- tricular septum. Am J Cardiol 59(5):482–484
tricular heart. Transatrial approach. J Thorac Cardiovasc Francois K, Tamim M et al. (2005) Is morbidity influenced
Surg 78(3):423–430 by staging in the fontan palliation? A single center
Driscoll DJ (2007) Long-term results of the Fontan opera- review. Pediatr Cardiol 26(4):350–355
tion. Pediatr Cardiol 28(6):438–442 Franklin RC, Spiegelhalter DJ et al. (1991) Double-inlet ven-
Driscoll DJ, Offord KP et al. (1992) Five- to fifteen-year tricle presenting in infancy. I. Survival without definitive
follow-up after Fontan operation. Circulation 85(2): repair. J Thorac Cardiovasc Surg 101(5):767–776
469–496 Freedom RM, Benson LN et al. (1986) Subaortic stenosis,
du Plessis AJ, Chang AC et al. (1995) Cerebrovascular acci- the univentricular heart, and banding of the pulmo-
dents following the Fontan operation. Pediatr Neurol nary artery: an analysis of the courses of 43 patients
12(3):230–236 with univentricular heart palliated by pulmonary
Durongpisitkul K, Porter CJ et al. (1998) Predictors of artery banding. Circulation 73(4):758–764
early- and late-onset supraventricular tachyarrhyth- Gaynor JW, Collins MH et al. (1999) Long-term outcome of
mias after Fontan operation. Circulation 98(11): infants with single ventricle and total anomalous pul-
1099–1107 monary venous connection. J Thorac Cardiovasc Surg
Earing MG, Cetta F et al. (2005) Long-term results of the 117(3):506–513; discussion 513–514
Fontan operation for double-inlet left ventricle. Am Gaynor JW, Bridges ND et al. (2002) Predictors of outcome
J Cardiol 96(2):291–298 after the Fontan operation: is hypoplastic left heart
Ebert PA (1984) Staged partitioning of single ventricle. syndrome still a risk factor? J Thorac Cardiovasc Surg
J Thorac Cardiovasc Surg 88(6):908–913 123(2):237–245
Edie RN, Ellis K et al. (1973) Surgical repair of single ventri- Gentles TL, Gauvreau K et al. (1997a) Functional outcome
cle. J Thorac Cardiovasc Surg 66(3):350–360 after the Fontan operation: factors influencing late
Edwards JE (1960) Congenital malformations of the heart morbidity. J Thorac Cardiovasc Surg 114(3):392–403;
and great vessels. In: Gould SE (ed) Pathology of the discussion 404–405
heart. Charles C Thomas, Springfield Gentles TL, Mayer JE Jr et al. (1997b) Fontan operation in
Edwards JE, Burchell HB (1949) Congenital tricuspid five hundred consecutive patients: factors influencing
atresia: a classification. Med Clin North Am 33: early and late outcome. J Thorac Cardiovasc Surg
1117–1196 114(3):376–391
Erek E, Yalcinbas YK et al. (2006) Current management of Gerelli S, Boulitrop C et al. (2012) Bidirectional cavopulmo-
late failure after classic Fontan modifications: Fontan nary shunt with additional pulmonary blood flow: a
conversion. Thorac Cardiovasc Surg 54(1):21–25 failed or successful strategy? Eur J Cardiothorac Surg
Feldt RH, Driscoll DJ et al. (1996) Protein-losing enteropa- 42(3):513–519
thy after the Fontan operation. J Thorac Cardiovasc Girod DA, Fontan F et al. (1987) Long-term results after the
13 Surg 112(3):672–680 Fontan operation for tricuspid atresia. Circulation
Fenton KN, Siewers RD et al. (2003) Interim mortality in 75(3):605–610
infants with systemic-to-pulmonary artery shunts. Glenn WW (1958) Circulatory bypass of the right side of
Ann Thorac Surg 76(1):152–156; discussion 156–157 the heart. IV. Shunt between superior vena cava and
Fermanis GG, Ekangaki AK et al. (1992) Twelve year experi- distal right pulmonary artery; report of clinical appli-
ence with the modified Blalock-Taussig shunt in neo- cation. N Engl J Med 259(3):117–120
nates. Eur J Cardiothorac Surg 6(11):586–589 Goldberg CS, Schwartz EM et al. (2000) Neurodevelopmental
Fernandez G, Costa F et al. (1989) Prevalence of reopera- outcome of patients after the fontan operation: a com-
tion for pathway obstruction after Fontan operation. parison between children with hypoplastic left heart
Ann Thorac Surg 48(5):654–659 syndrome and other functional single ventricle
Fiore AC, Turrentine M et al. (2007) Fontan operation: a lesions. J Pediatr 137(5):646–652
comparison of lateral tunnel with extracardiac conduit. Gupta A, Daggett C et al. (2004) Risk factors for persistent
Ann Thorac Surg 83(2):622–629; discussion 629–630 pleural effusions after the extracardiac Fontan proce-
Fontan F, Baudet E (1971) Surgical repair of tricuspid atre- dure. J Thorac Cardiovasc Surg 127(6):1664–1669
sia. Thorax 26(3):240–248 Hanley FL (1999) The one and a half ventricle repair-we can
Fontan F, Mounicot FB et al. (1971) “Correction” of tricuspid do it, but should we do it? J Thorac Cardiovasc Surg
atresia. 2 cases “corrected” using a new surgical tech- 117(4):659–661
nic. Ann Chir Thorac Cardiovasc 10(1):39–47 Harrison DA, Liu P et al. (1995) Cardiopulmonary function
Fontan F, Deville C et al. (1983) Repair of tricuspid atresia in in adult patients late after Fontan repair. J Am Coll
100 patients. J Thorac Cardiovasc Surg 85(5):647–660 Cardiol 26(4):1016–1021
Fontan F, Fernandez G et al. (1989) The size of the pulmo- Hess J, Kruizinga K et al. (1984) Protein-losing enteropathy
nary arteries and the results of the Fontan operation. after Fontan operation. J Thorac Cardiovasc Surg
J Thorac Cardiovasc Surg 98(5 Pt 1):711–719; discussion 88(4):606–609
719–724 Hopkins RA, Armstrong BE et al. (1985) Physiological ratio-
Fontan F, Kirklin JW et al. (1990) Outcome after a nale for a bidirectional cavopulmonary shunt. A versa-
“perfect” Fontan operation. Circulation 81(5): tile complement to the Fontan principle. J Thorac
1520–1536 Cardiovasc Surg 90(3):391–398
Chapter 13 · Definite Palliation of Functional Single Ventricle
459 13
Horowitz MD, Culpepper WS 3rd et al. (1989) Pulmonary cavopulmonary anastomosis). J Thorac Cardiovasc
artery banding: analysis of a 25-year experience. Ann Surg 129(3):569–575
Thorac Surg 48(3):444–450 Kaye MP (1975) Anatomic correction of transposition of
Hosein RB, Clarke AJ et al. (2007) Factors influencing early great arteries. Mayo Clin Proc 50(11):638–640
and late outcome following the Fontan procedure in Keith JD, Rowe RD et al. (1958) Heart disease in infancy and
the current era. The ‘Two Commandments’? Eur childhood. Macmillan, New York
J Cardiothorac Surg 31(3):344–352; discussion 353 Kim WH, Lim HG et al. (2005) Fontan conversion with
Hraska V, Duncan BW et al. (2005) Long-term outcome of arrhythmia surgery. Eur J Cardiothorac Surg 27(2):
surgically treated patients with corrected transposi- 250–257
tion of the great arteries. J Thorac Cardiovasc Surg Kirklin JK, Blackstone EH et al. (1986) The Fontan opera-
129(1):182–191 tion. Ventricular hypertrophy, age, and date of opera-
Huddleston CB (2007) The failing Fontan: options for surgi- tion as risk factors. J Thorac Cardiovasc Surg 92(6):
cal therapy. Pediatr Cardiol 28(6):472–476 1049–1064
Hussain ST, Bhan A et al. (2007) The bidirectional cavopul- Knight WB, Mee RB (1995) A cure for pulmonary arteriove-
monary (Glenn) shunt without cardiopulmonary nous fistulas? Ann Thorac Surg 59(4):999–1001
bypass: is it a safe option? Interact Cardiovasc Thorac Knott-Craig CJ, Danielson GK et al. (1995) The modified
Surg 6(1):77–82 Fontan operation. An analysis of risk factors for early
Iemura J, Oku H et al. (1997) Total extracardiac right heart postoperative death or takedown in 702 consecutive
bypass using a polytetrafluoroethylene graft. J Card patients from one institution. J Thorac Cardiovasc Surg
Surg 12(1):32–36 109(6):1237–1243
Imielski BR, Woods RK et al. (2013) Fontan fenestration clo- Koide M, Masakazu A et al. (1999) Thromboembolism
sure and event-free survival. J Thorac Cardiovasc Surg originating from the pulmonary artery stump after
145(1):183–187 Fontan operation. Jpn J Thorac Cardiovasc Surg
Jacobs ML (2003) The functional single ventricle and 47(7):346–349
Fontan’s operation. In: Mavroudis C, Backer CL (eds) Kopf GS, Kleinman CS et al. (1992) Fenestrated Fontan
Pediatric cardiac surgery. Mosby, Philadelphia, operation with delayed transcatheter closure of atrial
pp 496–523 septal defect. Improved results in high-risk patients.
Jacobs ML, Mayer JE Jr (2000) Congenital heart surgery J Thorac Cardiovasc Surg 103(6):1039–1047; discus-
nomenclature and database project: single ventricle. sion 1047–1048
Ann Thorac Surg 69(4 Suppl):S197–S204 Kouchoukos NT, Blackstone EH et al. (2003a) Aortic atre-
Jacobs ML, Pourmoghadam KK (2007) Thromboembolism sia and other forms of hypoplastic left heart physiol-
and the role of anticoagulation in the Fontan patient. ogy. In: Kirklin JW, Barrat-Boyes BG (eds) Cardiac
Pediatr Cardiol 28(6):457–464 surgery, vol 2. Churchill Livingstone, New York,
Jacobs ML, Rychik J et al. (1995a) Avoidance of subaortic pp 1377–1400
obstruction in staged management of single ventricle. Kouchoukos NT, Blackstone EH et al. (2003b) Tricuspid atre-
Ann Thorac Surg 60(6 Suppl):S543–S545 sia and management of single ventricle physiology. In:
Jacobs ML, Rychik J et al. (1995b) Results of Norwood’s Kirklin JW, Barrat-Boyes BG (eds) Cardiac surgery, vol 2.
operation for lesions other than hypoplastic left heart Churchill Livingstone, New York, pp 1113–1175
syndrome. J Thorac Cardiovasc Surg 110(5):1555– Kreutzer G, Galindez E et al. (1973) An operation for the
1561; discussion 1561–1562 correction of tricuspid atresia. J Thorac Cardiovasc
Jacobs ML, Rychik J et al. (1996a) Protein-losing enteropa- Surg 66(4):613–621
thy after Fontan operation: resolution after baffle fen- Kreutzer J, Keane JF et al. (1996) Conversion of modified
estration. Ann Thorac Surg 61(1):206–208 Fontan procedure to lateral atrial tunnel cavopulmo-
Jacobs ML, Rychik J et al. (1996b) Early reduction of the vol- nary anastomosis. J Thorac Cardiovasc Surg
ume work of the single ventricle: the hemi-Fontan oper- 111(6):1169–1176
ation. Ann Thorac Surg 62(2):456–461; discussion Kühne M (1906) Über zwei Fälle kongenitaler Atresie des
461–462 ostium venosum dextrum. Jahrbuch Kinderheilkunde
Jacobs ML, Pelletier GJ et al. (2008) Protocols associated Physische Erziehung 63:235–249
with no mortality in 100 consecutive Fontan proce- Kumar SP, Rubinstein CS et al. (2003) Lateral tunnel versus
dures. Eur J Cardiothorac Surg 33:626–632 extracardiac conduit Fontan procedure: a concurrent
Jayakumar KA, Addonizio LJ et al. (2004) Cardiac transplan- comparison. Ann Thorac Surg 76(5):1389–1396; dis-
tation after the Fontan or Glenn procedure. J Am Coll cussion 1396–1397
Cardiol 44(10):2065–2072 Laks H, Pearl JM et al. (1991) Partial Fontan: advantages of
Jonas RA, Castaneda AR (1988) Modified Fontan proce- an adjustable interatrial communication. Ann Thorac
dure: atrial baffle and systemic venous to pulmonary Surg 52(5):1084–1094; discussion 1094–1095
artery anastomotic techniques. J Card Surg 3(2):91–96 Lamberti JJ, Spicer RL et al. (1990) The bidirectional cavo-
Jonas RA, Castaneda AR et al. (1985) Single ventricle (sin- pulmonary shunt. J Thorac Cardiovasc Surg 100(1):22–
gle- or double-inlet) complicated by subaortic steno- 29; discussion 29–30
sis: surgical options in infancy. Ann Thorac Surg Lamberti JJ, Mainwaring RD et al. (1991) The Damus-
39(4):361–366 Fontan procedure. Ann Thorac Surg 52(3):676–679
Kaulitz R, Ziemer G et al. (2005) Prophylaxis of thromboem- Lardo AC, Webber SA et al. (1999) Fluid dynamic comparison
bolic complications after the Fontan operation (total of intra-atrial and extracardiac total cavopulmonary
460 R. Lange and J. Hörer
connections. J Thorac Cardiovasc Surg 117(4): duit cavopulmonary anastomosis. Ann Thorac Surg
697–704 62(5):1276–1282; discussion 1283
Laschinger JC, Redmond JM et al. (1996) Intermediate McFaul RC, Tajik AJ et al. (1977) Development of pulmo-
results of the extracardiac Fontan procedure. Ann nary arteriovenous shunt after superior vena cava-
Thorac Surg 62(5):1261–1267; discussion 1266–1267 right pulmonary artery (Glenn) anastomosis. Report of
LeBlanc JG, Ashmore PG et al. (1987) Pulmonary artery four cases. Circulation 55(1):212–216
banding: results and current indications in pediatric McKay R, Pacifico AD et al. (1982) Septation of the univen-
cardiac surgery. Ann Thorac Surg 44(6):628–632 tricular heart with left anterior subaortic outlet cham-
Lee JR, Choi JS et al. (2003) Surgical results of patients with ber. J Thorac Cardiovasc Surg 84(1):77–87
a functional single ventricle. Eur J Cardiothorac Surg Mendelsohn AM, Bove EL et al. (1994) Central pulmonary
24(5):716–722 artery growth patterns after the bidirectional Glenn
Lee JR, Kwak J et al. (2007) Comparison of lateral tunnel procedure. J Thorac Cardiovasc Surg 107(5):1284–
and extracardiac conduit Fontan procedure. Interact 1290
Cardiovasc Thorac Surg 6(3):328–330 Mertens L, Hagler DJ et al. (1998) Protein-losing enteropa-
Lemler MS, Ramaciotti C et al. (2006) The extracardiac lat- thy after the Fontan operation: an international multi-
eral tunnel Fontan, constructed with bovine pericar- center study. PLE study group. J Thorac Cardiovasc
dium: comparison with the extracardiac conduit Surg 115(5):1063–1073
Fontan. Am Heart J 151(4):928–933 Meyer DB, Zamora G et al. (2006) Outcomes of the Fontan
Lev M (1953) Autopsy diagnosis of congenital malformed procedure using cardiopulmonary bypass with aortic
hearts. Charles C Thomas, Springfield cross-clamping. Ann Thorac Surg 82(5):1611–1618;
Li JS, Yow E et al. (2007) Clinical outcomes of palliative sur- discussion 1618–1620
gery including a systemic-to-pulmonary artery shunt Mitchell ME, Ittenbach RF et al. (2006) Intermediate out-
in infants with cyanotic congenital heart disease: comes after the Fontan procedure in the current era.
does aspirin make a difference? Circulation J Thorac Cardiovasc Surg 131(1):172–180
116(3):293–297 Moodie DS, Ritter DG et al. (1984) Long-term follow-up in
Lins MF, Lins RF et al. (1981) Double cava-pulmonary anas- the unoperated univentricular heart. Am J Cardiol
tomosis for the correction of tricuspid atresia. Arq Bras 53(8):1124–1128
Cardiol 36(2):111–113 Morales DL, Dibardino DJ et al. (2005) Salvaging the fail-
Mahle WT, Wernovsky G et al. (1999) Impact of early ven- ing Fontan: lateral tunnel versus extracardiac con-
tricular unloading on exercise performance in preado- duit. Ann Thorac Surg 80(4):1445–1451; discussion
lescents with single ventricle Fontan physiology. J Am 1451–1452
Coll Cardiol 34(5):1637–1643 Murthy KS, Coelho R et al. (1999) Novel techniques of bidi-
Mainwaring RD, Lamberti JJ et al. (1995) Bidirectional rectional Glenn shunt without cardiopulmonary
Glenn. Is accessory pulmonary blood flow good or bypass. Ann Thorac Surg 67(6):1771–1774
bad? Circulation 92(9 Suppl):II294–II297 Muster AJ, Zales VR et al. (1993) Biventricular repair of
13 Marcelletti C, Corno A et al. (1990) Inferior vena cava- hypoplastic right ventricle assisted by pulsatile bidi-
pulmonary artery extracardiac conduit. A new form of rectional cavopulmonary anastomosis. J Thorac
right heart bypass. J Thorac Cardiovasc Surg 100(2):228– Cardiovasc Surg 105(1):112–119
232 Muller WHJ, Dammann JFJ (1952) Treatment of certail con-
Marcelletti CF, Hanley FL et al. (2000) Revision of previous genital malformations of the heart by creation of a
Fontan connections to total extracardiac cavopulmo- pulmaonic stenosis to reduce pulmonary hyperten-
nary anastomosis: a multicenter experience. J Thorac sion and excessive pulmonary blood flow (a prelimi-
Cardiovasc Surg 119(2):340–346 nary report). Surg Gynecol Obstet 95:213–219
Matsuda H, Kawashima Y et al. (1986) Evaluation of total Myers JL, Waldhausen JA et al. (1990) A reconsideration of
cavopulmonary shunt operation for single ventricle risk factors for the Fontan operation. Ann Surg
with common atrioventricular valve and left isomer- 211(6):738–743; discussion 744
ism. Am J Cardiol 58(1):180–182 Nakano T, Kado H et al. (2007) Excellent midterm outcome
Mavroudis C, Zales VR et al. (1992) Fenestrated Fontan with of extracardiac conduit total cavopulmonary connec-
delayed catheter closure. Effects of volume loading tion: results of 126 cases. Ann Thorac Surg 84(5):1619–
and baffle fenestration on cardiac index and oxygen 1625; discussion 1625–1626
delivery. Circulation 86(5 Suppl):II85–II92 Nakata T, Fujimoto Y et al. (2010) Atrioventricular valve
Mavroudis C, Stewart RD et al. (2005) Atrioventricular valve repair in patients with functional single ventricle.
procedures with repeat fontan operations: influence J Thorac Cardiovasc Surg 140(3):514–521
of valve pathology, ventricular function, and arrhyth- Newfeld EA, Nikaidoh H (1987) Surgical management of
mias on outcome. Ann Thorac Surg 80(1):29–36; dis- subaortic stenosis in patients with single ventricle and
cussion 36 transposition of the great vessels. Circulation 76(3 Pt
Mayer JE Jr, Bridges ND et al. (1992) Factors associated with 2):III29–III33
marked reduction in mortality for Fontan operations Norwood WI, Jacobs ML (1993) Fontan’s procedure in two
in patients with single ventricle. J Thorac Cardiovasc stages. Am J Surg 166(5):548–551
Surg 103(3):444–451; discussion 451–452 Ocello S, Salviato N et al. (2007) Results of 100 consecutive
McElhinney DB, Reddy VM et al. (1996) Revision of previous extracardiac conduit Fontan operations. Pediatr
Fontan connections to extracardiac or intraatrial con- Cardiol 28(6):433–437
Chapter 13 · Definite Palliation of Functional Single Ventricle
461 13
Ono M, Boethig D et al. (2006) Clinical outcome of patients Restivo A, Ho SY et al. (1982) Absent left atrioventricular
20 years after Fontan operation—effect of fenestra- connection with right atrium connected to morpho-
tion on late morbidity. Eur J Cardiothorac Surg logically left ventricular chamber, rudimentary right
30(6):923–929 ventricular chamber, and ventriculoarterial discor-
Ono M, Boethig D et al. (2007) Somatic development long dance. Problem of mitral versus tricuspid atresia. Br
after the Fontan operation: factors influencing catch-up Heart J 48(3):240–248
growth. J Thorac Cardiovasc Surg 134(5):1199–1206 Rodefeld MD, Ruzmetov M et al. (2005) Staged surgical
Oski JA, Canter CE et al. (1996) Embolic stroke after ligation repair of functional single ventricle in infants with
of the pulmonary artery in patients with functional unobstructed pulmonary blood flow. Eur
single ventricle. Am Heart J 132(4):836–840 J Cardiothorac Surg 27(6):949–955
Ostrow AM, Freeze H et al. (2006) Protein-losing enteropathy Rosenthal A, Dick M (1983) Tricuspid atresia. In: Adams FH,
after fontan operation: investigations into possible patho- Emmanoulidis GL (eds) Moss’ heart disease in infants,
physiologic mechanisms. Ann Thorac Surg 82(2):695–700 children and adolescents. Williams & Wilkins,
Ottenkamp J, Wenink AC (1989) Anomalies of the mitral Baltimore, pp 271–283
valve and of the left ventricular architecture in tricus- Rubino M, Van Praagh S et al. (1995) Systemic and pulmo-
pid valve atresia. Am J Cardiol 63(12):880–881 nary venous connections in visceral heterotaxy with
Ovroutski S, Alexi-Meskishvili V et al. (2003) Early and asplenia. Diagnostic and surgical considerations
medium-term results after modified Fontan operation based on seventy-two autopsied cases. J Thorac
in adults. Eur J Cardiothorac Surg 23(3):311–316 Cardiovasc Surg 110(3):641–650
Pacifico AD, Kirklin JK et al. (1985) Surgical management of Russo P, Danielson GK et al. (1988) Modified Fontan proce-
double inlet ventricle. World J Surg 9(4):579–589 dure for biventricular hearts with complex forms of dou-
Parikh SR, Hurwitz RA et al. (1991) Ventricular function in ble-outlet right ventricle. Circulation 78(5 Pt 2):III20–III25
the single ventricle before and after Fontan surgery. Rychik J, Murdison KA et al. (1991) Surgical management
Am J Cardiol 67(16):1390–1395 of severe aortic outflow obstruction in lesions other
Pereira NL, Shirali G (2005) Cardiac transplant following than the hypoplastic left heart syndrome: use of a pul-
failed Fontan or Glenn procedures. J Am Coll Cardiol monary artery to aorta anastomosis. J Am Coll Cardiol
46(7):1374–1375; author reply 1375–1376 18(3):809–816
Peterson RJ, Franch RH et al. (1984) Noninvasive determi- Sakakibara S, Tominaga S et al. (1972) Successful total cor-
nation of exercise cardiac function following Fontan rection of common ventricle. Chest 61(2):192–194
operation. J Thorac Cardiovasc Surg 88(2):263–272 Sakurai T, Kado H et al. (2010) The impact of extracardiac
Petrossian E, Reddy VM et al. (2006) The extracardiac con- conduit-total cavopulmonary connection on apicocaval
duit Fontan operation using minimal approach extra- juxtaposition. Eur J Cardiothorac Surg 38(4):439–444
corporeal circulation: early and midterm outcomes. Salazar JD, Zafar F et al. (2011) Fenestration during Fontan
J Thorac Cardiovasc Surg 132(5):1054–1063 palliation: now the exception instead of the rule.
Petrucci O, Khoury PR et al. (2010) Outcomes of the bidirec- J Thorac Cardiovasc Surg 140(1):129–136
tional Glenn procedure in patients less than 3 months Scheurer MA, Hill EG et al. (2007) Survival after bidirec-
of age. J Thorac Cardiovasc Surg 139(3):562–568 tional cavopulmonary anastomosis: analysis of preop-
Pizarro C, Mroczek T et al. (2006) Fontan completion in erative risk factors. J Thorac Cardiovasc Surg
infants. Ann Thorac Surg 81(6):2243–2248; discussion 134(1):82–89, 89.e1–2
2248–2249 Schreiber C, Horer J et al. (2007) Nonfenestrated extracar-
Praus A, Eicken A et al. (2008) Progressive intrapulmonary diac total cavopulmonary connection in 132 consecu-
shunting in a child after bidirectional Glenn operation tive patients. Ann Thorac Surg 84(3):894–899
only resolved after total cavopulmonary completion. Schwartz SM, Gordon D et al. (1996) Collagen content in
Int J Cardiol 128(1):e12–e15 normal, pressure, and pressure-volume overloaded
Puga FJ, Chiavarelli M et al. (1987) Modifications of the developing human hearts. Am J Cardiol 77(9):734–738
Fontan operation applicable to patients with left atrio- Shachar GB, Fuhrman BP et al. (1982) Rest and exercise
ventricular valve atresia or single atrioventricular hemodynamics after the Fontan procedure. Circulation
valve. Circulation 76(3 Pt 2):III53–III60 65(6):1043–1048
Putnam JB Jr, Lemmer JH Jr et al. (1988) Embolectomy for Shimazaki Y, Kawashima Y et al. (1980) Ventricular volume
acute pulmonary artery occlusion following Fontan characteristics of single ventricle before corrective
procedure. Ann Thorac Surg 45(3):335–336 surgery. Am J Cardiol 45(4):806–810
Rao PS (1977) Natural history of the ventricular septal Shinkawa T, Anagnostopoulos PV et al. (2011) Early results
defect in tricuspid atresia and its surgical implications. of the “clamp and sew” Fontan procedure without the
Br Heart J 39(3):276–288 use of circulatory support. Ann Thorac Surg 91(5):
Rao PS (1983) Further observations on the spontaneous 1453–1459
closure of physiologically advantageous ventricular Shiraishi S, Uemura H et al. (2005) The off-pump Fontan
septal defects in tricuspid atresia: surgical implica- procedure by simply cross-clamping the inferior caval
tions. Ann Thorac Surg 35(2):121–131 vein. Ann Thorac Surg 79(6):2083–2087; discussion
Reddy VM, Liddicoat JR et al. (1995) Primary bidirectional 2087–2088
superior cavopulmonary shunt in infants between 1 and Slavik Z, Webber SA et al. (1995) Influence of bidirectional
4 months of age. Ann Thorac Surg 59(5):1120–1125; dis- superior cavopulmonary anastomosis on pulmonary
cussion 1125–1126 arterial growth. Am J Cardiol 76(14):1085–1087
462 R. Lange and J. Hörer
Smolinsky A, Castaneda AR et al. (1988) Infundibular septal Thompson LD, Petrossian E et al. (1999) Is it necessary to
resection: surgical anatomy of the superior approach. routinely fenestrate an extracardiac fontan? J Am Coll
J Thorac Cardiovasc Surg 95(3):486–494 Cardiol 34(2):539–544
Soto B, Bertranou EG et al. (1979) Angiographic study of Uemura H, Yagihara T et al. (1998) Establishment of total
univentricular heart of right ventricular type. cavopulmonary connection without use of cardiopul-
Circulation 60(6):1325–1334 monary bypass. Eur J Cardiothorac Surg 13(5):504–
Sridhar A, Giamberti A et al. (2011) Fontan conversion with 507; discussion 508
concomitant arrhythmia surgery for the failing atrio- Uhl HSM (1952) A previously undescribed congenital mal-
pulmonary connections: mid-term results from a sin- formation of the heart: almost total absence of the
gle centre. Cardiol Young 21(6):665–669 myocardium of the right ventricle. Bull Johns Hopkins
Srivastava D, Preminger T et al. (1995) Hepatic venous Hosp 91:197
blood and the development of pulmonary arteriove- Uzark K, Lincoln A et al. (1998) Neurodevelopmental out-
nous malformations in congenital heart disease. comes in children with Fontan repair of functional
Circulation 92(5):1217–1222 single ventricle. Pediatrics 101(4 Pt 1):630–633
Stansel HC Jr (1975) A new operation for d-loop transposi- Van Mirop LHS, Wiglesworth FW (1962) Isomerismus of the
tion of the great vessels. Ann Thorac Surg 19(5):565– cardiac atria in the asplenie syndrome. Lab Invest
567 11:1303
Stefanelli G, Kirklin JW et al. (1984) Early and intermediate- van Praagh R, Plett JA et al. (1979) Single ventricle.
term (10-year) results of surgery for univentricular Pathology, embryology, terminology and classifica-
atrioventricular connection (“single ventricle”). Am tion. Herz 4(2):113–150
J Cardiol 54(7):811–821 Vogt KN, Manlhiot C et al. (2007) Somatic growth in children
Stein DG, Laks H et al. (1991) Results of total cavopulmo- with single ventricle physiology impact of physiologic
nary connection in the treatment of patients with a state. J Am Coll Cardiol 50(19):1876–1883
functional single ventricle. J Thorac Cardiovasc Surg Wallace MC, Jaggers J et al. (2011) Center variation in
102(2):280–286; discussion 286–287 patient age and weight at Fontan operation and
Stewart RD, Pasquali SK et al. (2012) Contemporary Fontan impact on postoperative outcomes. Ann Thorac Surg
operation: association between early outcome and 91(5):1445–1452
type of cavopulmonary connection. Ann Thorac Surg Warnes CA, Feldt RH et al. (1996) Protein-losing enteropa-
93(4):1254–1260; discussion 1261 thy after the Fontan operation: successful treatment
Tam CK, Lightfoot NE et al. (1989) Course of tricuspid atre- by percutaneous fenestration of the atrial septum.
sia in the Fontan era. Am J Cardiol 63(9):589–593 Mayo Clin Proc 71(4):378–379
Tandon R, Edwards JE (1974) Tricuspid atresia. A re- Webber SA, Horvath P et al. (1995) Influence of competi-
evaluation and classification. J Thorac Cardiovasc Surg tive pulmonary blood flow on the bidirectional supe-
67(4):530–542 rior cavopulmonary shunt. A multi-institutional study.
Tatum GH, Sigfusson G et al. (2006) Pulmonary artery Circulation 92(9 Suppl):II279–II286
13 growth fails to match the increase in body surface area Williams DB, Kiernan PD et al. (1984) Hemodynamic
after the Fontan operation. Heart 92(4):511–514 response to positive end-expiratory pressure following
Taussig HB (1936) The clinical and pathological findings in right atrium-pulmonary artery bypass (Fontan proce-
congenital malformation of the hearts due to defective dure). J Thorac Cardiovasc Surg 87(6):856–861
development of the right ventricle associated with tri- Williams JA, Bansal AK et al. (2007) Two thousand Blalock-
cuspid atresia or hypoplasia. Bull Johns Hopkins Hosp Taussig shunts: a six-decade experience. Ann Thorac
59:435 Surg 84(6):2070–2075; discussion 2070–2075
Taussig HB (1939) Cardiovascular anomalies: a single ven- Yamada K, Roques X et al. (2000) The short- and mid-term
tricle with a diminutive outlet chamber. J Tech results of bidirectional cavopulmonary shunt with addi-
Methods 19:120 tional source of pulmonary blood flow as definitive pal-
Tchervenkov CI, Tahta SA et al. (1998) Biventricular repair liation for the functional single ventricular heart. Eur
in neonates with hypoplastic left heart complex. Ann J Cardiothorac Surg 18(6):683–689
Thorac Surg 66(4):1350–1357 Yoshida M, Yamaguchi M et al. (2005) Appropriate addi-
Thiene G, Daliento L et al. (1981) Atresia of left atrioven- tional pulmonary blood flow at the bidirectional Glenn
tricular orifice. Anatomical investigation in 62 cases. Br procedure is useful for completion of total cavopulmo-
Heart J 45(4):393–401 nary connection. Ann Thorac Surg 80(3):976–981
463 14
References – 478
..Fig. 14.1 a, b
Anatomy and a
nomenclature of VSDs.
a Classification of the
septum: membranous
septum; muscular septum:
Muscular septum:
inlet-, trabecular and
outlet septum. b
Localization of inlet, Outlet septum
muscular, conoventricular Membranous
(perimembranous) and septum
14 conus defects
Trabecular septum
Inlet septum
Conus defect
Conoventricular
(perimembranous) Inlet defect
defect Muscular defects
Chapter 14 · Ventricular Septal Defects
465 14
14.1.1 efect of the Inlet Septum
D esis of tetralogy of Fallot) or rarely posteriorly
(Inlet VSD) (LVOT narrowing in interrupted aortic arch). An
isolated conoventricular VSD is the result of an
This defect lies directly behind the septal leaflet of insufficient development of the membranous sep-
the tricuspid valve and corresponds to the ven- tum and is therefore often also called a
tricular part of an atrioventricular septal defect or «membranous» VSD. Because of the usual exten-
AV canal. Accordingly, it is often found in associ- sion into the muscular part, «perimembranous
ation with trisomy 21. The fact that some also call VSD» may be the better term. This localization is
it an «AV canal-type VSD» is the cause of an the most frequent one by far (>80 %).
ongoing morphological debate between the vari- There is a close relation to the tricuspid valve in
ous schools of cardiac pathology (Spicer et al. the area of the commissure between the septal and
2013). Upstream its border is the tricuspid valve the anterosuperior leaflet. The conduction system
anulus. The conduction system usually lies rather lies within the posteroinferior border. If the mem-
superficial within the caudad muscular border. branous septum is missing completely, the superior
Chordae and papillary muscles of the sometimes border of the VSD reaches the base of the right/
atypically configured right-sided atrioventricular noncoronary cusps of the aortic valve. Various
valve may obscure the overview from the right extensions posteroinferiorly (muscular septum,
atrium. This can necessitate the detachment of a inlet portion) or anterosuperiorly (conus septum,
leaflet for secure closure (see below, 7 Sect. 14.3.1). Fallot type) can occur and may impede orientation.
If there is only a small perforation of the mem-
branous septum, the defect will be hemodynami-
14.1.2 Muscular VSD cally insignificant. The development of a
sometimes quite impressive bulge of the membra-
Such a VSD is circumferentially bordered by muscle. nous part may occasionally lead to subpulmonary
It can occur in any area of the trabeculated part of obstruction. The impression of such a so-called
the right ventricle. The view from the right ventricle aneurysm VSD is, however, more often created by
with its crossing trabeculations can simulate multi- an associated dysplasia of the tricuspid valve leaf-
ple defects. Hence, the true arrangement can be let next to the defect which may be attached to its
judged much better from the smooth left ventricular crest with several chordae. Septal and anterosep-
side. Defects located anteriorly toward the apex are tal leaflet tissue can form a tunnel and direct
also known as «apical» VSDs. They are usually mul- blood from the left ventricle directly into the right
tiple and reflect an incomplete differentiation of the atrium (Gerbode defect; Gerbode et al. 1958).
muscular septum. Spontaneous closure within the
first months of life is therefore seen frequently.
The primarily netlike structure of the inter- 14.1.4 Conus VSD
ventricular partition with numerous communica-
tions explains the commonly encountered This VSD is characterized by an incomplete closure
multitude of defects. Whereas this is often only an of the conus septum between the two great arteries
illusion in the midmuscular part, apical VSDs are and is sometimes also called «subpulmonary» or
multiple as a rule. A prominent disruption of the «outlet» VSD. With the upper rim being outlined
myocardial differentiation of the septum is collo- by the pulmonary valve, its caudad border is mus-
quially called a «Swiss-cheese-type.» Apart from a cular. The conduction system and the tricuspid
conoventricular VSD a separate muscular defect valve are far away. For embryological reasons, this
may coexist, the relevance of which often only defect is frequently seen with a common arterial
becomes evident if it is overlooked or deliberately trunk, the isolated form being very rare.
not closed at surgery for the leading defect.
14.2 Natural Course and Indications
14.1.3 Conoventricular VSD for Operation
This defect is situated between the true ventricu- The natural course depends on the size and local-
lar and the conus septum. The conus septum may ization of the VSD. According to the shunted vol-
deviate anteriorly (RVOT narrowing, embryogen- ume and the pulmonary vascular congestion
466 M.K. Heinemann
..Fig. 14.2 Transatrial closure of a VSD. Exposure of the VSD through the right atrium and the tricuspid valve. Shown is
the partial detachment of the septal tricuspid valve leaflet at the anulus to expose the superior rim of the defect. VSD
sutures in U-stitch technique
..Fig. 14.3 Right ventricular aspect of VSD closure. Opened right ventricle. The hatched area marks the part of the
septum bearing the conduction system. Here, sutures must be placed stringently on the right ventricular aspect and
away from the rim. Illustrated suture techniques are U-stitches and continuous suture. A combination is possible
placing several, usually only one to three, unbut- All stitches are now placed through the patch
tressed stitches in a longitudinal fashion along the in their original order. The patch is then carefully
rim. Under very delicate circumstances and in lowered onto the septal defect where it comes to
direct neighborhood of valve tissue, the use of fine rest on its right ventricular aspect like a lid. It
monofilament material is expedient, enabling bet- should therefore be tailored a bit larger than the
ter guidance. It is helpful to fill the aortic root actual defect size. When lowering the patch, great
intermittently with cardioplegia. This problematic care must be taken again that it does not obstruct
region is then overcome and one reaches the pos- any chordae or parts of the tricuspid valve.
terior aspect close to the tricuspid valve again, Retracting these delicate structures with fine
which is dealt with as described above. Usually, nerve hooks can be helpful. The sutures should
for closure of a nonrestrictive infant VSD, 10–15 only be tied after secure placement of the whole
sutures are needed to guarantee secure anchoring circumference of the patch.
of the patch, at the same time avoiding compro- If a tricuspid valve leaflet was detached for
mising neighboring structures. exposure’s sake, it is then reattached, ideally with
Woven Dacron is a well-proven patch mate- a fine monofilament running suture. Tricuspid
rial, being both tear-proof and flexible. PTFE is valve competence is tested in any case by instilling
preferred by some. Autologous pericardium (usu- fluid into the right ventricle. If necessary, recon-
ally impregnated with 0.6 % glutaraldehyde for structive measures to alleviate valve incompe-
10 min) and xenopericardium are controversial tence are needed (adaptation of commissures,
because of lower tear resistance. One should bear anuloplasty, but also replacement of awkward
in mind that after closure an enormous pressure VSD patch sutures).
load is inflicted on the patch and that the muscu- The left atrial vent catheter is now removed
lar part of the circumference is contracting with and the interatrial septum closed. We use absorb-
every systole, putting the whole suture line in this able monofilament material for closure of the right
area at risk. More recently, a tissue-engineered atriotomy in children. Before removing the aortic
matrix of porcine intestine (CorMatrix®) came cross clamp, the left heart is meticulously de-aired
into use also. through the cardioplegia site. For immediate
470 M.K. Heinemann
pulmonary artery with the aid of a circumferential changes can lead to sometimes dramatic effects.
textile band (Muller and Dammann 1952). This Before the band is eventually permanently fixed,
had been based on the observation that moderate the circulation must be given some time to adapt
pulmonary stenosis would positively influence the to the new resistances (. Fig. 14.4). Usually we
prognosis of patients with single ventricle. The see a minimum increase of 10–15 mmHg systolic
banding procedure enabled postponing correc- arterial pressure and a reduction of the heart rate
tion into an age beyond infancy and had become by 10 %. Pulmonary artery pressure does not
quite common practice. In the current era of sur- need to be measured routinely. In a univentricu-
gical treatment of VSD, pulmonary artery banding lar circulation, an arterial saturation between 75
has almost completely lost its former role, being and 80 % (under ventilation with room air and
only considered in the presence of absolute con- with an open chest!) and a decrease of pulmo-
traindications for extracorporeal circulation or in nary arterial pressure to about one third systemic
pronounced forms of a «Swiss-cheese-type» sep- is considered the benchmark. When palliating a
tum (see below, 7 Sect. 14.6.4). The technique still biventricular system, the arterial saturation
has its merits in the stepwise palliation of univen- should not drop below 85 %, but rather be 90 %.
tricular circulations with pulmonary vascular To prevent distal migration of the band with
congestion in the newborn and is therefore the danger of compromising the bifurcation and
described here. inducing asymmetrical pulmonary blood flow,
Contrary to the historical technique which was fixation sutures should be applied to the pulmo-
done through a left posterolateral thoracotomy, nary root. In biventricular circulations the pul-
access today is via a median sternotomy. Besides monary valve must not be jeopardized in any
offering the advantage of equal ventilation and manner. If the pulmonary trunk is very short, the
perfusion of both lungs, this route offers far better band can be excised concavely at the underside of
exposure, the possibility to establish cardiopulmo- the right-angled take-off of the right pulmonary
nary bypass in an emergency, as well as the cos- artery to prevent stenosis to the right side.
metic aspect of just one scar for all subsequent As the principle of palliation means transfer
operations. In addition, in cyanotic univentricular from one pathological state to another, which is
circulations, the development of post-thoracot- presumably better tolerated, the time span with a
omy chest wall-to-pulmonary collaterals compli- banded pulmonary artery should be kept to a
cating later Fontan type surgery is avoided. minimum. This is especially true for eventually
After displacement or partial resection of the curable congenital heart disease such as VSD or
thymus, the pericardium is incised over the great atrioventricular septal defect. The persistence of
14 vessels. The connective tissue between the aorta right-sided systemic pressures causes hypertro-
and the often rather short main pulmonary phy of the right ventricular muscle—up to the
artery is sparingly dissected over the width of the level of a hemodynamically relevant infundibular
intended band. Instead of the conventional stenosis persisting after debanding, causing addi-
braided textile bands which may migrate through tional problems. With the growth of the child, the
the vessel wall over time and lead to considerable banding becomes constantly more effective and
adhesions in any case, the use of smooth, inert may induce critical spells of arterial desaturation.
silicone, ideally reinforced with a Dacron mesh, This should be prevented by early de-banding not
or a PTFE band is recommended. Starting from later than 6 months post banding.
the historical Trusler’s formula (Trusler and
Mustard 1972), the initial length of the circum-
ference to be marked on the band should amount 14.6.2 VSD with Secondary Aortic
to 20 mm + 1 mm/kg body weight (e.g., 24 mm in Valve Insufficiency
a 4 kg baby). After marking, the band is pulled
around the pulmonary artery and fixed accord- The increasing prolapse of an aortic valve cusp
ing to the marked length. Depending upon the adjacent to a VSD can lead to distortion of the
hemodynamic situation, it can then be loosened valve with consecutive regurgitation. Small cono-
or tightened further. According to Poiseuille’s ventricular defects high in the septum or juxta-
law, the radius of a tube influences the flow arterial conus defects are at particular risk. Usually
through it in the fourth power. Thus, minimal it is the right coronary cusp which is closely
Chapter 14 · Ventricular Septal Defects
473 14
a b
..Fig. 14.4 a, b Pulmonary artery banding. a Provisional adjustment of the band at the pulmonary artery by
clamping. Direct distal pressure measurement. b Permanent fixation of the band with single sutures
related to the VSD, sometimes the non-coronary removed. Instillation of cardioplegia into the aor-
one or both (Tomita et al. 2005). A lack of support tic root gives a first impression of valve patency.
of the afflicted area as well as the Venturi effect are An intraoperative transesophageal echocardiog-
considered mechanisms contributing to progres- raphy will exactly define the success of the recon-
sive valvular insufficiency (Tatsuno et al. 1973; struction. In the presence of additional congenital
Tweddell et al. 2006; see above 7 Sect. 14.2). valve asymmetries, e.g., a bicuspid valve or
Closure of the defect before the development of advanced changes, prognosis is obviously limited.
valvular insufficiency or as long as it is only minor
has the best prognosis (Jian-Jun et al. 2006;
Kostolny et al. 2006; Saleeb et al. 2007; Tomita 14.6.3 VSD and Coarctation
et al. 2004). In these cases closure of the VSD is of the Aorta
often sufficient with the patch providing the nec-
essary support. If the valve has already undergone A VSD can be a defined, fixed part of a complex
morphological alterations, extension of the opera- malformation, e.g. in tetralogy of Fallot or com-
tion becomes necessary. mon arterial trunk. Often it is only loosely associ-
Reconstruction of the aortic valve according ated with a second malformation without this
to the technique described by Trusler has very constituting a direct syndromal relationship. The
good long-term results (Trusler et al. 1973, 1992). most common malformation encountered in the
After a transverse aortotomy above the sinotubu- presence of a VSD is coarctation of the aorta (see
lar junction, the aortic valve is inspected. One 7 chapter «Congenital Anomalies of the Great
usually finds an elongation of the cusp prolapsing Vessels», Sect. 21.2.1.1).
into the defect. To correctly judge the extent of the Whereas concomitant correction during a
asymmetry, it is recommended to pass a fine single procedure is well accepted for complex
monofilament suture through the noduli Arantii heart disease (Heinemann et al. 1990), the strat-
to elevate the valve («Frater stitch»; Frater 1967). egy for optimal treatment of VSD with aortic
The afflicted cusp will crimp along its pathologi- coarctation is still subject of debate (Gaynor
cally elongated part close to the commissure. A 2003). A two-staged approach is the historically
buttressed suture then fixes the resulting fold established one, in which the coarctation is cor-
transmurally to the neighboring aortic wall, thus rected first through a left lateral thoracotomy.
reestablishing the symmetry of the free cusp edges Then the VSD is closed transsternally in a second
(. Fig. 14.5). The central marking suture is then operation. In the presence of large defects, this
474 M.K. Heinemann
a b
..Fig. 14.5 a–d Trusler’s plasty in aortic valve insufficiency. Transverse incision of the aortic root. a «Frater stitch»
through the noduli Arantii. The right coronary cusp is elongated. b Tension on the «Frater stitch» creates crimping
14 of the elongated cusp into a fold close to the aortic wall. c Right and non-coronary cusp are attached to each other.
Then the fold is fixed transmurally with pledgeted sutures to gather the elongation. d Safety suture between the two
cusps, creating a cover
strategy can be complemented with pulmonary A disadvantage is the use of hypothermia which
artery banding during coarctectomy to gain some would not be necessary for VSD closure alone and
time until the second procedure. Disadvantages is therefore a considerable extension of the proce-
are the necessity of two different incisions, two dure with all associated risks. An increased rate of
anesthesias, and a longer or repeated hospital stay recoarctations, presumably because of worse expo-
in combination with a state of palliation. sure, has also been reported (Brouwer et al. 1996;
Technical advances have led to the increased Gaynor et al. 2000; Isomatsu et al. 2001). The suc-
endeavor of concomitant correction of an isolated cessful combination of both strategies is also pos-
VSD and aortic coarctation through a sternotomy sible: First, a standard coarctation repair is done
in analogy to the strategy in more complex heart through a lateral thoracotomy, to be followed—
disease. The aortic isthmus is then approached dur- after repositioning but during the same anesthe-
ing a short phase of deep hypothermia and, when sia—by VSD closure through a sternotomy,
necessary, circulatory arrest. During the cooling avoiding deep hypothermia (Kanter et al. 2007).
phase the aortic arch with its braches as well as the Our experience leads us to recommend the fol-
descending aorta can be dissected free extensively. lowing approach: in a small VSD and aortic coarc-
Chapter 14 · Ventricular Septal Defects
475 14
tation, the aorta is repaired first and the VSD to bulging of the sinus into the cardiac structures
closely followed and closed at a later date as neces- below, up to the point of perforation. Usually only
sary. If size and extent of a VSD necessitate early the right coronary sinus is afflicted, sometimes in
closure, this is regarded an indication for single combination with the adjacent half of the noncoro-
stage repair. If the aortic narrowing also extends nary one. Perforation ensues into the right atrium
into the aortic arch as a tubular hypoplasia, which or ventricle. Coexistence of a VSD bordering the
is often observed in these cases, sternotomy and aortic valve can also be part of the malformation,
the use of hypothermic techniques for extended leading to valvular insufficiency. An increased
aortic reconstruction are appropriate anyway. If a incidence of this combination has been observed
VSD/coarctation patient presents in poor condi- in Asia, giving an indirect hint to genetic predispo-
tion, even after compensation, we rather proceed sition (Brizard 2006; Sakakibara and Konno 1962,
with coarctation repair only, regardless of VSD size. 1968; Lee et al. 2013).
In adult patients an extra-anatomic bypass to The operative strategy consists of resection of
circumvent the coarctation can be a very good alter- the «aneurysm,» VSD closure, and aortic valve
native option (Myers et al. 2011; Reents et al. 2012). reconstruction. After inspection of the anatomy
through an aortotomy, an exposition from the
ventricular side, e.g., through an infundibulot-
14.6.4 «Swiss-Cheese-Type» VSD omy, is adequate in most cases. This allows for a
definitive discrimination between a conoventric-
The malformation somewhat colloquially called a ular and a pure conus defect and therefore the
«Swiss-cheese-type» VSD represents a profound supposed course of the conduction system. The
disturbance of the development of the muscular aneurysmatic tissue is resected, resulting in a
ventricular septum, primarily in its trabeculated larger defect in the right coronary sinus. The
part. The multiple connections between right and Dacron patch used for VSD closure is also utilized
left chamber existing during embryonic and fetal for reconstruction of the aortic root. First, com-
life do not close, resulting in several defects in the plete VSD closure is performed with the anchor-
muscular septum. In the case of anteroapically ing of the middle part of the patch directly into
located small communications, spontaneous clo- the insertion zone of the right coronary cusp in
sure within the first year of life is highly likely. the usual fashion. The excess patch length crani-
A combination of larger VSDs extending into ally can then be tailored into the aortic root defect
the midmuscular portion with a considerable with a continuous suture. In a classical conus
combined passage area can become hemodynami- defect, as is often seen in Asian patients, the whole
cally very significant at the time of the physiologi- procedure can be done transaortically. Asymmetry
cal postnatal drop in pulmonary vascular of a valve cusp may call for additional valve recon-
resistance. Number and location of these commu- struction or even replacement.
nications make their surgical exposure very diffi-
cult. A decision must be made between pulmonary
artery banding and a right ventriculotomy (Seddio 14.6.6 Catheter Interventional
et al. 1999), if pulmonary artery banding is not the Techniques
institutional approach in these cases anyway. A left
ventriculotomy should be avoided if at all possible. The endeavor to avoid a ventriculotomy for clo-
sure of hemodynamically highly significant mus-
cular defects led to the development of
14.6.5 VSD and Aneurysm interventional techniques by catheter (Lock et al.
of a Valsalva Sinus 1988; Okubo et al. 2001; Waight et al. 2002; Bacha
et al. 2005). Because of the limitations of vascular
Congenital aneurysm of a sinus of Valsalva repre- access in small children, these are often combined
sents a separation of the media of the afflicted sinus with open exposure of the heart through a thora-
from that of the hinge point of the respective aortic cotomy as so-called hybrid procedures and have
cusp (Edwards and Burchell 1957). The structural become well-established in several centers. Purely
weakness between the aortic root and the heart in percutaneous closure of a muscular VSD can be
combination with the aortic blood pressure leads regarded safe under certain conditions too.
476 M.K. Heinemann
Further refinement of the devices has mean- defects. Direct contact of the device with the
while led to their use for closure of conoventricu- neighboring structures of the VSD did lead to
lar/perimembranous defects as an alternative to some insufficiencies of the aortic and tricuspid
surgery. This was evaluated in several studies valves, which was to be expected. Pressure upon
(Michel-Behnke et al. 2005; Holzer et al. 2006; the inferior border of the VSD also induced higher
Butera et al. 2007a, b; Hirsch et al. 2007; Xunmin degree heart block up to the need of temporary
et al. 2007; Mo et al. 2011). Because secure place- transvenous stimulation over several weeks
ment of these devices in a defect needs appropri- (Butera et al. 2006; Walsh et al. 2006; Zhou et al.
ate protrusions at the borders of the right and left 2008). Contrary to surgical therapy the possibili-
ventricular aspect, several modifications of the ties to manage complications are limited, and
established models for atrial septal defects became even the theoretical benefit of a shorter hospital
necessary. The «Amplatzer membranous septal stay with low morbidity may then become obso-
occluder» shows recesses of the Nitinol meshwork lete. At present, elective interventional closure of
for the aortic valve (. Fig. 14.6). a conoventricular VSD should be limited to a few
This proved to be successful in older children centers and closely followed by rigid study proto-
and adults with accordingly relatively small cols (Butera et al. 2007a, b; Carminati et al. 2007).
..Fig. 14.6 «Amplatzer 5 4
membranous septal
occluder.» Lateral aspect.
Device mounted on the
catheter and expanded; left
ventricular disk between 3
and 4; aortic excess length 4
with 0.5 mm being smaller
than the septal one 3 with
5.5 mm. Right ventricular
disk between 1 and 5, equal
excess length of 2 mm.
Waist fitting into the VSD (2)
with a depth of 1.5 mm. A
«delivery sheath»; B «pusher
catheter»; C connector at
14 catheter; D screw coupling
at disk. The
dacron-polyester patch
material can be seen
through the Nitinol mesh
(Reproduced from Hijazi
et al. (2002); with
permission
A B C D
1 2 3
Chapter 14 · Ventricular Septal Defects
477 14
The limited financial means of emerging coun- typical scenario is spontaneous closure once the
tries have stimulated research to further modify pulmonary resistance drops.
occlusive devices for use in the setting of a hybrid
approach through a (limited) thoracotomy but
without cardiopulmonary bypass. Given the popu- 14.7 Perioperative Aspects
lation density of China and the frequency of VSDs
as isolated defects, impressive series were pub- For getting informed consent, besides the general
lished over the recent years (Xing et al. 2010, 2011; risks of open heart surgery, the typical risks of VSD
Zheng et al. 2009; Chen et al. 2013). The remark- closure must be mentioned: complete heart block
able early outcomes and the active participation in with pacemaker dependency, residual defect, and
their development have now brought these new valvular insufficiencies (aortic, tricuspid). On a
techniques to Europe (Schreiber et al. 2012). positive note, it should be emphasized that quality
However, long-term results remain to be evaluated. and expectancy of life as well as exercise capacity
can be considered normal after a successful
transatrial operation (Roos-Hesselink et al. 2004).
14.6.7 Pushing the Limits Recommendations for the prevention of infec-
tive endocarditis have evolved over the years.
Advancements in microtechnology have led to Antibiotic prophylaxis should be administered in
amazing developments like that of a telemetrically unrepaired VSD as well as any residual defects.
adjustable banding device (Bonnet et al. 2004). Depending on the material, endothelialization of
This plays no role for standard treatment of a a patch can be considered complete after
VSD. Transcutaneous tunneling of fixation 6 months, making aggressive antibiotic prophy-
sutures of a pulmonary band also serves the same laxis unnecessary thereafter (Backer et al. 1993;
purpose of avoiding operative readjustments Dajani et al. 1997; Wilson et al. 2007).
(Choudhary et al. 2006). During postoperative intensive care therapy,
Such notions are primarily generated in the principles for preventing so-called pulmonary
emerging countries which in theory do offer hypertensive crises must be followed, especially in
modern cardiac surgery, but where many patients young infants with a markedly raised preoperative
are presenting only late or in dismal condition Qp:Qs ratio (relaxation, NO application, etc.; see
due to infrastructural shortcomings. If primary 7 chapter «Critical Care in Pediatric Cardiac Surgery»,
closure of a VSD is attempted in the presence of Sect. 10.7 and Sect. 10.11). An early complete heart
markedly elevated but still responsive pulmonary block may be caused by swelling of the tissue,
vascular resistance, patches with a valve mecha- which will usually resolve within 10–14 days.
nism may be considered (Novick et al. 2005; During this time period, the indication for perma-
Zhang et al. 2007). In the event of a critical nent pacemaker implantation should be handled
increase in pulmonary artery pressure, these very restrictively. It is a recommended safety mea-
allow for an overflow of desaturated blood from sure to implant two sets of temporary ventricular
the right into the left ventricle and thus mainte- stimulation leads in any patient who has a higher
nance of systemic blood pressure at the expense degree heart block at the end of the operation.
of arterial oxygen saturation. We still use a sim- Even if closure of a VSD historically belongs to
ple fenestration of the Dacron patch with a the first successful cardiac procedures ever, it is by
standardized punch. Such an opening, usually no means a bagatelle or a beginner’s operation.
with a diameter of 3 mm, can be closed consecu- Nowadays, it is a requirement to limit this opera-
tively with a catheter intervention if necessary. tion only to centers with the necessary expertise
We feel this to be the safer approach—no risk of in the treatment of congenital heart disease, which
«valve» failure—as the limited fenestration is particularly important because the majority of
should reliably prevent a suprasystemic right VSD closures must be performed during the new-
ventricular pressure rather than prevent right- born period or in early infancy. This is the only
sided volume overload which rarely will occur in way to achieve the necessary quality regarding
these patients during long-term follow-up. The morbidity and mortality and long-term outcome.
478 M.K. Heinemann
Xunmin C, Shisen J, Jianbin G et al. (2007) Comparison of Zheng Q, Zhao Z, Zuo J (2009) A comparative study: early
results and complications of surgical and Amplatzer results and complications of percutaneous and surgi-
device closure of perimembranous ventricular septal cal closure of ventricular septal defect. Cardiology
defects. Int J Cardiol 120:28–31 114:238–243
Zhang B, Wu S, Liang J et al. (2007) Unidirectional mono- Zhou T, Shen XQ, Zhou SH (2008) Atrioventricular block: a
valve homologous aortic patch for repair of ventricu- serious complication in and after transcatheter clo-
lar septal defect with pulmonary hypertension. Ann sure of perimembranous ventricular septal defects.
Thorac Surg 83:2176–2181 Clin Cardiol 31:368–371
14
481 15
References – 524
484 G. Ziemer and R. Kaulitz
the trunk by tightening the purse-string suture. carried out via transinfundibular access. The
Depending on the bypass strategy, a solitary incision is performed in a longitudinal direc-
venous cannula is inserted via the right atrial tion in continuation of the center line of the
auricle or two cannulae are used for both SVC pulmonary artery. It is not necessary to extend
and IVC individually. During the cooling the incision beyond the infundibulum into the
period, the dissection of the pulmonary artery right ventricular cavity. Former descriptions
is completed. The clamping of the aorta is car- including their drawings show ample incisions
ried out in deep hypothermia or whenever car- in direction of the apex and beyond the infun-
dioplegia is planned to be delivered. In dibulum cause right ventricular dysfunction. If
circulatory arrest the venous cannula is a transanular patch plasty is not necessary, the
removed routinely, and the right atrium is infundibulotomy should start strictly inferior
incised and inspected. In cases with an intact to the anulus. Cutting the crista supraventricu-
atrial septum, temporary atrioseptostomy in laris should be avoided also.
the area of the fossa ovalis is performed. This In neonates, the spatial relations are more
helps venting the left heart and finally de-airing critical than in older patients. As the sutures of
also. the outflow tract patch consume relatively
Immediately after closure of the VSD, in more of the ventricular wall, the course of the
cases where DHCA was employed, systemic RIVA/LAD has to be considered at any time,
reperfusion may start with a reduced flow rate with any stitch of the RVOT patch suture. A
of 25 % of the calculated flow returning the retrospective study attributed the strikingly
venous blood by the cardiotomy sucker. The increased mortality after performing surgery
temperature of the perfusate should be main- of the tetralogy of Fallot in neonates at that
tained at 20°, and rewarming should start only time to a possible ischemia of the area provided
after release of the aortic cross clamp, allowing by the RIVA/LAD without showing pathologi-
for coronary perfusion. This may happen cal signs of a complete occlusion (DiDonato
immediately after the left heart is de-aired et al. 1991). Placing two superficial sutures at
through the atrioseptostomy and the proximal the collapsed right ventricle before infundibu-
ascending aorta, and the atrial septum is closed. lotomy provides a guideline for the incision
The venous cannula is reinstalled after closing projected in the longitudinal axis of the pulmo-
the atriotomy. For patients after DHCA, after nary artery.
release of the aortic clamp, the pressure of the
perfusion may stay at <30 mmHg for 10 min.
With flow rates of >50 %, the rewarming can be
A retractor placed in the apical angle of the
continued at temperatures of 25°. During the
infundibulotomy helps in identifying the intra-
15 rewarming period, the patch of the right ven-
cardial anatomy.
tricular/transanular outflow tract can be sewed
if necessary. Usually the time of the circulatory
arrest needed for a transinfundibular correc-
tion of a TOF lies between 20 and 30 min. In today’s approach of neonatal and infant
For patients operated upon in normother- tetralogy repair, transection rather than resection
mia or mild hypothermia with bicaval cannula- of abnormal and potentially obstructing muscle
tion, de-airing of the left heart is performed bundles is required. After sectioning some bundles
immediately after VSD and ASD closure, fol- of muscles in the area of the lateral insertion of the
lowed by release of the aortic cross clamp, deviated conus septum, the so-called dynamic
allowing for myocardial reperfusion and component of stenosis is usually eliminated. More
rewarming as necessary. The remainder of the incisions, or eventual resection of muscles, may be
repair is performed on the beating heart. necessary in presence of «jet lesion,» especially in
older kids, which can be found at spots of distinct
kTransinfundibular Repair flow acceleration. Although jet lesions are located
The classical surgical technique of tetralogy of very often at the conus septum, a resection of mus-
Fallot associated with a pulmonary stenosis is cles is not recommended because of the aortic
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
491 15
valve closely behind the middle part of the septum. of the valve to the anulus, entering now from
Only a sparing resection of the fibrotic component the atrium and placed in the area of the antero-
is required and suggested. septal commissure (A in . Fig. 15.3b). Placing a
Regarding the long-term results, the actual rigid sucker through the VSD facilitates visual-
Achilles’ heel of tetralogy repair is the pulmonary ization and technical accessibility. Lowering the
valve. In symptomatic patients with TOF, there patch into the field, the next stitches should be
almost always is a valvular component of the placed close to the aortic valve (B in . Fig. 15.3b),
obstruction, which requires surgery. In fact a followed by sutures postero-caudal on the side
complete resection is only considered in cases of of the right ventricle, far apart from the defect
an acommissural cartilaginous membrane. A (C in . Fig. 15.3b). It is imperative not to touch
stepwise separation using Hegar dilatators should or enclose the medial papillary muscle by the
be performed even at tenuous tricuspidal suture (Lansici muscle), because a resulting
designed valves. More recently intraoperative insufficiency of the tricuspid valve will not be
balloon dilation combined with pulmonary valve tolerated hemodynamically by the hypertro-
sparing and reconstructive techniques are sug- phic and hyperdynamic right ventricle in the
gested without having produced superior inter- early postoperative period. After four to five
mediate results, yet. stitches, ensuring the critical area, the patch
The diameter of the anular ring can be suffi- should be sewn in the posterior cranial angle
ciently determined preoperatively by echocar- using an additional suture (D in . Fig. 15.3b).
diography and may be taken as a measurement for This area has some irregular trabeculae and was
the extent of dilation rather than the calculated usually incised during the former resection/
diameter related to the surface of the body. The incision of the infundibulum. To avoid intra-
use of dilatators larger than the diameter of the mural residual VSDs and to compensate the
anulus minus the standard variance is not recom- missing relative solid endocardium, the stitches
mended. The use of bigger dilatators can lead to should be applied very profoundly. Reaching
paracommissural tears in the wall of the pulmo- the lower edge of the conus septum, the stitches
nary artery and the anulus. It is absolute impor- should be applied also strong but more superfi-
tant to handle the valve tissue gently and to cial to avoid any injury of the aortic valve situ-
maintain/preserve the sufficient valve. But it has ated directly behind the conal septum (E in
to be considered that the anulus of the pulmonary . Fig. 15.3b).
valve, in contrast to all other heart valves, is com- Patch closure of the infundibulotomy (iso-
pletely muscular. Therefore, regurgitation can lated at the infundibulum or extending transa-
develop caused by an aneurysmatic dilation of the nular) may be performed during rewarming on
infundibulum and anulus even in the case of an bypass with the heart beating.
originally preserved tenuous tricuspidal designed
valve (see also below, 7 Sect. 15.2: «Long-term
Complications After TOF Repair»).
For VSD closure, we prefer using a Dacron For patch closing the infundibulotomy, it is
patch. The patch is sutured using a running absolutely necessary to use two suckers to
polypropylene suture (5/0 or occasionally 6/0 obtain a blood free and sufficiently clean situs.
in neonates, 5/0 in infants and young children, Any distortion has to be avoided in order to
later 4/0). prevent any stenosis especially in the area of
Despite different techniques of sutures and the critical distal angle in the MPA or at the
patch materials, it is expedient to apply some anulus, respectively.
principles of surgery to a VSD closure
(. Fig. 15.3). The suture should commence at
the deepest and farthest point, at the area of the
tricuspid anulus for the transinfundibular kTransanular right ventricular outflow tract
approach. In the case of a defect reaching the patch
tricuspid valve (perimembranous malalign- We almost exclusively use polytetrafluorethylene
ment VSD), the first U-stitch of any suture (0.4 mm thick) as reconstruction material for the
should be placed preferably through the orifice right ventricular outflow tract. An alternative can
492 G. Ziemer and R. Kaulitz
a b
..Fig. 15.3 a Transinfundibular approach for tetralogy of Fallot repair. The septal and parietal branches, respectively,
of the conus septum are already incised. In general the edges of the VSD cannot be visualized simultaneously at one
15 glance. b Semi-schematic depiction of the circumference of the VSD. Segment A aortic tricuspidal angle; segment B inlet
septum with the bundle of His running at the edge of the ventricular septum, sometimes place of insertion of isolated
tricuspid valve chordae; segment C trabecular septum (smooth surface); segment D conus septum with the aortic valve
directly behind it; segment E crista supraventricularis with irregular and sometimes very deep trabeculae. c Distance and
depth of the stitches from the defect to the different parts of the circumference of the VSD
be autologous pericardium, homograft, xenograft, branches by the patch and to achieve a sufficient
or Dacron. In case of a sufficiently wide pulmonary dilatation at the same time, we occasionally use
valve anulus (>2 standard variation compared to third- or half-pipe-shaped pieces produced by lon-
normal), the integrity of anulus and valve may be gitudinal incised appropriate- sized PTFE grafts
retained by using only a strict infundibular patch. (diameter 6–8 mm). The given convexity of the
But in the case of a severely hypoplastic or atretic graft/vascular prosthesis prevents the distortion or
anulus, a transanular patch has to be used. The occlusion of the vascular lumen even using a cor-
incision is carried out into the pulmonary bifurca- responding wide patch. These bulged reconstruc-
tion, at least to the origin of the right pulmonary tions adapted itself to the growing vascular
artery or in case of a pulmonary coarctation even structure by time. A notable oversizing is not desir-
further into the left pulmonary artery (. Fig. 15.4). able; the diameter of the anulus may not exceed the
In order to avoid distortion of the bifurcation or its calculated item related to the surface of the body.
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
493 15
a b
..Fig. 15.4 a In case of a suspected pulmonary artery coarctation, the incision may reach into the left pulmonary
artery. b The bulging patch may be cut almost rectangular at its ends. The maximum diameter is located at the level of
the anulus
The suture line commences in the distal angle and kTransatrial correction
runs proximal to both sides in increments using a This technique had been already described in the
running suture (polypropylene). early 1960s (Hudspeth et al. 1963). But in the fol-
For the patch suture line, the stitches in the lowing three decades, the original transinfundibu-
pulmonary artery wall should be congruent to lar correction (Lillehei et al. 1955) was established
those in the patch, whereas the stitches performed (Kirklin and Karp 1970) and became accepted as
in the myocardium of the infundibulum are far the almost exclusively used method (Castaneda
wider and deeper than in the patch for reasons of et al. 1977). There were occasional reports in the
hemostasis. At the apical end of the patch, more literature using a transatrial correction, e.g., in case
length of the patch than in the myocardium is of coronary anomalies or as a secondary correction
used to retain the bulge of the patch. It is not nec- after primary palliation in older children (Brizard
essary to aim for full-thickness bites, as the main et al. 1998). The use of a «partial-transatrial» cor-
mechanical hold for the suture is provided by the rection with a transatrial closure of the VSD and a
epicardium. The adjacent coronary arteries must limited distal infundibulotomy had been also
not be compromised, especially the LAD. The established in some centers (Edmunds et al. 1976;
covering epicardium overlying the coronary has Kawashima et al. 1985; Atallah-Yunes et al. 1996).
to be incised occasionally, giving relief of tension With a growing number of patients undergo-
which may have occurred. ing reoperation after transinfundibular repair, a
There are different suggestions regarding tech- renewed interest in transatrial approach came up,
nical innovation to replace the valvular function hoping to preserve right ventricular integrity and
by some working groups, e.g., the so-called mon- thereby long-term function. During the last
ocusp valve by the Loma Linda group (Gundry 25 years, our strategy moved also toward this
et al. 1994). This technique utilizes insertion of a direction; beyond the early postnatal phase, it
semilunar cusp of the same material as the transa- became our preferred access for repair of uncom-
nular patch (autologous pericardium or 0.1 mm plicated tetralogy of Fallot.
thick PTFE membrane). Originally this idea came After performing a right atriotomy, the VSD
up in the early 1970s but did not show any ongo- can be seen through the tricuspid valve after
ing effects on the pulmonary regurgitation except retracting the septal leaflet and anterior anulus
an early postoperative improved function of the with the assistance of two small retractors, set
valve (Ionescu et al. 1979). John Brown from Riley asserted at 11 and 2 o’clock position in the sur-
Children’s reported midterm results on improved geon’s view. The VSD is located far more cranially
function of the monocusp valve without any compared to a perimembranous VSD. The over-
major regurgitation in 48 % of his patients after riding aortic valve is seen very clearly through the
10 years (Brown et al. 2007). VSD (. Fig. 15.5).
494 G. Ziemer and R. Kaulitz
of reoperation, while the alternative, namely using most patients with peripheral pulmonary artery
a conduit, will definitely lead to later reoperations stenosis after TOF repair, pulmonary insuffi-
in all patients. ciency is the second leading symptom. Therefore,
in these cases, pulmonary valve replacement
should be combined with a patch plasty (see
15.2.2 Pulmonary Stenosis 7 Sect. 15.2.3).
and Bifurcation/Left In patients less than 5 years of age, we rarely
Pulmonary Artery Stenosis implant pulmonary valves unless severe right
ventricular dilatation and dysfunction is present.
The rare event of pulmonary arteries being pri- These patients are rather candidates for adequate
marily very small can result in a significant post- patch reconstruction of RVOT/MPA/pulmonary
operative elevation of right ventricular pressure to bifurcation.
start with.
Recurrent or residual stenosis at the origin
of the left pulmonary artery, at the site of the 15.2.3 Pulmonary Regurgitation
former ductus entrance, is discussed as
Coarctatio pulmonalis in 7 Chapter 21, 15.2.3.1 Consequences of Pulmonary
«Anomalies of the Great Intrathoracic Vessels». Regurgitation
The insertion of the BT shunt can cause periph- Pulmonary «valve» insufficiency is described in
eral branch stenosis requiring patch plasty or up to 90 % of the patients after correction of
catheter intervention (balloon angioplasty ± tetralogy of Fallot. The right ventricular vol-
stent implantation). The same intervention ume load and dilatation are clinically compen-
might be necessary if the stenosis at the distal sated for many years, but reduced exercise
patch end of the RVOT patch plasty is compro- tolerance will develop in adolescent patients
mising the vessel lumen or causing bifurcation with an increased risk for exercise-induced
stenosis to the right and or left pulmonary arrhythmia. Because of the interventricular
artery. Decision-making for interventional dila- interaction also the left ventricular function
tion ± stent placement versus surgery should might be impaired. The increase in right ven-
take the state of the repaired RVOT into account: tricular conduction delay aggravates the ven-
if no further surgery for the RVOT is planned, tricular dyssynchrony. Relevant ventricular
catheter intervention may be the treatment arrhythmia turned out to be associated with
modality of choice. If in the near future, for older age at operation, higher proportion of
example, a conduit replacement was planned ventricular fibrosis, and extended infundibu-
anyways, it may be performed sooner and now lotomy/ventriculotomy; ventricular dysrhyth-
15 would include bifurcation plasty also. mia, right ventricular dilatation, and QRS
Reconstructive procedures at the pulmonary prolongation are associated with the risk for
bifurcation can cause kinking by vessel remodel- sudden death (Gatzoulis et al. 1995a; b; Norgard
ing even years after primary surgical correction. et al. 1996). An overview of post tetralogy
Elongation of the right ventricular outflow patch repair RV p athophysiology was published by
plasty into the pulmonary bifurcation can cause Andrew Redington, whose group took the lead
distortion of the pulmonary arteries resulting in in these investigations (Redington 2006).
relevant Doppler sonographic pressure gradients. Increasing right ventricular dilatation in the
As a catheter intervention approach, balloon context of pulmonary regurgitation is the main
angioplasty alone can rarely relieve the stenosis, indication for reoperation. During recent years,
stent implantation is required; surgical reinter- criteria for intervention have been continuously
vention allows usually restoration of the geometry discussed: currently right ventricular enddiastolic
of the bifurcation (. Fig. 15.6). Replacement of volume on MRI >140 ml/m2 and a regurgitation
the patch by a new one and individual techniques fraction of >35 %. A right ventricular volume
to solve the distortion are recommended (Fraser index of less than 160 ml/m2 is said to allow nor-
et al. 1995), sometimes with reinsertion of the left malization of right ventricular volume and func-
pulmonary artery (McElhinney et al. 1998a). In tion after relief of volume load. Right ventricular
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
497 15
a b
..Fig. 15.6 Internal plasty for pulmonary bifurcation stenosis. a Potential outer appearance of bifurcation with
proximal branch stenosis (not to be externally dissected), may be anatomical or functional. b Excision of neighboring
RPA/LPA wall, creating a whole thickness defect, extended by stay stitches. c By suturing the edges of the defect, a V-Y
plasty is performed, enlarging both LPA and RPA ostia
dilatation (<120 ml/m2) is now often used as the Our own follow-up data in 101 patients after
cutoff value to preserve the right ventricular func- infant TOF repair could demonstrate that the
tion; in addition to right ventricular end systolic transatrial approach will not reduce the incidence
volume index, development of tricuspid valve of pulmonary valve replacement at 12 years. An
regurgitation or presence of branch pulmonary undefined subgroup of patients after transinfun-
artery stenosis will influence the decision for sur- dibular repair employing transanular RVOT
gery also (Lee et al. 2012). The optimal timing for patch also required valve replacement somewhat
surgical intervention remains often difficult to earlier, but this did not influence the result at
define. 12 years when compared to the exclusively
Pulmonary insufficiency is probably not only transatrially repaired patients (unpublished data
determined by the integrity of the valve anulus per Ziemer and Nagy 2010).
se but also by dilatation of the subvalvular infun-
dibular region. Resection and incision of muscle 15.2.3.2 Surgical Pulmonary Valve
bundles of the right ventricular outflow tract can Replacement
cause dilatation of the infundibulum that promotes In symptomatic patients with pulmonary regurgi-
anulus dilatation with valve insufficiency regardless tation, valve replacement is necessary; to prevent
of the approach chosen (del Nido 2006). right ventricular functional decline, indication is
498 G. Ziemer and R. Kaulitz
given even in asymptomatic patients (criteria see tricular fibrillation at the beginning of surgery.
above). The operation is performed employing The RVOT surgery will follow as described above
normothermic extracorporal circulation with the and below.
heart beating. Bicaval cannulation is preferred. In The right ventricular outflow tract is opened;
cases with severe scarring, the right atrium may the patch material after a transanular patch proce-
not be dissected and a large single atrial cannula dure is removed. In presence of an aneurysm prox-
used instead. As in these operations the heart is imal/apical to the old RVOT patch, the incision is
not displaced during the procedure, a two-stage carried out farther into it until good RV muscle is
cannula seems not to have an advantage over large reached; the distal incision can be shorter.
solitary venous cannula. After transatrial correction, the incision is
Residual intracardiac shunts (PFO, residual comparable to that for primary transinfundibular
ASD, residual VSD, PDA) have to be definitely repair (. Fig. 15.7a). For valve replacement, a pul-
excluded preoperatively. Otherwise they have to monary homograft is preferred; an aortic homo-
be corrected during cardiac arrest or induced ven- graft might be useful in patients with pulmonary
a b
15
..Fig. 15.7 Implantation of the homograft for pulmonary valve replacement in repaired TOF. a Prospective sutures for
anastomosis are marked as dotted lines. The distal suture line goes around the RPA, indicating that the corresponding
sinus of the valve to be implanted needs to be excised accordingly. The proximal anastomosis can posteriorly reach the
VSD patch insertion. The infundibulotomy may be extended into the ventricle if an aneurysm is present. b After creation
of the distal anastomosis, the posterior part of the proximal anastomosis was performed, assuring proper valve function
by saline injection. c To further preserve valve geometry and function, the proximal anastomosis is enlarged anteriorly
with a trapezoid patch. Plication of an aneurysmal infundibulum is performed at the same time («right ventricular
reduction plasty»)
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
499 15
hypertension but bears the risk of earlier and are valve incompetence and more frequently dis-
more extensive calcification. Pulmonary homo- tal conduit stenosis by fibrosis at the Contegra
grafts with small diameters (10–18 mm) have a pulmonary anastomosis leading to reoperation.
limited availability. Leaving the conduit too long can result in angu-
Since 1966, the homograft develops to the gold lation and conduit obstruction. In addition,
standard of right ventricular outflow tract recon- proximal aneurysmal dilatation in presence of
struction. However, reintervention has to be distal stenosis develops more often than in
expected because of shrinkage, calcification, and homografts or porcine xenografts (Holmes et al.
valve dysfunction, especially in young patients; 2012). The overall need for reintervention was
accelerated degeneration was described in patients calculated as 19 % after 4 years. Postoperative
less than 1 year at implantation. Freedom from administration of ASA is supposed to reduce
right ventricular outflow tract reintervention was neointimal proliferation and stenosis at distal
calculated as 67 % at 10 years and 47 % at 15 years anastomosis (Brown et al. 2006). For primary
(Yuan et al. 2008). Multiple right ventricular- insertion in patients less than 2 years of age,
pulmonary artery conduit revisions may be bovine jugular vein conduit revealed superior
required in patients now surviving to adulthood. long-term results when compared to both aortic
Different from conduit placement/replace- and pulmonary homografts in a large multi-insti-
ment in patients with pulmonary atresia or for/ tutional study (Poynter et al. 2013).
after Rastelli surgery, in TOF/pulmonary stenosis
patients requiring pulmonary valve replacement, 15.2.3.3 Percutaneous Pulmonary
the valve can be positioned in an almost orthotopic Valve Implantation (PPVI)
position. The technical details during implanta- The limited lifespan of right ventricular-
tion of the homograft valve include the placement pulmonary artery conduits is leading to multiple
of the proximal suture lines at the former valve conduit revisions historically all requiring cardio-
anulus, at the transition from ventricle to pulmo- pulmonary bypass. PPVI allows to extend conduit
nary artery. The distal suture lines or anastomosis life span and postpones the next surgical revision.
are placed first and then checked for proper orien- Thereby, it decreases the number of surgical pro-
tation and function of the valve by injecting nor- cedures required during lifetime. This strategy
mal saline into the graft. The proximal sutures limits right ventricular volume and/or pressure
often need a patch insertion at the anterior hemi- overload. Indications include right ventricular
circumference to avoid valve distortion dilatation in presence of moderate pulmonary
(. Fig. 15.7c). insufficiency, outflow tract obstruction with right
Sometimes the somewhat oversized homo- ventricular pressure greater than two thirds sys-
grafts have an excessive relative length due to the temic or compromised systolic right ventricular
length requirements for the commissures to function. An important selection criteria is a pos-
assure good valve function. In these patients the itive coronary safety evaluation to avoid to com-
valve needs to be oriented in a way that after exci- promise coronary artery perfusion by compression
sion of one Valsalva sinus of the valve, the right (McElhinney et al. 2010).
pulmonary artery will originate out of it close to The procedure is usually performed as percu-
the valve level (. Fig. 15.7b). taneous valve implantation. Prestenting prepares
Bioprosthetic valves—although most widely the landing zone for the implant and reduces the
used in the adult population in any valve loca- risk of stent fractures and valve dysfunction;
tion—provide limited durability in younger upper limit of the diameter is 21 mm (Bonhoeffer
patients because calcification and valve dysfunc- et al. 2000; Lurz and Bonhoeffer 2008). The
tion are accelerated. hybrid implantation is described in patients with
™Bovine jugular vein conduits (Contegra™) difficult catheter course or limited vessel access.
are available in adequate sizes (12–22 mm) and The subxiphoid incision allows directly passage
can yield a functionally good result (Brown et al. of the delivery system into the right ventricle
2006). Other reports showed them to be (Simpson et al. 2011). The transinfundibular
functionally worse when compared to homo-
implantation requires a sternotomy; on the other
grafts (Bautista-Hernandez et al. 2008; Meyns hand, a valve with a larger diameter can be
et al. 2004). Problems during midterm follow-up implanted (Schreiber et al. 2006; Berdat and
500 G. Ziemer and R. Kaulitz
Carrel 2006). Once complete sternotomy is indication for prophylactic surgical intervention
required for this «hybrid» appoach in a rester- with replacement of the ascending aorta.
notomy setting with an enlarged heart, the If the aortic root/ascending aorta diameter
advantage over a plain surgical approach may reaches more than 40 mm, MRI or CT should be
become rather questionable and may have to be performed annually.
reserved for very special cases where extracorpo- Another aspect of postoperatively persistent
real circulation is clearly detrimental. aortopathy is the aortic valve regurgitation
observed in 6–20 %; this might accompany the
aortic root dilatation (Mongeon et al. 2013; Niwa
15.2.4 Right Ventricular Aneurysm et al. 2002). An indication for surgical interven-
tion can develop; this should be reconstructive at
The concept of exclusion of the dyskinetic seg- first line (see 7 Chapter «Acquired Lesions of the
ment of the ventricular wall in patients with car- Aortic Valve», Sect. 24.3.2.2 and Sect. 24.5.1).
diomyopathy of various underlying causes was
used as the basis for right ventricular outflow tract
plasty with resection of the hypokinetic infundib-
ular wall to reduce the right ventricular volume. 15.3 Tetralogy of Fallot
Del Nido describes the technique performing an with Pulmonary Atresia
incision along the right ventricular anterior wall (TOF/PA)
down to the apex if deemed necessary, plicating
and resecting the thinned anterior wall («right 15.3.1 Nomenclature
ventricular remodeling,» del Nido 2006). In a ran- and Pathoanatomy
domized trial, this right ventricular remodeling in
addition to pulmonary valve replacement did not Morphologic characteristics of TOF/PA are:
provide any additional benefit with regard to right 55 Large malalignment ventricular septal
ventricular ejection fraction or midterm func- defect.
tional result (Geva et al. 2010). 55 Anterior deviation of the infundibular
septum, hypoplasia of the infundibulum (as
in classical TOF) and blind-ending outflow
15.2.5 ortic Root Dilatation
A tract, or anteriorly deviated and overriding
and Aortic Valve Insufficiency aorta, that is in contact with the anterior right
After Tetralogy Repair ventricular wall, the infundibulum may be
absent (as in truncus arteriosus).
In conotruncal malformations, the aortic root is 55 Right ventricular hypertrophy.
15 primarily enlarged; after 10–12 years after surgi-
cal repair during infancy, the indexed aortic root The intracardiac anatomy resembles that of
diameter tends to normalize (Francois et al. 2010). tetralogy of Fallot. It is known from fetal echocar-
Structural abnormalities similar to those in diography that in some of these patients, the right
patients with Marfan syndrome have been ventricular outflow tract was initially patent and
described in those patients operated during child- became functionally and later anatomically atretic
hood (Tan et al. 2006). Nearly one third of adult during fetal life. An alternative terminology
patients are expected to have an aortic root diam- names this malformation «pulmonary atresia/
eter of more than 40 mm after TOF correction. VSD.» While this term describes the main fea-
Aortic root dilatation can reach a diameter up to tures of the malformation sufficiently, regarding
45–50 mm in adolescent or adult patients. In a the intracardiac anatomy, it is rather nonspecific.
multicenter study in adults with TOF correction, Therefore, in accordance with Van Praagh, we
29 % revealed aortic root a diameter above 40 mm; prefer the term tetralogy of Fallot with pulmonary
the indexed observed to expected ratio was greater atresia, being one end of the tetralogy spectrum of
than 1.5 in nearly 7 % (Mongeon et al. 2013). which the other end is marked by double outlet
Aortic root complications as aortic dissection are right ventricle/pulmonary stenosis (TOF type).
an extremely rare complication (Kim et al. 2005; Tetralogy of Fallot with pulmonary atresia
Rathi et al. 2005). This explains that there is no sometimes coexists with microdeletion 22q11.
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
501 15
15.3.1.1 Intracardiac Anatomy There is a wide spectrum of the pulmonary
The right ventricular outflow tract may by artery morphology—from normal developed pul-
obstructed by an imperforate pulmonary valve monary arteries with an atretic connection to the
(membranous atresia). More often a muscular right ventricle and ductal blood supply to absent
obstruction is present either at the os infundibuli intrapericardial pulmonary arteries and multiple
or immediately subvalvular. While there is no pulmonary collateral arteries from the aorta
luminal connection from the right ventricular (. Fig. 15.8).
outflow tract to the pulmonary artery, most often Rabinovitch et al. (1981) describe three sources
the pulmonary trunk at its blind origin is in tissue of arterial blood supply with different connection
continuation with the epi-myocardium. to the pulmonary arteries: bronchial artery collat-
The ventricular septal defect may be consid- erals take their origin from bronchial arteries and
ered perimembranous; with anterior extension are connected to pulmonary arteries inside the
due to the malalignment of the septum in TOF lung. Direct aortic collaterals arising from the
type, it can create a doubly committed situation. In descending aorta supply a lobe or a segment after
rare cases the septal defect might become restric- entering the lung at the hilum. Indirect aortic col-
tive if tricuspid valve tissue tags cover the defect; laterals arise from major branches of the aortic
this situation would become comparable with pul- arch (e.g., subclavian artery or internal mammary
monary atresia with intact septum. The aorta is in artery) and connect to the central pulmonary
overriding position, rarely connected only to the arteries. The collateral vessels may become stenotic
right ventricle, leading to a situation as in double at the site of anastomosis with the pulmonary
outlet ventricle. There is no correlation between artery; this protects the vascular bed from high
intracardiac anatomy and morphology of pulmo- flow and pressure damage by otherwise unob-
nary artery branching or pulmonary arterial sup- structed flow. Reduction of pulmonary blood flow
ply (Thiene et al. 1977; Anderson et al. 1991). in early infancy will decrease the number of alveoli
of the corresponding part of the lung in almost all
15.3.1.2 Morphology patients; lung function tests demonstrated a
of the Pulmonary Arteries smaller lung volume only in a few patients.
In early pregnancy, around the 40th day, the vas- In the presence of major aortopulmonary col-
cular plexus of the lung buds connect to the sys- lateral arteries (MAPCA), the central and periph-
temic segmental arteries; these originate from the eral pulmonary arteries are hypoplastic (Haworth
dorsal aorta. Additionally, there is antegrade and Macartney 1980). Vessel stenosis might be
blood flow from the right ventricle to the pulmo- found at the aortic origin or at the anastomosis
nary arteries originating from the sixth branchial with the pulmonary artery in nearly 60 %. The
arches; during this period, there is obviously a vessel origin is mainly found at the descending
dual blood supply for the pulmonary paren- thoracic aorta at the level of the left main bron-
chyma. As the segmental arteries normally invo- chus (in left aortic arch) or carina (in right aortic
lute, the only pulmonary blood supply is provided arch). MAPCA may also arise from the branches
by the pulmonary arteries. of the aorta (e.g., subclavian artery) or rarely from
In patients with TOF-pulmonary atresia, this the common aortic trunk. They connect to the
development is disturbed as part of the cardiac central pulmonary arteries, to lobar or segmental
malformation and characterized by collateral sys- pulmonary arteries within the lung, or any pul-
temic arterial blood supply in presence of a wide monary artery supplying the lung independently.
spectrum of pulmonary artery morphology. In the In this way MAPCA may supply nearly 45 % of
presence of hypoplastic or even absent central pul- the bronchopulmonary segments directly and
monary arteries, the segmental arteries persist pro- 50 % via the central pulmonary arteries, while 5 %
viding a variable amount of pulmonary blood of the lung parenchyma have a double blood sup-
supply. The aortopulmonary collaterals can con- ply (Haworth 1990).
nect to central pulmonary artery, to lobar or seg- Based on the pulmonary artery anatomy and
mental pulmonary arteries, or enter the lungs blood supply (dominant via native pulmonary
without connection to the pulmonary arteries; arteries or MAPCA, rarely dual), five subgroups
sometimes they perfuse parts of the contralateral of pulmonary atresia have been defined for clas-
lung also (Haworth and Macartney 1980). sification of pulmonary artery morphology :
502 G. Ziemer and R. Kaulitz
a b c
..Fig. 15.8 Morphology of pulmonary perfusion in tetralogy of Fallot/pulmonary atresia. a, b group I, ductal-dependent
pulmonary blood supply, no MAPCA, with or without an identifiable pulmonary trunk (unifocal lung perfusion); c group II,
hypoplastic intrapericardial pulmonary arteries, connected to all pulmonary segments; multiple segments will have dual
blood supply; d group III, extremely hypoplastic intrapericardial pulmonary arteries with multiple MAPCA-dependent
blood supply of the lung segments; e group IV e, absent intrapericardial pulmonary arteries; pulmonary blood supply
exclusively by MAPCA; f group V, absence of any angiographically identifiable pulmonary arteries or MAPCA, pulmonary
blood supply via diffuse small collateral vessels only. While group I may be considered a unifocal lung perfusion, groups
II–V represent situations with multifocal lung perfusion (Modified after Castaneda et al. (1994) and completed after Griselli
et al. (2004); both used with permission)
15 55 Group I (. Fig. 15.8a, b): ductal-dependent MAPCA, pulmonary blood supply via diffuse
pulmonary blood supply, no MAPCA, with small collateral vessels only (Griselli et al.
or without an identifiable pulmonary trunk 2004).
(unifocal lung perfusion).
55 Group II (. Fig. 15.8c): hypoplastic intraperi- While group I may be considered a unifocal
cardial pulmonary arteries, connected to all lung perfusion, groups II–V represent situations
pulmonary segments; multiple segments will with multifocal lung perfusion.
have dual blood supply.
55 Group III (. Fig. 15.8d): extremely hypoplas-
tic intrapericardial pulmonary arteries with 15.3.2 Clinical Presentation
multiple MAPCA-dependent blood supply of and Diagnosis
the lung segments.
55 Group IV (. Fig. 15.8e): absent intrapericar- Onset and severity of clinical symptoms will
dial pulmonary arteries; pulmonary blood depend on the type of pulmonary blood supply;
supply exclusively by MAPCA. the interventricular communication is large with
55 Group V (. Fig. 15.8f): absence of any angio- obligatory right-to-left shunt. If pulmonary blood
graphically identifiable pulmonary arteries or flow is ductus dependent, closure of the ductus
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
503 15
after birth will rapidly cause cyanosis, hypoxemia, vide information on origin and course of
acidosis, and death if not treated; prenatal diagno- MAPCAs; this diagnostic information is also
sis of pulmonary atresia will allow starting prosta- provided by computerized tomography (Lofland
glandin infusion immediately after birth, 2004). However, complete clarification of pulmo-
preventing arterial desaturation. In presence of nary blood supply can only be obtained by angi-
MAPCAs, the clinical presentation can vary ography. This includes selective catheterization
between moderate and no cyanosis at all. The lat- of the ductus and MAPCAs at any level of origin
ter even with congestive heart failure, if the from the aorta. Retrograde pulmonary venous
MAPCAs are unobstructed. Some of the MAPCAs wedge angiography allows the identification of
may become stenotic or occlude leading to an central pulmonary arteries if they are present.
increase in cyanosis during infancy or childhood. Pressure measurement and test occlusion of the
Large unrestrictive MAPCAs can cause pulmo- MAPCAs identify the perfusion pattern of the
nary hypertension in the corresponding lung seg- lung segments and the individual contribution to
ment with secondary pulmonary vascular oxygenation. For surgical decisions, the perfu-
obstructive disease. Hyperperfused and under- sion pattern of all 20 lung segments has to be
perfused segments can coexist adjacent to each investigated.
other. As in other cyanotic cardiac anomalies,
additional aortopulmonary collateral blood sup-
ply may develop. While the vessels of this addi- 15.3.4 Surgical Management
tional pulmonary blood supply connect to the of Tetralogy of Fallot
pulmonary arteries at precapillary level, MAPCAs with Pulmonary Atresia
connect at the hilum or farther upstream at seg-
mental level. 15.3.4.1 Ductus-Dependent
Clinical findings are not so impressive; a con- Pulmonary Blood Flow
tinuous murmur might be audible in patients Definitive primary repair is possible in ductus-
with relevant MAPCAs, more localized in patients dependent pulmonary lung perfusion with con-
with just patent ductus arteriosus. fluent, sufficiently developed pulmonary
Historically, it was said that Helen Taussig’s arteries. Closure of the ductus or stenosis at the
detection of a murmur in her TOF patients who pulmonary end might induce a critical situation
were doing well gave her the idea that an artificial with cardiac decompensation; in a critical situa-
ductus arteriosus, the later Blalock-Taussig shunt, tion, palliation with a BT shunt may be pre-
would be beneficial for those patients without a ferred. Dilatation and stenting of the stenotic
murmur. The idea most probably originated in lis- ductus was suggested also (Gibbs et al. 1992,
tening to MAPCAS rather than to the rare long- Gewillig et al. 2004). In patients with bilateral
term patent ductus arteriosus in unifocal lung ductus and discontinuity of the pulmonary
perfusion or a ductus in any other severe form of arteries, primary neonatal repair is recom-
tetralogy (Richard Van Praagh, personal commu- mended; the discontinuity can be bridged by a
nication). PTFE conduit with mobilization of both pulmo-
nary arteries into the hilus. If in older infants a
homograft is used, its intact pulmonary bifurca-
15.3.3 Diagnostic Procedures tion should be preserved and may be used for
reconstruction of the bifurcation; the risk of
Radiological findings represent the typical con- early homograft stenosis has to be considered
figuration of the heart («coeur-en-sabot,» French (Shanley et al. 1993).
for «Boot-shaped heart») with the lack of the pul- Primary repair is possible if 15–18 lung seg-
monary trunk and irregular pulmonary vascular ments are in connection to the central pulmonary
markings. arteries. The postoperative right-to-left ventricu-
Echocardiography allows the definition of the lar pressure ratio is of prognostic value. If only
intracardiac anatomy; often it is possible to iden- 11–14 segments could be connected, there is a
tify the central pulmonary arteries in continuity. high risk for right ventricular pressure overload
Cardiac magnetic resonance imaging can pro- after corrective surgery. Patient selection after
504 G. Ziemer and R. Kaulitz
careful and extensive diagnosis is a quite impor- Our preference for PTFE tubes in primary con-
tant point. Less than 10 segments do not allow for duit placement in babies is the relative ease at
a subsystemic right ventricular pressure (Reddy reoperation for the unavoidable conduit chance in
et al. 1995). any case.
Primary repair should be electively per- Reconstruction of the right ventricular out-
formed in the second week of life. In patients flow tract allows potentially a better growth of
with a membranous atresia or in those with a the pulmonary arteries compared to a shunt
short muscular atresia but tissue continuity (the procedure but needs cardiopulmonary bypass
majority of patients), surgical correction is com- and bears a higher risk. The risk of pulmonary
parable with transinfundibular correction of artery distortion after BT shunt is avoided
uncomplicated tetralogy of Fallot avoiding pri- using a central shunt which we prefer in
mary conduit implantation. Restenosis might patients with small-sized pulmonary arteries
develop at «anular» level, which even after the anyways.
best repair remains the most narrow and there- If there is coarctation of the pulmonary artery
fore most critical part of the RVOT reconstruc- associated with closure of the ductus, extended
tion. A longitudinal infundibular incision will be incision into the pulmonary branch 3–4 mm dis-
extended into the pulmonary trunk through the tal to the insertion of the ductus is necessary (see
atretic valve or muscular segment. In order to . Fig. 15.4a)
reduce the risk of early reoperation in patients
with muscular atresia, we mobilize the hypoplas- 15.3.4.2 Surgical Management
tic pulmonary trunk a couple of millimeters, in Multifocal Lung Perfusion
divide it at mid-length, and reanastomose it into Depending on the individual anatomic situa-
the distal end of the infundibulotomy with tion, different surgical strategies are conceiv-
absorbable sutures (e.g., PDS). A transanular able. The decision has to be made in early
patch completes the reconstruction, still leaving infancy as some MAPCAs might become ste-
the «anulus» as the most narrow but sufficiently notic or atretic leaving segments without perfu-
wide part for the time being. Sufficient growth of sion, while other lung segments might develop
the autochthonous tissue has to be anticipated. pulmonary vascular obstructive disease with
Before RVOT reconstruction, closure of the irreversible pulmonary hypertension already
VSD follows the procedure described in tetral- during childhood. The aim is to establish ante-
ogy of Fallot with pulmonary stenosis or truncus grade pulmonary perfusion from the central
arteriosus. pulmonary arteries for as many lung segments
In patients with complete atresia or severe as possible.
hypoplasia of the pulmonary trunk, implantation The surgical options for treatment of multifo-
15 of a conduit is necessary. For this, only limited cal lung perfusion are:
mobilization of the pulmonary arteries will be 55 Palliation with peripheral shunt procedures
performed; as the branch pulmonary arteries are 55 Staged peripheral unifocalization followed
often small, additional elongation/distortion by by central unifocalization and conduit place-
mobilization should be avoided. The conduit ment from the right ventricle with or with-
might be a homograft or xenograft or—for us out complete VSD closure (Puga et al. 1983,
preferable—a PTFE tube of 10–12 mm diameter; 1989; Sawatari et al. 1989; Sullivan et al.
a functioning valve at the right ventricular out- 1988)
flow tract is not necessary during infancy. The 55 Primary central operation and later unifocal-
main disadvantage for any neonatal or early ization with the option of catheter-based
infancy conduit implantation is the need for con- interventions as dilatation or coil occlusion:
duit replacement after 4–5 years because of out- with aortopulmonary shunt insertion (Iyer
growth. Comparing long-term results after and Mee 1991; Watterson et al. 1991) and
homograft and xenograft conduit implantation in with right ventricular to pulmonary artery
patients less than 2 years of age, a multicentric conduit without VSD closure (Rome et al.
Congenital Heart Surgeons’ Society Study more 1993)
recently showed advantages for the bovine jugular 55 Primary total correction (Lofland 2004;
vein xenografts employed (Poynter et al. 2013). Reddy et al. 1995)
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
505 15
kPalliative shunt procedure (Watterson et al. 1991). Bilateral thoracotomies
Peripheral shunt procedures have to be integrated are used for peripheral unifocalization as subse-
in the further surgical strategy; their indication is quent procedures. Later on right ventricle to pul-
mostly seen in patients with absent intrapericar- monary artery conduit with or without VSD
dial pulmonary arteries with severe or increasing closure may follow.
cyanosis. This patient group has the less favorable Boston Children’s Hospital favored the
prognosis (Griselli et al. 2004). approach of a primary conduit procedure during
infancy (Rome et al. 1993) choosing valved or
kStaged peripheral unifocalization nonvalved conduits for right ventricle to pulmo-
The concept of stepwise unifocalization was prop- nary artery connection to encourage growth of
agated by Francisco Puga (Puga et al. 1983, 1989), the central pulmonary arteries. The usually larger
Jarda Stark and Marc de Leval (Sullivan et al. size of this connection should allow for more pro-
1988), Yasuharu Imai (Sawatari et al. 1989), and nounced pulsatility, eventually giving a better
Yasunaru Kawashima (Yahagira et al. 1996). impulse for PA growth. A further benefit may be
Staged unifocalization tries to interconnect as the advantage of a streaming effect, leading mainly
many intrapulmonary arteries as possible in a desaturated venous blood into the pulmonary
stepwise procedure—directly or by using pros- arteries.
thetic material. The created confluence of this
interconnection is anastomosed to an aortopul- kPrimary unifocalization
monary shunt. Unifocalization on both sides of Primary unifocalization as a corrective procedure in
the lung is performed via lateral thoracotomies. early infancy (age 3–4 months) was favored by
In a later procedure, a right ventricular to pulmo- Frank Hanley and Mohan Reddy (Reddy et al.
nary artery conduit is implanted as a T- or 1995). Basic principle is complete mobilization of all
Y-shaped prosthesis/graft. The VSD might be MAPCAs (. Fig. 15.9). They preferred one-stage
closed completely or with a fenestrated patch. repair via median sternotomy. Other groups (Moritz
Collateral vessels have to be occluded by coils et al. 1996; Luciani et al. 1997) recommended bilat-
as they can support relevant left-to-right shunt. eral thoracotomies in the fourth intercostal space
If VSD closure (even only fenestrated) is the («clamshell» incision) establishing a more radical
positive endpoint in these staged unifocalization approach for the extensive dissection of the lung
procedures, success varies in the studies cited hilus. Their patients, however, were older.
from 12 to 60 % only. Before going on bypass, complete dissection of
the hilum in both lungs is required. Dissection of
kPrimary central procedures pre- and retrobronchial MAPCA is facilitated by
In the presence of central pulmonary arteries, anterior luxation of the lungs, if hemodynamically
with or without continuity, a central procedure as tolerated. As the majority of the MAPCAs originate
a primary approach is the first step of choice. from the middle part of the descending aorta, sinus
Thereby not only antegrade flow into the true pul- transversus incision of the posterior pericardium
monary arteries is established but also access for between the ascending aorta and the vena cava
potential catheter intervention made possible. It superior gives access to the infracarinal triangle.
may consist of a central aortopulmonary shunt or The collaterals are cut immediately at their aortic
of a RV to PA conduit. Both procedures may be takeoff. These vessels own a fragile structure, simi-
extended with central PA patch plasties if an addi- lar to a mixture of vein and ductal tissue, and they
tional localized stenosis cannot be taken care of require most delicate handling. After completion of
with the prosthetic anastomosis alone. the dissection and transection, they might be
Royal Children’s Hospital in Melbourne pre- guided into the intrapericardial space by separate
ferred a primary central aortopulmonary shunt retrophrenical but prehilar pericardial incisions.
(Iyer and Mee 1991). As in extremely hypoplastic Hanley and Reddy recommend to dissect,
central pulmonary arteries the shunt procedure disconnect, and unifocalize as many collater-
might be technically difficult, they introduced als as possible before establishing cardiopul-
what was called the «Melbourne shunt»: a direct monary bypass, only employing it earlier when
end-to-side anastomosis of the hypoplastic main desaturation and/or hemodynamic instabil-
pulmonary artery with the ascending aorta ity requires it (Reddy et al. 1995). Lofland
506 G. Ziemer and R. Kaulitz
recommends to perform the complete dissection Richard Jonas prefers a minimal mobilization
and unifocalization already during normother- of the MAPCA and an end-to-side connection
mic extracorporal circulation with the heart beat- with the central, intrapericardial pulmonary
ing (Lofland 2004). He claims superior and more arteries (Jonas 2004). Another approach avoids
extensive dissection on one hand with a safe envi- any manipulations of the MAPCA and preserves
ronment on the other. their native/original course to the lung by isolat-
ing the aortic segment with its MAPCA takeoffs.
This aortic segment is then connected to the right
All authors emphasize the importance of a ventricle using a conduit. The aorta is recon-
customized approach in any individual patient structed by using an adequate aorto-aortic vascu-
to unifocalize the collaterals and reconstruct lar graft (Abella et al. 2004).
the central pulmonary arteries at the same
time. kSeparation of the circulation with complete
or fenestrated VSD patch
The ultimate goal of surgery is to obtain a complete
separation of systemic and pulmonary circulation
The pattern of anastomosis favored by Reddy with occlusion of any intracardial connections.
and Hanley shows a long segment side-to-side Limiting factor is the capacity/cross section of the
junction of the vessels (see . Fig. 15.9) to gain a pulmonary vascular bed available for the right ven-
larger circumference to accommodate the cardiac tricular output. The resistance of either or both the
output. The anastomosis between right ventricle proximal unifocalized hypoplastic vessels or dis-
and pulmonary artery and any intracardiac pro- tally the arterioles may not accommodate a cardiac
cedure if necessary is performed during cardio- output at subsystemic pressures. The postoperative
plegic cardiac arrest. increased pressure ratios of the right and left ven-
tricles become the predictors of the mid- and long-
lasting outcome of the patients. Therefore,
management of the VSD is the key to successfully
attack this problem. In contrast to the tetralogy of
Fallot with a stenosis of the pulmonary artery and
a dynamic and regressive component of the intra-
operative high right ventricular pressure, the intra-
operative and early postoperative pressure of the
unifocalized vessels is anatomically and function-
ally fixed. A prediction of the postoperative right
15 ventricular pressure due to the connected lung seg-
ments (at least 10 of the 20 segments; Reddy et al.
1995) or other indices calculating the area or cross
section of the lung vessels (e.g., Nakata index;
Nakata et al. 1984) is vague and of limited value for
the final decision.
An assessment based on a preoperative angi-
ography is, in principle, suitable to estimate the
option of a definitive closure preoperatively. The
final decision has to be taken by the measure-
ments of the intraoperative pressure conditions of
the ventricles and/or great arteries.
..Fig. 15.9 Suggested patterns of unifocalization. In case of suprasystemic values of the right
Right: end-to-side anastomosis. This kind of anastomosis ventricle, the VSD patch should be fenestrated
requires only minimal mobilization of the MAPCA («major or—if necessary in very rare cases—be removed
aortopulmonary collateral arteries»). Left: a side-to-side
anastomosis after extended complete mobilization of
completely. Richard Jonas suggested fenestration
MAPCA, using the MAPCA itself as an augmentation of the of the VSD patch only if RV pressures exceed
central pulmonary artery 110–120 % of systemic values (Jonas 2004).
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
507 15
Mohan Reddy and Frank Hanley based their arteries as possible by enhancing antegrade flow.
decision for VSD management on a complicated MAPCA are incorporated only if deemed abso-
but in their hands useful intraoperative procedure: lutely necessary for a significant part of the pul-
after completion of the unifocalization and the monary perfusion, in our hands rather reluctantly.
distal connection of the future conduit, they per-
form an isolated lung perfusion via the conduit.
The flow of the lung perfusion pump should equal 15.4 Tetralogy of Fallot
the theoretical cardiac output of the patient. If the with Absent Pulmonary Valve
pulmonary vascular bed can accept the calculated («Absent Pulmonary Valve
cardiac output with a pulmonary artery pressure Syndrome» APVS)
of <25 mmHg, the VSD closure can be tolerated
hemodynamically. In case of pressure values 15.4.1 Definition
between 25 and 40 mmHg, a fenestration should
be created. A VSD closure is contraindicated at Absent pulmonary valve syndrome is a rare car-
values above 40 mmHg (Reddy et al. 1997). diac anomaly. The most common association (in
A primary elective fenestration of a VSD patch >80 %) is given with tetralogy of Fallot. Extremely
is performed only in rare cases. Even with unfa- rare is the isolated absent pulmonary valve syn-
vorable results of the preoperative angiography, drome or the association with tricuspid atresia.
different and advantageous conditions at the end Two to 6 % of patients with TOF present with
of the operation may occur. With suprasystemic absent pulmonary valve syndrome. It is character-
pressure of the right ventricle, a secondary fenes- ized by rudimentary, hypoplastic pulmonary
tration is created using an aortic puncher in nor- valve cusps resembling a wide-open fibrous ring
mothermia and electrically induced ventricular at the level of an almost normal-sized anulus. It is
fibrillation. The diameter of 2.8 mm in neonates associated with severe dilatation of the main pul-
and 4 mm in infants allows a sufficient decom- monary trunk and branch pulmonary arteries in
pression of the right ventricle without the risk of a presence of severe pulmonary regurgitation and
lung flooding later on. In case of an angiographi- mild to moderate pulmonary stenosis; tracheo-
cally frugal pulmonary vascular bed to start with, bronchial abnormalities result from bronchial
we do not perform a VSD patch closure at all. compression by the pulmonary arteries which
may lead to respiratory compromise already in
15.3.4.3 Destiny of the Unifocalized the immediate postnatal period. On the other
MAPCAs hand, patients with APVS may only have minor
The original enthusiasm for unifocalization based respiratory symptoms and moderate cyanosis for
on MAPCA/PA reconstruction was significantly several years.
dampened by reports of angiographic long-term
results of the Royal Children’s Hospital in
Melbourne (d’Udekem et al. 2005; Nørgaard et al. 15.4.2 Anatomy
2006). Sixty-five patients were examined after a
complete repair partially based on MAPCAs. In patients with absent pulmonary valve syn-
Comparing former and current angiographic drome and TOF, the intracardiac anatomy is char-
results, constantly unfavorable outcomes of the acterized by the large subaortic malalignment
incorporated MAPCAs were shown. Either most of VSD, anterior deviation of the conal septum, and
the vessels which were open early postoperatively moderate right ventricular outflow tract obstruc-
were occluded now or did not show any growth tion with a somewhat hypoplastic, fibrotic anulus
neither relatively nor absolute over the time. and rudimentary cusps and moderate-to-severe
In default of any long-term studies to date, a pulmonary insufficiency. The aneurysmal dilata-
competent judgment regarding the relevance of a tion of the pulmonary arteries might extend into
perfusion of individual MAPCA or MAPCA in its first- and second-order branches. Severe bron-
general is difficult. It is common sense to first con- chial obstruction might result from the long-
nect the original pulmonary arteries independent standing in utero compression by the tortuous
of their size to develop as much diameter and dilated pulmonary arteries; this might extend
peripheral distribution of original pulmonary from the main stem bronchi into the hilar regions
508 G. Ziemer and R. Kaulitz
(Milanesi et al. 1984; Rabinovitch et al. 1982). At (Taragin et al. 2006). Thoracic CT can complete
birth severe respiratory compromise might the preoperative airway assessment (Vincenti
develop and is associated with significant perina- et al. 2012).
tal morbidity and mortality. Urgent neonatal cor- Prenatal diagnosis has become important.
rective surgery might be indicated including Fetal case series described prenatal and postnatal
pulmonary artery arterioplasty. survival rates of less than 20 %. Cause of death
Absence of ductus arteriosus is a rather typical includes termination of pregnancy, fetal heart
finding in APVS (Emmanouilides et al. 1978). failure, respiratory decompensation, and chromo-
Ductal agenesis was thought to be protective in somal abnormalities (Wertaschnigg et al. 2013).
the setting of severe pulmonary regurgitation and
large VSD as a patent duct would result in huge
aortopulmonary shunting, the regurgitant absent 15.4.4 Therapy
pulmonary valve acting as in severe aortic insuf-
ficiency. Consequently, severe biventricular vol- 15.4.4.1 Preoperative Management
ume overload and heart failure would develop Compression of the tracheobronchial tree can
(Zach et al. 1979). It was assumed that the elevated result in severe respiratory distress before surgery
right ventricular pressure in utero leads to under- requiring intubation and high-pressure ventila-
development of the pulmonary valve and the to- tion. The respiratory situation might become
and-fro flow across the right ventricular outflow worse because of secondary barotrauma and the
tract and consecutive dilatation of the pulmonary risk of atelectasis, obstructive emphysema, or
arteries that cause tracheobronchial compression pneumothoraces. Special ventilation modalities
and malacia. The most severe compromise of pul- including belly or prone position management are
monary vasculature and airway development dur- used (Takabayashi et al. 2005). Emergency ster-
ing fetal life may result in airway occlusion and notomy and temporary chest wall plasty might
fluid retention in the distal lung segments. widen the intrathoracic space and might improve
In association with tetralogy of Fallot, micro- the preoperative ventilatory status (Heinemann
deletion 22q.11.2 is the most common chromo- and Hanley 1993). Ultimate therapeutic option is
somal abnormality. the venovenous extracorporal membrane oxygen-
ation for preoperative stabilization (Kirshbom
and Kogon 2004).
15.4.3 Diagnostic Procedures
15.4.4.2 Surgical Strategies
Intensity of diagnostic procedures depends on the Corrective surgery is planned as a one-stage
clinical presentation: some infants present as repair to treat cardiac and bronchial disease.
15 «pink» Fallot without respiratory symptoms even While TOF repair is a standardized procedure
if pulmonary artery dilatation is present; later (7 Sect. 15.1.6.2), extension of reduction pulmo-
they might become symptomatic with airway dis- nary arterioplasty depends on the varying degree
ease or bronchospasm. Others develop severe of vessel dilatation and actual airway obstruction.
respiratory compromise and become ventilator We prefer the extensive posterior and anterior
dependent as early as in the immediate postnatal reduction arterioplasty as suggested by Roger
period. Chest radiograph will demonstrate car- Mee’s group (Stellin et al. 1983) (see . Fig. 15.10).
diac enlargement, lobar atelectasis, and areas of Corrective surgery is performed with cardio-
obstructive emphysema. In neonates and infants, pulmonary bypass and moderate hypothermia
the intracardiac anatomy is comprehensively and intermittent cold-blood cardioplegia or as in
described by echocardiography; a cardiac cathe- our preference for neonates and infants in limited
terization is not indicated. deep hypothermic circulatory arrest and hypo-
Older, less symptomatic patients from both thermic cardiac arrest. Aortic cannulation for car-
the bronchial and the cardiac side may be candi- diopulmonary bypass should be performed far
dates for elective bronchoscopy defining airway distal in the ascending aorta or better in the prox-
involvement, also. imal aortic arch. This may allow to transsect the
MRI can be used to illustrate the cardiac anat- ascending aorta in order to facilitate pulmonary
omy and extension and pattern of airway disease artery reconstruction.
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
509 15
Surgical treatment of the pulmonary arteries trunk will also be plicated posteriorly. It may
depends on the varying degree and extension of even be transsected and somewhat shortened.
pulmonary dilatation. Both right and left pulmo- The reconstruction of the right ventricular out-
nary arteries as well as the main pulmonary flow tract can be managed in different ways, the
artery should be circumferentially dissected in individual surgeon’s preference for tetralogy
part before going on bypass and completely repair: transanular patch, monocusp valve,
before cross clamping the aorta. Pulmonary homograft valved conduit, Contegra jugular vein
bifurcation should be opened by an anterior valved conduit, or Gore-Tex conduit. In infancy a
y-shaped incision; the anterior wall of main, left, valved conduit is not really necessary even in sit-
and right pulmonary artery is partially resected uation of respiratory distress; an oversized homo-
and the posterior wall plicated, starting even far- graft or Contegra conduit might compromise
ther distal in the pulmonary hilum than the ante- tracheal bifurcation. Reduced infundibular resec-
rior resection did reach (. Fig. 15.10). While an tion is performed for relief of obstruction of the
anterior reduction plasty only reduces pulmo- right ventricular outflow. In neonates and infants,
nary artery diameter, the addition of the poste- we prefer transanular patch to ensure luminal
rior inner plication not only further diminishes reconstruction. Patients over 5 years receive
the diameter in a symmetric way, more impor- valved homografts or bovine jugular veins.
tantly it separates and elevates the pulmonary Decisions between 1 and 5 years, rarely to be
artery from the affected bronchi. During vessel made, may be again very individual.
closure, the lumen of the pulmonary artery Intraoperative bronchoscopy allows for assess-
should be calibrated by Hegar dilatators to pre- ment of the central respiratory tract and residual
vent overdoing the diameter reduction. In neo- tracheobronchial obstruction; if indicated airway
nates and infants, we use 8 mm Hegars. A decompression can be achieved by retrosternal
reduction to the correctly age and weight matched suspension of the pulmonary artery as long as not
diameters of 5–6 mm would increase the risk of dissected circumferentially (Bove et al. 1972;
iatrogenic obstruction. The distal pulmonary Alsoufi et al. 2007).
a b
..Fig. 15.10 Complete circumferential reduction plasty of pulmonary artery employing endoluminal posterior plication
and anterior resection. a The planned excision of the anterior wall (dotted line) extends from hilum to hilum in right and
left pulmonary artery and also in a T-shaped fashion into the pulmonary trunk/main pulmonary artery. Decision-making
for how much to resect as well as for the actual reconstruction may be facilitated by transsecting and re anastomosing the
ascending aorta. b After generous endoluminal everting plication of the posterior wall and resection of the anterior wall,
the reconstruction is completed by direct suture of the anterior wall, employing absorbable suture material. Introducing a
somewhat oversized Hegar dilatator into the arteries during reconstruction may secure proper sizing (not shown in figure)
(Modified from Stellin et al. (1983); with permission by Elsevier)
510 G. Ziemer and R. Kaulitz
a b
Subaortic conus Subpulmonary conus
Subaortic conus Subpulmonary conus
Posterior
Ventricular septal defect Anterior Ventricular Posterior
Trabeculum septomarginalis septal defect Anterior
Trabeculum septomarginalis
..Fig. 15.11 Double outlet right ventricle with the interventricular communication between the anterior and posterior
limb of the septomarginal trabeculation. The muscular outlet septum is a right ventricular structure and can fuse with the
anterior limb of the septomarginal trabeculation in subaortic VSD a or the posterior limb in subpulmonary VSD b
512 G. Ziemer and R. Kaulitz
15
..Fig. 15.12 Schematic drawing of RVOT in coronal plane in DORV with subpulmonary VSD. Dotted lines show the
course of suture lines of the future tunnel patch. a In Kawashima operation the intracardiac tunnel patch is running pos-
terior to the pulmonary valve along the subarterial conus. b In Patrick-McGoon and Doty operation, the intraventricular
tunnel lies anterior to the pulmonary artery
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
515 15
contraindication for this approach. Thus, the intra- Transposition and D-Transposition of the Great
cardiac conduit runs between tricuspid and pulmo- Arteries», Sect. 19.2.5.2. Coronary artery anomalies
nary valve and needs sufficient space between them: and the position of the great arteries in DORV
at least half of the aortic diameter in order to pre- can complicate the procedure further; as said
vent creation of subaortic o bstruction. Surgical above, patients with a side-by-side position of
access for this operation is an infundibular incision the great arteries may be more suitable for intra-
that allows inspection of the intervalvar space and cardiac rerouting repair. With side-by-side posi-
also the sub AV-valve apparatus to exclude tion of the great arteries, the Lecompte
straddling chordae (see 7 Chapter «Anomalous
maneuver should be combined with a transloca-
Pulmonary Venous Connections and Congenital tion of the pulmonary bifurcation to the right
Defects of the Atria, the Atrioventricular Septum, and pulmonary artery; the original pulmonary
the Atrioventricular valves», Sect. 12.5). The tunnel bifurcation can be closed directly or with a
patch should be redundant to prevent subaortic ste- patch. In the rare situation of posterior position
nosis. Its suture line may be reinforced and/or com- of the aorta, a Lecompte maneuver might be
prised with deep suture bites where it passes resected unnecessary (Van Praagh et al. 1970; Wilkinson
conus septum or VSD enlargement areas. et al. 1975; Tanaka et al. 1993). Coronary artery
anomalies have to be suspected more often than
kRastelli operation in transposition of the great arteries; most often
The intracardiac tunnel patch is implanted is a common origin of the LAD and the right
between the VSD and aorta passing the subpul- coronary artery from the anterior sinus. This
monary region. The connection from the right relates to coronary pattern type AB1 according
ventricle to the pulmonary artery is created via a to the SAUER classification (Gittenberger-de
valved or nonvalved conduit (Rastelli et al. 1969) Groot et al. 1983) or type 1L 2RCX of
or as a direct connection form the right ventricle Quaegebeur’s classification (Quaegebeur 1986).
to the pulmonary artery simultaneously with a Even with normal origin of the coronary arter-
Lecompte maneuver (Lecompte and Bex 1985); ies, their course might be tortuous around the
the pulmonary trunk has been transsected and is arterial root before the coronary arteries reach
closed at valvular level to avoid a residual the atrioventricular groove in a «looping pat-
pulmonary stump which may become a source tern» (Jonas 1998; Planche et al. 1998). This has
for systemic thromboemboli. See also 7 Chapter to be considered during the early postoperative
«Congenitally Corrected Transposition and period when changes in pre- and afterload could
D-Transposition of the Great Arteries», Sect. 19.2.6.4. cause aortic and pulmonary root dilatation and
increased tension on the reimplanted coronary
arteries compromising myocardial perfusion.
This may result in an unstoppable vicious cycle
Rastelli surgery for DORV with normal
of ischemic myocardial failure despite originally
pulmonary valve should only be considered in
undisturbed coronary perfusion. These patients
those patients who reveal very unusual
react very unfavorably to «fluid resuscitation»
coronary pattern or the awkward «double
in face of a deteriorating arterial blood pressure.
outlet both ventricles» (see below).
Side-by-side relation of the great arteries is
not infrequently associated with subaortic
obstruction, aortic valve hypoplasia, or hypopla-
kPatrick-McGoon and Doty operation sia of the ascending aorta. Resection of the conal
Nowadays these techniques are seldom used. septum and patch enlargement of the infundibu-
Intraventricular rerouting is yielded with a tun- lum might be necessary. In presence of anular
nel running anterior to the pulmonary valve hypoplasia, a transanular patch (for the neopul-
(Patrick-McGoon operation) or with a tube graft monary artery) is needed.
(Doty operation) (. Fig. 15.12b). Patients with aortic arch obstruction includ-
ing hypoplasia, interruption, or coarctation often
kSpecial features of arterial switch operation undergo separate surgical arch repair as a stage
in DORV one palliation, but also one-stage repair is recom-
The technique of the arterial switch operation is mended with even better survival as claimed
described in 7 Chapter «Congenitally Corrected (Kleinert et al. 1997; Schwarz et al. 2013).
516 G. Ziemer and R. Kaulitz
55 Mild right ventricular hypoplasia is often these patients (Giglia et al. 1992; 1993). With a
present in patients with membranous atresia non- or less stenotic coronary system in right
and a well-developed infundibulum. ventricular- dependent circulation to start with,
55 Severe right ventricular hypoplasia with an the RV to coronary connections diminish or may
obliterated infundibulum represents the other even disappear, as seen also in our own experience.
end of the morphological spectrum; the apex Therefore, in most of the so-called right ventricu-
volume is often taken up by severe hypertro- lar-dependent coronary circulation, dependency
phy of papillary and trabecular muscles. This is rather a term used for coronary flow originating
situation is complicated by right ventricle- from the right ventricle, while definitive depen-
dependent coronary artery circulation. dency is present in only a few cases with proximal
55 The intermediate form can be characterized stenosis of a solitary coronary artery or stenoses in
by a borderline right ventricular size with an both the right and left coronary artery.
infundibular portion; the trabecular portion
might be reduced. Major or minor right 15.6.2.3 Pulmonary Valve
ventricle-coronary arterial communications and Pulmonary Arteries
can be present (Alwi 2006). The atretic pulmonary valve may be well devel-
oped with fused commissures, but most often it is
Patients with dysplastic or severely insufficient only rudimentary and appears as a fibrous mem-
tricuspid valve or Ebstein’s anomaly (present in brane. The anulus frequently is hypoplastic.
5–10 %) and concomitant right ventricular dilata- Typically the pulmonary trunk and branches dis-
tion need separate judgment. tal to the atretic valve are normal; hypoplastic
Hypertrophic myocardium, myocardial fiber branch pulmonary arteries or arborization abnor-
disarray, and endocardial fibrosis are discussed malities are a rare finding (nearly 6 %). Also sig-
for the restrictive right ventricular physiology nificant aortopulmonary collaterals are usually
during long-term follow-up; neonatal endocar- absent. As in any right ventricular obstructive
dial fibrosis is a rare finding and may be related to lesion, pulmonary artery coarctation/LPA steno-
volume loading of the left ventricle or unstable sis may develop (Luhmer and Ziemer 1993).
coronary perfusion in right ventricular-derived
perfusion of the left and right ventricle. 15.6.2.4 Ductus Arteriosus
The morphology of the arterial duct is important
15.6.2.2 Coronary Arteries regarding the option of ductal stenting (origin
Coronary artery abnormalities are found in from the aorta, tortuosity, shape and length,
30–60 % of patients with pulmonary atresia with diameter).
intact ventricular septum; right ventricular
15 hypertension promotes the persistence of embry-
onic right ventricle-coronary artery fistulas 15.6.3 Symptoms and Diagnostic
(Freedom et al. 2005). They are less frequent in the Procedures
well-developed ventricle and can be diagnosed
using fetal echocardiography allowing right ven- Neonatal cyanosis with closure of the arterial duct
tricular decompression. Right ventricular-depen- is the leading symptom, so prostaglandin infusion
dent coronary artery circulation is characterized immediately after birth is indicated. Usually fetal
by retrograde high-pressure hypoxic perfusion; echocardiography has allowed for prenatal diag-
turbulent flow may cause endothelial lesions pre- nosis.
disposing to stenosis, occlusion, or secondary Precise morphologic diagnosis as well as esti-
ostial atresia, an ongoing process even postnatally mation of right ventricular size, tricuspid valve
(Gittenberger-de Groot et al. 1988). The presence size, and calculation of the initial z-score can be
of right ventricular-dependent coronary circula- obtained with transthoracic echocardiography.
tion is only a contraindication for right ventricular Right ventricular inlet length or area can be mea-
decompression when stenosis is present in both sured leading to an enddiastolic volume calcula-
the right and left coronary systems; this could tion. This, however, has some limitations in
result in myocardial steal and infarction. A pallia- presence of severe myocardial hypertrophy
tive single-ventricle repair is recommended in resulting from complete right outflow tract
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
519 15
obstruction. For detailed evaluation of the right the right ventricle. Both measures promote
ventricle, nature of pulmonary atresia, morphol- growth of the right ventricle and tricuspid valve.
ogy of the infundibulum, and arterial duct angi- They consist of pulmonary valvotomy, balloon
ography may be necessary when echocardiography valvuloplasty (if necessary repeatedly) or right
is incomplete. Angiography may be also neces- ventricular outflow tract patch in combination
sary to reveal detailed coronary artery anatomy as with a systemic-to-pulmonary shunt if oxygen-
coronary fistula and/or stenosis involving both ation is insufficient. The second step of surgery
the right and/or left coronary artery have to be has to decide between biventricular repair and
considered especially in patients with hypoplastic palliation following the Fontan principle and
right ventricle and suspicion of coronary abnor- depends more on the initial right ventricular size
malities. This is especially advisable in major and tricuspid valve z-score than on the growth
right ventricle-coronary arterial connections capacity of the right ventricle as ventricular
described in echocardiography in order to define enlargement might result from volume load by
number, size, and course of the vessels; right ven- tricuspid and right ventricular insufficiency more
triculography documents retrograde filling of the than real growth. The morphology of the coro-
aorta as well as significant stenosis. Additional nary arteries with fistula and stenosis and right
antegrade selective coronarography can be per- ventricle-dependent coronary artery circulation
formed also. precludes a procedure with right ventricular
decompression when more than one coronary
artery or a single coronary artery reveals stenosis.
15.6.4 Surgical Management Most patients undergo surgery, and only in
selected patients with membranous atresia a
15.6.4.1 Introduction catheter-based intervention can be performed
Historically rather poor results of surgical treat- employing membrane perforation followed by
ment for PA/IVS up to the 1980s may be attrib- balloon valvotomy; catheter-derived stenting of
uted to neglect of coronary anatomy and aiming the ductus arteriosus is an alternative option to
too often and too early at a biventricular repair. surgical shunt procedure in order to avoid pro-
Now stratified management algorithms have been longed treatment with prostaglandin (Udink ten
used for surgical decision-making and corre- Cate et al. 2013). Neointimal proliferation requires
spond to the morphologic classification (Hoashi redilatation of the ductus or replacement by a
et al. 2012; Alwi 2006). Surgical strategy depends shunt. The need for a systemic-to-pulmonary
on size and morphology of the right ventricle (tri- shunt procedure for sufficient pulmonary blood
partite right ventricle), nature of valvar atresia, flow shows some association with the right ven-
size and function of the tricuspid valve, and tricular size but does not preclude biventricular
presence of right ventricle-dependent coronary repair during later follow-up. In some patients
artery circulation. The goal of surgical repair is this will initially cause a period of left ventricular
separation of systemic venous and pulmonary volume overload and congestive heart failure.
venous circulation using either univentricular or The surgical approach will be dealt with sepa-
biventricular repair. The one-and-a-half ventricle rately for mild, moderate, and severe hypoplasia as
repair is the surgical option in patients with bor- well as for presence of right ventricular-dependent
derline right ventricular size. One-and-a-half coronary circulation. Thereafter, surgical contro-
ventricle repairs for PA/IVS have been first per- versies are discussed. Any surgical procedure com-
formed by Hillel Laks and his group. At that time prising more then an isolated shunt implantation
4 of 15 patients were still presented in the biven- requires support of extracorporeal circulation.
tricular repair group, although surgery also
included a Glenn anastomosis and an adjustable 15.6.4.2 Mild Right Ventricular
ASD (Billingsley et al. 1989). Hypoplasia
As it is rather unreliable to decide early on In patients with mild right ventricular hypoplasia
about the final strategy, primary palliation in neo- as described with a tricuspid valve z-score >−2,5,
nates shall keep all therapeutic options open. This well-developed infundibulum, most often mem-
first stage surgery aims at improvement of oxy- branous atresia, and usually absence of major
genation and to establish antegrade flow through right ventricle to coronary artery connections, a
520 G. Ziemer and R. Kaulitz
15
a b
30-45°
..Fig. 15.13 a Ex situ created PTFE shunt anastomosis with the patch plasty; b redundant shunt course around the
right ventricular infundibulum
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
521 15
kOne-and-a-half ventricle repair (Gerelli et al. 2012). During that time, further
Considering initial tricuspid valve z-score, the evaluation for the preferential one-and-a-half
one-and-a-half ventricle repair is preferable in ventricle option is possible.
these patients. This stage II procedure consists of
the bidirectional Glenn anastomosis with closure kModified Fontan Surgery
or restriction of the atrial septal defect. The cen- Details of Fontan Surgery are outlined in 7 Chapter
tral shunt from stage I neonatal palliation is «Definite Palliation of Functionally Single Ventricle»,
explanted. Sect. 13.6 and Sect. 13.7. It may be mentioned for
This procedure can be applied as early as patients after central shunt and RVOT patch that
6 months of age, although adequate oxygenation the RVOT has to be occluded/divided from the
and low pulmonary artery pressures may allow pulmonary circulation if not done so at the time of
waiting into the second year of life. Glenn procedure. Then the tricuspid valve should
The decision-making of whether the moderate be excised or at least made completely insufficient
hypoplastic right ventricle can pump the IVC in order to prevent suprasystemic pressures in the
blood with normal preload may be made by indi- RV that could provoke rhythm problems, extremely
vidual estimation of right ventricular volume and unfavorable in a Fontan state.
function; however, most important are the hemo-
dynamics after probatory occlusion of the atrial 15.6.4.4 Severe Right Ventricular
septal defect during preoperative cardiac cathe- Hypoplasia
terization. Right atrial pressures should not Severe right ventricular hypoplasia is character-
exceed 15 mmHg with impaired cardiac output. ized by an initial neonatal tricuspid valve z-score
Published data see reduced right ventricular func- <−4; the trabecular portion is often absent, the
tion and a right atrial pressure of more than infundibulum diminutive or absent (muscular
12 mmHg or more than twice the left atrial pres- atresia), and the right ventricle unipartite, the
sure at probatory ASD occlusion as indication for only open part being a diminutive RV inlet por-
univentricular approach (Alwi et al. 2000). tion. For long-term treatment, these patients are
Z-values of the tricuspid valve outside the neona- best served with a Fontan procedure (Hanley
tal period are rather unreliable predictors. et al. 1993). In these diminutive right ventricles,
major right ventricle to coronary artery connec-
kBidirectional Glenn anastomosis tions are common and may show on angiography
The bidirectional Glenn anastomosis as the only stenosis or interruptions. The association with
stage two surgery is the procedure of choice in suprasystemic right ventricular pressure leads to
any borderline small right ventricle. It allows the term right ventricular-dependent coronary
reduction of the ventricular volume load and bal- circulation. Often the significance of right ventri-
ances systemic and pulmonary blood flow for a cle to coronary artery connections, the actual RV
long time. This strategy has the advantage of dependency is unpredictable. The Boston
encouraging right ventricular and pulmonary Children’s Hospital group found only two ste-
artery growth and provides some pulsatile pul- notic/interrupted coronary arteries or a stenotic/
monary blood flow by conducting the inferior atretic single coronary artery defining actual
caval and hepatic venous blood through the right dependency with immediate death after RV
ventricle without increasing right atrial pres- decompression (Giglia et al. 1992; 1993).
sures; with the superior vena cava–pulmonary The duct-dependent pulmonary blood flow
artery anastomosis, one third to one half of the requires an aortopulmonary shunt procedure. As
systemic venous return diverts directly to the atrial septostomy is only rarely necessary in neo-
pulmonary circulation. The Glenn procedure can nates, an alternative primary procedure could be
be performed at the age of 6 months or later if the interventional ductal stenting. This, however,
arterial oxygenation remains adequate. We sug- leaves the RV under high pressure and therefore
gest to always leave the aortopulmonary shunt in our mind is only indicated when significance of
open for better postoperative oxygenation as tol- coronary stenosis requires an undecompressed
erated by PA pressures. This may allow to post- RV. Prior to any decision for or against decom-
pone the decision for the Fontan procedure for a pression, coronary arterial anatomy has to be
mean of 6 years as shown by Pascal Vouhe’s group investigated angiographically in detail.
522 G. Ziemer and R. Kaulitz
If the univentricular surgical pathway is cho- tic stenosis by bulging of the hypertensive right
sen, decompression of the right ventricle by par- ventricle (Razzouk et al. 1992). Beyond the neo-
tial or complete tricuspid valvectomy only can be natal period, other surgical considerations like
considered using cardiopulmonary bypass. patch closure of the tricuspid valve or right ven-
With going on bypass, the ductus arteriosus is tricular thromboexclusion to prevent coronary
closed, and the pulmonary shunt anastomosis is steal have been reported (Waldman et al. 1984;
created; as pulmonary coarctation has to be sup- Williams et al. 1991). To improve coronary perfu-
posed, the pulmonary shunt anastomosis should be sion during infancy, an aorta to right ventricle
extended into the left pulmonary artery conduit was considered (Freeman et al. 1993;
(. Fig. 15.14). For decompression of the right ven- Laks et al. 1995). In addition, this conduit should
tricle, the hypoplastic tricuspid valve will be cause right ventricular decompression during sys-
resected. Then atrial septectomy is performed, com- tole; clear indication and long-term results are
plemented by coronary sinus incision for definite missing (Laks and Plunket 2001). However, the
and reliable creation of permanent unrestrictive Fontan procedure allows for coronary perfusion
interatrial communication (Schraut et al. 1974). with oxygenated blood coming from the left
At the age of 6–8 months, a bidirectional Glenn atrium via tricuspid valve to perfuse the coronary
anastomosis is performed followed by completion arteries during right ventricular systole.
of the Fontan circulation as outlined in 7 Chapter Heart transplantation was considered as an
«Definite Palliation of Functionally Single Ventricle», alternative strategy in these patients (Rychik et al.
Sect. 13.5.5 and Sect. 13.7. The functional result can 1998), but is not a realistic concept for primary
be expected to be the same as for other Fontan surgical intervention in the neonatal period. It
patients with the left ventricle as the systemic ven- may remain a secondary option once myocardial
tricle. Patients with persistent RV-dependent cir- dysfunction leads to clinical deterioration.
culation, however, will do worse (Giglia et al.
1993). 15.6.4.6 Surgical Controversies
and Therapeutic Alternatives
15.6.4.5 Right Ventricular-Dependent
Coronary Circulation kClosure of coronary fistulas during primary
If proximal coronary stenoses are present, tricus- neonatal surgery
pid valvectomy for right ventricular decompression After preoperative diagnosis and intraoperative
can cause myocardial ischemia and ventricular identification by epicardial echocardiography,
arrhythmia and remains a high-risk procedure this concept suggests ligation of right ventricle to
with high mortality. If left not decompressed, coronary artery connections off bypass before
change in ventricular geometry can cause subaor- right ventricular decompression. Probatory liga-
15 tions of coronary connections with 7/0 sutures are
immediately and individually checked for result-
ing wall motion abnormalities by transesophageal
echocardiography. The idea is to induce antegrade
coronary flow taking away the high-pressure
hypoxic coronary flow, which might induce the
observed secondary coronary lesions. In their
experience ligation did not produce visual myo-
cardial consequences or wall motion abnormali-
ties during intraoperative echocardiographic
surveillance (Foker et al. 2008). The authors claim,
as others before (Hausdorf et al. 1987; Rychik
et al. 1998), that these noncritical connections
contribute to early postoperative myocardial dys-
function. The risk of this concept is, however, that
..Fig. 15.14 Anastomosis of the PTFE shunt with the
hypoplastic pulmonary artery and enlargement of the left
instead of awaiting the spontaneous closure of
pulmonary artery by oblique shunt insertion in patients these small vessels, actual autochthonous coro-
with presumed pulmonary coarctation nary arteries are damaged.
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
523 15
kExtensive right ventricular muscle regardless of the surgical strategy with uni- or
resection (right ventricular «overhaul») biventricular repair; oxygen consumption was
Originally from Melbourne, Australia, and then positively correlated to initial tricuspid valve
in Cleveland, Ohio, Roger Mee’s group suggested z-score (Karamlou et al. 2013). Patients after uni-
in hypoplastic RV with open infundibulum to ventricular palliation present with similar results
perform an extensive transatrial and transinfun- as other Fontan patients characterized by inade-
dibular myectomy to enlarge the diastolic right quate chronotropic response and limited cardiac
ventricular cavity. The idea was to improve right reserve and unfavorable ventricular filling (Romeih
ventricular growth for biventricular repair et al. 2012). Only patients with mild right ventricu-
(Pawade et al. 1993). While some patients who lar hypoplasia (tricuspid valve z-score <−2.5) and
may have been candidates for biventricular repair with two or more right ventricular segments bring-
anyways may have become ready for repair earlier ing them to a favorable end of the spectrum of
than without myectomy, the aim of the procedure PA-IVS might show normal exercise capacity
to make more patients eligible for biventricular (Romeih et al. 2012; Karl 2012). Late clinical fol-
repair could not be reached (Bryant et al. 2008). low-up after biventricular repair describes compli-
cations due to tricuspid or pulmonary insufficiency
kClosed («off-pump») pulmonary valvotomy with right ventricular dilatation or arrhythmia; a
during primary surgery small or hypertrophied right ventricle predisposes
These procedures stem from an era where morbidity to restrictive physiology with compromise of sys-
from extracorporeal circulation in neonates over- temic cardiac output (Hoashi et al. 2012). Careful
whelmed the early postoperative management. The initial management and selection for those who
last who published on this track, going even further, undergo biventricular repair are indicated. And
were Laks and Plunket (2001): they suggested off- even in selected patients, biventricular repair may
pump infundibulectomy and transanular outflow cause systemic venous congestion and low output
tract patch insertion. We think that with today’s tech- because of increased pulmonary vascular resis-
nology, a technically perfect procedure overcomes tance or decreased left ventricular filling; leaving
the disadvantages of extracorporeal circulation. the ASD open or fenestrated for right-to-left
shunting can stabilize the hemodynamic situation.
kCatheter-based interventions For borderline right ventricular size, bet-
In patients with membranous atresia, a catheter- ter outcome may be achieved with a one-and-
based procedure can be considered with perfora- half ventricle or univentricular repair
tion of the atretic plate followed by repeated (Karamlou et al. 2013).
balloon dilatation. A high technical success rate of
80–90 % can be expected in selected patients
(Alwi et al. 2000), but only a relative small pro- 15.7 Double-Chambered Right
portion of patients with pulmonary atresia and Ventricle (DCRV)
intact ventricular septum can be considered for
this procedure (Parsons et al. 1991; Bichell 1999). 15.7.1 Definition
In some patients this procedure has to be com-
bined with a surgical shunt procedure or later a Double-chambered right ventricle is an uncom-
right ventricular outflow tract patch resulting in a mon congenital heart defect and potentially pro-
hybrid biventricular repair (Hoashi et al. 2012). gressive disease although natural history is
Stenting of the arterial duct is recommended in unknown. The underlying morphologic substrate
selected patients as an alternative to prolonged is supposed to be congenital, while the course of
prostaglandin administration and perhaps shunt the disease resembles an acquired disease (Hartman
procedure (Alwi 2006). et al. 1964). The incidence varies from 0.5 to 2 %.
proximal chamber becomes the high-pressure, and morphologically excellent. Late ventricular
hypertrophied chamber; the distal chamber has dysfunction and arrhythmia are rarely described
low pressure. The anomalous muscle bundle tra- (Said et al. 2012).
verses the right ventricular cavity and shows a
midventricular fibrous ridge, hypertrophied mod-
erator band, or hypertrophied papillary muscles. References
Severity and location of the obstructing muscular
is variable (Alva et al. 1999; Wong et al. 1991). Abbot ME (1936) Atlas of congenital heart disease.
American Heart Association, New York. Reprint (2006)
A VSD is associated in more than 80 %, most
McGill-Queen’s University Press, Montreal, Canada
often a perimembranous type of VSD. Abella RF, De La Torre T, Mastropietro G et al. (2004) Primary
Double-chambered right ventricle might repair of pulmonary atresia with ventricular septal
develop postoperatively after VSD closure or after defect and major aortopulmonary collaterals: a useful
correction of TOF with the typical morphologic approach. J Thorac Cardiovasc Surg 127:193–202
Alsoufi B, Williams WG, Hua Z et al. (2007) Surgical out-
criteria (Moran et al. 1998).
comes in the treatment of patients with tetralogy of
fallot and absent pulmonary valve. Eur J Cardiothorac
Surg 31:354–359
15.7.3 Symptoms and Diagnostic Alva C, Ho SY, Lincoln CR et al. (1999) The nature of the
Procedures obstructive muscular bundles in double-chambered
right ventricle. J Thorac Cardiovasc Surg 117:1180–
1189
In childhood a systolic murmur is leading to diag-
Alwi M (2006) Management algorithm in pulmonary atre-
nosis by echocardiography. In infancy dyspnea and sia with intact ventricular septum. Cathet Cardiovasc
failure to thrive or cyanosis might rarely develop. Int 67:679–686
Diagnosis is made usually before the age of 10 years. Alwi M, Geetha K, Bilkis AA et al. (2000) Pulmonary atre-
Late diagnosis in adulthood might be accompanied sia with intact ventricular septum percutaneous
radiofrequency-assisted valvotomy and balloon dila-
by dyspnea and syncope or angina (McElhinney et
tion versus surgical valvotomy and Blalock Taussig
al. 2000). The description of an anomalous right shunt. J Am Coll Cardiol 35:468–476
ventricular bundle needs regular follow-up because Anderson RH, Becker AE, Wilcox BR, Macartney FJ, Wilkinson
of well-known progression; sometimes ß-blocker JL (1983) Surgical anatomy of double-outlet right ven-
medication might be indicated in this situation tricle—a reappraisal. Am J Cardiol 52:555–559
Anderson RH, Devine WA, Del Nido P (1991) The surgical
(Arai et al. 2001).
anatomy of tetralogy of fallot with pulmonary atresia
Echocardiography usually allows the diagno- rather than pulmonary stenosis. J Card Surg 6:41–58
sis and description of associated lesions in pediat- Aoki M, Forbess JM, Jonas RA, Mayer JE Jr, Castaneda AR
ric patients (Hoffman et al. 2004). In adults the (1994) Result of biventricular repair for double-outlet
echocardiographic diagnosis might be incomplete right ventricle. J Thorac Cardiovasc Surg 107:338–349
15 and needs confirmation by right heart catheter- Arai N, Matsumoto A, Nishikawa N et al. (2001) Beta-blocker
therapy improved symptoms and exercise capacity in
ization or—predominantly—MRI (Kilner et al. a patient with dynamic intra-right ventricular obstruc-
2002). tion: an atypical form of double-chambered right ven-
tricle. J Am Soc Echocardiogr 14:650–653
Ashburn DA, Blackstone EH, Wells WJ et al. (2004)
Determinants of mortality and type of repair in neo-
15.7.4 Surgical Approach nates with pulmonary atresia and intact ventricular
septum. J Thorac Cardiovasc Surg 127:1000–1008
Indications for surgical intervention are clinical Atallah-Yunes NH, Kavey RE, Bove EL et al. (1996)
symptoms, increasing right ventricular gradi- Postoperative assessment of a modified surgical
approach to repair of tetralogy of fallot. Long-term
ent (exceeding 40 mmHg at rest) and associated
follow-up. Circulation 94:II22–II26
anomalies as a VSD with relevant left-to-right Bacha EA, Scheule AM, Zurakowski D et al. (2001) Long-
shunt, or aortic regurgitation. For relief of term results after early primary repair of tetralogy of
intraventricular obstruction, usually a com- fallot. J Thorac Cardiovasc Surg 122:14–61
bined transatrial-transventricular or transatrial Barbero-Marcial M, Tamanati C, Jatene MB et al. (1998)
Double-outlet right ventricle with nonrelated ventric-
approach is chosen; in pediatric patients often
ular septal defect: surgical results using the multiple
the transventricular approach is indicated for patches technique. Heart Surg Forum 1:125–129
accurate demonstration of the right ventricular Bautista-Hernandez V, Kaza AK, Benavidez OJ, Pigula FA
obstruction. The surgical result is functionally (2008) True aneurysmal dilatation of a contegra con-
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
525 15
duit after right ventricular outflow tract reconstruc- Cooley DA, Hallman GL (1966) Intrapericardial aortic-right
tion: a novel mechanism of conduit failure. Ann Thorac pulmonary arterial anastomosis. Surg Gynecol Obstet
Surg 86:1976–1977 122:1084–1086
Belli E, Serraf A, Lacour-Gayet F et al. (1998) Biventricular d’Udekem Y, Alphonso N, Nørgaard MA et al. (2005)
repair for double-outlet right ventricle. Results and Pulmonary atresia with ventricular septal defects and
long-term follow-up. Circulation 98:II360–II365 major aortopulmonary collateral arteries: unifocaliza-
Berdat PA, Carrel T (2006) Off-pump pulmonary valve replace- tion brings no long-term benefits. J Thorac Cardiovasc
ment with the new shelhigh injectable stented pulmonic Surg 130:1496–1502
valve. J Thorac Cardiovasc Surg 131:1192–1193 Davignon AL, Greenwold WE, DuShane JW, Edwards JE
Bichell D (1999) Evaluation and management of pulmo- (1961) Congenital pulmonary atresia with intact ven-
nary atresia with intact ventricular septum. Curr Opin tricular septum. Clinicopathologic correlation of two
Cardiol 14:60–66 anatomic types. Am Heart J 62:591–602
Billingsley AM, Laks H, Boyce SW, George B, Santulli T, del Nido PJ (2006) Surgical management of right ven-
Williams RG (1989) Definitive repair in patients with tricular dysfunction late after repair of tetralogy of
pulmonary atresia and intact ventricular septum. fallot: right ventricular remodeling surgery. Semin
J Thorac Cardiovasc Surg 97:746–754 Thorac Cardiovasc Surg Pediatr Card Surg Annu
Blalock A, Taussig HB (1945) Surgical treatment of malfor- 9:29–34
mations of the heart in which there is pulmonary ste- deLeval MR, McKay R, Jones M, Stark J, Macartney FJ (1981)
nosis or pulmonary atresia. JAMA 128:189–202 Modified Blalock-Taussig shunt. Use of subclavian artery
Bonhoeffer P, Boudjemline Y, Saliba Z et al. (2000) orifice as flow regulator in prosthetic systemic-pulmonary
Percutaneous replacement of pulmonary valve in a artery shunts. J Thorac Cardiovasc Surg 81:112–119
right-ventricle to pulmonary-artery prosthetic conduit Delius RE, Rademecker MA, de Leval MR, Elliott MJ, Stark
with valve dysfunction. Lancet 356:1403–1405 J (1996) Is a high-risk biventricular repair always
Bove EL, Shaher RM, Alley R, McKneally M (1972) Tetralogy preferable to conversion to a single ventricle repair?
of fallot with absent pulmonary valve and aneurysm of J Thorac Cardiovasc Surg 112:1561–1568
the pulmonary artery: report of two cases presenting as Di Donato RM, Jonas RA, Lang P, Rome JJ, Mayer JE Jr,
obstructive lung disease. J Pediatr 81:339–343 Castaneda AR (1991) Neonatal repair of tetralogy of
Brandt PW, Calder AL, Barratt-Boyes BG, Neutze JM (1976) fallot with and without pulmonary atresia. J Thorac
Double outlet left ventricle. Morphology, cineangiographic Cardiovasc Surg 101:126–137
diagnosis and surgical treatment. Am J Cardiol 38:897–909 Dodge-Khatami A, Backer CL, Holinger LD, Baden HP,
Brizard CP, Mas C, Sohn YS, Cochrane AD, Karl TR (1998) Mavroudis C (1999) Complete repair of tetralogy of fal-
Transatrial-transpulmonary tetralogy of fallot repair is lot with absent pulmonary valve including the role of
effective in the presence of anomalous coronary arter- airway stenting. J Card Surg 14:82–91
ies. J Thorac Cardiovasc Surg 116:770–779 Doty DB (1986) Correction of Taussig-Bing malformation
Brock RC (1948) Pulmonary valvotomy for the relief of con- by intraventricular conduit. J Thorac Cardiovasc Surg
genital pulmonary stenosis. Report of three cases. Br 91:133–138
Med J 1:1121–1126 Edmunds LH Jr, Saxena NC, Friedman S, Rashkind WJ,
Brown JW, Ruzmetov M, Okada Y et al. (2001) Surgical results Dodd PF (1976) Transatrial repair of tetralogy of fallot.
in patients with double outlet right ventricle: a 20-year Surgery 80:681–688
experience. Ann Thorac Surg 72:1630–1635 Emmanouilides G, Thanopoulos B, Siassi B et al. (1978)
Brown JW, Ruzmetov M, Rodefeld MD, Vijay P, Darragh RK Agenesis of ductus arteriosus associated with the
(2006) Valved bovine jugular vein conduits for right syndrome of tetralogy of fallot and absent pulmonary
ventricular outflow tract reconstruction in children: an valve. Am J Cardiol 37:404–409
attractive alternative to pulmonary homograft. Ann Fallot EL (1888) Contribution a l’anatomie pathologique de
Thorac Surg 82:909–916 la maladie bleue (cyanose cardiaque). Marseille Med
Brown JW, Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW 25: 77, 138, 207, 341, 403
(2007) Right ventricular outflow tract reconstruction with Foker JE, Setty SP, Berry J et al. (2008) Treatment of right
a polytetrafluoroethylene monocusp valve: a twelve-year ventricle to coronary artery connections in infants
experience. J Thorac Cardiovasc Surg 133:1336–1343 with pulmonary atresia and intact ventricular septum.
Bryant R 3rd, Nowicki ER, Mee RB et al. (2008) Success J Thorac Cardiovasc Surg 136:749–756
and limitations of right ventricular sinus myectomy Francois k, Zagout M, Bove T et al. (2010) The fate of the
for pulmonary atresia with intact ventricular septum. aortiv root after early repair of tetralogy of Fallot. Eur J
J Thorac Cardiovasc Surg 136:735–742 Cardiothorac Surg. 37:1254–1258
Bull C, de Leval MR, Mercanti C, Macartney FJ, Anderson RH Fraser CD Jr, Latson LA, Mee RB (1995) Surgical repair of
(1982) Pulmonary atresia and intact ventricular septum: severe bilateral branch pulmonary artery stenosis.
a revised classification. Circulation 66:266–272 Ann Thorac Surg 59:738–740
Castaneda AR, Freed MD, Williams RG, Norwood WI (1977) Freedom RM, Anderson RH, Perrin D (2005) The signifi-
Repair of tetralogy of fallot in infancy. Early and late cance of ventriculo-coronary arterial connections in
results. J Thorac Cardiovasc Surg 74:372–381 the setting of pulmonary atresia with an intact ven-
Castaneda AR, Jonas RA, Mayer JE, Hanley FL (1994) Cardiac tricular septum. Cardiol Young 15:447–468
surgery of the neonate and infant. W.B.Saunders Freeman JE, DeLeon SY, Lai S, Fisher EA, Ow EP, Pifarré R
Company, Philadelphia, p 218 (1993) Right ventricle-to-aorta conduit in pulmonary
526 G. Ziemer and R. Kaulitz
atresia with intact ventricular septum and coronary Hanley FL, Sade RM, Blackstone EH et al. (1993) Outcomes
sinusoids. Ann Thorac Surg 56:1393–1395 in neonatal pulmonary atresia with intact ventricu-
Fyler DC (1992) Tetralogy of fallot. In: Fyler DC (ed) Nada’s lar septum. A multi-institutional study. J Thorac
pediatric cardiology. Hanley & Beltus, Philadelphia, Cardiovasc Surg 105:406–423
p 472, Mosby Year Book, St. Louis Baltimore Boston Hartman AF, Goldring D, Carlsson E (1964) Development of
Chicago London Philadelphia Sydney Toronto right ventricular aberrant muscular bands. Circulation
Gatzoulis MA, Clark AL, Cullen S, Newman CG, Redington 30:679–685
AN (1995a) Right ventricular diastolic function 15 to Hausdorf G, Graevinghoff L, Keck EW (1987) Effects of per-
35 years after repair of tetralogy of fallot. Restrictive sisting myocardial sinusoids on left ventricular per-
physiology predicts superior exercise performance. formance in pulmonary atresia with intact ventricular
Circulation 91:1775–1781 septum. Eur Heart J. 8:291–296
Gatzoulis MA, Till JA, Somerville J, Redington AN (1995b) Haworth SG (1990) Pulmonary vascular development.
Mechanoelectrical interaction in tetralogy of fallot. In: Long WA (ed) Fetal and neonatal cardiology. WB
QRS prolongation relates to right ventricular size and Saunders, Philadelphia, pp 51–63
predicts malignant ventricular arrhythmias and sud- Haworth SG, Macartney FJ (1980) Growth and develop-
den death. Circulation 92:231–237 ment of pulmonary circulation in pulmonary atresia
Gerelli S, Boulitrop C, Van Steenberghr M et al. (2012) with ventricular septal defect and major aortopulmo-
Bidirectional cavopulmonary shunt with additional nary collateral vessels. Br Heart J 44:14–24
pulmonary blood flow: a failed or successful strategy? Heinemann MK, Hanley FL (1993) Preoperative manage-
Eur J Cardiothorac Surg 42:513–519 ment of neonatal tetralogy of fallot with absent pul-
Geva T, Gauvreau K, Powell AJ, Cecchin F, Rhodes J, Geva J, monary valve syndrome. Ann Thorac Surg 55:172–174
del Nido P (2010) Randomized trial of pulmonary valve Hennein HA, Mosca RS, Urcelay G, Crowley DC, Bove EL
replacement with and without right ventricular remod- (1995) Intermediate results after complete repair of
eling surgery. Circulation 122(11 Suppl):S201–S208 tetralogy of fallot in neonates. J Thorac Cardiovasc
Gewillig M, Boshoff DE, Dens J, Mertens L, Benson LN Surg 109:332–344
(2004) Stenting the neonatal arterial duct in duct- Hew CC, Daebritz SH, Zurakowski D, del Nido PI, Mayer JE
dependent pulmonary circulation: new techniques, Jr, Jonas RA (2002) Valved homograft replacement of
better results. J Am Coll Cardiol. 43:107–112 aneurysmal pulmonary arteries for severely symptom-
Gibbs JL, Rothman MT, Rees M, Parsons JM, Blackburn ME, atic absent pulmonary valve syndrome. Ann Thorac
Ruiz CE (1992) Stenting of the arterial duct: a new Surg 73:1778–1785
approach to palliation for pulmonary atresia. Br Heart Hirji A, Bernasconi A, McCrindle B et al. (2010) Outcomes of
J 67: 240–245 prenatally diagnosed tetralogy of fallot: implications
Giglia TM, Mandell VS, Connor AR, Mayer JE Jr, Lock JE of valve sparing repair versus transannular patch. Can
(1992) Diagnosis and management of right ventricle- J Cardiol 26:E1–E6
dependent coronary circulation in pulmonary atresia Hirsch JC, Mosca RS, Bove EL (2000) Complete repair of
with intact ventricular septum. Circulation 86:1516– tetralogy of fallot in the neonate: results in the mod-
1528 ern era. Ann Surg 232:508–514
Giglia TM, Jenkins KJ, Matitiau A, Mandell VS, Sanders SP, Hoashi T, Kagisaki K, Kitano M et al. (2012) Late clinical fea-
Mayer JE, Lock JE (1993) Influence oft right heart size tures of patients with pulmonary atresia or critical pul-
on outcome in pulmonary atresia with intact ventricu- monary stenosis with intact ventricular septum after
lar septum. Circulation 88:2248–2256 biventricular repair. Ann Thorac Surg 94:833–841
15 Gittenberger-de Groot AC, Sauer U, Bindl L, Babic R, Essed Hoffman P, Wójcik AW, Rózański J (2004) The role of echo-
CE, Buehlmeyer K (1988) Competition of coronary cardiography in diagnosing double chambered right
arteries and ventriculo-coronary arterial communica- ventricle in adults. Heart 90:789–793
tions in pulmonary atresia with intact ventricular sep- Holmes AA, Co S, Human DG, LeBlanc JG, Campbell AIM
tum. Int J Cardiol. 18:243–258 (2012) The contegra conduit: late outcomes in right
Gittenberger-de Groot AC, Sauer U, Oppenheimer-Dekker ventricular outflow tract reconstruction. Ann Pediatr
A, Quaegebeur JM (1983) Coronary arterial anatomy Cardiol 5:27–33
in transposition of the great arteries: a morphology Hraska V (2000) A new approach to correction of tetral-
study. Pediatr Cardiol 4(Suppl I):15–24 ogy of fallot with absent pulmonary valve. Ann Thorac
Goor DA, Lillehei CW (1975) Congenital malformations of Surg 69:1601–1602
the heart. Grune & Stratton, New York Hu R, Zhang H, Xu Z et al. (2013) Late outcome for the
Griselli M, McGuirk SP, Winlaw DS et al. (2004) The influence surgical management of absent pulmonary valve
of pulmonary artery morphology on the results of syndrome in infants. Interact Cardiovasc Thorac Surg
operations for major aortopulmonary collateral arter- 16:792–796
ies and complex congenital heart defects. J Thorac Hudspeth AS, Cordall AR, Johnston FR (1963) Transatrial
Cardiovasc Surg 127:251–258 approach to total correction of tetralogy of fallot.
Gundry SR, Razzouk AJ, Boskind JF, Bansal R, Bailey LL Circulation 27:796–800
(1994) Fate of the pericardial monocusp pulmonary Ionescu MI, Tandon AP, Macartney FJ (1979) Long-term
valve for right ventricular outflow tract reconstruc- sequential hemodynamic evaluation of right ventricu-
tion. Early function, late failure without obstruction. lar outflow tract reconstruction using a valve mecha-
J Thorac Cardiovasc Surg 107:908–912 nism. Ann Thorac Surg 27:426–434
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
527 15
Iyer KS, Mee RB (1991) Staged repair of pulmonary atresia Lacour-Gayet F (2008) Intracardiac repair of double outlet
with ventricular septal defect and major systemic to right ventricle. Semin Thorac Cardiovasc Surg Pediatr
pulmonary artery collaterals. Ann Thorac Surg 51:65–72 Card Surg Annu 11:39–43
Jonas RA (1998) The arterial switch operation for transposi- Laks H, Castaneda AR (1975) Subclavian arterioplasty for
tion of the great arteries. Oper Tech Thorac Cardiovasc the ipsilateral Blalock-Taussig-shunt. Ann Thorac Surg
Surg: Comp Atlas 3:238–253 19:319–321
Jonas RA (2004) Comprehensive surgical management of Laks H, Plunket MD (2001) Surgical management of pul-
congenital heart disease. Hodders Arnold, London monary atresia with intact ventricular septum. Prog
Karamlou T, Poynter JA, Walters HL et al. (2013) Long-term Pediatr Cardiol 13:183–197
functional health status and exercise test variables for Laks H, Gates RN, Grant PW et al. (1995) Aortic to right ven-
patients with pulmonary atresia with intact ventricu- tricular shunt for pulmonary atresia and intact ventric-
lar septum: a congenital heart surgeons society study. ular septum. Ann Thorac Surg 59:342–347
J Thorac Cardiovasc Surg 145:1018–1027 Lecompte Y, Bex JP (1985) Repair of transposition of the
Karl TR (2012) Pulmonary atresia with intact ventricu- great arteries with ventricular septal defect and
lar septum: how good is a biventricular repair? Eur left ventricular outflow tract obstruction. J Thorac
J Cardio Thorac Surg 42:56 Cardiovasc Surg 90:151–152
Karl TR, Sano S, Porniviliwan S, Mee RB (1992) Tetralogy of Lee C, Jacobs JP, Lee C et al. (2012) Surgical pulmonary
fallot: favourable outcome of neonatal transatrial, trans- valve insertion—when, how, and why. Cardiol Young
pulmonary repair. Ann Thorac Surg 54:903–907 22:702–707
Kawashima Y, Fujita T, Miyamoto T, Manabe H (1971) Lev M, Bharati S, Meng CC et al. (1972) A concept of
Intraventricular rerouting of blood for the correction double-outlet right ventricle. J Thorac Cardiovasc Surg
of Taussig-Bing malformation. J Thorac Cardiovasc 64:271–281
Surg 62:825–829 Lillehei CW, Cohen M, Warden HE et al. (1955) Direct vision
Kawashima Y, Matsuda H, Hirose H et al. (1985) Ninety intracardiac surgical correction of the tetralogy of fal-
consecutive corrective operations for tetralogy of lot, pentalogy of fallot, and pulmonary atresia defects;
fallot with or without minimal right ventriculotomy. report of first ten cases. Ann Surg 142:418–442
J Thorac Cardiovasc Surg 90:856–863 Lofland GK (2004) Pulmonary atresia, ventricular septal
Kilner PJ, Sievers B, Meyer GP, Ho SY (2002) Double- defect, and multiple aorta pulmonary collateral arter-
chambered right ventricle or sub-infundibular steno- ies. Semin Thorac Cardiovasc Surg Pediatr Card Surg
sis assessed by cardiovascular magnetic resonance. Annu 7:85–94
J Cardiovasc Magn Reson 4:373–379 Luciani GB, Wells WJ, Khong A, Starnes VA (1997) The clam-
Kim WH, Seo JW, Kim SJ et al. (2005) Aortic dissection late shell incision for bilateral pulmonary artery recon-
after repair of tetralogy of fallot. Int J Cardiol 101:515– struction in tetralogy of fallot with pulmonary atresia.
516 J Thorac Cardiovasc Surg 113:443–452
Kim N, Friedberg MK, Silverman NH (2006) Diagnosis and Luhmer I, Ziemer G (1993) Coarctation of the pulmonary
prognosis of fetuses with double outlet right ventricle. artery in neonates. Prevalence, diagnosis, and surgical
Prenat Diagn 26:740–745 treatment. J Thorac Cardiovasc Surg 106:889–894
Kirklin JW, Karp RB (1970) The tetralogy of fallot. From a sur- Lurz P, Bonhoeffer P (2008) Percutaneous implantation
gical viewpoint. W.B.Saunders, Philadelphia, p 76 of pulmonary valves for treatment of right ven-
Kirklin JW, DuShane JW, Patrick RT et al. (1955) Intracardiac tricular outflow tract dysfunction. Cardiol Young
surgery with the aid of a mechanical pump-oxygenator 18:260–267
system (Gibbon type): report of eight cases. Proc Staff Mackie AS, Gauvreau K, Perry SB, del Nido PJ, Geva T (2003)
Meet Mayo Clin 30:201–206 Echocardiographic predictors of aortopulmonary col-
Kirsch RE, Glatz AC, Gaynor JW et al. (2013) Results of elec- laterals in infants with tetralogy of fallot and pulmo-
tive repair at 6 months or younger in 277 patients with nary atresia. J Am Coll Cardiol 41:852–857
tetralogy of Fallot: a 14-years experience at a single McElhinney DB, Parry AJ, Reddy VM, Hanley FL, Stanger P
center. J Thorac Cardiovasc Surg 147(2):713–717 (1998a) Left pulmonary artery kinking caused by out-
Kirshbom PM, Kogon BE (2004) Tetralogy of fallot with flow tract dilatation after transannular patch repair of
absent pulmonary valve syndrome. Semin Thorac tetralogy of fallot. Ann Thorac Surg 5:1120–1126
Cardiovasc Surg Pediatr Card Surg Annu 7:65–71 McElhinney DB, Reddy VM, Silverman NH, Brook MM,
Kleinert S, Sano T, Weintraub RG et al. (1997) Anatomic Hanley FL (1998b) Atrioventricular septal defect with
features and surgical strategies in double-outlet right common valvar orifice and tetralogy of fallot revisited:
ventricle. Circulation 96:1233–1239 making a case for primary repair in infancy. Cardiol
Klinner W, Zenker R (1965) Experience with correction of Young 8:455–461
fallot’s tetralogy in 178 cases. Surgery 57:353–357 McElhinney DB, Chatterjee KM, Reddy VM (2000) Double-
Klinner W, Pasini M, Schaudig A (1962) Anastomose chambered right ventricle presenting in adulthood.
zwischen System und Lungenarterie mit Hilfe von Ann Thorac Surg 70:124–127
Kunststoffprothesen bei cyanotischen Herzvitien. McElhinney DB, Hellenbrand WE, Zahn EM et al. (2010)
Thoraxchirurgie 10:68–75 Short- and medium- term outcomes after transcath-
Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp eter pulmonary valve placement in the expanded
RB (2003) Kirklin/Barratt Boyes cardiac surgery, 3rd multicenter US Melody valve trial. Circulation
edn. Churchill Livingstone, Philadelphia, p 982 122:507–516
528 G. Ziemer and R. Kaulitz
Mearns AJ, Deverall PB, Kester RC (1978) Revascularization Pawade A, Capuani A, Penny DJ, Karl TR, Mee RB (1993)
of an arm for incipient gangrene after Blalock-Taussig Pulmonary atresia with intact ventricular septum: sur-
anastomosis. Br J Surg 65:467–468 gical management based on right ventricular infun-
Meyns B, Van Garsse L, Boshoff D et al. (2004) The contegra dibulum. J Card Surg 8:371–383
conduit in the right ventricular outflow tract induces Pfaundler M, Schlossmann A (1926) Handbuch der
supravalvular stenosis. J Thorac Cardiovasc Surg Kinderheilkunde, vol I–IV, 4th edn. Vogel, Leipzig
128:834–840 Planche C, Lacour-Gayet F, Serraf A (1998) Arterial switch.
Milanesi O, Talenti E, Pellegrino PA, Thiene G (1984) Abnormal Pediatr Cardiol 19:297–307
pulmonary artery branching in tetralogy of fallot with Plunkett MD, Bond LM, Geiss DM (1998) Use of the “chim-
“absent” pulmonary valve. Int J Cardiol 6:375–380 ney patch” technique for Norwood stage I procedures.
Mongeon F, Gurvitz MZ, Broberg CS et al. (2013) Aortic root Ann Thorac Surg 66:1438–1439
dilatation in adults with surgically repaired tetralogy Potts WD, Smith S, Gibson S (1946) Anastomosis of the
of fallot. Circulation 127:172–179 aorta to a pulmonary artery for certain types of con-
Moran AM, Hornberger LK, Jonas RA, Keane JF (1998) genital heart disease. JAMA 132:629
Development of a double-chambered right ventricle Poynter JA, Eghtesady P, McCrindle BW et al. (2013) Effect
after repair of tetralogy of fallot. J Am Coll Cardiol of conduit type and size on right ventricle-pulmonary
31:1127–1133 artery conduit durability in infants less than two years
Moritz A, Marx M, Wollenek G, Domanig E, Wolner E (1996) of age: A Congenital Heart Surgeons’ Society study.
Complete repair of PA-VSD with diminutive or discon- Ann Thorac Surg 96:1695–1702
tinuous pulmonary arteries by transverse thoracoster- Preminger TJ, Sanders SP, van der Velde ME, Castañeda
notomy. Ann Thorac Surg 61:646–650 AR, Lock JE (1994) “Intramural” residual interventricu-
Najm HK, Van Arsdell GS, Watzka S et al. (1998) Primary lar defects after repair of conotruncal malformations.
repair is superior to initial palliation in children with Circulation 89:236–242
atrioventricular septal defect and tetralogy of fallot. Puga FJ, McGoon DC, Julsrud PR, Danielson GK, Mair DD
J Thorac Cardiovasc Surg 116:905–913 (1983) Complete repair of pulmonary atresia with
Nakata S, Imai Y, Takanashi Y et al. (1984) A new method nonconfluent pulmonary arteries. Ann Thorac Surg
for the quantitative standardization of cross-sectional 35:36–44
areas of the pulmonary arteries in congenital heart Puga FJ, Leoni FE, Julsrud PR, Mair DD (1989) Complete
diseases with decreased pulmonary blood flow. repair of pulmonary atresia, ventricular septal defect,
J Thorac Cardiovasc Surg 88:610–619 and severe peripheral arborization abnormalities of
Niwa K, Siu SC, Webb GD et al. (2002) Progressive aortic the central pulmonary arteries. Experience with pre-
root dilatation in adults after repair of tetralogy of fal- liminary unifocalization procedures in 38 patients.
lot. Circulation 106:1374–1378 J Thorac Cardiovasc Surg 98:1018–1028
Nölke L, Azakie A, Anagnostopoulos PV et al. (2006) The Quaegebeur JM (1986) The arterial switch operation.
lecompte maneuver for relief of airway compression in Rationale, results, and perspectives. Thesis. Leiden
absent pulmonary valve syndrome. Ann Thorac Surg University, The Netherlands
81:1802–1807 Qureshi SA, Kirk CR, Lamb RK, Arnold R, Wilkinson JL (1988)
Nørgaard MA, Alphonso N, Cochrane AD et al. (2006) Balloon dilatation of the pulmonary valve in the first
Major aorto-pulmonary collateral arteries of patients year of life in patients with tetralogy of fallot: a prelimi-
with pulmonary atresia and ventricular septal defect nary study. Br Heart J 60:232–235
are dilated bronchial arteries. Eur J Cardiothorac Surg Rabinovitch M, Herrera-deLeon V, Castaneda AR, Reid L
15 29:653–658 (1981) Growth and development of the pulmonary
Norgård G, Gatzoulis MA, Moraes F et al. (1996) vascular bed in patients with tetralogy of fallot with
Relationship between type of outflow tract repair and or without pulmonary atresia. Circulation 64:1234–
postoperative right ventricular diastolic physiology 1249
in tetralogy of fallot. Implications for long-term out- Rabinovitch M, Grady S, David I et al. (1982) Compression
come. Circulation 94:3276–3280 of intrapulmonary bronchi by abnormally branching
Ong J, Brizard CP, DÚdekem Y et al. (2012) Repair of atrio- pulmonary arteries associated with absent pulmonary
ventricular septal defect associated with tetralogy of valves. Am J Cardiol 50:804–813
fallot or double outlet right ventricle: 30 years of expe- Rastelli GC, Wallace RB, Ongley PA (1969) Complete repair
rience. Ann Thorac Surg 94:172–178 of transposition of the great arteries with pulmonary
Parry AJ, McElhinney DB, Kung GC et al. (2000) Elective stenosis. A review and report of a case corrected by
primary repair of acyanotic tetralogy of fallot in early using a new surgical technique. Circulation 39:83–95
infancy: overall outcome and impact on the pulmo- Rathi VK, Doyle M, Williams RB et al. (2005) Massive aortic
nary valve. J Am Coll Cardiol 36:2279–2283 aneurysm and dissection in repaired tetralogy of fal-
Parsons JM, Rees MR, Gibbs JL (1991) Percutaneous laser lot; diagnosis by cardiovascular magnetic resonance
valvotomy with balloon dilatation of the pulmonary imaging. Int J Cardiol 101:169–170
valve as primary treatment for pulmonary atresia. Br Razzouk AJ, Freedom RM, Cohen AJ et al. (1992) The rec-
Heart J 66:36–38 ognition, identification of morphologic substrate,
Patrick DL, McGoon DC (1968) An operation for double- and treatment of subaortic stenosis after a Fontan
outlet right ventricle with transposition of the great operation. An analysis of twelve patients. J Thorac
arteries. J Cardiovasc Surg (Torino) 9:537–542 Cardiovasc Surg 104:1750–1753
Chapter 15 · Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract
529 15
Reddy VM, Liddicoat JR, Hanley FL (1995) Midline one- monary valvotomy and central aortopulmonary shunt
stage complete unifocalization and repair of pulmo- for pulmonary atresia and intact ventricular septum.
nary atresia with ventricular septal defect and major Circulation 82:SIV157–SIV163
aortopulmonary collaterals. J Thorac Cardiovasc Surg Shanley CJ, Lupinetti FM, Shah NL et al. (1993) Primary
109:832–844 unifocalization for the absence of intrapericardial
Reddy VM, Petrossian E, McElhinney DB et al. (1997) One- pulmonary arteries in the neonate. J Thorac Cardiovasc
stage complete unifocalization in infants: when should Surg 106:237–247
the ventricular septal defect be closed? J Thorac Simpson KE, Huddleston CB, Foerster S, Nicholas R, Balzer
Cardiovasc Surg 113:858–866 D (2011) Successfull subxyhoid hybrid approach for
Redington AN (2006) Physiopathology of right ventricular placement of a Melody percutaneous pulmonary
failure. Semin Thorac Cardiovasc Surg Pediatr Card valve. Catheter Cardiovasc Interv. 78:108–111
Surg Annu 9:3–10 Skovránek J, Goetzová J, Samánek M (1976) Changes
Robinson JD, Rathod RH, Brown DW et al. (2011) The evolv- in muscle blood flow and development of the arm
ing role of intraoperative balloon pulmonary valvu- following the Blalock-Taussig anastomosis. Cardiology
loplasty in valve sparing repair of tetralogy of fallot. 61:131–137
J Thorac Cardiovasc Surg 142:1367–1373 Sluysmans T, Neven B, Rubay J et al. (1995) Early balloon
Rome JJ, Mayer JE, Castaneda AR, Lock JE (1993) Tetralogy of dilatation of the pulmonary valve in infants with
fallot with pulmonary atresia. Rehabilitation of diminu- tetralogy of fallot. Risks and benefits. Circulation
tive pulmonary arteries. Circulation 88:1691–1698 91:1506–1511
Romeih S, Groenink M, Roest AAW et al. (2012) Exercise Sokól S, Narkiewicz M, Billewicz OJ (1969) Subclavian
capacity and cardiac reserve in children and adolescents steal syndrome after Blalock-Taussig anastomoses.
with corrected pulmonary atresia with intact ventricular Cardiovasc Surg (Torino) 10:350–354
septum after univentricular palliation and biventricular Stellin G, Jonas RA, Goh TH, Brawn WJ, Venables AW, Mee
repair. J Thorac Cardiovasc Surg 143:569–575 RB (1983) Surgical treatment of absent pulmonary
Rychik J, Levy H, Gaynor JW, DeCampli WM, Spray TL (1998) valve syndrome in infants: relief of bronchial obstruc-
Outcome after operations for pulmonary atresia with tion. Ann Thorac Surg 36:468–475
intact ventricular septum. J Thorac Cardiovasc Surg Stellin G, Milanesi O, Rubino M et al. (1995) Repair of tetral-
116:924–931 ogy of fallot in the first six months of life: transatrial
Sachweh J, Däbritz S, Didilis V et al. (1998) Pulmonary versus transventricular approach. Ann Thorac Surg
artery stenosis after systemic-to-pulmonary shunt 60:S588–S591
operations. Eur J Cardiothorac Surg 14:229–234 Stenonis DN (1671) Embryo monstro affinis Parisii dissec-
Said SM, Burkhart HM, Dearani JA et al. (2012) Outcomes tus. In: Bartholini T (ed) Acta Medica et Philosophica
of surgical repair of double-chambered right ventricle. Hafniensia. Petrus Haubold, Kopenhagen, pp 200–203
Ann Thorac Surg 93:197–200 Stewart RD, Backer CL, Young L, Mavroudis C (2005)
Sandifort E (1777) Observationes anatomico pathologicae, Tetralogy of fallot: results of a pulmonary valve-spar-
caput primum. Eyk et Vygh, Lugduni Batavorum, pp 1–38 ing strategy. Ann Thorac Surg 80:1431–1438
Sandor GG, Cook AC, Sharland GK et al. (2002) Coronary Sullivan ID, Wren C, Stark J et al. (1988) Surgical unifocal-
artery abnormalities in pulmonary atresia with intact ization in pulmonary atresia and ventricular septal
ventricular septum diagnosed during fetal life. Cardiol defect. A realistic goal? Circulation 78:III5–III13
Young 12:436–444 Suzuki K, Ho SY, Anderson RH et al. (1998) Interventricular
Sawatari K, Imai Y, Kurosawa H, Isomatsu Y, Momma K communication in complete atrioventricular septal
(1989) Staged operation for pulmonary atresia and defect. Ann Thorac Surg 66:1389–1393
ventricular septal defect with major aortopulmonary Takabayashi S, Shimpo H, Miyake Y, Onoda K, Amano H,
collateral arteries. New technique for complete unifo- Maruyama K (2005) Postoperative prone position
calization. J Thorac Cardiovasc Surg 98:738–750 management of tetralogy of fallot with absent pul-
Schraut W, Lin CY, de la Fuente D, Arcilla R, Replogle RL monary valve syndrome. Jpn J Thorac Cardiovasc Surg
(1974) Avoiding postoperative dysrhythmias and 53:150–153
venous obstruction following Mustard’s operation. Tan JL, Gatzoulis MA, Ho SY (2006) Aortic root disease in
Technical considerations. Ann Thorac Surg 18:142–155 tetralogy of fallot. Curr Opin Cardiol 21:569–572
Schreiber C, Bauernschmitt R, Augustin N (2006) Tanaka AC, Atik E, Ikari NM et al. (1993) Transposition of
Implantation of a prosthesis mounted inside a self- the great arteries with posterior aorta. Arq Bras Cardiol
expandable stent in the pulmonary valvular area with- 60:339–342
out use of cardiopulmonary bypass. Ann Thorac Surg Taragin BH, Berdon WE, Printz B (2006) MRI assessment
81:e1–e3 of bronchial compression in absent pulmonary valve
Schwarz F, Blascgczok HC, Sinzobahamvya N et al. (2013) syndrome and review of the literature. Pediatr Radiol
The Taussig-Bing anomaly: long-term results. Eur 36:71–75
J Cardiothorac Surg 44(5):821–827 Taussig HB (1948) Tetralogy of fallot especially the care of
Sellors TH (1948) Surgery of pulmonary stenosis; a case in the cyanotic infant and child. Pediatrics 1:307
which the pulmonary valve was successfully divided. Taussig HB, Bing RJ (1949) Complete transposition of the
Lancet 26:988 aorta and a levoposition of the pulmonary artery;
Shaddy RE, Sturtevant JE, Judd VE, McGough EC (1990) clinical, physiological, and pathological findings. Am
Right ventricular growth after transventricular pul- Heart J 37:551–559
530 G. Ziemer and R. Kaulitz
Thiene G, Bortolotti U, Gallucci V, Valente ML, Volta SD Waterston DJ (1962) Treatment of fallot’s tetralogy in chil-
(1977) Pulmonary atresia with ventricular septal dren under 1 year of age. Rozhl Chir 41:181–183
defect. Further anatomical observations. Br Heart Watterson KG, Wilkinson JL, Karl TR, Mee RB (1991) Very
J 39:1223–1233 small pulmonary arteries: central end-to-side shunt.
Till K, Dave HH, Comber M, Bauersfeld U, Petre R (2011) Ann Thorac Surg 52:1132–1137
Realignment of the ventricular septum using partial Wertaschnigg D, Jaeggi M, Chityat D et al. (2013) Prenatal
direct closure of the ventricular septal defect in tetral- diagnosis and outcome of absent pulmonary valve
ogy of fallot. Eur J Cardiothorac Surg 40:1016–1019 syndrome: contemporary single-center experi-
Udink Ten Cate F, Sreeram N, Hamza H et al. (2013) Stenting ence and review of the literature. Ultrasound Obstet
the arterial duct in neonates and infants with con- Gynecol 41:162–167
genital heart disease and duct-dependent pulmonary Wilkinson JL, Arnold R, Anderson RH, Acerete F (1975)
blood flow. Catheter Cardiovasc Interv 82(3):E233–E243 “Posterior” transposition reconsidered. Br Heart
Van Praagh R (1968) What is the Taussig-Bing malforma- J 37:757–766
tion? Circulation 38:445–449 Williams WG, Burrows P, Freedom RM et al. (1991)
Van Praagh R (1989) Etienne-Louis Arthur fallot and his Thromboexclusion of the right ventricle in children
tetralogy: a new translation of Fallot’s summary and with pulmonary atresia and intact ventricular septum.
a modern reassessment of this anomaly. Eur J Cardio- J Thorac Cardiovasc Surg 101:222–229
Thorac Surg 3:381–386 Wong PC, Sanders SP, Jonas RA et al. (1991) Pulmonary
Van Praagh R, Van Praagh S, Nebesar RA, Muster AJ, Sinha valve-moderator band distance and association with
SN, Paul MH (1970) Tetralogy of fallot: underdevelop- development of double-chambered right ventricle.
ment of the pulmonary infundibulum and its sequelae. Am J Cardiol 68:1681–1686
Am J Cardiol 26:25–33 Yagihara T, Yamamoto F, Nishigaki K et al. (1996)
Vida VL, Padalino MA, Maschietto N, Biffanti R, Anderson Unifocalization for pulmonary atresia with ventricu-
RH, Milanesi O, Stellin G (2012) The balloon dilatation lar septal defect and major aortopulmonary collateral
of the pulmonary valve during early repair of tetralogy arteries. Thorac Cardiovasc Surg 112:392–402
of fallot. Catheter Cardiovasc Interv 80:915–921 Yoshimura N, Yamaguchi M, Ohashi H et al. (2003)
Vincenti M, Jacquot A, Guillaumont S et al. (2012) Thoracic Pulmonary atresia with intact ventricular septum:
computed tomography in absent pulmonary valve strategy based on right ventricular morphology.
syndrome. Pediatr Int 54:938–941 J Thorac Cardiovasc Surg 126:1417–1426
Vishnevsky A, Donetsky D (1960) A new method of the Yuan SM, Mishaly D, Shinfeld A, Raanani E (2008) Right
creation of the interarterial anastomosis in patients ventricular outflow tract reconstruction: valved con-
with the congenital cyanotic heart defects. Khirurgia duit of choice an clinical outcomes. J Cardiovasc Med
36:39–42 9:327–337
Waldman JD, Lamberti JJ, Mathewson JW, George L (1984) Zach M, Beitzke A, Singer H, Höfler H, Schellmann B (1979)
Surgical closure of the tricuspid valve for pulmonary atre- The syndrome of absent pulmonary valve and ventric-
sia, intact ventricular septum, and right ventricle to coro- ular septal defect—anatomical features and embryo-
nary artery communications. Pediatr Cardiol 5:221–223 logical implications. Basic Res Cardiol 74:54–68
15
531 16
Anomalies of the Left
Ventricular Outflow Tract
Viktor Hraška and Joachim Photiadis
References – 573
534 V. Hraška and J. Photiadis
Cuspis noncoronaria
A. coronaria dextra
536 V. Hraška and J. Photiadis
sc
Rig
ul
ba
Crist
sinus, NCS nonkoronarer
ar
ht ve
sinus (Picture: Sud et al.
a supraventrikularis
rior mitral
leaflet
Interv
RCS
Left atrium
valve
en
Ante
tricul
ar
se
ptu
m
M
s
Ri
u
sc
ou
gh
(p ent
ulo
ran
os ri
-m
tv
b emb
t) cl
Mem CS ranous
N
e
tum
sep trial
A
Atriu
m dextru
m
the ventricular muscle which makes up 45 % of the free wall of the left ventricle. The commissure
the circumference. between the left and the right coronary cusps lies
The entire right coronary sinus lies adjacent adjacent to the corresponding commissure of the
to the right ventricular outflow tract. Inferiorly, it pulmonary valve, and there is an area of dense
relates to the interventricular septum. The non- fibrous tissue between these commissures (Tsang
coronary sinus lies adjacent to the right and to et al. 2006).
the left atrial chambers and the interatrial sep-
tum. Inferiorly, the commissure between the
right and the noncoronary cusps is related to the 16.2 Valvular Aortic Valve Disease
16 membranous septum and to the penetrating
atrioventricular bundle. The bundle of His passes 16.2.1 Introduction
through the interventricular septum beneath the
noncoronary cusps, near its junction with the Congenital aortic valve stenosis accounts for
right coronary leaflet. The left part of the non- 3–5 % of all patients with congenital heart disease.
coronary sinus inserts into the anterior mitral The incidence in males is up to five times higher
leaflet. The commissure between the noncoro- than in females. Valvular aortic stenosis has a wide
nary and the left coronary leaflets is positioned spectrum of anatomical and clinical variations.
above the area of aortic-mitral valve continuity. Most patients, especially those with bicuspid aor-
The left coronary sinus lies adjacent to the left tic valves, will remain asymptomatic during child-
atrium on the right and to the lateral aspect of the hood. Later in life, as the leaflets become thickened
base of the left ventricle on the left. Inferiorly, it and fibrotic, they may need surgical treatment.
relates to the anterior leaflet of the mitral valve. Critical aortic stenosis in newborns and infants
To the right, this is the only part of the aortic root lies at the other end of the spectrum. These
that does not relate to a cardiac chamber but to patients represent a distinct and challenging group
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
537 16
with severe obstruction at valvular level and duc- The unicuspid valve usually has an eccentric,
tus-dependent systemic circulation. They may pinhole orifice with one defined commissure, or
become severely ill when the ductus closes, mani- no commissural attachment laterally to the aortic
festing with low cardiac output, acute renal failure, wall at all (Roberts 1973). Even in the absence of
and severe metabolic acidosis. commissural fusion, the valve is severely stenotic
Many other cardiac anomalies are associated from birth. The dysplastic bicuspid or tricuspid
with valvular aortic stenosis, including interrup- aortic valves may have a myxomatous appearance
tion of the aortic arch with posterior malalign- and thick cusps with a variable degree of periph-
ment of the ventricular septal defect (see 7 Chapter eral commissural fusion. This type of valve in sys-
«Congenital Anomalies of the Great Vessels», Sect. tole produces a dome with a central opening. It is
21.2.1.2), multiple left-sided obstructions such as believed that swollen, thickened leaflets, rather
supramitral or mitral stenosis (see 7 Chapter than fusion of commissures, cause the obstruc-
«Anomalous Pulmonary Venous Connections and tion (Ho et al. 1988). The aortic anulus and
Congenital Defects of the Atria, the Atrioventricular ascending aorta are usually hypoplastic. In gen-
Septum, and the Atrioventricular Valves», Sect. 12.2.4; eral, immaturity (gelatinous, myxomatous primi-
Sect. 12.3.5.2), underdevelopment of the left ven- tive tissue) and incomplete development (poorly
tricular cavity (see 7 Chapter «Surgery for Aortic defined leaflets, bicuspid or unicuspid morphol-
Atresia, Hypoplastic Left Heart Syndrome, and ogy, borderline aortic anulus) are typical features
Hypoplastic Left Heart Complex», Sect. 17.6.8), hypo- of critical neonatal aortic valve stenosis (McKay
plasia of the ascending aorta and aortic arch (see et al. 1992; Roberts and Ko 2006).
7 Sect. 21.2.1), and coarctation of the aorta (see
7 Sect. 21.2.1.1). In critical aortic stenosis, impor- 16.2.2.2 Pathophysiology
tant endocardial fibroelastosis may be presented. The postnatal course depends on the combination
of the severity of outflow tract obstruction and
the function and development of the left ventricle
16.2.2 ortic Stenosis in Neonates
A and shunts on the atrial and ductal level. There are
and Infants two potential scenarios.
If stenosis at valvular level is mild and the left
16.2.2.1 Anatomy ventricle is well developed with no obvious dys-
A reduced cross-section area in critical aortic ste- function, systemic circulation remains stable
nosis is the result of (1) deficiency or absence of despite closure of the arterial duct. Over time, the
one or more commissures, leading to a unicuspid left ventricular hypertrophy develops without
or bicuspid aortic valve; (2) immaturity with myx- signs of endocardial fibroelastosis.
omatous change and thickening of the valve More severe forms of valvular aortic stenosis
cusps, with or without commissural fusion/non- cause increased ventricular afterload, resulting
separation; and (3) hypoplasia of the valvular in increased ventricular wall stress and work-
anulus (. Fig. 16.4). load. This provides the stimulus for concentric
a b c
..Fig. 16.4 a–c Types of congenital valvular aortic stenosis. a Tricuspid valve. b Bicuspid valve with anterior and pos-
terior pocket. c Unicuspid valve
538 V. Hraška and J. Photiadis
hypertrophy of the left ventricle in order to nor- and degree of fibroelastosis should be estimated.
malize left ventricular wall stress, keeping an All cardiac structures should be assessed in two
appropriate left ventricular ejection fraction. The planes, and the Z-score for each parameter should
pressure gradient across the stenotic valve in addi- be calculated. The Doppler gradient across the
tion to the increase in myocardial wall thickness stenotic aortic valve can be greatly underesti-
may cause a mismatch between coronary and mated in a situation of low cardiac output with
myocardial perfusion pressure, potentially leading depressed left ventricular contractility and right
to myocardial ischemia, arrhythmias, and infarc- to left shunt at ductus level. Cardiac catheteriza-
tion. Endocardial fibroelastosis, as focal or diffuse tion to gain additional anatomic information is
cartilage-like fibroelastic thickening of the mural rarely necessary.
endocardium, may develop as a consequence of
chronic in utero or postnatal subendocardial isch- 16.2.2.4 Indications for Surgery
emia. Critically ill neonates should be stabilized by
This process could severely impair the systolic aggressive treatment, including mechanical venti-
and diastolic function of the left ventricle. If left lation, inotropic support, and correction of meta-
ventricular hypertrophy is incapable of normaliz- bolic acidosis, while patency of the ductus is
ing wall stress, afterload mismatch develops. At maintained by prostaglandins. After stabilization,
that point there is no further preload recruitable a trial discontinuation of prostaglandins can be
stroke volume, and any increase of afterload undertaken if the structures on the left side are
results in a progressive decrease in stroke volume. well developed, left ventricle function is adequate,
As a result, there will be elevated left ventricular and the degree of aortic valve stenosis is not
end-diastolic pressure and inadequate antegrade severe. No intervention is needed if ductal closure
flow through the left ventricle outflow tract. The is tolerated. On the other hand, dependency on
left ventricle starts to be more dilated than hyper- the ductal circulation is a clear indication for
trophied. Both systemic and coronary perfusions urgent valve centered measures.
become dependent on the patent ductus arterio-
sus. As the ductus begins to close after birth, in 16.2.2.5 Decision-Making
these cases, signs of circulatory collapse develop The key issue is to decide whether structures on
with hypotension, oliguria, and metabolic acido- the left side of the heart are adequate to sustain
sis (Jonas 2004). the systemic circulation, or whether they may
become adequate with available surgical
16.2.2.3 Clinical Presentation approaches, leading to a biventricular type of
and Diagnostics repair. Neonates with a well-developed mitral
Neonates and infants with critical aortic valve ste- valve, left ventricle, and aortic root will benefit
nosis and ductus-dependent circulation present from biventricular repair. At the other end of the
with varying degrees of cyanosis and reduced spectrum, there are patients with severe hypopla-
16 peripheral perfusion. Systolic ejection murmur sia of the mitral valve, left ventricle, and aortic
may or may not be present, depending on the root, who are suitable only for single-ventricle
function of the left ventricle and the amount of repair (Norwood procedure). The most challeng-
blood flow across the aortic valve. Occasionally, a ing group consists of patients with a borderline
neonate will present with circulatory collapse fol- left ventricle (Corno 2005). Correctly choosing
lowing ductal closure. the appropriate surgical pathway at the very
Neonates and infants with severe, noncritical beginning is critically important and contributes
aortic stenosis, without ductal dependency, may to an overall improvement in outcome for patients
present with a history of irritability, failure to with critical aortic stenosis. Converting the failed
thrive, and poor feeding within several weeks. The biventricular repair to Norwood Stage I is associ-
remaining cases with less severe aortic stenosis will ated with increased mortality (Rhodes et al. 1991).
be asymptomatic for the time being (Jonas 2004). The decision-making process in critical aortic ste-
Echocardiography and Doppler flow studies nosis, when deciding between a uni- or biventric-
are essential diagnostic tools. Apart from ana- ular type of repair, should include the following
tomic diagnosis, ventricular function should be considerations with respect to morphometric and
assessed, and the severity of aortic valve stenosis hemodynamic parameters:
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
539 16
1 . Morphometric parameters adequate to perform biventricular correction
The adequacy of the left ventricle is based (Eicken et al. 2010; Colan et al. 2006; Hickey et al.
on assessment of inflow and outflow structures 2007; Alsoufi et al. 2007).
and on the size of the left ventricle. In neonates The main limit of all recommendations and
with isolated aortic stenosis, the assessment by studies was that in the comparison between the
way of Rhodes score may predict the suitability biventricular and univentricular type of repair,
of a biventricular approach (Rhodes et al. the only surgical approach taken into consider-
1991). The scoring system is based on the aor- ation for biventricular repair was the aortic val-
tic root dimensions indexed to body surface votomy. All data shows that death after aortic
area (<3.5 cm2/m2), the ratio of the long-axis valvotomy is increasingly likely in patients with
dimension of the left ventricle to the long-axis smaller aortic valves and in the presence of endo-
dimension of the heart (<0.8), the indexed cardial fibroelastosis. Aortic valvotomy does not
mitral valve area (<4.75 cm2/m2), and left ven- address either of these morphologic issues, so it is
tricular mass (<35 g/m2). If one or more of not surprising that attempting an aortic valvot-
these factors are present, a univentricular omy in the face of an increasingly small aortic
approach should be considered. valve and increasing endocardial fibroelastosis
Reduced dimension of the mitral valve ori- will be completely ineffective. Other surgical
fice (<9 mm), a small diameter of the aortic options available in neonates with critical aortic
anulus (<5 mm) (Leung et al. 1991), and a car- stenosis such as the Ross or Ross-Konno proce-
diac apex not formed by the left ventricle dures, including the resection of fibroelastosis,
(Karl et al. 1990) are recognized risk factors have not been taken in consideration. The Ross
for mortality if a biventricular approach is procedure normalizes the left ventricular outflow
undertaken. The degree of endocardial fibro- tract dimensions and hemodynamics and allows
elastosis and previously recognized risk pre- easy access to the left ventricular myocardium for
dictors for hospital mortality in neonates with resection of endocardial fibroelastosis. Thereby, it
critical aortic stenosis (younger age, left ven- directly addresses the three most important mor-
tricular length, lower Z-score of the aortic phologic risk factors for biventricular repair: the
valve diameter, presence of moderate or more presence and degree of endocardial fibroelastosis,
tricuspid regurgitation) were identified in a the left ventricular length, and the lower Z-score
multi-institutional study conducted by the of the aortic valve diameter (Hanley et al. 2001).
Congenital Heart Surgeons’ Society (Lofland The decision-making protocol for critical aortic
et al. 2001). stenosis in our institution emphasizes assessment
2. Hemodynamic parameters of the inflow structures of the left ventricle, the
Left ventricular end-diastolic pressure length of the left ventricle, the flow characteristic in
>20 mmHg, left ventricular ejection fraction the ascending aorta, and the presence and degree of
<40 % (Pelech et al. 1987), and indexed left endocardial fibroelastosis (Hraska et al. 2007a, b).
ventricular end-diastolic volume <20 mL/m2 Single-ventricle palliation can be contem-
of body surface area (Keane et al. 1983) were plated if the mitral valve anulus is smaller than
clearly associated with death if biventricular 7 mm (more than–2z); if the apex of the heart is
repair was undertaken. The presence of total not formed by the LV, with diminished left ven-
or predominant antegrade blood flow in the tricular length and developed endocardial fibro-
ascending aorta and transverse aortic arch elastosis; and if flow in the ascending aorta is
correlated with survival after a biventricular predominantly reversed. Left ventricular outflow
type of repair (Kovalchin et al. 1998). structures, such as hypoplasia of the aortic anulus
and presence of endocardial fibroelastosis, are not
16.2.2.6 erman Pediatric Heart
G contraindications for biventricular repair if a Ross
Center Protocol (Deutsches operation is an option and the endocardial fibro-
Kinderherzzentrum (DKHZ) elastosis can be resected (Aszyk et al. 2012).
Protokoll) Historically, our treatment protocol was sur-
Proposed formulas have limited clinical value, gically driven. Recently, we have preferred an
and at this point there is no agreement on what interdisciplinary consensus-based approach.
combination of left-sided structures would be Critically ill neonates are stabilized by aggressive
540 V. Hraška and J. Photiadis
..Fig. 16.5 Treatment
protocol for critical aortic
valve stenosis at the Ger- Critical AoS
man Pediatric Heart Center,
Sankt Augustin. AoS aortic
stenosis, LV left ventricle,
EFE endocardial fibroelas- Bad LV function Good LV function EFE and/or Small Ao
tosis, Ao aorta, BV balloon
valvotomy, OV open val-
votomy
Ross-Konno
«Gentle» BV OV procedure
resuscitation, while patency of the ductus is edges of both thickened bicuspid cusps are taut
maintained by prostaglandins. A trial discontin- with no extra length and equal in length to the
uation of prostaglandins should be undertaken. diameter of the aortic root, they cannot open
Intervention (balloon or open valvotomy) is completely and thereby produce obstruction
indicated on a semi-elective basis if ductal clo- (Tsang et al. 2006). The aortic anulus is usually of
sure is tolerated. Dependency on the ductal cir- adequate size. Abnormal tricuspid aortic valves
culation is a clear indication for semi-urgent may not be obstructive during early infancy, but
intervention. If the LV function is depressed, a may become stenotic later in life due to leaflet
ballooning with a balloon not larger than 70 % of thickening and calcification.
diameter of the aortic anulus is performed to
slightly increase the effective orifice area of the 16.2.3.2 Pathophysiology
aortic valve, creating a minimal risk of regurgita- Pressure overload leads to the development of con-
tion. This so-called gentle ballooning is used as centric hypertrophy of the left ventricle to compen-
an intermittent step to stabilize the patient before sate for increased left ventricular wall stress. Even
open valvotomy. If the LV function is not severely though myocardial oxygen consumption and coro-
depressed, surgery is the method of choice nary blood flow normalize at rest, there are many
(Hraska et al. 2012). alterations of coronary circulation in the hypertro-
In case of a hypoplastic aortic anulus and phied left ventricle, with development of a noncom-
developed but resectable endocardial fibroelasto- pliant left ventricle susceptible to ischemia.
sis, a Ross-Konno operation can be considered. A Myocardial perfusion pressure is reduced because of
Ross option is considered also if balloon valvot- reduced aortic pressure (both systolic and diastolic)
omy or open valvotomy fails (. Fig. 16.5). on one side and elevated left ventricular end-diastolic
16 pressure on the other side. During exercise there may
be development of subendocardial ischemia, causing
16.2.3 Aortic Stenosis in Children angina-like symptoms. Ineffective increase of cardiac
output may lead to syncope (Jonas 2004).
16.2.3.1 Anatomy
Although all anatomic variations are possible, the 16.2.3.3 Clinical Presentation
valve is bicuspid in about 70 % of aortic valve ste- and Diagnosis
nosis cases in children, with the two remaining Typically, children with aortic stenosis may remain
commissures arranged as anterior and posterior, asymptomatic, despite a significant gradient. With
or left and right. There may be a third raphe or further progression of stenosis, symptoms of
false residual commissure. There are usually vari- breathlessness, syncope, and angina will develop
able degrees of peripheral fusion of one or both at some point. This is accompanied by a constant
commissures, creating a stenosis (Roberts 1973). risk of bacterial endocarditis and sudden death.
Stenosis can occur without commissural fusion, The ECG demonstrates left ventricular hyper-
simply as a result of thickened or myxomatous trophy, with T-wave inversion in V6, eventually
cusps and a bicuspid configuration. If the free with ST changes.
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
541 16
ECHO and Doppler flow studies are highly in asymptomatic patients with a peak gradient
reliable to estimate aortic valve gradient, mor- between 40 and 60 mmHg, in order to promote
phology of the valve, and functional and morpho- aortic anulus growth (. Fig. 16.6).
metric parameters of the left ventricle. In the
presence of additional defects and multilevel ste-
nosis, catheterization is indicated. 16.2.4 Aortic Insufficiency
In patients with normal left ventricular function, in Childhood
the severity of aortic stenosis is graded according to
well-established Doppler gradient measurements. 16.2.4.1 Anatomy
The stenosis is considered to be mild if the peak The morphology depends on the etiology. In
Doppler gradient is <40 mmHg or the mean gradi- children, aortic regurgitation may be associated
ent is <25 mmHg. If peak Doppler gradient is up to with certain congenital heart diseases (ventricu-
60 mmHg, or the mean gradient between 25 and lar septal defect, truncus arteriosus, tetralogy of
40 mmHg, the stenosis is considered to be moderate. Fallot, subaortic membrane, etc.) or with bicus-
If the maximum gradient is greater than 60 mmHg, pid aortic valves, with or without coarctation
or the mean gradient >40 mmHg, the stenosis is (Bonderman et al. 1999). Currently, postinter-
severe (Hossack et al. 1980; Baumgartner et al. 2010). vention aortic regurgitation with resting aortic
stenosis is frequently seen. Predominant mor-
16.2.3.4 Indications for Surgery phological factors contributing to aortic regur-
Surgery for aortic valve stenosis is indicated in gitation after balloon dilatation of the aortic
symptomatic patients when the peak Doppler valve are a combination of several pathologies:
gradient exceeds 40 mmHg, and LV hypertrophy anterior commissural avulsion, cusp dehiscence
and ECG changes are notable. In asymptomatic with retraction (presumably caused by long-
patients, a peak gradient more than 60 mmHg is standing balloon-induced tear), cusp tear or
an indication for procedure. Although each perforation, central incompetence due to calci-
patient should be evaluated individually, evidence fied cusps and sinus of Valsalva dilation, defi-
suggests that early intervention before myocardial cient cusps, and free cuspal edge adhesion to the
changes develop is beneficial (Jonas 2004). aortic wall (Solymar et al. 1992; Bacha et al.
Currently intervention/surgery is indicated even 2001; Jonas 2004).
..Fig. 16.6 Treatment
protocol for aortic valve
stenosis and insufficiency Aortic Insufficiency
at the German Pediatric Aortic Stenosis
Symptoms +/–
Heart Center, Sankt Augus- Symptoms +/–
AI ≥ Moderate
tin. LVEDD left ventricular Gradient >40 mm Hg
LVEDD ≥ 3 Z-score
end-diastolic diameter, AVR
aortic valve replacement
Surgery
Reconstruction of valve
Prolapse appears to be the most frequent cusp breathlessness, syncope, and angina may develop.
pathology in pure aortic regurgitation. This There is constant risk of bacterial endocarditis.
pathology may also coexist with root dilatation. The ECG demonstrates the left ventricular
Most importantly, prolapse may be induced by hypertrophy, with left ventricular strain or isch-
reduction of root diameters, particularly at the emia.
sinotubular level (de Kerchove et al. 2008; ECHO and Doppler flow studies are highly
Jeanmart et al. 2007). reliable in estimating aortic valve regurgitation,
Anuloaortic ectasia may be the result of dilata- morphology of the valve, and functional and
tion of the aortic root due to tissue disorder morphometric parameters of the LV. Aortic valve
(Marfan syndrome, Ehlers-Danlos syndrome, insufficiency is scored as 0–4 (none, trivial, mild,
Turner syndrome) (Dervanian et al. 1998). This moderate, and severe). The proximal aortic regur-
process begins in the sinuses of Valsalva with pear- gitation jet diameter to aortic anulus diameter
shaped aneurysmal enlargement of the aortic root, ratio offers more precise quantification of aortic
creating valvular incompetence by dilatation at the insufficiency, even in children. Catheterization is
commissural level. Another cause of aortic regur- rarely indicated.
gitation is rheumatic process with shortening of
the cusps between their free edges and the anular 16.2.4.4 Indications for Surgery
attachments. This results in rolling of the free The timing for surgery in patients with chronic
edges and central incompetence. Less common aortic regurgitation is controversial. Accepted
courses are bacterial endocarditis, myxomatous indications are the presence of symptoms, increas-
degeneration, spontaneous cusp rupture, etc. Pure ing significant LV dilation with or without dys-
congenital aortic insufficiency is very rare. function, or both in the setting of moderate or
even worse AR.
16.2.4.2 Pathophysiology of Aortic In order to achieve complete recovery of the
Regurgitation left ventricular function, surgery should be
Chronic aortic regurgitation represents a condi- performed before the Z-score of the preopera-
tion of combined volume overload and pressure tive left ventricular end-diastolic dimension is
overload. The balance between afterload excess, no greater than 4 (. Fig. 16.6) (Tsang et al.
preload reserve, and hypertrophy cannot be 2006). A further echocardiographic estimate of
maintained indefinitely in many patients, and more than moderate aortic regurgitation is, for
afterload mismatch and/or depressed contractility example, a jet length more than two-thirds back
ultimately result in a reduction in ejection frac- into the left ventricle, or if insufficiency induced
tion, first into the low normal range and then reversed flow is detectable in the descending
below normal. With time, during which the ven- aorta.
tricle develops progressive chamber enlargement
and a more spherical geometry, depressed myo-
16 cardial contractility predominates over excessive 16.2.5 Simple Reconstruction
loading as the cause of progressive systolic dys-
function. This can progress to the extent that the 16.2.5.1 Open Valvulotomie
full benefit of surgical correction of the regurgi- in Neonates and Infants
tant lesion in terms of recovery of left ventricle The operation is performed on cardiopulmonary
function and improved survival can no longer be bypass with mild hypothermia (32 °C) (Jeanmart
achieved (Bonow et al. 1998; Jonas 2004). et al. 2007; Hraska and Murin 2012).
The heart is approached through median ster-
16.2.4.3 Clinical Presentation notomy. The ductus arteriosus is dissected. After
and Diagnosis systemic heparinization, the patient is cannulated
With the advent of balloon valvotomy, patients for cardiopulmonary bypass with two venous
originally treated for aortic stenosis later present cannulas and with an arterial cannula in the distal
with predominant aortic regurgitation. The ascending aorta. The bypass is commenced, and
majority of children remain asymptomatic. With the patent ductus arteriosus is occluded with a
the progression of declining systolic function or snare. A left atrial vent is placed through right
elevated filling pressures of the left ventricle, pulmonary veins. Alternatively, vent could be
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
543 16
placed through interatrial septum after opening easier. In order to maximize the effective orifice
of the right atrium. The aortic cross-clamp is area, especially in a bicuspid scenario, the com-
applied and antegrade cold crystalloid cardiople- missurotomy can be extended in circumferential
gia is delivered. An oblique, inverted hockey stick fashion beyond the commissure into the aortic
type of incision is made from the anterior aspect wall, splitting the aortic wall in two layers (Ilbawi
of the aorta toward the noncoronary sinus of et al. 1991). Incision into a false raphe should be
Valsalva. The aortic valve is carefully examined. A avoided; however, debridement of the raphe usu-
small cardiotomy sucker placed through the valve ally improves mobility of the cusps with subse-
orifice can improve inspection of the valve. The quent enlargement of the effective orifice area of
fused/nondivided commissures are carefully the valve. In older patients, bicuspidalization even
divided with a knife, ensuring that the individual of an unicuspid valve can be considered (Schäfers
cusps created are well supported and not liable to et al. 2008). Nevertheless, the design of a bicuspid
prolapse. The incision is short, usually 1–2 mm in aortic valve has a limited prognosis with potential
length. Even a short incision may result in an ade- failure and the invariable development of stenosis
quate opening of the outflow orifice, because the (Robicsek et al. 2004).
flow is a function of the fourth power of the If completion of the commissurotomy does
radius. Obstructive myxomatous and fibrous not result in adequate opening, despite the nor-
nodules on the leaflets are removed. This implies mal anulus dimension, conversion to a three-
careful and meticulous thinning/shaving of cusps cusp valve should be considered. Usually, there is
with a knife. Frequently, a cleavage plane is identi- a prominent, thickened, even calcified false
fied between the fibrosis and what seems to be raphe. The false raphe is incised. Thickened and
thin pliable aortic cusp tissue. Properly performed immobile parts of the newly created cusps are
«shaving» greatly improves mobility of the cusps completely removed, leaving as much native
and increases the effective orifice area of the aortic mobile tissue as possible. Autologous or bovine
valve. The false raphe, if present as the ultimate pericardial augmentation of these two cusps is
remnant of a nondeveloped commissure, does not performed. Augmentation of the third cusp
provide any lateral support for the cusp and depends on the effectiveness of coaptation with
should not be incised. After performing the val- the already augmented cusps. Usually, the sym-
vulotomy, the aortotomy is closed with a running metric augmentation of all cusps involved pro-
suture, the left side of the heart is de-aired, and vides sufficient overlapping of cusps and
the aortic cross-clamp is removed. After rewarm- optimizes the height and width of the intercusp
ing, the patient is weaned off cardiopulmonary triangle, which governs the area of cusp apposi-
bypass. Transesophageal echocardiographic tion (Hraska and Murin 2012).
assessment of left ventricle function, aortic valve Alternatively, the raphe between the other
function, and flow characteristic in the left ven- unsupported cusp is incised, and a pericardial tri-
tricle outflow tract should be carried out rou- angle patch is folded and sutured along both edges
tinely. The ductus arteriosus should always be of the divided raphe and vertically to the aortic
ligated, if the patient is stable. If cardiac output is wall to provide support in the diastole, preventing
not adequate, even on aggressive inotropic sup- cusp prolapse (Tolan et al. 1997, . Fig. 16.8).
port, the arterial duct can be left open and main- Dysplastic bicuspid aortic valves are suitable
tained patent on prostaglandins or intraoperatively for tricuspidalization using most of available
stented to provide additional systemic perfusion native tissue for creation of two adjacent cusps
from the right ventricle. Under these circum- while adding a third cusp from pericardium. One
stances, bilateral pulmonary artery banding or both native cusps are detached from the anulus
should be considered as well (. Fig. 16.7). and reattached to anatomically ideal position, cre-
ating new commissures. Subsequently, cusp from
16.2.5.2 Open Valvulotomie in pericardium is fashioned in between (Pretre et al.
Childhood/Tricuspidalization 2006).
of the Aortic Valve If there is a risk of aortic narrowing at direct aor-
The management of the operation is similar to totomy closure, the supravalvular aortic segment
that described for neonates and infants. should be augmented with a patch instead. Should
Visualization of the aortic valve anatomy is much there be post stenotic dilatation of the ascending
544 V. Hraška and J. Photiadis
a b
16
..Fig. 16.7 a The fused/nonseparated commissures are incised and the incision is extended in a circumferential
fashion beyond and above the commissures into the ventriculoaortic junction. b The incision is deepened in the muscle
underneath the commissure between the right and left leaflets. c Myxomatous nodules and areas of leaflet thickening
are thinned out. d Releasing of the rudimentary commissure. e Enlargement of the supravalvular area with a patch
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
545 16
a b
..Fig. 16.8 a View of a congenital bicuspid aortic valve showing one larger cusp with a median raphe. b Incision of
the median raphe. c A pericardial triangle (inset) is folded and sutured along both edges of the divided raphe and verti-
cally to the aortic wall to provide support in the diastole
aorta, wedge resection of the anterior part of aorta is (Borghi et al. 1999; McCrindle et al. 2001). Both
performed to restore optimal sinotubular dimension. are palliative procedures: sooner or later, reinter-
vention is likely. Whereas patients undergoing
16.2.5.3 Discussion: Simple open valvotomy are more likely to have residual
Reconstruction stenosis, those undergoing balloon valvotomy are
The therapeutic goal is to relieve the aortic more likely to develop severe insufficiency
obstruction and provide lasting freedom from (Lofland et al. 2001). Predominant postvalvotomy
residual or recurrent stenosis, without causing pathology is an important predictor of the long-
significant aortic regurgitation. Satisfactory early term outcome. Mild aortic stenosis is usually very
outcomes have been reported in neonates and well tolerated, and patients have normal exercise
infants with critical aortic stenosis treated with tolerance (Mitchell et al. 2003). The need for aor-
both balloon valvotomy and open valvotomy tic valve replacement can be prolonged by
546 V. Hraška and J. Photiadis
LCO RCO
LCO RCO
..Fig. 16.9 a, b The prolapse of the fused leaflet is corrected by a triangular resection only. LCO left coronary artery
orifice, RCO right coronary artery orifice
3. More extensive cusp destruction, with defi- the sinotubular bar. The running 6–0 mono-
cient cusps, requires an extension procedure filament suture line is carried from the center
using a pericardial patch. The length of the half of the native cusp to each commissure and
moon-shaped patch should be 15–20 % greater then up onto the aortic wall 2–3 mm above the
16 than the diameter of the aorta. The 20 % addi- original commissural insertion, staying away
tional length accounts for a reduction in the from the coronary ostia. With each patch in
pericardial free cusp edge width from a purse place, the newly constructed pericardial cusp
string effect with a running polypropylene edges are trimmed to provide a uniform cusp
suture. The height is governed by the extent depth. The commissures are suspended and
of augmentation and anticipated height of the reinforced with 5–0 monofilament sutures
newly created commissure. The destroyed part (Caspi et al. 1994; Polimenakos et al. 2010;
of the cusp is removed, and the patch is tai- Hraska and Murin 2012) (. Fig. 16.10). A
lored to achieve a «normal» morphology of the small ascending aorta should be enlarged with
newly created cusp. A deliberate overcorrec- a patch, especially if a three-cusp extension is
tion in the cusp reconstruction is attempted to performed, to avoid stenosis at the sinotubular
provide additional material to extend the line junction.
of coaptation of the cusps 3–5 mm above the 4. Mild dilatation of the anulus can be man-
line of original coaptation and 2–3 mm above aged with a subcommissural anuloplasty.
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
549 16
a
LCO RCO
LCO RCO
..Fig. 16.10 a, b The deficient shallow cusp is augmented with a glutaraldehyde-treated pericardial patch suspended
to the aortic wall, to achieve normal depth and length. At the commissures, an overlap is created, thereby ensuring
optimal coaptation and thus addressing the commissural separation. LCO left coronary artery orifice, RCO right coronary
artery orifice
This reduces the circumference of the anu- is suitable for a valve-sparing remodeling
lus and increases cusp coaptation without technique, the so-called Yacoub procedure
interfering with the range of motion of the (Sarsan and Yacoub 1993). The native valve
cusps. The amount of plication depends on is preserved; all three sinuses of Valsalva are
the level at which the sutures are placed. excised. The scalloped Dacron graft is sewn
The lower the sutures are placed at the com- proximally to the residual aortic sinus tissue
missures, the greater the anular plication around the aortic cusps and commissures.
and degree of cusp coaptation. Horizontal The coronary arteries are reimplanted to the
mattress sutures with pledgets are placed newly created sinuses (see 7 Chapter «Acquired
through the anulus on each side of the com- Lesions of the Aortic Valve», Sect. 24.5.1.2).
missure and tied. Care is taken to avoid One putative advantage of the remodeling
contact with the cusps and coronary ostium approach is that the graft billows, thereby
(Cosgrow et al. 1991). mimicking the natural sinuses of Valsalva.
5. Aortic regurgitation resulting from restricted This allows more natural cusp motion (Leyh
cusp motion due to dilatation in the sinotu- et al. 1999) and should theoretically reduce
bular junction with no aortic anulus dilatation cusp-closing stresses and thereby enhance
550 V. Hraška and J. Photiadis
a b
..Fig. 16.11 a–c Ross operation: a The aorta is transected just above the sinotubular junction, and the pulmonary
autograft is harvested taking care to avoid injury to the LCA, the PA valves, and septal perforator of the LAD. b The right
and left coronary buttons are formed and dissected for maximal mobilization. The pulmonary autograft is implanted in
the left ventricular outflow tract using a running suture technique. c The left and right coronary arteries are reimplanted
into the neoaortic trunk. The neoaortic reconstruction is completed by proximal anastomosis with the ascending aorta.
16 A pulmonary homograft is used for reconstruction of the right ventricular outflow tract
placing the sinuses of the homograft into the 16.2.6.4 ortic Root Replacement
A
appropriate position. A mobile pulmonary artery with an Aortic Homograft
bifurcation facilitates construction of this anasto- Homograft aortic root replacement is currently
mosis. The posterior aspect of the proximal suture used for treatment of complicated bacterial endo-
line between the right ventricular outflow tract carditis with aortic root abscesses and in the man-
and the homograft is performed by placing shal- agement of complex left ventricular outflow tract
low bites avoid the coronaries. Anteriorly, the obstructions, when Ross-Konno is not an option.
suture line takes the full thickness of the muscle of The operation is performed using a standard
the right ventricular outflow tract. After de-airing, bypass cannulation technique. We provide myo-
the aortic clamp is released. An ECHO may be cardial preservation preferentially through the
routinely performed after weaning the patient off coronary sinus.
bypass (Mohan Reddy et al. 1998; Elkins et al. The proximal transverse aortotomy is per-
1994) (. Fig. 16.11). formed. The coronary arteries are harvested on
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
553 16
large buttons with as much adjacent aortic wall tis- sion. A variety of valvuloplasty techniques have
sue as possible. Full mobilization of the coronaries is demonstrated good early and intermediate results;
achieved. The aortic valve cusps are excised and these techniques range from commissurotomy,
root enlargement (Konno incision) is performed if reattachment of a torn leaflet, resuspension of
necessary. An aortic homograft of an appropriate cusps, thinning of a dysplastic valve, removal of
size is chosen. It is always possible to use larger dysplastic nodules, and symmetrical pericardial
homograft than native aortic root. The homograft is augmentation of cusps to a reduction anuloplasty.
then sutured to the left ventricular outflow tract Aortic valve repair is safe, with early mortality
opening using a continuous suture. Sewing muscle approaching zero and with 3-, 5-, and 18-year
to muscle is avoided by anticlockwise rotating the freedom from valve replacement of 90, 75, and
homograft 120°, so that the right coronary artery 60 % (McMullan et al. 2007; Alsoufi et al. 2006;
ostium faces the recipient’s left coronary artery. The Polimenakos et al. 2010).
second running adventitial suture over the remnant With this time frame, aortic valve repair is
of the aortic wall reinforces the first one. The left considered to be a good temporary solution as it
coronary artery button is sewn into the enlarged offers reduction of regurgitation and stenosis and
ostium of the homograft’s right coronary artery. The stabilization of the ventricular dimensions until
right anterior commissure is marked on the outside the patients grow older, at which time the full
of the homograft to avoid inadvertent injury to the range of possible treatment options, including
valve leaflet during the implantation of the right mechanical valve or the Ross procedure, might be
coronary artery button. The homograft is then anas- used if necessary (Bacha et al. 2001; Tweddell et al.
tomosed to the distal aorta using a continuous 2005a, b; Caspi et al. 1994; Alsoufi et al. 2006). The
suture. The aortic root is pressurized by cardiople- selection of the most appropriate substitute in
gia, and the right coronary artery is reimplanted to children with irreparable aortic valve lesions
the appropriate site, avoiding rotation, tension, or remains controversial, with all the available
kinking (Clarke 1991). options having certain drawbacks. The pulmonary
autograft procedure is an alternative to a mechani-
16.2.6.5 Discussion Complex cal prosthesis, but disproportionate dilation of the
Reconstruction neoaortic root, autograft regurgitation, and the
The ideal operation for aortic valve insufficiency need for pulmonary homograft exchanges remain
would preserve the native aortic valve and as much concerns (Elkins et al. 1996; Hörer et al. 2009).
of its functional unit (comprising the left ventricu- The Ross procedure is not a cure for aortic
lar outflow tract, the anulus, the sinus of Valsalva, valve disease. The Ross procedure should not be
and the sinotubular junction) as possible. Surgical considered if the pulmonary valve is deformed,
options in children with aortic valve disease remain damaged by acquired disease, or compromised by
limited. There is no ideal valve substitute; therefore, previous surgical procedures. Furthermore, certain
valve repair should take preference over any type of connective tissue disorders, such as the Marfan
valve replacement if a reduction of aortic regurgita- syndrome, probably affect the pulmonary valve
tion and/or stenosis can be accomplished to an and also disqualify it from consideration for auto-
acceptable level. A mild degree of postrepair resid- grafting. Results achieved with the Ross operation
ual hemodynamic lesions is well tolerated in the in rheumatic aortic valve disease were disappoint-
long run. The repaired valve has growth potential ing. The great advantages of the Ross procedure are
and no anticoagulation is needed. Not all valves are the superior hemodynamic performance, the
amenable to repair. Anatomic elements necessary growth potential, and the lack of need for antico-
for valve repair include a sufficient anulus, mobile agulant therapy (Al-Halees et al. 2002). The Ross
cusps, and the ability to achieve coaptation without procedure even allows replacement of stenotic or
inducing stenosis. Repair of congenitally bicuspid regurgitant aortic valves earlier in life, thus avoid-
aortic valves by tricuspid conversion confers better ing repeated surgical reinterventions, which may
outcomes, probably due to reduction in leaflet stress provide only short-term palliation and potentially
load and improved flow patterns (Odim et al. 2005). exacerbate ventricular function (Mohan Reddy
The best results are obtained when sufficient et al. 1998; Marino et al. 1999; Ohye et al. 2001).
native aortic tissue, including the hinge point of Root replacement technique, exclusively used for
the native leaflet, remains prior to leaflet exten- small children, demonstrated excellent midterm
554 V. Hraška and J. Photiadis
functional results with a mortality rate <5 % in The risk of autograft failure in these specific
both simple and complex left heart lesions, even in subsets of patients remains to be determined, and
neonates and infants (Stelzer et al. 1998; Marino mechanical valve replacement should be consid-
et al. 1999; Ohye et al. 2001). Eighty-eight to 95 % ered as a reasonable option. On the other hand,
freedom from more than trivial neoaortic regurgi- patients with mixed severe aortic stenosis and
tation or valve replacement at a 5-year follow-up insufficiency might benefit more from a Ross
can be expected (Elkins 1996; Hraska 2004; Elkins operation than a repair (Bacha et al. 2001).
et al. 1998; Takkenberg et al. 2009; Brown et al. Freedom from pulmonary homograft replace-
2009). ments after the Ross procedure is about 90 % at
In adults, excellent midterm outcome can be 12-year follow-up (Elkins et al. 2001). These results
expected, when aortic root was enforced or sub- are superior to other right-sided reconstructions,
coronary implantation technique was used most probably because an orthotopically placed
(Charitos et al. 2009). The main concern is dilata- pulmonary homograft provides the ideal hemody-
tion of the neoaortic root leading to progression namics and flow pattern. Nevertheless, reopera-
of aortic regurgitation, especially in the settings of tion is likely to be necessary in very young children
geometric mismatch of the aortic and pulmonary in whom more accelerated degeneration of the
roots and bicuspid, chronically regurgitant aortic homograft can be expected. Remodeling of the left
valves (David et al. 1996; Laudito et al. 2001). ventricle after the Ross procedure, with normal-
The adjustment of the diameter of the aortic ization of the left ventricle diastolic diameter index
anulus and/or the sinotubular junction of the and left ventricle mass index, can be expected over
aorta seems to be an important factor in prevent- a 3–12 months period in every patient without the
ing neoaortic regurgitation after the Ross proce- associated lesion (Hraska et al. 2004). The long-
dure. The sinotubular junction can easily be term outcome of the Ross operation is better than
adjusted to the appropriate diameter by reducing other aortic valve replacement options for chil-
the diameter of the ascending aorta before the dren, but the unknown long-term outlook of auto-
autograft-ascending aorta anastomosis is finished. graft replacement and degeneration of homograft
Adjustment of the diameter of the aortic anulus placed to the RVOT remind us that the Ross pro-
by plication or anular reinforcement should be cedure results in a two-valve pathology.
considered only in older children (over 15 years Mechanical valve replacement in children
old) (Brown et al. 2010b). is complicated by the need for systemic anti-
If the growth of the patient is not an issue, it was coagulation, by lack of anular growth, and by
suggested encasing the autograft in a Dacron tube patient-prosthesis mismatch (see also 7 Chapter
to prevent dilatation (Slater et al. 2005; David et al. «Acquired Lesions of the Aortic Valve», Sect. 24.5.5.7).
2000). Mechanical adaptation phenomena in the Operative mortality is low, ranging between 0 and
wall of the autograft associated with elastic fiber 5 % (Ibrahim et al. 1994; Mazzitelli et al. 1998),
fragmentation are another potential risk factor for and is associated with poor preoperative status.
16 Ross failure (De Sa et al. 1999; Carr-White et al. Long-term survival rates ranging from 85 to 64 %
2000; Ishizaka et al. 2003), which call for decreas- at 20-year follow-up reflect the complexity of the
ing wall stress by aggressive postoperative control underlying congenital disease, being more favor-
of blood pressure (Hraska et al. 2004). able for isolated aortic stenosis (Alexiou et al.
Patients with bicuspid valve, chronic aortic 2000; Champsaur et al. 1997). The anticoagula-
insufficiency, and a dilated aortic root are less tion management in infants and small children is
suitable candidates for a Ross operation. It is pos- particularly cumbersome. In the adolescent age
sible that aortic insufficiency alters the geometry group, noncompliance with activity restriction
and tissue characteristic of the subvalvular left and medical regiments, and high-risk behavior,
ventricle outflow tract, with propensity to postop- makes the use of a mechanical valve less attractive.
erative dilatation of the neoaortic root and pro- Although 93 % freedom from thromboembolism
gression of aortic insufficiency. Another at 20-year follow-up was reported, the risks of
possibility is that these patients have an inherent thromboembolism, valve thrombosis, anticoag-
abnormality of the pulmonary anulus, or valves ulation-related hemorrhage, and prosthetic valve
with mucocystic degeneration of the stroma endocarditis remain long term (Alexiou et al.
(Laudito et al. 2001). 2000; Vongpatanasin et al. 1996). Placement of a
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
555 16
prosthetic valve in small children requires anu- Other rare causes of subvalvular obstruction
lar enlargement to accommodate an adequate are abnormal attachments of the mitral valve,
prosthesis which may result in late ventricular dys- accessory tissue, abnormal insertion of the mitral
function (Sharma et al. 2004). Repeated prosthetic papillary muscle, abnormal muscular bands
valve replacement is unavoidable during child- within the left ventricular outflow tract, and
hood, because of somatic growth and left ventricu- space-occupying lesions in the left ventricular
lar outflow obstruction. Earlier referral for redo is outflow tract. Shone and associates described a
warranted before the onset of ventricular dysfunc- complex of subvalvular aortic stenosis in associa-
tion. Freedom from surgical reintervention rates tion with supravalvular mitral ring, parachute
range from 70 to 91 % at 10-year follow-up and mitral valve, and coarctation of the aorta (see also
53 to 86 % at 20-year follow-up (Mazzitelli et al. 7 Chapters «Anomalous Pulmonary Venous
1998; Alexiou 2000). On the other hand, mechani- Connections and Congenital Defects of the Atria, the
cal aortic valve replacement might be a lifelong Atrioventricular Septum, and the Atrioventricular
solution, mainly for patients where large sizes Valves», Sect. 12.3.5.4 and «Congenital Anomalies of
(≥21 mm) of the valve can be inserted. The major- the Great Vessels», Sect. 21.2.1.1) (Shone et al. 1963).
ity of survivors are in the NYHA I class, leading Rarely seen hypertrophic obstructive cardiomy-
normal or nearly normal lives. Mechanical aor- opathy usually creates dynamic subvalvular
tic valve replacement, with optional enlargement obstruction due to opposition of the anterior leaf-
of the aortic root and long-term anticoagulation, let of mitral valve against the asymmetrically
remains a good alternative to the Ross procedure. hypertrophied ventricular septum.
Other alternatives for valve replacement are
unappealing, including a tissue valve with rapid
degeneration in children, or an allograft with no 16.3.2 Anatomy
growth potential. Aortic homograft replacement
of the aortic valve is a reasonable alternative in the Fixed subaortic obstruction can be discrete or dif-
case of endocarditis. Unfortunately, the durability fuse. The discrete form represents about 70 % of
of an aortic homograft is limited, with an 82 % cases and is characterized by a thin, fibromuscular
5-year freedom from reintervention (Lupinetti shelf located at the area of aortomitral continuity
et al. 2003). Furthermore, the risk at reoperation with the extension toward the interventricular
is not negligible and warrants that they be used septum.
with caution. The allograft may be densely calci- It is typically seen in patients with a more
fied, sternal reentry may be perilous, the coronary acute angle between the long axis of the left ven-
ostia may need to be reimplanted again, and the tricle and the aorta. This angulation leads to
aortic anulus may have scarred down excessively. imbalance in shear forces which causes the prolif-
eration of tissue that forms the membrane
(Kleinert and Geva 1993; Jonas 2004). There may
16.3 Subaortic Stenosis be an abnormal anatomic relationship with the
mitral valve, which is displaced anteriorly and
16.3.1 Introduction rotates in a clockwise direction.
Diffuse subvalvular aortic stenosis is caused
Subvalvular stenosis is an obstruction of the left by a circumferential narrowing which commences
ventricular outflow tract below the aortic valve. at the anular level and extends downward for
The most common form is a fixed obstruction due 1–3 cm into the left ventricle cavity.
to a discrete membrane or a diffuse, tunnel-like It is often a secondary lesion seen after previ-
obstruction. This condition occurs in patients ous resection of a discrete subvalvular aortic
with associated congenital heart disease, including membrane. Scarring from the initial resection in
ventricular septal defect, coarctation of the aorta, conjunction with an abnormal-shaped left ven-
interrupted aortic arch, atrioventricular canal, and tricular outflow tract may result in progressive
others. The lesion may develop in patients with fibromuscular proliferation and creation of a left
these defects before operation, but also may appear ventricular outflow tract tunnel.
and progress significantly after surgical correction Hypertrophic obstructive cardiomyopathy is
of the associated defects (Gersony 2001). characterized by primary asymmetric ventricular
556 V. Hraška and J. Photiadis
septal hypertrophy and a dynamic obstruction third of all cases. Contrary to the discrete form,
associated with abnormal systolic anterior motion the tunnel type of obstruction is often seen as a
of the anterior leaflet of the mitral valve. The his- part of complex lesions, even in neonates and
tological hallmark of this disease is the presence infants. Hypertrophic obstructive cardiomyopa-
of disorganized and bizarrely shaped hypertro- thy is rarely seen in the pediatric population;
phied myocytes (Goodwin et al. 1960). symptoms usually develop in the second or third
decade of life.
An ECHO is the method of choice for visual-
16.3.3 Pathophysiology izing the narrowed left ventricular outflow tract,
to discriminate between discrete and diffuse
Pressure overload leads to concentric hypertro- forms, to measure gradient, and to evaluate the
phy of the left ventricle and myocardial hypoper- function of the aortic valve and the functional and
fusion with the same sequels as seen in valvular morphometric parameters of the left ventricle.
aortic stenosis. Catheterization, with direct gradient measure-
Increased shear stress due to turbulence might ment, is useful in assessing complex, multiple lev-
cause endocardial injury and subsequent prolifera- els of obstruction of the left ventricular outflow
tion and fibrosis (Cape et al. 1997). The membrane tract and the aorta.
which results creates more turbulence, further accel-
erating the process of proliferation and fibrosis. The
turbulence can also cause injury to the aortic valve 16.3.5 Indications for Surgery
leaflets, with subsequent thickening and distortion
resulting in aortic insufficiency. As compared to Symptoms associated with left ventricular outflow
other congenital heart defects, discrete subvalvular tract obstruction (syncope, angina, diminished
aortic membrane is virtually never recognized in exercise tolerance, etc.) are indications for surgery
early infancy, but appears to be an «acquired» lesion(. Fig. 16.12). The surgical intervention is
secondary to flow disturbances in the left ventricular
indicated when the peak Doppler gradient across
outflow tract (Gewillig et al. 1992). the left ventricular outflow tract is 30 mmHg or
more in discrete subvalvular aortic membrane
and a more than 60 mmHg (mean gradient
16.3.4 Clinical Presentation ≥40 mmHg) in tunnel-type obstruction. Patients
and Diagnosis with peak gradient <30 mmHg and no significant
left ventricle hypertrophy are followed closely for
Isolated discrete subvalvular aortic membrane is progression, especially in the first several years of
typically seen in older children with symptoms life. Surgery is also advocated in infants and chil-
similar to those of the valvular variety. Trivial or dren in the presence of aortic regurgitation
mild aortic regurgitation is present in about two- regardless of gradient (Jonas 2004).
16
..Fig. 16.12 Treatment
protocol for subvalvular
aortic stenosis at the Ger- Symptoms
man Pediatric Heart Center,
Sankt Augustin, according
to peak gradients mea- Gradient > 30 mm Hg
sured, symptoms, or noted in the discrete type
aortic regurgitation (AR)
Surgery
Gradient > 60 mm Hg
in the tunnel type
AR
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
557 16
Indication for operation in hypertrophic The technique is similar to that utilized for diffuse
obstructive cardiomyopathy is driven by the fibromuscular stenosis (Hraska et al. 2007b).
progression of symptoms despite the maximum Complications of this procedure include the
medical therapy. In symptomatic patients, a creation of an iatrogenic VSD, if resection is
peak Doppler gradient above 50 mmHg under excessively deep, damage to the conduction sys-
resting conditions or with provocation, prefera- tem if the resection is made under the noncoro-
bly using physiological stress, is an indication nary leaflet, and damage to the aortic cusps due
for surgery. In asymptomatic patients, a peak to carefree manipulation with instruments
Doppler gradient >75–100 mmHg at rest is an (. Fig. 16.13).
indication for surgery (Maron et al. 2003;
Dearani et al. 2005). 16.3.6.2 Modified Konno Operation
The aorta is opened vertically with an extension
toward the intercoronary commissure, staying
16.3.6 Surgical Correction away from the right coronary artery. An oblique
incision is made into the infundibulum of the
16.3.6.1 Resection of a Discrete right ventricle. A right-angled instrument is
Subaortic Membrane passed through the aortic valve to guide an inci-
The approach, the bypass strategy, and myocar- sion into the infundibular septum. Because of
dial protection are the same as in other opera- the extreme thickness of the septum, the inci-
tions for aortic valve surgery. An oblique, sion is always challenging. The incision should
inverted hockey stick type of incision is made remain above the muscle of Lancisi in order to
from the anterior aspect of the aorta toward the avoid the conduction system. The incision is
noncoronary sinus of Valsalva. The right coro- enlarged leftward below the pulmonary valve.
nary cusp is retracted anteriorly, exposing the Working back and forth between the aortic
subvalvular area. Alternatively, a nasal speculum approach and the septal incision, the incision is
can be used to improve exposure. The extent and extended toward the intercoronary commis-
relationship of the membrane to the surrounding sural triangle without injuring the cusps of the
structures, especially to the aortic valve and to aortic valve. Extra care is taken when resecting
the anterior leaflet of the mitral valve, are care- tissue from the inferior edge of the incision, to
fully assessed. The membrane is either grasped reduce the risk of injury to the His bundle. At
with forceps or stabilized by a skin hook. Caution this point, any fibrous subaortic tissue is
should be exercised not to damage the aortic resected. A patch of glutaraldehyde- treated
valve by instruments. Under the right coronary pericardium or Gore-Tex is sutured with inter-
cusp, close to the intercoronary commissure, a rupted pledgeted sutures (or a running suture)
radial incision is made through the white fibrous to close the created ventricular septal defect,
tissue only to its junction with the muscle. with all the sutures remaining on the right ven-
Combination of sharp and blunt dissection is tricular aspect of the septum. The incision in
used to peel the membrane off the muscle all the the aorta and the free wall of the right ventricu-
way round the entire circumference of the sub- lar outflow tract is then closed with a patch of
aortic region. If the membrane is attached to the pericardium (Jahangiri et al. 2000).
aortic valve cusps, careful sharp dissection Alternatively, the right ventricular aspect of the
should be performed to remove any fibrous tis- right ventricular outflow tract can be exposed
sue which could obstruct the left ventricular out- working through the right atrium and the tri-
flow tract. The location of the conduction tissue cuspid valve. After pulling away the septal leaf-
near the membranous septum should be identi- let of the tricuspid valve, the septal incision is
fied, and any deep, sharp dissection must be made, starting above the insertion of the Lancisi
avoided. muscle, with an extension toward the pulmo-
A myectomy is routinely performed to enlarge nary valve. The risk of damaging the conduc-
the left ventricular outflow tract and to reduce the tion system is thereby lessened. The rest of the
likelihood of recurrence. A wedge of muscle under operation is the same as described above
the left half of the right coronary cups is excised. (. Figs. 16.14, 16.15, and 16.16).
558 V. Hraška and J. Photiadis
a b
..Fig. 16.13 a, b Diagram of a the pathoanatomy and b the resection of a subvalvular diaphragm, extreme form of
discrete membranous subvalvular aortic stenosis
a b
16 ..Fig. 16.17 Ross-Konno procedure. a The aortic anulus is enlarged by incising the interventricular septum (dashed
line) between the left and right commissures. The length of the incision is frequently limited to just beyond the anulus.
A myectomy may also increase the orifice area, limiting the extent of the ventriculoplasty incision. b The autograft is
sutured to the margins with interrupted pledgeted sutures. A short apron is left on the autograft to fill the ventriculo-
plasty site. c Coronary buttons are reimplanted into the autograft. d Completed reconstruction
ple interrupted sutures are placed circumferentially repaired. The defect in the right ventricular outflow
through the aortic anulus, the Dacron patch, and tract is closed with a pericardial patch, covering the
the sewing ring of the prosthesis, and the valve is entire Dacron patch and draining any leakages into
seated and secured in place. Potential bleeding sites the right ventricle (. Fig. 16.18).
in the corner of the anulus-patch junction are dou-
ble sutured with individual interrupted sutures to 16.3.6.5 esection of the Conal Septum/
R
prevent hemorrhage from this site. The overlapping Fibromuscular Stenosis
Dacron patch is trimmed and used for enlargement After opening the aorta using an inverted hockey
and closure of the aortotomy incision. The aortic stick type of incision, the subvalvular area is
clamp is released, and any leakages in the patch are carefully inspected. The fibromuscular stenosis
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
561 16
a b
..Fig. 16.18 Surgical techniques for aortoventriculoplasty (AVP, Konno-Rastan). a Incision to open the aorta and
right ventricular outflow tract (dashed line). b Longitudinally opened aorta and transverse opening of the right ven-
tricle below the pulmonary valve, longitudinal incision into the interventricular septum after transection of the aortic
valve anulus, and resection of the dysplastic valve. c Sewn in trigonal patch plasty for the subaortic area and previously
implanted aortic valve prosthesis. d Completed left ventricular patch plasty and sewing technique of sewing in the
patch to enlarge the right ventricular outflow tract. e Completed AVP (LC left coronary artery, RC right coronary artery)
562 V. Hraška and J. Photiadis
or ridge of muscle projecting into the left ven- 16.3.7 Discussion Subvalvular
tricular outflow tract is excised as widely as pos- Obstruction
sible without interfering with the aortic valve
integrity and the conduction system. Two paral- On the basis of the underlying physiology and
lel incisions are made into the anterolateral morphology of a discrete subaortic membrane
aspect of the ventricular outflow tract. The first (Cape et al. 1997), any surgical treatment should,
incision is made beneath the intercoronary com- in addition to relieving the subaortic stenosis,
missural triangle, and the second one is made address the anatomic abnormality causing
beneath the belly of the right coronary cusp. The increased septal shear stress (Serraf et al. 1999a,
incisions are extended apical as far down as b). Discrete subvalvular aortic membrane can be
needed, and the chunk of muscle in between is cured in most patients by membranectomy
excised with scissors. accompanied by myectomy. Aggressive resection
of all structures causing flow turbulence (anoma-
16.3.6.6 Resection of Hypertrophic lous septal insertion of the mitral valve, accessory
Obstructive Cardiomyopathy/ mitral valve tissue, anomalous papillary muscle,
Extensive Myectomy anomalous muscular band, etc.) (Marasini et al.
in Hypertrophic Obstructive 2003) and removal of pathological tissue from the
Cardiomyopathy valve leaflets and from the subcommissural
The operation is conducted in a similar way to the trigones may prevent development or progres-
resection of fibromuscular stenosis, but the extent sion of aortic regurgitation and may eliminate the
of the septal myectomy is deeper. The septal substrate for recurrent obstruction. In patients
myectomy is guided by ECHO findings with par- without a myectomy, the left ventricle outflow
ticular emphasis on the thickness of the ventricu- tract gradient increased postoperatively at a
lar septum and the anatomy and function of the greater rate than those with a myectomy
mitral valve. Before bypass, direct measurement (Barkhordarian et al. 2007). Early mortality is
of the pressure gradient is performed to make a low, less than 5 % with the survival rate above
reliable comparison with post myectomy mea- 90 % at 15 years (Parry et al. 1999). Late mortality
surements. Particular attention is paid to myocar- is influenced by anatomic factors, namely, by
dial protection of the severely hypertrophied hypoplastic aortic anulus and concomitant mitral
myocardium. After opening the aorta and identi- stenosis (Lupinetti et al. 1992; Parry et al. 1999).
fication of the friction lesion caused by the oppo- Freedom from reoperation is about 85 % at
sition of the anterior leaflet of the mitral valve, 15 years. Recurrence of significant restenosis is
two parallel incisions are made in the septum associated with the immediate postoperative gra-
under the left, lateral half of the right coronary dient across the left ventricle outflow tract
sinus. These incisions are connected superiorly ≥30 mm, with the mean delay for reappearance of
under the aortic cusp, and a deep wedge of muscle a gradient leading to surgery approximately
16 is resected beyond the tip of the anterior leaflet of 4 years (Lupinetti et al. 1992; Rayburn et al. 1997).
the mitral valve to abolish the dynamic obstruc- The mild or moderate aortic insufficiency preop-
tion due to the systolic anterior motion of the eratively has a negative impact on the progression
anterior leaflet of the mitral valve. This classical of aortic insufficiency postoperatively. Therefore,
resection is extended leftward toward the mitral in the presence of aortic regurgitation, surgery is
valve and apically to the base of the papillary mus- recommended regardless of gradient to prevent
cle. In the presence of a midcavity obstruction, the development of aortic regurgitation or to
the apical portion of the myectomy is wider and improve aortic valve function (Parry et al. 1999;
includes the distal third of the right side of the Brauner et al. 1997). Higher incidence of com-
septum. All attachments between the papillary plete heart block reflects a too aggressive
muscles and septum are divided. After weaning approach during extended, circumferential
from bypass, direct measurement of the pressure myectomy.
gradient and TEE is performed to evaluate the Tunnel or multilevel subaortic stenosis repre-
effectiveness of the myectomy. A resting mean sents a more challenging cause of left ventricular
gradient of <20 mmHg is acceptable (Dearani outflow tract obstruction, occurring in isolation
et al. 2005). or in association with or secondary to correction
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
563 16
of complex congenital defects (double-outlet right prosthetic mismatches occurring with somatic
ventricle, transposition of the great arteries, inter- growth. Pannus formation is particularly likely to
ruption of the aortic arch, complete atrioventricu- cause problems in patients, who bear small pros-
lar canal, resection of subaortic membrane, etc. theses. Unfortunately, adequately sized valves
(Jonas et al. 1994; van Arsdell et al. 1995; Belli suited to patients’ body size do not eliminate the
et al. 1996). Ideally, relief of left ventricular risk of pannus ingrowth, due to children’s ability
obstruction should allow preservation of the to create pseudointima far more than adults.
aortic valve function, allow for growth of the aor- Careful attention should be given to maximizing
tic valve, and lessen the possibility of a subsequent the prosthesis effective orifice area according to
operation. The size and function of the aortic patient size without posing the additional risk of
valve determine the appropriate surgical tech- compromising the native pulmonary anulus,
nique. In isolated, simple forms, with a normal impinging on the pulmonary valve or the right
aortic valve, extended myectomy may be effective. coronary. Only an adult size prosthesis (21 mm
In complex forms with a normal aortic valve, or larger) should be inserted; otherwise patient-
the modified Konno procedure is effective for the prosthesis mismatch will inevitably develop,
relief of diffuse subvalvular aortic stenosis and resulting in aortic stenosis even in the presence
can safely be performed in infants and children. of a normally functioning valve. There is a non-
The risk of damaging the conduction tissue is trivial risk of pulmonary complications after the
minimal (Jahangiri et al. 2000). Children with Konno procedure related to an initial injury to
multilevel left ventricular outflow tract obstruc- the pulmonary valve, disruption of the muscular
tion consisting of subaortic narrowing and aortic subpulmonary conus, or distortion of the pulmo-
valve stenosis may require initial palliation to nary anulus by the right ventricular patch. The
relieve the critical level of obstruction. This may pulmonary valve is quite exposed to injury dur-
involve open/balloon aortic valvotomy, and/or ing the procedure because of the very limited
subaortic membrane and muscle resection. Often space between the right coronary ostia and the
this initial approach will achieve medium-term lateral commissure of the aortic valve. In an
clinical improvement despite some degree of effort to avoid injury to the coronary ostia, the
residual stenosis or insufficiency avoiding a more surgeon places his anular transecting incision as
extensive operation in the first years of life. If close to the commissure as possible, risking dam-
enlargement of the aortic root and aortic valve aging the pulmonary valve. Another problem
replacement is indicated to relieve the obstruc- seen with the Konno procedure is development
tion, the Konno or Ross-Konno operations should of complete heart block with an incidence of
be considered. about 10 % (De Vivie et al. 1993). The recovery of
The anterior aortoventriculoplasty described ventricular function after the Konno procedure
by Konno and Rastan (Konno et al. 1975; Rastan is analogous to that seen after aortic valve
and Kaney 1976) addresses all levels of left ven- replacement alone (Sharma et al. 2004). The
tricle outflow tract obstruction and is associated Konno procedure in children is an effective
with a significant decline in left ventricle outflow option for enlargement of the left ventricular
tract gradient, stabilization of left ventricular outflow tract in the face of multilevel obstruc-
function, and improvement in functional class. tion, but morbidity tends to be higher compared
Operative mortality for this procedure has with the Ross-Konno operation. If adult size
declined over the last two decades to less than prosthesis cannot be implanted, the Konno pro-
10 %, with survival at 10 years close to 90 % (Suri cedure should not be considered.
et al. 2006). The operation is designed to offer The Ross-Konno procedure is an excellent
definitive correction of complex left ventricle technique to treat complex multilevel left ventric-
outflow tract obstruction, but in children the ular outflow tract obstruction even in neonates
hazard phase for aortic reoperation is constant, and infants with significant anular and subanular
with the 10-year survival rate, free of reopera- hypoplasia (Hraska et al. 2010).
tion, approximately 60–80 % (Suri et al. 2006; Recently, the Ross-Konno procedure has
Erez et al. 2002). Apart from thrombosis and found application in patients with an interrupted
endocarditis, the reoperative risk is related either aortic arch as the initial procedure or after pri-
to prosthesis obstruction by pannus or to patient- mary repair in patients with a severely restricted
564 V. Hraška and J. Photiadis
subaortic area, hypoplastic aortic anulus, and accomplished with mortality less than 5 % and
bicuspid valve. The Ross-Konno procedure low morbidity (Hraska et al. 2010).
increases our therapeutic choices for neonates or Early complications are especially related to
infants with critical aortic stenosis who show malignant arrhythmias (nonsustained ventricu-
unacceptable results following open valvotomy or lar tachycardia, nonsustained supraventricular
balloon valvotomy. Some newborns with critical tachycardia, etc.). The incidence of complete
aortic stenosis, a hypoplastic aortic anulus, and heart block varies from 0 to 6 % (Marino et al.
significant endocardial fibroelastosis can be effec- 1999). The freedom from reoperation is between
tively treated with the Ross-Konno procedure and 60 and 70 % at 5 years, being less favorable for
resection of endocardial fibroelastosis, thus avoid- small children due to earlier homograft failure.
ing single-ventricular palliation (Aszyk et al. The choice of conduit appeared to impact the
2012). need for replacement. The aortic homograft
The Ross-Konno procedure allows easy access rather than pulmonary homograft and smaller
to the left ventricular myocardium for resection of homograft size are factors adversely affecting
endocardial fibroelastosis, normalizing the left homograft longevity. The policy is to place the
ventricular outflow tract and the long axis of the largest pulmonary homograft possible at the time
left ventricle. of procedure.
The type of enlargement of the left ventricular There are disadvantages of the Ross-Konno
outflow tract depends on the presence of a sig- procedure as well. The nature of the operation
nificant ventricular septal defect or on the magni- places two valves at risk for single-valve disease.
tude of discrepancy of the aortic valve and However, many pediatric patients are not candi-
pulmonary artery size. If a significant ventricular dates for a Konno operation because of anatomic
septal defect is present, the incision of the conal considerations and the lack of readily available,
septum is extended toward the ventricular septal appropriately sized prostheses. Particularly in the
defect, and patch closure of the ventricular septal growing pediatric patient, the need for pulmo-
defect provides outflow tract enlargement prior nary conduit replacement is high. Replacement
to the autograft implantation. If there is primary of a pulmonary conduit is, however, less difficult
muscular obstruction, the ventriculoplasty inci- than repeated aortic root replacement. Other
sion is patched by an extension of attached infun- concerns regarding the use of the Ross-Konno
dibular free wall muscle harvested with the procedure in children include the uncertain
autograft. Occasionally, in very small babies, the long-term outcome of the pulmonary autograft
thickness of the right ventricular muscle can in the aortic position. The pulmonary autograft
restrict inflow through the tricuspid valve or demonstrates durability without developing aor-
cause recurrent outflow obstruction beneath the tic stenosis, aortic insufficiency, or progressive
autograft. In these patients, patch enlargement of dilatation. Enlargement of the aortic anulus par-
the left ventricular outflow tract is preferable. If allels somatic growth. However, it is unclear
16 there is a significant mismatch between the pul- whether use of the Ross-Konno procedure at an
monary anulus and a truly hypoplastic aortic earlier age alters the natural history of complex
anulus (≤4 mm in Ø), excision and reimplanta- left ventricular outflow tract disease. It is also not
tion of the coronary ostia can be more difficult known if the risk of late reoperation on the neo-
since extensive enlargement of the aortic anulus is aortic valve is higher, if performed in the neo-
necessary and the coronary arteries are close nate, infant, or child because of aortic root sinus
together. Often there is associated severe arch dilation and valve distortion over time. Based on
hypoplasia, coarctation, and a ventricular septal experience from arterial switch operations for
defect. In these patients, a modified Norwood transposition of the great arteries, the neonatal
type of repair with Rastelli modification and baf- pulmonary valve might rapidly be able to adapt
fling of the ventricular septal defect to the pulmo- to the aortic position. Long-term changes in left
nary valve is simpler, although ventricular septal ventricular mechanics await further study.
defect enlargement should be performed in all Despite the technically demanding nature of the
cases to prevent development of a restrictive operation, the Ross-Konno procedure is the
pathway to the pulmonary artery (Serraf et al. method of choice for the multilevel type of left
1999a, b). The Ross-Konno procedure can be ventricle outflow tract obstruction, especially in
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
565 16
newborns and infants (Marino et al. 1999; Ohye cal approaches that include a more extended resec-
et al. 2001; Hraska et al. 2004, 2010; Brown et al. tion of the midventricular septum, relief of papillary
2006; Aszyk et al. 2012). muscle anomalies, and routine use of intraoperative
Isolated left ventricular septal myectomy pro- transesophageal echocardiography.
vides excellent outcomes for symptomatic patients
with severe obstructive hypertrophic cardiomyopa-
thy. After surgery, significant and persistent 16.4 Supravalvular Aortic Stenosis
improvement in symptoms and exercise capacity
can be expected. The operation is performed with 16.4.1 Introduction
low operative mortality (reported to be 1–3 % and
even less in the most recent cases) in patients of all Supravalvular aortic stenosis is a complex anomaly
ages, including children, at those centers which of the entire aortic root with predominant stenosis
have the most experience with this procedure. at the sinotubular junction. This represents the least
Surgical risk may be higher in patients with a prior common form of left ventricular outflow tract
myectomy, or those undergoing additional cardiac obstruction and occurs in about 5–8 % of patients
surgical procedures. Complications such as com- (Samanek et al. 1989). The underlying cause of con-
plete heart block (requiring a permanent pace- genital supravalvular aortic stenosis is a loss-of-
maker) and iatrogenic ventricular septal perforation function mutation of the elastin gene on
have become uncommon (equal to or less than chromosome 7q11.23 (Keating 1995). In patients
1–2 %), while partial or complete left bundle-branch with Williams syndrome, the elastin gene is deleted
block is an inevitable consequence of the muscular or disrupted together with a number of neighbor-
resection and is not associated with adverse ing genes that probably are important for the other
sequelae. Intraoperative guidance with echocar- features of the syndrome (elfin-face, mild mental
diography (transesophageal or with the transducer retardation, hypercalcemia) (Nickerson et al. 1995),
applied directly to the right ventricular surface) is whereas in patients with familial, non-Williams
standard and is useful in assessing the site and supravalvular aortic stenosis, the elastin gene only is
extent of the proposed myectomy, the structural subjected to a loss-of-function translocation or
features of the mitral valve, and the effect of muscu- point mutation (Chowdhury and Reardon 1999).
lar resection on systolic anterior movement of Patients with «sporadic» supravalvular aortic steno-
mitral valve and mitral regurgitation (Maron et al. sis are either members of a family carrying an elas-
2003). The incidence of recurrent left ventricular tin gene mutation with a subclinical phenotype, or
outflow tract gradient is less that 5 %. Recurrent carrying the elastin gene defect as a new mutation
obstruction is associated with limited myectomy at (Keating 1995). The resulting decrease in elastin
the initial operation, midventricular obstruction, expression during development is associated with
anomalies of papillary muscles, and ventricular an increased number of elastin lamellae and exces-
remodeling. The recurrence rate of left ventricle sive accumulation of collagen and smooth muscle
outflow tract obstruction after successful myectomy cells in the vascular wall, resulting in formation of
in pediatric patients is higher (Theodoro et al. 1996). severely thickened and rigid arterial vessels (Li et al.
Technically, the extended septal resection might be 1998; O’Connor et al. 1985). Elastin arteriopathy is
compromised by the small size of the aorta and lim- not a localized process but rather a generalized dis-
ited visibility of the midventricular region. Left ven- ease of both the systemic and pulmonary arterial
tricular remodeling, such as changes in wall systems (Stamm et al. 1997, 2001).
thickness and reduction of cavity size, might be
another explanation for the increased need for
reoperation in pediatric patients (Maron and Spirito 16.4.2 Anatomy
1998). A repeat myectomy can be performed with
excellent outcomes. Mitral valve replacement (in Supravalvular aortic stenosis is categorized
the absence of intrinsic mitral valve disease), even in broadly as either diffused (23 %) or localized
the presence of a relatively thin ventricular septum, (77 %). In the localized type, there is a severe nar-
is not recommended. Carefully performed surgical rowing of the sinotubular ridge. The outer
septal reduction is the preferred method. The need diameter of the aorta may be normal or reduced,
for reoperation may be reduced with current surgi- giving an hourglass appearance to the ascending
566 V. Hraška and J. Photiadis
aorta. The coronary arteries may be either dilated While echocardiography is diagnostic in many
due to the high systolic pressure proximal to the cases, it may be inadequate as the only diagnostic
sinotubular junction or stenotic because of the tool in more complex forms of supravalvular aor-
thickening of the wall of the sinuses of Valsalva, or tic stenosis with involvement of the aorta, the aor-
they develop atherosclerosis. On occasion, the free tic arch, and head vessels. In order to clarify the
edge of the aortic valve cusps almost completely anatomy in any complex form of supravalvular
adheres to the sinotubular junction, effectively aortic stenosis and to delineate any existing
isolating the coronary artery from the sinus of branch pulmonary artery stenosis, cardiac cathe-
Valsalva and the lumen of the aorta. Abnormalities terization and angiography are indicated. If there
of the aortic valve are present nearly in half of the is evidence for an obstruction of the coronary
cases. Thickening of the aortic cusps and aortic ostia, coronary angiography should be performed
insufficiency due to the high systolic pressure and with great care. Magnetic resonance imaging may
bicuspid aortic valve is most commonly presented possibly also represent an attractive alternative to
(Stamm et al. 1997; van Son et al. 1994b). In the invasive angiography.
diffuse form, the entire ascending aorta and the
brachiocephalic vessels may be thickened, with
narrowed lumen. There may by supravalvular nar- 16.4.5 Indications for Surgery
rowing of the main pulmonary artery and central
and peripheral pulmonary artery stenosis. An unfavorable natural history due to accelerated
coronary artery atherosclerosis, damage of the
aortic valve, and the high risk of sudden death is a
16.4.3 Pathophysiology reasonable argument for proceeding with early
surgery, before the left ventricular hypertrophy
The basic pathophysiology of supravalvular aortic has become severe.
stenosis is similar to other types of congenital aor- A peak Doppler gradient above 40 mmHg,
tic stenosis. Left ventricular hypertrophy and aortic insufficiency, and signs of compromised
ischemic myocardial damage due to coronary coronary artery blood flow are indications for
ostial stenoses, decreased blood flow into the cor- surgery (. Fig. 16.19).
onary sinuses, or hypertension-related coronary
arteriosclerosis may present in early childhood
and accelerate with age (van Son et al. 1994b). The 16.4.6 Surgical Management:
risk of sudden death secondary to myocardial Symmetric Restoration
hypoperfusion and subsequent fibrillation is of Distorted Aortic Root
higher in comparison with other forms of left ven-
tricular outflow tract obstruction (van Son et al. A single, asymmetric patch technique
1994a). In case of pulmonary artery stenosis, right (. Fig. 16.20a), enlarging only the noncoronary
16 ventricular hypertension and hypertrophy sinus in conjunction with resection of the stenotic
develop. ridge, does not address the constriction of the
thickened aortic wall at the level of the cusp hinge
point. This original method is rarely used any
16.4.4 Clinical Presentation more. Currently, the preferred method is the
and Diagnosis symmetric anatomic restoration of the aortic root
addressing all involved sinuses of Valsalva. The
The disease occurs as an isolated defect or as a risk of developing postoperative aortic regurgita-
part of William’s syndrome in association with tion is minimal despite the significant enlarge-
mental retardation, elfin facies, failure to thrive, ment of the diameter of the sinotubular junction.
and occasionally infantile hypercalcemia All techniques require a cardiopulmonary bypass
(Williams et al. 1961). The clinical appearance with cardioplegic arrest of the heart. Deep
corresponds to other forms of left ventricular out- hypothermia with circulatory arrest and, alterna-
flow tract obstruction. Limited capacity, syncope, tively, hypothermic low-flow bypass with direct
and angina symptoms may increase during the perfusion of the brachiocephalic vessels are used
course and may be associated with detectable for diffuse forms where reconstruction of the aor-
myocardial ischemia or heart failure. tic arch or origins of the head vessels is required.
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
567 16
..Fig. 16.19 Treatment
protocol for supravalvular
aortic stenosis at the Ger- Symptoms
man Pediatric Heart Center,
Sankt Augustin. AI aortic
valve insufficiency
Gradient > 40 mm Hg
Surgery
AI ≥ mild
Symmetric three-patch
Coronary ischemia or non-patch
technique
..Fig. 16.20 Surgical
techniques to correct a
supravalvular aortic ste-
nosis. a Enlargement of
b
the noncoronary sinus by
a diamond-shaped patch.
b Insertion of an inverted
bifurcated patch with
enlargement of the right
sinus and noncoronary
sinus (Doty plastic). c Tran-
section of the ascending
aorta and extending all
the sinuses using patch RCA
and reanastomosis of the LCA
aorta (Brom technique). d
Transection of the ascend-
ing aorta, incision of all c d
sinus and the commissures
of the aortic valve comple-
mentary ascending aorta,
and direct reanastomosis.
e Incisions performed into
the aortic root relative
to coronary arteries for
both Brom and tri-sinus
Doty techniques. LCA left
coronary artery, RCA right
coronary artery
RCA
LCA
568 V. Hraška and J. Photiadis
16.4.6.1 Inverted Bifurcated Patch taken to stay away from the coronary ostia; thus
An inverted Y-shaped vertical incision is made into the incisions are made off-center of the sinuses
the aorta (. Fig. 16.20b), extending to the noncor- (. Fig. 16.20e). Three complementary vertical
onary and right coronary sinus and crossing the incisions are made in the distal aorta, out of phase
thickened sinotubular ridge. It is important to stay with the proximal incisions. This allows interdigi-
away from the origin of the right coronary artery to tation of the proximal and distal flaps when the
prevent any problems with the suturing of the aorta is reanastomosed. A zigzag anastomosis
patch in close proximity to the coronary ostium. enlarges the aortic root without the use of pros-
An inverted bifurcated patch made of autologous thetic material (Myers et al. 1993; Hraska and
or bovine pericardium is trimmed. The goal is to Murin 2012).
create bulging new sinuses of Valsalva. The tips of
the patch are placed into the apex of each incision, 16.4.6.4 Diffuse Forms
near the hinge point of the aortic valve. The patch In the diffuse form of supravalvular aortic steno-
is sutured into the anterior sinuses of Valsalva and sis, the aortic enlargement should be extended
the remaining ascending aorta (Doty et al. 1977). into the ascending aorta or beyond as required, to
Doty later developed the bifurcated three sinus relieve the gradient. The pericardial patch is
technique by incising all three sinuses and using inserted along the entire ascending aorta and the
one part of the patch bifurcation as enlargement undersurface of the aortic arch distal to the left of
of the noncoronary sinus as before, while the the subclavian artery whenever a significant
other patch «leg» not only enlarges the right coro- degree of hypoplasia is suspected. In many cases
nary sinus to the left of the right coronary ostium of diffuse supravalvular aortic stenosis, the ori-
but enlarges also the left coronary sinus to the left gins of the supra-aortic vessels are also stenotic,
of the left coronary ostium. This is achieved by exposing the patients to the risk of cerebral isch-
suturing the right/left coronary commissure to the emia. Patch enlargement of the proximal branch
inner side of the left patch «leg,» similar as sug- arteries should be performed in association with
gested later for all commissures in the David tech- augmentation of the aortic arch (Pretre et al.
nique for aortic valve reconstruction (Doty 1985). 1999). In some patients, complex tube graft con-
structions bypassing the aortic arch (ascending-
16.4.6.2 Symmetric Three-Patch descending aortic conduit), with additional grafts
Technique supplying the supra-aortic branches, could be
In the case of extensive narrowing of the left coro- effective (see 7 Chap. 19).
nary sinus, a three-patch technique is preferable
(. Fig. 16.20c). The aorta is transected immediately
above the level of the commissures. Vertical inci- 16.4.7 Discussion: Supravalvular
sions are made into each of the three sinuses, staying Aortic Stenosis
away from the origin of the coronaries. Three tear-
16 drop-shaped patches from the pericardium are Congenital supravalvular aortic stenosis is not a
sutured into each of the sinuses of Valsalva. The simple lesion. The underlying elastin arteriopathy
ascending aorta is opened longitudinally on its ante- is a generalized disease of both the pulmonary and
rior surface. An anterior patch is used to supplement systemic arteries. Furthermore, the «supravalvu-
this aortotomy, enlarging the reanastomosis of the lar» stenosis has profound effects on the architec-
ascending aorta (Brom 1988). It is important that ture and function of the aortic valve itself. In
the patches are not too broad, because this may addition to left ventricular pressure overload, there
result in commissural splaying with valvular insuffi- is always a risk of myocardial ischemia due to cor-
ciency (Hraska and Murin 2012). onary hypertension and premature arteriosclerosis,
or obstruction to coronary blood flow as part of
16.4.6.3 Symmetric Reconstruction the aortic root malformation. Indication for oper-
Technique Without a Patch ation and surgical treatment strategies must take
The aorta is transected at the narrowest point all these features into account, so that an optimal
above the commissures (. Fig. 16.20d). Any thick- long-term outcome can be achieved (Stamm et al.
ened area on the distal aorta is excised. Three ver- 1997, 2001). Optimal surgical management should
tical incisions are made into each sinus of Valsalva, strive for preservation and restoration of the aortic
similar to those in a three-patch technique. Care is valve and root, rather than simply for relief of the
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
569 16
supravalvular stenosis. The more symmetric treatment of pulmonary artery obstructions, with a
reconstructions of the aortic root with three-sinus pericardial patch placed throughout both pulmo-
reconstructions result in superior hemodynamics, nary arteries from the right to the left hilum, offers
a more physiological flow pattern, and they are a good long-term outcome. In patients with gener-
associated with a lower mortality rate and fewer alized obstructive arteriopathy with systemic and
reoperations than only augmentation of the non- pulmonary artery stenoses and suprasystemic right
coronary sinus. To avoid aortic regurgitation due ventricular pressure, preoperative dilation of
to oversizing of the reconstructed sinuses, the peripheral and central pulmonary artery stenoses
appropriate patch size can be calculated based on should be considered to decrease the right ventricle
the age-normalized circumference of the aortic pressure load prior to the ischemic insult of surgical
root, but in most cases, it is sufficient to use the repair of supravalvular aortic stenosis (Stamm et al.
circumference of the distal aortic end as a guide- 2000). The severe thickening of the arterial wall in
line (Stamm et al. 1999, 2001; Hazekamp et al. infradiaphragmatic arteries and localized stenoses
1999). Management of abnormalities of the aortic of mesenteric and renal arteries have been reported
valve, the subaortic region, and the coronary arter- in up to 30 % of the patients with supravalvular aor-
ies is critical to the long-term outcome of these tic stenosis (Zalzstein et al. 1991; Rein et al. 1993).
patients (McElhinney et al. 2000). Involvement of It is important to diagnose concomitant stenoses
the aortic valve, including stenosis or an abnormal prior to surgery for supravalvular aortic stenosis, so
number of cusps, is presented in one-third of that the consequences of peri- and postoperative
patients with supravalvular aortic stenosis. hypoperfusion of the abdominal organs can be
Degenerative changes in the valve are common, minimized (Stamm et al. 2001). Severe generalized
most probably due to abnormal stresses on and arterial forms and associated aortic valve disease
restriction of the cusps in the setting of impaired are correlated with late death and the need for reop-
distensibility of the sinotubular junction eration. The long-term survival rate is between 70
(Braunstein et al. 1990). Reconstruction of the and 97 % and freedom from reoperation is above
valve is the preferable method of treatment. The 65 % at 20 years, respectively. Reoperations are
Ross procedure is the method of choice, if aortic more due to aortic valve problems than progressive
valve replacement is indicated. supra-aortic stenosis. The patient’s quality of life is
Stenosis or atresia of a coronary ostium and generally good, with the majority of patients in the
proximal coronary artery may occur, necessitat- New York Heart Association functional class I (van
ing either ostial endarterectomy, patch enlarge- Son et al. 1994a; Stamm et al. 1999; Hazekamp et al.
ment of coronary ostial stenoses, or coronary 1999; Brown et al. 2002).
bypass grafting (Martin et al. 1988). Early surgi-
cal intervention is indicated to prevent acceler-
ated coronary artery disease, its detrimental
ischemic effects on the myocardium, and dissec- 16.5 Sinus Valsalva Aneurysm
tion of the ascending aorta or coronary arteries
(van Son et al. 1994b). Any obstruction to coro- 16.5.1 Introduction and Anatomy
nary blood flow should be identified preopera-
tively, and patency of both coronary arteries The sinus of Valsalva is defined as that portion of
should be confirmed by intraoperative probing in the aortic root between the aortic valve anulus
every case. and the sinotubular ridge. The sinus wall of the
In diffuse supravalvular aortic stenosis, aortic aorta is thinner than the distal aorta and is there-
enlargement and extensive endarterectomy should fore subject to various anomalies, the most com-
be extended into the ascending aorta or beyond as mon being aneurysm of sinus Valsalva.
required, to relieve the gradient. The long-term The term «sinus of Valsalva aneurysm» should
survival of patients with diffuse supravalvular aor- not be mistakenly used for what is called an aortic
tic stenosis is similar to that of patients with the dis- root aneurysm, for example, in Marfan syndrome,
crete form, although the operative risk is increased or as part of any other acquired multianeurysmal
(Stamm et al. 1999). A number of patients with aortic disease (see 7 Chapters «Acquired Lesions of
William’s syndrome or other forms of elastin arteri- the Aortic Valve», Sect. 24.3.1 and «Aneurysm and
opathy have stenoses of the pulmonary arteries in Dissection of the Thoracic and Thoracoabdominal
addition to supravalvular aortic stenosis. Surgical Aorta», Sect. 27.2.2).
570 V. Hraška and J. Photiadis
16
16.5.3 Indications for Surgery
aortic insufficiency, the valve-sparing techniques ischemia. ECHO and Doppler flow studies are
are preferable. David operation may provide bet- highly reliable in estimating morphology, flow
ter support for the anulus and remaining aortic in the tunnel, and functional and morphomet-
root tissue than Yacoub operation. Otherwise ric parameters of the left ventricle. The presence
complete replacement of the aortic root with of paravalvular regurgitation renders it difficult
composite graft may be the best option. to evaluate the status of native aortic valve by
ECHO. Catheterization is useful in identifying
the coronary ostia.
16.6 Aortico-Left Ventricular Tunnel
Brabham KR, Roberts WC (1990) Fatal intrapericardial rup- German-Dutch Ross Registry (2009) Autograft rein-
ture of sinus of Valsalva aneurysm. Am Heart forcement to preserve autograft function after the
J 120:1455–1456 ross procedure: a report from the german-dutch ross
Brauner R, Laks H, Drinkwater DCJ, Shvarts O, Eghbali K, registry. Circulation 120(11 Suppl):S146–S154
Galindo A (1997) Benefits of early surgical repair in fixed Chartrand CC, Saro-Servando E, Vobecky JS (1999) Long-
subaortic stenosis. J Am Coll Cardiol 30:1835–1842 term results of surgical valvuloplasty for congenital val-
Braunstein PW, Sade RM, Crawford FA, Oslizlok PC (1990) var aortic stenosis in children. Ann Thorac Surg
Repair of supravalvular aortic stenosis. Ann Thorac 68:1356–1359
Surg 50:700–707 Chowdhury T, Reardon W (1999) Elastin mutation and car-
Brewer RJ, Deck D, Capati B, Nolan SP (1976) The dynamic diac disease. Pediatr Cardiol 20:103–107
aortic root. Its role in aortic valve function. J Thorac Chu SH, Hung CR, How SS et al. (1990) Ruptured aneu-
Cardiovasc Surg 72:413–417 rysms of sinus Valsalva in Oriental patients. J Thorac
Brom AG (1988) Obstruction of the left ventricular outflow Cardiovasc Surg 99:288
tract. In: Khonsari S (ed) Cardiac surgery: safeguards Clarke DR (1991) Extended aortic root replacement with
and pitfalls in operative technique. Aspen, Rockville, cryopreserved allografts: do they hold up? Ann Thorac
pp 276–280 Surg 52:669–675
Brown JW, Ruzmetov M, Vijay P, Turrentine MW (2002) Coady MA, Rizzo JA, Hammond GL et al. (1997) What is the
Surgical repair of congenital supravalvular aortic ste- appropriate size criterion for resection of thoracic aor-
nosis in children. Eur J Cardiothorac Surg 21:50–56 tic aneurysms? J Thorac Cardiovasc Surg 113:476–491
Brown JW, Ruzmetov M, Vijay P, Rodefeld MD, Turrentine Colan SD, McElhinney DB, Crawford EC, Keane JF, Lock JE (2006)
MW (2006) The Ross-Konno procedure in children: Validation and re-evaluation of a discriminant model pre-
outcomes, autograft and allograft function, and reop- dicting anatomic suitability for biventricular repair in neo-
erations. Ann Thorac Surg 82:1301–1307 nates with aortic stenosis. J Am Coll Cardiol 47:1858–1865
Brown DW, Chong EC, Gauvreau K, Keane JF, Lock JE, Corno AF (2005) Borderline left ventricle. Eur J Cardiothorac
Marshall AC (2008) Aortic wall injury as a complication Surg 27:67–73
of neonatal aortic valvuloplasty: incidence and risk Cosgrow DM, Rosenkranz ER, Hender WG et al. (1991)
factors. Circ Cardiovasc Interv 1:53–59 Valvuloplasty for aortic insufficiency. J Thorac
Brown JW, Ruzmetov M, Shahriari A, Rodefeld MD, Cardiovasc Surg 102:571–576
Mahomed Y, Turrentine MW (2009) Midterm results of David TE, Omran A, Webb G, Rakowski H, Armstrong S, Sun
Ross aortic valve replacement: a single-institution Z (1996) Geometric mismatch of the aortic and pul-
experience. Ann Thorac Surg 88:601–607 monary roots causes aortic insufficiency after the
Brown DW, Dipilato AE, Chong EC, Lock JE, McElhinney DB Ross procedure. J Thorac Cardiovasc Surg 112:1231–
(2010a) Aortic valve reinterventions after balloon 1239
aortic valvuloplasty for congenital aortic stenosis:
David TE, Omran A, Ivanov J, Armstrong S, de Sa MPL,
intermediate and late follow-up. J Am Coll Cardiol Sonnenberg B, Webb G (2000) Dilatation of the pulmo-
56:1740–1749 nary autograft after the Ross procedure. J Thorac
Brown JW, Ruzmetov M, Shahriari AP, Rodefeld MD, Cardiovasc Surg 119:210–220
Mahomed Y, Turrentine MW (2010b) Modification of the David TE, Armstrong S, Ivanov J, Feindel CM, Omran A,
Ross aortic valve replacement to prevent late autograft Webb G (2001) Results of aortic valve-sparing opera-
dilatation. Eur J Cardiothorac Surg 37:1002–1007 (2) tions. J Thorac Cardiovasc Surg 122:39–46
Cameron DE, Vricella LA (2005) Valve-sparing aortic root Davies RR, Gallo A, Coady MA, Tellides G, Botta DM, Burke B,
replacement in Marfan syndrome. Semin Thorac Coe MP, Kopf GS, Elefteriades JA (2006) Novel measure-
Cardiovasc Surg Pediatr Card Surg Annu 8:103–111 ment of relative aortic size predicts rupture of thoracic
Cape EG, Vanauker MD, Sigfusson G, Tracy TA, del Nido PJ aortic aneurysm. Thorac Surg 81:169–177
16 (1997) Potential role of mechanical stress in etiology de Kerchove L, Glineur D, Poncelet A, Boodhwani M, Rubay
of pediatric heart disease: septal shear stress in sub- J, Dhoore W, Noirhomme P, El Khoury G (2008) Repair
aortic stenosis. J Am Coll Cardiol 30:247–254 of aortic leaflet prolapse: a ten-year experience. Eur
Carr-White GS, Afoke A, Birks EJ, Yacoub MH (2000) Aortic J Cardiothorac Surg 34:785–791
root characteristic of human pulmonary autografts. De Sa M, Moshkovitz Y, Butany J, David TE (1999) Histologic
Circulation 102(Suppl III):III 15–III 21 abnormalities of the ascending aorta and pulmonary
Caspi J, Ilbawi MN, Roberson DA, Piccione W, Monson DO, trunk in patients with bicuspid aortic valve disease:
Wajafi H (1994) Extended aortic valvuloplasty for clinical relevance to the Ross procedure. J Thorac
recurrent valvular stenosis and regurgitation in chil- Cardiovasc Surg 118:588–596
dren. J Thorac Cardiovasc Surg 107:1114–1120 De Vivie ER, Borowski A, Mehlhorn U (1993) Reduction of
Champsaur G, Robin J, Tronc F, Curtil A, Ninet J, Sassolas F, the left-ventricular outflow-tract obstruction by aorto-
Vedrinne C, Bozio A (1997) Mechanical valve in aortic ventriculoplasty—long-term results of 96 patients.
position is a valid option in children and adolescents. Thorac Cardiovasc Surg 41:216–223
Eur J Cardiothorac Surg 11:117–122 Dearani JA, Danielson GK (2005) Septal myectomy for
Charitos EI, Hanke T, Stierle U, Robinson DR, Bogers AJ, obstructive hypertrophic cardiomyopathy. Semin
Hemmer W, Bechtel M, Misfeld M, Gorski A, Boehm JO, Thorac Cardiovasc Surg Pediatr Card Surg Annu
Rein JG, Botha CA, Lange R, Hoerer J, Moritz A, Wahlers 2005:86–91
T, Franke UF, Breuer M, Ferrari-Kuehne K, Hetzer R, Dervanian P, Mace L, Folliguet TA, Di Virgilio A (1998)
Huebler M, Ziemer G, Takkenberg JJ, Sievers HH, Surgical treatment of aortic root aneurysm related to
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
575 16
Marfan syndrome in early childhood. Pediatr Cardiol Grande-Allen KJ, Cochran RP, Reinhall PG, Kunzelman KS
19:369–373 (2000) Re-creation of sinuses is important for sparing
Detter C, Fischlein T, Feldmeier C, Nollert G, Reichart B (2001) the aortic valve: a finite element study. J Thorac
Aortic valvotomy for congenital valvular aortic stenosis: Cardiovasc Surg 119:753–763
a 37-year experience. Ann Thorac Surg 71:1564–1571 Han RK, Gurofsky RC, Lee KJ et al. (2007) Outcome and
Doty DB (1985) Supravalvular aortic stenosis. Extended growth potential of left heart structures after neonatal
aortoplasty. In: Doty DB (ed) Cardiac surgery. Loose- intervention for aortic valve stenosis. JACC 50:25
leaf for updating. Year Book Medical Publishers, Hanley FL. Discussion of: Loland GK, McCrindle BW, Williams
Chicago WG, Blackstone EH, Tchervenkov CI, Sittiwankul R,
Doty DB (1987) Replacement of the aortic valve with cryo- Jonas RA (2001) Critical aortic stenosis in the neonate: a
preserved aortic valve allograft: considerations and multi-institutional study of management, outcomes,
techniques in children. J Card Surg 2:129–136 and risk factors. J Thorac Cardiovasc Surg 121:25–26
Doty DB, Polansky DB, Jenson CB (1977) Supravalvular aor- Harkness JR, Fitton TP, Barreiro CJ et al. (2005) A 32-year
tic stenosis: repair by extended aortoplasty. J Thorac experience with surgical repair of sinus of Valsalva
Cardiovasc Surg 74:362–731 aneurysms. J Card Surg 20:198–204
Duran CMG (1993) Pericardium in valve operations. Ann Hawkins JA, Minich LL, Shaddy RE, Tani LY, Orsmond GS,
Thorac Surg 56:1–2 Sturtevant JE, McGough EC (1996) Aortic valve repair
Duran CMG, Gometza B, Shaid M, Al Halees Z (1998) and replacement after balloon valvuloplasty in chil-
Treated bovine and autologous pericardium for aortic dren. Ann Thorac Surg 61:1355–1358
valve reconstruction. Ann Thorac Surg 66:S166–S169 Hazekamp MG, Kappetein AP, Schoof PH et al. (1999) Brom’s
Edwards JE, Burchell HB (1957) The pathological anat- three-patch technique for repair of supravalvular aor-
omy of deficiencies between the aortic root and the tic stenosis. J Thorac Cardiovasc Surg 118:252–258
heart, including aortic sinus aneurysms. Thorax Hickey EJ, Caldarone CA, Blackstone EH et al. (2007) Critical
12:125–139 left ventricular outflow tract obstruction: the dispro-
Eicken A, Georgiev S, Balling G, Schreiber C, Hager A, Hess portionate impact of biventricular repair in borderline
J (2010) Neonatal balloon aortic valvuloplasty-predictive cases. J Thorac Cardiovasc Surg 134:1429–1437
value of current risk score algorithms for treatment strat- Ho SY, Anderson RH (1988) The morphology of the aortic
egies. Catheter Cardiovasc Interv 76:404–410 valve with regard to congenital malformations. In:
Elkins RC, Knott-Craig CJ, Ward KE, McCue C, Lane MM Dunn JM (ed) Cardiac valve disease in children. Elsevier
(1994) Pulmonary autograft in children: realized Science Publishing Co., New York, pp 3–21, Annals 2000
growth potential. Ann Thorac Surg 57:1387–1393 supplement
Elkins RC, Lane MM, McCue C (1996) Pulmonary autograft Hörer J, Hanke T, Stierle U, Takkenberg JJ, Bogers AJ,
reoperation: incidence and management. Ann Thorac Hemmer W, Rein JG, Hetzer R, Hübler M, Robinson DR,
Surg 62:450–455 Sievers HH, Lange R et al. (2009) Neoaortic root diam-
Elkins RC, Knott-Craig CJ, Ward KE, Lane MM (1998) The eters and aortic regurgitation in children after the
Ross operation in children: 10-years experience. Ann Ross operation. Ann Thorac Surg 88:594–600
Thorac Surg 65:496–502 Horváth P, Balaji S, Skovránek S, Hucin B, de Leval MR, Stark
Elkins RC, Lane NM, McCue C (2001) Ross operation in chil- J et al. (1991) Surgical treatment of aortico-left ven-
dren: late results. J Heart Valve Dis 10:736–741 tricular tunnel. Eur J Cardiotharac Surg 5:113
Erez E, Kanter KR, Tam VKH, Williams W (2002) Konno aorto- Hossack KF, Neutze JM, Lowe JB et al. (1980) Congenital
ventriculoplasty in children and adolescents from valvar aortic stenosis. Natural history and assessment
prosthetic valves to the Ross operation. Ann Thorac for operation. Br Heart J 43:561–573
Surg 74:122–126 Hovaquimian H, Cobanoglu A, Starr A et al. (1988) Aortico-
Ewert P, Bertram H, Breuer J et al. (2011) Balloon valvulo- left ventricular tunnel: a clinical review and new surgi-
plasty in the treatment of congenital aortic valve ste- cal classification. Ann Thorac Surg 45:109
nosis—a retrospective multicenter survey of more Hraska V, Murin P (2012) Surgical management of congeni-
than 1000 patients. Int J Cardiol 149:182–185 tal heart disease I: complex transposition of great
Ferreira AC, de Marchena E, Mayor M et al. (1996) Sinus of arteries right and left ventricular outflow tract obstruc-
Valsalva aneurysm presenting as myocardial infarction tion, Ebstein’s anomaly. A video manual. Springer,
during dobutamine stress test. Cathet Cardiovasc Heidelberg
Diagn 39:400–402 Hraska V, Krajci M, Haun C et al. (2004) Ross and Ross-
Frater RW (1967) The prolapsing aortic cusp: experimental Konno procedure in children and adolescents: mid-
and clinical observations. Ann Thorac Surg 3:63–67 term results. Eur J Cardiothorac Surg 25:742–747
Gersony WM (2001) Natural history of discrete subvalvular Hraska V, Photiadis J, Arenz C. Open valvotomy for aortic
aortic stenosis: management implications. J Am Coll valve stenosis in newborns and infants. (2007a) Multi-
Cardiol 38:843–845 media Man Cardiothorac Surg. Available at: 7 http://
Gewillig M, Daenen W, Dumoulin M et al. (1992) Rheologic mmcts.ctsnetjournals.org/cgi/collection/left_heart_
genesis of discrete subvalvular aortic stenosis: a lesions. Accessed 19 June 2007
Doppler echocardiographic study. J Am Coll Cardiol Hraska V, Photiadis J, Arenz C (2007b) Surgery for subvalvular
19:818–824 aortic stenosis—resection of discrete subvalvular aor-
Goodwin JF, Hollman A, Cleland WP et al. (1960) Obstructive tic membrane MMCTS. 7 http://mmcts.ctsnetjournals.
cardiomyopathy simulating aortic stenosis. Br Heart o rg / c gi / co nte nt / f u l l / 2 0 0 7 / 0 7 2 3 / m m c t s. 2 0 0 6 .
J 22:403–414 002303
576 V. Hraška and J. Photiadis
Hraska V, Photiadis J, Murin P, Poruban R, Asfour B (2008) in fixed subaortic stenosis. J Am Coll Cardiol 22:
Ross-Konno operation. MMCTS. 7 http://mmcts.ctsnet 1501–1508
journals.org/cgi/content/full/2008/0915/mmcts. Konno S, Imai J, Iida Y et al. (1975) A new method for pros-
2008.003160 thetic valve replacement in congenital aortic stenosis
Hraska V, Lilje C, Kantorova A, Photiadis J, Fink C, Asfour B, associated with hypoplasia of the aortic valve ring.
Lacour-Gayet F, Schneider M, Haun C (2010) Ross- J Thorac Cardiovasc Surg 70:909–917
Konno procedure in children: midterm results. World Kovalchin JP, Brook MM, Rosenthal GL, Suda K, Hoffman
J Pediatr Congenit Heart Surg 1:28–33 JIE, Silverman NH (1998) Echocardiographic hemody-
Hraska V, Sinzobahamvya N, Haun CH, Photiadis J, Arenz C, namic and morphometric predictors of survival after
Schneider M, Asfour B (2012) The long-term outcome two-ventricle repair in infants with critical aortic ste-
of open valvotomy for critical aortic stenosis in neo- nosis. J Am Coll Cardiol 32:237–244
nates. Ann Thorac Surg 94(5):1519–1526 Kunzelman KS, Grande KJ, David TE, Cochran RE, Verner ED
Ibrahim M, Cleland J, O’Kane H, Gladstone D, Mullholland (1994) Aortic root and valve relationships. Impact on
C, Craig B (1994) St Jude Medical prosthesis in chil- surgical repair. J Thorac Cardiovasc Surg 107:162–170
dren. J Thorac Cardiovasc Surg 108:52–56 Laudito A, Brook MM, Suleman S, Bleiweis MS, Thompson
Ilbawi MN, DeLeon SY, Wilson WR J et al. (1991) Extended LD, Hanley FL, Mohan Reddy V (2001) The Ross proce-
aortic valvuloplasty: a new approach for the manage- dure in children and young adults: a word of caution.
ment of congenital valvar aortic stenosis. Ann Thorac J Thorac Cardiovasc Surg 122:147–153
Surg 52:663–668 Leung MP, McKay R, Smith A, Anderson RH, Arnold R (1991)
Ishizaka T, Devaney EJ, Ramsburgh SR, Suzuki T, Ohyo RG, Critical aortic stenosis in early infancy. Anatomic and
Bove EL (2003) Valve sparing aortic root replacement echocardiographic substrates of successful open val-
for dilation of the pulmonary autograft and aortic vulotomy. J Thorac Cardiovasc Surg 101:526–535
regurgitation after the Ross procedure. Ann Thorac Leyh RG, Schmidtke C, Sievers H-H, Yacoub MH (1999)
Surg 75:1518–1522 Opening and closing characteristics of the aortic valve
Jahangiri M, Nicholson IA, del Nido PJ, Mayer JE, Jonas RA after different types of valve-preserving surgery.
(2000) Surgical management of complex and tunnel- Circulation 100:2153–2160
like subaortic stenosis. Eur J Cardiothorac Surg Li DY, Faury G, Taylor DG, Davis EC, Boyle WA, Mecham RP
17:637–642 et al. (1998) Novel arterial pathology in mice and humans
Jeanmart H, de Kerchove L, Glineur D, Goffinet JM, Rougui I, hemizygous for elastin. J Clin Invest 102:1783–1787
Van Dyck M, Noirhomme P, El Khoury G (2007) Aortic Lofland GK, McGrindle BW, Williams WG, Blackstone EH,
valve repair: the functional approach to leaflet pro- Tchervenkov CI, Sittiwankul R, Jonas RA (2001) Critical
lapse and valve-sparing surgery. Ann Thorac Surg aortic stenosis in the neonate: a multi-institutional
83:746–751 study of management, outcomes, and risk factors.
Jonas RA (2004) Left ventricular outflow tract obstruction: J Thorac Cardiovasc Surg 121:10–27
aortic valve stenosis, subaortic stenosis, supravalvular Lukacs L, Bartek I, Haan A et al. (1992) Ruptured aneurysms
aortic stenosis. In: Jonas RA (ed) Comprehensive surgi- of the sinus of Valsalva. Eur J Cardiothorac Surg 6:15
cal management of congenital heart disease. Arnold, Lupinetti FM, Pridjian AK, Callow LB, Crowley DC,
London, pp 320–340 Beekman RH, Bove EL et al. (1992) Optimum treat-
Jonas RA, Quaegebeur JM, Kirkim JW, Blackstone EH, ment of discrete subaortic stenosis. Ann Thorac Surg
Daicoff G et al. (1994) Outcomes in patients with inter- 54:467–470
rupted aortic arch and ventricular septal defect. Lupinetti FM, Duncan BW, Lewin M, Dyamenahalli U,
J Thorac Cardiovasc Surg 107:1099–1113 Rosenthal GL (2003) Comparison of autograft and
Karamlou T, Shen I, Alsoufia B, Burch G, Reller M, Silberbach allograft aortic valve replacement in children. J Thorac
M, Ungerleider RM (2005) The influence of valve phys- Cardiovasc Surg 126:240–246
16 iology on outcome following aortic valvotomy for Marasini M, Zannini L, Ussia GP, Pinto R (2003) Discrete sub-
congenital bicuspid valve in children: 30-year results aortic stenosis. incidence, morphology and surgical
from a single institution. Eur J Cardiothorac Surg impact of associated subaortic anomalies. Ann Thorac
27:81–85 Surg 75:1763–1768
Karl TR, Sano S, Brawn WJ, Mee RB et al. (1990) Critical aor- Mariani MA, Waterbolk TW, Strengers JL, Ebels T (1997)
tic stenosis in the first month of life: surgical results in Aortic valvuloplasty for aortic insufficiency associated
26 infants. Ann Thorac Surg 50:105–109 with ventricular septal defect. J Thorac Cardiovasc
Keane JF, Norwood WI, Bernhard WF (1983) Surgery for Surg 114:851–853
aortic stenosis in infancy. Circulation 68(Suppl 3):182 Marino BS, Wernovsky G, Rychlik J, Bockoven JR, Godinez
Keane JF, Driscoll DJ, Gersony WM, Hayes CJ, Kidd L, RI, Spray TL (1999) Early results of the Ross procedure
O’Fallon WM et al. (1993) Second natural history study in simple and complex left heart disease. Circulation
of congenital heart defects. Results of treatment of 100(Suppl II):II 162–II 166
patients with aortic valvar stenosis. Circulation Maron BJ, Spirito P (1998) Implications of left ventricular
87(Suppl 1):I16–I27 remodeling in hypertrophic cardiomyopathy. Am
Keating MT (1995) Genetic approaches to cardiovascular J Cardiol 81:1339–1344
disease: supravalvular aortic stenosis, Williams syn- Maron BJ, McKenna WJ, Danielson GK et al. (2003) American
drome, and long-QT syndrome. Circulation 92:142–147 College of Cardiology/European Society of Cardiology
Kleinert S, Geva T (1993) Echocardiographic morphome- clinical expert consensus document on hypertrophic
try and geometry of the left ventricular outflow tract cardiomyopathy. J Am Coll Cardiol 42:1687–1713
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
577 16
Martin MM, Lemmer JH, Shaffer E, Dick M, Bove EL (1988) Nickerson E, Greenberg F, Keating MT, McCaskill C, Shaffer
Obstruction to left coronary artery blood flow second- LG (1995) Deletions of the elastin gene at 7q11.23
ary to obliteration of the coronary ostium in supraval- occur in approximately 90% of patients with Williams
vular aortic stenosis. Ann Thorac Surg 45:16–20 syndrome. Am J Hum Genet 56:1156–1161
Massih TA, Vouhé PR, Mauriat P, Mousseaus E, Sidi D, Nicks R, Cartmill T, Bernstein L (1970) Hypoplasia of the
Bonnet D (2002) Replacement of the ascending aorta aortic root. Thorax 25:339–346
in children: a series of fourteen patients. J Thorac O’Connor WN, Davis JB, Geissler R, Cottrill CM, Noonan JA,
Cardiovasc Surg 124:411–413 Todd EP (1985) Supravalvular aortic stenosis: clinical
Mazzitelli D, Guenther T, Schreiber C, Wottke M, Michel J, and pathologic observations in six patients. Arch
Meisner H (1998) Aortic valve replacement in children: Pathol Lab Med 109:179–185
are we on the right track? Eur J Cardiothorac Surg Odim J, Laks H, Allada V, Child J, Wilson S, Gjertson D (2005)
13:565–571 Results of aortic valve sparing and restoration with
McCrindle BW (1996) Independent predictors of immedi- autologous pericardial leaflet extension in congenital
ate results of percutaneous balloon aortic valvotomy heart disease. Ann Thorac Surg 80:647–654
in childhood. Am J Cardiol 77:286–293 Ohye RG, Gomez CA, Ohye BJ, Goldberg CS, Bove EL (2001)
McCrindle BW, Blackstone EH, Williams WG et al. (2001) Are The Ross/Konno procedure in neonates and infants:
outcomes of surgical versus transcatheter balloon val- intermediate-term survival and autograft function.
votomy equivalent in neonatal critical aortic stenosis? Ann Thorac Surg 72(3):823–830
Circulation 104(Suppl 1):I-152–I-158 Parissis JT, Bistola V, Kostakis GD et al. (2004) Giant unrup-
McElhinney DB, Petrossian E, Tworetzky W, Silverman NH, tured aneurysm of right Valsalva sinus detected by
Hanley FL (2000) Issues and outcomes in the manage- magnetic resonance imaging. Int J Cardiol 96:299–301
ment of supravalvar aortic stenosis. Ann Thorac Surg Parry AJ, Kovalchin JP, Suda K, McElhinney DB, Wudel J,
69:562–567 Silverman NH, Reddy VM, Hanley FL (1999) Resection
McKay R, Smith A, Leung MP, Arnold R, Anderson RH (1992) of subaortic stenosis; can a more aggressive approach
Morphology of the ventriculoaortic junction in critical be justified? Eur J Cardiothorac Surg 15:631–638
aortic stenosis. Implications for hemodynamic func- Pelech AN, Dyck JD, Trusler GA, Williams WG, Olley PM, Rowe
tion and clinical management. J Thorac Cardiovasc RD, Freedom RM (1987) Critical aortic stenosis. Survival
Surg 104:434–442 and management. J Thorac Cardiovasc Surg 94:510–517
McMullan DM, Oppido G, Davies B, Kawahira Y, Cochrane Polimenakos AC, Sathanandam S, Elzein C, Barth MJ,
AD, d‘Udekem d’Acoz Y, Penny DJ, Brizard CP et al. Higgins RS, Ilbawi MN (2010) Aortic cusp extension
(2007) Surgical strategy for the bicuspid aortic valve: valvuloplasty with or without tricuspidization in chil-
tricuspidization with cusp extension versus pulmo- dren and adolescents: long-term results and freedom
nary autograft. J Thorac Cardiovasc Surg 134:90–98 from aortic valve replacement. J Thorac Cardiovasc
Mitchell BM, Strasburger JF, Hubbard JE, Wessel HU et al. Surg 139:933–941
(2003) Serial exercise performance in children with Pretre R, Arbenz U, Vogt PR, Turina MI (1999) Application of
surgically corrected congenital aortic stenosis. Pediatr successive principles of repair to correct supravalvular
Cardiol 24:319–324 aortic stenosis. Ann Thorac Surg 67:1167–1169
Miyamoto T, Sinzobahamvya N, Wetter J, Kallenberg R, Pretre R, Kadner A, Dave H, Bettex D, Genoni M (2006)
Brecher AM, Asfour B, Urban AE et al. (2006) Twenty Tricuspidisation of the aortic valve with creation of a
years experience of surgical aortic valvotomy for critical crown-like annulus is able to restore a normal valve
aortic stenosis in early infancy. Eur J Cardiothorac Surg function in bicuspid aortic valves. Eur J Cardiothorac
30:35–40 Surg 29:1001–1007
Mohan Reddy V, Rajasinghe HA, Teitel DF, Haas GS, Hanley Quiang GJ, Dong ZX, Xing XG et al. (1994) Surgical treat-
FL et al. (1996) Aortoventriculoplasty with the pulmo- ment of ruptured aneurysm of the sinus of Valsalva.
nary autograft: the “Ross-Konno” procedure. J Thorac Cardiol Young 4:347
Cardiovasc Surg 111:158–167 Ralph JD (1998) Aortic valve replacemnt: prosthesis. In: Kaiser
Mohan Reddy V, McElhinney DB, Phoon CK, Brook MM, LR, Kron IL, Spray TL (eds) Mastery of cardiothoracic sur-
Hanley FL et al. (1998) Geometric mismatch of pulmo- gery. Lippincott-Raven, Philadelphia, pp 361– 368
nary and aortic annuli in children undergoing the Ross Rastan H, Kaney J (1976) Aortoventrikuloplasty, a new
procedure: implications for surgical management and technique for the left ventricular outflow tract
autograft valve function. J Thorac Cardiovasc Surg 115: obstruction. J Thorac Cardiovasc Surg 71:920
1255–1263 Rayburn ST, Netherland DE, Heath BJ et al. (1997) Discrete
Moore M (1997) Pericardial tissue stabilized by dye- membranous subaortic stenosis: improved results
mediated photooxidation: a review article. J Heart after resection and myectomy. Ann Thorac Surg
Valve Dis 6:521–526 64:105–109
Moore P, Egito E, Mowrey H, Perry SB, Lock JE, Keane JF Reich O, Tax P, Marek J et al. (2004) Long term results of per-
(1996) Midterm results of balloon dilation of congeni- cutaneous balloon valvuloplasty of congenital aortic
tal aortic stenosis: predictors of success. J Am Coll stenosis: independent predictors of outcome. Heart
Cardiol 27:1257–1263 90:70–76
Myers JL, Waldhausen JA, Cyran SE, Gleason MM, Weber Rein AJ, Preminger TJ, Perry SB, Lock JE, Sanders SP (1993)
HS, Baylen BG (1993) Results of surgical repair of con- Generalized arteriopathy in Williams syndrome: an
genital supravalvular aortic stenosis. J Thorac intravascular ultrasound study. J Am Coll Cardiol
Cardiovasc Surg 105:281–288 21:1727–1730
578 V. Hraška and J. Photiadis
Rhodes LA, Colan SD, Perry SB, Jonas RA, Sanders SP (1991) Stamm C, Kreutzer C, Zurakovski D, Nollert G, Friehs I,
Predictors of survival in neonates with critical aortic Mayer JE, Jonas RA, del Nido PJ (1999) Forty-one years
stenosis. Circulation 84:2325–2335 of surgery for congenital supravalvar aortic stenosis.
Roberts WC (1973) Valvular, subvalvular and supravalvar J Thorac Cardiovasc Surg 118:874–885
aortic stenosis. Cardiovasc Clin 5:97–126 Stamm C, Friehs I, Moran AM, Zurakowsky D, Bacha E, Mayer
Roberts WC, Ko JM (2006) Clinical and morphologic features JE, Joans RA, del Nido PJ (2000) Surgical treatment of
of the congenitally unicuspid acommissural stenotic bilateral outflow tract obstruction in elastin arteriopa-
and regurgitant aortic valve. Cardiology 108:79–81 thy. J Thorac Cardiovasc Surg 120:755–763
Robicsek F, Thubrikar MJ, Cook JW, Fowler B (2004) The con- Stamm C, Friehs I, Ho SY, Moran AM, Jonas RA, del Nido PJ
genitally bicuspid aortic valve: how does it function? (2001) Congenital supravalvular aortic stenosis: a sim-
Why does it fail? Ann Thorac Surg 77:177–185 ple lesion? Eur J Cardiothorac Surg 19:195–202
Robinson BV, Brzenzinska-Rajszys G, Weber HS, Ksiazyk J, Stelzer P, Weinrauch S, Tranbaugh RF (1998) Ten years of
Fricker EJ, Fischer DR, Ettedgui JA (2000) Balloon aortic experience with the modified Ross procedure. J Thorac
valvotomy through a carotid cutdown in infants with Cardiovasc Surg 115:1091–1100
severe aortic stenosis: results of the multi-centric reg- Sud A, Parker F, Magilligan DJ (1984) Anatomy of the aortic
istry. Cardiol Young 10:225–232 root. Ann Thorac Surg 38:76–79
Samanek M, Slavik Z, Zborilova B et al. (1989) Prevalence, Suri RM, Dearani JA, Schaff HV, Danielson GK, Puga FJ
treatment and outcome of heart disease in live/born (2006) Long-term results of the Konno procedure for
children: a prospective analysis of 91, 823 live-born complex left ventricular outflow tract obstruction.
children. Pediatr Cardiol 10:205 J Thorac Cardiovasc Surg 132:1064–1071
Sands MP, Rittenhouse EA, Mohri H, Merendino KA (1969) Swanson M, Clark RW (1974) Dimensions and geometric
An anatomical comparison of human, pig, calf and relationships of the human aortic valve as a function
sheep aortic valves. Ann Thorac Surg 8:407–414 of pressure. Circulation 35:871–882
Sarsan MAI, Yacoub M (1993) Remodeling of the aortic valve Takach TJ, Reul GJ, Duncan JM (1999) Sinus of Valsalva
annulus. J Thorac Cardiovasc Surg 105:435–438 aneurysm or fistula: management and outcome. Ann
Schäfers HJ, Aicher D, Riodionycheva S, Lindinger A, Rädle- Thorac Surg 68:1573–1577
Hurst T, Langer F, Abdul-Khaliq H (2008) Bicuspidization Theodoro DA, Danielson GK, Feldt RH, Anderson BJ (1996)
of the unicuspid aortic valve: a new reconstructive Hypertrophic obstructive cardiomyopathy in pediatric
approach. Ann Thorac Surg 85:2012–2018 patients results of surgical treatment. J Thorac
Serino W, Andrade JL, Ross D et al. (1983) Aortico-left ven- Cardiovasc Surg 112:1589–1597
tricular communication after closure: late postopera- Tolan MJ, Daubeney PE, Slavik Y et al. (1997) Aortic valve
tive problems. Br Heart J 49:501 repair of congenital stenosis with bovine pericardium.
Serraf A, Piot JD, Bonnet N et al. (1999a) Biventricular repair Ann Thorac Surg 63:465–469
approach in Ductus-dependent neonates with hypo- Trusler GA, Moes CAF, Kidd BSL (1973) repair of ventricular
plastic but morphological normal left ventricle. J Am septal defect with aortic insufficiency. J Thorac
Coll Cardiol 33:827–834 Cardiovasc Surg 66:394–403
Serraf A, Zoghby J, Lacour-Gayet F, Houel R, Belli E, Galleti Tsang VT, de Leval MR (2006) Surgery of the left ventricular
L, Planche C et al. (1999b) Surgical treatment of sub- outflow tract. In: Tsang and DeLeval, Stark, DeLeval and
aortic stenosis: a seventeen-year experience. J Thorac Tsang, Surgery for Congenital Heart Defects, 3rd
Cardiovasc Surg 117:669–678 Edition, Wiley, London, UK, pp 489–514
Sharma GK, Wojtalik M, Siwinska A, Mrozinski B, Pawelec- Turley K, Silverman NH, Teitel D et al. (1982) Repair of
Wojtalik M, Bartkowski R (2004) Aortoventriculoplasty aortico-left ventricular tunnel in the neonate: surgical,
and left ventricle function: long-term follow-up. Eur anatomic and echocardiographic considerations.
J Cardiothoracic Surg 26:29–136 Circulation 65:1015
16 Shone JD, Sellers RD, Anderson RC et al. (1963) The devel- Tweddell JS, Pelech AN, Frommelt PC et al. (2005a) Complex
opmental complex of “parachute mitral” valve, supra- aortic valve repair as a durable and effective alternative
valvular ring of the left atrium, subaortic stenosis and to valve replacement in children with aortic valve dis-
coarctation of the aorta. Am J Cardiol 11:714–725 ease. J Thorac Cardiovasc Surg 129:551–558
Silver MA, Roberts WC (1985) Detailed anatomy of the nor- Tweddell JS, Pelech AN, Jaquiss RDB, Frommelt PC,
mally functioning aortic valve in hearts of normal and Mussatto LA, Hoffmann GM, Litwin SB (2005b) Aortic
increased weight. Am J Cardiol 55:454–461 valve repair. Semin Thorac Cardiovasc Surg Pediatr
Slater M, Shen I, Welke K, Komanapalli Ch, Ungerleider R (2005) Cardiac Surg Annu 2005:112–121
Modification to the Ross procedure to prevent autograft van Arsdell GS, Williams WG, Boutin C, Trusler GA, Coles JG,
dilatation. Semin Thorac Cardiovasc Surg 2005:181–184 Rebeyka IM, Freedom RM et al. (1995) Subaortic steno-
Solymar L, Sudow G, Berggren H, Erikson B (1992) Balloon sis in the spectrum of atrioventricular septal defects.
dilation of stenotic aortic valve in children. J Thorac J Thorac Cardiovasc Surg 110:1534–1542
Cardiovasc Surg 104:1709–1713 van Son JA, Danielson GK, Puga FJ (1994a) Supravalvar aor-
Sreeram N, Franks R, Arnold R et al. (1991) Aortico-left ven- tic stenosis. Long-term results of surgical treatment.
tricular tunnel: long term outcome after surgical J Thorac Cardiovasc Surg 107:103
repair. J Am Coll Cardiol 17:950 van Son JA, Edwards WD, Danielson GK (1994b) Pathology
Stamm C, Li J, Ho SY, Redington AN, Anderson RH (1997) of coronary arteries, myocardium and great arteries in
The aortic root in supravalvular aortic stenosis. supravalvar aortic stenosis. J Thorac Cardiovasc Surg
J Thorac Cardiovasc Surg 114:16–24 108:21–28
Chapter 16 · Anomalies of the Left Ventricular Outflow Tract
579 16
van Son JAM, Danielson GK, Schaff HV et al. (1994c) Long- Williams JC, Barrat-Boyes BG, Lowe JB (1961) Supravalvar
term outcome of surgical repair of ruptured sinus of aortic stenosis. Circulation 24:1311–1318
Valsalva aneurysm. Circulation 90:II20 Yacoub MH, Khan H, Starvi G, Shinebourne E, Radley-Smith
Vongpatanasin W, Hillis LD, Lange RA (1996) Prosthetic R (1997) Anatomic correction of the syndrome of pro-
heart valves. N Engl J Med 335:407–416 lapsing right coronary aortic cusp, dilatation of the
Vricella LA, Williams JA, Ravekes WJ, Holmes KW, Dietz HC, sinus of Valsalva, and ventricular septal defect. J Thorac
Gott VL, Cameron DE (2005) Early experience with Cardiovasc Surg 113:253–261
valve-sparing aortic root replacement in children. Ann Zalzstein E, Moes CAF, Musewe NN, Freedom RM (1991)
Thorac Surg 80:1622–1627 Spectrum of cardiovascular anomalies in Williams-
Waldner P, Dhillon R, Taylor JF et al. (1996) An alternative Beuren syndrome. Pediatr Cardiol 12:219–223
method for repair of aortico-left ventricular tunnel Zeevi B, Keane JF, Castaneda AR, Perry SB, Lock JE (1989)
associated with severe aortic stenosis presenting in a Neonatal critical valvar aortic stenosis. A comparison of sur-
newborn. Eur J Cardiothorac Surg 10:380–382 gical and balloon dilation therapy. Circulation 80:831–839
581 17
References – 594
Chapter 17 · Surgery for Aortic Atresia, Hypoplastic Left Heart Syndrome
583 17
17.1 Cardiac Pathoanatomy also be hypoplastic, however, not as severely as
the ascending aorta, as they receive the blood
Aortic atresia is defined by a complete occlusion flow for the supra aortic vessels.
of the aortic valve and a hypoplastic ascending Pulmonary artery and ductus arteriosus are
aorta. The isolated form with two normally big and well developed. The septum primum is
developed ventricles is extremely rare and exclu- typically muscularized and thickened. Sometimes
sively combined with a nonrestrictive ventricular it is shifted toward the left atrium: atrial septum
septal defect and a normally developed mitral malposition. Without severe restriction at the
valve. level of the foramen ovale, the left atrium is
The term «hypoplastic left heart syndrome» smaller than the right one.
was introduced by Noonan and Nadas in 1958. It In most cases, the leading lesion is a valvular
describes a spectrum of malformations in which hypoplasia/obstruction either of the mitral or of
the left ventricle is unable to support the systemic the aortic valve. As a consequence of the reduced
circulation due to its size. In these cases, not only blood flow, growth of the left ventricle and its
the left ventricle but also its adjacent structures, adjacent structures is reduced or had stopped at
mitral valve, aortic valve, and ascending aorta, are some stage. This has been proven in chicken
severely hypoplastic. A combination of hypoplas- embryos. By obstructing their mitral valve, hypo-
tic AV and semilunar valve features (mitral steno- plastic left heart syndrome could be produced
sis or atresia, aortic stenosis or atresia) defines the (Harh et al. 1973). The same process could be
hypoplasia of the left ventricle and its adjacent observed by fetal echocardiography in aortic ste-
structures. nosis in humans, originally described by L. Allan
Traditionally four subtypes are differentiated: and coworkers at Guy’s Hospital in London
55 Mitral atresia, aortic atresia (Allan et al. 1989). They found a critical aortic
55 Mitral hypoplasia/stenosis, aortic atresia stenosis with severely impaired left ventricular
55 Mitral atresia, aortic hypoplasia/stenosis contractility in a fetus in the 22nd week of gesta-
55 Mitral hypoplasia/stenosis, aortic hypoplasia/ tion. At 32 weeks of gestation, the ventricle was
stenosis not grown but small and highly echogenic, as in
typical hypoplastic left heart syndrome. Its
Further lesions leading to hypoplasia of the dimensions were the same as in the 22nd week of
left ventricle during fetal live are: gestation. Our group made the same observa-
55 Critical aortic coarctation tions in our fetal cardiac program. The hypothe-
55 Asymmetric septation in unbalanced AV canal sis of a valvular lesion as the reason for
55 Straddling mitral valve ventricular hypoplasia is the basis for fetal valvu-
55 Complex ventricular septal defect (VSD) and lar interventions. If such an intervention (bal-
hypoplastic or interrupted aortic arch loon valvuloplasty) is done early, an otherwise
55 Multiple stenotic lesions in series in the left lost ventricle could be rehabilitated (Makikallio
ventricular system (Shone syndrome) et al. 2006)
55 Isolated Severe Mitral Stenosis This also explains that there is no strict cutoff
line between critical aortic stenosis and hypoplas-
Borderline cases without a primary valvular tic left heart syndrome with a spectrum of border-
lesion were defined as hypoplastic left heart com- line cases concerning the size of the left ventricle.
plex by C. Tchervenkov in 1998.
In hypoplastic left heart syndrome, the
ascending aorta is usually very thin walled and 17.2 Extracardiac Malformations
fragile. Especially in aortic atresia with retro-
grade perfusion, functionally the hypoplastic Congenital cardiac malformations are frequently
ascending aorta can be looked at as a common combined with extracardiac malformations. For
coronary artery. A diameter of less than 2 mm is hypoplastic left heart syndrome, rates reported
most frequent in these cases. If there is antegrade are between 12 and 37 %.
blood flow (aortic stenosis), it is usually bigger in While one post mortem study shows an
diameter. Aortic arch and aortic isthmus may overall malformation rate in hypoplastic left
584 R. Mair and E. Sames-Dolzer
aortic arch with the supraaortic branches and the A.pulmonalis Ascending aorta
first 10–15 mm of the descending aorta are dextra
dissected and mobilized, as well as the ductus
arteriosus. Marking stitches are placed on the PTFE Ductus
ascending aorta and the main pulmonary artery at prosthesis arteriosus
the planned anastomotic sites to avoid distortion. A.pulmonalis
sinistra
If it is planned to perfuse via the brachioce-
Truncus
phalic trunk, it has to be decided whether this is pulmonalis
done by direct cannulation or through a small
PTFE tube prosthesis (Pigula et al. 1999). Heparin
is now administered. In case prosthesis is used for V.cava
cannulation, the end-to-side anastomosis between superior
a 3.5 mm PTFE prosthesis and the brachioce-
phalic trunk is now performed.
A.pulmonalis sinistra
A.pulmonalis dextra
Truncus pulmonalis
..Fig. 17.3 Aortic arch reconstruction with a patch RV-PA-Conduit
between superior vena cava and the common sected and the distal end of the conduit is sewn
atrium. Once shunt or conduit is closed, the ven- over transversely. If the distal anastomosis has
tricle does no longer pump into the pulmonary been made on the right side, the conduit must be
circulation. Systemic oxygen saturation after a removed completely. The site of the anastomosis
bidirectional Glenn shunt is usually between 80 can be used for the bidirectional cavopulmonary
and 85 %. This is similar to the situation after stage anastomosis. Usually it has to be enlarged by
I palliation, but it is achieved with a significantly 2–3 mm. In case of a stenosis of the pulmonary
smaller workload of the ventricle. So the efficiency arteries, this has to be repaired simultaneously
of heart labor is optimized and the ventricle either by a patch plasty or, if there is compression
should be better prepared for Fontan completion. under the aortopulmonary truncus, with a stent
placed intraoperatively.
17.6.3.2 Timing of the Procedure While the Glenn anastomosis can be per-
The timing of the bidirectional cavopulmonary formed off pump in cases with a left-sided conduit
anastomosis varies from center to center. As the anastomosis, shunt or conduit anastomoses on
flow through the pulmonary vessels is reduced by the right pulmonary artery at stage I surgery
the Glenn procedure, it is mainly an outweighing require normothermic extracorporeal circulation
between potential further growth of the pulmonary (beating heart) for stage II.
arteries on the one side and early unloading the
ventricle to keep the ventricular performance and
possibly reduce interstage mortality on the other 17.6.4 Stage III: Fontan Operation
side. Many authors recommend an interval of
6 months between stage I and stage II. In our unit, The completion of the bidirectional cavopulmo-
we think it is important to unload the systemic ven- nary anastomosis, namely, from partial to total
tricle early as it is a right ventricle. Regarding the cavopulmonary connection, is the third step in
pulmonary vascular resistance, the earliest possible definite surgical palliation for univentricular
time would be after 6 weeks. We prefer an interval hearts. The operative technique is described in
of 3–4 months between stage I and stage II. 7 Chapter «Definite Palliation of Functional Single
Ventricle» Sect. 13.7.3. The age of operation
17.6.3.3 Operative Technique depends to some extent on the method used for
As a rule, we generally perform a simple bidirec- this Fontan completion (lateral tunnel or extra-
tional Glenn shunt. We do not perform Hemi- cardiac conduit). As we prefer a fenestrated extra-
Fontan techniques. Surgery is described in cardiac conduit, we usually perform the procedure
7 Chapter «Definite Palliation of Functional Single during the third year of life. The patient should
Ventricle» Sect. 13.7.2.1 and Sect. 13.7.2.2. A special weigh 12 kg, so that PTFE tube prosthesis of
feature in hypoplastic left heart syndrome with 20 mm in diameter can be used. The following
aortic atresia is that great attention must be paid aspects made the extracardiac conduit the method
in order to not compress the small aorta, while of choice for us.
clamping the right pulmonary artery during the In this version, we avoid major suture lines on
bidirectional cavopulmonary anastomosis. This the atrial wall. Only when closing the junction of
17 could result in severe ventricular failure. the inferior vena cava (IVC), atrial tissue may be
incorporated in the suture line. In addition, no
kTechnique after a Blalock-Taussig shunt part of the atrial wall is under the elevated sys-
Besides the abovementioned aspects, there is no temic venous pressure employing this technique.
difference to other univentricular hearts with aor- This should minimize rhythm problems in this
topulmonary shunts. cohort of patients.
Using an extracardiac conduit, the aorta is not
kTechnique after RV-PA conduit to be cross clamped, and there is no period of
The RV-PA conduit is cross clamped and divided. ischemia for the heart in this procedure. We deem
The proximal end is sewn over very close to the this to be especially important in face of a right
infundibular anastomosis, so that no blind stump ventricle supporting the systemic circulation.
is left. If the distal conduit anastomosis has been Furthermore, an extensive dissection would be
made on the left side, this region has to be dis- necessary to cross clamp the aorta after a Norwood
Chapter 17 · Surgery for Aortic Atresia, Hypoplastic Left Heart Syndrome
591 17
procedure if at all possible after complex neoaor- occurred. The preoperative status clearly
tic reconstruction. influences postoperative outcome.
55 Introduction of the RV-PA conduit. The severe
changes in pathophysiology, creating a physi-
17.6.5 Results of Univentricular ology similar to a PAB, lead to a more stable
Palliation condition especially in the early postoperative
period. We are well aware that the RV-PA
The total mortality of univentricular palliation conduit is not shown to be superior in larger
comprises the mortalities of each surgical stage as studies; however, it clearly helped us to
well as the interstage mortality. improve our results.
Before the Norwood operation (stage I), neo- 55 Evolution of extracorporeal circulation. We
nates and infants with hypoplastic left heart syn- replaced deep hypothermic circulatory arrest
drome suffer from univentricular circulation with initially by selective antegrade cerebral perfu-
increasingly nonrestrictive pulmonary blood flow sion and later on by complete body perfusion
resulting in diastolic systemic runoff and conse- via an additional arterial cannula in the
quently poor systemic perfusion. supradiaphragmatic aorta. This change in
The result of a successful Norwood procedure perfusion technique makes the operation
is a balanced univentricular circulation. Pulmonary comparable to other procedures using extra-
blood flow is restricted, either in form of shunt corporeal circulation and reduces the detri-
physiology (when a BT shunt is used) or in form of mental effects of intraoperative ischemia.
a functional pulmonary artery banding (PAB)
(using an RV-PA conduit). This leads to some relief Summarizing these changes, the significant
of ventricular volume load; however, the ventricle improvement of the results over time can be
has still to be considered volume overloaded until explained.
stage II surgery is performed. The problems of bal- Postoperative morbidity can be caused by aor-
ancing the two circulations after extensive pallia- tic arch stenosis, which can be kept low by using
tive surgery on extracorporeal circulation attribute the right patch form. In our series, aortic arch
to the higher mortality compared to corrective reoperations were required in 2.5 %, and cathlab
neonatal surgery. interventions in 6 % of patients. Stenoses of the
In the early days of Norwood procedures pulmonary arteries seem to be more common in
performed, the survival rate was somewhere the RV-PA conduit group (about 12 % in our
between 50 and 70 %. Today, several centers come series) than with Blalock-Taussig shunts. They
up with rates above 90 %. In our group, the sur- can usually be addressed at the time of the bidi-
vival rate of the Norwood procedure improved rectional cavopulmonary anastomosis.
from initially 66 % in 1998 to 87.4 % in a consec- The bidirectional cavopulmonary anastomosis
utive series of 206 unselected patients since 2003 is technically a rather straightforward procedure
(complete body perfusion by add. cannulation that reduces ventricular volume load. The mortal-
of the descending aorta). As independent risk ity of the Glenn shunt ranges between 0 and 2 %.
factors for mortality after the Norwood proce- This operation contributes only minimally to the
dure, we identified age older than 20 days at time overall mortality of the univentricular palliation
of operation, the anatomic subgroup of unbal- pathway.
anced AV canal and former trial of biventricular This consideration is also valid for the com-
repair. pletion to the total cavopulmonary connection.
Apart from the learning curve of the surgeon The extracardiac version of the Fontan palliation
and other responsible team members, we identi- can be safely performed without cross clamping
fied the following factors to have influenced the the aorta, gaining near normal arterial saturation
improvements of our results: by the same cardiac workload. Mortality rate is
55 Prenatal diagnostics. Prenatally identified between 0 and 2 %.
patients usually come to the specialized cen- Causes for death between stage I and stage II
ter in better condition than those who get can be:
diagnosed only once severe cardiac failure or 55 Residual or recurrent anatomic lesions like a
circulatory shock after ductal closure restrictive interatrial septum, obstruction of
592 R. Mair and E. Sames-Dolzer
the aortic arch or stenosis of the BT shunt, Aortic arch repair, aortopulmonary anasto-
RV-PA conduit or pulmonary artery. mosis, and atrial septectomy are done together
55 Trivial gastrointestinal infections can sud- with the bidirectional cavopulmonary anastomo-
denly lead to dehydration and circulatory sis at stage II, which now may be called compre-
instability. hensive stage II. The number of centers applying
55 Respiratory infections can rapidly cause this procedure, as well as the numbers of cases, is
severe respiratory insufficiency and death. still small. Therefore, a fair comparison with the
Norwood procedure is difficult.
A strict home monitoring program looking at It is the advantage of this method that it avoids
certain parameters (pulse oximetry, weight gain, cardiopulmonary bypass, deep hypothermia, and
food habits) was introduced by the Milwaukee circulatory arrest in a newborn for a palliative
group and showed significant advantage procedure only. Consequently, this approach less-
(Ghanayem et al. 2003). The early performance of ens the surgical trauma for stage I palliation. This
the bidirectional cavopulmonary anastomosis at could be of increased importance in high-risk
the age of 3–4 months is also commonly seen as patients especially in low birth weight or imma-
an advancement and is practiced at our unit. Our ture neonates.
own experience showed that the – what we call – In some centers, it is therefore used predomi-
banded physiology of the RV-PA conduit was able nantly in this high-risk group of patients.
to lower the interstage mortality in comparison to Pizarro and coworkers however found out that
the BT shunt group. there is no significant difference in survival in
Only few reliable data are published about the high-risk patients whether a Norwood procedure
interstage mortality between stages II and III. Death or a hybrid approach is used (Pizarro et al. 2007).
can be caused by compromised ventricular func- They also reported that between stage I and stage
tion, cardiac failure due to multiple aortopulmo- II procedures, a substantial number of reinterven-
nary collaterals, or rhythm disturbances. Overall, tions concerning stents and pulmonary artery
lethal events seem to be rare in this period. band were necessary. The comprehensive stage II
55 Mitral valve area >4.75 cm2/m2 monary anastomosis are the same as in a con-
55 Left ventricular long axis dimension to long ventional Norwood procedure. An intracardiac
axis of the heart >0.8 baffle is necessary to direct the left ventricular
55 Left ventricular mass > 35 g/m2 flow through the VSD into the pulmonary artery,
55 Aortic root diameter 3.5 cm/m2 similar to Rastelli procedure. A valved or non-
valved conduit is used to reestablish the continu-
These parameters are valid only for critical ity between RV and central pulmonary arteries.
aortic stenosis. This procedure can be performed as a com-
The left ventricular outflow tract however can plete repair in one stage, as we have done it suc-
be enlarged surgically by a Konno or Ross-Konno cessfully in our unit several times. Other authors
procedure and is therefore of very limited value as recommend a two-stage procedure:
a criteria for biventricular repair in critical aortic The first step would be a conventional
stenosis. Norwood procedure. In second step, the intracar-
These criteria are also of very limited value in diac baffle is made and the RV-PA conduit is
aortic coarctation with hypoplastic left ventricle. implanted.
For coarctation and hypoplastic left heart com-
plex, Tchervenkov and coworkers showed, that
even smaller ventricles than those selected by the
Rhodes criteria are amenable for biventricular References
repair, if certain requirements are fulfilled
55 Good left ventricular function Akintürk H, Michel-Behnke I, Valeske K et al. (2002)
55 No mitral or aortic valve stenosis Stenting of the arterial duct and banding of the pul-
monary arteries. Basis for the combined Norwood
55 No endocardial fibroelastosis
stage 1 and 2 repair in hypoplastic left heart.
55 Antegrade blood flow in the ascending aorta Circulation 105:1099–1103
and the proximal aortic arch Allan LD, Sharland G, Tynan M (1989) The natural history
of hypoplastic left heart syndrome. Int J Cardiol
Undisturbed antegrade blood flow through 25:341–343
Allan LD, Maxwell DJ, Carminati M, Tynan MJ (1995)
the mitral valve is also crucial for the success of
Survival after fetal aortic balloon valvuloplasty.
biventricular repair in these cases. It is a require- Ultrasound Obstet Gynecol 5:90–91
ment for left ventricular growth. That means that Arzt W, Wertaschnigg D, Veit I, Klement F, Gitter R, Tulzer G
any ASD should to be closed. Furthermore, all (2011) Intrauterine aortic valvuloplasty in fetuses with
obstructions in the outflow tract – aortic root, critical aortic stenosis: experience and results of 24 pro-
cedures. Ultrasound Obstet Gynecol 37(6):689–695
ascending aorta, aortic arch, and isthmus – must
Bacha EA, Daves S, Hardin J et al. (2006) Single-ventricle
be removed aggressively. palliation for high risk neonates: the emergence of an
Different criteria were elaborated by van Son alternative hybrid stage I strategy. J Thorac Cardiovasc
for the repair of the unbalanced AV canal. Van Surg 131:163–171
Son and coworkers found that a left ventricular Bailey L, Conception W, Shattuck H, Huang L (1986) Method
of heart transplantation for treatment of hypoplastic
volume index greater than 15 ml/m2 is required
left heart syndrome. J Thorac Cardiovasc Surg 92:1–5
for biventricular repair of this malformation. A
17 big VSD component and a dysplastic mitral valve
Bridges ND, Jonas RA, Mayer JE, Flanagan MF, Keane JF,
Castaneda AR (1990) Bidirectional cavopulmonary
component worsen the prognosis of biventricular anastomosis as interim palliation for high- risk Fontan
repair (Van Son et al. 1997). candidates. Circulation 82(Suppl IV):170–176
Cayler GG, Smeloff EA, Miller GE (1970) Surgical palliation
for hypoplastc left side of the heart. N Engl J Med
282:780–783
17.6.9 ortic Atresia and Normally
A Doty DB, Knott HW (1977) Hypoplastic left heart syn-
Sized Left Ventricle drome. Experience with an operation to establish
functionally normal circulation. J Thorac Cardiovasc
Surg 74:624–630
This rare form of aortic atresia can only exist
Ghanayem NS, Hoffman GM, Mussatto KA et al. (2003)
with an unrestrictive VSD. A biventricular Home surveillance program prevents interstage mor-
reconstruction is possible in this malformation. tality after the Norwood procedure. J Thorac
The principles of aortic arch repair and aortopul- Cardiovasc Surg 126:1367–1377
Chapter 17 · Surgery for Aortic Atresia, Hypoplastic Left Heart Syndrome
595 17
Glauser TA, Rorke LB, Weinberg PM, Clancy RR (1990) Norwood WI, Lang P, Hansen DD (1983) Physiologic repair
Congenital brain anomalies associated with hypoplas- of aortic atresia – hypoplastic left heart syndrome. N
tic left heart syndrome. Pediatrics 85:984–990 Engl J Med 308:23–26
Griselli M, McGuirk SP, Stümper O et al. (2006) Influence of Ohye RG, Sleeper LA, Mahony L, Newburger JW et al.
surgical strategies on outcome after the Norwood pro- (2010) Comparison of shunt types in the Norwood
cedure. J Thorac Cardiovasc Surg 131:416–426 procedure for single-ventricle lesions. N Engl J Med
Harh JY, Paul MH, Gallen WJ et al. (1973) Experimental pro- 362(21):1980–1992
duction of hypoplastic left heart syndrome in the Pigula FA, Siewers RD, Nemoto EM (1999) Regional perfu-
chick embryo. Am J Cardiol 73:51–56 sion of the brain during neonatal aortic arch recon-
Hofer A, Haizinger B, Geiselseder G, Mair R, Rehak P, struction. J Thorac Cardiovasc Surg 117:1023–1024
Gombotz H (2005) Monitoring of selective antegrade Pizarro C, Derby CD, Baffa JM, Murdison KA, Radtke WA
cerebral perfusion using near infrared spectroscopy in (2007) Improving outcome of high-risk neonates with
neonatal aortic arch surgery. Eur J Anaesthesiol hypoplastic left heart syndrome: hybrid procedure or
22:293–298 conventional surgical palliation? Eur J Cardiothorac
ImotoY KH, Shiokawa Y, Minami K, Yashui H (2001) Surg 33(4):613–618
Experience with the Norwood procedure without cir- Pizarro C, Murdison KA, Radtke WA, Derby CD (2008) Stage
culatory arrest. J Thorac Cardiovasc Surg 122:879–882 II reconstruction after hybrid palliation for high-risk
Mair R, Tulzer G, Sames E et al. (2003) Right ventricular to patients with a single ventricle. Ann Thorac Surg
pulmonary artery conduit instead of modified Blalock- 85(4):1332–1338
Taussig shunt improves postoperative hemodynamics Rhodes LA, Colan SD, Perry SB, Jonas RA, Sanders P (1991)
in newborns after the Norwood operation. J Thorac Predictors of survival in neonates with critical aortic
Cardiovasc Surg 126:1378–1384 stenosis. Circulation 84:2325–2335
Makikallio K, McElhinney DB, Levine JC et al. (2006) Fetal Sano S, Ishino K, Kawada M et al. (2003) Right ventricle-
aortic valve stenosis and the evolution of hypoplastic pulmonary artery shunt in first stage palliation of
left heart syndrome: patient selection for intervention. hypoplastic left heart syndrome. J Thorac Cardiovasc
Circulation 113:1401–1405 Surg 129:504–509
Mavroudis C, Harrison H, Klein JB et al. (1988) Infant ortho- Sano S, Ishino K, Kado H et al. (2004) Outcome of right-
topic cardiac transplantation. J Thorac Cardiovasc ventricle–to-pulmonary artery shunt in first stage pal-
Surg 96:912–924 liation of hypoplastic left heart syndrome: a
Michel-Behnke I, Akintuerk H, Marquardt I et al. (2003) multi-institutional study. Ann Thorac Surg 78:1951–
Stenting of the ductus arteriosus and banding of the 1957
pulmonary arteries: basis for various surgical strate- Tchervenkov CI, Tahta SH, Jutra LC, Beland MJ (1998)
gies in newborns with multiple left heart obstructive Biventricular repair in neonates with hypoplastic left
lesions. Heart 89:645–650 heart complex. Ann Thorac Surg 66:1350–1357
Natowicz M, Chatten J, Clancy R et al. (1988) Genetic disor- Tworetzky W, McElhinney DB, Reddy VM, Brook MM, Hanley
ders and major extracardiac anomalies associated FL, Silverman NH (2001) Improved surgical outcome
with hypoplastic left heart syndrome. Pediatrics after fetal diagnosis of hypoplastic left heart syn-
82:698–706 drome. Circulation 103(9):1269–1273
Noonan JA, Nadas AS (1958) The hypoplastic left heart syn- Tworetzky W, Wilkins-Haug L, Jennings RW et al. (2004)
drome: an analysis of 101 cases. Pediatr Clin N Am Balloon dilatation of severe aortic stenosis in the fetus:
5:1029–1056 potential for prevention of hypoplastic left heart syn-
Norwood WI, Kirklin JK, Sanders SP (1980) Hypoplastic left drome: candidate selection, technique, and results of
heart syndrome: experience with palliative surgery. successful intervention. Ciculation 110:2125–2131
Am J Cardiol 45:87–91 Van Son JAM, Phoon CK, Silverman NH, Haas GS (1997)
Norwood WI, Lang P, Castaneda AR, Campbell DN (1981) Predicting feasibility of biventricular repair of right
Experience with operations for hypoplastic left heart dominant unbalanced atrioventricular canal. Ann
syndrome. J Thorac Cardiovasc Surg 82:511–519 Thorac Surg 63:1657–1663
597 18
References – 605
18 A1/I A2/II A3 A4
..Fig. 18.1 Truncus arteriosus types according to classification by Van Praagh and Van Praagh (A1–A4) and by Collett
and Edwards (I–IV). Types A1 and A2 are similar to types I–III. Type A1/I common origin of pulmonary arteries from the
truncus, type A2/II separates right and left branch pulmonary arteries from the dorsal truncus; type III is similar to type
II, but with further distance of the ostia of the separate pulmonary arteries (not shown). Type A3, only the branch pul-
monary artery contralateral to aortic arch sidedness arises directly from the truncus. The other branch pulmonary artery
(in this case the left) is connected through a usually narrow, ductus-like vessel with the aortic arch. Type A4, the truncus
continues via a ductus arteriosus into the descending aorta. The right and left pulmonary arteries come off the truncus
before the ductus. The ascending aorta is small. It appears to arise from the main pulmonary artery. The aortic arch is
interrupted. For type IV see text
Chapter 18 · Common Arterial Trunk
599 18
is no main pulmonary artery. Aortopulmonary 18.4 Pathophysiology
collaterals supply the lungs; therefore, this form is
now classified into the group pulmonary atresia The lungs are perfused during systole and diastole
with ventricular septal defect. with systemic pressures. This becomes more
prominent with fall of pulmonary vascular resis-
tance which increases symptoms. Truncal valve
18.3.2 Truncal Valve regurgitation reduces diastolic pressure which
leads to diastolic retrograde flow in the abdomi-
The truncal valve resembles a fused pulmonary nal aorta. A pressure gradient across the truncal
and aortic valve. The number of cusps varies from valve is rather attributed to these hemodynamics
two to six. Rarely there are more than four indi- than a morphological narrowing. Despite
vidual cusps. More commonly some cusps or increased pulmonary blood flow, a mild cyanosis
commissures are not completely developed, is secondary to the fact that both ventricles eject
which can result in significant truncal valve regur- into the truncal artery. An irreversible pulmonary
gitation. Additionally these cusps can be myxo- hypertension can result as early as in the sixth
matous which allows placement of stitches for month of life (Marciletti et al. 1976).
reconstruction.
18.5 Diagnostics
18.3.3 Ventricular Septal Defect
Physical examination and chest radiographs show
The ventricular septal defect is located usually signs of increased pulmonary blood flow.
directly underneath the truncal valve and has Echocardiography is usually sufficient for diagno-
some distance from the tricuspid valve. Therefore, sis (and indication for surgery). Cardiac catheter-
the risk of AV block after closure of the ventricu- ization is indicated to evaluate pulmonary
lar septal defect is low. In rare cases, when the resistance in patients older than 3 months.
ventricular septal defect is very small, closure can
create left ventricular outflow tract obstruction.
18.6 Operation
18.3.4 Coronary Arteries 18.6.1 Preoperative Management
Often the origin of the coronary arteries is Truncus arteriosus is a cyanotic heart defect,
abnormal; however, the course is usually normal. which does not improve with prostaglandins.
The origins can be located very close to the ori- When there is associated interrupted aortic arch,
gins of the right and left pulmonary arteries. The the patent ductus arteriosus has no tendency to
left anterior descending (LAD) branch can origi- close. Rarely patients present with circulatory col-
nate from the right coronary and run across the lapse or acidosis. More commonly heart failure
infundibulum – like in tetralogy of Fallot (Suzuki secondary to increased pulmonary blood flow is
et al. 1989). found. Medical therapy – except for short-term
stabilization to optimize the preoperative status –
is not beneficial.
18.3.5 Associated Anomalies
Most commonly the right aortic arch (25 %) and 18.6.2 Indication for Surgery
aberrant right subclavian artery (5–10 %) are and Timing
found (Calder et al. 1976). Interrupted aortic arch
(type A and B) is also frequently associated (10– Diagnosis is an indication for surgical correction.
15 %). In up to 90 % of cases, a DiGeorge syn- After stabilization in the intensive care unit, the
drome can be found (Jahangiri et al. 2000; Sano procedure should be performed within the first
et al. 1990). 2–3 weeks of life.
600 B. Asfour and L. Duebener
a b
c d
e f
..Fig. 18.2 a Intraoperative situs after cannulation in truncus arteriosus type A1/I and lines of incision for excision of
the pulmonary arteries. b With the heart arrested, the truncus is transected with preservation of the coronary arteries.
In the next step, the right ventricle will be incised between stay sutures. c The excision site of the pulmonary artery is
closed. The right ventricular outflow tract is incised between stay sutures. The pulmonary artery bifurcation is marked
with a stay suture. d The excision of the pulmonary arteries from the common truncus arteriosus (type A2/II, III) is
accomplished by transection of the truncus at the level of the pulmonary arteries. e The truncus is transected, and the
bifurcation of the pulmonary arteries is separated. f The ventricular septal defect is closed with interrupted sutures with
pledgets or with a continuous suture. As the bundle of His is not located in the inferior border of the ventricular septal
defect, the sutures can be placed safely there. g Patch in place for closure of ventricular septal defect
602 B. Asfour and L. Duebener
a b
..Fig. 18.3 a Implantation of a valved homograft. The distal anastomosis has already been accomplished. Proximally
one third of the circumference of the homograft is anastomosed to the right ventricle. b The reconstruction of the right
ventricular outflow tract is completed proximally with an autologous pericardial patch
..Fig. 18.4 Trimming of the proximal end of a valveless polytetrafluoroethylene graft. The insert shows that the distal
level of beveling is in a 90° angle to the proximal one
struction, the cannula is advanced into the innominate artery. For reconstruction the
innominate artery and snared with a tourniquet. descending aorta is controlled with a Castaneda
At 18 °C cardiopulmonary bypass flow is reduced clamp, and both supraaortic branches (left com-
to 30–50 % of calculated whole body flow. A right mon carotid artery, left subclavian artery) are
radial artery line is used for invasive blood pres- clamped with small neurosurgical Yasargil clips.
sure monitoring. The goal is a pressure of Weaning from cardiopulmonary bypass is
30–35 mmHg to prevent potential cerebral edema. carried out in standard fashion with low dose of
To improve exposure the arterial cannula can be dopamine (4–6 μg/kg kg/min). The chest is
connected through a polytetrafluoroethylene closed primarily. Other groups perform rou
shunt (3.5 mm), which is anastomosed to the tinely a secondary chest closure – especially in
604 B. Asfour and L. Duebener
a b
..Fig. 18.5 a–c Repair of truncus arteriosus with associated interrupted aortic arch. a, b The pulmonary arteries are
excised from the truncus along the depicted lines, and the ductus arteriosus is transected. c The proximal ascending
aorta and the proximal ductus arteriosus are oversewn
very small patients (body weight <2.5 kg) – after hypertension is more common in late infancy
1–3 days. (Bando et al. 1996). Therefore, a thin catheter
should be placed via the right ventricular out-
flow tract into the pulmonary artery. Otherwise
18.6.8 Special Postoperative clinical criteria can be used to diagnose pulmo-
Problems nary hypertension (e.g., high central venous
18 pressure, arterial desaturation, tachycardia,
Postoperative care can be challenging because hypotension, acidosis, oliguria). It is our policy
of pulmonary hypertension and right ventricu- to keep the patients ventilated during the first
lar dysfunction (secondary to the ventriculot- postoperative night, or up 48 h postoperatively,
omy). to lower the pulmonary vascular resistance.
Before considering medical treatment of pul- Nitric oxide (10–40 ppm) is administered if
monary hypertension, the operative result there is an otherwise therapy-resistant elevation
should be evaluated, and a large residual ven- of pulmonary vascular resistance. Only rarely
tricular septal defect should be excluded by extracorporeal membrane oxygenation is used.
echocardiography. Life-threatening pulmonary (Aguilar JM et al. 2015)
Chapter 18 · Common Arterial Trunk
605 18
References tricular outflow tract reconstruction. Eur J Cardiothorac
Surg 20:95–103
Jahangiri M, Zurakowski D, Mayer JE, del Nido PJ, Jonas RA
Aguilar JM, Garcia E, Arlati FG et al. (2015) Repair of type III
(2000) Repair of the truncal valve and associated inter-
common arterial trunk with modified Barbero-Marcial
rupted arch in neonates with truncus arteriosus.
technique. J Thorac Cardiovasc Surg 150:e69–70
J Thorac Cardiovasc Surg 119:508–514
Bando K, Turrentine MW, Sharp TG et al. (1996) Pulmonary
Jonas RA (1998) Myocardial protection for neonates and
hypertension after operations for congenital heart dis-
infants. Thorac Cardiovasc Surg 46(Suppl 2):288–291
ease: analysis of risk factors and management. J Thorac
Marciletti C, McGoon DC, Mair DD (1976) The natural his-
Cardiovasc Surg 112:1600–1607
tory of truncus arteriosus. Circulation 54:108–111
Barbero-Marcial M, Riso A, Atik E et al. (1990) A technique for
Pexieder T (1995) The conotrucus and its septation at the
correction of truncus arteriosus types I and II without extra-
advent of the molecular biology era. In: Clark EB,
cardiac conduits. J Thorac Cardiovasc Surg 99:364–369
Markwald RR, Takao A (eds) Developmental mecha-
Calder L, Van Praagh R, Van Praagh S et al. (1976) Truncus
nisms of heart disease. Futura Publishing Co, Armonk,
arteriosus communis. Clinical, angiographic and patho-
pp 227–248
logic findings in 100 patients. Am Heart J 92:23–38
Sano S, Brawn WJ, Mee RB (1990) Repair of truncus arteriosus
Chen JM, Glickstein JS, Davies RR et al. (2005) The effect of
and interrupted aortic arch. J Cardiac Surg 5:157–162
repair technique on postoperative right-sided
Suzuki A, Sy H, Anderson RH, Deanfield JE (1989) Coronary
obstruction in patients with truncus arteriosus.
arterial and sinusal anatomy in hearts with a common
J Thorac Cardiovasc Surg 129:559–568
arterial trunk. Ann Thorac Surg 48:792–797
Collet RW, Edwards JE (1949) Persistent truncus arteriosus:
Van Praagh R, Van Praagh S (1965) The anatomy of com-
a classification according to anatomic types. Surg Clin
mon aorticopulmonary trunk (truncus arteriosus com-
North Am 29:1245
munis) and its embryonic implications. A study of 57
Danton MH, Barron DJ, Stumper O et al. (2001) Repair of
necroscopy cases. Am J Cardiol 16:406–426
truncus arteriosus: a considered approach to right ven-
607 19
Congenitally Corrected
Transposition
and D-Transposition
of the Great Arteries
Sabine H. Daebritz and Michel Ilbawi
References – 636
Chapter 19 · Congenitally Corrected Transposition and D-Transposition of the Great Arteries
609 19
19.1 Congenitally Corrected mal»] looping; and D, dextroposition of the aorta,
Transposition of the Great respectively) (Van Praagh 1992; Van Praagh and
Arteries (ccTGA) Van Praagh 1966, 1967; see also below, 7 Sect. 19.2.3,
«Embryology and Anatomy»). CcTGA can present
19.1.1 Pathology without other intracardiac malformations (1–2 %;
Allwork et al. 1976). Associated cardiac malforma-
Congenitally corrected transposition of the great tions as ventricular septal defects (VSD), valvar and
arteries (ccTGA) is a rare cardiac malformation subvalvar pulmonary stenosis, as well as dysplasia of
with an incidence of 0.5 % of congenital heart the tricuspid valve are frequently present. The
defects (CHD). Morphologically, it consists of abnormal location of the conduction system predis-
atrioventricular as well as ventriculoarterial discor- poses to rhythm disturbances.
dance. The systemic venous, morphologically the Most patients with ccTGA have situs solitus
right atrium, is connected to the morphologically with levocardia. The morphologic right ventricle
left ventricle via the mitral valve; the left ventricle (mRV) is left sided; the morphologic left ventricle
in turn is connected to the pulmonary valve and (mLV) is right sided (L-loop). In about 25 % of
the pulmonary artery. On the other hand, the pul- cases, the lesion is associated with dextro- or
monary venous atrium (morphologically left) mesocardia. In situs inversus (about 5 % of cases),
drains into the morphologically right ventricle via there is a mirror-image anatomy with the apex of
the tricuspid valve. Thus, the right ventricle func- the heart pointing to the right side, which has to
tions as systemic ventricle and ejects via the aortic be distinguished from isolated dextrocardia in
valve into the aorta. This double discordance results situs solitus. In situs solitus and ccTGA, the aorta
in a physiologically corrected circulation whereby is left and anterior to the posteriorly positioned
the unoxygenated blood flows to the lungs and the pulmonary trunk (L-malposition). In situs inver-
oxygenated blood to the body (. Fig. 19.1). sus and ccTGA, the aorta is positioned right and
In most of the cases of ccTGA, there is situs sol- anterior to the pulmonary trunk (I, D, D).
itus with a segmental {SLL-} anatomy (S, situs soli-
tus; L, levo, left-sided looping of the cardiac tube; 19.1.1.1 entricular Septal Defect
V
and L, left-sided malposition of the aorta at the level (VSD)
of the aortic valve, respectively). Less frequently, A VSD is the most common associated cardiac
there is situs inversus with segmental {IDD-} anat- malformation in ccTGA and is present in about
omy (I, situs inversus; D, dextro [right sided, «nor- 70 % of all patients with ccTGA (Warnes 2006).
a b
Ao
Ao PA LA
PA
LA
Tricuspid valve
RA
Systemic ventricle,
anatomical
RA right ventricle
LV
Mitral valve
RV
Subpulmonic ventricle,
anatomical left ventricle
..Fig. 19.1 a, normal heart; b, Congenitally corrected transposition of the great arteries (ccTGA). Ao aorta, LA left
atrium, LV left ventricle, PA pulmonary artery, RA right atrium, RV right ventricle
610 S.H. Daebritz and M. Ilbawi
Although a VSD can take up most of the ventricu- by subvalvar as well as valvar components. A sub-
lar septum, there is typically a single, nonrestric- valvar stenosis can additionally be due to accessory
tive perimembranous VSD of at least moderate mitral valve tissue or fibrotic tissue resulting in a
size, i.e., has no pressure gradient across it. The diffuse hypoplastic fibromuscular tunnel. If a VSD
VSD may extend into the inlet or into the infun- is present, accessory valvar tissue may prolapse
dibular septum. Multiple VSDs can be present. A from the systemic ventricle through the defect into
big, subaortic VSD reaching into the perimembra- the LV outflow tract or the pulmonary valve.
nous septum is frequently associated with a sig- Abnormal chordal attachments onto the crest of the
nificant pulmonary stenosis or with pulmonary septum can also be present. At the level of the pul-
atresia. Rarely, there is an atrioventricular septal monary valve, morphologic obstruction is caused
defect (AVSD) with or without a (subpulmonary) by fused and/or thickened dysplastic valve leaflets.
left ventricular outflow tract obstruction (LVOTO). In some cases there is even pulmonary valve atresia.
and ventricular dysfunction is supported by the dence of mRV dysfunction was 32 % of 65 patients
observation that an increase in LV pressure fol- older than 18 years with ccTGA; 55 patients had
lowing pulmonary artery banding shifts the inter- undergone conventional repair (Piran et al. 2002).
ventricular septum towards the RV and leads to a Development of progressive right ventricular
decrease in tricuspid regurgitation (van Son et al. dysfunction with progressive increase of tricuspid
1996; Daebritz et al. 2001). regurgitation after classic repair, led to treatment
There is an increasing incidence of progressive strategies that aim at incorporating the mLV into the
deterioration of right ventricular function and systemic circulation. The recruitment of the mLV
failure with increasing age. In the first five decades, into the systemic circulation is achieved by rerouting
56 % of all patients with ccTGA and associated of the pulmonary venous and the systemic venous
major defects have moderate to severe deteriora- blood return by an atrial switch procedure (Senning
tion of right ventricular function. This late pre- or Mustard operation) and rerouting of the arterial
sentation could also occur in 32 % of patients connection by an arterial switch operation or a
without significant associated defects (Graham Rastelli operation. These so- called double-switch
et al. 2000). It is due primarily to lack of natural operations (Ilbawi et al. 2002) result in anatomic
anatomic adaptation of the mRV to the high- repair, creating atrioventricular and ventriculoarte-
pressure systemic circulation, exaggerated by lim- rial concordance (see below). The major advantage
ited coronary reserve to the right ventricular of this strategy is the immediate decompression of
myocardium due to single coronary artery supply the mRV, which becomes the low-pressure pulmo-
(Hornung et al. 1998; Hauser et al. 2003) or by nary ventricle. The decompression results in shifting
complete AV block causing an increase in stroke of the ventricular septum towards the mRV and thus
volume and secondary dilatation of the tricuspid reduces any tricuspid regurgitation, making valve
valve anulus (Voskuil et al. 1999; van Son et al. repair or replacement unnecessary.
1995). In case of dysplasia of the tricuspid valve or
Ebstein-like malformation, the onset of mRV fail-
ure can be accelerated due to the mechanisms and 19.1.3 Diagnostics
interactions described above.
Progression of preexisting regurgitation or the Prenatal or postnatal diagnosis of ccTGA is usually
onset of new one can occur after traditional physi- made by echocardiography (Allan et al. 1994).
ologic repair (VSD closure, relief of LVOT) and is Additional diagnostic details may be obtained by
reported in 42 % of postoperative patients (Hraska transesophageal echocardiography. Advanced non-
et al. 2005). The decrease of the mLV pressure after invasive diagnostic tools, such as 3D echocardiog-
physiologic correction leads to a shift of the sep- raphy, 3D CT scan, and 3D MRI, are helpful in
tum to the left towards the mLV with consequent selected more complex cases. Cardiac catheteriza-
increase in tricuspid regurgitation and volume tion gives precise assessment of hemodynamics,
overload of the mRV. Therefore, tricuspid valvulo- such as the degree of pulmonary stenosis, shunt
plasty after classic repair is not an optimal solution volumes of one, or more VSDs (Qp/Qs), and
because it is either technically not feasible or is enables calculation of the pulmonary vascular
likely (van Son et al. 1995) to fail if the right ven- resistance. If an anatomical correction is planned,
tricle remains as systemic ventricle (Acar et al. coronary arteriography is helpful, especially in redo
1998). Although the results of primary tricuspid surgery, but not absolutely necessary. Measurement
valve replacement have improved, particularly if of the mRV and mLV pressures and assessment of
surgery is performed early at the onset of first signs mLV muscle mass has to be performed in all
of mRV dysfunction (van Son et al. 1995), they are patients in whom pulmonary artery banding for
19 still not satisfactory, because valve replacement retraining or anatomical correction is planned.
does not address progressive mRV dysfunction. In
a series of 14 patients with initial tricuspid valve
replacement (Hraska et al. 2005), the 1- and 5-year 19.1.4 Surgical Treatment Strategies
survival rates were 71 and 53 %; the 1 and 5 years
rate of freedom from repeat valve replacement Until the end of the 1980s, the surgical treatment
were 100 and 40 %. Eleven of these patients (79 %) strategies of ccTGA were limited to the repair of
had dysfunction of the mRV. The reported inci- associated defects, resulting in a classic or physio-
Chapter 19 · Congenitally Corrected Transposition and D-Transposition of the Great Arteries
613 19
logic repair. Thereafter, treatment options incorpo-
and to prevent from development of pul-
rating the mLV into the systemic circulation, monary vascular obstructive disease. In this
so-called the anatomical correction, were intro- group of patients, the band is tightened until
duced due to disappointing long-term results of thethe pulmonary artery pressure drops to about
physiologic repair (see 7 Sect. 19.1.2, «Clinical Pre-
one-third of the systemic pressure. It is also
sentation and Natural History») (Ilbawi et al. 1990).
used in patients who are destined for double-
The classic repair, in which the mRV remains as switch operation (i.e., atrial and arterial switch
systemic ventricle, carries the risk of unpredictable
or atrial switch plus Rastelli). Another indica-
onset and progression of mRV dysfunction. In con- tion for banding is the retraining of the mLV
trast, anatomic correction carries the potential dis-
in older patients with low mLV pressure,
advantages of the venous switch operation. who are planned for an anatomical correc-
Currently, the anatomic repair has become the tion. Pulmonary artery banding could also be
standard approach in spite of absence of long-term considered a definitive palliation in selected
results proving its advantages. . Fig 19.2 gives apatients with unfavorable anatomy, to establish
decision algorithm of the currently applied criteria
«balanced» circulation as seen in some patients
for surgical treatment of ccTGA. with valvar or subvalvar pulmonary stenosis
(naturally banded) who have a very good natu-
19.1.4.1 Palliative Procedures ral palliation without any intervention (Hraska
Depending on morphology and hemodynamics, et al. 2005).
an initial palliative procedure such as systemic- In retraining of the left ventricle and PAB,
to-pulmonary shunt or pulmonary artery band- the PAB is tightened to achieve mLV pres-
ing may be necessary. sure of 80–100 % of the systemic pressure.
Frequently, a repeat stepwise banding is nec-
kModified Blalock–Taussig-Shunt essary because the left ventricle may not ini-
Newborns with severe pulmonary artery stenosis tially tolerate tight banding. These patients are
or pulmonary atresia (ductal dependant pulmo- usually sick and require prolonged stay in the
nary circulation) need early palliation with intensive care unit for ventilatory and hemo-
systemic-to-pulmonary artery shunt, to allow for dynamic stabilization. This is due to the fact
growth of the patient making physiology or ana- that the response of the mLV to acute increase
tomic repair (Senning/Mustard operation, in afterload and volume shifting through
Rastelli) easier to perform. It is also needed in an intracardiac shunt is unpredictable and
newborns in whom a septation or rerouting of the is dependent on the age of the patients and
ventricles cannot be performed (i.e., huge VSD, other perioperative variables that could lead to
noncommitted VSD) and thus are destined for short- and long-term mLV dysfunction (Quinn
eventual Fontan palliation. et al. 2008).
The insertion of a modified Blalock–Taussig The common approach to perform PAB is via
shunt is performed via median sternotomy. The median sternotomy. We use a 4-mm polytetra-
graft is preferably a thin-walled 3.5–4 mm fluoroethylene band and adjust it by serial pul-
polytetrafluoroethylene stretch graft, which is monary artery pressure measurements proximal
interposed between the proximal part of the right and distal to the band. Transesophageal echo-
subclavian or distal innominate artery and the cardiogram is used to evaluate mLV function at
right or main pulmonary artery. The use of car- the time of banding. An externally adjustable
diopulmonary bypass machine is usually not banding device might be helpful particularly
needed unless the patient becomes hemodynam- with regard to longterm stepwise training occa-
ically unstable when the pulmonary artery is par- sionally needed in older children, although its
tially clamped. use has been discouraging and has not been
widely accepted (Bonnet et al. 2004; Daebritz
kBanding of the pulmonary artery et al. 1999). Recently, a newer adjustable band
Pulmonary artery banding (PAB) is performed has been applied effectively for stepwise banding
in patients with a large VSD and pulmonary in 11 patients with ccTGA (DiBardino et al.
overcirculation, to allow some somatic growth 2012).
614 S.H. Daebritz and M. Ilbawi
ccTGA
TVregurg?
No acute consequence
Associated defects Right ventricular
Clinical follow up
dysfunction
Not septable heart TV-dysplasia VSD Pulmonary stenosis Age <15-18 years TVrec/TVR
mLV-training (PAB) + +
Higher grade TVregurg Non-restrictive VSD? mLV-training (PAB)
reduction of TVregurg PS? VSD? HTX
reduction of TVregurg
Severe PS?
HTX PAB PA/VSD? Mild to moderate PS
Fontan-palliation
Double Switch Cyanosis? Balanced state
once PAB is outgrown Neonatal mBTS
Resection of PS,
VSD closure
..Fig. 19.2 Algorithm for the surgical management of congenitally corrected transposition of the great arteries. Hori-
zontal arrow l No; vertical arrow l Yes; ccTGA congenitally corrected transposition of the great arteries, HTX cardiac trans-
plantation, mBTS modified Blalock–Taussig shunt, mLV morphologic left ventricle, PA pulmonary atresia, PAB pulmonary
artery banding, PS pulmonary stenosis, TVregurg tricuspid valve regurgitation, TV tricuspid valve, TVR tricuspid valve
replacement, TVrec tricuspid valve reconstruction, VSD ventricular septal defect
because the closure in anatomic repair is done on may be due to inadequate coronary perfusion
the right ventricular side of the septum. The tech- caused by the sudden increase of the afterload or
nique of implantation of pacemaker systems is by the induced hypertrophy. The reduced coro-
extensively described in see 7 Chapter «Device nary reserve in older patients compared to young
Therapy of Rhythm Disorders», Sect. 30.3. It has to be children may lead to greater ischemic damage of
kept in mind that pacemaker wires with screws the myocardium following the sudden increase in
have to be used if they are inserted into the mor- afterload and consequently impaired short- and
phologically left ventricle because it lacks the tra- long-term ventricular function (Poirier et al. 2004;
beculation which normally holds the wires in place. Quinn et al. 2008). However, training of the left
In children with a body weight <15 kg, a DDD ventricle already in early infancy, as performed in
system with epimyocardial wires and an epigas- the «rapid two-stage switch operation,» is also
tric battery implantation onto the posterior fascia associated with a long-term impairment of left
of the rectus muscle is recommended. Over 15 kg ventricular function (see 7 Sect. 19.2.5.3, «Choice of
of body weight, thin, transvenous electrodes the Operative Procedure»). Another unanswered
implanted through the right subclavian vein may question is how fast should the pulmonary artery
prevent thrombosis of the left innominate vein. be banded. It seems that a multistage slow tighten-
The battery is placed under the right pectoral ing of the band to appropriate pressure levels is
muscle on the thoracic wall. better than a one-stage very tight initial banding. It
allows healthier left ventricular hypertrophy and
19.1.4.3 Retraining the Left Ventricle prepares the left ventricle better for short- and
for Anatomic Repair long-term performance. The measurement of left
Following physiologic repair of ccTGA, progressive ventricle mass by MRI is a reliable way to judge the
failure of the systemic right ventricle may occur preparedness of the left ventricle (Duncan et al.
with or without tricuspid valve regurgitation. 2003; Langley et al. 2003; Poirier et al. 2004).
Retraining of the pulmonary LV to become a sys-
temic ventricle and subsequent anatomic correction 19.1.4.4 Anatomical Correction
has been tried in D-TGA with RV failure following In the physiologic repair, the emphasis of the treat-
venous switch operation (Daebritz et al. 2001; ment strategies is placed on the management of the
Mavroudis and Backer 2000; Mee 1986; Poirier and associated malformation and their complications.
Mee 2000; Poirier et al. 2004; Quinn et al. 2008; The mRV and the tricuspid valve remain in the sys-
Brawn et al. 2008). The concept is based on progres- temic circulation. Disappointment with the long-
sive tightening of PAB that gradually allows the left term performance of the right ventricle and tricuspid
ventricle to acquire the necessary muscle mass, pos- valve as systemic structures and resultant heart fail-
terior wall thickness, and normal geometry of its ure led to the introduction of anatomic repair which
cavity as well as of the interventricular septum. incorporates the left ventricle and mitral valve into
Normalizing the septal orientation, namely, bulging the systemic circulation. The systemic venous blood
towards the right ventricle improves the function of is rerouted into the mRV, and the pulmonary venous
the tricuspid valve and right ventricle. blood is rerouted into the mLV by a venous switch
Which patients are suitable for a retraining of operation (Senning or Mustard operation) (see
the left ventricle for anatomical repair is still largely 7 Sect. 19.2.5.1, «Atrial Switch Operation»). In patients
an unanswered question due to the limited num- without pulmonary outflow obstruction (without
ber of cases reported. One major issue is to what LVOTO), the ventriculoarterial discordance is coun-
age a successful retraining of the left ventricle is teracted by an additional arterial switch operation.
still possible. Most series suggest the left ventricle On the other hand, the venous switch operation has
19 loses its adaptability to become a systemic ventri- to be completed by a Rastelli operation in those
cle by 10–15 years of age (Mavroudis and Backer patients with LVOTO, pulmonary stenosis or atresia,
2000; Poirier and Mee 2000), although there are and VSD. Both variations comprise the so-called
occasional case reports of successful retraining of double-switch operation or the Ilbawi operation
the mLV in patients up to early adulthood (Ilbawi et al. 1990; Yagihara et al. 1994). The surgical
(23 years) (Padalino et al. 2000). The age limitation techniques are described in detail in 7 Sect. 19.2.5,
may be due to potential damage of the ventricle by «Surgical Management», and Sect. 19.2.6.4,
the PAB after a certain age. Although the mecha- «Transposition of the Great Arteries with Left Ventricular
nism is unclear, failure of the mLV after retraining Outflow Tract Obstruction» .
Chapter 19 · Congenitally Corrected Transposition and D-Transposition of the Great Arteries
617 19
Although there are no absolute contraindica- tional Glenn leading to a hemi-Mustard procedure
tions for the anatomical repair of ccTGA, certain has also been advocated as part of the double-
morphologic features are regarded as relative con- switch operation in order to assist right heart
traindications. In candidates for a Senning/ hemodynamics. Its benefits also include improved
Mustard–Rastelli operation, the size and location conduit life, reduced venous obstruction, decreased
of the VSD can limit the feasibility of connecting sinus node dysfunction, and technical simplicity
the left ventricle via the VSD to the aorta. Thus, a (Malhotra et al. 2011) (. Figs. 19.3 and 19.4).
Senning/Mustard–Rastelli operation cannot be Patients with coronary anomalies such as
performed successfully if the VSD is very distant to single or intra-aortomural coronary artery with
the aortic valve in the muscular or apical septum. or without signs of myocardial ischemia, as well
In patients with small VSD, enlargement of the as those with subsystemic LV pressure and those
defect to accommodate left ventricular stroke vol- with atrial arrhythmias, are considered subopti-
ume without obstruction has resulted in ventricu- mal candidates for anatomic correction (Langley
lar dysfunction and increased incidence of et al. 2003; Poirier et al. 2004; Mee 2005).
complete AV block (Ilbawi et al. 2002; Shin’oka
et al. 2007). Another relative contraindication is 19.1.4.5 Alternative Procedures
the insertion of chordae of one or both AV valves In patients, in whom biventricular repair is not
on the crest of the VSD or at the infundibular sep- possible due to a straddling AV valve, hypoplasia
tum, whereby tunneling of the mLV via the VSD to of one ventricle, or a difficult anatomy of one or
the aorta may disturb the functional integrity of more VSDs, the Fontan palliation is an alternative
one or both AV valves. Severe hypoplasia of the surgical approach (see 7 Chapter «Definite Palliation
mRV or the mLV makes the patients candidates for of Functional Single Ventricle», Sect. 13.6). Compared
a Fontan palliation. In mild hypoplasia of the mRV, with traditional physiologic repair, the Fontan
a «double-switch» operation, in combination with palliation provides better midterm results in this
bidirectional pulsatile Glenn procedure, 1½ ven- subgroup of patients. This may be due to the fact
tricle repair is recommended. The use of bidirec- that in these cases, the left ventricle remains in the
IVC RV
IVC
RV
LV
LV
systemic circulation providing additional energy 50 % at 10 years of follow-up. The major risk factor
source. Another alternative is 1½ ventricle repair, for long-term poor outcome of physiologic tradi-
in which a limited relief of LVOTO is combined tional repair was preexisting tricuspid valve regurgi-
with a pulsatile bidirectional Glenn anastomosis tation (Hraska et al. 2005; Shin’oka et al. 2007).
(Mavroudis et al. 1999). In this strategy, it is Survival rate of patients with preoperatively com-
important to keep the pressure in the mLV high, petent tricuspid valve was 72 % at 30 years.
but subsystemic to prevent septal shift towards
the mLV and consequent tricuspid regurgitation 19.1.5.2 Anatomical Correction
and progressive deterioration of mRV function. The discouraging long-term results of physiologic
This strategy may be used in combination with a repair and the progressive failure of the mRV in
Rastelli operation for patients who are not suit- the systemic circulation have prompted the adop-
able for an atrial switch due to the location and tion of anatomic repair as the treatment of choice.
size of the VSD or abnormal subvalvar attach- Excluding the learning curve results, short- and
ments to the crest of the septum. A better alterna- midterm outcome of anatomic repair has been excel-
tive in these patients is aortic translocation (see lent (Devaney et al. 2003; Duncan et al. 2003; Imai
7 Sect. 19.2.6.4, «Nikaidoh Operation»). The last et al. 1994; Imamura et al. 2000; Ilbawi et al. 2002;
treatment option with or without prior surgery is Langley et al. 2003; Shin’oka et al. 2007; Hiramatsu
orthotropic heart transplantation (see 7 Chapter et al. 2012). The operative mortality is reported as
«Heart and Heart-Lung transplantation», Sect. low as 0–7 % (Duncan et al. 2003, 46 patients, 26
37.1.5.2). However, heart donor shortage, particu- «double- switch» operations, 20 Senning–Rastelli
larly in neonates and children, and the morbidity procedures; Karl et al. 1997, 14 patients, all «double-
of lifelong immunosuppression have to be taken switch» operations; Ilbawi et al. 2002, Langley et al.
into account when deciding on optimal treatment 2003, 54 patients, 29 «double-switch» operations,
strategy for each individual patient. 22 Senning–Rastelli procedures, 3 Senning tun-
nel operations; Hiramatsu et al. 2012, 90 patients,
72 atrial switch–Rastelli procedures, 18 «double-
19.1.5 Results switch» operations). Hiramatsu reported a follow-
up of 90 patients with double-switch operation for
19.1.5.1 Physiologic Repair congenitally corrected transposition. Survival was
There are many reported results of classical physi- 75.7 % for patients with atrial switch and intracar-
ologic repair of ccTGA. They show a low early diac rerouting (Rastelli operation) and 83.3 % when
mortality and consistently emphasize the problem a combined arterial and atrial switch were used for
of progressive failure of the mRV in the systemic those with double switch at 20 years postopera-
circulation (Acar et al. 1998; Graham et al. 2000; tively (Hiramatsu et al. 2012). Langley et al. (2003)
Ilbawi et al. 2002; Shin’oka et al. 2007; van Son et al. reported a series of 54 patients with a 5- and 9-year
1995; Voskuil et al. 1999; Yeh et al. 1999). The larg- survival of 94.4 %, 89.7 %, respectively, and demon-
est series published (Hraska et al. 2005) reports 123 strated reduction of tricuspid valve regurgitation by
patients with physiologic repair. Survival after 1, 5, the shift of the interventricular septum to the mRV
10, and 15 years was 84 %, 75 %, 68 %, and 61 %, after anatomical correction. Similar findings were
respectively. Patients with preoperative tricuspid reported by others. None of the patients had to be
regurgitation had the worst outcome. A dysfunc- reoperated for tricuspid regurgitation (Devaney
tion of the mRV was found in 44 % of all patients. et al. 2003; Duncan et al. 2003; Imamura et al.
Freedom from mRV dysfunction after 5, 10, 15, 2000; Langley et al. 2003; Shin’oka et al. 2007). The
and 20 years (after physiologic repair) was 88 %, incidence of post-op complete AV block is quite
19 73 %, 57 %, 43 %, and 39 %, respectively. variable (2–5 %) and is lower following anatomic
Dysfunction of the tricuspid valve was present in corrections, but increased when aggressive enlarge-
42 % of the patients on follow-up, and freedom ment of the VSD is needed to prevent potential sub-
from development of tricuspid valve dysfunction aortic stenosis in venous switch/Rastelli operation
after 5, 10, 15, and 20 years was 91 %, 69 %, 52 %, (Shin’oka et al. 2007).
42 %, and 37 %, respectively. Sano et al. (1995) New onset of left ventricular dysfunction has
reported a mortality rate of 17 %, significant tricus- been reported post anatomic repair. The exact etiol-
pid regurgitation of 71 %, and mRV dysfunction of ogy is not clear but could be related to long cross-
Chapter 19 · Congenitally Corrected Transposition and D-Transposition of the Great Arteries
619 19
clamp time, inadequate myocardial protection, pulmonary circulations such as those with a VSD
injury to septal perforators, other coronaries, sub- and moderate pulmonary stenosis can be followed
aortic stenosis, or suboptimal coronary arteries without intervention as long as they are free of
translocation (Langley et al. 2003; Duncan et al. clinical symptoms and do not develop mRV dys-
2003; Imamura et al. 2000; Shin’oka et al. 2007). function, although such conservative approach is
Also, moderate regurgitation of the neoaortic valve not recommended any more. Patients with ccTGA
can occur following double-switch operation and without associated defects can also be observed
may be related to pulmonary artery banding caus- without intervention. A good alternative, how-
ing proximal pulmonary trunk dilatation and dis- ever, is to apply a loose PAB in the neonatal period
ruption of the sinotubular bar (Langley et al. 2003) to keep the pressure in the mLV elevated to pre-
Occasional neoaortic regurgitation may occur with- serve septal geometry and keep LV trained to per-
out prior banding (Quinn et al. 2008). Restoration form as systemic ventricle in case anatomic repair
of sinotubular bar and anuloplasty using a double is needed at an older age. Long-term results of this
suture line at the anular level can be used to mini- aggressive approach are not available yet.
mize the incidence of regurgitation and consequent
left ventricular failure. The incidence of complica-
tions related to the venous switch operation such as 19.2 Dextro-(D-)Transposition
arrhythmias and venous obstruction has decreased of the Great Arteries (D-TGA)
markedly following the introduction of the modifi-
cations with hemi Mustard by Frank Hanley’s group 19.2.1 Epidemiology
(Malhotra et al. 2011) (. Figs. 19.3 and 19.4).
D-TGA is the second most common cyanotic car-
diac malformation after tetralogy of Fallot. It has
19.1.6 Summary an incidence of around 10 % of all congenital
heart malformations which occur in about 8/1,000
The current surgical strategy of choice in ccTGA live births (Sellke et al. 2005). The overall ratio of
is the anatomical correction/repair. However, male to female newborns with TGA is 2:1 and is
long-term data are still limited to small series, so increased to 3:1 in D-TGA with intact ventricular
there remains uncertainty with regard to the per- septum, «simple» TGA (Fyler 1980).
formance of the mLV in the systemic circulation,
to the development of neoaortic valve regurgita-
tion, and to the potential complications of venous 19.2.2 Embryology and Pathology
switch operation, although the later has decreased
markedly with recent modifications. Risk factors In normal hearts, the aorta is posterior and to the
such as RV dysfunction and tricuspid regurgita- right, and the pulmonary artery is anterior and to
tion favor strongly anatomic repair. Certain sub- the left (N position of Van Praagh). In transposi-
groups of patients, however, are not suitable for tion or malposition, the aorta is anterior to the
anatomical correction. These include older pulmonary artery at the semilunar valve levels. If
patients, in whom a retraining of the mLV cannot the aorta is anterior and to the right, it is
be performed successfully. These patients are still D-transposition of the great arteries
candidates for a classical repair or heart transplan- (D-malposition of Van Praagh); if it is anterior
tation. If the physiologic repair is deemed neces- and to the left, the transposition is L-transposition
sary, it is advantageous to aim at leaving mLV of the great arteries (L-transposition of Van
pressure at least half of the systemic pressure, to Praagh) regardless of the relationship of the great
preserve the geometry of the mRV and interven- arteries to the ventricles they arise from.
tricular septum. In younger patients, in whom D-TGA is considered a conotruncal malfor-
anatomical correction is contraindicated because mation, i.e., a malformation in which the outflow
of morphological features, i.e., straddling of an AV tracts of both ventricles and their respective semi-
valve, an unfavorite location of the VSD or a hypo- lunar valves are affected. The aorta and the aortic
plastic ventricle, the Fontan palliation is an alter- valve arise from the mRV. There is a subaortic
native with good results. Asymptomatic patients muscular conal septum separating the aortic valve
with associated defects but balanced systemic and from the tricuspid valve. The pulmonary artery
620 S.H. Daebritz and M. Ilbawi
and the pulmonary valve arise from the mLV, and ence of a VSD increases the probability of further
there is a fibrous continuity between the mitral associated malformations. If a VSD is hemody-
valve and the pulmonary valve (no conus), a rela- namically significant, the pulmonary artery is
tion which is normally present with the aortic larger in diameter compared to the aorta, and the
valve. Normally, the pulmonary valve is the only ratio can reach 3:1. RVOT obstructions can be
valve, which is not in fibrous continuity with the extensive and tunnel-like. Multilevel of obstruc-
other cardiac valves, because it is separated by the tions of the systemic (aortic) outflow with hypo-
conal septum. In TGA this applies to the aortic plasia of the aortic arch or coarctation of the aorta
valve. The great vessels are almost anterior–poste- is more frequent in TGA with VSD compared to
rior to each other, and the pulmonary artery is simple TGA and may be associated with hypo-
larger in diameter. Frequently, the commissures of plastic right ventricle at the extreme end of the
the two outflow valves are facing each other, and spectrum precluding biventricular repair in some
the coronary arteries arise laterally and posteriorly cases. The presence of interrupted aortic arch is
on the right and left side of the aortic root from the also possible, but rare (see, 7 Sect. 19.2.6.5,
facing sinuses. These abnormalities are due to lack «Transposition with Right Ventricular Outflow
of absorption of the subaortic conus prenatally Obstruction and Hypoplasia of the Aortic Arch or
leaving the aortic valve and aorta anteriorly. Coarctation of the Aorta»).
As a result, there is atrioventricular concor- The coronary arteries have abnormal patterns
dance and ventriculoarterial discordance due to in 25–30 % of patients with TGA. These abnormal-
the normal position of the RV and abnormal ante- ities are more frequently observed in patients with
rior position of the aorta. Using the segmental associated VSD or double-outlet right ventricle
nomenclature of Van Praagh and Van Praagh (DORV) and side-to-side position of the great ves-
(1966), simple D-transposition is described as (S, sels (see 7 Sect. 19.2.6.6, «Taussig-Bing Anomaly»;
D, D), with S being situs solitus (normal atrial Gittenberger-de Groot et al. 1983; Sim et al. 1994).
position), D dextro (normal) looping of ventri- There are several classifications of the coro-
cles, and D dextro position of great arteries. nary morphology, based on the nomenclature of
Associated malformations are present in 25 % the sinus, from which the coronaries arise. In the
and consist of a VSD with or without obstruction usual coronary pattern, the coronaries come off of
of the left or right ventricular outflow tract, hypo- the respective posterior right and left sinus,
plasia of the aortic arch or coarctation of the aorta whereas the anterior sinus does not give rise to
(see 7 Sect. 19.2.6.5, «Transposition with Right any coronary artery. The axes of the great vessels
Ventricular Outflow Obstruction and Hypoplasia of and the coronary arteries are in a right angle. The
the Aortic Arch or Coarctation of the Aorta»), and most common anomaly is a circumflex artery
coronary artery abnormalities. arising from the right coronary artery out of the
Anatomic LVOTO is rare in simple TGA, right posterior sinus. In these cases the circumflex
TGA with an intact ventricular septum. Often, a artery runs behind the two great vessels into its
subvalvar LVOTO is just a functional obstruction supply area. In cases with single coronary artery,
caused by bulging of the ventricular septum to both right and left coronary arteries arise from a
the left. It decreases after anatomical correction single ostium. Frequently there is an associated
due to the decrease in right ventricular pressure small additional ostium that gives rise to a conal
and resultant septal shift towards the right ven- branch. Only very rarely, there is no second
tricle. This form of LVOTO is not a contraindica- ostium at all. Another very important variation of
tion to arterial switch operation. The pulmonary coronary anatomy is an intramural course of a
valve can be abnormal. Rarely, it is dysplastic; coronary artery, present in 2–3 % of cases
19 more frequently it is bicuspid with or without (Gittenberger-de Groot et al. 1986; Sachweh et al.
significant stenosis. The subpulmonary area may 2002) regardless of branching pattern. The intra-
be obstructed with accessory valvar tissue or mural part of the coronary artery characteristi-
abnormal attachment of the mitral valve (as in cally runs the first millimeters within the aortic
Taussig–Bing anomaly). It could also be due to wall and does not have its own separate wall.
fibromuscular ridge or tunnel-like obstruction. Typically, the coronary artery crosses one aortic
VSD in TGA can be located anywhere in the valve commissure within the aortic wall and its
ventricular septum (25 % incidence). The pres- ostium is slanted and beveled. Therefore, the
Chapter 19 · Congenitally Corrected Transposition and D-Transposition of the Great Arteries
621 19
Cx Cx Cx
R R
R
p
L
L
L
1LC×2R 1L2RC× 2RC×L r l
Cx Cx
Cx a
Cx
R
L L L
R R R
L
1C×2RL 1RLC× 1RL2C× 1R2C×L
R
L R
R L
p
Cx
R
Cx
r l
L
L
R a
1RLC× 2LC×R
..Fig. 19.5 Coronary morphology. a Most common pattern of coronary arteries in D-transposition of the great
arteries; b Rare pattern of coronary branching with single ostium. a anterior, Cx circumflex artery, l left, L left anterior
descending artery, p posterior, r right, R right coronary artery
ostium cannot be excised in the regular fashion at rings are the longer systemic and the shorter
the time of the arterial switch operation, because pulmonary circulations. The two circulations
of the common wall of the aorta and the coronary are in series. In contrast, in D-TGA, pulmo-
in the area of excision of the button. The varia- nary and systemic circulations are in parallel;
tions of coronary anatomy are depicted in the oxygenated blood recirculates in the pul-
. Fig. 19.5. monary vessels, and the deoxygenated blood
recirculates in the systemic vessels. Survival
after birth is possible only if there is mixing of
19.2.3 Physiology blood between the two circulations. The mix-
ing can occur at the level of foramen ovale or
The normal circulation resembles the figure of an ASD, a persistent ductus arteriosus, or a
8: The heart is in the cross middle and the VSD (. Fig. 19.6 ). The mixture of the blood
622 S.H. Daebritz and M. Ilbawi
through these shunts usually ensures adequate pressure leads to a shift of the ventricular septum
arterial oxygen saturation. The most important towards the mLV, a phenomenon that becomes
shunt is the one on the atrial level. If this is too apparent echocardiographically by the fourth
small, the atrial connection can be enlarged by week of life. This natural decrease in muscle mass
a balloon atrioseptostomy. This can be done of the LV is important for timing of the arterial
without fluoroscopy but under echocardio- but not the atrial switch operation.
graphic control. Usually, an ASD ensures ade-
quate mixing and survival for the first year of
life. Very rarely, adequate mixing of oxygen- 19.2.4 Diagnostics
ated and unoxygenated blood does not occur
in spite of wide open atrial communication, Diagnoses of D-TGA can be established prena-
and maintenance of open ductus arteriosus tally by the characteristic absence of crossing of
with PGE1 might be needed. In the era of the the great arteries on fetal echocardiogram and
atrial switch operation, i.e., the Sennning or the origin of the aorta from the anterior right
Mustard operations, children with D-TGA ventricle. After birth there is no typical heart
were operated on between 3 months and 1 year murmur, and the cyanosis can be mild due to
of age, surviving until the procedure with just adequate mixing while the ductus is still open.
an ASD. Rarely, in the so-called nonmixers, Newborns present with unspecific clinical symp-
urgent arterial switch operation may be indi- toms as tachypnea, failure to thrive, and cyanosis
cated. while feeding. Associated, noncardiac malfor-
At birth, patients with D-TGA with or without mations are extremely rare in simple D-TGA. The
a VSD have systemic pressures in both ventricles diagnosis is typically made by echocardiography.
because of the elevated pulmonary vascular resis- Cardiac catheterization is usually not necessary,
19 tance of fetal circulation. Both right and left ven- neither in simple D-TGA nor in D-TGA with
tricles, therefore, are adapted to pump blood VSD even in patients with unclear coronary
against systemic vascular resistance. In the neona- artery anatomy.
tal period, the right ventricle maintains muscular Today’s surgical therapy is always the arterial
hypertrophy, while the left ventricle gradually switch operation, for which no additional preop-
loses muscle mass as the pulmonary arterial resis- erative diagnostics besides echocardiography are
tance decreases. The resulting decrease in LV necessary.
Chapter 19 · Congenitally Corrected Transposition and D-Transposition of the Great Arteries
623 19
Coronary anomalies suspected but not diag- done in the neonatal period or could be delayed
nosed preoperatively can be thoroughly evaluated until the child is older as long as the oxygen sat-
and managed at the time of surgery, as they do not uration is satisfactory. Its main advantage is
constitute contraindication to arterial switch opera- avoidance of any patch material. On the other
tion. In case of complex LVOTO or other malfor- hand, it utilizes the atrial septum and right atrial
mations, which potentially necessitate a change of wall to redirect the venous blood (Senning
operative strategy or even a univentricular pathway, 1959). The surgical principle of the Mustard pro-
cardiac catheterization may be indicated, although cedure (Mustard 1964) is to create an inner tun-
in most cases 2 or 3D echo can provide this infor- nel from the ostia of the caval veins to the
mation without the need for cardiac catheterization. posteriorly lying mitral valve and to reroute the
Sometimes, an MRI can add useful informa- pulmonary venous blood around this inner tun-
tion regarding left ventricular mass in cases of nel to the anteriorly positioned tricuspid valve
delayed presentation (after 3 weeks of life). (. Figs. 19.7, 19.8, 19.9, 19.10, 19.11, 19.12, 19.13,
19.14, 19.15, 19.16, 19.17, 19.18, 19.19, and 19.20).
In contrast to the Senning, which utilizes viable
19.2.5 Surgical Management tissue, it uniformally utilizes prosthetic patch
material.
Surgical management of D-TGA has undergone For extracorporeal circulation, the venous
changes over the years. These changes reflect the cannulae are placed into the inferior caval and
current basic principles of surgery for congenital into the innominate vein or preferably in the
malformations, namely, that an early, complete, superior vena cava; the arterial cannula is inserted
and anatomic repair should be performed when- into the ascending aorta. The operation is mostly
ever possible. done using mild to moderate hypothermia with-
Historically, the initial therapeutic steps were out circulatory arrest.
palliative and consisted of an atrioseptostomy In the Senning operation, the aorta is cross-
without the heart and lung machine (Blalock and clamped, and the venous inflow is excluded.
Hanlon 1950) and switching the IVC and RPV Cardiac arrest is maintained with crystalloid or
location using a homograft (Baffes 1956; O’Shea blood cardioplegia. The atrium is dissected
et al. 1983). The development of cardiopulmo- anterior to the orifices of the venae cavae and
nary bypass and its widespread application opened longitudinally. The incision is extended
enabled the introduction of the atrial switch from the anterior wall of the IVC to base of the
operation (Senning 1959; Mustard 1964) and atrial appendage. A band of atrial wall tissue of
later the arterial switch anatomic correction 2–3 cm is created by longitudinal incision of the
(Jatene et al. 1975, 1976). left atrium just anterior to the entry of the right
pulmonary veins. This band of atrial tissue
19.2.5.1 trial Switch Operation
A forms the anterior circumference of the respec-
(Senning and Mustard tive caval veins. The residual atrial septum is cut
Procedures) anteriorly, and the coronary sinus is split open
The two types of atrial switch operations were towards the left atrium. A segment of atrial sep-
first and successfully performed by Senning in tum between the AV valves is left intact. The
1958 and Mustard in 1963. Their principle is to rim of the coronary sinus and the posteriorly
switch the venous inflow to both atria so that the displaced atrial septum is sutured anterior to
unoxygenated blood goes to the LV and pulmo- left pulmonary veins using running monofila-
nary circulation while the oxygenated blood ment sutures. In patients with a deficient atrial
goes to the RV and systemic circulation septum (native ASD or s/p Rashkind), a septum
(. Fig. 19.6). The outcome is a physiologic circu- defect is replaced with a patch of any kind most
lation without the normal anatomical repair, commonly pericardium or Gore-Tex. The roof-
where the right ventricle remains the systemic ing of the left pulmonary veins is completed
ventricle, an anatomy and pathophysiology suturing of the anterior rim of the atrial wall
comparable to the findings in ccTGA or L-TGA band to the remnant of the septal rims between
(see . Fig. 19.1). The Senning procedure can be the two atrioventricular valves.
624 S.H. Daebritz and M. Ilbawi
19
tant to divide any tissue tethering or pulling on After implantation of the coronary arteries,
the pulmonary arteries to allow successful the Lecompte maneuver (Lecompte et al. 1981) is
Lecompte maneuver without pulmonary artery performed, in which the pulmonary bifurcation is
kinking or distortion (see below). After cross- advanced in front of the distal aorta by passing the
clamping of the aorta and achieving cardioplegic aorta posteriorly between the two main pulmo-
arrest, the right atrium is opened and a left heart nary artery branches. The next step is the end-to-
vent is placed via the atrial septal defect or persis- end anastomosis of the ascending aorta with the
tent foramen ovale. The aorta is cut distal to the reconstructed neoaortic root. Discrepancy
sinotubular junction, clearly distal to the coro- between neoaortic root and ascending aortic
nary ostia, and after inspection, the coronary diameter can be adjusted by taking smaller bites
arteries are excised with a wide U-shaped aortic on the distal small aorta. It may be helpful to
wall button. This should be done under direct make two longitudinal incisions at the cut end of
visualization from the inside and the initial cut to the distal aorta to decrease the discrepancy and
be limited to the aortic wall, to decrease potential achieve appropriate orientation. The atrial septum
damage to the coronary arteries especially in case is closed with a running double suture after
of an intramural course. If the coronary ostium is removing the vent; the right atrium is closed in
very close to the commissure, the commissure is the same way. After de-airing, the cross-clamp is
mobilized and an adequate coronary button is removed. The areas of excision of the coronary
harvested. The commissure may be resuspended buttons are patched with fresh or glutaraldehyde-
later during the reconstruction of the neopulmo- fixed autologous pericardium. Augmentation
nary root. with two separate patches is preferred, but a sin-
The coronaries are mobilized for 5–10 mm gle, large pantaloon patch can be used as well.
peripherally. Sometimes it is necessary to sacrifice Other patch materials have not proven of value
a conal branch to gain sufficient mobility. The and have led to stenosis. The reconstructed proxi-
pulmonary artery is cut 2–3 mm proximal to mal neopulmonary artery is then anastomosed
bifurcation. The coronary buttons are approxi- end to end with the distal pulmonary artery using
mated to the neoaorta to select the optimal place 6/0 polydioxane sutures. Polypropylene can be
for reimplantation. In difficult coronary anatomy, used as well. At the end of the procedure, coro-
it is useful to mark the best position for reimplan- nary arteries are inspected for flow, and the myo-
tation prior to division of the great vessels. It is cardium for color. The success of the operation is
important to choose the position on the neoaorta
rather a little distal than too proximal as the risk
of kinking caused by very proximal implantation
is higher than the risk of stretching of the elastic
neonatal coronaries caused by distal placement.
The aorta is pierced with a knife at the selected
position. Care is taken that the cusps of the semi-
lunar valve are away to avoid damage to the valve
(neoaortic valve). The hole is stretched with a fine
instrument and a 4-mm punch is used to achieve
a rounded hole. The implantation is performed
with an absorbable running 7/0 polydioxane
suture or with similar size nonabsorbable mono-
filament suture (Daebritz et al. 2000). Some sur-
19 geons prefer the excision of a U-shaped hole;
others perform a trapdoor plasty, in which no tis-
sue is removed and the hole is created by a wing-
door incision (. Fig. 19.21). The later techniques
are advantageous in some cases because they
allow modifications in the orientation of the coro-
nary artery as needed to obtain optimal angle
especially when the anatomy is complex. ..Fig. 19.21 Trap door technique
Chapter 19 · Congenitally Corrected Transposition and D-Transposition of the Great Arteries
629 19
visible right away by the good filling of the coro-
nary arteries and the pink myocardium of both
ventricles.
In intramural course of a coronary artery
(. Fig. 19.22), the aortic wall has to be excised
beyond the commissure together with the coro-
nary artery. The commissure is detached and
resuspended later to the autologous pericardial
patch used for the reconstruction of the pulmo-
nary trunk. Some authors recommend the split-
ting and unroofing of the intramural part of the
coronary artery (Asou et al. 1994).
Several techniques have been used for single ..Fig. 19.22 Intramural course of right coronary artery,
coronary artery or both coronary arteries arising giving rise for left circumflex artery also
in proximity from the same sinus. These tech-
niques include separation of both coronary ostia arterial switch operation are superior when
(. Fig. 19.23) and separate reimplantation or compared with the atrial switch operation
reverse flap (. Fig. 19.24). Rarely used is the in (Muller et al. 2013). The incidence of obstruc-
situ exclusion technique (. Fig. 19.25). tive pulmonary vascular disease in D-TGA has
markedly decreased over the years due to ear-
kResults lier surgical repair using the anatomic correc-
Hospital mortality in patient with simple tion (Newfeld et al. 1974; Rivenes et al. 1998;
D-TGA, undergoing arterial switch operation, Squarcia and Macchi 2011).
is less than 2 % in most experienced institu-
tions, even in small babies with low birth weight 19.2.5.3 Choice of the Operative
(Blume et al. 1999; Daebritz et al. 2000; Roussin Procedure
et al. 2007; Fricke et al. 2012; Khairy et al. 2013). The atrial switch is almost an obsolete procedure
Most patients do not need any cardiac medica- (Williams et al. 2003). The arterial switch opera-
tion and have normal exercise tolerance (Muller tion is indicated in almost all cases of D-TGA
et al. 2013). Coronary artery stenosis or independent of coronary artery pattern such as
obstruction is rare (5 %), and freedom from intramural course or single coronary artery or the
reoperation at 10 years is >90 % (Daebritz et al. presence of a bicuspid but pliable and nonthick-
2000; Kado et al. 1994; Fricke et al. 2012; Khairy ened pulmonary (neoaortic) valve (van Praagh
et al. 2013) but is lower in patients with associ- 1992). It can also be performed as primary proce-
ated cardiac malformations (Fricke et al. 2012). dure successfully in patients with closed ductus
Pulmonary stenoses caused by the Lecompte arteriosus and decreased pulmonary artery pres-
maneuver occur in 5–10 % of cases. sure safely up to an age of 8 weeks (Edwin et al.
Development of regurgitation of the neoaortic 2010), provided the left ventricular muscle mass is
valve (Losay et al. 2001; Marino et al. 2006; Lim within normal limits. Isolated cases with hypotro-
et al. 2013) occurs in up to 5 % of patients and is phy of the left ventricle and a decreased posterior
more common after pulmonary artery banding. wall thickness to as low as 2–3 mm were success-
Rhythm disturbances are rare compared to the fully operated on as late as 12 weeks of age after an
atrial switch operation and are mostly supra- intraoperative temporary pulmonary artery band-
ventricular in origin (Rhodes et al. 1995; Khairy ing was used to assess, whether or not the left ven-
et al. 2013; Bonhoeffer et al. 1997; Castaneda tricle is able to sustain systemic pressure (Däbritz
et al. 1994; Di Donato et al. 1989; Hovels-Gurich et al. 1997). All patients within this age range tol-
et al. 1997; Mayer et al. 1990; Planche et al. erated the procedure, nullifying the need for two-
1998; Pretre et al. 2001; Prifti et al. 2002; step arterial switch operation in early infancy. In
Quaegebeur et al. 1986; Sellke et al. 2005; borderline cases, beyond 12 weeks of age, an arte-
Wernovsky et al. 1995; Williams et al. 1997; rial «rapid two-stage» switch operation is utilized,
Fricke et al. 2012). Moreover, exercise tolerance in which the left ventricle is retrained in 1–2 weeks
and quality of life of patients undergoing the by tight pulmonary artery banding and implanta-
630 S.H. Daebritz and M. Ilbawi
a b
a b
..Fig. 19.24 a, b Surgery for single coronary artery abnormality (reverse flap)
tion of a modified Blalock–Taussig shunt if a dra- Banding for retraining of the left ventricle
matic drop of the arterial saturation occurs with after a failed atrial switch or for preparation for
banding (see above; Castaneda et al. 1994; Lacour- a double-switch procedure in ccTGA (see
Gayet et al. 2001; Sellke et al. 2005). While this 7 Sect. 19.1.4.3, «Retraining the Left Ventrilcle for
rapid two-step retraining acutely induces suffi- Anatomic Repair») is age dependent and seems to
cient LV myocardial hypertrophy during the first be possible up to early teenage age (Poirier and
19 6–8 months of life, it results in impaired LV func- Mee 2001; Sellke et al. 2005). Deterioration of
tion and contractility, which, has not shown pro- left ventricular function has been described
gression progress over a period of 0.5–6 years of after late banding and retraining procedures in
observation (Boutin et al. 1994). Banding also most of these cases (Daebritz et al. 2001). In this
increases the incidence of neoartic valve regurgi- age group, the retraining is frequently per-
tation (Jenkins et al. 1991). Because of these draw- formed in several steps over a time period of
backs of banding, it is better to perform primary months and years before the atrial switch is
arterial switch operation even up to 6 weeks of life. taken down and replaced with an arterial switch
Chapter 19 · Congenitally Corrected Transposition and D-Transposition of the Great Arteries
631 19
a b
operation (Daebritz et al. 2001; Imai et al. 2001; patients need early corrective surgery. On the
Mee 2005). other hand, there can be reduced pulmonary
blood flow with variable degree of cyanosis.
These cases can be stabilized by the administra-
19.2.6 Complex D-Transposition tion of prostaglandins if the ductus is still
of the Great Arteries responsive.
Diagnosis is established primarily by echocar-
19.2.6.1 Definition and Epidemiology diography, demonstrating the morphology of a
The term «complex TGA» opposite to «simple TGA» VSD, the conal septum, the coronary arteries, the
refers to D-TGA with associated malformations AV valves, and the aortic arch. In most cases, a
such as a VSD, ventricular outflow tract obstruc- cardiac catheterization is redundant and can be
tion caused by a deviation of the conal septum, and avoided, as it does not give additional informa-
Taussig–Bing or aortic arch anomalies. D-TGA tion. However, it may be necessary for functional
with a perimembranous VSD (incidence 10–15 %) assessment of the different cardiac structures,
is inconsistently classified with complex TGA (Fyler pressure measurements, and calculation of pul-
1980). Complex forms of D-TGA associated with monary vascular resistance. In addition, cardiac
hypoplasia of one ventricle or with malformations of catheterization should always be performed in
the AV valve like straddling are not dealt with in this patients with complex TGA who have had prior
chapter as they are not amenable for biventricular palliative procedures.
correction but usually undergo primary palliation
leading eventually to a Fontan-type operation. 19.2.6.3 Transposition of the Great
Arteries with Ventricular
19.2.6.2 Pathophysiology Septal Defect
and Diagnostics Up to 50 % of patients with D-TGA have a VSD,
The clinical symptoms of complex D-TGA cover which is often small and closes spontaneously, so
a broad spectrum and depend on the presence of that only about 25 % of these patients have a VSD
associated outflow tract obstruction and hypo- which needs to be surgically addressed (Daebritz
plasia of various structures. On one hand, there et al. 2000; Moene et al. 1985).
can be pulmonary over circulation with reduced In the presence of a large, nonrestrictive VSD,
systemic perfusion and its attendant symptoms the left ventricle remains trained to support the
of heart failure and low cardiac output. These systemic circulation. The arterial switch operation
632 S.H. Daebritz and M. Ilbawi
kNikaidoh Operation
The principle of this operation is that the entire
aortic root is uprooted and displaced posteriorly
into the pulmonary root and the LVOT
(. Figs. 19.29 and 19.30). Potential advantages are
the achievement of an anatomical position for the
outflow of the LVOT without a tunnel. It also
brings the RV-PA connection with or without con-
duit into a more anatomical position. In addition,
the operation can be applied to patients who are
not suitable for a Rastelli operation due to unfa-
vorable location or size of the VSD. Since the first
description of the technique in 1984, the operation
has gained increasing application recently because
..Fig. 19.28 Rastelli operation. Continuity between
right ventricle and pulmonary valve is established with a
of the reported incidence of LVOT obstruction
valved conduit. Individual anatomy dictates whether con- and resultant LV dysfunction following the Rastelli
duit runs distally to the right or the left of the aorta operation. After initiation of cardiopulmonary
634 S.H. Daebritz and M. Ilbawi
a b
..Fig. 19.29 a, b Nikaidoh operation. a The aortic root is excised and mobilized together with the coronary arteries
left in situ, if anatomically possible. The pulmonary artery is cut close to the valve. b The left ventricular outflow tract is
enlarged by dividing the ventricular septum starting from the pulmonary valve up to the VSD. The anterior leaflet of the
mitral valve is shown
a b
19
..Fig. 19.30 a, b Nikaidoh operation. a After mobilization of the aortic root, the aorta is translocated posteriorly
and about 30–50 % of the aortic root circumference of it sutured into the combined opening of RVOT and pulmonary
anulus posteriorly. The LVOT is reconstructed and enlarged with a generously tailored VSD patch. b Finally, the suture
of the aortic root is completed for the remaining 50 % to two-third of the circumference by the superior rim of the VSD
patch
Chapter 19 · Congenitally Corrected Transposition and D-Transposition of the Great Arteries
635 19
bypass, the coronary arteries are excised with a others are in favor of the Rastelli procedure
button as in arterial switch operation (s.e.). In (Brown et al. 2011).
some cases, with favorable anatomy, the coronary
arteries can be transferred en bloc with the aortic 19.2.6.5 ransposition with Right
T
root without need for excision and reattachment as Ventricular Outflow
was originally described by Nikaidoh (1984). The Obstruction and Hypoplasia
aortic root is uprooted with or without the coro- of the Aortic Arch or
nary arteries and aortic valve. The LVOT is Coarctation of the Aorta
enlarged by incising the conal septum longitudi- RVOTO in TGA is due to either a malalignment
nally towards the VSD or, if VSD is absent, towards of the conal septum in association with a cono-
the cavity of the LV. The aortic autograft is then ventricular (perimembranous) VSD or hypoplas-
sutured into the native LVOT. The LVOT is recon- tic subaortic conus as in DORV. The presence of a
structed with a triangular piece of PTFE sutured to conal septum under the pulmonary valve sepa-
the autograft distally and septum proximally, func- rating the pulmonary from the mitral valve
tionally closing the enlarged VSD. If they have results in Taussig–Bing anomaly with incidence
been excised from the aorta, the coronary arteries of 5–7 % of all TGA (Pigott et al. 1987). Subaortic
are reimplanted into the neoaortic root as in an obstruction due to conal septum deviation or
arterial switch operation. In the classic Nikaidoh hypoplastic tunnel results in hypoplasia or atresia
operation, the RVOT is reconstructed with a patch, of downstream structures. Thus, RVOTO is usu-
but a valved conduit can be used as well (homo- ally associated with hypoplasia of the aortic arch
graft or bovine jugular vein conduit). The conduit and coarctation or interruption. The incidence of
lies in a physiological position. A Lecompte arch anomalies is around 7–10 % in TGA with
maneuver is performed depending on the anatomy VSD and is increased in DORV and TGA or in
of the great vessels and is usually not recom- Taussig–Bing anomaly (see 7 Sect. 19.2.6.6,
mended in side-by-side position. «Taussig-Bing Anomaly») (Pigott et al. 1987). These
lesions are often ductal dependant and need pros-
kResults for Rastelli and Nikaidoh taglandin administration soon after birth.
Operations
Rastelli operation carries a low operative mor- kSurgical therapy
tality of less than 5 %, but has a potential long- RVOTO due to conal septum deviation or subaor-
term morbidity caused by LVOTO and right tic conus is best treated by patch enlargement of
ventricular outflow tract obstruction (RVOTO). the RVOT anteriorly. Coarctation or interruption
Up to 50 % of patients require a reintervention of is treated with end-to-end anastomosis and patch
the RVOT at 5 years (cardiac catheterization or enlargement of the arch and the ascending aorta
operation), although in some series freedom at the time of VSD closure and arterial switch
from reoperation is 86 % at 5 years and 74 % at operation. Surgery may be performed in deep
10 years (Brown et al. 2011). For the LVOT, hypothermic circulatory arrest (DHCA) or as we
reoperation rates of 5–10 % have been reported prefer with regional cerebral perfusion avoiding
from single centers (Hörer et al. 2007; Kreutzer DHCA. The patching of the ascending aorta min-
et al. 2000; Brown et al. 2011; Lim et al. 2013); a imizes the difference in caliber between proximal
European multicentre study showed the inci- neoaorta and distal aorta. Diffuse hypoplasia of
dence of long-term LVOTO to be around 5.2 % all structures, especially the aortic valve, may
(Hazekamp et al. 2010). RVOT morbidity is also require Damus–Kaye–Stensel operation or modi-
expected after the Nikaidoh operation, but fied Norwood approach. In case of hypoplasia of
LVOT should not be at risk for reoperation. The the entire arch, patch augmentation with autolo-
low rate for LVOT and RVOT reoperation led to gous pericardium or pulmonary homograft
an increased application of the Nikaidoh opera- extending from the ascending aorta along the
tion in this patient population. However, so far arch to beyond the coarcation is recommended.
there are no long-term results that support the Postoperative supravalvar pulmonary stenosis or
Nikaidoh operation in favor of Rastelli opera- obstruction due to Lecompte maneuver requires
tion (Yeh et al. 2007). One study reports superi- stenting or surgical patching in a small number of
ority of the Nikaidoh (Hu et al. 2008), while cases. Technically it is advantageous to augment
636 S.H. Daebritz and M. Ilbawi
the pulmonary end-to-end anastomosis ventrally ment of RVOTO later on. Usually, this occurs in
with a patch of autologous pericardium. the first postoperative year and is treated with a
patch augmentation like in tetralogy of Fallot. The
19.2.6.6 Taussig–Bing Anomaly Lecompte maneuver may have to be modified as it
The term Taussig–Bing anomaly refers to may add to the incidence of supravalvar stenosis
double-outlet right ventricle (DORV) with because of the side-to-side position of the great
D-transposition of the great artery in which the vessels when the aortic arch and a coarctation are
VSD is committed to the pulmonary valve (sub- present, a concomitant patch augmentation of the
pulmonary) and the great arteries are side by aortic arch (see 7 Sect. 19.2.6.5, «Transposition with
side. The aorta is on the right and slightly ante- Right Ventricular Outflow Obstruction and Hypoplasia
rior to the pulmonary artery. There is variable of the Aortic Arch or Coarctation of the Aorta») with a
degree of pulmonary valve overriding of the sep- patch of pulmonary artery homograft or another
tum (see also 7 Sect. 19.2.6.5). patch material is performed. Rarely a two-staged
TGA with DORV is also associated with coro- procedure may be needed (Daebritz et al. 2000)
nary anomalies such as single ostium or intramu- and has reasonable results. Thus, a one-stage cor-
ral artery in 27 % of cases (Uemura et al. 1995). rection is considered the procedure of choice
Abnormal mitral valve attachments to the LVOT (Tchervenkov and Korkola 2001; Tchervenkov
and hypoplasia of the aortic arch often combined et al. 1997).
with an aortic coarctation are frequently present
(Alsoufi et al. 2008). kResults of surgical therapy
Despite complexity of these lesions, the results are
kSurgical therapy comparable with those of the arterial switch oper-
The correction normally consists of an arterial ation in larger series (Daebritz et al. 2000;
switch operation with VSD closure. The VSD is Tchervenkov and Korkola 2001; Lim et al. 2013),
tunneled towards the pulmonary valve (the neo- with an operative mortality of 7 % (Vogel et al.
aortic valve). The close proximity of the VSD to 1984; Blume et al. 1999; Takeuchi et al. 2001;
the valve facilitates such connection (Mavroudis Wetter et al. 2004; Soszyn et al. 2011). There is an
et al. 1996). The VSD can be closed via the right increased rate of reoperation in patients with
atrium, a right-sided ventriculotomy, or via the hypoplasia of the aortic arch and the RVOTO and
pulmonary (neoaortic) valve. Our former routine is significantly higher than after the arterial switch
trans-neoaortic approach led to very good results operation for simple D-transposition (Alsoufi
(Daebritz et al. 2000). In this technique, the et al. 2008; Rodefeld et al. 2007; Soszyn et al. 2011;
stitches for the VSD suture are placed on the right Lim et al. 2013).
side on the lower rim of the VSD by passing the
needle prior to each stitch through the VSD onto
the right side. The patch has to be sutured in such References
a way that the free edge come to be on the right
ventricular side along the inferior and posterior Acar P, Sidi D, Bonnet D, Aggoun Y, Bonhoeffer P, Kachaner
J (1998) Maintaining tricuspid valve competence in
border. This means that the patch and sutures are
double discordance. Heart 80:479–483
not visible from the left side once the VSD is Allan LD, Sharland GK, Milburn A (1994) Prospective diagno-
closed. This can only be achieved with a running sis of 1,006 consecutive cases of congenital heart dis-
suture technique along the anterior and superior ease in the fetus. J Am Coll Cardiol 23:1452–1458
VSD boarder. Due to the subpulmonary position Allwork SP, Bentall HH, Becker AE (1976) Congenitally cor-
rected transposition of the great arteries: morpho-
of the VSD, the sutures are placed on the upper
19 rim into the pulmonary or neoaortic anulus. The
logic study of 32 cases. Am J Cardiol 38:910–923
Alsoufi B, Cai S, Williams WG (2008) Improved results with
drawback of this technique is potential damage to single-stage total correction of Taussig-Bing anomaly.
the neoaortic valve because the sutures are very Eur J Cardiothorac Surg 33:244–250
close to the valve. More recently the VSD is closed Asou T, Karl TR, Pawade A, Mee RBB (1994) Arterial switch:
translocation of the intramural coronary artery. Ann
through the right atrium most of the time. RVOT
Thorac Surg 57:461–465
reconstruction is needed initially; however, as the Baffes TG (1956) A new method for surgical correction of
native anatomic pulmonary valve is smaller than transposition of the aorta and pulmonary artery. Surg
normal, the patients are prone to future develop- Gynecol Obstet 102:227–233
Chapter 19 · Congenitally Corrected Transposition and D-Transposition of the Great Arteries
637 19
Bajpai P, Shah S, Misri A, Rao S, Suresh PV, Maheshwari S DiBardino DJ, Kleeman K, Bove EL (2012) A method of
(2011) Assessment of operability in d-transposition of transcutaneously adjustable pulmonary artery band-
great arteries with ventricular septal defect: a practical ing for staged left ventricular retraining. J Thorac
method. Ann Pediatr Cardiol 4:41–44 Cardiovasc Surg 144:553–556
Blalock A, Hanlon CR (1950) The surgical treatment of com- Di Donato RM, Wernovsky G, Walsh EP (1989) Result of the
plete transposition of the aorta and pulmonary artery. arterial switch operation for transposition of the great
Surg Gynecol Obstet 90:1 arteries with ventricular septal defect. Circulation
Blume ED, Altmann K, Mayer J (1999) Evolution of risk fac- 80:1689–1705
tors influencing early mortality of the arterial switch Duncan BW, Mee RBB, Mesia CI (2003) Results of the dou-
operation. J Am Coll Cardiol 33:1702–1709 ble switch operation for congenitally corrected trans-
Bonhoeffer P, Bonnet D, Piechaud JF (1997) Coronary position of the great arteries. Eur J Cardiothorac Surg
artery obstruction after arterial switch operation for 24:11–12
transposition of the great arteries in newborns. J Am Ebenroth ES, Hurwitz RA (2002) Functional outcome of
Coll Cardiol 29:202–206 patients operated for d-transposition of the great
Bonnet D, Corno AF, Sidi D (2004) Early clinical results of the arteries with the Mustard procedure. Am J Cardiol
telemetric adjustable pulmonary artery banding 89:353–356
FloWatch-PAB. Circulation 110(Suppl 1):II158–II163 Edwin F, Mamorare H, Brink J et al. (2010) Primary arterial
Boutin C, Wernovsky G, Sanders SP, Jonas RA, Castaneda switch operation for transposition of the great arteries
AR, Colan SD (1994) Rapid two-stage arterial switch with intact ventricular septum—is it safe after three weeks
operation, evaluation of left ventricular systolic of age? Interact Cardiovasc Thorac Surg 11(5):641–644
mechanics late after an acute pressure overload stim- Freedom RM (1999) Congenitally corrected transposition
ulus in infancy. Circulation 90:1294–1303 of the great arteries: definitions and pathologic anat-
Brawn WJ, Barron DJ, Jones TJ (2008) The fate of the omy. Prog Pediatr Cardiol 10:3–16
retrained left ventricle after double switch procedure Fricke TA, d’Udekem Y, Richardson M et al. (2012) Outcomes
for congenitally corrected transposition of the great of the arterial switch operation for transposition of the
arteries. Semin Thorac Cardiovasc Surg Pediatr Card great arteries: 25 years of experience. Ann Thorac Surg
Surg Annu 2008:69–73 94(1):139–145
Brown JW, Ruzmetov M, Huynh D et al. (2011) Rastelli oper- Friedberg DZ, Nadas AS (1970) Clinical profile of patients
ation for transposition of the great arteries with ven- with congenitally corrected transposition of the great
tricular septal defect and pulmonary stenosis. Ann arteries: a study of 60 cases. N Engl J Med 282:1053–
Thorac Surg 91(1):188–193 1059
Castaneda AR, Jonas RA, Mayer JE Jr (1994) D-transposition Fuller S, Rajagopalan R, Jarvik GP et al. (2010) J. Maxwell
of the great arteries. In: Castaneda AR, Jonas RA, Mayer Chamberlain Memorial Paper for congenital heart sur-
JE, Hanley FL (eds) Cardiac surgery of the neonate and gery. Deep hypothermic circulatory arrest does not
infant. Saunders, Philadelphia, pp 409–438 impair neurodevelopmental outcome in school-age
Cebotari S, Lichtenberg A, Tudorache I (2006) Clinical children after infant cardiac surgery. Ann Thorac Surg
application of tissue engineered human heart valves 90(6):1985–1994
using autologous progenitor cells. Circulation Fyler DC (1980) Report of the New England regional infant
114(Suppl):I132–I137 cardiac program. Pediatrics 65:375
Däbritz S, Engelhardt W, von Bernuth G, Messmer BJ (1997) Gadzoulis MA, Walters J, McLaughlin PR (2000) Late arrhyth-
Trial of pulmonary artery banding: a diagnostic crite- mias in adults with the Mustard procedure for transpo-
rion for “one-stage” arterial switch in simple transposi- sition of the great arteries: a surrogate marker for right
tion of the great arteries beyond the neonatal period. ventricular dysfunction. Heart 84:409–415
Eur J Cardiothorac Surg 11:112–116 Gittenberger-de Groot AC, Sauer U, Oppenheimer-Dekker
Daebritz SH, Sachweh JS, Tiete A, Engelhardt W, von A, Quaegebeur J (1983) Coronary artery anatomy in
Bernuth G, Messmer BJ (1999) Experience with an transposition of the great arteries: a morphologic
adjustable pulmonary artery banding device in two study. Pediatr Cardiol 4(Suppl):15
cases: initial success-midterm failure. Thorac Cardiovasc Gittenberger-de Groot AC, Sauer U, Quaegebeur J (1986)
Surg 47:51–52 Aortic intramural coronary artery in three hearts with
Daebritz SH, Nollert G, Sachweh JS, Engelhardt W, von transposition of the great arteries. J Thorac Cardiovasc
Bernuth G, Messmer BJ (2000) Anatomical risk factors Surg 91:566–571
for mortality and cardiac morbidity after arterial switch Graham TP Jr, Bernard YD, Mellen BG (2000) Long-term
operation. Ann Thorac Surg 69:1880–1886 outcome in congenitally corrected transposition of
Daebritz SH, Tiete AR, Sachweh JS, Engelhardt W, von Bernuth the great arteries: a multi-institutional study. J Am Coll
G, Messmer BJ (2001) Systemic right ventricular failure Cardiol 36:255–261
after atrial switch operation: midterm results of conver- Hauser M, Bengel FM, Hager A (2003) Impaired myocardial
sion into an arterial switch. Ann Thorac Surg 71:1255–1259 blood flow and coronary flow reserve of the anatomi-
Devaney EJ, Charpie JR, Ohye RG, Bove EL (2003) Combined cal right systemic ventricle in patients with congeni-
arterial switch and Senning operation for congenitally tally corrected transposition of the great arteries.
corrected transposition of the great arteries: patient Heart 89:1231–1235
selection and intermediate results. J Thorac Cardiovasc Hazekamp MG, Gomez AA, Koolberger DR et al. (2010)
Surg 125:500–507 Surgery for transposition of the great arteries,
638 S.H. Daebritz and M. Ilbawi
v entricular septal defect and left ventricular outflow Jatene A, Fontes VF, Paulista PP (1976) Anatomic correction
tract obstruction: European Congenital Heart Surgeons of transposition of the great vessels. J Thorac
Association multicentre study. Eur J Cardiothorac Surg Cardiovasc Surg 72:364
36:699–706 Jenkins KJ, Hanley FL, Colan SD, Mayer JE Jr, Castaneda AR,
Hiramatsu T, Matsumura G, Konuma T (2012) Long-term Wernovsky G (1991) Function of the anatomic pulmo-
prognosis of double-switch operation for congenitally nary valve in the systemic circulation. Circulation
corrected transposition of the great arteries. Eur 84(Suppl):III173–III179
J Cardiothorac Surg 42(6):1004–1008 Kado H, Asoh T, Imoto Y, Shiokawa Y, Yamasaki M, Yasui H
Hörer J, Schreiber C, Dworak E (2007) Long-term results (1994) Reoperation for transposition of the great arter-
after the Rastelli repair for transposition of the great ies: mid-term results and reoperation after arterial
arteries. Ann Thorac Surg 83:2169–2175 switch operation. Rinsho Kyebu Geka 14:192–197
Hornung TS, Bernard EJ, Jaeggi ET, Howman-Giles RB, Karl TR, Weintraub RG, Brizard CP, Cochrane AD, Mee RB
Celermajer DS, Hawker RE (1998) Myocardial perfusion (1997) Senning plus arterial switch operation for dis-
defects and associated systemic ventricular dysfunc- cordant (congenitally corrected) transposition. Ann
tion in congenitally corrected transposition of the Thorac Surg 64:495–502
great arteries. Heart 80:322–326 Khairy P, Clair M, Fernandes SM , Blume ED, Powell AJ,
Hosseinpour AR, McCarthy KP, Griselli M, Sethia B, Ho SH Newburger JW, Michael J. Landzberg MJ, Mayer JE
(2004) Congenitally corrected transposition: size of (2013) Cardiovascular outcomes after the arterial
the pulmonary trunk and septal malalignment. Ann switch operation for D-transposition of the great
Thorac Surg 77:2163–2166 arteries. Circulation 127:331–339
Hovels-Gurich HH, Seghaye MC, Dabritz S, Messmer BJ, von Kreutzer C, de Vivie J, Oppido G (2000) Twenty-five-year
Bernuth G (1997) Cardiological and general health sta- experience with Rastelli repair for transposition of the
tus in preschool- and school-age children after neona- great arteries. J Thorac Cardiovasc Surg 120:211–223
tal arterial switch operation. Eur J Cardiothorac Surg Lacour-Gayet F, Piot D, Zoghbi J (2001) Surgical management
12:593–601 and indication of left ventricular retraining in arterial
Hraska V, Duncan BW, Mayer JE, Freed M, del Nido PJ, Jonas switch for transposition of the great arteries with intact
RA (2005) Long-term outcome of surgically treated ventricular septum. Eur J Cardiothorac Surg 20:824–829
patients with corrected transposition of the great Langley SM, Winlaw DS, Stumper O (2003) Midterm results
arteries. J Thorac Cardiovasc Surg 129:182–191 after restoration of the morphologically left ventricle
Hu SS, Liu ZG, Li SJ (2008) Strategy for biventricular outflow to the systemic circulation in patients with congeni-
tract reconstruction: Rastelli, REV, or Nikaidoh proce- tally corrected transposition of the great arteries.
dure? J Thorac Cardiovasc Surg 135:331–338 J Thorac Cardiovasc Surg 125:1229–1241
Huhta JC, Maloney JD, Ritter DG, Ilstrup DM, Feldt RH (1983) Lecompte Y, Zannini L, Hazan E (1981) Anatomic correction
Complete atrioventricular block in patients with atrio- of transposition of the great arteries: new technique
ventricular discordance. Circulation 67:1374–1377 without use of a prosthetic conduit. J Thorac
Huhta JC, Danielson GK, Ritter DG, Ilstrup DM (1985) Cardiovasc Surg 82:629–631
Survival in atrioventricular discordance. Pediatr Cardiol Lim HG, Kim WH, Lee JR , Kim YJ (2013) Long-term results of
6:57–60 the arterial switch operation for ventriculo-arterial dis-
Ilbawi MN, DeLeon SY, Backer CL, Duffy CE, Muster AJ, cordance. Eur J Cardiothorac Surg 43:325–334
Zales VR, Paul MH, Idriss FS (1990) An alternative Losay J, Touchot A, Serraf A (2001) Late outcome after arte-
approach to the surgical management of physiologi- rial switch operation for transposition of the great
cally corrected transposition with ventricular septal arteries. Circulation 104:1–121
defect and pulmonary stenosis or atresia. J Thorac Lundstrom U, Bull C, Wyse R, Somerville J (1990) The natu-
Cardiovasc Surg 100:410–415 ral and “unnatural” history of congenitally corrected
Ilbawi MN, Ocampo CB, Allen BS, Barth MJ, Roberson DA, transposition. Am J Cardiol 65:1222–1229
Chiemmongkoltip P, Arcilla RA (2002) Intermediate Malhotra SP, Reddy YM, Qiu M, Pirolli PJ, Barboza L,
results of the anatomic repair for congenitally corrected Reinhartz O, Hanley FL (2011) The hemi-Mustard/bidi-
transposition. Ann Thorac Surg 73:594–599 rectional Glenn atrial switch procedure in the double-
Imai Y, Sawatari K, Hoshino S, Ishihara K, Nakazawea M, switch operation for congenitally corrected
Momma K (1994) Ventricular function after anatomic transposition of the great arteries: rationale and mid-
repair in patients with atrioventricular discordance. term results. J Thorac Cardiovasc Surg 141:162–170
J Thorac Cardiovasc Surg 107:1272–1283 Marino BS, Wernovsky G, McElhinney DB (2006) Neo-aortic
19 Imai Y, Seo K, Aoki M, Shin’oka T, Hiramatsu K, Ohta A (2001) valvular function after the arterial switch. Cardiol
Double-switch operation for congenitally corrected Young 16:481–489
transposition. Semin Thorac Cardiovasc Surg Pediatr Martin RP, Queshri SA, Ettedgui JA (1990) An evaluation of
Card Surg Ann 4:16–33 right and left ventricular function after anatomical
Imamura M, Drummond-Webb JJ, Murphy DJ Jr (2000) correction and intra-atrial repair operations for com-
Results of the double switch operation in the current plete transposition of the great arteries. Circulation
era. Ann Thorac Surg 70:100–105 82:808–816
Jatene AD, Fontes VF, Souza LCB (1975) Successful ana- Mavroudis C, Backer C (2000) Arterial switch after failed
tomic correction of transposition of the great arteries. atrial baffle procedures for transposition of the great
A preliminary report. Arg Bros Cardiol 28:461–464 arteries. Ann Thorac Surg 69:851–857
Chapter 19 · Congenitally Corrected Transposition and D-Transposition of the Great Arteries
639 19
Mavroudis C, Backer CL, Muster AJ (1996) Taussig-Bind- Poirier NC, Mee RB (2001) Left ventricular reconditioning
anomaly: arterial switch versus Kawashima intraven- and anatomical correction for systemic right ventricu-
tricular repair. Ann Thorac Surg 61:1330–1338 lar dysfunction. Semin Cardiovasc Surg Pediatr Card
Mavroudis C, Backer CL, Kohr LM (1999) Bidirectional Surg Ann 3:198–215
Glenn shunt in association with congenital repairs: the Poirier NC, Yu JH, Brizard CP, Mee RBB (2004) Long-term
1½ ventricular. Ann Thorac Surg 68:976–981 results of left ventricular reconditioning and anatomic
Mayer JE, Sanders SP, Jonas RA, Castaneda AR, Wernovsky correction for systemic right ventricular dysfunction
G (1990) Coronary artery pattern and outcome of arte- after atrial switch procedures. J Thorac Cardiovasc
rial switch operation for transposition of the great Surg 127:975–981
arteries. Circulation 82(Suppl IV):IV-139–IV-145 Presbitero P, Somerville J, Rabajoli F, Stone S, Conte MR
Mee RBB (1986) Severe right ventricular failure after Mustard (1995) Corrected transposition of the great arteries
or Senning operation two stage repair: pulmonary without associated defects in adult patients: clinical
banding and switch. J Thorac Cardiovasc 92:385–390 profile and follow up. Br Heart J 74:57–59
Mee RB (2005) The double switch operation with accent on Pretre R, Tamisier D, Bonhoeffer P (2001) Results of the
the Senning component. Semin Thorac Cardiovasc arterial switch operation in neonates with transposed
Surg Pediatr Card Surg Annu 2005:8:57–65 great arteries. Lancet 357:1826–1830
Moene RJ, Oppenheimer-Dekker A, Wenink ACG (1985) Prifti E, Crucean A, Bonacchi M (2002) Early and long
Morphology of ventricular septal defect in complete term outcome of the arterial switch operation for
transposition of the great arteries. Am J Cardiol transposition of the great arteries: predictors and
55:1566 functional evaluation. Eur J Cardiothorac Surg
Müller J, Hess J, Hörer J , Hager A (2013) Persistent superior 22:864–873
exercise performance and quality of life long-term Quaegebeur JM, Rohmer J, Ottenkamp J (1986) The arterial
after arterial switch operation compared to that after switch operation: an 8-year experience. J Thorac
atrial redirection. Int J Cardiol 166:381–384 Cardiovasc Surg 92:361–384
Mustard WT (1964) Successful two-stage correction of Quinn DW, McGuirk SP, Metha C (2008) The morphologic
transposition of the great vessels. Surgery 55:469–472 left ventricle that requires training by means of pul-
Newfeld EA, Paul MM, Muster AJ, Idriss FS (1974) Pulmonary monary artery banding before the double-switch pro-
vascular disease in complete transposition of the great cedure for congenitally corrected transposition of the
arteries: a study of 200 patients. Am J Cardiol 34:75 great arteries is at risk for late dysfunction. J Thorac
Nikaidoh H (1984) Aortic translocation and biventricular Cardiovasc Surg 135(5):1137–1144
outflow tract reconstruction. A new surgical repair for Rastelli GC, Wallace RB, Ongley PA (1969) Complete repair
transposition of the great arteries associated with ven- of transposition of the great arteries with pulmonary
tricular septal defect and pulmonary stenosis. J Thorac stenosis. A review and report of a case corrected by
Cardiovasc Surg 88:365 using a new surgical technique. Circulation 39:83
O’Shea MA, Williams WG, McLaughlin PR et al. (1983) Rhodes LA, Wernovsky G, Keane JF (1995) Arrhythmias and
Management of transposition after the Baffes proce- intracardiac conduction after the arterial switch oper-
dure: a case report and review of our experience. Ann ation. J Thorac Cardiovasc Surg 109:303–310
Thorac Surg 35:430–435 Rivenes SM, Grifka RG, Feltes TF (1998) Development of
Oechslin E, Jenni R (2000) 40 years after the first atrial obstructive pulmonary vascular disease in
switch procedure in patients with transposition of the D-transposition of the great arteries after neonatal
great arteries: long-term results in Toronto and Zurich. arterial switch operation. Tex Heart Inst J 25:201–205
Thorac Cardiovasc Surg 48:233–237 Rodefeld MD, Ruzmetov M, Vijay P, Fiore AC, Turrentine
Oelert H (1991) Modification of the Mustard operation for MW, Brown JW (2007) Surgical results of arterial switch
surgical treatment of complete transposition by creat- operation for Taussig-Bing anomaly: is position of
ing confluence of the caval veins. Cardiol Young 1: great arteries a risk factor? Ann Thorac Surg 83:1451–
71–79 1457
Padalino MA, Stellin G, Brawn WJ (2000) Arterial switch Roussin R, Belli E, Bruniaux J (2007) Surgery for transposi-
operation after left ventricular retraining in the adult. tion of the great arteries in neonates weighing less
Ann Thorac Surg 70:1753–1757 than 2,000 grams: a consecutive series of 25 patients.
Pigott JD, Chin AJ, Weinberg PM (1987) Transposition of Ann Thorac Surg 83:173–178
the great arteries with aortic arch obstruction. Sachweh JS, Tiete AR, Jockenhoevel S (2002) Fate of intra-
Anatomical review and report of surgical manage- mural coronary arteries after arterial switch operation.
ment. J Thorac Cardiovasc Surg 94:82–86 Thorac Cardiovasc Surg 50:40–44
Piran S, VeldtmannG SS, Webb GD, Liu PP (2002) Heart fail- Sano T, Riesenfeld T, Karl TR, Wilkinson JL (1995)
ure and ventricular dysfunction in patients with single Intermediate-term outcome after intracardiac repair
or systemic right ventricles. Circulation 105:1189–1194 of associated cardiac defects in patients witch atrio-
Planche C, Lacour-Gayet F, Serraf A (1998) Arterial switch. ventricular and ventriculoarterial discordance.
Pediatr Cardiol 19:297–307 Circulation 92:272–278
Poirier NC, Mee RBB (2000) Left ventricular recondition- Sellke FW, del Nido PJ, Swanson SJ (2005) Transposition of
ing and anatomical correction for systemic right ven- the great arteries. In: Sellke F, Swanson S, del Nido P
tricular dysfunction. Semin Thorac Cardiovasc Surg (eds) Sabiston & Spencer surgery of the chest. Elsevier,
3:198–215 Amsterdam, pp 2133–2151
640 S.H. Daebritz and M. Ilbawi
Senning A (1959) Surgical correction of transposition of arterial switch operation for failing systemic ventricle
the great vessels. Surgery 45:966 after atrial inversion operation. J Thorac Cardiovasc
Shin’oka T, Kurosawa H, Imai Y (2007) Outcomes of defini- Surg 111:342–347
tive surgical repair for congenitally corrected transpo- Vogel M, Freedom RM, Smallhorn JF (1984) Complete
sition of the great arteries or double outlet right transposition of the great arteries and coarctation of
ventricle with discordant atrioventricular connections: the aorta. Am J Cardiol 53:1627–1632
risk analyses in 189 patients. J Thorac Cardiovasc Surg Voskuil M, Hazekamp MG, Kroft LJM (1999) Postsurgical
133:1318–1328 course of patients with congenitally corrected trans-
Shumaker HB Jr (1961) A new operation for transposition position of the great arteries. Am J Cardiol 83:558–562
of the great vessels. Surgery 50:773–777 Warnes CA (2006) Transposition of the great arteries.
Sim EK, van Son JAM, Edwards WD, Julsrud PR, Puga FJ Circulation 114:2699–2709
(1994) Coronary artery anatomy in complete transpo- Webb CL (1999) Congenitally corrected transposition of
sition of the great arteries. Ann Thorac Surg 57:890– the great arteries: clinical features, diagnosis and
894 prognosis. Prog Pediatr Cardiol 10:17–30
Soszyn N, Fricke TA, Wheaton GR et al. (2011) Outcomes of Wells WJ, Blackstone E (2000) Intermediate outcome after
the arterial switch operation in patients with Taussig- Mustard and Senning procedures: a study by the
Bing anomaly. Ann Thorac Surg 92(2):673–679 Congenital Heart Surgeons Society. Semin Thorac
Squarcia U, Macchi C (2011) Transposition of the great Cardiovasc Surg Pediatr Card Surg Ann 3:186–197
arteries. Curr Opin Pediatr 23(5):518–522 Wernovsky G, Mayer JE, Jonas RA (1995) Factors influenc-
Takeuchi K, McGowan FX, Moran AM (2001) Surgical out- ing early and late outcome of the arterial switch oper-
come of double-outlet right ventricle with subpulmo- ation for transposition of the great arteries. J Thorac
nary VSD. Ann Thorac Surg 71:49–53 Cardiovasc Surg 109:289–302
Tchervenkov CI, Korkola SJ (2001) Transposition complexes Wetter J, Sinzobahamvya N, Blaschczok HC (2004) Results
with systemic obstruction. Semin Thorac Cardiovasc of arterial switch operation for primary total correc-
Surg Pediatr Card Surg Ann 4:71–82 tion of the Taussig-Bing anomaly. Ann Thorac Surg
Tchervenkov CI, Tahta SA, Cecere R, Beland MJ (1997) 77:41–46
Single-stage arterial switch with aortic arch enlarge- Williams WG, Quaegebeur JM, Kirklin JW, Blackstone EH
ment for transposition complexes with aortic arch (1997) Outflow obstruction after the arterial switch
enlargement. Ann Thorac Surg 64:1776–1781 operation: a multiinstitutional study. J Thorac
Uemura H, Yagihara T, Kawashima Y (1995) Coronary arte- Cardiovasc Surg 114:975–990
rial anatomy in double-outlet right ventricle with sub- Williams WG, McCrindle BW, Ashburn DA, Jonas RA, Mavroudis
pulmonary VSD. Ann Thorac Surg 59:591–597 C, Blackstone EH, the members of the Congenital Heart
van Praagh R (1992) Segmental approach to diagnosis. In: Surgeon’s Society (2003) Outcomes of 829 neonates with
Fyler DC (ed) Nadas’ pediatric cardiology. Hanley & complete transposition of the great arteries 12–17 years
Belfus, Philadelphia, pp 27–35 after repair. Eur J Cardiothorac Surg 24:1–10
Van Praagh R, Van Praagh S (1966) Isolated ventricular Yagihara T, Kishimoto H, Isobe F, Yamamoto F, Nishigaki K,
inversion: a consideration of the morphogenesis, defi- Matsuki O, Uemura H, Kamiya T et al. (1994) Double
nition and diagnosis of nontransposed and trans- switch operation in cardiac anomalies with atrioven-
posed great arteries. Am J Cardiol 17:395 tricular and ventriculoarterial discordance. J Thorac
van Praagh R, van Praagh S (1967) Anatomically corrected Cardiovasc Surg 107:351–358
transposition of the great arteries. Br Heart J 29:112–119 Yeh TJ, Conelly MS, Coles JG (1999) Atrioventricular discor-
van Son JAM, Danielson GK, Huhta JC (1995) Surgery for dance: results of repair in 127 patients. J Thorac
congenital heart disease. Late results of systemic atrio- Cardiovasc Surg 117:1190–1203
ventricular valve replacement in corrected transposi- Yeh T Jr, Ramaciotti C, Leonard SR, Roy L, Nikaidoh H (2007)
tion. J Thorac Cardiovasc Surg 109:642–643 The aortic translocation (Nikaidoh) procedure: mid-
van Son JAM, Reddy M, Silverman NH, Hanley FL (1996) term results superior to the Rastelli procedure.
Regression of tricuspid regurgitation after two-stage J Thorac Cardiovasc Surg 133:461–469
19
641 20
Congenital Anomalies
of the Coronary Arteries
and Coronary Diseases
of Children and Adolescents
Christoph Haller and Christian Schlensak
References – 657
Chapter 20 · Congenital Anomalies of the Coronary Arteries and Coronary Diseases
643 20
20.1 Introduction ALCAPA syndrome, is a rare congenital disorder.
In 1959, its incidence originally was stated to be
The development of the embryonic coronary artery only 1 in 300,000 live births (Keith 1959). In 1980,
circulation is complex and remains not fully under- the New England Regional Infant Cardiac
stood. Early theories suggest that the main coronary Program revealed 1 in roughly 100,000 and more
arteries develop from endothelial tissue growing out recently a study from Ireland 1 in 4,243 live births
of the aorta forming so-called coronary buds (Brotherton and Philip 2008; Fyler 1980). It was
(Hutchins et al. 1988). Newer animal models point first reported by Brooks in 1886 based on two
to a retrograde connection to the aorta (Waldo et al. cases. However, it was interpreted as a functional
1990). Coronary capillaries emanate from the pro- vein returning blood back into the pulmonary
epicardium forming a subepicardial capillary bed system (Brooks 1885). Almost 50 years later, it was
(Bernanke and Velkey 2002). The coronary arteries up to Bland, White, and Garland to describe the
then arise from a capillary ring that encircles the clinical features (Bland et al. 1933). Therefore,
outflow tracts and invade the aortic wall second- ALCAPA was originally known as Bland-White-
arily. Thereby, basic anlagen can be seen in all three Garland syndrome. The etiology is suspected to be
coronary sinuses that are subject to apoptotic mech- an abnormal aorticopulmonary septation process.
anisms (Waldo et al. 1990). A recent report could ALCAPA syndrome usually is an isolated defect.
further prove that coronary arteries may arise from There have been several reports that show coinci-
angiogenic sprouts of the sinus venosus by develop- dences with mitral valve arcade (Su et al. 2011) and
mental reprogramming (Red-Horse et al. 2010). other mitral valve pathologies, patent ductus arterio-
Coronary abnormalities are rare disorders occur- sus (Awasthy et al. 2010), atrial or ventricular septal
ring in approximately 1 % of all patients (Yamanaka defects (Kajihara et al. 2009; Schneider et al. 2006), or
and Hobbs 1990). It is still unclear whether this over- aortopulmonary window with interrupted aortic
or underestimates the real incidence since most arch (McMahon et al. 2002). Considering the differ-
studies are biased by referral patterns and asymp- ent anatomical variations in ALCAPA, a classifica-
tomatic patients may remain undetected (Angelini tion by Smith et al. was proposed in 1989 (Smith et al.
2002). Considering the anatomical variations of the 1989). Similar to the Leiden classification, the sinuses
normal coronary system, pathology may be even are named corresponding to the nonfacing sinus of
more variable. Alterations have been reported with the pulmonary artery. Most common is an origin of
regard to number, location, size and angle of the the left c oronary artery from sinus 1 (right-handed
ostia, coronary artery size, course, arteriolar ramifi- sinus). Other possible takeoffs are sinus 2, the supra-
cations and termination, as well as secondary abnor- commissural wall of sinus 1 and the nonfacing sinus,
malities (Angelini 2002; Ogden 1970). and posterior aspects of pulmonary artery branches.
Describing coronary variations requires a def- Coronary perfusion in utero is unremarkable.
inition of the normal anatomy. Herein, we rely on This changes early postnatally when the high pul-
the common description as reviewed by Loukas monary vascular resistance still allows for ante-
et al. (2009) and the definitions of the Congenital grade perfusion of the anomalous artery but
Heart Surgery Nomenclature and Database Proj- supplies the myocardial mass with hypoxic blood.
ect (Dodge-Khatami et al. 2000). The aortic Later on, with continuing reduction of pulmonary
sinuses are described as suggested by the Leiden vascular resistance, antegrade flow into the anom-
classification (Gittenberger-de Groot and Sauer alous artery will be reduced or even reversed. This
1983) and Smith et al. (1989). may cause a myocardial perfusion deficit. In case
of flow reversal, a coronary steal effect with left-
to-right shunting can be seen. The coronary blood
20.2 nomalous Pulmonary Origins
A flow depends highly on increased right coronary
of the Coronaries blood flow and on the collateral network between
the right and left coronary artery. Stenosis of the
20.2.1 nomalous Left Coronary
A left coronary origin can further alter coronary
Artery from the Pulmonary hemodynamics (Dodge- Khatami et al. 2000).
Artery (ALCAPA) According to the extent of these intercoronary
collaterals, an infantile type with little and an
The anomalous origin of the left coronary artery adult type with good collateralization are defined
from the pulmonary artery, also known as (Agustsson 1962).
644 C. Haller and C. Schlensak
The demand-supply mismatch leads to myocar- lish a two coronary system with antegrade perfusion.
dial damage and its sequelae such as v entricular dys- It is not surprising that ligation compared with estab-
function, ventricular dilation, ventricular aneurysm, lishment of a two coronary system performed worse
mitral valve insufficiency due to papillary muscle (Bunton et al. 1987). Therefore, the most physiologic
ischemia, and malignant arrhythmias. If the collateral and anatomical correction, the direct reimplantation
blood flow is satisfactory, ALCAPA can stay unrecog- as described by Neches et al. (1974) is the favorable
nized up to an advanced age (Tsigkas et al. 2011). therapeutic strategy in ALCAPA. If despite all surgi-
Once coronary blood supply is insufficient, cal sophistication a direct anastomosis cannot be
Bland-White-Garland syndrome patients usually achieved, several different options have been
present with signs of heart failure such as increased reported. Most of them deal with poor mobilization
diaphoresis, dyspnea with feeding, tachycardia, and of the coronary. The most relevant strategies will be
failure to thrive. It can be difficult to distinguish discussed below. Surgical techniques of ligation of
between dilated cardiomyopathy and ALCAPA. the coronary artery and bypassing of the vessel are
Specific electrocardiographic changes have been nowadays rarely used and should be reserved for
defined by Johnsrude et al. (1995) as Q-wave width special anatomical variations and individual cases.
≥30 ms in lead I and Q-wave depth ≥3 mm and ST Special attention has to be paid not only to
segment amplitude in lead aVL. The diagnostic value administration of cardioplegia but also to persistent
was challenged by Chang et al. (2001) who added coronary malperfusion once extracorporeal circu-
echocardiographic findings of an RCA to aortic anu- lation has been established. Adequate myocardial
lus ratio greater than 0.14, increased echogenicity of protection during correction is crucial to the insuf-
papillary muscles and Doppler color flow through the ficiently perfused myocardium. As soon as cardio-
left coronary artery to electrocardiographic changes. pulmonary bypass starts, left-to-right shunting
Nevertheless, ALCAPA can be diagnosed by echocar- increases since the pulmonary arterial pressure
diography alone if there is awareness of the examiner. drops. Therefore, the main pulmonary artery or in
In addition to the confirmation of the aberrant artery, complex cases the right and left pulmonary arteries
an increased diameter of the right coronary artery have to be clamped immediately to avoid further
can be seen. Indirect changes such as signs of myocar- coronary steal. During delivery of cold blood car-
dial dysfunction and mitral valve insufficiency can dioplegia, our strategy includes clamping both the
complete the clinical picture. In rare cases, coronary aorta and the pulmonary artery. Other possible
angiography and MRI viability studies can help in approaches act by direct clamping of the left coro-
securing the diagnosis (Dodge-Khatami et al. 2002). nary artery at its origin or cardioplegia infusion via
At least within the first 3 years of life, any patient pre- the aorta and the pulmonary artery simultaneously.
senting with the clinical signs of dilated cardiomyop-
athy should have diagnostic evaluation to rule out 20.2.1.1 Direct Reimplantation
ALCAPA. of the Anomalous Left
Surgical correction is indicated once the diag- Coronary Artery
nosis is made. The infantile type is characterized by Today, most of the patients presenting with Bland-
insufficient collateral perfusion and has a fatal prog- White-Garland syndrome can be treated with early
nosis if left untreated. Ongoing myocardial damage correction via direct reimplantation (Turley et al.
due to chronic hypoperfusion occurs and hinders 1995). Surgical access is gained by standard median
postoperative improvement of left ventricular func- sternotomy. We prefer establishment of extracor-
tion. Ultrastructural changes could be detected in poreal circulation with bicaval venous cannulation.
specimen taken intraoperatively (Shivalkar et al. Cardioplegia is administered as described
1994) as well as post mortem (Smith et al. 1989). above. After adequate myocardial protection, the
Left ventricular recovery can be achieved best by pulmonary artery is transected and the aberrant left
early detection and early correction. coronary artery identified. The ostium is carefully
20 Early operative strategies aimed at a reduction of excised leaving a large pulmonary wall button. The
left-to-right shunting and an elimination of coronary most challenging part starts with mobilization of
steal. Several techniques have been reported includ- the coronary artery. Special attention has to be paid
ing ligation of the left coronary artery which leads to when choosing the adequate localization for aortic
an increase of its perfusion pressure (Sabiston et al. reimplantation. Tension should be avoided by any
1960). The corrective measure, however, is to estab- means, but kinking or distortion may as well lead to
Chapter 20 · Congenital Anomalies of the Coronary Arteries and Coronary Diseases
645 20
devastating consequences. The aortic wall can be 20.2.1.2 Coronary Button Transfer
punched or incised in a flap-like fashion (trapdoor with Lecompte Maneuver
technique) to get a suitable opening (. Fig. 20.1). The farther away the anomalous coronary artery
Anastomosis is usually done with 6/0 or 7/0 is located, the more technically challenging is the
polydioxanone sutures. Finally, the defect of the direct reimplantation. Malankar et al. proposed a
pulmonary wall has to be closed by autologous or technique combining an extended coronary but-
xenogenic pericardium. Polytetrafluoroethylene ton transfer with Lecompte maneuver (Malankar
(PTFE) or polyester (Dacron®) patches can be used et al. 2010). The pulmonary trunk is transected
as well. Owed to the pathophysiology, early reperfu- close to the pulmonary bifurcation. The anomalous
sion and minimal cross-clamping time can mini- artery then has to be excised generously to ensure
mize myocardial stress. Therefore, coronary a tension-free anastomosis. The aorta has to be
circulation should be reestablished as soon as transected above the sinotubular junction and lon-
proper left coronary reinsertion is achieved. gitudinally incised opposite to the aberrant artery.
a
b
..Fig. 20.1 a–d Direct reimplantation in ALCAPA syndrome. a Incision of the pulmonary trunk. b Excision of the
coronary ostium. c Reimplantation into the ascending aorta. d Pulmonary trunk closure
646 C. Haller and C. Schlensak
a b
e
Pericardial patch
..Fig. 20.3 a–e Takeuchi repair via intrapulmonary tunneling. a Operative site with incision marks corresponding to
anomalous coronary artery. b Flap-like incision of the pulmonary trunk. c Creation of an aortopulmonary window. d
Posterior displacement of pulmonary wall flap and tunnel creation. e Pulmonary trunk patch augmentation
a
b
..Fig. 20.4 a–d Aortocoronary anastomosis using the left subclavian artery. a Access can be gained by left
anterolateral thoracotomy. b Proximal ligation of the anomalous coronary artery. c, d End-to-side or end-to-end
anastomosis of the subclavian artery with the anomalous coronary artery
PA
Posterior
Anterior
Right
LAD
LCX
RCA
AO
..Fig. 20.5 Anomalous origin of the left coronary artery from the right sinus of Valsalva. LAD left anterior descending
artery, LCX left circumflex artery, PA pulmonary artery, RCA right coronary artery, AO aorta
incised following the intramural course. The high as it might be suspected from necropsy
intercoronary commissure needs to be detached studies, surgery should be indicated cautiously.
and resuspended. Outcome of the procedure Surgical correction is justified in symptomatic
regarding patency of the coronary is excellent. patients. Even though the risk of sudden cardiac
The downside is the potential of aortic valve death is higher with exercise, there still remains a
incompetence, which might warrant aortic valve residual risk with restriction from exertion. We
replacement (Romp et al. 2003). Therefore, recommend evaluation of prophylactic ICD
creation of a neo-ostium has been suggested
implantation in conservatively managed patients.
(Davies et al. 2009; Mumtaz et al. 2011). However, nowadays most authors suggest pri-
Coronary reimplantation or pulmonary mary surgical therapy in asymptomatic left coro-
translocation are other options used in select nary artery arising from the right sinus of
cases. Valsalva (Davies et al. 2009).
These surgical procedures can be performed
easily. Nevertheless, reported outcomes are diffi-
cult to interpret . Most studies are based on small 20.3.2 ight Coronary Artery
R
patient numbers and lack a long follow-up. from Left Sinus of Valsalva
Adverse events differ in frequency between 0 and
42 % (Penalver et al. 2012). In view of the fact The incidence of this anomaly is equal or higher
that the risk of sudden cardiac death is not as than the anomalous origin of the left coronary
652 C. Haller and C. Schlensak
artery from the right sinus of Valsalva (Taylor 20.3.3 ther Anomalous Aortic
O
et al. 1992; Yamanaka and Hobbs 1990). Origins of the Coronaries
Although data is scarce, the rate of sudden car-
diac deaths in these patients is lower and sur- Individual origins have been described for both the
gery should be indicated with caution. In left anterior descending and the circumflex artery
contrast to an anomalous left coronary artery, an from the right coronary sinus or from the right
asymptomatic right coronary artery from the coronary artery itself (. Fig. 20.7). There are reports
left sinus of Valsalva may be treated conserva- of all three coronary vessels arising separately from
tively with medical therapy and restriction from the right or left sinus of Valsalva, respectively
exercise. Symptomatic patients should undergo (Panduranga and Al-Mukhaini 2012; Surana et al.
surgery, which follows the techniques described 2012). Since numbers of patients are very small,
for an anomalous left coronary artery treatment strategies have to be evaluated for each
(. Fig. 20.6). patient dependent on individual risk factors.
LAD b
a LCX LAD
LCX
PA PA
RCA
RCA =
AO AO
..Fig. 20.6 a, b Scheme of anomalous origins of the right coronary artery from the left sinus of Valsalva; LAD left
anterior descending artery, LCX left circumflex artery, PA pulmonary artery, RCA right coronary artery, AO aorta
LCX
LCX
AO LCX AO
RCA AO RCA
= RCA
20
..Fig. 20.7 a–c Scheme of anomalous courses of the circumflex artery. The artery may originate either with a
separate ostium, a common ostium, or as a branch of the right coronary artery. LCX left circumflex artery, RCA right
coronary artery, LAD left anterior descending artery, PA pulmonary artery, AO aorta
Chapter 20 · Congenital Anomalies of the Coronary Arteries and Coronary Diseases
653 20
20.4 ongenital Atresia of the Left
C 20.5 Single Coronary Artery
Main Stem
Single coronary arteries are defined as a coro-
There have been early reports of atresia of the nary artery system that arises from a single coro-
left coronary ostium (Byrum et al. 1980; nary ostium supplying the entire myocardium
Mullins et al. 1972; van der Hauwaert et al. (. Fig. 20.9). Since the anomaly is considered
1982). One of the earliest surgical corrections benign and symptoms are extraordinarily rare,
by saphenous vein grafting is described by diagnosis is usually made accidentally. Incidence
Mullins et al. in 1972 (Mullins et al. 1972). is reported with 0.04 % (Dodge-Khatami et al.
Collaterals from the right coronary artery pro- 2000). Associations with congenital malforma-
vide perfusion of the left coronary system tions have been described in transposition of
(. Fig. 20.8). Atresia of the left coronary artery the great arteries, tetralogy of Fallot, truncus
can be differentiated from a single coronary arteriosus, arteriovenous fistula, endocardial
artery by coronary angiography showing a ret- fibroelastosis, and bicuspid aortic valve (Dodge-
rograde perfusion of the left coronary system. Khatami et al. 2000). The classification proposed
Therefore, patients present with signs similar by Smith et al. (Smith 1950) is usually applied.
to those of Bland-White-Garland syndrome, It divides the pattern in three subtypes. Type I
such as syncope, failure to thrive, angina, dys- refers to a single coronary artery that follows the
pnea, or myocardial infarction (Musiani et al. course of one of the two regular arteries. In type
1997). However, they miss the feature of left II, the course follows the normal distribution of
coronary artery steal, namely left-to-right both coronary vessels. Anomalous courses that
shunt into the pulmonary artery. Surgery is cannot be assigned to either type I or II are clas-
indicated once the diagnosis is established. sified as type III. The Congenital Heart Surgery
Therapeutic strategies aim at creating a two Nomenclature and Database Project follows a
coronary system. Most commonly, coronary more detailed classification based on the one
artery bypass grafting is performed. Left main suggested by Smith and described by Sharbaugh
trunk reconstruction has been described, too. in 1974 (Dodge-Khatami et al. 2000; Sharbaugh
LCX
LAD
LCX
PA LAD
PA
AO RCA
AO
RCA
and White 1974). Other more detailed and Congenital Heart Surgery Nomenclature and
complex classifications have been created, too Database Project refers to an angiographic classi-
(Ogden 1970). fication by Sakakibara et al. who differentiate
The risk of sudden cardiac death is only between a proximal and a distal type (Sakakibara
increased in patients with an anomalous course et al. 1966).
in between the great vessels. This may be the only Patients usually present with signs and symp-
indication for surgery once a single coronary toms due to left-to-right shunting. The size of the
artery is diagnosed. If needed, coronary bypass fistula correlates with its clinical manifestation.
grafting is the most common therapeutic Most patients are asymptomatic and are referred
approach. because of an unusual murmur during ausculta-
tion. Since drainage to the right atrium or ventricle
is common, signs of right heart volume overload
20.6 Coronary Arteriovenous can be seen. Nonspecific signs such as fatigue, dys-
Fistulas pnea, angina, endocarditis, arrhythmias, and myo-
cardial ischemia or infarction may precede
Coronary arteriovenous fistulas are defined as congestive heart failure. A rare complication of
connections between the coronary arteries and coronary arteriovenous fistulas is endocarditis.
the cardiac chambers or the great vessels, bypass- Diagnosis is made by echocardiography,
ing the myocardial capillary bed. Small fistulas are which can be helpful in larger fistulas, showing
quite frequent and are considered normal. They the extent and course of the vessel. Angiography is
usually neither result in clinical signs and symp- regarded as gold standard, since shunt volumes
toms nor do they increase in size and therefore can be quantified and exact course, origin, drain-
commonly remain undetected (Yamanaka and age, and size of the fistula can be observed. CT or
Hobbs 1990). Spontaneous closure may occur in MRI can give additional valuable information
some cases (Qureshi 2006). before initiating therapy.
Coronary arteriovenous fistulas with relevant Spontaneous closures of coronary fistulas,
intracardiac shunts are rare. Incidence is specified although rare, have been reported (Griffiths et al.
between 0.18 and 0.87 % (Angelini et al. 2002; 1983; Hackett and Hallidie-Smith 1984; Schleich
Yamanaka and Hobbs 1990). As with most coro- et al. 2001). Indications for therapeutic closure are
nary anomalies, reports of incidence are biased symptomatic malformations and signs of heart
since asymptomatic patients may circumvent failure, myocardial ischemia, or when large but
diagnostic detection. Coronary fistulas have first clinically still asymptomatic shunt volumes are
been described by Krause in 1865 (Krause 1865). detected. If asymptomatic, therapeutic strategies
Björck et al. described the first surgical approach remain controversial due to the potentially benign
(Biorck and Crafoord 1947). Most commonly, this course of the diseases.
anomaly is considered congenital even though fis- There are several treatment options using
tulas can be acquired by trauma or may be sec- interventional approaches as well as surgical tech-
ondary to surgical or interventional procedures. niques. Since its first description in the 1980s,
Associated congenital malformations are catheter-based closure of coronary fistulas was
described, such as atrial and ventricular septal performed numerously. A multitude of different
defects, tetralogy of Fallot, double-outlet right devices have been used, ranging from detachable
ventricle, pulmonary or aortic stenosis, mitral balloons and coils of various types to Amplazer
regurgitation, and patent ductus arteriosus duct occluders (Qureshi 2006; Wiegand et al.
(Fernandes et al. 1992). 2009). The risk of interventional procedures is
Most coronary fistulas, approximately embolization of the device, especially in high-flow
50–60 %, arise from the right coronary artery, fol- fistulas. Surgical approaches aim at direct closure
20 lowed by the left anterior descending (~35 %) and of the anomaly. Depending on size, location, and
the circumflex artery (~18 %) (Dodge-Khatami myocardial perfusion, closure can be achieved
et al. 2000; Gowda et al. 2006). Fistulas usually with or without cardiopulmonary bypass.
drain to the right side, most commonly to the Especially in large fistulas with impaired myocar-
right ventricle, followed by the right atrium. The dial perfusion, a trans ventricular approach with
anomaly may present isolated or multiple. The closure of its drainage site should be considered.
Chapter 20 · Congenital Anomalies of the Coronary Arteries and Coronary Diseases
655 20
If the coronary vessel is considerably dilated,
aneurysmorrhaphy or resection with interposi-
tion or bypass grafting may be needed.
Outcome of interventional and surgical pro-
cedures is excellent with only low mortality
rates (Mavroudis et al. 1997; Qureshi 2006).
Treatment strategies should be evaluated care-
fully and discussed in an interdisciplinary team
to guarantee optimal care.
Incidence ranges between 1.5 and 5 % (Syed cate antiplatelet or anticoagulant treatment even
and Lesch 1997). Congenital aneurysms only though there are no clear criteria for initiation of
form a minor part. Atherosclerosis is reported as therapy. If symptomatic, surgical therapy is indi-
the main cause of aneurysms, although numbers cated. Especially if patients are at increased risk
vary depending on the study population. Kawasaki of rupture, e.g., in case of giant aneurysms or
disease, for example, mainly affects Japanese and rapid progression, if there are signs of local com-
Korean people, but increasing incidence has been pression or if additional coronary artery pathol-
reported for the United States, too (Newburger ogies exist, surgery should not be delayed. In
and Fulton 2004; Taubert 1997). Other associa- case of asymptomatic, moderately sized aneu-
tions have been reported for systemic vasculitis rysms, therapy may not be necessary and close
(e.g., Churg-Strauss syndrome, polyarteritis follow-up combined with medical treatment can
nodosa, syphilis, systemic lupus erythematosus, be sufficient.
Takayasu’s arteritis), connective tissue disorders Via a median sternotomy, all aneurysms
(e.g., Marfan’s or Ehlers-Danlos syndrome), bac- should be accessible. Exposure of the left main
terial infections, drugs, or trauma (Cohen and coronary artery can be challenging. Strategies
O’Gara 2008; Hartmann et al. 2012; Syed and include coronary artery bypass grafting with liga-
Lesch 1997). tion, resection, or marsupialization of the aneu-
The right coronary artery is most commonly rysm. Off-pump coronary artery bypass
affected accounting for 40–87 % of coronary techniques have been reported (Emaminia et al.
aneurysms (Cohen and O’Gara 2008; Cohn 2007). 2011; Ishikawa et al. 2004), as well as interven-
Among 978 patients examined by Swaye et al. tional approaches.
(1983), the proximal and middle segments of the
right coronary artery were most frequently
involved, followed by the proximal left anterior 20.8.1 Kawasaki Disease
descending and the circumflex artery. Left main
coronary aneurysms have been described but are Kawasaki disease, also known as mucocutaneous
considerably rare. lymph node syndrome, is the leading cause of
Clinical presentation depends on size of the acquired heart disease in children in the United
aneurysm and can range from asymptomatic States and Japan (Taubert 1997). First described
patients to signs of myocardial ischemia, con- by Kawasaki (1967) in 1967, the disease is an acute
gestive heart failure, or even sudden cardiac febrile vasculitis of medium-sized vessels. Its role
death. There is an increased risk of thrombosis, in pediatric cardiac surgery is related to the occur-
thromboembolism, fistula formation, and rup- rence of coronary artery aneurysms which can
ture (Cohen and O’Gara 2008). Large aneu- affect 15–25 % of untreated children (Newburger
rysms may be seen on chest x-rays or during et al. 2004). The mainstays of therapy are anti-
routine echocardiography. Standard imaging is inflammatory drugs, intravenous immunoglobu-
made with CT or MRI, which offers the possibil- lin, anticoagulation, and TNF-α inhibition.
ity of reconstructing the extent of the dilated Administration of intravenous immunoglobulin
vessel and its relation to adjacent structures. can reduce the prevalence of coronary artery
Coronary angiography is usually performed abnormalities (Durongpisitkul et al. 1995). In
adding information on coronary anatomy, coex- Kawasaki disease, impressive regression of aneu-
isting atherosclerotic coronary artery disease, rysms has been reported, depending on initial
and associated fistulas. aneurysmal size (McNeal-Davidson et al. 2013).
It is not clear if patients with coronary aneu- Regression has been described in 55 % of affected
rysms with associated atherosclerotic coronary patients during follow-up (Kato et al. 1996). This
artery disease are at higher risk for adverse is commonly due to myointimal proliferation,
20 events than those without atherosclerosis. causing stenosis of the coronary. In giant coronary
Nevertheless, coronary aneurysms show worse aneurysms, antithrombotic management most
outcomes when compared to the general popula- commonly includes low-dose aspirin combined
tion (Baman et al. 2004; Demopoulos et al. 1997; with warfarin, aiming at an international normal-
Hartnell et al. 1985; Swaye et al. 1983). ized ratio of 2.0–2.5 (Gordon et al. 2009;
Conservative regimes in larger aneurysms advo- Newburger et al. 2004).
Chapter 20 · Congenital Anomalies of the Coronary Arteries and Coronary Diseases
657 20
Operative therapy should be considered if artery. Ann Thorac Surg 76:1429–1434. doi:10.1016/
there is left main trunk occlusion or obstruction S0003-4975(03)00897-X
Ben Ali W, Metton O, Roubertie F et al. (2009) Anomalous
of a main coronary artery. Giant aneurysms tend origin of the left coronary artery from the pulmonary
to have a higher risk of developing stenosis later artery: late results with special attention to the mitral
on, as well as occlusion of the artery or rupture of valve. Eur J Cardiothorac Surg 36:244–248.
the aneurysm itself. A common therapeutic doi:10.1016/j.ejcts.2009.03.014; discussion 248–9
approach is coronary artery bypass grafting. Graft Bernanke DH, Velkey JM (2002) Development of the coro-
nary blood supply: changing concepts and current
patency has been reported as good, even though
ideas. Anat Rec 269:198–208. doi:10.1002/ar.10139
there is little data on long-term outcomes (Tsuda Bharati S, Chandra N, Stephenson LW et al. (1984) Origin of
2009; Tsuda and Kitamura 2004, 2007). the left coronary artery from the right pulmonary
artery. J Am Coll Cardiol 3:1565–1569. doi:10.1016/
S0735-1097(84)80298-3
Biorck G, Crafoord C (1947) Arteriovenous aneurysm on
References the pulmonary artery simulating patent ductus arteri-
osus botalli. Thorax 2:65–74
Agustsson MH (1962) Anomalous origin of left coronary Bland EF, White PD, Garland J (1933) Congenital anomalies
artery from pulmonary artery. JAMA 180:15. of the coronary arteries: report of an unusual case
doi:10.1001/jama.1962.03050140017004 associated with cardiac hypertrophy. Am Heart
Alexi-Meskishvili V, Nasseri BA, Nordmeyer S et al. (2011) J 8:787–801. doi:10.1016/S0002-8703(33)90140-4
Repair of anomalous origin of the left coronary artery Bossert T, Walther T, Doll N et al. (2005) Anomalous origin of
from the pulmonary artery in infants and children. the right coronary artery from the pulmonary artery
J Thorac Cardiovasc Surg 142:868–874. doi:10.1016/j. combined with aortic valve stenosis. Ann Thorac Surg
jtcvs.2011.04.006 79:347–348. doi:10.1016/j.athoracsur.2003.08.016
Angelini P (2002) Coronary artery anomalies – current clin- Brooks HS (1885) Two cases of an abnormal coronary artery of
ical issues: definitions, classification, incidence, clinical the heart arising from the pulmonary artery: with some
relevance, and treatment guidelines. Tex Heart Inst remarks upon the effect of this anomaly in producing
J 29:271–278 cirsoid dilatation of the vessels. J Anat Physiol 20:26–29
Angelini P (2007) Coronary artery anomalies: an entity in Brotherton H, Philip RK (2008) Anomalous left coronary
search of an identity. Circulation 115:1296–1305. artery from pulmonary artery (ALCAPA) in infants: a
doi:10.1161/CIRCULATIONAHA.106.618082 5-year review in a defined birth cohort. Eur J Pediatr
Angelini P, Villason S, Chan AV, Diez G (1999) Normal and 167:43–46. doi:10.1007/s00431-007-0423-1
anomalous coronary arteries in humans. Coronary Bunton R, Jonas RA, Lang P et al. (1987) Anomalous origin
Artery anomalies: a comprehensive approach. Lippincott of left coronary artery from pulmonary artery. Ligation
Williams & Wilkins, Philadelphia, p 27–79 versus establishment of a two coronary artery system.
Angelini P, Velasco JA, Flamm S (2002) Coronary anoma- J Thorac Cardiovasc Surg 93:103–108
lies: incidence, pathophysiology, and clinical relevance. Byrum CJ, Blackman MS, Schneider B et al. (1980)
Circulation 105:2449–2454. doi:10.1161/01.CIR.00000 Congenital atresia of the left coronary ostium and
16175.49835.57 hypoplasia of the left main coronary artery. Am Heart
Armer RM, Shumacker HB Jr, Lurie PR, Fisch C (1963) Origin J 99:354–358
of the left coronary artery from the pulmonary artery Chang RR, Allada V, Articles O (2001) Electrocardiographic
without collateral circulation: report of a case with a and echocardiographic features that distinguish anom-
suggested surgical correction. Pediatrics 32:588–593 alous origin of the left coronary artery from pulmonary
Awasthy N, Marwah A, Sharma R, Dalvi B (2010) Anomalous artery from idiopathic dilated cardiomyopathy. Pediatr
origin of the left coronary artery from the pulmonary Cardiol 22:3–10. doi:10.1007/s002460010142
artery with patent ductus arteriosus: a must to recog- Chugh SS, Reinier K, Balaji S et al. (2009) Population-based
nize entity. Eur J Echocardiogr 11:E31. doi:10.1093/ analysis of sudden death in children: the oregon sud-
ejechocard/jeq039 den unexpected death study. Heart Rhythm 6:1618–
Azakie A, Russell JL, McCrindle BW et al. (2003) Anatomic 1622. doi:10.1016/j.hrthm.2009.07.046
repair of anomalous left coronary artery from the pul- Cleuziou J, Haas F, Schreiber C et al. (2006) Hypoplastic left
monary artery by aortic reimplantation: early survival, heart syndrome with anomalous origin of the right
patterns of ventricular recovery and late outcome. coronary artery. Ann Thorac Surg 81:341–343.
Ann Thorac Surg 75:1535–1541. doi:10.1016/ doi:10.1016/j.athoracsur.2004.09.057
S0003-4975(02)04822-1 Cohen P, O’Gara PT (2008) Coronary artery aneurysms: a
Baman TS, Cole JH, Devireddy CM, Sperling LS (2004) Risk review of the natural history, pathophysiology, and
factors and outcomes in patients with coronary artery management. Cardiol Rev 16:301–304. doi:10.1097/
aneurysms. Am J Cardiol 93:1549–1551. doi:10.1016/j. CRD.0b013e3181852659
amjcard.2004.03.011 Cohn L (2007) Cardiac surgery in the adult. 3rd edn.
Barth MJ, Allen BS, Gulecyuz M et al. (2003) Experience McGraw Hill Professional, New York, NY, p 1584
with an alternative technique for the management of Collins N, Colman J, Benson L et al. (2007) Successful per-
anomalous left coronary artery from the pulmonary cutaneous treatment of anomalous left coronary
658 C. Haller and C. Schlensak
artery from pulmonary artery. Int J Cardiol 122:e29– Grayzel DM, Tennant R (1934) Congenital atresia of the tri-
e31. doi:10.1016/j.ijcard.2006.11.075 cuspid orifice and anomalous origins of the coronary
D’Alessandro LC, Di Lorenzo M (1976) Single coronary arteries from the pulmonary artery. Am J Pathol
artery originating from the left pulmonary artery of a 10:791–794.1
“truncus arteriosus communis” in a living 39 year-old- Griffiths SP, Ellis K, Hordof AJ et al. (1983) Spontaneous
patient. G Ital Cardiol 6:939–945 complete closure of a congenital coronary artery fis-
Davies JE, Burkhart HM, Dearani JA et al. (2009) Surgical tula. J Am Coll Cardiol 2:1169–1173
management of anomalous aortic origin of a coronary Hackett D, Hallidie-Smith KA (1984) Spontaneous closure
artery. Ann Thorac Surg 88:844–847. doi:10.1016/j. of coronary artery fistula. Br Heart J 52:477–479
athoracsur.2009.06.007; discussion 847–8 Harris KM, Henry JT, Rohman E et al. (2010) Sudden death
Del Nido PJ, Duncan BW, Mayer JE et al. (1999) Left ven- during the triathlon. JAMA 303:1255–1257.
tricular assist device improves survival in children with doi:10.1001/jama.2010.368
left ventricular dysfunction after repair of anomalous Hartmann M, Wajon EMCJ, van Houwelingen GK et al. (2012)
origin of the left coronary artery from the pulmonary Giant coronary aneurysm in Churg-Strauss syndrome.
artery. Ann Thorac Surg 67:169–172 Euro Interv 8:760–761. doi:10.4244/EIJV8I6A116
Demopoulos VP, Olympios CD, Fakiolas CN et al. (1997) The Hartnell GG, Parnell BM, Pridie RB (1985) Coronary artery
natural history of aneurysmal coronary artery disease. ectasia. Its prevalence and clinical significance in 4993
Heart 78:136–141 patients. Br Heart J 54:392–395
Dodge-Khatami A, Mavroudis C, Backer CL (2000) Hutchins GM, Kessler-Hanna A, Moore GW (1988)
Congenital heart surgery nomenclature and database Development of the coronary arteries in the embry-
project: anomalies of the coronary arteries. Ann onic human heart. Circulation 77:1250–1257.
Thorac Surg 69:S270–S297 doi:10.1161/01.CIR.77.6.1250
Dodge-Khatami A, Mavroudis C, Backer CL (2002) Imamura M, Dossey AM, Jaquiss RDB (2011) Reoperation
Anomalous origin of the left coronary artery from the and mechanical circulatory support after repair of
pulmonary artery: collective review of surgical ther- anomalous origin of the left coronary artery from the
apy. Ann Thorac Surg 74:946–955 pulmonary artery: a twenty-year experience. Ann
Durongpisitkul K, Gururaj VJ, Park JM, Martin CF (1995) The Thorac Surg 92:167–172. doi:10.1016/j.athorac-
prevention of coronary artery aneurysm in Kawasaki sur.2011.02.074; discussion 172–3
disease: a meta-analysis on the efficacy of aspirin and Ishikawa S, Wakamatsu S, Tange S et al. (2004) Combination
immunoglobulin treatment. Pediatrics 96:1057–1061 therapy for adult coronary artery aneurysm with off-
Eckart RE, Scoville S, Campbell CL et al. (2004) Sudden pump coronary artery bypass grafting and vein graft-
death in young adults: a 25-year review of autopsies in coated stent. Jpn Heart J 45:157–161
military recruits. Ann Intern Med 141:829–834. Johnsrude CL, Perry JC, Cecchin F et al. (1995) Differentiating
doi:10.1016/S0084-3954(07)70139-2 anomalous left main coronary artery originating from
El-Rassi I, Soueide A, Sweid O, Chabb B (2012) Transfer the pulmonary artery in infants from myocarditis
technique of an anomalous coronary artery from the and dilated cardiomyopathy by electrocardiogram. Am
anterior pulmonary artery. Ann Thorac Surg 93:1738– J Cardiol 75:71–74. doi:10.1016/S0002-9149(99)80531-1
1740. doi:10.1016/j.athoracsur.2011.12.046 Juilliére Y, Berder V, Suty-Selton C et al. (1995) Isolated
Emaminia A, Bandettini WP, Arai AE, Horvath KA (2011) Off-pump myocardial bridges with angiographic milking of the
surgery for giant right coronary artery aneurysms. J Card left anterior descending coronary artery: a long-term
Surg 26:596–599. doi:10.1111/j.1540-8191.2011.01332.x follow-up study. Am Heart J 129:663–665
Fernandes ED, Kadivar H, Hallman GL et al. (1992) Kajihara N, Asou T, Takeda Y, Kosaka Y (2009) Bland-White-
Congenital malformations of the coronary arteries: Garland syndrome with ventricular septal defect: late
the Texas Heart Institute experience. Ann Thorac Surg presentation. Gen Thorac Cardiovasc Surg 57:547–549.
54:732–740. doi:10.1016/0003-4975(92)91019-6 doi:10.1007/s11748-009-0427-z
Fyler DC (1980) Report of the New England regional infant Kato H, Sugimura T, Akagi T et al. (1996) Long-term conse-
cardiac program. Pediatrics 65:375–461 quences of Kawasaki disease. A 10- to 21-year follow-
Ginde S, Earing MG, Bartz PJ et al. (2012) Late complications up study of 594 patients. Circulation 94:1379–1385
after Takeuchi repair of anomalous left coronary artery Katsumata T, Westaby S, Surgery C et al. (1999) Anomalous
from the pulmonary artery: case series and review of left coronary artery from the pulmonary artery: a sim-
literature. Pediatr Cardiol 33:1115–1123. doi:10.1007/ ple method for aortic. The Annals of Thoracic Surgery
s00246-012-0260-5 4975:4–5
Gittenberger-de Groot AC, Sauer U (1983) Coronary arte- Kawasaki T (1967) Acute febrile mucocutaneous syndrome
rial anatomy in complete transposition of the great with lymphoid involvement with specific desquama-
arteries: a morphologic study. Pediatr Cardiol 4:15–23 tion of the fingers and toes in children. Arerugī
20 Gordon JB, Kahn AM, Burns JC (2009) When children with 16:178–222
Kawasaki disease grow up: myocardial and vascular Keith JD (1959) The anomalous origin of the left coronary
complications in adulthood. J Am Coll Cardiol artery from the pulmonary artery. Br Heart J 21:149–
54:1911–1920. doi:10.1016/j.jacc.2009.04.102 161
Gowda RM, Vasavada BC, Khan IA (2006) Coronary artery Kory WP, Buck BE, Pickoff AS et al. (1984) Single coronary
fistulas: clinical and therapeutic considerations. Int artery originating from the right pulmonary artery.
J Cardiol 107:7–10. doi:10.1016/j.ijcard.2005.01.067 Pediatr Cardiol 5:301–306
Chapter 20 · Congenital Anomalies of the Coronary Arteries and Coronary Diseases
659 20
Kramer JR, Kitazume H, Proudfit WL, Sones FM (1982) Mumtaz MA, Lorber RE, Arruda J et al. (2011) Surgery for
Clinical significance of isolated coronary bridges: anomalous aortic origin of the coronary artery. Ann
benign and frequent condition involving the left ante- Thorac Surg 91:811–814. doi:10.1016/j.athorac-
rior descending artery. Am Heart J 103:283–288 sur.2010.11.002; discussion 814–5
Krause W (1865) Über den Ursprung einer accessorischen Musiani A, Cernigliaro C, Sansa M et al. (1997) Left main
A. coronaria cordis aus der A. pulmonalis. Zeitschrift coronary artery atresia: literature review and thera-
für Ration Med 225–227 peutical considerations. Eur J Cardiothorac Surg
Kuroczynski W, Kampmann C, Kayhan N et al. (2008) 11:505–514
Anomalous origin of the left coronary artery from the Mustafa I, Gula G, Radley-Smith R et al. (1981) Anomalous
pulmonary artery: mid-term results after surgical cor- origin of the left coronary artery from the anterior aor-
rection. Clin Res Cardiol 97:266–271. doi:10.1007/ tic sinus: a potential cause of sudden death. Anatomic
s00392-007-0621-x characterization and surgical treatment. J Thorac
Li D, Wu Q, Sun L et al. (2005) Surgical treatment of giant Cardiovasc Surg 82:297–300
coronary artery aneurysm. J Thorac Cardiovasc Surg Nair KKS, Zisman LS, Lader E et al. (2003) Heart transplant
130:817–821. doi:10.1016/j.jtcvs.2005.04.004 for anomalous origin of left coronary artery from pul-
Limbourg M (1937) Über den Ursprung der Kranzarterien monary artery. Ann Thorac Surg 75:282–284; discus-
des Herzens aus der Arteria Pulmonalis. Beitr zu path sion 284–5
Anat u zu allg Path 100:191–194 Neches WH, Mathews RA, Park SC et al. (1974) Anomalous
Loukas M, Groat C, Khangura R et al. (2009) The normal and origin of the left coronary artery from the pulmonary
abnormal anatomy of the coronary arteries. Clin Anat artery: a new method of surgical repair. Circulation
22:114–128. doi:10.1002/ca.20761 50:582–587. doi:10.1161/01.CIR.50.3.582
Malankar DP, Chauhan S, Das S, Bisoi AK (2010) How to do Newburger JW, Fulton DR (2004) Kawasaki disease. Curr
it: repair of anomalous left coronary artery from pul- Opin Pediatr 16:508–514
monary artery (ALCAPA) with coronary button transfer Newburger JW, Takahashi M, Gerber MA et al. (2004) Diagnosis,
and Lecompte maneuver. J Card Surg 25:225–227. treatment, and long-term management of Kawasaki dis-
doi:10.1111/j.1540-8191.2009.00994.x ease: a statement for health professionals from the
Mavroudis C, Harrison H, Klein JB et al. (1988) Infant ortho- Committee on Rheumatic Fever, Endocarditis and
topic cardiac transplantation. J Thorac Cardiovasc Kawasaki Disease, Council on Cardiovascular Disease in
Surg 96:912–924 the Young, American Heart Association. Circulation
Mavroudis C, Backer CL, Rocchini AP et al. (1997) Coronary 110:2747–2771. doi:10.1161/01.CIR.0000145143.19711.78
artery fistulas in infants and children: a surgical review Novick WM, Li XF, Anic D et al. (2009) Anomalous left coro-
and discussion of coil embolization. Ann Thorac Surg nary artery from the pulmonary artery: intermediate
63:1235–1242 results of coronary elongation. Interact Cardiovasc
McMahon CJ, DiBardino DJ, Undar A, Fraser CD (2002) Thorac Surg 9:814–818. doi:10.1510/icvts.2009.208215
Anomalous origin of left coronary artery from the Ogden JA (1970) Congenital anomalies of the coronary
right pulmonary artery in association with type III aor- arteries. Am J Cardiol 25:474–479
topulmonary window and interrupted aortic arch. Ojala T, Salminen J, Happonen J-M et al. (2010) Excellent
Ann Thorac Surg 74:919–921 functional result in children after correction of anoma-
McNeal-Davidson A, Fournier A, Scuccimarri R et al. (2013) The lous origin of left coronary artery from the pulmonary
fate and observed management of giant coronary artery artery – a population-based complete follow-up study.
aneurysms secondary to Kawasaki disease in the Province Interact Cardiovasc Thorac Surg 10:70–75. doi:10.1510/
of Quebec: the complete series since 1976. Pediatr icvts.2009.209627
Cardiol 34:170–178. doi:10.1007/s00246-012-0409-2 Ozer N, Deniz A, Doğan R (2008) Left anterior descending
Meyer BW, Stefanik G, Stiles QR et al. (1968) A method of coronary artery originating from the pulmonary
definitive surgical treatment of anomalous origin of artery: a rarity suspected during echocardiography.
left coronary artery. A case report. J Thorac Cardiovasc Türk Kardiyol Derneği arşivi Türk Kardiyol Derneğinin
Surg 56:104–107 yayın organıdır 36:181–183
Mirchandani S, Phoon CKL (2005) Management of anoma- Panduranga P, Al-Mukhaini M (2012) Absent left main with
lous coronary arteries from the contralateral sinus. Int anomalous origin of all three coronary arteries from
J Cardiol 102:383–389. doi:10.1016/j.ijcard.2004.10.010 left aortic sinus: a previously undescribed combina-
Mohiddin SA, Begley D, Fananapazir L (1999) Myocardial tion of coronary anomalies. Heart Lung Circ 21:824–
bridging in children with hypertrophic cardiomyopa- 827. doi:10.1016/j.hlc.2012.04.015
thy. N Engl J Med 341:288–290 Peñalver JM, Mosca RS, Weitz D, Phoon CKL (2012)
Möhlenkamp S, Hort W, Ge J, Erbel R (2002) Update on Anomalous aortic origin of coronary arteries from the
myocardial bridging. Circulation 106:2616–2622. opposite sinus: a critical appraisal of risk. BMC
doi:10.1161/01.CIR.0000038420.14867.7A Cardiovasc Disord 12:83. doi:10.1186/1471-2261-
Morgagni G (1761) De sedibus et causis morborum per ana- 12-83
tomen indagatis. Apud M. C. Compère, lutetiae Qureshi SA (2006) Coronary arterial fistulas. Orphanet
Mullins CE, El-Said G, Mcnamara DG et al. (1972) Atresia of J Rare Dis 1:51. doi:10.1186/1750-1172-1-51
the left coronary artery ostium: repair by saphenous Redelmeier DA, Greenwald JA (2007) Competing risks of
vein graft. Circulation 46:989–994. doi:10.1161/01. mortality with marathons: retrospective analysis. BMJ
CIR.46.5.989 335:1275–1277. doi:10.1136/bmj.39384.551539.25
660 C. Haller and C. Schlensak
Red-Horse K, Ueno H, Weissman IL, Krasnow MA (2010) Surana SP, Doddamani S, Swaminathan A et al. (2012)
Coronary arteries form by developmental reprogram- Anomalous coronary circulation: left anterior descend-
ming of venous cells. Nature 464:549–553. doi:10.1038/ ing and left circumflex coronary arteries arising from
nature08873 the right sinus of valsalva. Echocardiography 29:E102–
Roberts WC, Robinowitz M (1984) Anomalous origin of E104. doi:10.1111/j.1540-8175.2011.01610.x
the left anterior descending coronary artery from the Swaye PS, Fisher LD, Litwin P et al. (1983) Aneurysmal coro-
pulmonary trunk with origin of the right and left cir- nary artery disease. Circulation 67:134–138.
cumflex coronary arteries from the aorta. Am J Cardiol doi:10.1161/01.CIR.67.1.134
54:1381–1383 Syed M, Lesch M (1997) Coronary artery aneurysm: a
Rodefeld MD, Culbertson CB, Rosenfeld HM et al. (2001) review. Prog Cardiovasc Dis 40:77–84
Pulmonary artery translocation: a surgical option for Takeuchi S, Imamura H, Katsumoto K et al. (1979) New sur-
complex anomalous coronary artery anatomy. Ann gical method for repair of anomalous left coronary
Thorac Surg 72:2150–2152 artery from pulmonary artery. J Thorac Cardiovasc Surg
Romp RL, Herlong JR, Landolfo CK et al. (2003) Outcome of 78:7–11
unroofing procedure for repair of anomalous aortic Tashiro T, Todo K, Haruta Y et al. (1993) Anomalous origin of
origin of left or right coronary artery. Ann Thorac Surg the left coronary artery from the pulmonary artery.
76:589–595; discussion 595–6 New operative technique. J Thorac Cardiovasc Surg
Sabiston DC, Neill CA, Taussig HB (1960) The direction of 106:718–722
blood flow in anomalous left coronary artery arising Taubert KA (1997) Epidemiology of Kawasaki disease in
from the pulmonary artery. Circulation 22:591–597 the United States and worldwide. Prog Pediatr Cardiol
Sahin T, Bozyel S, Acar E et al. (2012) A young patient with 6:181–185. doi:10.1016/S1058-9813(97)00188-4
coronary artery anomaly, whose left anterior descend- Tavaf-Motamen H, Bannister SP, Corcoran PC et al. (2008)
ing artery originated from the pulmonary artery, Repair of anomalous origin of right coronary artery
underwent cardiac arrest. Cardiovasc J Afr 23:e15–e18. from the left sinus of Valsalva. Ann Thorac Surg 85:2135–
doi:10.5830/CVJA-2012-037 2136. doi:10.1016/j.athoracsur.2007.07.006
Sakakibara S, Yokoyama M, Takao A et al. (1966) Coronary Taylor AJ, Rogan KM, Virmani R (1992) Sudden cardiac death
arteriovenous fistula. Nine operated cases. Am Heart associated with isolated congenital coronary artery
J 72:307–314 anomalies. J Am Coll Cardiol 20:640–647
Schleich JM, Rey C, Gewillig M, Bozio A (2001) Spontaneous Tsigkas GG, Kasimis GA, Karantalis V et al. (2011) Anomalous
closure of congenital coronary artery fistulas. Heart origin of the left main coronary artery from the main
85:E6 pulmonary artery in an elderly patient. J Card Surg
Schneider T, Rickli H, Gliech V, Maeder M (2006) Bland- 26:66–68. doi:10.1111/j.1540-8191.2010.01155.x
White-Garland syndrome and atrial septal defect – Tsuda E (2009) Coronary artery bypass grafting for coronary
rare association and diagnostic challenge. Clin Res artery stenosis caused by Kawasaki disease. Expert Rev
Cardiol 95:295–300. doi:10.1007/s00392-006-0369-8 Cardiovasc Ther 7:533–539. doi:10.1586/erc.09.29
Sese A, Imoto Y (1992) New technique in the transfer of an Tsuda E, Kitamura S (2004) National survey of coronary
anomalously originated left coronary artery to the artery bypass grafting for coronary stenosis caused by
aorta. Ann Thorac Surg 53:527–529 Kawasaki disease in Japan. Circulation 110:II61–II66.
Shah AS, Milano CA, Lucke JP (2000) Anomalous origin of doi:10.1161/01.CIR.0000138194.61225.10
the right coronary artery from the left coronary sinus: Tsuda E, Kitamura S, Kimura K et al. (2007) Long-term
case report and review of surgical treatments. patency of internal thoracic artery grafts for coronary
Cardiovasc Surg 8:284–286 artery stenosis due to Kawasaki disease: comparison
Sharbaugh AH, White RS (1974) Single coronary artery. of early with recent results in small children. Am Heart
Analysis of the anatomic variation, clinical importance, J 153:995–1000. doi:10.1016/j.ahj.2007.03.034
and report of five cases. JAMA 230:243–246 Turley K, Szarnicki RJ, Flachsbart KD et al. (1995) Aortic
Shinkawa T, Yamaguchi M, Yoshimura N, Oka S (2002) implantation is possible in all cases of anomalous ori-
Anomalous origin of the left coronary artery from the pul- gin of the left coronary artery from the pulmonary
monary artery A case using the autologous pulmonary artery. Ann Thorac Surg 60:84–89. doi:10.1016/S0003-
arterial wall graft. Eur J Cardiothorac Surg 21:105–107 4975(95)00355-X
Shivalkar B, Borgers M, Daenen W et al. (1994) ALCAPA syn- Ural E, Bildirici U, Celikyurt U et al. (2009) Long-term progno-
drome: an example of chronic myocardial hypoperfu- sis of non-interventionally followed patients with iso-
sion? J Am Coll Cardiol 23:772–778 lated myocardial bridge and severe systolic compression
Smith JC (1950) Review of single coronary artery with of the left anterior descending coronary artery. Clin
report of 2 cases. Circulation 1:1168–1175. Cardiol 32:454–457. doi:10.1002/clc.20570
doi:10.1161/01.CIR.1.5.1168 Van der Hauwaert LG, Dumoulin M, Moerman P (1982)
20 Smith A, Arnold R, Anderson RH et al. (1989) Anomalous ori- Congenital atresia of left coronary ostium. Br Heart
gin of the left coronary artery from the pulmonary J 48:298–300
trunk. Anatomic findings in relation to pathophysiology Villa E, Brancaccio G, Carotti A et al. (2005) Circumflex coro-
and surgical repair. J Thorac Cardiovasc Surg 98:16–24 nary artery from right pulmonary artery in hypoplastic
Su LS, Burkhart HM, O’Leary PW, Dearani JA (2011) Mitral left heart syndrome. Ann Thorac Surg 80:1919–1920.
valve arcade with concomitant anomalous left coro- doi:10.1016/j.athoracsur.2004.06.034
nary artery from the pulmonary artery. Ann Thorac Surg Waldo KL, Willner W, Kirby ML (1990) Origin of the proximal
92:e121–e123. doi:10.1016/j.athoracsur.2011.06.010 coronary artery stems and a review of ventricular vas-
Chapter 20 · Congenital Anomalies of the Coronary Arteries and Coronary Diseases
661 20
cularization in the chick embryo. Am J Anat 188:109– Wren C, Sullivan JJO, Wright C et al. (2000) Sudden death in
120. doi:10.1002/aja.1001880202 children and adolescents. Heart 83:410–413
Wiegand G, Sieverding L, Kaulitz R, Hofbeck M (2009) Yamanaka O, Hobbs RE (1990) Coronary artery anomalies
Transarterial and transvenous approach for transcath- in 126,595 patients undergoing coronary arteriogra-
eter closure of a large coronary artery fistula with the phy. Cathet Cardiovasc Diagn 21:28–40
Amplatzer vascular plug. Pediatr Cardiol 30:172–175. Yetman AT, McCrindle BW, MacDonald C et al. (1998)
doi:10.1007/s00246-008-9266-4 Myocardial bridging in children with hypertrophic cardio-
Williams IA, Gersony WM, Hellenbrand WE (2006) Anomalous myopathy – a risk factor for sudden death. N Engl J Med
right coronary artery arising from the pulmonary artery: 339:1201–1209. doi:10.1056/NEJM199810223391704
a report of 7 cases and a review of the literature. Am
Heart J 152:1004.e9–17. doi:10.1016/j.ahj.2006.07.023
663 21
Congenital Anomalies
of the Great Vessels
Gerhard Ziemer and Renate Kaulitz
References – 713
Chapter 21 · Congenital Anomalies of the Great Vessels
665 21
21.1 Introduction duct (later arterial ligament), has a rather typically
diminished diameter at birth. In the old days, this
There is a vast spectrum of congenital anomalies led to the naming «isthmus», in analogy to a geo-
affecting the major intrathoracic arteries. These graphical term for the narrow land connection
anomalies may be the result of a complicated between the Peloponnese peninsula and the Greek
embryologic development from primitive ventral mainland, the Isthmus of Corinth. Developmentally,
and two dorsal aortas and their interconnecting the relative aortic narrowing can be explained with
branchial arches (Dische et al. 1975). They also may the small fraction of circulating blood passing this
result from hemodynamic pathophysiology second- aortic segment in fetal circulation.
ary to intracardiac lesions interfering with originally The obstructive lesion at this site had been
normal or abnormal ontogenetic vessel develop- described for some time as stenosis of the aortic isth-
ment. For the final pathoanatomy, we may distin- mus. Although isolated significant hypoplasia of the
guish between two principle groups of lesions. One isthmus, resulting in hemodynamic obstruction,
group comprises autochthonous lesions of great ves- does occur, the typical lesion to deal with is a rather
sels (narrowings, interruptions, atresias, abnormal distinct narrowing, a coarctation at the junction of
connections) which result in hemodynamic symp- aortic isthmus and the further descending aorta, the
toms, while the other group reveals anomalous posi- latter receiving most of its blood flow in utero from
tionings of the aorta and its branches causing the arterial duct. When aortic coarctation is accom-
symptoms by compressing neighboring structures. panied by aortic arch hypoplasia, either only distal
Anatomical shortcuts leading to hemody- or complete arch hypoplasia, frequently intracardiac
namic shunts will only be dealt with in this chap- congenital heart disease is associated (▶ Chapter
ter when they occur as isolated lesions. «Ventricular Septal Defects», Sect. 14.6.3). In cases of
We will proceed according to the following arch hypoplasia, the isthmus usually has more pro-
division/classification: nounced hypoplasia as well.
The localized narrowing/stenosis is at the cra-
nial circumference of the open arterial duct or
55 Anomalies of the aorta later the arterial ligament where it is connecting
55 Anomalies resulting in aortic to the aorta. Former classifications distinguished
hypoplasia/stenosis between pre- and post-ductal coarctations as well
55 Anomalies causing compression of as empirically between adult or infant coarctation
neighboring organs types. According to the lasso theory, backed by his-
55 Anomalies of the pulmonary artery
tology (Elzenga et al. 1986), as well as by the flow
55 Anomalies resulting in pulmonary
theory (Rudolph et al. 1972), these classifications
artery hypoplasia/stenosis
should not be used anymore.
55 Anomalies causing compression of
Pathogenesis of aortic coarctation starts with
neighboring organs
abnormal fetal hemodynamics. It may or may not be
55 Congenital shortcuts involving major
associated with intracardiac left-sided obstructive
intrathoracic vessels
lesions. These lesions result in decreased blood flow
55 Left to right shunt
in the ascending aorta, aortic arch, and isthmus and
55 Right to left shunt
consequently increased flow through the main pul-
monary artery and finally the arterial duct into the
descending aorta. This increased flow causes ductal
21.2 Anomalies of the Aorta myoepithelial cells to migrate within the whole cir-
cumference of the arterial duct into the adjacent
21.2.1 nomalies Resulting in Aortic
A descending aorta. These prostaglandin-sensitive
Hypoplasia and/or Stenosis cells contract postnatally when the prostaglandin
serum level drops and the oxygen saturation rises.
21.2.1.1 Coarctation of the Aorta/ This contraction, physiologically meant to close the
Aortic Isthmus Stenosis ductus, in the presence of ductal cells in the aortic
Without being obstructive, the most proximal part circumference, will also narrow or even close the
of the descending aorta, between takeoff of the left aortic lumen. The mechanism for this localized ste-
subclavian artery and the junction of the arterial nosis can be viewed as a protrusion of the aortic wall
666 G. Ziemer and R. Kaulitz
into the lumen, caused by a lasso, leading to an ther on remain clinically asymptomatic for
intraluminal membrane once the inverted wall is months or years. A good clinical exam, however,
fibrosed. The term «coarctatio aortae», aortic coarc- should suffice to detect the disease early on (weak
tation, is a description of this process: the Latin pulses in lower extremities, hypertension of upper
word coarcere means pulling together. extremities, systolic murmur best heard paraver-
An important acute therapeutic option to tebrally next to the left scapula). Surgery in these
secure lower body perfusion in critical neonatal cases is elective; however, it should not be delayed
aortic coarctation is medical reopening of the significantly, as complications of long-standing
arterial duct and thereby diminishing the restric- not operated aortic coarctation, like irreversible
tion in the adjacent aorta: a prostaglandin infu- arterial hypertension or left ventricular hypertro-
sion paralyzes the ductal myoepithelial cells in the phy, reduce life expectancy.
ductus and aorta. A concomitant aortic arch The mechanism and cause of these late dys-
hypoplasia can be explained by fetal hemodynam- functions remain unclear. A humoral pathophysi-
ics; however, at no point can it be treated medi- ologic chain, based on the currently known
cally. It remains a point of discussion as to whether mediators for arterial hypertension, has been
or not a hypoplastic arch segment has growth excluded, and the existence of an intrinsic vascu-
potential once the aortic coarctation has been lar pathology is being speculated upon (Pedersen
taken care of (DeLeon et al. 1991; Siewers et al. et al. 2011). The patient group followed in this
1991). Therefore the surgical approach, to simul- paper, however, had been operated on between
taneously take care of both the coarctation and 1965 and 1985. Median age at repair had been
the hypoplastic arch if present, is the safe strategy. 10 years and 44 years at follow-up.
An isolated aortic coarctation can present clin- As technological advances in the field of MR
ically in various forms. The most serious form is techniques are being applied on a wider scale also
critical neonatal aortic coarctation. The acutely for the study of the post-coarctation aortas, subtle
developing aortic stenosis at ductal closure within but considerable differences can be detected in
the first days of life may lead to cardiac decompen- comparison to the normal aorta but also between
sation. Lack of collateral flow to the lower body different repair techniques, which might influ-
adds to rapidly increasing acidosis and multi- ence the long-term prognosis of these patients. It
organ failure. Intensive care treatment comprising becomes clear that abnormal pulse wave propaga-
of prostaglandin infusion, also, may stabilize the tion, even in the absence of measurable pressure
patient within hours. Surgical treatment can be gradients, is strongly influencing the cardiac
safely delayed and metabolic compensation and hemodynamics and the aortic pressure and flow
urine production can be anticipated. If medical conditions (Kenny et al. 2010). These abnormal
treatment does not result in immediate clinical hemodynamics may especially persist in those not
improvement of the patient, and echocardiogra- quite precisely defined moderately hypoplastic
phy shows only mild or no ductal opening, surgery arches in long-term follow-up (Liu et al. 2010; Lee
is indicated with urgency. An increased mortality et al. 2012).
has to be anticipated in these cases. A palliative Patients with native coarctation of the aorta
catheter procedure if more readily available than continuously develop a more and more complex
surgery in dramatic cases may be considered. and complete system of collaterals contributing to
More recent work has identified that patients lower body perfusion. Main collateral courses are
with prenatally diagnosed isolated coarctation the mammary and intercostal arteries as well as
have a more severe anatomical form of coarcta- thoracic wall muscle artery branches. They are
tion, often accompanied by borderline hypoplas- able to significantly diminish or even abolish an
tic left heart structures than the neonates with original pressure gradient at rest. Pulse quality
coarctation diagnosed only after the first week of and exercise pressure gradient will still be a source
age. The prenatally diagnosed neonates were more for clinical diagnosis.
likely to require extensive arch reconstruction
21 under ECC, and they had longer hospital stays kSurgical Access
(McCandless et al. 2012). An aortic coarctation Standard approach for coarctation surgery is a
that remains silent at ductus closure, not causing posterolateral thoracotomy in the fourth intercos-
hemodynamic or metabolic symptoms, may fur- tal space (. Fig. 21.1). In neonates with a
Chapter 21 · Congenital Anomalies of the Great Vessels
667 21
tions or plasties are planned. An advantage of
young and very young age is excellent elasticity of
the aorta. Special care has to be taken not to injure
the nerves next and close to the aorta (vagus
nerve, recurrent laryngeal nerve, phrenic nerve).
The extent of the dissection required depends on
the repair technique planned as well as on indi-
vidual pathoanatomic features. While only a lim-
ited dissection is necessary for a patch plasty (in
neonates, infants, and small children nowadays
rarely indicated), a resection of the coarctation to
be followed by an end-to-end anastomosis
requires an extensive dissection/mobilization of
the distal aortic arch as well as of the proximal or
even farther descending aorta. The first two sets
of intercostal arteries originating directly off the
isthmus are usually mobilized and temporarily
encircled with vessel loops, or they may be even
closed with a clip.
The Abbott artery coined by Robert Gross
after Maude Abbott (the author of the first book
on pathoanatomy of congenital heart defects
..Fig. 21.1 The curved, 4–5 cm skin incision needs suf-
ficient distance to both tips of the scapula and spine.
(Abott 1936)) quite often shows up as an anoma-
Closer to the spine, its course is almost parallel to it lous artery in coarctation. It originates from the
back of the aortic isthmus and can lead to quite
disturbing bleedings in case of accidental rupture.
bodyweight <3 kg, thoracotomy in the third inter- Even in a case of a supposedly «simple» patch
costal space may be advantageous. A limited, plasty, this could be troublesome.
curved skin incision starting 1 cm below the tip of A further abnormality asking for clinical com-
the left scapula and pointing cranially to end about plications is the aberrant subclavian artery, also
1 cm lateral to the vertebra gives sufficient access. called lusorian artery (lusorian from Latin lusus
Thoracotomy in neonates and infants is rather naturae, capricious act of nature; also, God’s joke).
simple, right in the middle of the intercostal space An aberrant subclavian artery arises as the last
chosen. When dividing the chest wall muscles, supra-aortic branch off the aorta and takes its
special care has to be taken in older children, ado- course to the opposite side of the arch passing
lescents, and adults, as intramuscular arteries as behind the esophagus. As its involvement in the
part of the collateral artery system in coarctation actual aortic narrowing of the coarctation is not
may have reached significant size leading to unde- clear at clinical presentation, extremity blood
sirable bleeding. pressure measurements may be misleading.
Neonates, infants, and small children can be Intraoperatively, cross-clamping of the distal aor-
ventilated with routine tracheal intubation for tic arch in these cases obstructs flow to both sub-
coarctation surgery. For older children some- clavian arteries (normal and aberrant) and
times, but for adults always, double-lumen intu- theoretically impairs the perfusion of the spinal
bation for isolated right-sided ventilation is cord even more than in cases of normal subclavian
required. arteries. A study aimed to determine the influence
Intrathoracic dissection starts with sharp or of this anomaly on coarctation repair in five neo-
electrocautery division of the mediastinal pleura nates did not result in paraplegia which most cer-
covering the proximal descending aorta. tainly can be attributed to a short cross-clamp time
Dissection extends further to the subclavian (Hjortdal et al. 2003), as it is our own experience.
artery and reaches caudally for about three inter- Another not infrequent variation of aortic
costal spaces, at times as far as the diaphragm. arch branching is the isolated origin of the left
The latter is required when long-segment resec- vertebral artery coming off as the third branch
668 G. Ziemer and R. Kaulitz
between left common carotid artery and left sub- repair, but other studies recommend heparin in a
clavian artery. While it neither is a health issue on dosage of 100 U/kg KG (Wright et al. 2005).
its own, nor should it complicate the procedure, it Both aortic cross-clamp and release lead to
must not be confused with the left-sided common significant and acute changes in left ventricular
carotid artery when the arch is dissected from the afterload. Whereas the mature myocardium toler-
left side. This would lead to an insufficient mobili- ates such an increase in the afterload, immature
zation of a hypoplastic aortic arch. neonates may develop acute cardiac decompensa-
A truncus bicaroticus which sometimes is tion (bradycardia and asystole). It is mandatory to
associated with a hypoplastic aortic arch should timely prevent this complication with pharmaco-
be diagnosed preoperatively also. Again, dissect- logical measures. In the presence of concomitant
ing from the left side, it could be mistaken for a atrial- and/or ventricular septal defects, aortic
left-sided carotid artery, and one could unneces- cross-clamping can be better tolerated as the
sarily feel required to further dissect for a bra- blood can be shunted into the pulmonary circula-
chiocephalic trunk. tion, thereby relieving the left ventricle’s acute vol-
In general all of these anomalies should be ume load and pressure overload. Release of the
described preoperatively by echocardiography in aortic cross-clamp is accompanied by an acute
neonates and infants or with any type of angiogra- decrease in coronary perfusion. This should be
phy in older patients. prevented by volume loading concomitant to
Regardless of the technique of repair chosen, clamp release. Immediate or intermittent clamp-
cross-clamping of the aorta is necessary. Steps of ing with stepwise reopening of the aorta can be
the procedure and the required close communica- necessary and useful.
tion with anesthesia may be described in detail. Following the correction, the intraoperative
gradient for the mean pressure should not exceed
2 mmHg measured in a femoral artery line. In
The invasive arterial blood pressure infants, a strong pulse in the descending aorta as
measurement has to be performed on the well as absence of a palpable thrill over the anas-
right arm (we prefer right radial artery) tomosis suggest a good result. An almost
because at least temporary occlusion of the unchanged blood pressure in the radial artery
left subclavian artery occurs during most after release of the descending aortic cross-clamp
procedures (Cave: aberrant right subclavian is extremely suspicious for a significant residual
artery). stenosis.
Intraoperative measurements seem to be not
very predictive for a successful operation in terms
An additional invasive blood pressure mea- of residual or recurrent coarctation. A recent ret-
surement distal of the coarctation in neonates, rospective study suggests that a noninvasive sys-
infants, and small children is not required as tolic gradient >13 mmHg at hospital discharge
long as the expected cross-clamp time for the has excellent discriminative accuracy in identify-
descending aorta will be safely less than 30 min. ing patients in whom subsequent recoarctation
If a longer clamp time has to be expected, espe- developed (Kumar et al. 2011). The authors also
cially in older patients or in cases of reoperation, frequently observed rapid growth of both the
a relevant ischemia of the spinal cord has to be ascending and the transverse aortas, which was
avoided, and it is essential to document the dis- associated with improvement in gradient over
tal perfusion during a test clamping. A distal time.
descending aortic/femoral artery mean perfu- The mediastinal pleura is closed with a con-
sion pressure of less than 40–50 mmHg at pro- tinuous suture. A significant pericardial effusion
batory cross-clamp in these cases requires an may have been drained by pericardial incision
assist procedure, e.g., partial left heart bypass or ventral of the ductus Botalli or the phrenic nerve
even full ECC, to as much as possible avoid already before aortic cross-clamping.
21 paraplegia, one of the worst complications of In neonates and infants, we do not place chest
this operation. tubes unless an air leak is present. The preplaced
While in our practice we do not heparinize pericostal absorbable sutures shall be tied during
during simple aortic cross-clamp for coarctation an inflation hold of the lungs.
Chapter 21 · Congenital Anomalies of the Great Vessels
669 21
The individual surgical techniques are dis- anastomosis after incision of the aortic arch should
cussed below: expand the concavity of the hypoplastic arch. Dis-
section usually starts at the proximal descending
Surgical Techniques for Coarctation of aorta and is continued circumferentially around
the Aorta the left subclavian artery. In absence of any side
55 Resection and end-to-end anastomosis branches at the proximal subclavian artery, with
55 Resection and extended end-to-end the exception of some adventitia vessels, a fast
anastomosis exposure can be expected.
55 Patch plasty (indirect Vossschulte
plasty)
It is important to dissect in direct contact to
55 Direct Vossschulte plasty
the adventitia of the aorta and its side
55 Interposition graft
branches in order to avoid any injuries to the
55 Extra-anatomic bypass
esophagus, the thoracic duct, and nerves
55 Subclavian-aortic (Blalock-Park)
(vagus, recurrent laryngeal). Sharp dissection
55 Aorto-aor tic (ascending to
with scissors or electrocautery is preferential
descending aorta)
over blunt dissection in order to avoid
55 Subclavian flap plasty (Waldhausen)
tearing off of aortic side branches. Bleeding
55 Reverse subclavian flap plasty
can be much better taken care of if they
originate from clean cuts rather than from
tears. Avoid spreading tissue close to arteries.
kResection and end-to-end anastomosis
This technique was independently developed but
successfully performed for the first time by Clar- The next step is to circumferentially dissect the
ence Crafoord (Crafoord and Nylin 1945) in distal aortic arch as well as the isthmus and also the
Stockholm, Sweden (October 19, 1944) and shortly arterial duct or ligament. The Abbott artery as men-
thereafter by Robert Gross (1945a) in Boston, tioned above is the only side branch which may
Massachusetts (July 6, 1945), after the first Boston originate from the back of the aorta (see «general
patient, operated on June 28, 1945 had died (Bob remarks» and Lerberg 1981) and which has to be
Replogle, personal communication). Since then it detected and occluded. The presence of a lusorian
was made more sophisticated and adjusted to the artery should have been detected preoperatively. A
different ages and types of pathology. Today, for separate origin of a vertebral artery in between the
neonates and infants, this is the most frequently takeoffs of the left common carotid and left subcla-
performed technique for coarctation repair. vian artery is more difficult to be recognized by pre-
The major advantage of this technique, besides operative echocardiography. Being aware of the
avoiding artificial material, is the complete resection possibility of this anomaly avoids confusion. The
of the ductal tissue that may be present in the com- circumferentially exposed aortic arch is retracted
plete circumference of the aorta at the coarctation with a loose vessel loop; we prefer leading the loop
site. Any remaining ductal tissue in the aortic wall ventral to the arch and dorsal around the isthmus
may become responsible for most of the early reste- inferiorly and dorsal around the subclavian artery
nosis following coarctation repair in neonates superiorly. In case of an extreme hypoplasia, the
(Ziemer et al. 1986). Extending this technique, it can loop should go around the arch proximal to the left
be also applied for taking care of accompanying carotid artery to optimize exposure (. Fig. 21.2).
hypoplasia of the distal or even proximal aortic arch. These maneuvers especially improve complete
mobilization of the back of the aortic arch.
kExtended end-to-end anastomosis The ductus arteriosus may be dissected before
In these cases, an extensive mobilization of the aor- or after the maneuver described above, depending
tic arch from the left even beyond the origin of the on the mobilization achieved versus required. In
left carotid artery – sometimes beyond the brachio- neonates, most often the duct is kept open with
cephalic trunk and the ascending aorta – is neces- prostaglandin infusion. In these cases, the ductus
sary as well as a quite distal dissection of the tissue is very vulnerable and manipulation should
descending aorta circumferentially. The following be avoided as much as possible. Once the decision
670 G. Ziemer and R. Kaulitz
a b
..Fig. 21.2 a, b The circumferentially exposed aortic arch is retracted with a vessel loop, crossing the arch ventral and
distal to the left common carotid artery. This course of the vessel loop allows for extensive dissection and mobilization
in preparation for an extended end-to-end anastomosis
Aorta
for closure is made, a circumferential polypropylene necessary, while clamping the aorta, they can be
suture is placed superficially into the ductal wall or closed either temporarily using Yasargil clips or
the ligamentum arteriosum on the pulmonary side, permanently with titanium clips. For temporary
which is tied simFultaneously with discontinuing occlusion in older patients, a vessel loop maneu-
the prostaglandin infusion if present. ver may be also adequate. We prefer a definitive
On the aortic side, a clip is applied and the duct collateral closure as a primary step with or without
will be cut. Then or already before, a prophylactic transection, depending on the exposure achieved.
second clip can be applied on the pulmonary side After complete mobilization, but before any
(. Fig. 21.3). Dividing the duct improves the aortic resection, the mechanical tension of the
approach to the descending aorta, which should be future anastomosis should be estimated by approx-
mobilized beyond the next two or three sets of inter- imation of the future resection sites, each being
costal arteries. In case of a good exposure, neither the grabbed with forceps. This also could be done dur-
clip on the aortic side nor duct division is required at ing a test clamping phase by approximating the
this point. After placing the aortic clamps, the duct proximal and distal clamps. Mobilization may be
21 can be cut en bloc with coarctectomy. extended thereafter if necessary to reduce the risk
The intercostal arteries of a neonate are easily of anastomosis strictures caused by mechanical
identified and clearly structured, but with advanc- tension. Cross-clamping the aorta proximally
ing age, they become big, winding, and friable. If always includes the subclavian artery employing
Chapter 21 · Congenital Anomalies of the Great Vessels
671 21
a b
..Fig. 21.4 a–c Clamps in place after coarctectomy and arch incision a. Dorsal «inner» suture line of the anastomosis
performed with the clamps approximated b. Final anastomosis, clamps removed c
one curving clamp. In cases of more proximal arch line with the suture material available. Even inter-
hypoplasia, the aortic cross-clamp will include the rupted single suture lines tended to scar down as
left subclavian and carotid artery (. Fig. 21.4a, b). materials like silk provoked a rather heavy foreign
Now the complete resection of the juxtaductal body reaction. Nowadays circumferential anasto-
segment of the aorta is performed. The cutting edges mosis with absorbable (polydioxanone suture, PDS
should be free of any ductal tissue. If further resec- [Ethicon]; neonates, 7/0; infants, 6/0; children and
tion and/or anastomosis is necessary, the distal adolescents, 5/0) and with nonabsorbable monofil-
clamp may have to be moved more caudally. Into the ament sutures (polypropylene up to 6/0) shows a
arch, an aortotomy is commenced in the concavity normal potential of growing, even when employed
of the aortic arch and it is extended to cover the as a continuously running suture. The 6/0 polypro-
complete hypoplastic segment. In case of a now pylene sutures obviously rupture during growth.
enlarged proximal circumference in comparison to For adults we use 4/0 polypropylene sutures.
the circumference of the descending aorta, a dorsal A closure of the isthmus followed by an end-
incision can be performed into the descending aorta to-side anastomosis of the descending aorta to the
out of the distal resection site to create a harmonic aortic arch (neonates, infants) provides a techni-
E-E-anastomosis including the subclavian artery. cal variation as described by Frank Hanley’s group
Occasionally a separate implantation of the subcla- (Rajasinghe et al. 1996) (. Fig. 21.5).
vian artery is performed distal to or directly into the
aortic anastomosis to facilitate aortic reconstruction. kIndirect Vossschulte plasty
In the 1960s and 1970s, a direct E-E-anastomosis In 1957 Vossschulte described a technique to
was not routinely performed in children, owing to enlarge the isthmus and the coarctation site by a
the fear of lack of growth of a circumferential suture plasty with an artificial patch, originally Dacron,
672 G. Ziemer and R. Kaulitz
a b
..Fig. 21.5 Alternative repair of neonatal aortic coarctation, complicated by distal aortic arch hypoplasia: Hanley repair.
a All vascular structures involved are circumferentially dissected and mobilized: aortic arch, distal ascending aorta, aortic arch
branches, descending aorta as far as possible, and arterial duct. Sites of resection or incision are indicated by dotted lines.
b Left subclavian and left carotid artery as well as proximal arch are occluded with fine Satinsky-type clamp allowing for unre-
stricted flow in brachiocephalic trunk. The aortic isthmus is definitely closed with two clips, the ductus and all ductal tissue con-
taining descending aorta are resected, and the aortic arch is longitudinally incised in its concavity. Pulmonary end of the ductus
is closed with clip and/or suture. c Final sites after end-to-side anastomosis of the descending aorta to underside of aortic arch in
its concavity (From Rajasinghe et al. (1996); used with permission)
later polytetrafluoroethylene (Vossschulte 1957). rysms and risk of dissection (Aebert et al. 1993).
The coarctation membrane was usually excised and This may be caused by the weakened aortic wall
the intima-media defect was often, but not always, following the resection of the intima-media mem-
oversewn. This technique originally was employed brane. It also may be due to the different elasticity
only for older children, adolescents, and adults. properties (anisoelasticity) of the patch material
For this patch-plasty, the so-called indirect used compared with the aortic wall, especially
Vossschulte plasty, a mobilisation of the aorta is when weakened by membrane resection (De
not necessary. This is advantageous in a case of Santo et al. 1987; Rheuban et al. 1986). There are
significant tortuous collaterals or major adhesions no reports on late aneurysm formation after
caused by former procedures. The length of the coarctation patch plasty in neonates or infants.
isthmus is unchanged and there is no risk of ste- In fact this kind of repair seems to experience a
nosis caused by mechanical tension (. Fig. 21.6). renaissance. Carl Backer and Gus Mavroudis used
21 The disadvantages of this technique became the procedure as an elective procedure in all
obvious over time in the follow-up of patients patients age 2–16 years, but unlike the original
receiving this operation at an age of more than method, the intima-media membrane will remain
14 years: all studies report development of aneu- untouched (Mavroudis and Backer 2003).
Chapter 21 · Congenital Anomalies of the Great Vessels
673 21
a b
c d
..Fig. 21.6 a–d Indirect isthmus (patch-) plasty according to Vossschulte: after proximal and distal aortic cross-
clamping and temporary occlusion of major collaterals entering the clamped aortic segment, longitudinal aortotomy is
performed, well covering the aortic isthmus and the coarctation site a. The membrane may be kept in place or cut out b.
If cut out, a continuous suture of the intima and media may cover the defect and reinforce the weakened wall c. Sutur-
ing the patch into place, standard rules apply: avoid oversizing and no tapered edges d. Nowadays, this technique
should be applied only exceptionally in lack of any other options
a b
B A
A
C
D D B
..Fig. 21.7 a, b Direct plasty according to Vossschulte. a Longitudinal incision commences in the aortic arch and
extends throughout the isthmus beyond the coarctation into the proximal descending aorta. b Suture of the longitudi-
nal incision in a crosswise fashion
advantage, as the long-term result depends on the tissue or insufficient proximal extension during
quality and diameter of the original anastomosis the primary operation) can usually be redone with
anyways. And again, smaller people have smaller a further more extended resection combined with
vessels! a proximal enlargement. If necessary, a sternot-
omy and extracorporeal circulation with circula-
tory arrest have to be utilized. Beyond the infant
The normal aorta is a rather delicate organ age, this kind of recurrent stenosis should not be
(Heinemann et al. 1997). treated in that way. Adhesions make the dissection
more difficult and can cause severe damage by
accidental injuries of related structures (phrenic
The operation should be performed in accor- nerve, recurrent laryngeal nerve, esophagus, tho-
dance with state-of-the-art general principles of racic duct). A repeat resection in these patients
vascular surgery. Outside neonatal, infant, and beyond infant age increases the mechanical ten-
child age, the operation should be only per- sion of the vessels because of a postoperative
formed in simple clamping, when a sufficient fibrosis with the aortic tissue not being elastic any-
arterial pressure in the descending aorta is con- more. This leads to a higher risk of another recur-
tinuously monitored (see above). In all other rent stenosis, if the re-resected aortic ends could
cases, a temporary bypass procedure should be be directly anastomosed anyways. Today these re-
employed. coarctations are a primary indication for interven-
tional dilatation and stent placement. It should be
kExtra-anatomic bypass in aortic isthmus kept in mind, however, that even after stent place-
stenosis/coarctation of the aorta ment, surgery may be necessary. Therefore, the
Extra-anatomic bypass procedures should not be length of the stent should be only as long as neces-
used in primary operations of an aortic isthmus sary to not further complicate future reoperation.
stenosis in children or adolescents. However, occa- In patients beyond infancy with a simple stric-
sionally it may be advisable even for older children ture of an anastomosis (without interventional
in repeatedly recurrent stenosis of the Isthmus, the option/success) and normal aortic arch and sub-
distal, or even in the proximal aortic arch to clavian artery, a Blalock-Park operation (bypass
employ an extra-anatomic bypass as a functional between left subclavian artery and descending
cure. For adults with a long-segment hypoplasia of aorta) through a left-sided thoracotomy can be
21 the isthmus, an extra-anatomic bypass procedure performed as a successful and simple treatment
can be appropriate even as a primary operation. (Blalock and Park 1944).
A recurrent stenosis in the first weeks after In general, the post-ductal descending aorta
neonatal operation (caused by remodeling duct can be laterally clamped in a generous way because
Chapter 21 · Congenital Anomalies of the Great Vessels
675 21
of the poststenotic reduced arterial pressure. We section of the descending aorta and the distal
recommend an extended circumferential expo- bypass anastomosis should be performed in accor-
sure of the aorta in the region of anastomosis or at dance to the abovementioned principles for the
least a vessel loop maneuver proximal and distal Blalock-Park bypass (Blalock and Park 1944).
of the planned anastomosis in elderly patients. Others prefer the intra-abdominal aorta for
After clamping, using an adequate large Satinsky the distal bypass anastomosis (Levy Praschker
clamp, the arterial pressure of the distal aorta has et al. 2008). For us, this approach should be
to be checked. Especially in redo procedures, an reserved for specific and exceptional cases due
additional monitoring line for the arterial pres- to the complexity of the additional intra-
sure should be placed in the femoral artery or abdominal/retroperitoneal exposure. To opti-
descending aorta in addition to the always inva- mize length and position of the intrapericardial
sively monitored right radial artery pressure. course of the bypass, recommendation is to
The prosthesis to subclavian artery anastomo- use two separated prostheses for the proximal
sis should be in end-to-side technique at the prox- and distal anastomosis which then can be
imal intrathoracic segment of the artery. If further adjusted and anastomosed in ideal length and
growth of the patient has to be expected, the pros- position, not compromising any intrapericar-
thesis (polytetrafluoroethylene or Dacron) should dial structure.
be given some redundancy. This, however, should
not lead to kinking! kWaldhausen-Plasty («subclavian-flap-
Of special value is the extra-anatomic bypass plasty»)
in cases of accompanying hypoplasia of the aortic Waldhausen’s subclavian flap technique requires
arch (so-called complex recoarctation). Here, the dissection and transection of the left subclavian
bypass directly connects the ascending and artery, as in the original Blalock-Taussig shunt.
descending aorta. Employing the longitudinally split open subcla-
For this ascending to descending aortic vian artery as an in situ patch was the first long-
bypass, several courses may be considered: term successful technique of correcting neonatal
To achieve a bypass in an «orthotopic» but coarctation (Waldhausen and Nahrwold 1966).
still extraanatomic position, in which the bypass Reports on obstructions of growth and mal-
runs parallel and anterior to the aortic arch, usu- functioning of the left arm following the section
ally extracorporeal circulation is required as of the subclavian artery, as already historically
some retrocardiac dissection is necessary. If reported for the classical Blalock-Taussig shunt,
extracorporeal circulation is needed anyways, to may be considered as a detriment. This is also true
treat associated intracardiac lesions, aortic dis- for the potential late occurrence of a subclavian
section can be performed on the beating heart steel phenomenon. The fact of leaving ductal tis-
without any additional myocardial ischemia. sue in the aorta is an important cause of rather
Daebritz et al. (1999) report about five patients early recurrence of coarctation in up to 25 % of
who received an orthotopic bypass in case of a neonates operated upon (Ziemer et al. 1986).
complex recoarctation through a left-sided tho- Today the Waldhausen plasty may be
racotomy without extracorporeal circulation. employed in infant coarct reoperations when
Some groups suggest an extra-anatomic trans- resection and end-to-end techniques or artificial
pericardial connection between the ascending and patch plasties have failed without interventional
descending aorta through a median sternotomy treatment options available. This may be espe-
(McKellar et al. 2007; Burkhart et al. 2011). The cially true when repair is required in cases of sep-
proximal anastomosis is performed on the right sis and the exceptional case of local mycotic
side of the ascending aorta employing a side-biting aneurysm.
clamp. For the distal anastomosis, the descending After dissection, the subclavian artery is tran-
aorta is dissected transpericardially under careful sected proximal to the origin of the vertebral
retraction of the cardiac apex, using the OPCAB artery. The further incision line is performed
tools (e.g. vacuum, see also ▶ Chapter «Coronary along the lateral side of the subclavian artery,
Artery Disease», Sect. 22.4.2.1, and . Fig. 22.19). extended beyond the isthmus and the coarctation
These groups claim an excellent approach to the into the descending aorta (. Fig. 21.8a, b). Once
supradiaphragmal descending aorta. The local dis- sutured into place, it has to be made sure that the
676 G. Ziemer and R. Kaulitz
a b
c d
..Fig. 21.8 a–d Classic a, b and «inverse» c, d subclavian flap plasty. The incision extends clearly beyond the coarcta-
tion. As well as with the Vossschulte plasty, the membrane stays intact, or if resected, it is sewn from the inside (see
. Fig. 21.6c) a. After turning down of the laterally incised proximal left subclavian artery, it is sewn into the incised aorta
as a pedicled patch b. The «inverse» Waldhausen plasty is useful as a complementary technique in case of a long-seg-
ment hypoplastic distal aortic arch. In this technique, the left subclavian artery has to be incised medially to create the
pedicled patch required for the arch c. The localized coarctation is dealt with by a resection and end-to-end anastomo-
sis, not requiring much dissection d
subclavian flap reaches well beyond the ductal tis- we first would reevaluate the options for re-resec-
sue containing descending aortic wall, potentially tion and end-to-end anastomosis. A prosthetic
bypassing the later on occurring constriction of patch would be considered also before a subcla-
the ductal tissue at this location. vian flap in redo surgery as long as the segment to
Michel Ilbawi’s group modified the original be patched does not look severely scared and
Waldhausen technique by adding a direct therefore showing potential for future growth.
Vossschulte plasty (see . Fig. 21.7a, b) to create an The distally disconnected proximal left sub-
enlargement of the lumen and a reduction of clavian artery as a pedicled biological flap for the
length to be bridged by the subclavian flap (Allen aorta with growth potential is used also as an
et al. 2000). Asano in principle adds resection of additional or independent component of different
the coarctation with direct suture to be completed techniques (Zannini et al. 1993). The «reverse
by the subclavian flap (Asano et al. 1998). subclavian flap» technique (. Fig. 21.8c, d) as
Currently we would see an indication for the described by Hart and Waldhausen (1983) is
«subclavian flap» technique only in the rare meant to be used in cases of a pre-isthmal aortic
21 infant who may require surgery for recoarctation stenosis. However the incidence of this variation
late after neonatal coarctectomy and end-to-end is minimal. This technique is also described by
anastomosis. But even in these rare events for Kanter to correct a hypoplasia of the distal aortic
surgery in the era of interventional cardiology, arch (Kanter et al. 2001).
Chapter 21 · Congenital Anomalies of the Great Vessels
677 21
As usual, it is important to transect the left
subclavian relatively distally, as the length of the In case of an extreme hypoplasia of the
proximal subclavian stump should bridge not proximal or the complete aortic arch, defined
only the segment to the left carotid but extend as less than 25 % of the diameter of the
into the proximal few millimeters of this vessel ascending aorta, transsternal surgery
as well. To create the reverse flap, the incision is employing extracorporeal circulation and if
carried along its medial border facing the left necessary deep hypothermic circulatory arrest
carotid artery, and it is continued into the con- also is recommended even in the absence of
vexity of the hypoplastic arch into the facing any additional intracardiac defects.
aspect of the left carotid artery. Distal dissection
and transsection, however, must not impair col-
lateralsation. Therefore both thyrocervical trunk A particularly unfavorable arch branching
and costocervical trunk as important collaterali- variant, especially for decision-making regarding
sation arterial branches of the subclavian artery surgical approach, is the common origin of the
have to be saved. brachiocephalic trunk and the left common
A more recent variant for this indication fol- carotid artery (truncus bicaroticus). When asso-
lows the same technical principle, but preserves ciated with coarctation, the distal aortic arch is
the left subclavian artery: After incision of left extremely long and accordingly hypoplastic. The
subclavian artery, convexity of the aortic arch, available techniques of extended plasties of the
and the left carotid artery, a prosthetic patch is aortic arch with or without E-E anastomosis may
interposed. In this way, the distal arch is work very well, when the clamped arch allows for
enlarged and both the takeoffs of the arteries are a good proximal lumen for which to end the
pushed distally (Dave et al. 2012). In shorter plasty in. An intimate relationship of brachioce-
distal arch hypoplasia, both the cut open left phalic trunk and left carotid artery with a severely
subclavian artery and left common carotid hypoplastic distal arch from its origin may have a
artery may be sutured not only to the opened transsternal approach to be considered also.
distal arch but also to each other, creating a so-
called V/Y plasty which is used in vascular sur- kAortic coarctation as a part of complex
gery for vessel stenoses immediately behind intracardiac anomalies
bifurcations (own experience). This creates a Only about a third of isolated coarctation surgery
common trunk for the left subclavian and com- used to be done in patients with normal intracar-
mon carotid artery which at the same time diac anatomy (Ziemer et al. 1986). As already
enlarges the distal arch. mentioned above, the most important causes for a
hypoplastic isthmus or arch including coarctation
kAssociated hypoplasia of the aortic arch of the aorta are pathologic intracardiac flow pat-
A certain degree of hypoplasia of the aortic arch is terns developing during fetal life. Therefore coarc-
frequently associated with coarctation of the tation is rather more frequently part of an
aorta. The normal diameters of the aortic arch intracardiac defect (Anderson et al. 1983; Rudolph
segments are described in percentage in relation et al. 1972). Furthermore, coarctation is especially
to the ascending aorta. The values are set by mor- a complementary part of complex cardiac defects
phologists, being 60 %, 50 %, and 40 % for the with any left ventricular outflow tract obstruction
proximal and distal aortic arch and the aortic or complete underdevelopment of cardiac struc-
isthmus, respectively (Moene et al. 1982). tures serving the systemic circulation. Those typi-
There are no clear morphological cutoff values cal complex heart diseases are:
established which would indicate surgical therapy 55 Shone complex (see ▶ Sect. 12.3.5.4; 16.3.1; 17.1)
for segments below these values. While some 55 Taussig-Bing anomaly (see ▶ Sect. 19.2.6.6)
studies have shown a potential for growth, other 55 Unbalanced atrioventricular septal defects
authors like us prefer the primary correction of (see ▶ Sect. 12.1.5.8; 13.6; 13.7.5.1)
the hypoplastic aortic arch. The «extended» E-E 55 Various forms of univentricular hearts .
anastomosis or the «reverse subclavian flap» (see (see ▶ Sect. 13.3.3; 13.7.1.2)
above) may enlarge the distal and if necessary the These complex congenital heart defects com-
proximal aortic arch also. bined with coarctation of the aorta usually
678 G. Ziemer and R. Kaulitz
become symptomatic in the neonatal period and of more than 10 °C to proceed with coarctectomy
require immediate medical and surgical attention. in patients planned for intracardiac repair also.
While the extent of the first surgery, correction or While any simultaneous intracardiac and coarc-
palliation, is to be assessed individually, surgical tation repair can be done via median sternotomy, this
correction of the aortic coarctation is always access clearly facilitates an additional repair of a prox-
demanded. If only the correction of the aortic imal hypoplasia of the aortic arch. This can be per-
arch/coarctation of the aorta is planned without formed either as a long extended anastomosis,
any intracardiac repair, a left-sided thoracotomy comprising the entirely cut open concavity of the aor-
may be performed. This approach also allows for tic arch (. Fig. 21.9), an anastomosis comparable to
pulmonary artery banding if required. an interrupted aortic arch repair (. Fig. 21.11), or one
Correction of intracardiac defects employing may perform a direct E-S anastomosis between the
cardiopulmonary bypass in neonates requires a descending aorta and the ascending aorta after clip
median sternotomy. Moderate hypothermia or if closing of the isthmus (. Fig. 21.5). While we find it
necessary deep hypothermic circulatory arrest almost always possible to perform some type of direct
with or without (our preference) isolated brain autologous connection/anastomosis, in certain cases,
perfusion may be used. If deep hypothermic cir- a patch plasty with either synthetic material as
culatory arrest is planned, arterial cannulation for polytetrafluoroethylene, a tissue-engineered patch, or
extracorporeal circulation is in the ascending pulmonary homograft wall may be performed.
aorta. In case of planning isolated cerebral perfu- An interesting patch material available for
sion also, the arterial access can be established arch reconstruction is autologous pulmonary
either via the brachiocephalic trunk using a PTFE artery wall harvested from the usually enlarged
prosthesis or via a direct cannulation of the distal pulmonary arteries in these settings (Roussin
ascending aorta on the right side. In both cases, et al. 2002). The postulated advantages as com-
the aortic clamp can be placed during cardiac/cir- pared to other patch materials used have not been
culatory arrest to allow for «isolated» brain perfu- proven yet. Furthermore, it has to be evaluated
sion via the brachiocephalic trunk. In neonates, whether the postulated superior growth potential
once on pump, the proximal descending aorta can for the whole arch justifies the risk to impair the
be easily reached after performing the pericardial integrity of pulmonary valve and/or branches.
incision during cooling. To facilitate circumferen- The group from Texas Children’s Hospital
tial dissection of the descending aorta farther dis- recently reported a novel technique to address the
tally, a C-clamp (Cooley clamp – «C» either hypoplastic aortic arch in patients beyond infancy,
representing Cooley or the form/shape of the when the elasticity of the involved vascular struc-
clamp) can be placed distal to the isthmus after tures could prevent an «extended» end-to-end
having sufficiently lowered the core temperature reconstruction (McKenzie et al. 2011). In fact this
to 25 °C. During this clamp phase, flow of ECC technique is a mirror image of their previously
should be lowered to 50 %. described «advancement flap» (Elgamal et al.
The cooling phase on ECC should be used to 2002). They transect the distal ascending aorta at
dissect and mobilize the aortic arch branches as the level of the arch. A longitudinal incision on the
far as possible and loop them with tourniquets. right anterolateral margin of the proximal ascend-
During cooling, a temperature gradient mea- ing aorta creates a tongue or flap, which is then
sured between intraesophageal, nasopharyngeal, anastomosed to the longitudinally incised under-
or tympanal on one side and intrarectal or intra- surface, the concavity of the aortic arch. The oper-
vesical (urine bladder) on the other side has to be ation is completed by a limited resection of
noted. High gradients may indicate an inadequate coarctation and isthmus, which is followed by an
circulation of the lower part of the body requiring end-to-end anastomosis incorporating the distal
an extended period of cooling (sometimes signifi- end of the ascending aortic patch/tongue. The
cantly longer than the usual 20 min used in any advantages speculated about with this kind of
case). In cases of undisputed anatomic and hemo- operation is the avoidance of foreign material
21 dynamic significant descending aortic narrow- bearing the risk of pseudoaneurysm formation.
ings, the temperature gradient on pump is not of Further speculation is a better elastic reservoir
importance. In cases of preoperatively question- function of the proximal aorta. Besides these theo-
able coarctations, we use a temperature gradient retical advantages, this approach has also practical
Chapter 21 · Congenital Anomalies of the Great Vessels
679 21
a b
Truncus brachiocephalicus
A. carotis communis sin.
Aortic
isthmus
Pulmonary
artery
..Fig. 21.9 a–c Trans-median sternotomy repair of aortic coarctation, with hypoplasia of the isthmus and the aortic arch.
Cannulation of the ascending aorta close, better opposite to the planned anastomosis/enlargement of the arch. Cannulation of
the ascending aorta is close, better opposite to the planned anastomosis/enlargement of the arch. The resection and incision
lines are visualized a. The arterial cannula is removed during deep hypothermic circulatory arrest (not always necessary). The
coarctation and isthmus are resected, the ductus transected, and the concavity of the aortic arch incision extends into the dis-
tal ascending aorta b. Final image after extended E-E anastomosis leading to enlargement of the arch also c
advantages in cases of recurrent coarctation With reaching the desired rectal temperatures
beyond infancy, where the challenging mobiliza- (<20 °C) and a cooling time of at least 20 min to
tion of the previous extensively manipulated ensure equal temperature distribution, the extra-
descending aorta can be avoided by performing corporeal circulation is arrested after the tempo-
the necessary extensive mobilization solely around rary closure of the arch vessels with tourniquets.
the proximal structures; but this technique always The blood is drained via the venous cannula into
requires extracorporeal circulation. the reservoir of the heart-lung machine. Usually
680 G. Ziemer and R. Kaulitz
a b
A. subclavia dextra
A. carotis communis dextra
A. carotis communis sinistra
A. subclavia sinistra
A. lusoria dextra
A. subclavia sinistra
..Fig. 21.10 a–d Classification of interrupted aortic arch (Celoria and Patton 1959)
early mortality is influenced by prenatal diagnosis, the systemic circulation has to be ensured as a first
which helps to present these patients in best possi- step of palliation as in the Norwood procedure.
ble condition for surgery, avoiding any period of Isolated correction of an interrupted aortic
metabolic and/or circulatory decompensation. The arch via a lateral thoracotomy required reopera-
diagnosis of an interrupted aortic arch dictates indi- tion in all of nine patients as reported by Karl
cation for urgent surgery in the stabilized patient. et al. (1992).
Despite the first neonatal repair of IAA/VSD Median sternotomy is the access of choice in
being performed already in 1970, selective correc- total repair of IAA/VSD.
21 tion of the aortic arch combined with temporary After completing the sternotomy, one can
pulmonary artery banding had been the method of often see only a very thin or missing thymus as a
choice for quite some years. We nowadays recom- sign of DiGeorge syndrome (microdeletion 22q11)
mend this only in cases of a single ventricle where especially with IAA type B. These children tend to
Chapter 21 · Congenital Anomalies of the Great Vessels
683 21
a b
arterial line
venous line
..Fig. 21.11 a–c Surgery for IAA type B. Cannulation: the delicate ascending aorta is cannulated on its right side, while the
descending aorta is perfused via the ductus arteriosus a. After removing one or both arterial cannulas, the E-S anastomosis
between the descending aorta and the distal part of the ascending aorta is performed, after complete resection of the ductal
tissue. The proximal incision should be carried out at the posterior-medial aspect of the distal ascending aorta b. The com-
pleted anastomosis, which can reach into the left carotid artery proximally and the left subclavian artery distally c
develop hypocalcemia early postoperatively which Alternatively, the ascending aorta/right carotid
demand special attention. artery/brachiocephalic trunk can be perfused via a
Similar to repair of complex aortic coarcta- PTFE tube anastomosed in an end-to-side fashion.
tion, the extra- and intracardiac procedure can be Routine use of a single ascending aortic cannula
performed in deep hypothermic circulatory arrest has also been described (Vouhé et al. 1990).
with or without an isolated cerebral perfusion. As said before, cannulation of the proximal
A special feature of extracorporeal circulation aorta can be performed also indirectly using a
in repair of IAA is double arterial cannulation: the polytetrafluoroethylene prosthesis sewn E-S to
ascending aorta is directly cannulated, while the the brachiocephalic trunk. For direct cannula-
cannula for the descending aorta is introduced via tion, it is important to choose the right-sided
the arterial duct. Flow to both cannulas is provided aspect of the ascending aorta in order to facili-
by a Y-connector. The selected cannulas have a size tate the aorto-aortic anastomosis, which ideally
not exceeding 8F, which is advantageous even in will be just opposite to the place of cannulation.
the case of a very small aorta (. Fig. 21.11a). These cannulations can be used as the only
684 G. Ziemer and R. Kaulitz
source for reperfusion and the rewarming pro- intending to only incise it. The purse-string sutures
cess after repair. of the cannulation site or additional sutures placed
We perfuse the distal aorta usually with a direct at the aortic wall during the cooling period can be
cannulation of the duct. Alternatively the cannula- helpful for orientation to perform a correct anas-
tion can be installed in a large pulmonary artery, tomosis free of torsion. Both the aortic ends are
closing off both branches of the pulmonary artery approximated after setting the initial stitches of a
with tourniquets when starting extracorporeal cir- continuous suture using a slowly resorbable mate-
culation. This, however, carries a risk of develop- rial (polydioxanone 7/0). Alternatively, both edges
ing acute pulmonary valve insufficiency on bypass. of the vessel can be fixed first followed by sewing
In preparing direct cannulation of the ductus, a the backside from the inside also (. Fig. 21.11b, c).
tourniquet is placed around the ductus. The can- A multicenter Congenital Heart Surgeons’
nula can be inserted through an oblique incision at Society study (McCrindle et al. 2005) investigated
the pulmonary end of the ductus. It is then pushed the potential technical causes of a recurrent obstruc-
in direction to the descending aorta and fixed with tion of the aortic arch in the context of the arch
the tourniquet, thereby closing the ductus. The reconstruction in 472 patients. A partial direct anas-
transection of the ductus is performed conve- tomosis complemented by a patch plasty was
niently during the cooling period, and the pulmo- reported as the optimal therapy. Using a polytetra-
nary stump is oversewn. Now the descending fluoroethylene patch was identified as an isolated
aorta is more easily accessible. If direct cannula- risk factor for later obstruction, and they advise to
tion of the ductus is chosen, the venous cannula use autologous pericardium or a homograft as an
should be inserted before, to be immediately able ideal patch material. In view of the heterogeneity of
to commence extracorporeal circulation. the participants and the few definite anatomic details
If not done already before going on extracorpo- of type and location of the restenosis described, the
real circulation, complete mobilization of the aorta results should be applied with caution.
and the supra-aortic branches should be carried In earlier times, when mobilization of the aorta
out during the cooling period, as described for and the supra-aortic branches was not extensively
complex coarctation before. A right-sided lusorian performed, different techniques to accomplish aor-
artery may have to be transected sometimes but tic continuity were suggested. For example, the duct
not necessarily; it can be used also as a «reverse was preserved (when persisting in older infants) or
subclavian flap» (s.a, surgery of an isolated coarcta- the left, distally detached carotid artery was turned
tion) for an aortic arch repair. In our experience, down to form a new arch (Monro et al. 1989).
we never needed to transect a left-sided subclavian Long-term results of the historical series were
artery during a correction of an IAA type B but is deemed acceptable, and it was concluded that
advocated generously by others to facilitate perfor- these techniques may eventually be also used
mance of the anastomosis. Under no circum- (Monro et al. 1996).
stances, both the left-sided subclavian artery and a The interposition of a prosthesis between the
right-sided lusorian artery should be transected, or ascending and descending aorta can be done
at least one has to be reimplanted. After mobiliza- without extended mobilization but will inevitably
tion out of the retroesophageal bed, a transected require an even more complicated reoperation in
right-sided lusorian artery can be anastomosed these originally very small patients.
orthotopically E-S to the right carotid artery, As a technical variation of a direct anastomo-
thereby creating a brachiocephalic trunk. sis, a side-to-side anastomosis between the left
After the cooling period is completed, the carotid and the left subclavian artery may be per-
supra-aortic branches are occluded with tourni- formed (in case of a type B). To obtain a wider
quets while deep hypothermic circulatory arrest is communication, the incision should be extended
initiated and all cannulas are removed. about 5–6 mm cranial. Although described in his
After complete resection of all ductal tissue textbook, Castaneda’s group didn’t see any need to
visible, the circumference of the distal aorta is ever perform it (Castaneda et al. 1994).
21 adjusted. The distal ascending aorta will be incised After closing the ventricular septal defect and if
on its left dorsolateral side if necessary including necessary, an additional communication at the atrial
the left carotid artery (type B). Placing and manag- level the cannula is reinserted in the ascending aorta,
ing the incision line in an empty vessel can be dif- which is now sufficient enough for the complete cir-
ficult. Beware of cutting through the vessel while culation. The various techniques of closing a ventric-
Chapter 21 · Congenital Anomalies of the Great Vessels
685 21
ular septal defect are described in detail in ▶ Chapter increased RV pressure after banding can accentuate
«Ventricular Septal Defects», Sect. 14.4. Here, the rel- the subaortic obstruction and result in cardiac failure.
evant characteristics of the posterior malalignment In IAA, the majority of authors recommend a
ventricular septal defect and the associated subaortic resection. The former group from Marie-
stenosis will be addressed more closely. Lannelongue (Serraf et al. 1996), as well as Vaughn
There are four routes to access the typical pos- Starnes’ group (Salem et al. 2000), claims that with
terior malalignment VSD in IAA. Certain authors their special suture technique they are able to pull
exclusively describe a right transventricular the subaortic septum from the left into the right
(Castaneda et al. 1994; Vouhé et al. 1990), a right ventricle. We doubt the effectiveness of this approach
transatrial (Bove et al. 1993; Serraf et al. 1996), a and deem the additional resection to be the most
transaortic, or a transpulmonary (Luciani et al. important part of the surgical treatment. For exam-
1996) approach. We think it is important to be ple, a short, thick septum cannot just be pulled over.
able to employ all four approaches as in the indi- We are not alone with this opinion (Jonas 2004).
vidual case it may be necessary to simultaneously The resection of the conus septum can be per-
use different routes for different parts of an other- formed via different approaches as mentioned above.
wise difficult accessible VSD. It is extremely important to visualize the defect, espe-
The decision to surgically address the subaortic cially in the region of the aortic anulus to avoid any
region is critical. A significantly deviated septum injury of the aortic valve in spite of a necessary resec-
can be clearly identified by preoperative echocar- tion close to the anulus. In an ideal manner, the
diography. However, there may be no subaortic resection should be performed right up to the anulus
pressure gradient preoperatively. Even immediately in a way that a suture of the septal patch can catch the
after correction, the flow conditions of the left out- rest of the muscle and the anulus. Using a small hook
flow tract may appear regular, while after or a deep-rooted suture to pull the conal septum, a
1–2 months, certainly in between the first years, a better exposure of the narrowing muscle for save
stenosis may develop in these cases (Apfel et al. resection can be achieved. In that case we perform
1998). Data to support clear decision-making at pri- usually an oblique infundibulotomy to inspect the
mary surgery do not exist. Some preoperative echo subaortic area, and occasionally we inspect through
studies try to predict development of a left ventricu- the aortic valve as well if doubt about the quality and
lar outflow tract obstruction by measurements of quantity of resection or potential damage exists. A
the original anatomy in absence of a preexisting sufficient and reliable transaortic resection is not
gradient. Suggested data indicating the need for possible and not advisable. The usually small aortic
surgical therapy are an absolute subaortic diameter anulus in addition to a frequently present bicuspid
of less than 3.5 mm and a subaortic area of <0.7 cm2/ valve limits exposure and increases the risk of dam-
m2 (Apfel et al. 1998) as well as a diameter of the age to the valve during resection, while inspection
aortic valve anulus of <4.5 mm (Salem et al. 2000). may be helpful in decision-making.
As shown by a Congenital Heart Surgeons’ In IAA/VSD with multistage hypoplasia of the
Society multicenter study (Jonas et al. 1994), a pri- left ventricular outflow tract, it is expedient to
mary subaortic muscle resection is connected with apply the surgical principle as suggested by Yasui
higher mortality. As in other multicenter studies (Yasui et al. 1987) and Ilbawi (Ilbawi et al. 1988).
before, a clear cause relation was not communi- The blood flow of the left ventricle is guided
cated. Postoperative subaortic stenosis is reported through the ventricular septal defect into the pul-
to occur in 26–57 % of most studies (Geva et al. monary artery, and an end-to-side anastomosis
1993). Therefore, the decision to perform a primary between the proximal pulmonary artery and the
subaortic muscle resection is left with the individ- ascending aorta respectively the concavity of the
ual surgeon operating on an individual patient, and aortic arch is performed. An additional conduit
it should include prognostic aspects also. connects the right ventricle with the pulmonary
This thought process is also important when artery. We call this variation also «Norwood-
treating coarctation/VSD with the VSD being of the Rastelli» procedure, knowing that the «Rastelli» is
posterior malalignment type (Kreutzer and Van inverse (Steger et al. 1998). The technical possi-
Praagh 2000). Should the malalignment be defini- bilities/options for an aortopulmonary connec-
tively identified, simultaneous intra- and extracar- tion are wide and manifold. Some groups perform
diac repair should be preferred over pulmonary a Norwood-like construction using the classical
artery banding and coarctation repair, as the or modified Damus-Kaye-Stansel anastomosis
686 G. Ziemer and R. Kaulitz
(McElhinney et al. 1997) or the modification by Different from other aortic lesions in pediatric
Lamberti (Lamberti et al. 1991). cardiac surgery, being mainly congenital defects
The Ross-Konno operation is an alternative to with a clearly explanation of embryologic genesis,
the Norwood-Rastelli procedure when lack of the «midaortic syndromes» are addressed also as
retrosternal space becomes an issue – an advantage being acquired (Takayasu arteritis) as a secondary
particularly in neonates. How the long-term fate of result of systemic diseases like neurofibromatosis
the autograft in aortic position is comparable or or Williams-Beuren syndrome (Connolly et al.
inferior to the Norwood/Damus-Kaye-Stansel anas- 2002). Arnot and Louw (1973) assume a missing
tomosis remains open for discussion. Still in discus- fusion of the bilateral dorsal arteries in the seg-
sion is also the choice between a staged approach for ments affected. Because of the almost regularly
these patients versus a primary repair. High-volume observed anomalies of the renal arteries in the
Norwood centers seem to prefer a temporary single- abdominal form (stenosis, multiple arteries) other
ventricle circulation like a classical Norwood proce- authors think of a renal developmental disorder
dure followed by secondary repair (inverse Rastelli causing this syndrome. Given the different gender
part) within 1 year. In order to avoid any interstage distribution of the Takayasu arteriitis and the
mortality, we prefer primary repair. noninflammatory related hypoplasia, a clear dis-
tinction from the inflammatory arteriitis is seen
kComplications (Eibenberger et al. 1993). The segmental postisth-
In case of a relapse, the subaortic stenosis is associated mal hypoplasia caused renovascular hypertonus
with higher mortality. Further complications worth analog the typical ISTA, but it is rarely accompa-
mentioning are a restenosis in the region of the aortic nied by congenital heart disease. Mesenteric isch-
arch and the airway compression. In the Toronto Sick emia is also observed only in rare cases. The
Children’s Hospital experience, greatest survival histological examinations predominantly show
occurred in those patients with uncomplicated IAA dysplastic changes of the media, intima, and espe-
who had repair since 1993 (5-year survival, 83 %). cially along the lamina elastica interna.
Freedom from reintervention for arch obstruction As a rule the disease is diagnosed after some
was 60 % at 5 years (Oosterhof et al. 2004). years, mostly around the 20th year of life although
The restenosis rate is up to 20–50 % in the most the disease is also watched in infants. Our youngest
published studies. We have to differentiate between patient requiring thoracoabdominal aortic surgery
at least two types of restenosis: a stenosis localized was 3 years old, after previous catheter interventions.
to the anastomosis region may be treated sufficient If only the distal descending aorta is involved
by a balloon dilatation. A diffuse tubular stenosis only, a simple aorto-aortic prosthesis may bypass
requires an operative procedure. A patch plasty the affected segment via a lateral thoracic or tho-
during circulatory arrest should be performed. In racoabdominal access. More complex revascular-
adolescents and adults, an extra-anatomic bypass izations may be necessary in cases of renal and
between ascending and descending aorta can be a visceral artery stenosis. The latter are usually
successful and permanently sufficient alternative, compensated for by a natural Riolan anastomosis.
as described by us for complex or recurrent coarc- A reconstruction using the saphenous vein or
tation (Heinemann et al. 1997). a plastic/artificial prosthesis has been failed in
children. In the largest published study so far, an
21.2.1.3 Segmental Hypoplasia autotransplantation or the use of the iliac artery as
of the Aorta («Midaortic graft material for the diffuse stenosed renal artery
Syndrome») is recommended (Stanley et al. 2006). In our
As far as morphology and etiology are concerned, experience also a reimplantation, at least of the
segmental hypoplasia of the aorta is a rather heteroge- left renal artery, direct into an aorto-aortic bypass
neous disease. It is based on a focal and diffuse hypo- is a possible alternative.
plasia of the descending aorta distal to the isthmus.
Most often only the abdominal aorta is 21.2.1.4 Cervical Aortic Arch
21 affected in this syndrome, and therefore it is This malformation is an aortic arch that developed
mainly described in textbooks of vascular surgery. from the third instead of the fourth branchial artery.
Its presence, although very rare, as hypoplasia or It also can be looked at as a persistence of the third
stenosis of the thoracic or thoracoabdominal arch with involution of the fourth arch. As an iso-
aorta caused the discussion in this chapter. lated defect, it may not cause any symptoms except
Chapter 21 · Congenital Anomalies of the Great Vessels
687 21
a pulsation felt at the base of the neck. This anomaly descending aorta. A systemic-pulmonary form
is described on the right as well as on the left side. provides ductus-like flow to the pulmonary artery,
However, it is more often associated with short- while a pulmonary to systemic form may be a
path stenosis, similar to coarctation, tubular hypo- source for at least part of systemic flow. In a signif-
plasia, or tortuosity, which may require an adequate icant number of reported cases, the normal aorta,
therapy. In view of the rarity and the morphological developing from the fourth pharyngeal artery,
diversity of the obstructed cervical aortic arch, shows a coarctation, a hypoplasia of the isthmus,
there is not really a kind of standard therapy. In or an interruption. In that case the parallel run-
neonates an E-S anastomosis of the descending ning, abnormal arch is important in compensating
aorta to the ascending aorta should be performed. the perfusion like a collateral vessel. Also, both the
Establishing the anastomosis in the distal ascending distal end of the fifth arch and the normal isthmus
aorta will not compromise the main bronchus of can obtain ductal tissue causing hemodynami-
the same side as the arch. In elderly patients, a patch cally relevant coarctation (Atsumi et al. 2001). In
plasty similar to a Vossschulte plasty (McElhinney the latter case, a correction as in case of a classical
et al. 2000) or an extra-anatomic (prosthetic) bypass coarctation is required. If necessary, a hypoplasia
(Walker et al. 2002) is recommended. of the aortic arch may be repaired with/by a lon-
An aneurysmatic dilation of the cervical arch gitudinal incision following by an S-S anastomosis
has been described in some case reports. In cervi- of both arches (Lambert et al. 1999).
cal arches, this complication seems to occur much A persistent fifth aortic arch as a systemic-
earlier than in normal arches. Most cases were pulmonary connection has been reported in asso-
reported in patients at an age between 30 and ciation with pulmonary atresia or interrupted
40 years (Mitsumori et al. 2008). A primary struc- pulmonary arteries. The «ductus-dependent»
tural wall abnormality of the embryologic third perfusion of the lung is confirmed by a morphol-
aortic arch may be causing this (McElhinney et al. ogy persistent fifth aortic arch. This anomaly is
2000). Additionally, cervical arches can be part of mistakenly named, preoperatively as well as intra-
a complete or incomplete vascular ring, requiring operatively, an atypical ductus arteriosus. Actually
treatment as described below. the fifth aortic arch doesn’t own a connection to
the aortic isthmus in contrast to the ductus arte-
21.2.1.5 ersistence of the Fifth Arterial
P riosus. This is relevant in the preoperative treat-
Arch (Fifth Aortic Arch or ment, as this «atypical» ductus does not require
Double-Lumen Aortic Arch) prostaglandin to maintain patency. Nevertheless,
This anatomic variant shows again the complexity cases with a prostaglandin-dependent systemic-
of the embryological development of the great pulmonary fifth arch connection have been
intrathoracic vessels. In the classic way, a persis- reported (Zartner et al. 2000). In one case, a pul-
tent fifth arch runs parallel and above the normal monary coarctation could be verified histologi-
left aortic arch. The fifth arch does not carry side cally (Khan and Nihill 2006).
branches. As it may just lead to a double-lumen The third variation, a pulmonary-systemic
aortic arch, it is in itself asymptomatic. connection, is the rarest form. It is seen with an
The origin of the additional fifth aortic arch interrupted aortic arch associated with aortic atre-
is at the distal ascending aorta, respectively, at sia. The persistent fifth aortic arch between the
the proximal concavity of the aortic arch oppo- pulmonary artery and the brachiocephalic trunk
site to the brachiocephalic trunk. The distal con- supplies the ascending aorta with the coronary
nection is in the region of the aortic isthmus arteries as well as the proximal normal aortic arch.
(Van Praagh and Van Praagh 1969). Besides the In such cases there exists a typical arterial duct
original form reported by the VanPraaghs, other simultaneously which guarantees the perfusion of
variations are described associated with malfor- the descending aorta as in any interrupted arch.
mations of the pulmonary artery or the «normal» There are two additionally known sources for cor-
aortic arch. Related to the function of the malfor- onary blood supply in IAA/aortic atresia with a
mated vascular structure, different forms exist as persistent fifth arch. A second form is described as
systemic- systemic, systemic-pulmonary, pulmo- a pulmonary aortic fistula that runs between the
nary-systemic, and mixed forms. As a systemic pulmonary artery and the aortic root on the level
to systemic variant, the fifth arch can be a second of the sinus of Valsalva (Donofrio et al. 1995). The
or the only arch connecting the ascending and third variation is a cycle of Willis- dependent
688 G. Ziemer and R. Kaulitz
r etrograde collateral perfusion without any addi- such as the trachea and esophagus causing short-
tional vascular structure (Tannous et al. 2006). ness of breath and sometimes difficulties in swal-
Neither pulmonary atresia nor aortic atresia/ lowing. In infancy with predominantly liquid
IAA requires special technical features in com- food as mother’s milk and a rather soft trachea,
parison with the typical ductus-dependent circu- their most common symptoms are respiratory in
lation. Regarding construction of an aortic arch, it origin. With increasing age, the difficulties in
could be an intriguing choice to include the cut swallowing/dysphagia come forward with the
open fifth arch as an autologous tissue patch to introduction of solid food causing more pro-
obtain potential growth for the reconstructed new nounced dysphagia, especially because the rela-
aortic arch. But given the rareness of the disease tive narrowings of the vascular structures increase
and the uncertainty of the actual growth poten- with the growth.
tial, a Norwood variant should be the accepted The typical symptom is an inspiratory stridor
traditional and familiar treatment of choice. at rest accentuated at exertion; but a global respi-
ratory insufficiency with dependence on mechan-
ical ventilation may be possible as early as
21.2.2 ortic Anomalies Causing
A immediately postpartum. The difficulty in swal-
Compression of Neighboring lowing solid food is the typical symptom in adult-
Structures hood. However, patients who never experienced
normal swallowing before, may not be able to
report their objective dysphagia.
21.2.2.1 Foreword The greatest challenge in diagnosing vascular
At first sight vascular anomalies causing compres- anomalies is to think of them in the differential diag-
sion of neighboring structures do not appear to be nosis of the relative heterogeneous symptoms often
simply categorized because of their diversity. and much more frequently caused by other diseases.
Berdon is responsible for naming them «Vascular Modern imaging tools (CT/NMR) improve the
rings, slings, and things» (Berdon and Baker 1972). accuracy of localizing defects. The timely and up-to-
The expression, which substantiated the difficult date implementation of surgery brings about imme-
issue of a summary, is later on used as a title of a diate regression and abolishment of symptoms in
chapter of the pediatric cardiology textbook by Bob most patients with excellent long-term results.
Freedom (Moes and Freedom 1992). A compre- The variations in the course of the thoracic
hensive embryologically based classification was aorta do not necessarily cause symptoms.
published by Jesse Edwards 1948, Edwards 1953. Contrary to other congenital heart diseases,
The development of the aortic arch and its anomalies of intrathoracic great vessels are only
branches from two dorsal and two ventral parts operated upon when clinical symptoms are pres-
and their interconnecting pharyngeal arches/arter- ent. However, there are two exceptions:
ies is substantiated by embryology. The classifica- 55 An asymptomatic vascular anomaly
tion suggested by anatomists and embryologists is associated with intracardiac defects requiring
intellectually perfectly able to grasp, but they have surgery anyways
only isolated clinical relevance because of their 55 An asymptomatic complete double aortic
numerous possibilities. A definite simplified clas- arch as it will become symptomatic with age
sification serving >95 % of the clinically described and growth but surgery is much easier in
variations was proposed within the Congenital neonates and infants
Heart Surgery Nomenclature and Database Project A new clinical challenge is the increasing
in 2000 (Backer and Mavroudis 2000): number of asymptomatic vascular rings being
55 Double aortic arch diagnosed already during fetal life (Mehmet Gule-
55 Right-sided aortic arch/left-sided arterial cyuz, pers. communication).
ligament
55 Aberrant origin of the right-sided 21.2.2.2 istory of Surgery for
H
21 brachiocephalic trunk/innominate artery Vascular Ring Formations
causing tracheal stenosis The first successful division of a double aortic
The common objective of these anomalies is arch was performed by Robert Gross on June 9,
the compression of the neighboring structures 1945 using the term vascular ring in the publica-
Chapter 21 · Congenital Anomalies of the Great Vessels
689 21
1966). In our view, with the exception of an aber-
rant brachiocephalic trunk, for all compressing
thoracic vascular anomalies, the approach, a lateral
thoracotomy, should always be performed on the
side of the descending aorta. The argument in favor
of such an approach is to obtain safe intraoperative
control of the structures to be cut, especially of the
descending aorta. A complete resection starts at the
descending aorta and extends beyond the esopha-
gus on the other side if not limited by specific ana-
tomic structures/details we are not aware of.
..Fig. 21.12 First vascular ring / double aortic arch divi-
sion: Excerpt from Page 102 of Dr. Gross’s Surgical case
logbook #1. On 6-9-45, in his third of four Patients to oper-
ate upon, he divided “……ligamentum arteriosum Divi- The key is to achieve effective control of the
sion of medial root of left subclavian artery». (Courtesy Dr. most sensitive/critical structure – the
Robert and Carol Replogle) descending aorta – which is of course more
accessible on the side of the descending aorta.
tion (Gross 1945b). It had been case three of the
day, preceded by an appendectomy, a right herni-
orrhaphy, and followed by a pyloromyotomy The resection of a patent vascular segment can
(. Fig. 21.12). The forms/findings of aortic arch be carried out by side bite clamping or under cer-
anomalies causing this problem and their indi- tain circumstances in cross-clamping of the
vidual treatment have been well delineated by his descending aorta above and below the structure
group early on (Gross and Ware 1946; Gross and to be resected. To perform a continuous running
Neuhauser 1951). The radical resection of the suture to close the resected structure, flush to the
encircling vascular and fibrotic structures was aorta should take only a few minutes, and for that
recommended by Hallmann and Cooley 1966 there is no need for further protective measures
(Hallmann et al. 1966). The importance of the like including a left heart assist.
Kommerell diverticulum (Kommerell 1936) as an It has to be mentioned that Carl Backer and
independent cause for recurrent symptoms was Constantine Mavroudis perform a division of any
recognized by different working groups over the vascular ring irrespective of the side of the
years (Backer et al. 2002) and addressed now for descending aorta via a left-sided posterior thora-
primary surgery. This has been practiced by us for cotomy, following the same radical principles for
more than 25 years now. resection as we do. They claim no safety issues
The first sizeable study of video-assisted thora- and in addition the advantage of reimplantation
coscopic repair of vascular ring anomaly was pub- of the divided ipsilateral subclavian artery.
lished by Redmond Burke and his working group The immediate intraoperative sign of effectively
in 1995, at that time still in Boston (Burke et al. having taken care of the symptomatic vascular ring
1995). Between 2002 and 2004, the first six vascu- is the significant retraction of the divided ring struc-
lar rings were operated upon employing a totally tures. Retraction is facilitated by preceding careful
endoscopic robotic-assisted approach by Pedro del circumferential dissection of these structures. If
Nido’s group, also in Boston (Suematsu et al. 2005). retraction does not occur, any residual, peri-esoph-
These approaches, however, do not meet the ageal, soft, and tissue-related finest structures/
abovementioned principles of radical resection. fibrotic bands have to be cut or divided as well, even
if seemingly harmless. This should avoid the devel-
21.2.2.3 im of Surgery for Vascular
A opment of adhesive scarring from previous distor-
Rings tions followed by recurrences. The remaining
The basic aim of surgery is definite relief of external vascular stumps (lusorian artery, ligamentum arte-
compression to the trachea and esophagus. This riosum, hypoplastic aortic arch) will be placed per-
should be achieved with most extensive resection manently beyond the esophagus if it doesn’t happen
of the compressing structures, especially by «clean- on its own right after ring division with or without
ing» the retroesophageal space (Hallmann et al. additional dissection. Vascular rings are associated
690 G. Ziemer and R. Kaulitz
Lig.
arteriosum
Lig.
arteriosum
Aorta
Aorta
A. pulmonalis A. pulmonalis
..Fig. 21.14 a, b Double aortic arch. The most common form of double aortic arch: right-sided aortic arch with a
hypoplastic left arch. The hypoplastic arch can become extremely hypoplastic or even atretic either between the supra-
aortic vessels or distal to them a. The rare form of a dominant left-sided aortic arch with a hypoplastic retroesophageal
segment of the right-sided arch with the descending aorta on the left b
A. subclavia
sin.
Lig.
arteriosum
A. pulmonalis dext.
aortic diverticulum
A. pulmonalis sin.
692 G. Ziemer and R. Kaulitz
Kommerell diverticulum
A. lusoria
sin.
Aorta
A. pulmonalis
A. lusoria
sin.
Lig.
arteriosum
A. pulmonalis dext.
Kommerell diverticulum
A. pulmonalis sin.
694 G. Ziemer and R. Kaulitz
diverticulum. Transthoracic surgery for the diver- diagnostic and technical principles should con-
ticulum may be also preceded by subclavian tinue to be applied.
artery revascularization. We primarily revascu-
larize only in elderly patients.
21.2.2.8 Compression Caused
21.2.2.7 Less Common Findings by an Aberrant Origin
of a Vascular Ring of the Brachiocephalic
The diversity of these findings published mostly Trunk/Innominate Artery
as a pathological case report or as an isolated clin- This anomaly is caused by an atypical distal take-
ical case (Moes and Freedom 1993; Ziemer et al. off of the innominate artery. It does not form a
1983) is quite understandable given the complex vascular ring. The artery leaves the arch distally
embryologic development. As these forms posteriorly. Coming from the left, it takes a course
account for only about 1 % of the clinical cases ventral to the right around the trachea and head-
with vascular ring malformation, they can’t be ing into a posterior direction. This at times causes
focused on individually. Their common feature is a compression of the trachea. The ventral indenta-
an unusual branching of the duct (right sided or tion of the trachea seen by bronchoscopy is
doubled) or an uncommon site of interruption, pathognomonic for this anomaly; no other vascu-
leading to interesting made-up nomenclature lar anomaly causes an isolated indent like that
like: «retroesophageal left aberrant innominate (. Fig. 21.17a).
artery» (Moes et al. 1996). The above-discussed
21
Chapter 21 · Congenital Anomalies of the Great Vessels
695 21
This anomaly is mainly seen in infants and should show an extension of the tracheal lumen,
causes a typical stridor. Even episodes of short ideally with disappearance of the pulsations.
apnea are described.
For minor stridor symptoms, the treatment is
mostly conservative because with the development 21.3 Anomalies of the Pulmonary
of the cartilaginous rings during the second and Artery
third year of life symptoms diminish or may even
disappear completely. This may be also due to the 21.3.1 Obstructive Anomaly
changing size relation of the structures with growth. of the Pulmonary Artery:
A symptomatic, significant tracheal stenosis (70– Coarctatio Pulmonalis/
80 %) or apneic spells during infancy and early child- Pulmonary Artery
hood should be an indication for surgical treatment. Coarctation
An established therapy is the classical ventral
truncopexy – tacking the innominate artery to A proximal stenosis of the left pulmonary artery
the sternum (. Fig. 21.17c) (Gross and close to the arterial duct was already known in the
Neuhauser 1948) – or, as a complex and more 1950s, but a systematic pathoanatomical and
risky alternative, the resection followed by a pathophysiological summary was first published
proximal reimplantation of the innominate in 1990 by the group of Leyden (Elzenga and
artery into the proximal aortic arch or the distal Gittenberger-de Groot 1986; Elzenga et al. 1990).
ascending aorta (Hawkins et al. 1992). The so-called coarctatio pulmonalis/coarctation
Transsternal resection and proximal reimplanta- of the pulmonary artery can be seen as a counter-
tion is our method of choice to treat the symp- part of the coarctatio aortae/coarctation of the
tomatic aberrant origin of the brachiocephalic aorta. It is frequently seen being associated with
trunk (. Fig. 21.17b). We always employ extra- anomalies comprising right ventricular outflow
corporeal circulation and mild hypothermia for obstruction (Luhmer and Ziemer 1993). A
this procedure. During the procedure, NIRS is reversed flow direction in the prenatal arterial
used to monitor brain perfusion. duct leads - analog to coarctation of the aorta - to
The original surgical approach described by a lariat/lasso, like loop of invaded contractile duc-
Gross is the left thoracotomy (Gross and Neuhauser tal tissue/cells in the pulmonary artery wall,
1948), but several approaches are possible. Other which subsequently cause pulmonary artery
authors prefer a right anterolateral thoracotomy with coarctation. Contrary to coarctation of the aorta,
a submammary incision line (Mavroudis and Backer coarctatio pulmonalis is not known as an isolated
2003). We perform a median partial upper sternot- lesion. Its significance may only develop after cor-
omy for this procedure. The argument in favor of rection or palliation of an intracardiac lesion, as at
such access is the safe anchoring of the transsternal the time of surgery pulmonary coarctation was
sutures performing the pexy. The narrowed opera- not present or being thought of. With an ideal
tion field achieved by a lateral thoracotomy seems to early postoperative result of RVOT reconstruc-
make it awkward and insecure in our mind. tion and an unrestrictive pulmonary bifurcation
When we do on parents’ request a pexy rather for a couple of weeks after neonatal repair, sud-
than a translocation, we use three U-figured denly occurring right ventricular strain can be
sutures augmented with patches of 3.0 or 4.0. caused by acute narrowing to almost closure of
Instead of a mere truncopexy, we perform an aor- the proximal left pulmonary artery. If the postop-
totruncopexy (. Fig. 21.17.c): we place the first erative obstruction caused by plastic remodeling
suture subadventially at the distal ascending aorta, of ductal tissue occurs more slowly, it may be
the second at the base of the innominate artery, hemodynamically tolerated and stay clinically
and the third at the distal trunk just above the silent for a long time. Then, however, it may have
innominate vein. The sutures are fed transsternally led to the persistence of hypoplastic capillaries of
through the right part of the sternum and tight- the affected side during the critical phase of post-
ened fast after the removal of the retractor. With natal pulmonary development. This delay in
that the pulse oximeter measurement applied at development may complicate future surgical
the right arm should be unchanged, and the bron- reconstructions as a permanent increase of pul-
choscopy which should be performed obligatory monary vascular resistance has already occurred.
696 G. Ziemer and R. Kaulitz
This is of special importance in univentricular cir- arteries should be discussed, as neonatal Blalock-
culations which may require Fontan-type surgery. Taussig shunts or central shunts involving the
Severity of the ductal tissue causing left pul- pulmonary artery branches can lead to secondary
monary artery stenosis correlates with the severity stenosis (Sachweh et al. 1998).
of the right ventricular outflow tract obstruction.
That means that patients with pulmonary atresia
(with or without a ventricular septal defect) reveal 21.3.2 ulmonary Artery Anomaly
P
the most severe manifestations of pulmonary Causing/Being Associated
artery coarctation. In these patients, preoperative with Compression/
diagnostic procedure is often limited: in most Hypoplasia of Neighboring
cases, they require preoperative prostaglandin Structures: Pulmonary
infusion relaxing the myoepithelial cells. Artery Sling
Prostaglandin infusion in duct-dependent pulmo-
nary circulation cannot be temporarily stopped Pulmonary artery sling is an uncommon anomaly in
under very close clinical and echocardiography which the left pulmonary artery takes its origin from
surveillance to get information about the potential the common, main pulmonary artery far distal to
extent of pulmonary artery narrowing. the right of the trachea, then running behind the
Therefore, neonatal repair of pulmonary atre- trachea and in front of the esophagus to the left side
sia, which also includes a plastic reconstruction of (see . Fig. 21.18a). Characteristically, this anomaly
the main pulmonary artery in most patients, should causes an anterior dent of the esophagus. Contrary
also routinely include arterioplasty of the origin of to vascular rings caused by abnormal courses and
the left and/or right pulmonary artery (with right- connections of the aorta, leading to problems with
sided or bilateral duct) as recommended by some swallowing, this anomaly is more often associated
authors (Castaneda et al. 1994; Kirklin and Barratt- with pathological modifications of the respiratory
Boyes 1993; Luhmer and Ziemer 1993). system. Sometimes there may be only a temporary
For pulmonary atresia patients receiving a tracheomalacia in neonates, which does not require
systemic-pulmonary shunt as the primary opera- surgical attention. More often, however, the respira-
tion, the strategy is not clearly defined. In case of tory symptoms in the presence of a pulmonary
a limited narrowing, we recommend to address artery sling are caused by associated stenosis of the
surgery with the aid of cardiopulmonary bypass respiratory tract due to more or less hypoplastic and
and to perform a patch plasty of the pulmonary ring forming tracheal cartilages. As this has to be
coarctation including installation of distal anasto- expected in about 50 % of these patients, preopera-
mosis of the central shunt in the patched area. tive imaging of the respiratory tract is mandatory in
If the significance of a pulmonary coarctation all cases of pulmonary artery sling.
cannot be certified by echocardiography, it is ques- For a long time, a mechanical cause of the tra-
tionable to perform a prophylactic pulmonary arte- cheal stenosis was assumed because the accompa-
rioplasty. As many as 30 % of cases with right nying stenosis was mainly seen in the area of the
ventricular outflow tract obstruction revealed pul- left pulmonary artery crossing the trachea. The
monary artery coarctation (Luhmer and Ziemer length of the stenosis/hypoplasia, however, can
1993). Patients after shunt surgery should be very extend even far proximal, and hypoplastic com-
carefully and aggressively followed by cardiology plete ring cartilages can be present in the full
and cardiac surgery to recognize any changes in pul- length of the trachea. The ring cartilages can reach
monary perfusion in order to timely repair any distal the carina also. The main bronchial system
developing stenosis to maintain and provide a suffi- is mostly inconspicuous. The therapy of such an
cient and balanced pulmonary arterial development. anomaly should not only include the correction of
The issue of secondary pulmonary artery coarc- the pulmonary artery course but also address the
tation, occurring after neonatal surgery, is further narrowed airways. If necessary, the complete seg-
discussed in ▶ Chapter «Congenital Heart Disease ment of the stenosed trachea should be resected.
21 with Anomalies of the Right Ventricular Outflow Nowadays a median sternotomy is performed
Tract» Sect. 15.6.2.3; . Fig. 15.4; . Fig. 15.14». as access of choice, although the original opera-
In postoperative patients, differential diagno- tion described by Potts (Potts et al. 1954) was
sis of an iatrogenic stenosis of the pulmonary done through a left-sided thoracotomy.
Chapter 21 · Congenital Anomalies of the Great Vessels
697 21
a b
..Fig. 21.18 a, b Pulmonary artery sling before and after orthotopic reimplantation
Usually extracorporeal circulation is estab- and occlusion of the anteposed vessels occurred,
lished. In case of an isolated pulmonary artery sling orthotopic reimplantation regained its popularity.
without intracardiac disease to be taken care of, the Tracheal surgery, at times involving the proxi-
operation can be performed in normothermia and mal right main bronchus, is indicated as a simul-
with the heart beating. With good venous drainage taneous procedure, adequate bronchoscopic and
and an empty right heart, ventilation is not required imaging findings provided. In planning and per-
which facilitates retroaortic and retrotracheal dis- forming this part of surgery, some principles are
section and final translocation of the left pulmonary notable:
artery. Even without intrinsic tracheal pathology – 55 In contrast to adults, the trachea in children
i.e., only tracheomalacia – but also in case of patho- is much better vascularized, and therefore an
logical, but sufficiently wide complete tracheal extensive dissection of the trachea is possible
rings, the proximal segment of the left pulmonary without risking ischemic complications to the
artery should be dissected and transected close to its same extent.
origin. Thereafter orthotopic reimplantation into 55 In infants the trachea can be resected in
the normal position pretracheal and left anterolat- a length of up to eight to ten cartilages
eral has to follow (. Fig. 21.18b). (or about half of the complete length) and
The origin of the left pulmonary artery is trans- still be anastomosed E-E after adequate
located and reanastomosed E-S far proximal into mobilization.
the main pulmonary artery in what one would con- 55 The region of anastomosis remains critical for
sider an orthotopic position. Preferably the area of restenosis caused by granulation tissue.
the former insertion of the ligamentum arterio- 55 An asymptomatic trachea albeit consisting
sum/arterial duct is used. Potts in his original oper- completely of pathological ring cartilages
ation described the reanastomosis of the left does not require surgical treatment.
pulmonary artery E-E into its origin on the right of
the trachea, now, however, being in a pretracheal For tracheal surgery, the following techniques
position. This technique resulted in an unaccept- may be applied:
able high occlusion rate of the left pulmonary 55 Simple resection and E-E anastomosis
artery. After a period in the late 1980s, where ortho- 55 Sliding plasty
topic reimplantation had been already the preferred 55 Insertion of a tracheal autograft
method to deal with the sling, there was a period 55 Using autologous tissue (pericardium, costal
where during the resection of the trachea only an cartilage) as an augmentation of the
anteposition ventral to the trachea was performed, reconstructed trachea
leaving the origin of the left pulmonary artery
untouched. This was thought to facilitate the com- As mentioned above, flexibility and good vas-
plex tracheal and vascular surgery. When stenosis cularization of the trachea in children allow for an
698 G. Ziemer and R. Kaulitz
a b c
extended resection and end-to-end anastomosis. sional active extensions of the neck cannot be
Because of the simple feasibility of the resection, it avoided; however, a temporary fixation of the
is our preferred technique, which allows a resec- chin at the chest – as sometimes performed in the
tion even up to 50 % of tracheal length in case of adult thoracic surgery – is not indicated.
hypoplasia. An extended mobilization of the tra- In cases of more extensive stenosis (more than
chea above and below the length of resection is 50 % of the tracheal length), which prevents a
required, and the procedure is carried out by «simple» resection, a sliding plasty can be per-
inflection of the neck. In some cases, it was help- formed (Beierlein and Elliot 2006). The stenotic
ful for us to involve an ENT surgeon performing a segment of the trachea is cut obliquely in a very
laryngeal release. We perform the anastomosis huge angle, almost longitudinally. Cutting the tips
with interrupted polydioxanone sutures. To facili- of both ends (being an equivalent of some central
tate a fast healing tendency and to reduce the cre- resection), the remaining sides are shifted and
ation of obstructive granulation tissue, some sutured longitudinally (. Fig. 21.19). In those cases
surgeons felt an advantage in using faster reab- of an additionally preexisting stenosis of the right
sorbing suture material such as Vicryl®. We seal main bronchus, a sagittal incision starting at the
the anastomosis with fibrin glue containing an upper surface of the right bronchus heading proxi-
aminoglycoside antibiotic. Although used by oth- mal to the left side of the trachea facilitates con-
ers, we so far did not employ any other tracheal comitant treatment of this problem (Jonas 2004).
anastomosis-protecting feature like a pedicled The tracheal autograft technique described by
pericardial flap. We deem it appropriate to main- Backer (Backer et al. 1998) uses the resected tra-
tain the inflection of the neck up to 7 days after cheal segment once longitudinally incised as an
surgery. For that the preoperative customized expanding patch anteriorly for a partial E-E anas-
21 production of a plaster frame is recommended as tomosis. This procedure effectively shortens the
well as the application of sedation should be con- length of required resection (. Fig. 21.20).
tinued. Nevertheless an early extubation should The rare form of a completely, in its entire
be encouraged, i.e., at the morning of the first length, hypoplastic and stenotic trachea may
postoperative day. We are well aware that occa- mandate an alternative technique like that
Chapter 21 · Congenital Anomalies of the Great Vessels
699 21
a b c d
..Fig. 21.20 a–d Tracheal autograft technique: a segment resected out of the midst of the hypoplastic trachea is
sewn as a ventral patch after performing the direct anastomosis of the posterior tracheal hemicircumference
described by Idriss. As such, this is an extended and sixth day of life. This is followed by anatomical
patch plasty using autologous pericardium reach- closure, an involution of the duct tissue that finally
ing over the complete length of the trachea if nec- becomes the ligament arteriosum, a thin fibrotic
essary. This technique has a high rate of restenosis, structure between the aortic isthmus and the prox-
and it requires postoperative trachea stabilizing imal left pulmonary artery in adults. A ductus not
ventilation for at least 2 weeks (Idriss et al. 1984). functionally closed by 3 months of age will most
Good long-term results are reported on a small probably stay open as a persistent arterial duct.
series of patients undergoing a similar procedure Position (right/left), number (1–2), and size of
employing autologous costal cartilage as described the ductus can vary, especially in the aortic arch
by Forsen (Forsen et al. 2002). anomalies as discussed above. This is also true for
conotruncal as well as obstructive cardiac defects.
However, in most instances, the ductus is solitary
21.4 Left to Right Shunt and left sided, even in the case of a right aortic arch.
It is not the intention of this chapter to discuss the
21.4.1 Ductus Arteriosus Botalli particularities of the ductus arteriosus with regards
to associated anomalies, but we note the absence of
The ductus arteriosus Botalli, the arterial duct, is an the duct in patients with a truncus arteriosus type
essential part of normal fetal circulation. As a phys- A1–A2 as well in patients with an agenesis of the
iological shunt in prenatal life, it allows for about pulmonary valve. Histologically and functionally,
90 % of the right ventricular output (over 60 % of the ductus arteriosus is a unique vascular structure.
the combined right and left ventricular output) to In prenatal life, histologically the vessel is a large-
bypass the lungs, which are not inflated anyways. In sized muscular artery with the media almost com-
the descending aorta, it combines with about 10 % pletely missing elasticity. The cells of the tunica
of the combined cardiac output coming from the muscularis are located circular with unique recep-
aortic arch through the isthmus into the descend- tors, which react to postnatal changes (rising oxy-
ing aorta. The intrauterine diameter of the ductus is gen partial pressure, reduction of prostaglandin
comparable to the diameter of the descending aorta level in the blood). The muscle cells promote with
or the main pulmonary artery. Functional closure their contraction the functional closure in 80 % of
occurs soon after birth, usually between the first the newborns mostly in the first 24 h postnatally.
700 G. Ziemer and R. Kaulitz
Additionally, the intima of the ductus shows a Patent resp. persistent ductus arteriosus will be
very good developed gelatinous matrix, which separately discussed for three different age groups
seals the closing lumen during/at the muscle con- with distinct therapeutic approaches: premature
traction. The final closure occurs within some babies, infants/children, and adults. Furthermore,
weeks caused by apoptosis of the muscular cells the special situations of an atherosclerotic duct, an
and proliferation of fibrotic tissue. At first the clo- infected duct, and of an aneurysmal duct will be
sure is completed at the side of the pulmonary dealt with.
artery; an ampulla-like bulging remains on/at the
aortic side for a few months and might lead to an 21.4.1.1 Patent Ductus Arteriosus
aneurysm of the aorta in rare cases later in life. of the Premature Baby
The early phase of the closing process can be This type has to be seen as a delayed closure of a
completely reversed by a postnatal infusion of pros- histologically normal duct. The ductal tissue is just
taglandin l. On the other hand, the muscular cells in as immature as the baby, which postnatally makes
a ductus of a premature baby show a limited ability the ductal myocytes not respond timely to the
to react to oxygen, which is one reason for the high higher oxygen or lower prostaglandin levels, as it
rate of a patent ductus in this group of patients. would occur in term babies. Because of the com-
The patent ductus (spontaneous or maintained) mon association with respiratory insufficiency and
as part of a complex congenital heart disease will be arterial hypoxemia caused by an immature lung, it
discussed in the corresponding chapters. may be also speculated about an insufficient stimu-
lus for ductal closure in some patients. Depending
on the hemodynamic influence of the patent duct in
As definite spontaneous closure of the duct the individual patient, surgery is indicated
can be seen clinically as late as at the end of (increased left heart volume load by a large left-
the third month of life, this point of time is the right shunt with persistent pulmonary hyperten-
relevant date for the final distinction between sion). It cannot be ruled out that among the majority
a mere patent (before) and a persistent (after) of these patents with a histologically normal duct,
ductus arteriosus, interestingly both the occasional abnormal duct may be found which
abbreviated PDA. might otherwise have become a persistent arterial
duct later on. But this is without any clinical impact.
The first surgical treatment of a patent duct in
The persistent ductus arteriosus is a distinct a premature baby was reported by DeCancq in
pathological-clinical entity. In contrast to the nor- 1963. Because of the dramatic improvement of
mal ductal wall tissue, elastic fibers in the media the clinical condition after surgery, the procedure
are well developed. It almost resembles what can was rapidly accepted as the standard therapy for
be seen in the media of the normal aorta. For that this situation (DeCancq 1963). Indomethacin as a
reason, persisting patency of the duct cannot be medical therapy was introduced in 1976 (inhibi-
explained by a delayed or incomplete closing, but tion of prostaglandin synthesis), and because of
rather with a different (patho-) histology to start positive results and reduced risks, the medical
with. treatment remains the primary therapy in prema-
Historically, surgery for a persistent arterial duct ture neonates weighing more than 1,000 g.
was the first successful operation to repair congeni- With lack of sufficient data, the discussion
tal heart disease. It was Robert Gross from Boston whether primary surgery or primary medical
Children’s Hospital who performed ligation of a therapy should be preferred for premies <1,000 g
symptomatic large-caliber arterial duct via a left- is ongoing. The only randomized series of a
sided thoracotomy in a 7-year-old girl on August 26, controlled trial of very early prophylactic surgical
1938 (Gross and Hubbard 1939) (see also ▶ Chapter duct closure is more than 25 years old (Cassady
«The History of Cardiac Surgery» Sect. 1.5). et al. 1989). This study could not show a survival
When Porstmann closed a persistent arterial benefit for surgery; however, it showed a signifi-
21 duct in catheter technique in 1966, the arterial cant reduction in the incidence of necrotizing
duct also became the first congenital heart defect enterocolitis in the surgical group. Significantly
to successfully undergo definitive interventional worse neurosensory outcome was seen after sur-
treatment (Porstmann et al. 1967). gical duct closure versus medical therapy alone as
Chapter 21 · Congenital Anomalies of the Great Vessels
701 21
a result from the trial of indomethacin prophy- dure. Typically, these monitoring measures are
laxis in preterms (Kabra et al. 2007). Interestingly, already in place as part of the routine NICU care.
the surgically treated patients had received almost Positioning the baby on its right side is facili-
50 % more indomethacin than the nonsurgical, tated by its diapers in the radiant warming bed.
medicine-only group (0.64 mg/kg vs 0.45 mg/kg). There is no further bending of the chest necessary.
The authors of this large multicenter study com- The right side of the chest lies flat on the mattress.
prising of centers in Canada, Australia, New The posterolateral thoracotomy should be kept
Zealand, Hong Kong, and the USA state that PDA short according to the extreme elasticity of the tis-
ligation is a risk factor for poor long-term out- sue and may be no longer than 3.0 cm in prema-
comes in extremely low birth weight infants. They ture infants with a weight of about 500 g. The
also state that although indomethacin prophylaxis exposure of the site is obtained by careful retrac-
will prevent a few PDA ligations, most of these tion of the inflated and relative stiff lung. This
premies will not benefit from prophylactic indo- maneuver can compromise the cardiac function,
methacin and may even be harmed. and close monitoring regarding the development
In these patients, there is a persistent urgent of bradycardia is required. In case of a repetitive
need for controlled trials with long-term follow- compromise of the circulation and decrease of the
up to better delineate the role of surgical versus heart frequency <100/min, which may occur very
medical closure of a patent arterial duct. In cur- rapidly, all instruments should be removed out of
rent practice, a surgical procedure is usually only the chest immediately, and a few minutes of
considered by neonatologists after two or three recovery should be allowed for. The dissection
indomethacin or ibuprofen cycles, and a symp- should be minimized also. Only some local inci-
tomatic duct remains patent. Given the undesir- sions of the mediastinal pleura cranial and caudal
able, especially renal side effects of indomethacin of the ductus are needed to apply a clip. The
and an only 50 % primary success rate of the edematous subpleural tissue of very symptomatic
drug-induced closure especially in premature patients further simplifies the dissection. The use
with a weight less than 1,000 g and definitely with of surgical loupes (2.5 better 3.5 magnification) is
a weight less than 500 g, a primary surgical ther- only mentioned for completeness. Circumferential
apy should be considered. Logistically even the dissection of the ductus is not only unnecessary; it
transport of these patients to the OR can be chal- clearly should be avoided for isolated ductus clo-
lenging. If possible, the operation should take sure in premature infants. The ductal tissue is
place on the neonatal intensive care unit because extremely fragile so that any direct manipulation
of the unstable thermoregulatory proportions and should be avoided. The recurrent laryngeal nerve
the often-critical respiratory conditions. This embracing the duct ventral of the vagal nerve
strategy advocated by us for many years is sup- (. Fig. 21.21) shouldn’t be manipulated either.
ported by clinical studies. They also showed that The clip should be applied midway of the duct to
the often-noticeable retrolenticular dysplasia may avoid any narrowing to the aorta or left pulmo-
be the result of an uncontrolled application of nary artery. For that the pulse oximetry system of
oxygen during the transport. The working group the lower extremities is useful. The clip should
in Philadelphia recommends further providing completely close the ductus. In case of an appar-
care to patients of outlying hospitals in their facil- ently incomplete closure, the removal of the clip is
ities by «traveling surgeons» (Gould et al. 2003). not recommended because of the risk of duct lac-
We are focusing further on maintaining the thera- eration. Instead a second clip has to be placed. If
peutical surrounding not only by performing the the application of the first clip induces any bleed-
procedure in the ICU at the bedside, but we also ing of the ductus, a second and better a third clip
foster an uninterrupted care and monitoring application also, one to each side of the original
throughout this procedure by an experienced clip, may save the situation.
neonatologist and its NICU staff. The advancement of clip technology allows for
A direct measurement of the arterial blood this approach as a routine. We check any clip
pressure during operation is neither practical nor applier for undistorting clip application on a
necessary. The ECG and the pulse oximetry sys- string outside the operating field before using the
tem of the upper and lower extremities should very same applier to actually close the duct.
deliver sufficient information during the proce- Although the majority of the premie duct closures
702 G. Ziemer and R. Kaulitz
a b
N. vagus
..Fig. 21.21 a, b Surgical site for a patent ductus arteriosus in a premature baby. After minimal dissection above and
below the ductus, only one clip is applied in the midst of the ductus
are performed as clip closure, they still may be drain should be primarily inserted. After a running
referred to as «ligations». Actually the first report suture of the fascia, the skin can be closed directly.
on premie ductus clip closures referred to «liga- We avoid conventional intracutaneous suture of the
tion with a clip» 30 years ago (Traugott et al. 1980). immature skin as this is often traumatic in these
An unpublished poll within the European cases; we also avoid adhesive dressings. We prefer a
Congenital Heart Surgeons’ Association in May nonacrylic tissue glue (Dermabond®) as a substi-
2013 (personal communication) regarding duct tute for a skin suture and wound dressing.
closing preference resulted for premies less than Any alternative surgical approach as interven-
1,000 g in 20 clip closures only, five ligations only, tion or video-assisted thoracic closure is not lon-
and two ligations plus clip when 27 members ger indicated. Even pioneering centers of
responded. A retrospective study comprising six video-assisted thoracoscopic ductus closure in the
surgeons in two centers in New Zealand over 1990s went back to the conventional procedure in
5 years demonstrated a significant reduction in these patients (Jonas 2004).
the operative time for clip use, absence of bleed-
ing (18 % in ligature group), and a trend in lower 21.4.1.2 Persistent Ductus Arteriosus
postoperative morbidity (Mandhan et al. 2006). in Infants and Children
In a standard situation, the chest should be This is the typical aorta-like ductus arteriosus which
closed without any pleural drain. Preplaced perico- depending on its size and effectiveness can cause a
stal sutures are tied during a forced inflation of the significant left-right shunt leading to heart failure
lungs, in order to guarantee a complete expansion with or without increased pulmonary pressure.
of the lungs. Irrigating the wound with normal Beyond the third month of life, the ductus does not
21 saline during the first ventilations after chest clo- show any tendency toward a spontaneous closure,
sure may detect or exclude any air. Any attempt to and therefore the diagnosis of a hemodynamically
close small air leaks in the visceral pleura in order relevant ductus should define automatically an indi-
to avoid any drain is useless. In these cases, a pleural cation for transcatheter closure or surgery. The
Chapter 21 · Congenital Anomalies of the Great Vessels
703 21
modern Doppler sonographic technology enables to vessel clamps, the ductus is incised right above the
identify a persistent ductus as small as a diameter of most prominent part of the palpable device. After
1–2 mm. These tiny vessels are asymptomatic, and a removing the device, the primarily placed sur-
prophylactic treatment due to threatening complica- rounding sutures and/or ligatures are tied.
tions of/like an endarteritis remains controversial
(Thilén and Aström-Olsson 1997).
During the last 10 years, the interventional clo- Rescue of a closing device after embolisation
sure has been established as a standard procedure in into the left pulmonary artery
this age group, preferably with a body weight of 1. Acute embolization: lateral thoracotomy in
6 kg or more, which, however, seems not to be man- the fifth intercostal space and oblique inci-
datory. Open surgery is performed only in excep- sion in the left pulmonary artery in the
tional circumstances or due to complications (s.b.). interlobar fissure.
Since the introduction of the Rashkind device (duc- 2. Late embolization or late notice, that means
tus occluder, «umbrella») in the 1970s, the inter- after more than 2 weeks: transsternal
ventional techniques for ductus closure have longitudinal incision of the distal main
become much more safer but also more extensive pulmonary trunk into the proximal left
and especially more expensive. The rate of compli- pulmonary artery employing
cations is very low. The occasional rescue surgery to normothermic extracorporeal circulation.
retrieve a lost device out of the pulmonary artery or 3. Cave: before removing the embolized device,
aorta, however, is far more extensive than a primary the closure/division of the ductus is required.
simple operative closure or division of the ductus.
An acutely embolized device usually ends up
in the lower lobe artery of the left pulmonary Video-assisted thoracoscopic ductus closure as
artery; this can be removed directly out of the first done by Laborde (Laborde et al. 1993) may
interlobar fissure (upper/lower lobe) via a postero- offer a cosmetically favorable alternative to an
lateral thoracotomy in the fifth intercostals space open surgical procedure. But as currently indica-
and an oblique incision of the artery during a tem- tion for surgery is limited to very small patients or
porary tourniquet closure. If the dislocation was to those cases with an apparently complicated
originally unnoticed and the embolized device anatomy, video-assisted technique may be
may be in its new position for more than 2 weeks, extremely laborious, and the benefits of such a
we recommend access via sternotomy and the use procedure are not demonstrated. Technically, the
of normothermic extracorporeal circulation with trocars are placed in the third or fourth intercostal
the heart beating. With the transsternal approach, space if possible along a line.
the ductus Botalli has to be closed before or with The classical closure of the ductus is performed via
ongoing bypass. After a longitudinal incision of a posterolateral thoracotomy in the third or fourth
the distal main pulmonary artery and the proximal intercostal space. The following rule of thumb – sim-
left pulmonary artery, the device can be retracted. ilar to the coarctation operation – approximately
A device still sticking in the ductus, however, applies: the lighter the patient, the higher the inter-
with a huge residual shunt can be removed via ster- costal space to be selected (<3 kg, third intercostals
notomy using extracorporeal circulation also. An space; >4 kg, fourth intercostal space; in between
off-pump approach via a typical left lateral thora- gray zone). The lung is retracted ventral by a spatula,
cotomy in the fourth intercostal space as usual for and the mediastinal pleura is opened longitudinally
primary ductus closure is also possible. But addi- right over the proximal descending aorta. The ven-
tionally to the procedure as for primary surgical tral edge of the pleura is elevated by stay sutures pro-
closure, the intrapericardial left pulmonary artery tecting the lung with a wet gauze pad. With this the
and the descending aorta have to be exposed proxi- lung spatula can be removed as they are no longer
mal and distal to the ductus to be able to perform necessary. The identification of the ductus, even
clamping maneuvers. Depending on the position of though it seems to be simple, may not always be
the intraductal device, sutures or ligatures may have achieved at the first sight. The first glance often
to be preplaced. After clamping of the great vessels addresses the ductus as the aortic arch. The recurrent
(aorta, if necessary even distal and proximal of the laryngeal nerve branching from the phrenic nerve
ductus branching) using Cooley clamps or straight and embracing the ductus from ventral/caudal to
704 G. Ziemer and R. Kaulitz
and Astrom-Olsson 1997). Therefore, the assumed be documented by ultrasound performed two to
risk of endocarditis as indication for ductus closure three times daily in the neonate. If regression does
in hemodynamically irrelevant arterial duct is not not occur or a partial thrombosis without closure
valid anymore for quite some time. For the rarely of the ductus persists (in the area of the aneurysm),
presenting infective PDA, in analogy to valvular surgery should be considered . This may also be
endocarditis, first-line treatment is antibiotics, and true for the rare cases with symptoms (Koneti et al.
surgery is only indicated if sepsis persists and/or 2011). Many in utero detected ductal aneurysms
PDA-associated circulatory problems persist/arise. resolve with ductal closure and thrombosis with-
The objective of the surgical therapy would not out clinical sequelae (Rutishauser et al. 1977). The
only be closure of the ductus but also the complete principal operative steps are the same as described
resection of the infected (vascular) parts of the ves- in ▶ Sect. 21.4.1.1, «Patent Ductus Arteriosus of the
sel. Providing a safe operation with the aid of car- Premature Baby», and ▶ Sect. 21.4.1.2, «Persistent
diopulmonary bypass is recommended. Ductus Arteriosus in Infants and Children» as the
Before and during establishing the extracorpo- aneurysm is constrained to the extent of the duc-
real circulation, manipulations at the pulmonary tus, not involving the connecting great arteries.
artery should have to be avoided. After clamping The generally elongated run of the ductus is easier
the ductus on the aortic side and blocking of the to transect. If thrombus extending into the aorta is
peripheral pulmonary arteries, the pulmonary documented, the clamping of the isthmus and
artery itself may be opened to prevent peripheral proximal descending aorta with inspection of the
embolizations. After removing the vegetations, the aortic lumen is obligatory. Resected aneurysms
involved wall of the pulmonary artery is resected. may show histological anomalies, above all con-
If the ductal intima of the aortic side imposes itself nective tissue disease.
macroscopically as obviously infected, a local Aortic thromboembolism may occur even in
resection of this part is also necessary. In cases premature infants after clip application of a large
such as these, the approach via left anterior thora- ductus/ductus aneurysm. Aortic occlusion may
cotomy is suggested by Stejskal and Stark (1992) in be treated by a short-term medical rising of the
order to facilitate the establishment of extracorpo- blood pressure, pushing the acute/fresh thrombus
real circulation via the ascending aorta and the far peripheral by the bloodstream, in an ideal way
pulmonary trunk as well as the access to both the to the iliac/femoral vessels where it may dissolve
aortic isthmus and the left pulmonary artery. spontaneously. If the aorta remains symptomati-
cally obstructed or even occluded, surgical treat-
21.4.1.6 Aneurysm of the Ductus ment would be required, but was not necessary in
Arteriosus our own experience yet.
An aneurysm of the arterial duct may already be In our experience in two institutions over
present prenatally and demonstrable in fetal echo- 25 years, we have seen only one symptomatic aortic
cardiography. It also can manifest itself as a late thromboembolism during ductus surgery and one
complication of a persistent or incomplete closed spontaneous symptomatic pulmonary thrombo-
ductus, after surgery, intervention, and/or infection. embolism (Rauch et al. 2008), both in premature
infants. A surgically treated patient has been
kThe aneurysm of the neonatal ductus reported in one case elsewhere (Fripp et al. 1985).
With the advanced use of perinatal echocardiog-
raphy, a primary aneurysm is seen more often kLate Aneurysm
(mostly asymptomatic). The incidence in children An originally incomplete closure or a persistent
with genetic anomalies is higher. In 25 % of ductus Botalli can result in typical degenerative
patients, systemic diseases such as trisomy 21, tri- aneurysm with complications as thromboembo-
somy 13, Ehlers-Danlos syndrome, or Marfan’s lism and dissection/rupture at advanced age.
disease are present (Dyamenahalli et al. 2000). Besides the typical spindle-shaped aneurysm of the
Early on the indication for surgery was seen ductus, a diverticulum is also described compris-
21 very liberal because of an impressing diameter ing the aortic side of the ductus expanded like a
(Lund et al. 1992). Nowadays a primary conserva- trumpet. Misinterpretations as an aortic rupture of
tive treatment in the sense of watchful waiting is this structure after thoracic traumata are described.
recommended. The tendency of regression should At adult age the aneurysmatic dilatation can
Chapter 21 · Congenital Anomalies of the Great Vessels
707 21
involve the neighboring vessels and with that com- the proximal variation (type I, proximal defect,
plicate the therapy; however, the aneurysm even in . Fig. 21.23a), the window is directly located
late adult life can still be constrained to the original downstream of the sinotubular junction of both
duct area. Aneurysms of the pulmonary artery vessels connecting the left side of the ascending
connected with Eisenmenger syndrome caused by aorta with the right side of the main pulmonary
a patent ductus Botalli are also known in adults. artery. The distal variation is located farther
Clinical signs of a hemodynamically ineffective downstream linking the two great arteries at the
aneurysm of the ductus in adults are those of a tho- level of the takeoff of the right pulmonary artery.
racic mass: thoracic pressure sensation, a typical In relation to the aorta, the distal window can be
hoarseness indicating the affected recurrent laryn- identified in the back wall of the aorta (type II,
geal nerve. Bronchial obstruction in a 4 months old distal defect, . Fig. 21.23b).
(Roughneen et al. 1996) and vocal chord paralysis A third type, type III, is called the total defect
with phrenic nerve paralysis in a neonate (Berger (. Fig. 21.23c). Here, the complete aortic pulmonary
et al. 1960) have also been reported. Indications for septum between sinotubular junction and takeoff of
surgery are either hemodynamic reasons or symp- the right pulmonary artery is missing. Type IV is an
toms created by aneurysmal tumor growth. «intermediate version». The defect/window is
The operative procedure in adults may be located in between types I and II (. Fig. 21.23d).
complex as it may require aortic wall excision or The proximal and distal varieties correspond
a segmental resection and an implantation of a to the types I and II of the in most publications
prosthetic patch or a tube graft. The technical cited Richardson classification (Richardson et al.
details do not differ from principles of surgery of 1979). The third variety of the Richardson classifi-
an aneurysm of the descending aorta, and they cation matches what is now called hemitruncus,
may be straightforward also. an anomalous origin of the right or left pulmo-
nary artery directly from the aorta with two
essentially normal aortic and pulmonary semilu-
21.4.2 Aortopulmonary Window nar valves. The anomalous origin is usually oppo-
site to the side of the aortic arch.
21.4.2.1 Classification Associated cardiac malformations are present
This rare malformation of the great arteries can be in 50 % of the patients. Most of them are simple
defined as a communication between the ascend- isolated anomalies like a ventricular septal defect,
ing aorta and the main pulmonary artery with ASD type II, or a persistent ductus. Others are
almost no length, a «window», which is hemody- typical for the aortopulmonary window. An IAA
namically unrestrictive in most cases. (see also ▶ Sect. 21.2.1.2, «Interrupted Aortic Arch»)
Clinical symptoms and pathophysiology are as a very complex anomaly associated with aorto-
similar to truncus arteriosus, although these two pulmonary window was described first by Berry
defects derive from different embryological devel- et al. (1982). In this association, the IAA is usually
opments. While truncus arteriosus is a conotrun- of type A. The three branches of the aortic arch
cal malformation, the aortopulmonary window arise from the more or less normal-size ascending
results from an error in fusion of parts of the far- aorta. The right pulmonary artery seems to arise
ther distally located aortopulmonary septum. from the right side of the ascending aorta, while
The result is an oval hole between the ascend- the origin of the left pulmonary artery is located –
ing aorta and the pulmonary artery associated far away from that – at the left side of the pulmo-
with two separated and completely normal semi- nary trunk before the origin of the ductus Botalli.
lunar valves. Absence of the intracardiac ventric- The proximal type windows may be accompa-
ular septal defect and presence of these two nied by anomalies of the coronary ostia and their
semilunar valves are the only morphological dif- origins. The distal windows more often show
ferences to truncus arteriosus. complex spatial relations to the takeoff of the right
In the Congenital Heart Surgery classification, pulmonary artery.
Jacobs et al. (2000) suggested four different types Independent of their location, these defects
for aortopulmonary window based on three types cause a nonrestrictive left to right shunt on the arte-
as described earlier (Mori et al. 1978). These four rial level. This may result in a relative early rise in
types differ in their localization (. Fig. 21.23). In pulmonary vascular resistance (Doty et al. 1981). To
708 G. Ziemer and R. Kaulitz
a b
c d
..Fig. 21.23 a–d Congenital Heart Surgeons’ Society classification of aortopulmonary window (According to Mori
et al. 1978; Jacobs et al. 2000; used with permission)
prevent this, early corrective surgery in neonates sary. Cardiac catheterization is recommended in
should be considered even in compensated situa- patients beyond the third month of life to evaluate
tions. In truncus arteriosus type A, the primary mix- pulmonary vascular resistance.
ing occurs on the ventricular level, causing a mixed The diagnosis of an aortic pulmonary window
shunt; in case of an aortopulmonary window, there presents an indication for surgery. The only con-
is an exclusive left to right shunt with normal periph- traindication would be fixed Eisenmenger syn-
eral saturation, especially after reduction of neonatal drome in older patients.
pulmonary vascular resistance. As said before, how-
21 ever, pulmonary resistance may rise again early. 21.4.2.2 Surgical Treatment
Echocardiography is usually sufficient for The first successful surgical treatment dates back
diagnosis (Apitz et al. 2007). If coronary anomalies to 1948 when Robert Gross closed an aortopul-
cannot be ruled out, cardiac angiography is neces- monary window by double ligation. As depicted
Chapter 21 · Congenital Anomalies of the Great Vessels
709 21
in drawings in that publication, there obviously the aortic cross-clamp. If deep hypothermic cir-
had been some length to the window which allows culatory arrest is instituted, the place of cannula-
for this type of off-pump procedure (Gross 1952). tion is not as critical as the cross-clamp can be
Another procedure that was performed before the placed on either side of the cannula during arrest,
introduction of extracorporeal circulation was the and thereafter the defect is repaired.
division of the aortopulmonary connection after Venous cannulation depends on the presence
side clamping as reported by Scott and Sabiston of additional intracardiac defects to be taken care
(1953) and Flechter et al. (1954). Despite these of as well as on the perfusion technique used (with
reports about successful treatments, these tech- or without circulatory arrest). Independent of the
niques had limited application due to the anatom- anatomic location of the aortopulmonary window,
ical variations, and they remained risky. Only by both pulmonary arteries should be dissected cir-
employing extracorporeal circulation, this cardiac cumferentially and snared by tourniquets with ini-
defect can be routinely corrected in a safe and tiating extracorporeal circulation analog to the
reproducible manner with protection of the correction of the truncus arteriosus. In case of a
neighboring structures. From all the techniques, hemitruncus, only the aberrant pulmonary artery
some of them are still used. We may mention the coming off the ascending aorta has to be closed
separation and direct closure described by Cooley temporarily.
(Cooley et al. 1957) as well as the transaortic In contrast to the biarterial cannulation
(Deverall et al. 1969) patch closure. required for primary IAA, a single cannulation is
By definition this anomaly is a window, i.e., a sufficient in case of a Berry syndrome (s.a.)
communication without length, and it is not a because both parts of the aorta can be perfused
tubular structure. Sometimes, however, it can show via the nonrestrictive window. The temporary
some «depth», and its dimensions can be even closure of the pulmonary arteries with initiating
delineated in between the aorta and main pulmo- the extracorporeal bypass is self-evident.
nary artery. As the pulmonary artery is usually Dissection of the window can be carried out
enlarged, this anatomic communication can then during the cooling phase of bypass with the heart
be isolated with C-clamps on both the aortic and decompressed. In case of an aberrant right coro-
pulmonary sides without compromising the lumen nary artery out of the window or even out of the
of the great vessels. With that the window gets even pulmonary artery, its epicardial course may sug-
more of a length, and therefore the closure can be gest the best incision. With reaching the target
performed almost like a transection of the ductus temperature desired, the aorta is cross-clamped,
Botalli without installing cardiopulmonary bypass. and if applicable, cardioplegia is given. Thereafter
These must have been the circumstances of the first the window is opened, and aorta and main pul-
cases reported before the advent of cardiopulmo- monary artery are separated (. Fig. 21.24a, b).
nary bypass. This procedure may be still used in Relatively small (about 25 % of the aortal cir-
selected cases. One has to be absolutely certain that cumference) type I and type IV defects (see
the clamps will neither obstruct the lumina of the . Fig. 21.22a, d) can be closed directly at the aortic
pulmonary artery nor aorta and coronary arteries side. In case of an oval opening as in most cases, the
and they also will not interfere with the semilunar closure should be performed with a double-row
valves. If this is the case, suture closure of both suture parallel to the longitudinal diameter, given
sides will be safe. Even if we plan to go ahead with the attention to the valve, the coronary arteries, and
an off-pump approach in aortopulmonary win- the remaining diameter of the aorta.
dow, we have a circuit for extracorporeal circula- If the window is larger than 25 % of the aortic
tion ready to be connected on the OR table. circumference, patch plasties of both the aortic
In general, repair of an aortopulmonary win- defect as well as the pulmonary artery is recom-
dow is performed under direct vision as an on- mended, using pericardium or polytetrafluoroeth-
pump procedure with cardioplegic arrest. In case ylene. We use 0.4 mm thick polytetrafluoroethylene
of a proximal window, the ascending aorta may be (. Fig. 21.24b).
cannulated at the usual aspect. With a distal win- As an alternative, the window can be closed by a
dow, the cannulation should be performed as dis- patch either with a transaortic or pulmonary access
tal as possible, rather in the proximal aortic arch incorporating the risk of a remaining defect/leak as
to ensure a safe and nondistorting placement of in any intracavity closure of a communication.
710 G. Ziemer and R. Kaulitz
If the window is located more distally, it transaortic patch closure may be possible; how-
should be approached through an oblique inci- ever, right pulmonary artery reconstruction is less
sion of the aorta. The aortotomy facilitates the controlled and therefore less reliable.
exact excision of the posterior part of the window If an IAA is associated, the origins of the pul-
to the pulmonary artery. Confining the excision monary arteries are located far from each other,
to the dimensions of the window facilitates right detaining a direct reimplantation of the right pul-
pulmonary artery reconstruction, which should monary artery (. Fig. 21.25a). It is suggested to
be carried out with a patch plasty. A second patch connect the orifice of the right pulmonary artery
closes and reconstructs the aorta. A single intra-/ with the aortic pulmonary window via an intra-
a b
a A. pulmonalis sin. b
descending aorta
aortopulmonary window
ascending aorta
21
A. pulmonalis dext. intra aortic patch for tunnel of RPA
..Fig. 21.25 a, b Approach in case of an aortic pulmonary window associated with an interrupted aortic arch
Chapter 21 · Congenital Anomalies of the Great Vessels
711 21
aortic tunnel after performing a longitudinal aor- more or less severe cyanosis and/or insufficiency
totomy (. Fig. 21.25b). Anterior enlargement of of the heart.
the aorta may be necessary as already mentioned When viewed morphologically, these abnor-
in the original paper (Berry et al. 1982). After mal connections can be classified as simple or
resection of the ductal tissue, the distal aorta complex forms, depending on the number of arte-
should be anastomosed end to side to the almost rial inflow vessels (commonly pulmonary collat-
regular-sized ascending aorta. In our own limited eral arteries at times branching from the bronchial
experience, we preferred to reconstruct the aorta arteries). A solitary arterial inflow vessel identifies
and pulmonary artery separately with two patches. the simple form, while networks of arterial and
venous collaterals designate the complex form.
A more or less severe cyanosis without signs
21.5 Right to Left Shunt for intracardiac or known pulmonary disease is
typical for isolated congenital pulmonary arterio-
21.5.1 Pulmonary Arteriovenous venous fistula. Chest X-ray alterations of the lungs
Fistula in most cases are very discrete. The appearance of
intravenously injected air bubbles in the left
Pulmonary arteriovenous fistulas are a rare, but atrium detected by a contrast echocardiography is
correctable cyanotic disease. Their origins can be pathognomonic for the pulmonary arteriovenous
both congenital and acquired. The vast majority fistula. An additional CT or MRI or a conven-
of the congenital arteriovenous fistulas (about tional angiography is required to verify even the
70 %) are caused by an autosomal dominant anatomical details.
hereditary disease (hereditary hemorrhagic telan- Catheter interventional therapy can produce
giectasia, Rendu-Osler-Weber syndrome). This excellent results in cases of smaller fistulas with
telangiectasia primarily affects the skin and defined in- and outflow vessels. For the more
mucous membranes, but also visceral organs. It complex forms, surgical therapy is indicated.
rarely becomes symptomatic during the neonatal Closure of isolated large-sized fistulas can be per-
period, but does so with increasing age. About formed by ligation after careful dissection of these
35 % of the patients show pulmonary manifesta- very thin-walled and fragile fistulas. But more
tions (Gossage and Kanj 1998). often preference will be given for an anatomical
Acquired forms may be attributed to liver dis- lung resection (lobectomy, segmentectomy),
ease. It may also be seen in patients in the follow-up eliminating the abnormal connections and the
of pediatric/congenital cardiac surgery after cyanosis en bloc.
Kawashima- or bidirectional Glenn operation for In the extreme case of bilateral diffuse fistulas
the spectrum of univentricular hearts. In these causing severe cyanosis, bilateral lung
patients, hepatovenous blood is prevented from transplantation is the only feasible therapeutic
direct lung passage. The lack of a presumed «liver option (Svetliza et al. 2002).
factor» is thought to be the reason for the develop-
ment of these potentially ubiquitously existing pul-
monary fistulas. Frequently, however, these new 21.5.2 ulmonary Artery to Left
P
appearing fistulas may be restricted to one lobe only. Atrium Communication
These fistulous communications are dealt with
also in ▶ Chapter «Definite Palliation of Functional This very rare anomaly of a shunt between the right
Single Ventricle», Sect. 13.7.5.2. Due to their fre- pulmonary artery and the (superior aspect of the) left
quency and clinical implications for the Fontan atrium is reported in about 60 cases in the literature,
circulation, their treatment and first of all their most of them described beyond the neonatal period
primary prophylaxis continue to represent subject as a cyanotic disease which also may lead to systemic
of ongoing debate as to the optimal timing and thromboembolism (Chowdhury et al. 2005).
technical configuration of the total cavopulmo- According to a fatal case report (Verel et al.
nary connection both in the setting of normal or 1964), there have been two reports mentioning
azygos-dependent inferior vena cava drainage. one surgical survivor each before 1960 (Friedlich
Congenital fistulas are usually isolated pathol- et al. 1950; Taussig 1960), the earlier and first one
ogies. Depending on their size, they can cause a supposedly being operated by Alfred Blalock.
712 G. Ziemer and R. Kaulitz
RPA RPA
LPA LPA
aneurysm
aneurysm MPA MPA
RUPV LUPV RUPV LUPV
LA LA
RA RA
LV LV
RV RV
Type I Type II
RPA
RPA
pulmonary
venous confluens LPA LPA
RUPV
aneurysm
RLPV RUPV LUPV
aneurysm
RLPV
LLPV
LA LA
RA RA
LV LV
RV RV
..Fig. 21.26 Classification of the pulmonary artery to left atrium communication. Type I: direct connection between the
right pulmonary artery and the posterior aspect of the left atrium. Normal pulmonary veins. Type II: connection of the right
lower lobe pulmonary artery branch with the right inferior pulmonary vein («end to end») close to the left atrium. Agenesis of
the right lower lobe of the lung. Type III: connection with a retrocardiac pulmonary venous confluence that is not incorporated
into the left atrium. Type IV: aneurysmatic retrocardiac connection of the fistula receiving right pulmonary veins also. Normally
connected left pulmonary veins (According to de Souza e Silva et al. (1974) and Ohara et al. (1979); used with permission)
The neonatal form may manifest itself with a a proposed classification of three pathoanatomic
severe cyanosis, which cannot be managed conven- findings was already published in the 1970s by de
tionally. It has to be considered that this disease is Souza e Silva et al. (1974) and extended to four
probably underdiagnosed due to the lethal outcome types by O’hara et al. (1979; . Fig. 21.26).
immediately postpartum. This may change now The symptomatic neonatal form has already
with the increasing frequency of prenatal diagnosis, significant abnormal hemodynamics prenatally:
21 as it was the case in our experience (Meyberg- in addition to the obligatory right to left shunt of
Solomayer et al. 2009). This anomaly is currently the fistula there is flow reversal in the arterial
rarely mentioned in any of the important textbooks duct, leading to extreme biventricular cardiomeg-
of pediatric cardiologic or cardiac surgery although aly due to extreme volume overload of the heart.
Chapter 21 · Congenital Anomalies of the Great Vessels
713 21
At the same time, the pulmonary circulation The alternative strategy of an interventional
being prenatally already minimal may be even closure of the pulmonary artery to left atrial com-
further reduced. Cardiomegaly with the extremely munication is only recommended in neonates
enlarged left atrium may demand a detailed fetal achieving sufficient oxygenation and CO2 elimi-
echocardiography detecting the abnormal com- nation early postpartum. But in case of a continu-
munication. Immediately postnatal neonatal pul- ously deteriorating O2 saturation and a developing
monary vascular resistance may not allow any metabolic acidosis, immediate surgery is the most
pulmonary flow in this situation followed quickly competent measure to eliminate or prevent the
by asphyxia within the first minutes of life. metabolic decompensation within 15 min, proper
If the respiratory situation of the baby after planning provided.
birth cannot be stabilized within minutes after In patients at any age outside the immediate
spontaneous breathing or after intubation and neonatal period, who are less compromised by
ventilation, an operation via sternotomy is the defect, the fistula may be taken care off
required without delay. Anesthetic IV medication electively in a catheter-guided interventional
may have to be given directly into the right atrium setting or depending on the anatomy by surgery
after sternotomy. also.
A temporary/probationary occlusion of the
right pulmonary artery eliminating the left atrial
runoff may improve left pulmonary perfusion and References
gas exchange and facilitate the intraoperative
inspection of the anatomy. In case of a favorable Abbot ME (1936) Atlas of congenital heart disease.
anatomic variety (types I and II), a simple applica- American Heart Association, New York
Aebert H, Laas J, Bednarski P, Koch U, Prokop M, Borst HG
tion of a clip even theoretically is sufficient to (1993) High incidence of aneurysm formation follow-
close the connection. In complex forms with par- ing patch plasty repair of coarctation. Eur
ticipation of the pulmonary veins or with threat- J Cardiothorac Surg 7:200–204
ening loss of time, extracorporeal circulation Allen BS, Halldorsson AO, Barth MJ, Ilbawi MN (2000)
should be established. Modification of the subclavian patch aortoplasty for
repair of aortic coarctation in neonates and infants.
While the duct has to be temporarily snared Ann Thorac Surg 69:877–880
with ongoing extracorporeal circulation, it may Anderson RH, Lenox CC, Zuberbuhler JR (1983) Morphology
be opened as necessary once coming off bypass or of ventricular septal defect associated with coarcta-
it may stay closed. If closure is not tolerated as tion of the aorta. Br Heart J 50:176–181
indicated by oxygen saturation and gas exchange, Apfel HD, Levenbraun J, Quaegebeur JM, Allan LD (1998)
Usefulness of preoperative echocardiography in pre-
the duct should remain patent or even maintained dicting left ventricular outflow obstruction after pri-
open by infusion of prostaglandin. With decreas- mary repair of interrupted aortic arch with ventricular
ing pulmonary vascular resistance and matura- septal defect. Am J Cardiol 82:470–473
tion of the lungs within hours/days, the Apitz C, Kaulitz R, Sieverding L et al. (2007)
oxygenation will/may improve and the arterial Echokardiographische Diagnose des aortopulmona-
len Fensters. Ultraschall Med 28:189–194
duct can and should be closed. If the arterial duct Arnot RS, Louw JH (1973) The anatomy of the posterior wall
has to remain open at the end of surgery, we vote of the abdominal aorta. Its significance with regard to
for a preplaced snare at this time to facilitate duct hypoplasia of the distal aorta. S Afr Med J 47:899–902
closure on the PCICU. Asano M, Mishima A, Yamamoto S, Saito T, Manabe T (1998)
Modified subclavian flap aortoplasty for coarctation
repair in patients less than three months of age. Ann
Thorac Surg 66:588–589
Successful treatment of the prenatally Atsumi N, Moriki N, Sakakibara Y, Mitsui T, Horigome H,
diagnosed significant neonatal form requires Kamma H (2001) Persistent fifth aortic arch associated
preparation for potential immediate with type A aortic arch interruption. Histological study
establishment of extracorporeal circulation and morphogenesis. Jpn J Thorac Cardiovasc Surg
49:509–512
and cardiac surgical procedure to close the
Austin EH, Mayer JE, Jonas RA, Castaneda AR (1989) Aortic
abnormal communication and snare the atresia with normal left ventricle. Single-stage repair
arterial duct (cardiac surgery in «standby» in the neonate. J Thorac Cardiovasc Surg 97:392–395
with childbirth in the OR of cardiac surgery). Backer CL, Mavroudis C (2000) Congenital heart surgery
nomenclature and database project: vascular rings,
714 G. Ziemer and R. Kaulitz
tracheal stenosis, pectus excavatum. Ann Thorac Surg A randomized, controlled trial of very early prophylactic
69(4 Suppl):S308–S318 ligation of the ductus arteriosus in babies who weighed
Backer CL, Mavroudis C, Dunham ME, Holinger LD (1998) 1000 g or less at birth. N Engl J Med 320:1511–1516
Repair of congenital tracheal stenosis with a free tra- Castaneda AR, Jonas RA, Mayer JE Jr, Hanley FL (1994)
cheal autograft. J Thorac Cardiovasc Surg 115: Cardiac surgery of the neonate and infant. Saunders,
869–874 Philadelphia
Backer CL, Hillman N, Mavroudis C, Holinger LD (2002) Celoria GC, Patton RP (1959) Congenital absence of the
Resection of Kommerell’s diverticulum and left subcla- aortic arch. Am Heart J 58:407–413
vian artery transfer for recurrent symptoms after vas- Chowdhury UK, Diplomate NB, Kothari SS, Airam B,
cular ring division. Eur J Cardiothorac Surg 22:64–69 Subramaniam KG, Venugopal P (2005) Right pulmo-
Backer CL, Mavroudis C, Rigsby CK, Holinger LD (2005) nary artery to left atrium communication. Ann Thorac
Trends in vascular ring surgery. J Thorac Cardiovasc Surg 80:365–370
Surg 129:1339–1347 Connolly JE, Wilson SE, Lawrence PL, Fujitani RM (2002)
Barratt-Boyes BG, Nicholls TT, Brandt PW, Neutze JM (1972) Middle aortic syndrome: distal thoracic and abdominal
Aortic arch interruption associated with patent ductus coarctation, a disorder with multiple etiologies. J Am
arteriosus, ventricular septal defect, and total anoma- Coll Surg 194:774–781
lous pulmonary venous connection. Total correction in Cooley DA, McNamara DG, Latson JR (1957)
an 8-day-old infant by means of profound hypothermia Aorticopulmonary septal defect: diagnosis and surgi-
and limited cardiopulmonary bypass. J Thorac cal treatment. Surgery 42:101–120
Cardiovasc Surg 63:367–373 Craaford C, Nylin G (1945) Congenital coarctation of the
Beierlein W, Elliott MJ (2006) Variations in the technique of aorta and its surgical treatment. J Thorac Surg 14:347
slide tracheoplasty to repair complex forms of long- Daebritz S, Fausten B, Sachweh J et al. (1999) Anatomically
segment congenital tracheal stenoses. Ann Thorac positioned aorta ascending-descending bypass graft-
Surg 82:1540–1542 ing via left posterolateral thoracotomy for reopera-
Bellinger DC, Wypij D, Kuban KC et al. (1993) Developmental tion of aortic coarctation. Eur J Cardiothorac Surg
and neurological status of children at 4 years of age 16:519–523
after heart surgery with hypothermic circulatory arrest Dave H, Rosser B, Reineke K, Nguyen-Minh S, Knirsch W,
or low-flow cardiopulmonary bypass. Circulation Prêtre R (2012) Aortic arch enlargement and coarcta-
100:526–532 tion repair through a left thoracotomy: significance of
Berdon WE, Baker DH (1972) Vascular anomalies and the ductal perfusion. Eur J Cardiothorac Surg 41:
infant lung: rings, slings, and other things. Semin 906–912
Roentgenol 7:39–64 De Santo A, Bills RG, King H, Waller B, Brown JW (1987)
Berger M, Ferguson C, Hendry J (1960) Paralysis of the left Pathogenesis of aneurysm formation opposite pros-
diaphragm, left vocal cord, and aneurysm of the ductus thetic patches used for coarctation repair. An experi-
arteriosus in a 7-week-old infant. J Pediatr 56:800–802 mental study. J Thorac Cardiovasc Surg 94:720–723
Berry TE, Bharati S, Muster AJ et al. (1982) Distal aortopul- de Souza e Silva NA, Giuliani ER, Ritter DG, Davis GD, Pluth
monary septal defect, aortic origin of the right pulmo- JR (1974) Communication between right pulmonary
nary artery, intact ventricular septum, patent ductus artery and left atrium. Am J Cardiol 34:857–863
arteriosus and hypoplasia of the aortic isthmus: a newly DeCancq HG Jr (1963) Repair of patent ductus arteriosus in
recognized syndrome. Am J Cardiol 49:108–116 a 1417 gm infant. Am J Dis Child 106:402–410
Blalock A, Park EA (1944) The surgical treatment of experi- DeLeon MM, De Leon SY, Quinones JA et al. (1991)
mental coarctation (atresia) of the aorta. Ann Surg Management of arch hypoplasia after successful
119:445–456 coarctation repair. Ann Thorac Surg 63:875–880
Bove EL, Minich LL, Pridjian AK et al. (1993) The manage- Deverall PB, Lincoln JC, Aberdeen E, Bonham-Carter RE,
ment of severe subaortic stenosis, ventricular septal Waterston DJ (1969) Aortopulmonary window.
defect, and aortic arch obstruction in the neonate. J Thorac Cardiovasc Surg 57:479–486
J Thorac Cardiovasc Surg 105:289–295 Dische MR, Tsai M, Baltaxe HA (1975) Solitary interruption
Burke RP, Wernovsky G, van der Velde M, Hansen D, of the arch of the aorta. Clinicopathologic review of
Castaneda AR (1995) Video-assisted thoracoscopic eight cases. Am J Cardiol 35:271–277
surgery for congenital heart disease. J Thorac Donofrio MT, Ramaciotti C, Weinberg PM, Murphy JD
Cardiovasc Surg 109:499–507 (1995) Aortic atresia with interruption of the aortic
Burkhart HM, Dearani JA, Connolly HM, Schaff HV (2011) arch and an aortopulmonary fistulous tract: case
Ascending-descending posterior pericardial bypass of report. Pediatr Cardiol 16:147–149
complex coarctation of the aorta. Semin Thorac Doty DB, Richardson JV, Falkovsky GE, Gordonova MI,
Cardiovasc Surg Pediatr Card Surg Ann 14:116–119 Burakovsky VI (1981) Aortopulmonary septal defect:
Butera G, Manica JL, Chessa M et al. (2012) Covered-stent hemodynamics, angiography, and operation. Ann
implantation to treat aortic coarctation. Expert Rev Thorac Surg 32:244–250
Med Devices 9:123–130 Dyamenahalli U, Smallhorn JF, Geva T, Fouron JC, Cairns P,
21 Campbell M (1968) Natural history of patent ductus arte- Jutras L, Hughes V, Rabinovich M, Mason CA,
riosus. Br Heart J 30:4–13 Hornberger LK (2000) Isolated ductus arteriosus aneu-
Cassady G, Crouse DT, Kirklin JW, Strange MJ, Joiner CH, Godoy rysm in the fetus and infant: a multi-institutional expe-
G, Odrezin GT, Cutter GR, Kirklin JK, Pacifico AD et al. (1989) rience. J Am Coll Cardiol 36:262–269
Chapter 21 · Congenital Anomalies of the Great Vessels
715 21
Edwards JE (1948) Anomalies of the derivates of the aortic sus ligation in premature infants. Pediatrics
arch system. Med Clin N Am 32:925–949 112:1298–1301
Edwards JE (1953) A pathology of anomalies of thoracic Gross RE (1945a) Surgical correction for coarctation of the
aorta. J Clin Pathol 23:1240–1258 aorta. Surgery 18:673
Egan M, Holzer RJ (2009) Comparing balloon angioplasty, Gross RE (1945b) Surgical relief for tracheal obstruction
stenting and surgery in the treatment of aortic coarcta- from a vascular ring. New Engl J Med 233:586
tion. Expert Rev Cardiovasc Ther 7(11):1401–1412 Gross RE (1952) Surgical closure of an aortic septal defect.
Eibenberger K, Dock W, Metz V, Farres MT et al. (1993) Circulation 5:858–863
Stenoses and occlusions of the abdominal aorta in Gross RE, Hubbard JP (1939) Surgical ligation of a patent
patients less than 40-years old. Röfo 159:388–392 ductus arteriosus. Report of first successful case. JAMA
Elgamal ME, McKenzie ED, Fraser CD Jr (2002) Aortic arch 112:729
advancement: the optimal one-stage approach for Gross RE, Longino LA (1951) The patent ductus arteriosus;
surgical management of neonatal coarctation with observations from 412 surgically treated cases.
arch hypoplasia. Ann Thorac Surg 73:1267–1273 Circulation 1:125–137
Elzenga NJ, Gittenberger-de Groot AC (1986) The ductus Gross RE, Neuhauser EB (1948) Compression of the trachea
arteriosus and stenoses of the pulmonary arteries in by an anomalous innominate artery; an operation for
pulmonary atresia. Int J Cardiol 11:195–208 its relief. Am J Dis Child 75:570–574
Elzenga NJ, Gittenberger-de Groot AC, Oppenheimer- Gross RE, Neuhauser EB (1951) Compression of the trachea
Dekker A (1986) Coarctation and other obstructive or esophagus by vascular anomalies. Surgical therapy
aortic arch anomalies: their relationship to the ductus in 40 cases. Pediatrics 7:69–83
arteriosus. Int J Cardiol 13:289–308 Gross RE, Ware PF (1946) The surgical significance of aortic
Elzenga NJ, von Suylen RJ, Frohn-Mulder I, Essed CE, Bos E, arch anomalies. Surg Gynecol Obstet 83:435–440
Quaegebeur JM (1990) Juxtaductal pulmonary artery Hallman GL, Cooley DA, Bloodwell RD (1966) Congenital
coarctation. An underestimated cause of branch pul- vascular ring. Surg Clin North Am 46:885–892
monary artery stenosis in patients with pulmonary Hara Y, Ishiguro S, Maeda T et al. (1993) A case of surgical
atresia or stenosis and a ventricular septal defect. treatment of patent ductus arteriosus using left heart
J Thorac Cardiovasc Surg 100:416–424 bypass in an adult. Kyobu Geka 46:494–497
Fisher RG, Whigham CJ, Trinh C (2005) Diverticula of Hart JC, Waldhausen JA (1983) Reversed subclavian flap
Kommerell and aberrant subclavian arteries complicated angioplasty for arch coarctation of the aorta. Ann
by aneurysms. Cardiovasc Intervent Radiol 28:553–560 Thorac Surg 36:715–717
Fletcher G, Dushane JW, Kirklin JW, Wood EH (1954) Aortic- Hawkins JA, Bailey WW, Clark SM (1992) Innominate artery
pulmonary septal defect: report of a case with surgical compression of the trachea. Treatment by reimplanta-
division along with successful resuscitation from ven- tion of the innominate artery. J Thorac Cardiovasc
tricular fibrillation. Proc Staff Meet Mayo Clin 29:285–292 Surg 103:678–682
Forsen JW Jr, Lusk RP, Huddleston CB (2002) Costal carti- Heinemann MK, Ziemer G, Wahlers T, Kohler A, Borst HG
lage tracheoplasty for congenital long-segment tra- (1997) Extraanatomic thoracic aortic bypass grafts:
cheal stenosis. Arch Otolaryngol Head Neck Surg indications, techniques, and results. Eur J Cardiothorac
128:1165–1171 Surg 11:169–175
Friedlich A, Bing RJ, Blount SG Jr (1950) Physiological studies Hjortdal VE, Khambadkone S, de Leval MR, Tsang VT (2003)
in congenital heart disease; circulatory dynamics in the Implications of anomalous right subclavian artery in
anomalies of venous return to the heart including pul- the repair of neonatal aortic coarctation. Ann Thorac
monary arteriovenous fistula. Bull Johns Hopkins Hosp Surg 76:572–575
86:20–57 Idrees J, Keshavamurthy S, Subramanian S et al. (2014)
Fripp RR, Whitman V, Waldhausen JA, Boal DK (1985) Hybrid repair of Kommerell diverticulum. J Thorac
Ductus arteriosus aneurysm presenting as pulmonary Cardiovasc Surg 147:973–976
artery obstruction: diagnosis and management. J Am Idriss FS, DeLeon SY, Ilbawi MN, Gerson CR, Tucker GF,
Coll Cardiol 6:234–236 Holinger L (1984) Tracheoplasty with pericardial patch
Geva T, Hornberger LK, Sanders SP, Jonas RA, Ott DA, Colan for extensive tracheal stenosis in infants and children.
SD (1993) Echocardiographic predictors of left ven- J Thorac Cardiovasc Surg 88:527–536
tricular outflow tract obstruction after repair of inter- Ilbawi MN, Idriss FS, DeLeon SY, Muster AJ, Benson DW Jr,
rupted aortic arch. J Am Coll Cardiol 22:1953–1960 Paul MH (1988) Surgical management of patients with
Godart F (2011) Intravascular stenting for the treatment of interrupted aortic arch and severe subaortic stenosis.
coarctation of the aorta in adolescent and adult Ann Thorac Surg 45:174–180
patients. Arch Cardiovasc Dis 104:627–635 Imoto Y, Kado H, Shiokawa Y, Fukae K, Yasui H (1999)
Gold JP, Cohn LH (1986) Operative management of the cal- Norwood procedure without circulatory arrest. Ann
cified patent ductus arteriosus. Ann Thorac Surg Thorac Surg 68:559–561
41:567–568 Imoto Y, Kado H, Shiokawa Y, Minami K, Yasui H (2001)
Gossage JR, Kanj G (1998) Pulmonary arteriovenous mal- Experience with the Norwood procedure without
formations. A state of the art review. Am J Respir Crit circulatory arrest. J Thorac Cardiovasc Surg 122:
Care Med 158:643–661 879–882
Gould DS, Montenegro LM, Gaynor JW et al. (2003) A com- Ishino K, Kawada M, Irie H, Kino K, Sano S (2000) Single
parison of on-site and off-site patent ductus arterio- stage repair of aortic coarctation with ventricular
716 G. Ziemer and R. Kaulitz
s eptal defect using isolated cerebral and myocardial Lambert V, Blaysat G, Sidi D, Lacour-Gayet F (1999) Double-
perfusion. Eur J Cardiothorac Surg 17:538–542 lumen aortic arch by persistence of fifth aortic arch: a
Jacobs JP, Quintessenza JA, Gaynor JW, Burke RP, Mavroudis new case associated with coarctation. Pediatr Cardiol
C (2000) Congenital heart surgery nomenclature and 20:167–169
database project: aortopulmonary window. Ann Lamberti JJ, Mainwaring RD, Waldman JD et al. (1991)
Thorac Surg 69(4 Suppl):S44–S49 The Damus-Fontan procedure. Ann Thorac Surg
Johnson AM, Kron IL (1988) Closure of the calcified patent 52:676–679
ductus in the elderly: avoidance of ductal clamps and Langley SM, Chai PJ, Miller SE et al. (1999) Intermittent per-
shunts. Ann Thorac Surg 45:572–573 fusion protects the brain during deep hypothermic
Jonas RA (2004) Comprehensive surgical management of circulatory arrest. Ann Thorac Surg 68:4–12
congenital heart disease. Hodders Arnold, London Lee MGY, Kowalski R, Galati JC et al. (2012) Twenty-four-
Jonas RA, Quaegebeur JM, Kirklin JW, Blackstone EH, hour ambulatory blood pressure monitoring detects a
Daicoff G (1994) Outcomes in patients with inter- high prevalence of hypertension late after coarctation
rupted aortic arch and ventricular septal defect. A repair in patients with hypoplastic arches. J Thorac
multiinstitutional study. Congenital Heart Surgeons Cardiovasc Surg 144:1110–1116
Society. J Thorac Cardiovasc Surg 107:1099–1109 Lerberg DB (1981) Abbott’s artery. Ann Thorac Surg 33:415–416
Kabra NS, Schmidt B, Roberts RS, Doyle LW, Papile L, Levy Praschker BG, Mordant P, Barreda E et al. (2008) Long-
Fanaroff A, Trial of Indomethacin Prophylaxis in term results of ascending aorta-abdominal aorta
Preterms Investigators (2007) Neurosensory impair- extra-anatomic bypass for recoarctation in adults with
ment after surgical closure of patent ductus arteriosus 27-year follow-up. Eur J Cardiothorac Surg 34:805–809
in extremely low birth weight infants: results from the Liu JY, Kowalski R, Jones B, Konstantinov IE (2010)
Trial of Indomethacin Prophylaxis in Preterms. Moderately hypoplastic arches: do they reliably grow
J Pediatr 150:229–234, 234.e1 into adulthood after conventional coarctation repair?
Kanter KR, Vincent RN, Fyfe DA (2001) Reverse subclavian Interact Cardiovasc Thorac Surg 10:582–586
flap repair of hypoplastic transverse aorta in infancy. Luciani GB, Ackerman RJ, Chang AC, Wells WJ, Starnes VA
Ann Thorac Surg 71:1530–1536 (1996) One-stage repair of interrupted aortic arch,
Karl TR, Sano S, Brawn W, Mee RB (1992) Repair of hypo- ventricular septal defect, and subaortic obstruction in
plastic or interrupted aortic arch via sternotomy. the neonate: a novel approach. J Thorac Cardiovasc
J Thorac Cardiovasc Surg 104:688–695 Surg 111:348–358
Kenny D, Polson JW, Martin RP, Wilson DG, Caputo M, Luhmer I, Ziemer G (1993) Coarctation of the pulmonary
Cockroft JR, Paton JFR, Wolf AR (2010) Surgical artery in neonates. Prevalence, diagnosis, and surgical
approach for aortic coarctation influences arterial treatment. J Thorac Cardiovasc Surg 106:889–894
compliance and blood pressure control. Ann Thorac Luijendijk P, Bouma BJ, Groenink M et al. (2012) Surgical
Surg 90:600–604 versus percutaneous treatment of aortic coarctation:
Khan S, Nihill MR (2006) Clinical presentation of persistent new standards in an era of transcatheter repair. Expert
5th aortic arch: 3 new cases. Tex Heart Inst J 33:361–364 Rev Cardiovasc Ther 10:1517–1531
Kirklin JW, Barratt Boyes BB (1993) Cardiac surgery, 2nd Lund JT, Hansen D, Brocks V, Jensen MB, Jacobsen JR (1992)
edn. Churchill Livingstone, New York Aneurysm of the ductus arteriosus in the neonate:
Kommerell B (1936) Verlagerung des Oesophagus durch three case reports with a review of the literature.
eine abnorm verlaufende Arteria subclavia dextra Pediatr Cardiol 13(4):222–226
(Arteria lusoria). Fortschr Geb Roentgenstr 54: MacDonald MJ, Hanley FL, Reddy VM (2002) Arch recon-
590–595 struction without circulatory arrest: current clinical
Koneti NR, Kanchi V, Kandruja H, Jaishankar (2011) applications and results of therapy. Semin Thorac
Symptomatic aneurysm of ductus arteriosus in neo- Cardiovasc Surg Pediatr Card Surg Ann 5:95–103
nates. Ann Pediatr Cardiol 4:159–163 Mandhan PL, Samarakkody U, Brown S, Kukkady AK,
Kostelka M, Walther T, Geerdts I et al. (2004) Primary repair Blakelock R, Beasley S (2006) Comparison of suture
for aortic arch obstruction associated with ventricular ligation and clip application for the treatment of pat-
septal defect. Ann Thorac Surg 78:1989–1993 ent ductus arteriosus in preterm neonates. J Thorac
Kreutzer J, Van Praagh R (2000) Comparison of left ventric- Cardiovasc Surg 132:672–674
ular outflow tract obstruction in interruption of the Mavroudis C, Backer CL (2003) Pediatric cardiac surgery,
aortic arch and in coarctation of the aorta, with diag- 3rd edn. Mosby, St. Louis
nostic, developmental, and surgical implications. Am McCandless RT, Puchalski MD, Minich LL, Menon SC (2012)
J Cardiol 86:856–862 Prenatally diagnosed coarctation: a more sinister dis-
Kumar TK, Zurakowski D, Sharma R, Saini S, Jonas RA (2011) ease? Pediatr Cardiol 33:1160–1164
Prediction of recurrent coarctation by early postopera- McCrindle BW, Tchervenkov CI, Konstantinov IE et al. (2005)
tive blood pressure gradient. J Thorac Cardiovasc Surg Risk factors associated with mortality and interven-
142(5):1130–1136 tions in 472 neonates with interrupted aortic arch: a
Laborde F, Noirhomme P, Karam J, Batisse A, Bourel P, Saint Congenital Heart Surgeons Society study. J Thorac
21 Maurice O (1993) A new video-assisted thoracoscopic Cardiovasc Surg 129:343–350
surgical technique for interruption of patient ductus McElhinney DB, Reddy VM, Silverman NH, Hanley FL (1997)
arteriosus in infants and children. J Thorac Cardiovasc Modified Damus-Kaye-Stansel procedure for single
Surg 105:278–280 ventricle, subaortic stenosis, and arch obstruction in
Chapter 21 · Congenital Anomalies of the Great Vessels
717 21
neonates and infants: midterm results and techniques O’Donovan TG, Beck W (1978) Closure of the complicated
for avoiding circulatory arrest. J Thorac Cardiovasc patent ductus arteriosus. Ann Thorac Surg 25:463–465
Surg 114:718–725 Ohara H, Ito K, Kohguchi N et al. (1979) Direct communica-
McElhinney DB, Thompson LD, Weinberg PM, Jue KL, tion between the right pulmonary artery and the left
Hanley FL (2000) Surgical approach to complicated atrium. A case report and review of the literature.
cervical aortic arch: anatomic, developmental, and J Thorac Cardiovasc Surg 77:742–747
surgical considerations. Cardiol Young 10:212–219 Oosterhof T, Azakie A, Freedom RM, Williams WG,
McKellar SH, Schaff HV, Dearani JA et al. (2007) McCrindle BW (2004) Associated factors and trends in
Intermediate- term results of ascending-descending outcomes of interrupted aortic arch. Ann Thorac Surg
posterior pericardial bypass of complex aortic coarcta- 78:1696–1702
tion. J Thorac Cardiovasc Surg 133:1504–1509 Ozmen J, Granger EK, Robinson D, White GH, Wilson M
McKenzie ED, Klysik M, Morales DLS, Heinle JS, Fraser CD Jr, (2005) Operation for adult patent ductus arteriosus
Kovalchin J (2011) Ascending sliding arch aortoplasty: using an aortic stent-graft technique. Heart Lung Circ
a novel technique for repair of arch hypoplasia. Ann 14:54–57
Thorac Surg 91:805–810 Pedersen TA, Munk K, Andersen NH, Lundorf E, Pedersen
Merrill DL, Webster CA, Samson PC (1957) Congenital EB, Hjortdal VE, Emmertsen K (2011) High long-term
absence of the aortic isthmus. J Thorac Surg 33:311 morbidity in repaired aortic coarctation: weak associa-
Meyberg-Solomayer GC, Hofbeck M, Müller-Hansen I, tion with residual arch obstruction. Congenit Heart
Kaulitz R, Ziemer G (2009) Open heart surgery immedi- Dis 6:573–582
ately after birth following prenatal diagnosis of a large Pigula FA, Nemoto EM, Griffith BP, Siewers RD (2000)
right pulmonary artery to left atrium communication. Regional low-flow perfusion provides cerebral circula-
Prenat Diagn 29:718–720 tory support during neonatal aortic arch reconstruc-
Mitsumori Y, Tsuchiya K, Nakajima M, Fukuda S, Morimoto tion. J Thorac Cardiovasc Surg 119:331–339
H (2008) Surgical repair of left-sided cervical aortic Planché C, Lacour-Gayet F (1984) Aortic uncrossing for
arch aneurysm: case report and literature review. Ann compressive circumflex aorta. 3 cases. Presse Med
Vasc Surg 22:140–144 13:1331–1332
Moene RJ, Oppenheimer-Dekker A, Moulaert AJ, Wenink Porstmann W, Wierny L, Warnke HZ (1967a) Der Verschluß
AC, Gittenberger-de Groot AC, Roozendaal H (1982) des Ductus arteriosus persistens ohne Thorakotomie
The concurrence of dimensional aortic arch anomalies (vorläufige Mitteilung). Thoraxchir Vask Chir 15:
and abnormal left ventricular muscle bundles. Pediatr 199–203
Cardiol 2:107–114 Portsmann W, Wierny L, Warnke H (1967b) Closure of per-
Moes CAF, Freedom RM (1992) Rings, slings, and other sistent ductus arteriosus without thoracotomy. Ger
things. Vascular structures contributing to a neonatal Med Mon 12:259–261
“noose”. In: Freedom RM, Benson LN, Smallhorn JF (eds) Potts WJ, Holinger PH, Rosenblum AH (1954) Anomalous left
Neonatal heart disease. Springer, Berlin, pp 731–750 pulmonary artery causing obstruction to right main bron-
Moes CA, Freedom RM (1993) Rare types of aortic arch chus: report of a case. J Am Med Assoc 155:1409–1411
anomalies. Pediatr Cardiol 14:93–101 Rajasinghe HA, Reddy VM, van Son JAM et al. (1996)
Moes CA, Mawson JB, MacDonald C et al. (1996) Right aor- Coarctation repair using end-to-side anastomosis of
tic arch with retroesophageal left aberrant innominate descending aorta to proximal aortic arch. Ann Thorac
artery. Pediatr Cardiol 17:402–406 Surg 61:840–844
Monro JL, Bunton RW, Sutherland GR, Keeton BR (1989) Rauch R, Kaulitz R, Hofbeck M (2008) Pulmonary artery
Correction of interrupted aortic arch. J Thorac thrombosis in a premature infant. Ultraschall Med
Cardiovasc Surg 98:421–427 29:233–234
Monro JL, Delany DJ, Ogilvie BC, Salmon AP, Keeton BR Rheuban KS, Gutgesell HP, Carpenter MA et al. (1986) Aortic
(1996) Growth potential in the new aortic arch after aneurysm after patch angioplasty for aortic isthmic
non-end-to-end repair of aortic arch interruption in coarctation in childhood. Am J Cardiol 58:178–180
infancy. Ann Thorac Surg 61:1212–1216 Richardson JV, Doty DB, Rossi NP, Ehrenhaft JL (1979) The
Mori K, Ando M, Takao A, Ishikawa S, Imai Y (1978) Distal spectrum of anomalies of aortopulmonary septation.
type of aortopulmonary window: report of 4 cases. Br J Thorac Cardiovasc Surg 78:21–27
Heart J 40:681–689 Roques F, Hennequin JL, Sanchez B, Ridarch A, Rousseau H
Moore FD, Folkman J (1995) Robert Edward Gross 1905– (2001) Aortic stent-graft for patent ductus arteriosus
1988. A biographical memoir. National Academy of in adults: the aortic exclusion technique. Ann Thorac
Sciences. National Academies Press, Washington D.C. Surg 71:1708–1709
p. 129–149 Roughneen PT, Parikh P, Stark J (1996) Bronchial obstruc-
Morrow AG, Clark WD (1966) Closure of the calcified patent tion secondary to aneurysm of a persistent ductus
ductus. A new operative method utilizing cardiopul- arteriosus. Eur J Cardiothorac Surg 10:146–147
monary bypass. J Thorac Cardiovasc Surg 51:534–538 Roussin R, Belli E, Lacour-Gayet F et al. (2002) Aortic arch
Naoum JJ, Parenti JL, LeMaire SA, Coselli JS (2008) reconstruction with pulmonary autograft patch aorto-
Endovascular repair of a right-sided descending tho- plasty. J Thorac Cardiovasc Surg 123:443–448
racic aortic aneurysm with a right-sided aortic arch and Rudolph AM, Heymann MA, Spitznas U (1972)
aberrant left subclavian artery. Ann Thorac Surg Hemodynamic considerations in the development of
85:1074–1076 narrowing of the aorta. Am J Cardiol 30:514–525
718 G. Ziemer and R. Kaulitz
Rutishauser M, Ronen G, Wyler F (1977) Aneurysm of the Thilén U, Aström-Olsson K (1997) Does the risk of infective
nonpatent ductus arteriosus in the newborn. Acta endarteritis justify routine patent ductus arteriosus
Pediatr Scand 66:649–651 closure? Eur Heart J 18:503–506
Sachweh J, Däbritz S, Didilis V et al. (1998) Pulmonary Traugott RC, Will RJ, Schuchmann GF, Treasure RL (1980) A
artery stenosis after systemic-to-pulmonary shunt simplified method of ligation of patent ductus arterio-
operations. Eur J Cardiothorac Surg 14:229–234 sus in premature infants. Ann Thorac Surg 29:263
Salem MM, Starnes VA, Wells WJ et al. (2000) Predictors Van Praagh R, Van Praagh S (1969) Persistent fifth arterial
of left ventricular outflow obstruction following arch in man. Congenital double-lumen aortic arch. Am
single- stage repair of interrupted aortic arch J Cardiol 24:279–282
and ventricular septal defect. Am J Cardiol 86:1044– Verel D, Grainger RG, Taylor DG (1964) Communication of a
1047, A11 pulmonary artery with the left atrium. Br Heart
Schneider DJ, Moore JW (2006) Congenital heart disease J 26:856–858
for the adult cardiologist. Patent ductus arteriosus. Vossschulte K (1957) Isthmusplastik zur Behandlung der
Circulation 114:1873–1882 Aortenisthmusstenose. Thoraxchirurgie 4:443–450
Scott HW Jr, Sabiston DC Jr (1953) Surgical treatment for Vouhé PR, Macé L, Vernant F et al. (1990) Primary definitive
congenital aorticopulmonary fistula; experimental repair of interrupted aortic arch with ventricular septal
and clinical aspects. J Thorac Surg 25:26–39 defect. Eur J Cardiothorac Surg 4:365–370
Serraf A, Lacour-Gayet F, Robotin M et al. (1996) Repair of Waldhausen JA, Nahrwold DL (1966) Repair of coarctation
interrupted aortic arch: a ten-year experience. J Thorac of the aorta with a subclavian flap. J Thorac Cardiovasc
Cardiovasc Surg 112:1150–1160 Surg 79:532
Siewers RD, Ettedgui J, Pahl E, Tallman T, del Nido PJ (1991) Walker T, Heinemann MK, Nagy Z, Steil E, Ziemer G (2002)
Coarctation and hypoplasia of the aortic arch: will the Right-sided cervical aortic arch with stenosis – treat-
arch grow? Ann Thorac Surg 52:608–613 ment with an extra-anatomic bypass graft. Thorac
Stanley JC, Criado E, Upchurch GR Jr (2006) Pediatric Cardiovasc Surg 50:306–307
renovascular hypertension: 132 primary and 30 sec- Wright GE, Nowak CA, Goldberg CS, Ohye RG, Bove EL,
ondary operations in 97 children. J Vasc Surg 44: Rocchini AP (2005) Extended resection and end-to-
1219–1228 end anastomosis for aortic coarctation in infants:
Steger V, Heinemann MK, Irtel von Brenndorff C, Ziemer G results of a tailored surgical approach. Ann Thorac
(1998) Combined Norwood and Rastelli procedure for Surg 80:1453–1459
repair of interrupted aortic arch with subaortic steno- Yasui H, Kado H, Nakano E et al. (1987) Primary repair of
sis. Thorac Cardiovasc Surg 46:156–158 interrupted aortic arch and severe aortic stenosis in
Stejskal L, Stark J (1992) Surgical treatment of persistent neonates. J Thorac Cardiovasc Surg 93:539–545
ductus arteriosus complicated by bacterial endocardi- Zannini L, Gargiulo G, Albanese SB et al. (1993) Aortic
tis. Eur J Cardiothorac Surg 6:272–273 coarctation with hypoplastic arch in neonates: a spec-
Stewart JR, Kincaid OW, Edwards JE (1964) An atlas of vas- trum of anatomic lesions requiring different surgical
cular rings and related malformations of the aortic sys- options. Ann Thorac Surg 56:288–294
tem. Thomas, Springfield Zartner P, Schneider MB, Bein G (2000) Prostaglandin E1 sen-
Suematsu Y, Mora BN, Mihaljevic T, del Nido PJ (2005) sitive persistent fifth aortic arch type 2. Heart 84:142
Totally endoscopic robotic-assisted repair of patent Ziemer G, Luhmer I, Kallfelz HC, Oelert H (1983) Die seltene
ductus arteriosus and vascular ring in children. Ann Form eines Gefäßringes in Verbindung mit
Thorac Surg 80:2309–2313 Transposition der großen Arterien: Linksseitiger
Svetliza G, De la Canal A, Beveraggi E et al. (2002) Lung Aortenbogenverlauf bei Arteria lusoria und
transplantation in a patient with arteriovenous mal- rechtsseitig persistierendem Ductus Botalli [A rare
formations. J Heart Lung Transplant 21:506–508 form of vascular ring in association with transposition
Tannous HJ, Moulick AN, Jonas RA (2006) Interrupted aortic of the great arteries: left-sided course of the aortic
arch and aortic atresia with circle of Willis-dependent arch with arteria lusoria and a right-sided persistent
coronary perfusion. Ann Thorac Surg 82:e11–e13 Botalli’s duct]. Herz 8:170–174
Taussig HB (1960) Congenital malformations of the heart, Ziemer G, Jonas RA, Perry SB, Freed MD, Castaneda AR
vol 2, 2nd edn. Harvard University Press, Cambridge, (1986) Surgery for coarctation of the aorta in the neo-
MA, p 376 nate. Circulation 74:I25–I31
21
719 IV
Acquired Diseases of
the Heart and Great
Vessels
Contents
References – 739
..Fig. 22.3 Open
saphenous vein
procurement
726 J.T. Cremer et al.
V saphena magna
(Yoshikai et al. 2004). To harvest the LIMA as a essential for the use of this graft, can be gained by
skeletonized graft without concomitant vessels dividing the proximal pedicle from mediastinal
and perivascular tissue presents another tech- structures. This comprises division of the proxi-
nique which differs in several aspects from the
standard method (Canadas et al. 2005; . Fig. 22.8).
Harvesting starts with incision of the endotho-
racic fascia along the LIMA followed by encircling A hemodynamically relevant stenosis of the
the conduit – a vessel loop may be passed around it. proximal left subclavian artery is an absolute
Thereafter takedown over the entire length is contraindication for utilization of the LIMA as
achieved by dividing the side branches with hemo- conduit for coronary revascularization.
static clips. The main advantage of this technique
consists of more available graft length and a pre-
served venous circulation left in place, which may
improve conditions of wound healing. However
skeletonizing may be associated with more traumas
to the IMA due to the harvesting technique where a
no-touch procurement is almost impossible.
Difficulties in harvesting are likely in patients
with exostoses of the ribs, thoracic deformities,
systemic rheumatic diseases and in post radiation
or post thoracic trauma situations.
c d e
..Fig. 22.16 Technique of proximal anastomosis. a Punching of the side-clamped aorta. b–c Suture in running
fashion starting at the graft’s heel. d Parachuting of the graft. e Completion of the anastomosis
Isolated, discrete ostial stenoses of the left or right 22.4.6 Surgery of Coronary
coronary artery can be surgically treated by ostial Aneurysms
patch plasty. During cardioplegic arrest, the
ostium of the effected coronary vessel is exposed In adult patients coronary aneurysms can reach a
via aortotomy. The coronary artery is longitudi- diameter up to 15 cm (Mawatari et al. 2000). An
nally opened from the lesion up to the normal existing coronary aneurysm bears the risk of
738 J.T. Cremer et al.
spontaneous rupture or embolization of throm- avoided by the use of in situ IMA grafts in
bus material causing an indication for operation. combination with T or Y grafts.
There are different techniques for surgical
treatment. The aneurysm’s in- and outflow vessel
can be ligated with subsequent distal coronary 22.5 Significance of Coronary
bypass; however, techniques preserving the ves- Surgery and Current Studies
sel are feasible, also. After resection of the aneu- and Analyses
rysm, an end-to-end anastomosis can be
performed (Westaby et al. 1999). In cases where As an example for Western Europe, CABG data
this is not possible due to the resulting distance from Germany are described. To date around
between the two vessels ends, a vein segment can 50,000 cases of isolated coronary surgery are per-
be interposed (Firstenberg et al. 2000) (see also formed in Germany anually, with a 30-day mortal-
7 Chapter «Congenital Anomalies of the Coronary ity of <3 %. These numbers comprise all patients,
Arteries and Coronary Diseases in Children and including those with significant risk factors like
Adolescents», Sect. 20.8). poor left ventricular function, acute myocardial
infarction or redo bypass surgery. Around 10 % of
coronary procedures are performed under beat-
22.4.7 Coronary Revascularization ing-heart conditions without extracorporeal circu-
and Atherosclerotic lation (MIDCAB/OPCAB). The mean age for all
Ascending Aorta patients is 67.4 years with more than 10 % being
80 years and older. On average 3.1, distal anasto-
The presence of significant atherosclerosis of the moses are constructed utilizing a LIMA graft in
ascending aorta demands changes of the routine 92.3 % of cases.
surgical procedure especially to prevent cerebral In the United States, the proportions are com-
complication due to embolization of plaque mate- parable. Within the last decade, around 1.5 mil-
rial. In many cases the atherosclerosis is preopera- lion patients underwent isolated CABG according
tively known because of the findings in the chest to the registry of the Society of Thoracic Surgeons
x-ray, the angiogram or a thoracic CT scan. If (STSs). The postoperative mortality rate declined
ascending aortic wall atherosclerosis is an acci- to 1.9 % in 2009. The LIMA is used 95 % of cases
dental intraoperative finding, an epiaortic ultra- (ElBardissi et al. 2012).
sound or trans-esophageal echocardiography may The Synergy between PCI with Taxus and
be helpful to judge the importance of calcification Cardiac Surgery (SYNTAX) trial is a randomized
actually present. prospective study, comparing CABG and PCI in
The following measures are helpful in order to 1,800 patients with three-vessel disease and/or left
reduce manipulation of the calcified aorta, thereby main stenosis. For these patients, it demonstrated a
reducing the risk of embolization to a minimum significant superiority of bypass surgery concern-
(«no-touch philosophy»): ing the primary end point (rate of major adverse
55 In operations with extracorporeal circulation, cardiac and cerebrovascular events; Serruys et al.
the aortic cannula should be placed in the 2009). In the meantime the 3-year results of the
proximal arch or the right subclavian artery. SYNTAX trial are available which confirm the
Clamping of the aorta can be avoided by original results (Kappetein et al. 2011). This was
temporary hypothermic cardiac arrest and the first major multicenter randomized trial that
retrograde application of cardioplegia. compares CABG and PCI in the drug eluting stent
55 Alternatively the procedure can be performed era in patients with triple-vessel disease. Former
with the heart fibrillating in normothermia or studies like the Arterial Revascularization Therapy
moderate hypothermia (Beyersdorf et al. Study (ARTS trial, Serruys et al. 2005) and the
1990); however, this technique is associated Stent or Surgery trial (SOS trial, Stables et al. 2002)
with limited exposure of the target vessels. compared CABG to PCI with bare-metal stents.
55 The use of OPCAB technique avoids the risks The long-term results of the SYNTAX trial demon-
of inserting cannulas in this setting. strate impressively that with a growing complexity
Depending on the pattern of atherosclerotic of coronary lesions (quantified by the Syntax
22 lesions, any aortic anastomosis can be Score), surgery leads to a significant survival ben-
22 · Coronary Artery Disease
739 22
efit apart from lower incidence of myocardial than 1,000 patients using an internal thoracic artery/
infarctions, revascularization and recurrent angina radial artery T graft. Ann Surg 234:447–453
Barra JA, Bezon E, Mondine P et al. (2000) Surgical angio-
pectoris. Latest analyses of major registries prove plasty with exclusion of atheromatous plaques in case
concordantly with risk adjustment a survival ben- of diffuse disease of the left anterior descending
efit for surgical treated patients with triple-vessel artery: 2 years’ follow-up. Eur J Cardiothorac Surg
disease (Hannan et al. 2005, 2008). 17:509–514
Current interdisciplinary European (Task Benetti FJ, Rizzardi JL, Ann RJ (1980) Cirurgia coronaria
directa con bypass de vena safena sin circulación
Force and Kolh et al. 2010) and North American
extracorpórea o parada cardíaca: comunicación pre-
(Kushner et al. 2009) guidelines particularly take via. Rev F Arg Cardiol 8:3
the results of the SYNTAX trial into account. Aside Ben-Gal Y, Mohr R, Uretzky G et al. (2006) Drug-eluting
from recommendations for revascularization, it is stents versus arterial myocardial revascularization in
unmistakeably declared that an individual thera- patients with diabetes mellitus. J Thorac Cardiovasc
Surg 132:861–866
peutic strategy has to be developed in a formal
Beyersdorf F, Krause E, Sarai K et al. (1990) Clinical evaluation
heart team. This should result in an optimal ther- of hypothermic ventricular fibrillation, multi-dose blood
apy for every individual patient and avoid the use cardioplegia, and single-dose Bretschneider cardiople-
of PCI when there is clear recommendation for gia in coronary surgery. Thorac Cardiovasc Surg 38:20–29
surgical treatment (Hannan et al. 2010). If these Boening A, Schoeneich F, Lichtenberg A, Bagaev E, Cremer
guidelines will be followed, coronary bypass sur- JT, Klima U (2005) First clinical results with a 30° end-
to-side coronary anastomosis coupler. Eur
gery will be of paramount importance in industrial J Cardiothorac Surg 27:876–881
nations as demographic changes with an aging Boethig D, Minami K, Lueth JU, El-Banayosy A, Breymann T,
population will lead to a growing number of Koerfer R (2004) Intermittent aortic cross-clamping for
patients with complex coronary findings. They isolated CABG can save lives and money: experience
may be due just to advanced age, but also due to with 15307 patients. Thorac Cardiovasc Surg 52:147–
151
previous catheter interventions and/or surgery.
Bonacchi M, Prifti E, Giunti G, Salica A, Frati G, Sani G (2001)
Consequently the overuse of PCI may be limited. Respiratory dysfunction after coronary artery bypass
grafting employing bilateral internal mammary arter-
ies: the influence of intact pleura. Eur J Cardiothorac
References Surg 19:827–833
Booth J, Clayton T, Pepper J et al. (2008) Randomized, con-
Abu-Omar Y, Taggart DP (2009) The present status of off- trolled trial of coronary artery bypass surgery versus percu-
pump coronary artery bypass grafting. Eur taneous coronary intervention in patients with multivessel
J Cardiothorac Surg 36(2):312–321 coronary artery disease: six-year follow-up from the Stent
Acar C, Jebara VA, Portoghese M et al. (1992) Revival of the or Surgery Trial (SoS). Circulation 118:381–388
radial artery for coronary artery bypass grafting. Ann Brose S, Fabricius AM, Falk V et al. (2002) Comparison of
Thorac Surg 54:652–659 ultrasonic scalpel versus argon-beam and conven-
Acar C, Ramsheyi A, Pagny JY et al. (1998) The radial artery tional electrocautery for internal thoracic artery dis-
for coronary artery bypass grafting: clinical and angio- section. Thorac Cardiovasc Surg 50:71–73
graphic results at five years. J Thorac Cardiovasc Surg Buche M, Schroeder E, Gurné O et al. (1995) Coronary
116(6):981–989 artery bypass grafting with the inferior epigastric
Agrifoglio M, Dainese L, Pasotti S et al. (2005) Preoperative artery: midterm clinical and angiographic results.
assessment of the radial artery for coronary artery J Thorac Cardiovasc Surg 109:553–560
bypass grafting: is the clinical Allen test adequate? Calafiore AM, Di Giammarco G, Teodori G et al. (1996) Left
Ann Thorac Surg 79:570–572 anterior descending coronary artery grafting via left
Allen KB, Dowling RD, DelRossi AJ et al. (2000) anterior small thoracotomy without cardiopulmonary
Transmyocardial laser revascularization combined bypass. Ann Thorac Surg 61:1658–1665
with coronary artery bypass grafting: a multicenter, Canadas PPL, Canas AC, Romeo JLO, Martinez CIR,
blinded, prospective, randomized, controlled trial. Almodovar LFO, Hernandez MC (2005) Endothelium
J Thorac Cardiovasc Surg 119:540–549 histological integrity after skeletonized dissection
Amoroso G, Van Boven AJ, Crijns HJ (2001) Drug therapy or of the left internal mammary artery with ultrasonic
coronary angioplasty for the treatment of coronary scalpel. Interact Cardiovasc Thorac Surg 4:
artery disease: new insights. Am Heart J 141:22–25 160–162
Athanasiou T, Aziz O, Al-Ruzzeh S et al. (2004) Are wound Carpentier A, Guernonprez JL (1973) The aorto-coronary
healing disturbances and length of hospital stay radial artery bypass. A technique avoiding pathologi-
reduced with minimally invasive vein harvest? A meta- cal changes in graft. Ann Thorac Surg 16:111–121
analysis. Eur J Cardiothorac Surg 26:1015–1026 Christenson JT, Schmuziger M (1997) Sequential venous
Barner HB, Sundt TM III, Bailey M, Zang Y (2001) Midterm bypass grafts: results 10 years later. Ann Thorac Surg
results of complete arterial revascularization in more 63:371–376
740 J.T. Cremer et al.
Cisowski M, Morawski W, Drzewiecki J et al. (2002) Garrett HE, Dennis EW, DeBakey ME (1973) Aortocoronary
Integrated minimally invasive direct coronary bypass bypass with saphenous vein graft: seven-year follow-
grafting and angioplasty for coronary artery revascu- up. JAMA 223:792–794
larization. Eur J Cardiothorac Surg 22:261–265 Gründeman PF, Verlaan CWJ, van Boven WJ, Borst C (2004)
Cohn WE, Suen HC, Weintraub RM, Johnson RG (1998) The Ninety-degree anterior cardiac displacement in off-
“H” graft: an alternative approach for performing mini- pump coronary artery bypass grafting: the Starfish
mally invasive direct coronary artery bypass. J Thorac cardiac positioner preserves stroke volume and arte-
Cardiovasc Surg 115:148–151 rial pressure. Ann Thorac Surg 78:679–684
Connolly MW, Torrillo LD, Stauder MJ et al. (2002) Halkos ME, Puskas JD (2010) Off-pump coronary surgery:
Endoscopic radial artery harvesting: results of first 300 where do we stand in 2010. Curr Opin Cardiol 25:
patients. Ann Thorac Surg 74:502–506 583–588
Cremer J, Teebken OE, Simon A, Hutzelmann A, Heller M, Halkos ME, Kerendi F, Corvera JS et al. (2004) Myocardial
Haverich A (1998) Thoracic computed tomography prior to protection with postconditioning is not enhanced by
redo coronary surgery. Eur J Cardiothorac Surg 13:650–654 ischemic preconditioning. Ann Thorac Surg 78:961–
Cremer JT, Wittwer T, Böning A et al. (2000) Minimally inva- 969
sive coronary artery revascularization on the beating Hannan EL, Racz MJ, Walford G et al. (2005) Long-term out-
heart. Ann Thorac Surg 69:1787–1791 comes of coronary-artery bypass grafting versus stent
De Paulis R, De Notaris S, Scaffa R et al. (2005) The effect of implantation. NEJM 352:2174–2183
bilateral internal thoracic artery harvesting on superfi- Hannan EL, Wu C, Walford G et al. (2008) Drug-eluting
cial and deep sternal infection: the role of skeletoniza- stents vs. coronary-artery bypass grafting in multives-
tion. J Thorac Cardiovasc Surg 129:536–543 sel coronary disease. NEJM 358:331–341
Diegeler A (1999) Left internal mammary artery grafting to Hannan EL, Racz MJ, Gold J et al. (2010) Adherence of cath-
left anterior descending coronary artery my minimally eterization laboratory cardiologist to American
invasive direct coronary artery bypass approach. Curr College of Cardiology/American Heart Association
Cardiol 1:323–330 guidelines for percutaneous coronary intervention
Dion R, Glineur D, Derouck D et al. (2000) Long-term clini- and coronary artery bypass graft surgery: what hap-
cal and angiographic follow-up of sequential internal pens in actual practice? Circulation 121:267–775
thoracic artery grafting. Eur J Cardiothorac Surg Hartmann A, Reuss W, Burger W, Kneissl GD, Rothe W,
17:407–414 Beyersdorf F (1998) Endothelium-dependent and
Eckstein FS, Bonilla LF, Englberger L et al. (2002) The St endothelium- independent flow reserve in vascular
Jude Medical symmetry aortic connector system for regions supplied by the internal mammary artery
proximal vein graft anastomoses in coronary artery before and after bypass grafting. Eur J Cardiothorac
bypass grafting. J Thorac Cardiovasc Surg 123:777–782 Surg 13:410–415
Egloff L, Laske A, Siebenmann R, Studer M (2002) Coronary Heilmann CAU, Attmann T, Thiem A, Haffner E, Beyersdorf
artery revascularization using both ITAs as free con- F, Lutter G (2003) Gene therapy in cardiac surgery:
duits in patients with three-vessel disease – the intramyocardial injection of naked plasmid DNA for
lambda graft. Thorac Cardiovasc Surg 50:31–34 chronic myocardial ischemia. Eur J Cardiothorac Surg
ElBardissi AW, Aranki SR, Sheng S et al. (2012) Trends in iso- 24:785–793
lated coronary artery bypass grafting: an analysis of Holzhey DM, Jacobs S, Mochalski M et al. (2008) Minimally
the Society of Thoracic Surgeons adult cardiac surgery invasive hybrid coronary artery revascularization. Ann
database. J Thorac Cardiovasc Surg 143:273–281 Thorac Surg 86:1856–1860
Favoloro RG (1968) Saphenous vein autograft replacement Hueb W, Soares PR, Gersh BJ et al. (2004) The Medicine,
of severe segmental coronary artery occlusion. Ann Angioplasty, or Surgery Study (MASS-II): a random-
Thorac Surg 5:334–339 ized, controlled clinical trial of three therapeutic strat-
Fazel S, Borger MA, Weisel RD et al. (2004) Myocardial pro- egies for multivessel coronary artery disease. JACC
tection in reoperative coronary artery bypass grafting: 43:1743–1751
toward decreasing morbidity and mortality. J Card BARI Investigators (1997) Influence of diabetes on 5-years
Surg 19:291–295 mortality and morbidity in a randomized trial compar-
Feng ZZ, Shi J, Zhao XW, Xu ZF (2009) Meta-analysis of on- ing CABG and PTCA in patients with multivessel dis-
pump and off-pump coronary arterial revasculariza- ease. Circulation 96:1761–1769
tion. Ann Thorac Surg 87:757–765 Jansen EWL, Lahpor JR, Borst C, Gründeman PF, Bredée JJ
Firstenberg MS, Azoury F, Lytle BW, Thomas JD (2000) (1998) Off-pump coronary bypass grafting: how to use
Interposition vein graft for giant coronary aneurysm the octopus tissue stabilizer. Ann Thorac Surg 66:576–579
repair. Ann Thorac Surg 70:1397–1398 Jones EL (1991) Preparation of the internal mammary
Franke U, Wahlers T, Cohnert TU et al. (2001) Retrograde artery for coronary bypass surgery. J Cardiac Surg
versus antegrade crystalloid cardioplegia in coronary 6:326–329
surgery: value of troponin-I measurement. Ann Thorac Jones CM, Athanasiou T, Dunne N et al. (2006) Multi-slice
Surg 71:249–253 computed tomography in coronary artery disease. Eur
Freshwater IJ, Morsi YS, Lai T (2006) The effect of angle on J Cardiothorac Surg 30:443–450
wall shear stresses in a LIMA to LAD anastomosis: Kappetein AP, Feldman TE, Mack MJ et al. (2011)
numerical modeling of pulsatile flow. Proc Inst Mech Comparison of coronary bypass surgery with drug-
22 Eng H 220:743–757 eluting stenting for the treatment of left main and/or
22 · Coronary Artery Disease
741 22
three-vessel disease: 3-year follow-up of the SYNTAX thoracic artery grafting on survival during 20 postop-
trial. Eur Heart J 32:2125–2134 erative years. Ann Thorac Surg 78:2005–2014
Katznelson Y, Petchenko P, Knobel B, Cohen AJ, Kishon Y, Mack MJ, Emery RW, Ley LR et al. (2003) Initial experience
Schachner A (1996) Myocardial bridging: surgical with proximal anastomoses performed with a
technique and operative results. Mil Med 161:248–250 mechanical connector. Ann Thorac Surg 75:1866–1871
Khot UN, Friedmann DT, Pettersson G, Smedira NG, Li J, Ellis Malyshev M, Gladyshev I, Safuanov A, Siniukov D,
SG (2004) Radial artery bypass grafts have an increased Borovikov D, Rostovykh N (2004) Surgical angioplasty
occurrence of angiographically severe stenosis and of the left main coronary artery and/or proximal seg-
occlusion compared with left internal mammary arteries ment of the right coronary artery by pulmonary auto-
and saphenous vein grafts. Circulation 109:2086–2091 graft patch. Eur J Cardiothorac Surg 25:21–25
Kiaii B, Moon BC, Massel D et al. (2002) A prospective ran- Maniar HS, Sundt TM, Barner HB et al. (2002) Effect of tar-
domized trial of endoscopic versus conventional har- get stenosis and location on radial artery graft
vesting of the saphenous vein in coronary artery bypass patency. J Thorac Cardiovasc Surg 123:45–52
surgery. J Thorac Cardiovasc Surg 123:204–212 Mariani MA, D’Alfonso A, Grandjean JG (2004) Total arterial
Klima U, Falk V, Maringka M et al. (2003) Magnetic vascular off-pump coronary surgery: time to change our hab-
coupling for distal anastomosis in coronary artery its? Ann Thorac Surg 78:1591–1597
bypass grafting: a multicenter trial. J Thorac Cardiovasc Marinelli G, Chiappini B, Di Eusanio M et al. (2002) Bypass
Surg 126:1568–1574 grafting with coronary endarterectomy: immediate
Klima U, MacVaugh H, Bagaev E et al. (2004) Magnetic vas- and long-term results. J Thorac Cardiovasc Surg
cular port in minimally invasive direct coronary artery 124:553–560
bypass grafting. Circulation 110:II-55–II-60 Mawatari T, Koshino T, Morishita K, Komatsu K, Abe T (2000)
Kolessov VI (1967) Mammary artery-coronary artery anas- Successful surgical treatment of giant coronary artery
tomosis as method of treatment for angina pectoris. aneurysm with fistula. Ann Thorac Surg 70:1394–1397
J Thorac Cardiovasc Surg 54:535–544 Menon AK, Albes JM, Oberhoff M, Karsch RK, Ziemer G
Kuecherer HF (2002) Use of intravascular ultrasound in (2002) Occlusion versus shunting during MIDCAB:
clinical trials. Heart Drug 2:24–34 effects on left ventricular function and quality of anas-
Kunadian B, Thornley AR, Tanos M, Dunning J (2006) Should tomosis. Ann Thorac Surg 73:1418–1423
clopidogrel be stopped prior to urgent cardiac surgery? Mohr FW, Rastan AJ, Serruys PW et al. (2011) Complex
Interact Cardiovasc Thorac Surg 5:630–636 coronary anatomy in coronary artery bypass graft sur-
Kushner FG, Hand M, Smith SC Jr et al. (2009) 2009 focused gery: impact of complex coronary anatomy in modern
updates: ACC/AHA guidelines for the management of bypass surgery? Lessons learned from the SYNTAX
patients with ST-elevation myocardial infarction (updating trial after two years. J Thorac Cardiovasc Surg
the 2004 guideline and 2007 focused update) and ACC/ 141:130–140
AHA/SCAI guidelines on percutaneous coronary interven- Murphy GJ, Bryan AJ, Angelini GD (2004) Hybrid coronary
tion (updating the 2004 guideline and 2007 focused revascularization in the era of drug-eluting stents. Ann
update) a report of the American College of Cardiology Thorac Surg 78:1861–1867
Foundation/American Heart Association Task Force on Programm für Nationale Versorgungsleitlinien (2008)
Practice Guidelines. J Am Coll Cardiol 54:2205–2241 Chronische KHK. Langfassung. April 2008. Version 1.8.
Kwak YL (2005) Reduction of ischemia during off-pump ÄZQ – Redaktion Versorgungsleitlinien, Berlin;
coronary artery bypass graft surgery. J Cardiothorac 7 www.Versorgungsleitlinien.de
Vasc Anesth 19:667–677 Ngaage DL (2003) Off-pump coronary artery bypass graft-
Lapenna D, Mezzetti A, de Gioia S et al. (1994) Blood car- ing: the myth, the logic and the science. Eur
dioplegia reduces oxidant burden in the ischemic and J Cardiothorac Surg 24:557–570
reperfused human myocardium. Ann Thorac Surg Ochi M, Hatori N, Bessho R et al. (2001) Adequacy of flow
57:1522–1525 capacity of bilateral internal thoracic artery T graft.
Laube HR, Duwe J, Rutsch W, Konertz W (2000) Clinical Ann Thorac Surg 72:2008–2012
experience with autologous endothelial cell-seeded Oliveira SA, Lisboa LAF, Dallan LAO, Rojas SO, de Figueiredo
polytetrafluoroethylene coronary artery bypass grafts. LFP (2002) Minimally invasive single-vessel coronary
J Thorac Cardiovasc Surg 120:134–141 artery bypass with the internal thoracic artery and
Lemma M, Mangini A, Redaelli A, Acocella F (2005) Do car- early postoperative angiography: midterm results of a
diac stabilizers really stabilize? Experimental quantita- prospective study in 120 consecutive patients. Ann
tive analysis of mechanical stabilization. Interact Thorac Surg 73:505–510
Cardiovasc Thorac Surg 4:222–226 Ong ATL, Serruys PW (2006) Coronary artery bypass graft
Liska J, Jönsson A, Lockowandt U, Herzfeld I, Gelinder S, surgery versus percutaneous coronary intervention.
Franco-Cereceda A (1999) Arterial patch angioplasty Circulation 114:249–255
for reconstruction of proximal coronary artery steno- Ono M, Wolf RK, Angouras D, Schneeberger EW (2002)
sis. Ann Thorac Surg 68:2185–2189 Early experience of coronary artery bypass grafting
Loop FD, Lytle BW, Cosgrove DM et al. (1986) Influence of with a new self-closing clip device. J Thorac Cardiovasc
the internal-mammary-artery graft on 10-year survival Surg 123:783–787
and other cardiac events. NEJM 314:1–6 Posacioglu H, Atay Y, Cetindag B, Saribülbül O, Buket S, Hamulu
Lytle BW, Blackstone EH, Sabik JF, Houghtaling P, Loop FD, A (1998) Easy harvesting of radial artery with ultrasoni-
Cosgrove DM (2004) The effect of bilateral internal cally activated scalpel. Ann Thorac Surg 65:984–985
742 J.T. Cremer et al.
Puig LB, Ciongolli W, Cividanes GV et al. (1990) Inferior epi- Suma H, Tanabe H, Takahashi A et al. (2007) Twenty years
gastric artery as a free graft for myocardial revascular- experience with the gastroepiploic artery graft for
ization. J Thorac Cardiovasc Surg 99:251–255 CABG. Circulation 116:I-188–I-191
Rivetti LA, Gandra MA (1997) Initial experience using an Task Force on Myocardial Revascularization of the
intraluminal shunt during revascularization of the European Society of Cardiology (ESC) and the
beating heart. Ann Thorac Surg 63:1742–1747 European Association for Cardio-Thoracic Surgery
Rodriguez E, Ormont ML, Lambert EH et al. (2001) The role (EACTS), European Association for Percutaneous
of preoperative radial artery ultrasound and digital Cardiovascular Interventions (EAPCI), Kolh P, Wijns W,
plethysmography prior to coronary artery bypass Danchin N et al. (2010) Guidelines on myocardial
grafting. Eur J Cardiothorac Surg 19:135–139 revascularization. Eur J Cardiothorac Surg 2010(38
Ruengsakulrach P, Sinclair R, Komeda M, Raman J, Gordon Suppl):S1–S52
I, Buxton B (1999) Comparative histopathology of Toda K, Mackenzie K, Mehra MR et al. (2002) Revascularization
radial artery versus internal thoracic artery and risk in severe ventricular dysfunction (15 % ≤ LVEF ≤ 30 %):
factors for development of intimal hyperplasia and a comparison of bypass grafting and percutaneous
atherosclerosis. Circulation 100:II-139–II-144 intervention. Ann Thorac Surg 74:2082–2087
Rukosujew A, Reichelt R, Fabricius AM et al. (2004) Tonino PA, De Bruyne B, Pijls NH, FAME Study Investigators
Skeletonization versus pedicle preparation of the et al. (2009) Fractional flow reserve versus angiogra-
radial artery with and without the ultrasonic scalpel. phy for guiding percutaneous coronary intervention.
Ann Thorac Surg 77:120–125 N Engl J Med 360:213–224
Samani NJ, Erdmann J, Hall AS et al. (2007) Genomewide Tozzi P, Corno AF, von Segesser LK (2002) Sutureless coro-
association analysis of coronary artery disease. NEJM nary anastomoses: revival of old concepts. Eur
357:443–453 J Cardiothorac Surg 22:565–570
Scott NA, Knight JL, Bidsrup BP, Wolfenden H, Linacre RN, Tran HM, Paterson HS, Meldrum-Hanna W, Chard RB (1998)
Maddern GJ (2002) Systematic review of beating heart Tunnelling versus open harvest technique in obtain-
surgery with the octopus tissue stabilizer. Eur ing venous conduits for coronary bypass surgery. Eur
J Cardiothorac Surg 21:804–817 J Cardiothorac Surg 14:602–606
Serruys PW, Ong AT, van Herwerden LA et al. (2005) Five- Vineberg AM, Miller G (1951) Internal mammary coronary
year outcomes after coronary stenting versus bypass anastomosis in the surgical treatment of coronary
surgery for the treatment of multivessel disease: the artery insufficiency. Can Med Assoc J 64:204–210
final analysis of the Arterial Revascularization Watanabe G, Takemura H, Tomita S, Nagamine H, Kamiya H,
Therapies Study (ARTS) randomized trial. J Am Coll Kanamori T (2004) Skeletonized radial artery graft with
Cardiol 46:575–581 the St. Jude Medical Symmetry Bypass System (aortic
Serruys PW, Morice MC, Kappetein AP et al. (2009) connector system). Ann Thorac Surg 77:1550–1552
Percutaneous coronary intervention versus coronary- Westaby S, Vaccari G, Katsumata T (1999) Direct repair of
artery bypass grafting for severe coronary artery dis- giant right coronary aneurysm. Ann Thoray Surg
ease. NEJM 360:961–972 68:1401–1403
Shroyer AL, Grover FL, Hattler B et al. (2009) Veterans affairs Wippermann J, Albes JM, Bruhin R et al. (2004) Chronic
Randomized On/Off Bypass (ROOBY) Study Group. ultrastructural effects of temporary intraluminal
On-pump versus off-pump coronary-artery bypass shunts in a porcine off-pump model. Ann Thorac Surg
surgery. N Engl J Med 361:1827–1837 78:543–548
Shuhaiber JH, Evans AN, Masad MG, Geha AS (2002) Yilmaz AT, Ozal E, Barindik N, Günay C, Tatar H (2002) The
Mechanisms and future directions for prevention of results of radial artery Y-graft for complete arterial
vein graft failure in coronary bypass surgery. Eur revascularization. Eur J Cardiothorac Surg 21:794–799
J Cardiothorac Surg 22:387–396 Yoshikai M, Ito T, Kamohara K, Yunoki J (2004) Endothelial
SOS Investigators (2002) Coronary artery bypass surgery integrity of ultrasonically skeletonized internal tho-
versus percutaneous coronary intervention with stent racic artery: morphological analysis with scanning
implantation in patients with multivessel coronary electron microscopy. Eur J Cardiothorac Surg 25:
artery disease (the Stent or Surgery trial): a ran- 208–211
domised controlled trial. Lancet 360:965–970 Zacharias A, Habib RH, Schwann TA et al. (2004) Improved
Suma H, Fukumoto H, Takeuchi A (1987) Coronary artery survival with radial artery versus vein conduits in coro-
bypass grafting by utilizing in situ right gastroepiploic nary bypass surgery with left internal thoracic artery
artery: basic study and clinical application. Ann Thorac to left anterior descending artery grafting. Circulation
Surg 44:394–397 109:1489–1496
22
743 23
Left Ventricular
Reconstruction
and Conventional Surgery
for Cardiac Failure
Friedhelm Beyersdorf and Matthias Siepe
References – 756
a b
Thrombus
..Fig. 23.2 a, b. Elevation of left ventricular apex using pads and incision in the scar of the left ventricle lateral to the
left anterior descending coronary artery with a scalpel a; thrombotic material in the cavity of the left ventricle b
748 F. Beyersdorf and M. Siepe
23
..Fig. 23.5 Schematic drawings of the normal elliptical shape of the heart (left), the spherical shape of the dilated
heart after anterior-septal myocardial infarction (middle) and the reconstructed elliptical configuration after left
ventricular reconstruction (right)
a b
..Fig. 23.6 a After the second purse-string suture has been tied, b the remaining myocardial opening in the left
ventricle in most cases is less than 1–2 cm in size.
nonvital myocardium. Prophylactic or secondary dial opening effectively and create a new left ven-
implantation of a defibrillator is rarely necessary. tricular apex. Using these techniques, the left
After the purse-string suture has been tied, ventricle will regain its elliptical shape (. Fig. 23.5).
most often the myocardium of the left ventricle is If the first purse-string suture does not com-
already closed. If an opening remains, which is less pletely occlude the left ventricular myocardial
than 1–1.5 cm in diameter, further direct closure is defect, a second purse-string suture can be carried
possible: Three U-stitches (polypropylene sutures) out above the first one (. Fig. 23.6). With these
can be positioned at an angle of 60° to each other double- layer purse-string sutures, the opening
(. Fig. 23.4). They occlude the remaining myocar- almost always can be occluded.
750 F. Beyersdorf and M. Siepe
Area of Coaptation
Reduced
closure force
Ring dilatation
Increased tension
on the chordae
Enlarged distance between
the papillary muscles
..Fig. 23.8 a–c Normal functional anatomy with good coaptation areas a; development of functional mitral valve
insufficiency b; sutures in the anulus before and after tying with use of a complete mitral valve ring c
optimum preoperative hemodynamics is of upmost surgery, the poorer the outcome (Deja et al. 2000;
importance to the operative outcome. Whether a Labrousse et al. 2002). The fragility of the septal
preoperative phase of mechanical circulatory tissue where the patch is anchored is the main rea-
support as a routine treatment tool is beneficial son for residual shunts (up to 40 %) and causes
awaits investigation in future series. significant mortality rates (Deja et al. 2000; Pretre
Preoperatively, repetitive transesophageal et al. 1999).
echocardiography (TEE) and thorough hemody-
namic monitoring are mandatory. Peripheral 23.3.3.2 Surgical Strategy
organ function has to be screened and taken care Historically, the first successful post-infarct VSD
of as necessary. The hemodynamic status deter- closure was achieved by Denton Cooley in 1957
mines the time for surgery, with instability being (Cooley et al. 1957). Many surgical techniques
the indication for defect closure. This in itself is and perioperative tools have been described
one of the main prognostic factors for outcome. over the past 55 years to treat this defect and
The sooner a post-infarction VSD requires improve patient care. A preoperative IABP may
754 F. Beyersdorf and M. Siepe
23
..Fig. 23.9 Patch closure of an anterior (left Panels) and posterior (right Panels) post-infarction VSD. The access
through the infarcted LV free wall may or may not be closed with a second patch in anterior infarctions. For posterior
VSDs a patch repair of the LV access is always recommended (Adapted from Wahlers and Franke 2003)
be inserted and maintained for some days post- The techniques performed most often closely follow
operatively. Using general anesthesia and total the work of Dagget (Daggett et al. 1977). After inci-
hemodynamic monitoring including pulmonary sion in the left ventricular wall parallel to the LAD,
artery catheter and TEE, the operation is con- the defect is localized and a patch inserted using big
ducted employing full sternotomy using stan- stitches for Teflon-augmented single or running
dard cardiopulmonary bypass and cardioplegic sutures. The patch (of bovine pericardium or
arrest. Surgery using induced ventricular fibrilla- Dacron) should be large enough and placed broadly
tion has not proven to be superior to cardiople- overlapping the healthy myocardial tissue around
gic arrest (Deja et al. 2000; Labrousse et al. 2002; the VSD and its infracted borders. In anterior
Gula and Yacoub 1981). defects, access in the left anterior wall can be closed
The scar tissue of the infarction is routinely used using Teflon felt-augmented direct sutures or a sec-
for surgical access to the defect. Most described sur- ond patch. When choosing an incision in the poste-
gical procedures can be performed through an ante- rior wall for posterior defects, it is recommended to
rior or posterior scar region. In some patients always use a second patch for LV wall reconstruc-
trans-right atrial and trans-tricuspid valve access tion. The patch on the fragile septum issue can be
is possible. However, the right ventricular trabe secured using biologic glue material injected into
culae at times make localising the defect and safe the tissue (Daggett et al. 1977; Tanaka et al. 2001)
patch insertion difficult (Massetti et al. 2000). (. Fig. 23.9).
Chapter 23 · Left Ventricular Reconstruction and Conventional Surgery for Cardiac Failure
755 23
tated (. Fig. 23.11). The apex is then reconstructed
using four strips of Teflon felt – one on each ven-
tricular free wall side and one on each side of the
septum, excluding the infarcted VSD area once the
sutures are tied (Khonsari and Sintek 2008).
and more prospective data is necessary before DiCarli MF, Maddahi J, Roshar S et al. (1998) Long-term sur-
23 these alternative treatments are being accepted for vival of patients with coronary artery disease and left
ventricular dysfunction: implications for the role of
routine use. Some of these methods are still in the myocardial viability assessment in management deci-
experimental stage. These include dynamic car- sions. J Thorac Cardiovasc Surg 116:997–1004
diomyoplasty procedures (Lange et al. 1995), bio- DiDonato M, Sabatier M, Dor V, Toso A, Maioli M, Fantini F
logical pulsation with aortoplasty (Guldner et al. (1997) Akinetic versus dyskinetic postinfarction scar:
2000), skeletal muscle ventricle (Guldner et al. relation to surgical outcome in patients undergoing
endoventricular circular patch plasty repair. Am J Coll
2001), left ventricular constraint device (Konertz Cardiol 29:1569–1575
et al. 2001), myosplint implantation (Fukamachi DiDonato M, Sabatier M, Dor V et al. (2001) Effects of the Dor
and McCarthy 2005) and transmyocardial laser procedure on left ventricular dimensions and shape
revascularization (Lutter et al. 2002) – just to and geometric correlates of mitral regurgitation one
name a few. year after surgery. J Thorac Cardiovasc Surg 121:91–96
DiDonato M, Castelvecchio S, Menicanti L (2010) End-
systolic volumes following surgical ventricular recon-
struction impacts survival in patients with ischaemic
References dilated cardiomyopathy. Eur J Heart Fail 12: 375–381
Dor V (1997) Reconstructive left ventricular surgery for
postischemic akinetic dilatation. Semin Thorac
Athanasuleas CL, Stanley AWH, Buckberg GD et al. (2001) Cardiovasc Surg 9:139–145
Surgical anterior ventricular endocardial restoration Dor V (2004) Left ventricular reconstruction: the aim and
(SAVER) for dilated ischemic cardiomyopathy. Sem the reality after twenty years. J Thorac Cardiovasc Surg
Thorac Cardiovasc Surg 13:448–457 128:2351–2357
Athanasuleas CL, Buckberg GD, Stanley AWH, the RESTORE Dor V (2011) Favorable effects of left ventricular recon-
group et al. (2004) Surgical ventricular restoration in the struction in patients excluded from the Surgical
treatment of congestive heart failure due to postinfarc- Treatments for Ischemic Heart Failure (STICH) trial.
tion ventricular dilatation. JACC 44:1439–1445 J Thorac Cardiovasc Surg 141:905–916
Attia R, Blauth C (2010) Which patients might be suitable Elefteriades J, Edwards R (2002) Coronary bypass in left
for a septal occluder device closure of postinfarction heart failure. Semin Thorac Cardiovasc Surg 14:125–
ventricular septal rupture rather than immediate sur- 132
gery? Interact Cardiovasc Thorac Surg 11:626–629 Fukamachi K, McCarthy P (2005) Initial safety and feasibil-
Baldi A, Abbate A, Bussani R et al. (2002) Apoptosis and ity clinical trial of the myosplint device. J Cardiac Surg
postinfarction left ventricular remodeling. J Mol Cell 20:S43–S47
Cardiol 34:165–174 Gaudron P, Eilles C, Kugler I, Ertl G (1993) Progressive left
Bolling SF, Pagani FD, Deeb GM et al. (1998) Intermediate- ventricular dysfunction and remodeling after myocar-
term outcome of mitral reconstruction in cardiomy- dial infarction: potential mechanisms and early pre-
opathy. J Thorac Cardiovasc Surg 115:381–388 dictors. Circulation 87:755–763
Brandt B III, Wright CB, Ehrenhaft JL (1979) Ventricular sep- Gheorghiade M, Bonow RO (1998) Chronic heart failure in
tal defect following myocardial infarction. Ann Thorac the United States: a manifestation of coronary artery
Surg 27:580–589 disease. Circulation 97:282–289
Buckberg GD, Athanasuleas CL (2009) The STICH trial: mis- Gula G, Yacoub MH (1981) Surgical correction of complete
guided conclusions. J Thorac Cardiovasc Surg rupture of the anterior papillary muscle. Ann Thorac
138:1060–1064 Surg 32:88–91
Cooley DA, Belmonte BA, Zeis LB, Schnur S (1957) Surgical Guldner NW, Klapproth P, Großherr M et al. (2000)
repair of ruptured interventricular septum following Clenbuterol- supported dynamic training of skeletal
acute myocardial infarction. Surgery 41:930–937 muscle ventricles against systemic load. A key for pow-
Daggett WM, Guyton RA, Mundth ED, Buckley MJ, McEnany erful circulatory assist? Circulation 101:2213–2219
MT, Gold HK, Leinbach RC, Austen WG (1977) Surgery Guldner NW, Klapproth P, Großherr M et al. (2001)
for post-myocardial infarct ventricular septal defect. Biomechanical hearts: muscular blood pumps, per-
Ann Surg 186:260–271 formed in a 1-step operation, and trained under sup-
Deja MA, Szostek J, Widenka K, Szafron B, Spyt TJ, Hickey MS, port of clenbuterol. Circulation 104:717–722
Sosnowski AW (2000) Post infarction ventricular septal Jones RH (2001) Is it time for a randomized trial of surgical
defect; can we do better? Eur J Cardiothorac Surg 18:194–201 treatment of ischemic heart failure? J Am Col Cardiol
Deville C, Labrousse L, Coukroun E, Madonna F (2005) 35:1210–1212
Surgery for post-infarction ventricular septal defect Jones RH, Velazquez EJ, Michler RE et al. (2009) Coronary
(VSD): double patch and glue technique for early bypass surgery with or without surgical ventricular
repair. Multimedia Manual of Cardiothoracic Surg doi: reconstruction. New Engl J Med 360:1705–1717
10.1510/mmcts.2004.000562 Khonsari S, Sintek CF (2008) Mechanical complications of
Di Donato M, Castelvecchio S, Menicanti L (2010) End- myocardial infarction. In: Khonsari SSCF (ed) Cardiac
systolic volumes following surgical ventricular recon- surgery; safeguards and pitfalls in operative t echnique,
struction impacts survival in patients with ischaemic 4th edn. Lippincott Williams and Wilkins, Philadelphia,
dilated cardiomyopathy. Eur Heart J pp 178–179
Chapter 23 · Left Ventricular Reconstruction and Conventional Surgery for Cardiac Failure
757 23
Komajda M, Lutiger B, Madeira H, Thygesen K, Bobbio M, Pitt B, Zannad F, Remme WJ et al. (1999) The effect of spi-
Hildebrandt P, Jaarsma W, Riegger G, Ryden L, Scherhag ronolactone on morbidity and mortality in patients
A, Soler-Soler J, Remme WJ, On behalf of the CARMEN with severe heart failure. Randomized Aldactone
investigators and Co-ordinators (2004) Tolerability of Evaluation Study Investigators. N Engl J Med 341:
carvedilol and ACE-inhibition in mild heart failure. 709–717
Results of the CARMEN (carvedilol ACE-inhibitor remod- Pretre R, Ye Q, Grunenfelder J, Lachat M, Vogt PR, Turina MI
elling mild CHF evaluation). Eur J Heart Fail 6:467–475 (1999) Operative results of “repair” of ventricular septal
Konertz W, Dushe S, Hotz H et al. (2001) Safety and feasibility rupture after acute myocardial infarction. Am J Cardiol
of a cardiac support device. J Cardiac Surg 16:113–117 84:785–788
Labrousse L, Choukroun E, Chevalier JM, Madonna F, Reimer KA, Jennings RB (1979) The “wavefront phenome-
Robertie F, Merlico F, Coste P, Deville C (2002) Surgery non” of myocardial ischemic cell death. II. Transmural
for post infarction ventricular septal defect (VSD): risk progression of necrosis within the framework of isch-
factors for hospital death and long term results. Eur emic bed size (myocardium at risk) and collateral flow.
J Cardiothorac Surg 21:725–731 Lab Invest 40:633–644
Lange R, Sack FU, Voss B (1995) Dynamic cardiomyoplasty: Remme WJ (2003) Should ACE inhibition always be first-
indication, surgical technique, and results. Thorac line therapy in heart failure? Lessons from the CARMEN
Cardiovasc Surg 43:243–251 Study. Cardiovasc Drugs Ther 17:107–109
Lee EM, Roberts DH, Walsh KP (1998) Transcatheter closure Sallin EA (1969) Fiber orientation and ejection fraction in
of a residual postmyocardial infarction ventricular sep- the human left ventricle. Biophys J 9:954–964
tal defect with the Amplatzer septal occluder. Heart Shah PJ, Hare DL, Raman JS et al. (2003) Survival after myo-
80:522–524 cardial revascularization for ischemic cardiomyopathy:
Lee MS, Kozitza R, Mudrick D, Williams M, Lodge AJ, a prospective ten-year follow-up study. J Thorac
Harrison JK, Glower DD (2010) Intraoperative device Cardiovasc Surg 126:1320–1327
closure of postinfarction ventricular septal defects. Tanaka H, Hasegawa S, Sakamoto T, Sunamori M (2001)
Ann Thorac Surg 89:e48–e50 Postinfarction ventricular septal perforation repair with
Lutter G, Attmann T, Heilmann C, von Samson P, von Specht endoventricular circular patch plasty using double
B, Beyersdorf F (2002) The combined use of transmyo- patches and gelatin-resorcinol-formaldehyde biologi-
cardial laser revascularization (TMLR) and fibroblastic cal glue. Eur J Cardiothorac Surg 19:945–948
growth factor (FGF-2) enhances perfusion and The CONSENSUS Trial Study Group (1987) Effects of enala-
regional contractility in chronically ischemic porcine pril on mortality in severe congestive heart failure:
hearts. Eur J Cardio-Thorac Surg 22:753–761 results of the Cooperative North Scandinavian
Madsen JC, Daggett WM (1997) Postinfarction ventricular Enalapril Survival Study (CONSENSUS). N Engl J Med
septal defect and free wall rupture. In: Edmunds LH Jr 316:1429–1435
(ed) Cardiac surgery in the adult. McGrave-Hill, The RALES Investigators (1996) Effectiveness of
New York, pp 629–655 Spironolactone added to an Angiotensin-Converting
Massetti M, Babatasi G, Le PO, Bhoyroo S, Saloux E, Khayat Enzyme Inhibitor and a loop diuretic for severe
A (2000) Postinfarction ventricular septal rupture: chronic congestive heart failure (The Randomized
early repair through the right atrial approach. J Thorac Aldactone Evaluation Study [RALES]). Am J Cardiol 87:
Cardiovasc Surg 119:784–789 902–907
Maxey TS, Reece TB, Ellman PI et al. (2004) Coronary Thiele H, Kaulfersch C, Daehnert I, Schoenauer M, Eitel I,
artery bypass with ventricular restoration is superior Borger M, Schuler G (2009) Immediate primary trans-
to coronary artery bypass alone in patients with catheter closure of postinfarction ventricular septal
ischemic cardiomyopathy. J Thorac Cardiovasc Surg defects. Eur Heart J 30:81–88
127:428–434 Velazquez EJ, Lee KL, O’Connor CM, STICH investigators
Migrino RQ, Young JB, Ellis SG et al. (1997) End-systolic volume et al. (2007) The rationale and design of the surgical
index at 90 to 180 minutes into reperfusion therapy for treatment for ischemic heart failure (STICH) trial.
acute myocardial infarction is a strong predictor of early J Thorac Cardiovasc Surg 123:1540–1547
and late mortality. The Global Utilization of Streptokinase Wahlers T, Franke UF (2003) Ventricular septal defect and
and t-PA for Occluded Coronary Arteries (GUSTO)-I papillary muscle rupture; surgical options and results.
Angiographic Investigators. Circulation 96:116–121 Dtsch Med Wochenschr 128:810–814
Narula J, Haider N, Virmani R et al. (1996) Apoptosis in myocytes White HD, Norris RM, Brown MA, Brandt PW, Whitlock RM,
in end-stage heart failure. N Engl J Med 335:1182–1189 Wild CJ (1987) Left ventricular end-systolic volume as
Olivetti G, Abbi R, Quanini F et al. (1997) Apoptosis in the the major determinant of survival after recovery from
failing human heart. N Engl J Med 336:1131–1141 myocardial infarction. Circulation 76:44–51
Packer M, Coats AJ, Fowler MB et al. (2001) Effect of Yamaguchi A, Ino T, Adachi H et al. (1998) Left ventricular
carvedilol on survival in severe chronic heart failure. N volume predicts postoperative course in patients with
Engl J Med 344:1651–1658 ischemic cardiomyopathy. Ann Thorac Surg 65:434–438
759 24
References – 790
Chapter 24 · Acquired Lesions of the Aortic Valve
761 24
24.1 Introduction Don Shiley later developed a mechanical convex-
concave tilting valve. The first valve had an opening
Surgery on the aortic valve is now a routine cardiac angle of 60° and was on market from 1969 till 1986.
procedure performed in Germany (population The subsequent model with only one strut (mono-
>81,000,000) in 2010 in almost 11,700 patients strut), improved metal frame, and opening angle of
(Gummert et al. 2011). The etiology of aortic valve 70° was available from 1982; however, due to sporadic
disease is either aortic valve stenosis, aortic regurgita- reports of design failure and fracture, it was removed
tion, or a combination of these. Replacement options from the market in 1986. During 1977, St. Jude
include mechanical or biological prostheses. Biological Medical Company entered the market with the first
prostheses (xenografts or heterografts: tissue from b-leaflet valve with an opening angle of 85°.
another species) are available as stentless or as stented On July 24, 1962, Donald performed the first
valves. Stented prostheses utilize a stent frame to subcoronary aortic valve homograft replacement
mount the biological tissue that will provide the valve in London, England, employing a single suture
mechanism, while stentless prostheses utilize either a technique as personally suggested to him by
complete porcine aortic root or an aortic root com- Gunning and Duran (Ross 1962). Brian Barratt-
pletely constructed from xenopericardium of porcine Boyes, working independently, performed his first
or bovine origin. Biological aortic valve substitutes subcoronary aortic valve homograft implantation
also include homografts (homografts or allografts: tis- on August 23, 1962, employing a what he later
sue from the same species) and autografts (tissue from called a more modern double suture technique
the same individual) as in the Ross procedure. derived from his own experience (Barratt-Boyes
1964; Kirklin and Barratt-Boyes 1986). They both
used sterilized freeze-dried cadaveric homografts.
24.2 Historical Insights Marion Ionescu from Leeds, UK, developed an
autologous valve using tissue harvested from the
The first aortic valve intervention was performed by patient’s fascia lata mounted on a metal stent
the French surgeon Theodore Tuffier (1857–1929) frame. Later on, xenopericardium was utilized as
and published by Alexis Carrel. They performed the valve leaflet material. Carlos Duran and Alfred
first dilatation of a stenotic aortic valve on July 13, Gunning in Oxford concentrated their efforts on
1912 in a 26-year-old patient by invaginating the aor- conservation of xenograft tissue, and on September
tic wall through the aortic valve. Later on, Carrel per- 23, 1964, the first stented porcine valve could be
formed the first anastomosis between the left ventricle implanted. Alan Carpentier from Paris in cooper-
and the descending aorta in animal experiments, as a ation with Miles L. Edwards developed and later
mean to bypass a stenotic valve. No realistic attempts marketed their first biological valve in the late
were performed thereafter until the end of the Second 1960s. Quicksilver was initially employed for the
World War. During 1950, an aortic valve dilatator was conservation of the tissue utilized for the first bio-
developed by Charles Bailey. It presented a variation logical valves; however later on, quicksilver was
of the mitral valve dilator (originally developed by replaced by formaldehyde and glutaraldehyde.
Lord Brock) but incorporated a three-piece head that
could be rotated to match and eventually dilate/dis-
rupt the three commissures. On September 11, 1952, 24.3 General Knowledge
Charles A. Hufnagel (1917–1989) at Georgetown
University implanted a self-made valve in the The aortic root provides the connection between
descending aorta which resulted in a reduction of the left ventricle and the ascending aorta. The aor-
aortic valve regurgitation present by 70 %. Originally tic valve resides inside the aortic root and consists
the valve inside the valve cage was made from metal; of several structures. The anatomic and physio-
later on, however, it was replaced by a silicone sphere. logical borders between the aortic valve and the
On March 10, 1960, Dwight E. Harken (1910–1993) left ventricle are not identical (Sievers et al. 2012).
implanted a self-made mechanical aortic valve in the
anatomic position. The thoracic surgeon Albert Starr
and the valve engineer Miles Lowell Edwards devel- 24.3.1 Aortic Root Anatomy
oped a mechanical prosthesis originally meant for the
mitral position. Model 1260 was implanted for the The three leaflets provide the main closing mech-
first time in 1966 in aortic position. Viking Björk and anism of the aortic valve. The form of the leaflets
762 M. Misfeld et al.
and leaflet attachments give the aortic valve a of the coronary arteries, and the sinuses are termed
crown shape, and thus it is described as a «semilu- accordingly as left, right, and noncoronary sinus.
nar valve.» Anatomically a valve leaflet can be The areas where neighboring leaflet attach-
24 divided into three parts: ments run parallel are called commissure. The
55 The free margin, with a thickened circular area underneath each commissure is called an
node (nodulus Aranti), which provides the interleaflet triangle. The triangle between the
coaptation area to the corresponding neigh- right and noncoronary sinus is part of the mem-
boring valve leaflet branous septum and hosts the His bundle. This
55 The «belly» of the leaflet area is of special importance during aortic valve
55 The basal parts of the leaflet or leaflet attach- procedures, as injury here may lead to tempo-
ments rary or permanent conduction abnormalities,
potentially requiring implantation of a perma-
The leaflet attachments insert in the wall of the nent cardiac pacemaker system. Under the left/
aortic root and thus form a crown-shaped struc- noncoronary trigonum, the aortomitral curtain
ture, often called «anulus.» This description can be leads to the anterior mitral valve leaflet. The dis-
misleading as the word anulus refers to a c ircular tal border of the sinuses toward the ascending
structure in contrast to the «crown»-formed shape aorta as well as the top of the commissures forms
of the leaflet attachment (Sievers et al. 2012). a circular structure named «sinotubular junc-
The three bulges of the aortic wall are the sinuses tion» which separates the aortic root from the
of Valsalva. Two of the three sinuses host the origins ascending aorta (. Fig. 24.1) (Sievers et al. 2012).
C C
C
Conduction system
Aorto-mitral
(His bundle)
continuity
Anterior mitral
Muscular septum Membranous septum valve leaflet
Muscular septum
C = Commissure
D = Trigona
..Fig. 24.1 Schematic drawing of the aortic root and the left ventricular outflow tract. Injuries to the His bundle,
which runs in close proximity to the membranous septum on its left ventricular aspect, may cause temporary or perma-
nent postoperative conduction abnormalities. The aortomitral valve continuity reflects the close anatomic relationship
of the mitral and aortic valve in normal hearts
Chapter 24 · Acquired Lesions of the Aortic Valve
763 24
24.3.2 Aortic Valve Disease aortic stenosis, and thus it is not surprising that the
frequency of interventions in patients with bicus-
Aortic valve disease is classified in pure stenosis, pid aortic valves is much higher than in patients
pure regurgitation, and a combination of the with normal, tricuspid aortic valves (Roberts and
above, where both forms of disease and hemody- Ko 2005). Various phenotypes of bicuspid aortic
namics coexist. Aortic valve stenosis can be fur- valves have been classified, which allow a more
ther classified into valvular, subvalvular, and detailed classification of the pathology and recon-
supravalvular stenosis. struction techniques (Sievers and Schmidtke 2007).
Principally, all techniques employed for aortic
24.3.2.1 Aortic Valve Stenosis valve surgery in tricuspid aortic valves can also be
Before 1950, rheumatic aortic valve disease after employed in patients with bicuspid aortic valves.
rheumatic fever due to streptococcal infection was Bicuspid aortic valve repair can lead to satisfactory
the most frequent etiology for aortic valve disease short- and midterm results (Davierwalla et al.
(Rahimtoola and Frye 2000). After the introduction 2003; El Khoury et al. 2006). Similarly, valve-
of penicillin prophylaxis and treatment, rheumatic sparing aortic root replacement can also be per-
heart valve disease has been dramatically reduced formed in the setting of a bicuspid aortic valve.
in the western world. Here today, calcified aortic In the setting of bicuspid aortic valves, all con-
valve stenosis represents the most frequent form of ventional aortic valve replacement procedures
stenotic aortic valve disease, mainly due to the (mechanical, biological, homograft) can be per-
increased life expectancy of the population. This formed without concern. The anatomy of the cor-
form of disease, however, in contrast to the popular onary ostia, however, must be taken into special
belief, does not represent a mere time-dependent consideration, since in these patients, different to
degeneration process but rather an active disease the three leaflet setting, the two coronary ostia are
with aspects similar to the development of athero- often at a 180° angle apart, and as such some
sclerosis. Deposits of lipoproteins lead to a chronic matching and special orientation of the prosthesis
tissue inflammation and valve calcification at implantation might be required.
(Freeman and Otto 2005). There is however evi-
dence of a genetic component in the development 24.3.2.4 Natural History
of the aortic valve calcification (Probst et al. 2006). Calcified aortic valve stenosis represents a dynamic
process. Mild or moderate aortic valve stenosis can
24.3.2.2 Aortic Valve Regurgitation be well tolerated; however severe stenosis leads to
In the first half of the twentieth century, endocardi- increased morbidity and mortality. When combined
tis as complication of tertiary syphilis was often the with other comorbidities, often interdependent,
etiology of aortic valve regurgitation (Reader et al. such as low ventricular function, chronic renal dis-
1947). In the rheumatic form of aortic regurgita- ease, or advanced age, aortic valve stenosis can
tion, retracted, shortened aortic valve leaflets lead shorten life expectancy (Carabello and Paulus 2009).
to the development valve incompetence and pro- Patients with aortic valve insufficiency can be
gressive aortic valve regurgitation. Other reasons asymptomatic for quite some time. Once the
for the development of aortic regurgitation are patient becomes symptomatic, chronic dilatation
destruction of the aortic leaflets due to infective of the left ventricle is already present, and an
endocarditis, posttraumatic leaflet injury, as well as acceleration of the natural history and symptom
complications of type A aortic dissection. In many development takes place.
cases, dilatation of the aortic root cannot any lon-
ger warrant a sufficient closing mechanism of the
aortic valve leading to aortic valve regurgitation. 24.3.3 Diagnostic Modalities
for Aortic Valve Disease
24.3.2.3 Bicuspid Aortic Valve
A bicuspid aortic valve has an incidence of about Symptom development can often be misleading, as
2 % and is the most common congenital heart some patients develop symptoms even with mild
defect. Diverse factors play a role in the embryo- aortic valve disease, while others may be asymp-
genesis of a bicuspid aortic valve. There is a ten- tomatic even in presence of severe aortic stenosis or
dency for bicuspid aortic valves to develop calcified regurgitation. In patients with aortic valve disease,
764 M. Misfeld et al.
symptoms often include angina. Symptoms also for aortic valve surgery. It should be taken into con-
can be related to the development of heart failure, sideration that the transvalvular gradient depends
like dyspnea and orthopnea. Syncope often occurs on the systolic left ventricular function, and in
24 in patients with significant aortic valve stenosis. patients with severely depressed left ventricular
Some patients with severe aortic valve stenosis systolic function, severe aortic valve stenosis can
can present with gastrointestinal bleeding. The coexist with low transvalvular gradient. These
etiology of this has only recently been unmasked. patients also profit from aortic valve replacement
This clinical syndrome consists of gastrointestinal despite low contractile reserve (Quere et al. 2006).
angiodysplasia in combination with a bleeding With respect to the aortic valve area, there is a con-
disorder mainly attributed to von Willebrand fac- sensus that an area below 1.0 cm2 represents a
tor dysfunction and has been coined «Heyde syn- severe aortic valve stenosis as depicted in the ACC/
drome» (Pate et al. 2004); therapy of choice is AHA guidelines (Bonow et al. 2006) (. Table 24.1).
aortic valve replacement. Most patients with asymptomatic severe aor-
The clinical examination reveals a pathologi- tic valve stenosis will eventually develop clinical
cal systolic murmur at the second intercostal symptomatology within the first 5 years after the
space radiating to the base of the neck and carotid diagnosis and carry a risk for sudden death of
arteries in cases of aortic stenosis and an early about 1 %/year (Pellika et al. 2005). An exercise
diastolic murmur radiating to the heart apex in test can help differentiate and uncover symptoms,
patients with aortic regurgitation. as many «asymptomatic» patients will develop
Chest x-ray may reveal signs of left ventricular typical symptoms at exercise (Das et al. 2005).
hypertrophy or dilatation as well as calcification of Patients with acute, severe aortic insufficiency
the aortic valve. Echocardiography establishes the typically develop pulmonary congestion and hypo-
diagnosis of aortic valve disease, as well as the grading tension, which may lead to cardiogenic shock and
of its severity with measurement of transvalvular gra- should be referred for urgent surgical intervention.
dients, aortic valve area, and grade of valvar regurgi- The time point of surgical intervention in patients
tation. Aortic root dimensions, grade of calcification, with chronic aortic valve regurgitation is of great
as well as leaflet mobility and presence of vegetations importance. Surgical intervention in these patients
in cases of endocarditis can also be evaluated. should be performed before the left ventricular ejec-
Left heart catheterization with cardio- tion fraction falls below 55 % and before the (cor-
angiography provides invasive measurements of rected) left ventricular end diastolic diameter
transvalvular gradients as well as grading of the increases above 55 mm (Bekeredijan and Grayburn
aortic regurgitation. It should be performed prior 2005). According to the AHA/ACC guidelines,
to intervention in all patients to evaluate potential patients referred for surgical intervention before the
coexisting coronary artery disease that can be development of a depressed left ventricular function
treated at the time of operation. or left ventricular dilatation profit the most in terms
Magnetic resonance imaging is becoming more of long-term survival in comparison to patients
and more popular for the evaluation of patients operated after symptom development (Bonow et al.
with aortic valve disease; however, for the time 2006; Tornos et al. 2006). It is important to mention
being, it cannot replace echocardiography or left that patients with severe aortic regurgitation and
heart catheterization for valve disease severity grad- depressed left ventricular function also profit from
ing and lead on its own to indication for surgery. surgical intervention (Bhudia et al. 2007).
v entricular distention. Care should be taken when cle through the aortic valve can also provide a
cannulating the left coronary ostium as in some bloodless operating field. Some surgeons prefer
patients with short left main stem this can lead to the flooding of the operative field with CO2 (2–4 l/
24 selective cannulation of either the left anterior min). As CO2 is heavier than air, it displaces air in
descending or the circumflex artery and result in the operative field and may lead to decreased inci-
insufficient myocardial protection. In cases where dence of air embolism (Martens et al. 2008).
significant calcification or stenosis of the coronary
ostia, retrograde cardioplegia and cooling of the
patient on bypass should provide adequate myocar- 24.4.2 Exposure of the Aortic Valve,
dial protection. Alternatively coronary artery bypass Suturing of the Aortotomy,
grafting should be performed before aortic valve and De-airing
replacement and cardioplegia can be given via the
vein grafts. The aortic valve can be exposed through a trans-
Placement of a left ventricular venting cathe- verse incision above the sinotubular junction or an
ter via the right superior pulmonary vein or the S-shaped or hockey-stick-shaped incision from
interatrial groove should be considered. the sinotubular junction down to the noncoronary
Alternatively, antegrade venting of the left ventri- sinus (. Fig. 24.3). In some patients with bicuspid
a c
..Fig. 24.3 a–c Valve exposure after S-shaped aortotomy a or after supracommissural transverse aortotomy b, c
Chapter 24 · Acquired Lesions of the Aortic Valve
767 24
a b
..Fig. 24.4 a, b Closure of the aortotomy after prosthetic valve implantation employing two single layer running
atraumatic sutures coming from each end of the incision a; noncoronary sinus enlargement with the use of a pericardial
patch. The proximal patch is sutured to the prosthetic valve ring b
a b
24
..Fig. 24.10 a–c Reconstruction of a regurgitant aortic valve by plication of the prolapsing left-coronary cusps in the
area of the Aranti nodule a. Shortening of the free cusp margin with the use of double-layered over-and-over running
b or first mattress and then over-and-over running suture c. In both cases the sutures are finally passed through the aor-
tic wall and tied on the outside
attempted (Duran et al. 1995) using glutaraldehyde- which is then used to suture the piece of pericar-
treated autologous pericardium or xenopericar- dium as leaflet substitute, using 5/0 running
dium, similar as used for biological valves monofilament suture (. Fig. 24.12). Midterm
(. Fig. 24.11). For this, we prefer monofilament or results of aortic leaflet extension in patients with
PTFE sutures (5/0 or 6/0 size). According to the rheumatic aortic valve disease have shown satis-
extent of the leaflet shortening or calcifications, in factory results (Bozbuga et al. 2004; Grinda et al.
certain cases a complete leaflet can be replaced 2002); however, changes of the xenologous or
with pericardium. The diseased leaflet is resected autologous tissue properties overtime can influ-
leaving only a small rim at the level of the anulus, ence the long-term results (Cohen et al. 2007).
Chapter 24 · Acquired Lesions of the Aortic Valve
771 24
Further reconstruction techniques can be
used for decalcification of the aortic leaflets or
closing of fenestrations or perforations as there
are after healed aortic valve endocarditis, using
materials as described above.
a b
..Fig. 24.12 a–c Restoration of valve competence in a valve with asymmetric leaflet retraction. a Isolated retraction
of the left coronary cusp, while both the other cusps are intact. b The retracted cusp is almost completely resected leav-
ing only a small rim to suture to at the anulus attachment. It is then replaced with a pericardial patch using running
suture technique from commissure to commissure c
772 M. Misfeld et al.
a b c
24
..Fig. 24.13 a–c Pathomechanisms responsible for central aortic regurgitation due to dilatation. a Normal aortic root.
b Dilatation at the anulus level. c Dilatation at the sinotubular junction. Both result in inadequate valve closing due to
lack of coaptation of the cusps
using subcommissural 4/0 monofilament U-sutures excised with a 2 mm rim of aortic wall around them
reinforced with autologous pericardium or Teflon («button»). After measuring the anulus diameter,
felt pledgets can restore normal dimensions. In stay sutures are placed at the upper end of the com-
patients with basal anulus dilatation, anuloplasty missures and put under tension so that the commis-
can reduce the basal anulus diameter. The aortic sures stay in anatomical position and the leaflet
root in the area of the noncoronary sinus is dis- coaptation is satisfactory. The diameter of the
sected, and monofilament pledgeted U-sutures are Dacron vascular prosthesis to be used is determined
passed from the inside of the aorta through the aor- using Hegar sizers. The prosthesis is then cut in
tic wall and tied over a short strip of Dacron pros- three tonguelike forms (also: «tulip shape»), so that
thesis, leading to a shortening of the basal anulus the «tongues» replace the excised sinuses
circumference in this segment, ultimately reducing (. Fig. 24.15). The prosthesis is sutured to the rim
the diameter, also (. Fig. 24.14). of the aortic root wall left over after excising the
Midterm results of partial reconstruction of sinuses using 4/0 monofilament suture. It is impor-
the aortic root are satisfactory (Carr and Savage tant to note that the sutures should pass through the
2004; Langer et al. 2004). In contrast to mitral anulus and the attachment of the leaflets. The mobi-
valve reconstruction, there is limited experience lized coronary ostia are then further mobilized, and
with partial aortic root reconstruction, and thus the proper site of implantation is determined. Using
these patients require periodical follow-up electrocautery, holes are burned into the prosthesis,
(Minakata et al. 2004; Charitos et al. 2009b). and the coronary ostia are anastomosed to the pros-
In cases of complex aortic root dilatation thesis using 5/0 monofilament suture. Caution
with macroscopically intact valve leaflets, should be taken so that the coronary ostia after
valve-sparing aortic root replacement can be per- implantation should remain without tension and
formed. These techniques replace the wall of the distortion, even after filling of the heart. The suture
aortic root with a Dacron prosthesis, and the lines for reimplantation of the coronary ostia can be
valve tissue is sutured to the individually pre- reinforced with strips of pericardium or Teflon felt
shaped tube prosthesis (Yacoub procedure or pledgets. Mid- as well as long-term results are excel-
«remodeling procedure») or reimplanted inside a lent (Erasmi et al. 2003, 2007; Yacoub et al. 1998;
tube or «Valsalva» graft (David procedure or Hanke et al. 2009).
«reimplantation technique»). In patients with connective tissue disorders
(Marfan syndrome), the Yacoub procedure should be
kYacoub procedure performed with caution, because the aortic anulus
The Yacoub procedure was first described in 1982 will not be stabilized and can further dilate over time.
(Fagan et al. 1982). The aortic root is mobilized, and For the same reasons, this procedure may not be
the aortic sinuses are excised leaving a 2 mm rim. appropriate in cases of acute type A aortic dissection
Likewise, the coronary ostia are mobilized and or patients with very dilated aortic anuli.
Chapter 24 · Acquired Lesions of the Aortic Valve
773 24
kDavid operation
The David procedure, initially published by David
et al. in 1992, replaces the aortic root while preserv-
ing the native aortic valve. It also stabilizes the aortic
anulus (David and Feidel 1992). The aortic root is
dissected in the same manner as in the Yacoub pro-
cedure. The sinus walls are excised leaving a 3–4 mm
aortic wall rim at the anulus. The coronary ostia are
mobilized and excised with a «button» of aortic wall.
After determining the right prosthesis size/diameter
(see Yacoub operation), U-sutures (monofilament
..Fig. 24.14 a, b Aortic valve repair by subcommissural
narrowing for isolated noncoronary anulus dilatation. a Tef-
3/0 sutures with or without felt reinforcement) are
lon felt-reinforced U-sutures are passed from the noncoro- passed from inside out through the aortic wall, close
nary sinus through the cusps underneath the commissures to the aortic anulus and the base of the aorta. If pled-
and through the subcommissural trigona to the neighbor- geted sutures are used, care should be taken that the
ing (right and/or left coronary) sinus and tied down. b Dur- pledgets are not in contact to the valve leaflets.
ing an aortic anuloplasty, U-sutures reinforced with
pledgets are passed inside out through the anulus and
Alternatively, three base sutures only can be used.
sutured over a shortening Dacron strip, so that the anulus The commissures are placed inside the prostheses,
is plicated and reinforced (insert) and the U-sutures are then passed through the pros-
thesis (. Fig. 24.16). The commissure stay sutures
774 M. Misfeld et al.
the left ventricle, under direct vision, is washed ning suture is employed starting in the anterior
out extensively with saline solution. mitral leaflet ending in the anulus, where the
Thereafter, the valve orifice area is mea- suture line is anchored using a second suture. If
sured, taking into consideration that the vari- the left atrium is opened, this should be closed
ous prosthesis sizers for the different valves are using monofilament, running 4/0 or 5/0 suture.
not identical in size with each other (Bartels The diameter of the aortic root is now measured
et al. 1998). Each prosthesis sizer compares to again, and the rest of the patch is integrated in
the given size of the specific commercial valve the closure line of the aortotomy (. Fig. 24.19).
product only (. Fig. 24.18).
24.5.2.3 Subvalvular Stenosis
24.5.2.2 nlargement of the Aortic
E In most patients with aortic valve stenosis, sig-
Anulus nificant left ventricular hypertrophy coexists. In
If after meticulous anulus decalcification, an some of these patients, hypertrophy of the bulbo-
adequate-sized prosthesis cannot be implanted, spiralis muscle in the left ventricular outflow
enlargement of the aortic anulus should be tract can lead to additional left ventricular out-
considered. An incision in the noncoronary
flow tract obstruction. To avoid a residual left
sinus or the commissure between the left and ventricular outflow tract obstruction after aortic
noncoronary sinus in the anterior mitral leaflet valve replacement, an enlargement of the subval-
can be used for this purpose (Manouguian and vular part is indicated, via a myectromy similar
Seybold- Epting techniques: Manougian and but limited compared to that described by
Seybold-Epting 1979). After incision in this Bigelow or Morrow (Bigelow et al. 1966; Morrow
area, the base of the aortic root can be enlarged 1978, . Fig. 24.20). For extensive discussion of
using a 2–3 cm wide tear-shaped patch either surgery for congenital types of subvalvular steno-
from native or glutaraldehyde-treated pericar- sis see 7 Chapter «Anomalies of the Left Ventricular
dium or from Dacron; A4/0 monofilament, run- Outflow Tract», Sect. 16.3.7.
776 M. Misfeld et al.
a b c
..Fig. 24.19 a–c Enlargement of a small aortic root through a posterior patch plasty. a With an incision through the
left/noncoronary commissure, the incision is extended into the anterior mitral valve leaflet. b A 2–3 cm wide, tear-shaped
patch (native or glutaraldehyde fixated autologous or xenopericardium or other prosthetic material) is sutured into the
incision. The suture should start at the deepest point of the incision, in the anterior mitral valve leaflet, and progress till
just below the aortic anulus (asterisks). c The valve is then implanted with standard technique and the rest of the patch is
integrated in the aortotomy closure
Chapter 24 · Acquired Lesions of the Aortic Valve
777 24
a b c
..Fig. 24.20 a–c Treatment of subvalvular muscular obstruction with a Bigelow myotomy a, b or a Morrow myectomy
c. After exposure and visualization of the hypertrophied muscle, the incision is performed into the muscle lying under-
neath the right coronary anulus part. For the Bigelow myotomy, now only compression into the incision is applied by
the surgeon’s finger. For the Morrow myectomy, a trapezoid piece, a block of muscle is excised, taking care to avoid
injury to the conductance system more anteriorly
risks for bleeding and thromboembolism. The and it should ensure to implant the prosthesis low
prostheses most often implanted nowadays are of into the plane of the aortic anulus. The orientation
the bileaflet type and in some centers also the of the bileaflet prosthesis should be at a 90° angle
tilting-disk type. Mechanical valves are primarily to a virtual line connecting the coronary ostia.
indicated for implantation in younger patients, Valve-specific terms «supra,» «epi,» or «intra»-
who may wish to avoid a reoperation. Long-term anular implantation can be misleading as on one
studies show a risk for thromboembolic complica- hand the so-called supra-anular implantation still
tions between 0.5 and 4.4 % per patient-year and a leaves the prosthesis sitting on the anulus. On the
risk for major bleeding between 0.4 and 2.3 % per other hand, both techniques are possible with
patient-year (Lund et al. 2000; Santini et al. 2002). both prostheses, mechanical or b iological. A his-
This risk depends on various factors like coexist- torical and real supra-anular aortic valve implan-
ing diseases (prior thromboembolism or bleeding tation had been suggested in the 1970s, when in
disorders), patient age, and target anticoagulation severe forms of aortic valve endocarditis with
range. Patient self-management of anticoagulation anular abscesses, the valve prostheses was
has shown to reduce bleeding and thromboem- implanted high in the ascending aorta, even distal
bolic complications (Aagard et al. 2003; Kvidal to the coronary ostia. The coronary ostia had to be
et al. 2000b; Murdy et al. 2003). suture closed, and coronary perfusion was facili-
Implantation techniques: For the implantation tated with saphenous vein grafts (Danielson et al.
of mechanical and stented biological prostheses, 1974; Reitz et al. 1981).
similar principles apply. Principally these pros- An alternative to the U-suture technique is the
theses can be implanted either in intra-anular running suture technique (usually three monofil-
position (using everted, aortoventricular, U-type, ament sutures size 2/0, one per sinus (. Figs. 24.22
size 3/0 or 2/0, pledget mattress sutures) passed and 24.23).
first through adequate amount of anular tissue It is important to note that mechanical pros-
and thereafter through the prostheses. For better thesis has a circular ring that is implanted in a
orientation, sutures of alternating colors can be crown-shaped anulus. The ring of biological
used. The prosthesis is held using a special valve valves has a similar crown shape that resembles
stabilizer and lowered into position after all that of a symmetrical aortic anulus.
sutures have been passed through the ring of the At the base of the interleaflet trigone between
valve prosthesis (. Fig. 24.21). Tying down the the right and the noncoronary sinus lies the con-
sutures should be performed in an alternating duction system (His bundle). Care should be
crossover fashion to avoid tilting the prosthesis, taken not to cause injury in this area.
778 M. Misfeld et al.
..Fig. 24.21 Implantation
of a mechanical prosthesis
using everting or the more
commonly noneverting
mattress sutures. The com-
missure sutures are marked
and placed under tension to
facilitate exposure. The pros-
thesis is mounted on a
holder and held by the assis-
tant. The mattress sutures
are passed through the anu-
lus and then at similar dis-
tances through the
prosthesis
Chapter 24 · Acquired Lesions of the Aortic Valve
779 24
Especially the self-expanding devices need careful
sizing after decalcification, and in general the aor-
totomy should be performed more distally than in
conventional aortic valve replacement procedures.
The first sutureless prosthetic valve was developed
in the 1960s by George J. Magovern in cooperation
with the engineer Harry W. Cromie (Magovern
and Cromie 1963). The major problems with these
prostheses at that time were the risk of valve
thrombosis, the need for oral anticoagulation, and
the risk of endocarditis. The Magovern-Cromie
prostheses were implanted in the aortic as well as
in the mitral position. Anecdotal reports of
patients in whom this prosthesis was implanted
have been recently published (Zlotnick et al. 2008).
Reduced ischemia and cardiopulmonary
bypass times are major advantages for implantation
of sutureless valve prosthesis. This may be of spe-
cial importance for complex procedures or in
patients with high-risk profiles. However, studies
comparing outcome of sutureless prosthesis with
conventional prostheses are still to be published.
Another advantage of the sutureless prostheses
..Fig. 24.22 An alternative technique to the everting or may be the increased effective valve area that these
non-everting mattress sutures is the single suture or the type of prostheses offer, as they do not require a
twisting suture technique suturing ring which allows for larger inner open-
ing diameters. Patients with heavily calcified aor-
tic roots may especially benefit from implantation
of a sutureless valve prosthesis.
The sutureless valves currently used, either in
the clinical or in experimental setting, are the
3F-Enable Valve (3F ATS Medical, Minneapolis,
MN, USA), the Perceval S (Sorin Biomedica,
Saluggia, Italy), and the Intuity valve (Edwards
Lifesciences, Irvine, CA, USA). The final judg-
ment and acceptance of the sutureless valves will
depend on the safety and simplicity of implanta-
tion, as well as on the durability in comparison to
conventional biological valves.
a b c
24
..Fig. 24.24 a–c Techniques for the implantation of stentless bioprostheses. a Subcoronary. b Root replacement. c
The root inclusion technique
a d
24
..Fig. 24.25 a–e Aortic valve replacement with a homograft. a The sinuses of the homograft are excised. b The valve
is inverted. c The homograft is inverted into the left ventricular outflow tract, and the homograft basis is sutured to the
native anulus using an atraumatic suture. d Thereafter, the homograft is everted again, and the commissures are pulled
up into the aorta and sutured to the aortic wall using pericardial or Teflon pledgeted sutures. e The rim of the homograft
sinuses is sutured to the aortic wall using running suture in between commissures
24.5.5.1 Aortic Valve Replacement replacement at the time of primary coronary bypass
and Coronary Artery Bypass grafting should be performed also in the setting of
Grafting mild or moderate aortic valve disease (Rosenhek
Isolated coronary artery bypass grafting as well as et al. 2004; Smith et al. 2004). Intraoperative aortic
isolated aortic valve replacement are routine cardiac valve inspection may help to determine the degree
surgical interventions. The indications for combined of valve degeneration and facilitate the decision.
aortic valve replacement and CABG procedure are During combined CABG and aortic valve
the same as for isolated aortic valve replacement. As replacement, on extracorporeal circulation, the aor-
a mild aortic valve disease, however, can quickly tic valve is excised to start with, in order to allow for
progress to a severe form, c oncomitant aortic valve manipulation of the heart during CABG without the
Chapter 24 · Acquired Lesions of the Aortic Valve
783 24
risk of dislocation of calcium plaques. Thereafter, Long-term results are excellent (Hagl et al. 2003).
the distal coronary bypass anastomoses are per- Alternatively, a combination of a Dacron prosthe-
formed. This is followed by replacement of the aortic ses and a stentless biological valve can be used
valve and closure of the aortotomy. Finally, the grafts (Urbanski et al. 2003). If the aortic root is not
are anastomosed proximally. With the distal anasto- diseased, then a conventional aortic valve and
moses in place, it is possible to perfuse coronary ascending aorta replacement can be performed
arteries with cardioplegic solution distal to their which significantly simplifies the operative proce-
obstruction until the end of the ischemic period. dure (Houel et al. 2002; Sioris et al. 2004). In cases
of ascending aortic ectasia or mild dilatation, an
24.5.5.2 Aortic Valve Replacement aortoplasty can be performed using mattress
in Multiple Valve Disease sutures followed by running 3/0 monofilament
If more than one valve requires surgical attention, sutures over two Teflon strips for reinforcement
the calcified aortic valve is excised at first. (Bail et al. 2007; Robicsek et al. 2004; Walker et al.
Thereafter, interventions on the other valves are 2007). In cases of aortic root dilatation with a
performed in the following sequence: macroscopically normal aortic valve, a valve-
55 Mitral valve sparing David or Yacoub procedure is indicated.
55 Aortic valve
55 Tricuspid valve 24.5.5.4 Aortic Valve Replacement
55 Pulmonary valve in Advanced Age
Calcified aortic valve stenosis is a disease charac-
This sequence prevents embolization of calci- teristically seen with advanced age, senile aortic
fied aortic valve and/or wall plaques. It also allows stenosis. The aging of the overall population has
for tricuspid and pulmonary valve surgery to be led to an increase in the mean age of patients
performed after closure of the aortotomy and referred for aortic valve replacement. Although
while the heart is already being reperfused. the natural history for severe symptomatic aortic
Because of the anatomic proximity of the aortic valve stenosis carries a significant mortality and
and mitral valve anulus (. Fig. 24.1), mitral valve morbidity burden, almost a third of the elderly
replacement or mitral ring anuloplasty can lead to patients than could potentially benefit from sur-
reduction of the aortic valve diameter. Therefore, gery are not referred for surgical evaluation. The
mitral valve surgery should be performed before main reasons for this seem to be increased age
the aortic valve is replaced. The aortic valve anulus and reduced left ventricular function (Iung et al.
should be sized only after the mitral valve has been 2005). However, many studies have shown that
taken care of (replacement/reconstruction). although aortic valve replacement in the elderly
The techniques for mitral, tricuspid, or pul- carries a somewhat increased operative risk as
monary valve replacement during concomitant compared to younger patients, these patients do
aortic valve replacement are described in the cor- benefit from aortic valve replacement, also
responding sections of this book. (Carabello 2004; Charlson et al. 2006;
Chukwuemeka et al. 2006a; Langanay et al. 2006;
24.5.5.3 Concomitant Replacement Sundt et al. 2000; Varadarajan et al. 2006b). As
of Aortic Valve the perioperative risk of such procedures is
and Ascending Aorta increased in an emergency setting, especially in
During concomitant surgery on aortic valve and this patient population, a planned referral fort
ascending aorta, the decision should be made timely surgery should be sought (Kohl et al. 2007;
whether the aortic valve can be repaired or not Mistiaen et al. 2004). The increased perioperative
and whether only the tubular part of the ascending risk in this patient population should be commu-
aorta or the complete aortic root should be nicated with the patient during the informed
replaced, also. The classic Bentall procedure consent process, as often many patients decline
(Bentall and De Bono 1968) with reimplantation the procedure. High-risk patients should be
of the coronary arteries for the complete replace- offered a minimal invasive alternative (transfem-
ment of the aortic root and the ascending aorta oral or transapical aortic valve replacement; see
can be performed either with a composite 7 Sect. 24.8 «Alternative Therapeutic Interventions»
mechanical valve-graft conduit or in combination and Chapter «Minimally Invasive Cardiac Surgery»,
with a stented biological valve («bio Bentall»). 7 Sect. 26.3.4).
784 M. Misfeld et al.
24.5.5.5 Aortic Valve Replacement biotic prophylaxis may be used in these patients
and Dialysis (Elkayam and Bitar 2005; Hung and Rahimtoola
Patients with terminal renal insufficiency are 2007; Reimold and Rutherford 2003).
24 prone to develop calcified aortic valve stenosis. A detailed information for the pregnant
Because of the reduced life expectancy of these patient or the patient in childbearing age about
patients and the need for regular vascular access the risk during and after pregnancy as well as
for dialysis, the American Heart Association and during delivery and a close collaboration of
American College of Cardiology guidelines rec- cardiologists, cardiac surgeons, and gynecologists
ommend the implantation of biological valves in is of great importance.
these patients (ACC/AHA Guidelines, Bonow
et al. 2006). However, no significant survival dif- 24.5.5.7 he «Small» Aortic Valve
T
ference could be shown between biological or Anulus
mechanical valves in patients with terminal renal The term «prosthesis-patient mismatch» (PPM) was
insufficiency requiring dialysis (Herzog et al. first introduced by Rahimtoola in 1978 (Rahimtoola
2002). For these reasons, the decision between 1978) and describes the situation when the effective
mechanical or biological aortic valve replace- valve area of the prosthesis is smaller than that of the
ments should take into consideration the patient’s normal aortic valve for the given body size. The first
age and life expectancy, the option of renal trans- who examined relevance and implications of PPM
plantation, as well as the patient’s preferences. on the patient’s functional status after aortic valve
replacement was Pibarot (Pibarot et al. 1996).
24.5.5.6 Aortic Valve Replacement . Table 24.2 presents the grading criteria of PPM as
and Pregnancy described by Blais et al. (2003).
Mild or moderate aortic valve stenosis (aortic Although studies have shown that the pres-
valve area >1.0 cm2) rarely causes complications ence of PPM can have a negative effect on the
during pregnancy (Hameed et al. 2001). Severe early postoperative course (Kulik et al. 2006;
aortic valve stenosis can lead to increased mater- Tasca et al. 2006), this can be masked by many
nal morbidity and fetal mortality. Maternal mor- other factors. In special cases, the use of a smaller
tality is rare (Silversides et al. 2003). Usually these prosthesis may be justified. This is true for older
patients will require aortic valve replacement patients with small aortic roots (Arata et al. 2002;
during the first months after delivery. When Bortolotti et al. 2000). Important factors to facili-
patients with severe aortic valve stenosis develop tate the decision for the size of prosthesis are not
symptoms during pregnancy, this may lead to only the left ventricular functional status but also
interruption of pregnancy. Therefore, a balloon the patient’s daily level of activity. Small prosthe-
dilatation prior to surgical intervention may be ses should be avoided in tall or active patients, in
necessary. Important factors for the prognosis of order to decrease the early postoperative risk, as
the disease during pregnancy are left ventricular well as to facilitate late recovery of the left ven-
function, grade of the aortic valve stenosis, and tricular function (David 2005). In these patients,
history of previous left ventricular decompensa- aortic root enlargement procedures may allow for
tion or thrombolic complications. Aortic valve the implantation of a larger prosthesis without a
insufficiency is usually good tolerated during significant increase in operative risk (Peterson
pregnancy. et al. 2007).
Pregnancy does not cause acceleration of bio-
logical valve degeneration. Mechanical prostheses
however carry a certain thromboembolic risk as ..Table 24.2 Defining the severity of
«prosthesis-patient mismatch» by valve area
well as a risk for warfarin embryopathy. For these
indexed to body surface area (Blais et al. 2003)
reasons in patients wishing to become pregnant,
biological valves may be an alternative. Patients Definition Effective aortic valve area (cm2/m2)
with mechanical prostheses who become preg-
nant should be switched to unfractioned heparin Severe PPM <0.65
during the first trimester and after the 36th week Moderate PPM 0.65–0.85
of pregnancy in order to reduce the risk for warfa-
No PPM >0.85
rin embryopathy or bleeding. Perioperative anti-
Chapter 24 · Acquired Lesions of the Aortic Valve
785 24
24.6 I ntra- and Postoperative may also require increased filling pressures to main-
Course tain adequate cardiac output, and thus invasive
hemodynamic monitoring with a Swan-Ganz or left
24.6.1 Patient Monitoring atrial pressure catheter is of benefit and can aid post-
operative decision-making. Patients after aortic valve
Intraoperative monitoring of patients undergoing replacement may develop temporary atrial or ven-
aortic valve replacement is not different to other tricular arrhythmia, and thus, temporary pacemaker
routine cardiac surgery procedures. Apart from electrodes should not be prematurely removed.
the central venous access as well as invasive All patients after aortic valve interventions
arterial blood pressure monitoring, transesopha- should receive endocarditis prophylaxis as well as
geal echocardiography (TEE) plays nowadays a adequate anticoagulation as recommended in the
central role. TEE can evaluate the prosthesis or guidelines of the American Heart Association
native valve function, the anatomy of the aortic (Bonow et al. 2006).
root, the coronary ostia, ventricular function
when weaning from extracorporeal circulation, as 24.6.2.1 Anticoagulation
well as evaluate the de-airing of the heart. Patients with mechanical valves must receive life-
Especially during aortic valve reconstruction, long oral anticoagulation, whereas patients with
TEE can evaluate and document the postopera- biological valves may require anticoagulation only
tive result. for the first months after the procedure, if at all. The
degree of anticoagulation requirement can vary
from patient to patient according to the type and
24.6.2 Postoperative Management model of the implanted prosthesis as well as depend
on other comorbidities. . Table 24.3 presents an
The early postoperative management of patients after overview of the anticowagulation recommendations
aortic valve interventions does not deviate signifi- in patients after mechanical and biological aortic
cantly from that of patients undergoing other routine valve replacement (Bonow et al. 2006).
cardiac surgery procedures. The management of In patients already receiving warfarin, the
patients however with aortic valve stenosis and sig- addition of acetylsalicylic acid (ASA) is recom-
nificant left ventricular hypertrophy can be challeng- mended. Risk factors requiring a higher antico-
ing. During hypertensive phases, the risk of bleeding agulation target range are:
in these patients is increased as well as the myocar- 55 Atrial fibrillation
dium oxygen needs. For these reasons, strict blood 55 Reduced left ventricular function
pressure monitoring in these patients is advised. Due 55 History of thromboembolic event
to decreased myocardial compliance, these patients 55 Hypercoagulopathy
..Table 24.3 Recommendations of The American College of Cardiology and The American Heart Association
for anticoagulation of patients with aortic valve prostheses (Bonow et al. 2006)
Mechanical prostheses
High risk x x
Biological prostheses
High risk x x
786 M. Misfeld et al.
lead to in hospital mortality of up to 30 % (Moon valve fixation can occur due to incomplete valve
et al. 2001). In combination with prolonged anti- and anulus decalcification. The indications for
biotic therapy and healed focus, aortic valve reoperation for paravalvular leak depend on the
replacement is most often the therapy of choice. patients’ symptoms, the presence or absence of
In about half of these patients, the aortic root hemolysis, as well as the hemodynamic conse-
can be involved, also. In order to prevent rein- quences of the leak. In cases of a localized partial
fection, during reoperation of patients with detachment, this can be repaired with additional
prosthetic valve endocarditis, the use of pros- pledget or pericardium-reinforced, size 3/0
thetic materials should be limited as far as pos- Seralene or Mersilene U-sutures (. Fig. 24.26). In
sible. For this reason, the use of homografts can cases of multiple or extended detachment of the
be recommended, especially in prosthetic endo- prosthetic valve, a new valve replacement is rec-
carditis with anuli abscesses (Lytle et al. 2002; ommended.
Sabik et al. 2002). However, it remains unclear
which is the prosthesis of choice in these cases,
as the decision of the replacement prosthesis 24.7 Reoperations
depends on the complexity of the intervention
and the additional interventions that may be Aortic valve reoperations become more and
required to remove all infected tissues from the more part of the daily routine in cardiac surgery.
aortic root (Hagl et al. 2002; Leyh et al. 2004; This in most case is due to the degeneration of
Moon et al. 2001). biological prostheses, but there are also compli-
cations of mechanical and biological prostheses.
kParavalvular leak Although elective reoperations require some
A partial valve detachment with the consequence special surgical approaches, they are not associ-
of a paravalvular leak presents with an incidence ated with a significantly higher morbidity and
of about 1–2 %. In most cases an acute or cured mortality in experienced centers as long as they
endocarditis can lead to valve detachment and are limited to isolated aortic valve re-replace-
paravalvular leak; however, inadequate valve fixa- ments (Davierwala et al. 2006; Potter et al. 2005;
tion during the primary operation can also result Vigt et al. 2000).
to paravalvular leak development. Paravalvular Indications. Indications for aortic valve reop-
leaks are preferential sites for the development of erations follow the general guidelines and criteria
prosthetic endocarditis. Most often, inadequate for aortic valve surgery and depend on the
788 M. Misfeld et al.
presence of symptoms, other comorbidities, vention is absolutely indicated. In patients with sep-
hemodynamic parameters, as well as upon the tic cerebral embolization, the time point of surgical
need for other, concomitant, cardiac procedures. intervention is of great importance as the risk for
24 recurrent embolization should be weighed against
the risk of intracerebral bleeding during the proce-
24.8 Alternative Therapeutic dure due to the systemic heparinization required
Interventions for extracorporeal circulation. The emergency pro-
cedure in these cases should be performed either
The techniques for percutaneous aortic valve bal- within the first 24 h or after 4–6 weeks when the
loon valvuloplasty were developed in the 1980s as initial cerebral insult has stabilized. As cerebral
an alternative to conventional valve replacement, insult often lead to blood-brain barrier disorders, a
in order to offer symptomatic, high-risk, patients computed tomography may be performed prior to
with severe aortic valve stenosis a therapy option intervention in patients with cerebral insult.
(Cribier et al. 1986). Initial results showed a high Risk factors for aortic valve endocarditis are:
(~25 %) periprocedural complication rate, mor- 55 Bicuspid and degenerated calcified aortic
tality rates up to 8 %, and increased incidence of valves
early restenosis and rehospitalization (NHLBI 55 Past rheumatic fever
Balloon Valvuloplasty Registry Participants 55 Diabetes mellitus
1991). Since then however, with the use of refined 55 Congenital valve disease
techniques, results have significantly improved, 55 Prior endocarditis
and balloon valvuloplasty has now been shown to
improve early and midterm survival (Agarwal In about 8–20 % of all cases, dental procedures
et al. 2005). Studies have shown than combina- or other invasive interventions, respectively, were
tion of valvuloplasty and radiation may improve the main suspects of the initial bacteremia leading
restenosis rates. Nowadays however the interest to endocarditis.
in less invasive aortic valve interventions has Typical microorganisms causing endocarditis
shifted to minimal invasive aortic valve implanta- are (Bashore and Khandheria 2004):
tion via the transapical or transfemoral approach, 55 Staphylococcus aureus (32.4 %)
see 7 Chapter «Minimally Invasive Cardiac Surgery», 55 Streptococcus viridans (13.1 %)
Sect. 26.3.4. 55 Enterococcus faecalis (10.6 %)
a b
..Fig. 24.27 a Abscess of the noncoronary sinus penetrating toward the right atrium with destruction of the noncor-
onary cusp, also. b The aortic valve is excised, and the abscess cavity is closed with a pericardial patch
790 M. Misfeld et al.
a b c
24
..Fig. 24.28 a Of the aortomitral continuity. The attachment of the anterior mitral leaflet is completely detached
from the aortic wall, and the left atrium may be seen. b Reattachment of the base of the aortic root to the anterior mitral
leaflet using pericardium or pledget-reinforced U-sutures, only suggested in minor dehiscence. c To avoid tension on
the sutures, the defect, especially when large, should be closed with a pericardial patch
a b
..Fig. 24.29 a, b Subvalvular abscess extending into the muscular ventricular septum. a The wall of the abscess is
excised, and debridement of the abscess cavity is performed. b The cavity is closed with a pericardial patch using a run-
ning suture penetrating deep in the septal muscle
Deyerling W, Haverich A, Potel J, Hetzer R (1984) A suspen- Hagl C, Strauch JT, Spielvogel D et al. (2003) Is the Bentall
sion of fibrin glue and antibiotic for local treatment of procedure for ascending aorta or aortic valve replace-
mycotic aneurysms in endocarditis – an experimental ment the best approach for long-term event-free sur-
study. Thorac Cardiovasc Surg 32:369–372 vival? Ann Thorac Surg 76:698–703
24 Duebener LF, Stierle U, Erasmi A, German Ross Registry et al. Hameed A, Karaalp IS, Tummala PP et al. (2001) The effect of
(2005) Ross procedure and left ventricular mass regres- valvular disease on maternal and fetal outcome of preg-
sion. Circulation 112(SuppI):1415–1422 nancy. J Am Coll Cardiol 37:893–899
Duran CM, Gometza B, Kumar N, Gallo R, Bjorastad K (1995) Hanke T, Charitos EI, Stierle U et al. (2009) Factors associated
From aortic cusp extension to valve replacement with with the development of aortic valve regurgitation over
stentless pericardium. Ann Thorac Surg 60(2 time after two different techniques of valve-sparing aor-
Suppl):S428–S432 tic root surgery. J Thorac Cardiovasc Surg 137:314–319
Elkayam U, Bitar F (2005) Valvular heart disease and preg- Herzog CA, Ma JZ, Collins AJ (2002) Long-term survival of
nancy. Part I: native valves. J Am Coll Cardiol 46:223–230 dialysis patients in the United States with prosthetic
ElKhoury G, Vanoverschelde JL, Glineur D et al. (2006) Repair heart valves: should ACC/AHA practice guidelines on
of bicuspid aortic valves in patients with aortic regurgi- valve selection be modified? Circulation 105:1336–1341
tation. Circulation 114(SupplI):1610–1616 Houel R, Soustelle C, Kirsch M, Hillion ML, Renaut C, Loisance
Erasmi AW, Stierle U, Bechtel MJF, Schmidtke C, Sievers H-H, DY (2002) Long-term results of the Bentall operation
Kraatz E-G (2003) Up to 7 years’ experience with valve versus separate replacement of the ascending aorta
sparing aortic root remodeling/reimplantation for and aortic valve. J Heart Valve Dis 11:485–491
acute type A dissection. Ann Thorac Surg 76:99–104 Hung L, Rahimtoola SH (2007) Prosthetic heart valves and
Erasmi AW, Sievers H-H, Bechtel MJF, Hanke T, Stierle U, Mis- pregnancy. Circulation 107:1240–1246
feld M (2007) Remodeling or reimplantation for valve Iung B, Cachier A, Baron G et al. (2005) Decision-making in
sparing aortic root surgery? Ann Thorac Surg elderly patients with severe aortic stenosis: why are so
83(Suppl):S752–S756 many denied surgery? Eur Heart J 26:2714–2720
Erasmia A, Sievers H-H, Scharfschwerdt M, Eckel T, Misfeld Karck M, Siclari F, Wahlig H et al. (1990) Pretreatment of pros-
M (2005) In vitro hydrodynamics, cusp-bending defor- thetic valve sewing-ring with the antibiotic-fibrin seal-
mation, and root distensibility for different types of ant compound as a prophylactic tool against prosthetic
aortic valve-sparing operations: remodeling, sinus valve endocarditis. Eur J Cardiothorac Surg 4:142–146
prosthesis, and reimplantation. J Thorac Cardiovasc Kirklin JW, Barratt-Boyes BG (1986) Cardiac surgery. Wiley,
Surg 130:1044–1049 Chichester
Fagan A, Yacoub MH, Pillai R, Radley-Smith R (1982) Dacron Kolh P, Kerzmann A, Honore C, Comte L, Limet R (2007) Aor-
replacement of the ascending aorta and sinuses with tic valve surgery in octogenarians: predictive factors for
resuspension of the aortic valve and re-implantation of operative and long-term results. Eur J Cardiothorac
the coronary arteries: a new method for treatment of Surg 31:600–606
aneurismal or acute dissection of the aortic root. Pro- Kulik A, Burwash IG, Kapila V, Mesana TG, Ruel M (2006)
ceedings of the Joint International Cardiovascular and Long-term outcomes after valve replacement for low-
Thoracic Surgical Conference, Stockholm. Scand J Car- grading aortic stenosis: impact of prosthesis-patient
diovasc Surg 16:175 mismatch. Circulation 114(SuppI):1553–1558
Freeman RV, Otto CM (2005) Spectrum of calcified aortic Kvidal P, Bergström R, Hörte LG, Stahle E (2000a) Observed
valve disease. Pathogenesis, disease progression, and and relative survival after aortic valve replacement.
treatment strategies. Circulation 111:3316–3326 J Am Coll Cardiol 35:747–756
Grinda JM, Latremouille C, Berrebi AJ et al. (2002) Aortic Kvidal P, Bergström R, Malm T, Stahle E (2000b) Long-term
valve extension valvuloplasty for rheumatic aortic follow-up of morbidity and mortality after aortic valve
valve disease: midterm results. Ann Thorac Surg replacement with mechanical valve prosthesis. Eur
74:438–443 Heart J 21:1099–1111
Grube E, Schuler G, Bullesfeld L et al. (2007) Percutaneous aortic Langanay T, Verhove JP, Ocampo G et al. (2006) Current hos-
valve replacement for severe aortic stenosis in high-risk pital mortality of aortic valve replacement in octoge-
patients using the second- and current third-generation narians. J Heart Valve Dis 15:630–637
self-expanding CoreValve prosthesis: device success and Langer F, Aicher D, Kissinger A et al. (2004) Aortic valve
30-day clinical outcome. J Am Coll Cardiol 50:69–76 repair using a differentiated surgical strategy. Circula-
Grubitzsch H, Linneweber J, Kossagk C, Sanli E, Beholz S, tion 110:II67–II73
Konertz WF (2005) Aortic valve replacement with new- Leyh RG, Knobloch K, Hagl C et al. (2004) Replacement of the
generation stentless pericardial valves: short-term clini- aortic root for acute prosthetic valve endocarditis: pros-
cal and hemodynamic results. J Heart Valve Dis thetic composite versus aortic allograft root replace-
14:623–629 ment. J Thorac Cardiovasc Surg 127:1416–1420
Gummert JF, Funkat AK, Beckmann A et al. (2011) Cardiac Luciani GB, Casali G, Auriemma S, Santini F, Mazzucco A
surgery in Germany during 2010: a report on behalf of (2002) Survival after stentless and stented xenograft
the German Society for Thoracic and Cardiovascular aortic valve replacement: a concurrent, controlled trial.
Surgery. Thorac Cardiovasc Surg 59:259–267 Ann Thorac Surg 74:1443–1449
Hagl C, Galla JD, Lansman SL et al. (2002) Replacing the Lund O, Nielsen SL, Arildsen H, Ilkjaer LB, Pilegaard HK (2000)
ascending aorta and aortic valve for acute prosthetic Standard aortic St. Jude valve at 18 years: performance
valve endocarditis: is using prosthetic material contra- profile and determinants of outcome. Ann Thorac Surg
indicated? Ann Thorac Surg 74(Suppl):S1781–S1785 69:1459–1465
Chapter 24 · Acquired Lesions of the Aortic Valve
793 24
Lytle BW, Sabik JF, Blackstone EH, Svensson LG, Pettersson prosthetic valve hemodynamic performance and
GB, Cosgrove DM 3rd (2002) Reoperative cryopreserved patients clinical status. Can J Cardiol 12:379–387
root and ascending aorta replacement for acute aortic Potter DD, Sundt TM, Zehr KJ et al. (2005) Operative risk of
prosthetic valve endocarditis. Ann Thorac Surg reoperative aortic valve replacement. J Thorac Cardio-
74(Suppl):S1754–S1757 vasc Surg 129:94–103
Magovern GJ, Cromie HW (1963) Sutureless prosthetic heart Probst V, Le Scouamec S, Legendre A et al. (2006) Familial
valves. J Thorac Cardiovasc Surg 46(46):726–736 aggregation of calcific aortic valve stenosis in the west-
Manouguian S, Seybold-Epting W (1979) Patch enlargement ern part of France. Circulation 113:856–860
of the aortic valve ring by extending the aortic incision Quere JP, Monin JL, Levy F et al. (2006) Influence of preop-
to the anterior mitral leaflet. J Thorac Cardiovasc Surg erative left ventricular contractile reserve on postopera-
78:402–412 tive ejection fraction in low-gradient aortic stenosis.
Manouguian S, Abu-Aishah N, Neuzel J (1979) Patch enlarge- Circulation 113:1738–1744
ment of the aortic and mitral valve rings with aortic and Rahimtoola S (1978) The problem of valve prosthesis-
mitral double valve replacement. J Thorac Cardiovasc patient mismatch. Circulation 58:20–24
Surg 78:394–401 Rahimtoola SH (1998) Indications for surgery in aortic valve
Martens S, Neumann K, Sodemann C, Deschka H, Wimmer- disease. In: Yusuf S, Cairns JA, Camm AJ, Fallen EL, Gersh
Greinecker G, Moritz A (2008) Carbon dioxide field BJ (eds) Evidence based cardiology. BMJ Publishing,
flooding reduces neurologic impairment after open London, pp 811–832
heart surgery. Ann Thorac Surg 85:543–547 Rahimtoola SH, Frye RL (2000) Valvular heart disease. Circu-
McElhinney DB, Petrossian E, Tworetzky W, Silverman NH, lation 102:IV-24–IV-33
Hanley FL (2000) Issues and outcomes in the manage- Rankin JS, Hammill BG, Ferguson TB et al. (2006) Determi-
ment of supravalvular aortic stenosis. Ann Thorac Surg nants of operative mortality in valvular heart surgery.
69:562–567 J Thorac Cardiovasc Surg 131:547–557
Minakata K, Schaff HV, Zehr KJ et al. (2004) Is repair of aortic Reader GG, Romeo BJ, Webster B et al. (1947) The prognosis
valve regurgitation a safe alternative to valve replace- of syphilitic aortic insufficiency. Ann Intern Med
ment? J Thorac Cardiovasc Surg 127:645–653 27:584–595
Mistiaen W, Van Cauwelaert P, Muylaert P, Wuyts F, Harrisson Reimold SC, Rutherford JD (2003) Valvular heart disease in
F, Bortier H (2004) Risk factors and survival after aortic pregnancy. N Engl J Med 349:52–59
valve replacement in octogenarians. J Heart Valve Dis Reitz BA, Stinson EB, Watson DC et al. (1981) Translocation of
13:538–544 the aortic valve for prosthetic valve endocarditis. J Tho-
Moon MR, Miller DC, Moore KA et al. (2001) Treatment of endo- rac Cardiovasc Surg 81:212–218
carditis with valve replacement: the question of tissue ver- Roberts WC, Ko JM (2005) Frequency by decades of unicus-
sus mechanical prosthesis. Ann Thorac Surg 71:1164–1171 pid, bicuspid, and tricuspid aortic valves in adults hav-
Morrow AG (1978) Hypertrophic subaortic stenosis: opera- ing isolated aortic valve replacement for aortic stenosis,
tive methods utilized to relieve left ventricular out- with or without associated aortic regurgitation. Circula-
flow tract obstruction. J Thorac Cardiovasc Surg tion 111:920–925
76:423–430 Robicsek F, Cook JW, Reames MK, Skipper ER (2004) Size
Murday AJ, Hoschtitzky A, Mansfield J et al. (2003) A prospec- reduction ascending aortoplasty: is it dead or alive?
tive controlled trial of St. Jude versus Starr Edwards aortic J Thorac Cardiovasc Surg 128:562–570
and mitral valve prostheses. Ann Thorac Surg 76:66–73 Rosenhek R, Klaar U, Schemper M et al. (2004) Mild and
NHLBI Balloon Valvuloplasty Registry Participants (1991) Per- moderate aortic stenosis. Natural history and risk strati-
cutaneous balloon aortic valvuloplasty. Acute and fication by echocardiography. Eur Heart J 25:199–205
30-day follow-up results in 674 patients from the NHLBI Ross DN (1962) Homograft replacement of the aortic valve.
Balloon Valvuloplasty Registry. Circulation 84:2383–2397 Lancet 2:487
Pai RG, Varadarajan P, Kapoor N, Bansal RC (2007) Aortic Ross DN (1967) Replacement of the aortic and mitral valves
valve replacement improves survival in severe aortic with a pulmonary autograft. Lancet 2:956–958
stenosis associated with severe pulmonary hyperten- Sabik JF, Lytle BW, Blackstone EH, Marullo AG, Pettersson GB,
sion. Ann Thorac Surg 84:80–85 Cosgrove DM (2002) Aortic root replacement with cryo-
Pasquali SK, Shera D, Wernovsky G et al. (2007) Midterm out- preserved allograft for prosthetic valve endocarditis.
comes and predictors of reintervention after the Ross Ann Thorac Surg 74:650–659
procedure in infants, children, and young adults. J Tho- Santini F, Casali G, Viscardi F et al. (2002) The CarboMedics
rac Cardiovasc Surg 133:893–899 prosthetic heart valve: experience with 1,084 implants.
Pate GE, Chandavimal M, Naiman SC, Webb JG (2004) Hey- J Heart Valve Dis 11:121–126
de’s syndrome: a review. J Heart Valve Dis 13:701–712 Schmidtke C, Bechtel M, Hueppe M, Sievers HH (2001) Time
Pellikka PA, Sarano ME, Nishimura RA et al. (2005) Outcome course of aortic valve function and root dimensions
of 622 adults with asymptomatic, hemodynamically after subcoronary Ross procedure for bicuspid versus
significant aortic stenosis during prolonged follow-up. tricuspid aortic valve disease. Circulation
Circulation 111:3290–3295 104(Suppl):I21–I24
Peterson MD, Borger MA, Feindel CM, David TE (2007) Aortic Scognamiglio R, Negut C, Palisi M, Fasoli G, Dalla-Volta S
anular enlargement during aortic valve replacement: (2005) Long-term survival and functional results after
improving results with time. Ann Thorac Surg 83:2044–2049 aortic valve replacement in asymptomatic patients with
Pibarot P, Honos GN, Durand LG, Dumesnil JG (1996) The chronic severe aortic regurgitation and left ventricular
effect of prosthesis-patient mismatch on aortic bio- dysfunction. J Am Coll Cardiol 45:1025–1030
794 M. Misfeld et al.
Sievers HH (2002) Status of aortic valve reconstruction Taylor WJ, Thrower WB, Black H, Harken DE (1958) The surgi-
and Ross operation in aortic valve disease. Herz cal correction of aortic insufficiency by circumclusion.
27:435–444 J Thoracic Surg 35:192–205
Sievers HH, Schmidtke C (2007) A classification system for Tleyjeh IM, Abdel-Latif A, Rahbi H et al. (2007) A systematic
24 the bicuspid aortic valve from 304 surgical specimens. review of population-based studies of infective endo-
J Thorac Cardiovasc Surg 133:1226–1233 carditis. Chest 132:1025–1035
Sievers HH, Hanke T, Stierle U et al. (2006) A critical reappraisal of Tornos P, Sambola A, Permenyer-Miralda G, Evangelista A,
the Ross operation: renaissance of the subcoronary implan- Gomez Z, Soler-Soler J (2006) Long-term outcome of
tation technique? Circulation 114(SuppI):1504–1511 surgically treated aortic regurgitation: influence of
Sievers HH, Stierle U, Charitos EI et al. (2010a) Fourteen guideline adherence towards early surgery. J Am Coll
years’ experience with 501 subcoronary Ross proce- Cardiol 47:1012–1017
dures: surgical details and results. J Thorac Cardiovasc Urbanski PP, Diegeler A, Siebel A, Zacher M, Hacker RW
Surg 140:816–822, 822.e1-5 (2003) Valve stentless composite graft: clinical out-
Sievers HH, Stierle U, Charitos EI, German-Dutch Ross Registry comes and hemodynamic characteristics. Ann Thorac
et al. (2010b) Major adverse cardiac and cerebrovascular Surg 75:467–471
events after the Ross procedure: a report from the German- Varadarajan P, Kapoor N, Bansal RC, Pai RG (2006b) Survival
Dutch Ross Registry. Circulation 122(11 Suppl):S216–S223 in elderly patients with severe aortic stenosis is dramat-
Sievers HH, Hemmer W, Beyersdorf F, Working Group for ically improved by aortic valve replacement: results
Aortic Valve Surgery of German Society of Thoracic and from a cohort of 27 patients aged > or = 80 years. Eur
Cardiovascular Surgery et al. (2012) The everyday used J Cardiothorac Surg 30:722–727
nomenclature of the aortic root components: the tower Vogt PJ, Brunner-LaRocca H, Sidler P et al. (2000) Reopera-
of Babel? Eur J Cardiothorac Surg 41:478–482 tive surgery for degenerated aortic bioprostheses: pre-
Silversides CK, Coleman JM, Sermaer M, Farine D, Siu S dictors for emergency surgery and reoperative
(2003) Early and intermediate-term outcomes of preg- mortality. Eur J Cardiothorac Surg 17:134–139
nancy with congenital aortic stenosis. Am J Cardiol Vrandecic M, Fantini FA, Filho BG, de Oliveira OC, da Costa
91:1386–1389 Junior IM, Vrandecic E (2000) Retrospective clinical
Sioris T, David TE, Ivanov J, Armstrong S, Feindel CM (2004) analysis of stented vs. stentless porcine aortic biopros-
Clinical outcomes after separate and composite thesis. Eur J Cardiothorac Surg 18:46–53
replacement of the aortic valve and ascending aorta. Vrandecic M, Fantini FA, Filho BG, de Oliveira OC, da Costa
J Thorac Cardiovasc Surg 128:260–265 Junior IM, Vrandecic E (2002) Long-term results with the
Smith WT, Ferguson TB, Ryan T, Landolfo CK, Peterson ED (2004) Biocor-SJM stentless porcine aortic bioprosthesis.
Should coronary artery bypass graft surgery patients with J Heart Valve Dis 11:47–53
mild or moderate aortic stenosis undergo concomitant Walker T, Bail DH, Gruler M, Vonthein R, Steger V, Ziemer G
aortic valve replacement? A decision analysis approach to (2007) Unsupported reduction ascending aortoplasty:
the surgical dilemma. J Am Coll Cardiol 44:1241–1247 fate of diameter and of Windkessel function. Ann Tho-
Stamm C, Friehs I, Ho SY, Moran AM, Jonas RA, del Nido PJ rac Surg 83:1047–1053
(2001) Congenital supravalvar aortic stenosis: a simple Wilson W, Taubert KA, Gewitz M et al. (2007) Prevention of
lesion? Eur J Cardiothorac Surg 19:195–202 infective endocarditis. Guidelines from the American
Sundt TM, Bailey MS, Moon MR et al. (2000) Quality of life Heart Association. A Guideline from the American heart
after aortic valve replacement at the age of >80 years. Association Rheumatic Fever, Endocarditis, and Kawa-
Circulation 102(Suppl III):III70–III74 saki Disease Committee, Council on Cardiovascular Dis-
Takkenberg JJ, Eijkemans MJ, van Herwerden LA et al. (2003) ease in the Young, and the Council on Clinical
Prognosis after aortic root replacement with cryopre- Cardiology, Council on Cardiovascular Surgery and
served allografts in adults. Ann Thorac Surg 75:1482–1489 Anesthesia, and the Quality of Care and Outcome
Talwar S, Mohapatra R, Saxena A, Singh R, Kumar AS (2005) Research Interdisciplinary Working Group. Circulation
Aortic homograft: a suitable substitute for aortic valve 115:1–19
replacement. Ann Thorac Surg 80:832–838 Yacoub MH, Gehle P, Chandrasekaran V, Birks EJ, Child A,
Tarantini G, Buja P, Scognamiglio R et al. (2003) Aortic valve Radley-Smith R (1998) Late results of a valve-
replacement in severe aortic stenosis with left ventricular preserving operation in patients with aneurysms of
dysfunction: determinants of cardiac mortality and ventric- the ascending aorta and root. J Thorac Cardiovasc
ular function recovery. Eur J Cardiothorac Surg 24:879–885 Surg 115:1080–1090
Tasca G, Mhagna Z, Perotti S et al. (2006) Impact of prosthesis- Zlotnick AY, Shiran A, Lewis BS, Aravot D (2008) Images in
patient mismatch on cardiac events and midterm mor- cardiovascular medicine. A perfectly functioning
tality after aortic valve replacement in patients with Magovern-Cromie sutureless prosthetic aortic valve 42
pure aortic stenosis. Circulation 113:570–576 years after implantation. Circulation 117:e1–e2
795 25
Surgery for Acquired
AV-Valve Diseases
Parwis B. Rahmanian and Thorsten C.W. Wahlers
References – 828
Chapter 25 · Surgery for Acquired AV-Valve Diseases
797 25
25.1 History of AV-Valve Surgery reveals today the real quality and durability of
repair techniques developed and applied in the
The first successful mitral valve procedure in his- early days of reconstructive valve surgery.
tory was performed at the Peter Bent Brigham However, meanwhile reoperative mitral valve sur-
Hospital in Boston, Massachusetts, in 1923, by gery, accessed commonly via a median sternotomy
Elliot C. Cutler who used a teratomy knife placed but also via right and – less common – left lateral
from the apex of the left ventricle to open a stenotic thoracotomy, has become a standardized and safe
mitral valve by commissurotomy (Cohn 1994). procedure in most cardiac tertiary care centers.
Two years later, in 1925, Souttar performed a The proportion of tricuspid valve surgery com-
mitral valvulotomy from the left atrium using his pared to mitral valve procedures remains small,
finger to fracture the commissures (Comas et al. and the decrease of rheumatic diseases in indus-
2006). The next step in the evolution of mitral valve trial countries makes particularly tricuspid valve
surgery was depending on the development of car- replacement a rare procedure today. However, tri-
diopulmonary bypass. Following Braunwald’s cuspid valve reconstruction, most commonly to
research studies at the National Institute of Health treat tricuspid regurgitation due to pulmonary
in the 1950s, Starr and Edwards were the first to hypertension in patients with long-lasting mitral
implant a mitral valve prosthesis at the University valve regurgitation becomes more important.
of Oregon in 1961. Despite a relatively high rate of Different techniques of tricuspid valve reconstruc-
thromboembolic complications, the Starr-Edwards tion are described at the end of this chapter.
caged-ball prosthesis presented the gold standard
for mitral valve replacement for many years (Gao
et al. 2004). The caged-ball prosthesis was replaced 25.2 Open Surgical Access
by tilting disc prostheses and later on by bileaflet
prostheses in the 1980s. Another important 25.2.1 edian Sternotomy in
M
development in heart valve surgery was the estab- Primary and Redo Surgery
lishment of biological heart valve prostheses. In
1970, Hancock implanted the first stented porcine Mitral valve surgery can be accessed by a median
valve, and only 6 years later, the first generation of sternotomy (. Fig. 25.1) or lateral thoracotomy from
Carpentier-Edwards bovine pericardial valves was the right and less common from the left side. A right
introduced (see also 7 Chapter «The History of anterior thoracotomy is used for minimal-invasive
Cardiac Surgery», Sect. 1.9). approaches and usually limited to the AV valves and
Following the establishment of heart valve pros- does not allow concomitant procedures such as
theses for the treatment of mitral valve disease, CABG or aortic valve procedures. Particularly reop-
reconstructive techniques such as open commissur- erative mitral valve surgery, which needs to be
otomy became less important. However, the nega- applied to an increasing proportion of patients, can
tive side effects of prosthetic valve replacement, i.e., be performed more safely via median sternotomy.
the need for lifelong anticoagulation and the risk of However, redo sternotomy carries the risk to injure
thromboembolic complications associated with retrosternal structures such as the right ventricle or
mechanical valves and the high rate of degeneration the right atrium, and therefore transection of the
of biological prostheses, led to a revival of recon- posterior proportion of the sternal bone and dissec-
structive techniques particularly within the last two tion of the pericardial tissue needs to be performed
decades. One of the pioneers of mitral valve recon- carefully to avoid severe bleeding complications. In
struction is Alain Carpentier who applied a system- redo cases, we strongly recommend a preoperative
atic and reproducible approach to reconstructive CT scan of the chest to measure the distance between
mitral valve surgery (Carpentier 1977). the sternum and the cardiac structures. Sternotomy
With an aging patient population and a is performed with an oscillating saw while the respi-
broader application of valve reconstruction or rator is disconnected in order to deflate the lungs and
replacement, the rate of patients referred for redo thus unload the right v entricle. Once the posterior
mitral valve surgery is constantly increasing. layer of the sternal bone is cut, the parietal pleura and
Consequent long-term follow-up of patients the pericardium are dissected. If the pericardium
undergoing mitral valve repair and improving was not closed at the initial procedure, careful dissec-
imaging quality of echocardiographic assessment tion between the parietal pleura and the right atrium/
798 P.B. Rahmanian and T.C.W. Wahlers
25
b
ventricle is necessary. It is useful to open both pleural
spaces right after sternotomy to avoid tension on
structures such as the upper cava vein, the anony-
mous vein, the right ventricle, and the right atrium
when the sternal retractor is placed. Once the upper
and lower caval veins are dissected circularly and the
ascending aorta is freed from adhesive tissue, cardio-
pulmonary bypass can be established in a standard
fashion using bicaval venous cannulation. We only
advocate cannulation of the femoral vessels for estab-
lishment of cardiopulmonary bypass before sternot-
omy in patients with severe obliteration of the
retrosternal space and particularly in patients where
the ascending aorta has broad contact to the poste-
rior sternal layer. Once cardiopulmonary bypass is
established, the heart should be completely freed ..Fig. 25.2 a, b Opening of the left atrium in the
from adhesions to allow a good exposure of the interatrial groove. Epicardial fibrofatty tissue within the
mitral valve, which sometimes requires rotation or interatrial groove is dissected a and preserved to facilitate
primary closure of the left atriotomy b
elevation of the heart. In addition, complete dissec-
tion allows better application of topic cooling and de-
airing of the heart before releasing the cross-clamp. exposition of the mitral valve. While cardioplegic
solution is applied in an antegrade or retrograde
fashion, the left atrium is opened with a knife leaving
25.2.2 Atriotomy an edge of 5 mm towards the septum. The incision is
then extended caudally towards the lower cava and
The most common access to the mitral valve is an inci- cranially into the roof of the left atrium carefully
sion of the left atrium in the area of the interatrial avoiding an opening of the right atrium. Using a spe-
groove (sulcus interatrialis, Sondergaard’s groove) cial retractor with two to three hooks placed at the
(. Fig. 25.2) (Sondergaard et al. 1955). This can be septal and caudal part of the incision usually allows
achieved by dissection beginning from the upper left for a good circumferential exposure of the mitral
pulmonary vein while the assistant is pulling the valve (. Fig. 25.3). However, sometimes a sponge or
right atrium towards a left and ventral direction. The gauze needs to be placed under the heart to lift up
epicardial fatty tissue in the interatrial groove can be the subvalvular apparatus. If the visibility of the
dissected with scissors or a high-frequency electro- valve is not satisfactory, holding sutures in the area
cautery allowing separation of the left and right of the commissures of the mitral valve may
atrium 3–6 cm in length. The higher the incision is improve the exposition. We routinely place a spiral
located towards the atrial septum, the better the wire-reinforced suction catheter in the left inferior
Chapter 25 · Surgery for Acquired AV-Valve Diseases
799 25
a
a b
25
..Fig. 25.5 a Trans-right atrial, transatrioseptal access to the left atrium. The dotted line represents the incision of the
interatrial septum after right atriotomy. b Biatrial, transatrioseptal incision (the Dubost approach). Care has to be taken
not to injure the atrioventricular junction, the tricuspid valve, the aortic root, or the His bundle
approach may be the better choice. First, the right of the septum. Marking the different flaps of atrial
atrium is opened with a hook-shaped incision fol- tissue with stay stitches may help correct anatomic
lowed by an incision of the interatrial septum in the repositioning before closing the atriotomy.
fossa ovalis in a length of 3–5 cm between the origin However, the biatrial approach is associated with
of the upper cava vein and the caudal portion of the an increased rate of atrial conduction disturbances
fossa ovalis. With this approach, a good exposition and should therefore, as recommended in the origi-
of both AV valves can be achieved without extensive nal publication by Deloche, only be used if the direct
rotation of the heart (. Fig. 25.5a). However, this approaches are not suitable (Deloche et al. 1990).
approach may have negative impact on the blood
supply of the sinus node and may contribute to tem-
porary or permanent sinus node dysfunction 25.2.6 Reoperation: Considerations
(Guiraudon et al. 1991). Both incisions are closed
with 3-0 Prolene sutures in an overcast fashion. The rate of patients referred for reoperative mitral
valve surgery is constantly increasing. In these
patients, our standard approach is a median sternot-
25.2.5 Dubost Approach omy. After dissection of the posterior portion of the
sternum, either with an oscillating saw or with heavy
The classic biatrial incision, as described by Dubost, scissors, the pericardium is carefully dissected from
allows a good exposition of both the mitral and tri- the chest wall. If the pericardium was not closed dur-
cuspid valves (Dubost et al. 1966). The incision starts ing the original procedure, then the right ventricle
at the right upper pulmonary vein and goes through and right atrium may need to be dissected from the
the atrial septum as well as the ventral right atrial wall chest wall. To start with, we regularly open the pleural
(. Fig. 25.5b). A horizontal incision into the inter- space to the right in order to avoid too much tension
atrial sulcus can be added to improve exposition on structures like SVC, innominate vein, right atrium,
(Brawley 1980; Deloche et al. 1990). However, there and right ventricle when placing the retractor. Then
needs to be caution if the incision is extended, par- the ascending aorta and the superior and inferior
ticularly in patients with small space between the pul- caval veins are dissected circumferentially in a step-
monary veins because uncontrolled tension by the by-step fashion. In case of severe adhesions between
retractor may enlarge the incision unintentionally pericardial tissue and the right atrium, a more lateral
into direction of the atrioventricular junction, the transpericardial entry is chosen, while the ventral
aortic root, or the His bundle (Deloche et al. 1990). adhesions are left in place. In our opinion, an attempt
Closure of the Dubost incision needs to be per- to completely release the heart from adhesions should
formed carefully to ensure correct reapproximation be made in every case to facilitate rotation of the heart
Chapter 25 · Surgery for Acquired AV-Valve Diseases
801 25
for better exposure of the mitral valve. This further- Following median sternotomy and resection of
more allows topical cooling of the heart during isch- the thymus tissue, the pericardium is opened in an
emia and facilitates better de-airing before releasing inverse t-shaped fashion. After systemic heparin
the cross-clamp at the end of the mitral procedure. application (400 IE/kg bodyweight) and placement of
An alternative approach for reoperative mitral valve two 4-0 polypropylene stay stitches, a cannula is
surgery is a right anterolateral thoracotomy with or placed in the distal ascending aorta and secured with
without transfemoral cannulation for the establish- a tourniquet. Regarding venous cannulation, it is
ment of extracorporeal circulation. important to consider that the best exposition of the
mitral valve is achieved by total cardiopulmonary
bypass with bicaval cannulation. First, a 90° venous
25.2.7 Left or Right Lateral Approach cannula is placed in the SVC and secured by tourni-
quet. Following initiation of partial extracorporeal
Under rare circumstances, a mitral procedure will circulation, another tourniquet suture is placed in the
be performed using a left lateral thoracotomy. In right atrium near the inferior cava (IVC) junction. A
these cases, the left atrium will be opened by plac- second 90° cannula is placed into the IVC and con-
ing a diagonal incision between the base of the left nected to the venous line using a y-connector. Using
atrial appendage and the insertion of the left pul- this bicaval cannulation technique allows for enlarge-
monary veins. However, venous cannulation ment of the atriotomy in any direction for better
remains an issue and is usually performed femo- exposure of the mitral valve if necessary. Furthermore,
ral ± jugular veins. Right and left lateral thora- pulling the tourniquet of the IVC cannula moves the
cotomy approaches for minimally invasive, heart towards the surgeon also facilitating better
endoscopic, and robotic mitral surgery are exposure. Finally, tourniquets are placed around both
described in more detail in 7 Chapter «Minimally caval veins to establish total cardiopulmonary bypass.
Invasive Cardiac Surgery», Sect. 26.3.5. Placement of a catheter into the coronary sinus
transatrially allows for the application of retro-
grade cardioplegia at every time point during the
25.3 Closure of Left Atrial operation without changing exposure. The alter-
Appendage native route of antegrade cardioplegia administra-
tion via the aortic root most likely requires removal
The left atrial appendage can be closed from inside of the mitral retractors to allow a competent clo-
the left atrium during mitral valve surgery using a sure of the aortic valve during administration of
double 3-0 polypropylene suture. Alternatively, a 4-0 cardioplegic solution. Usually, we administer 4 °C
teflon pledgeted suture can be placed from outside cold blood cardioplegia solution as described by
to directly close the appendage. Another approach Buckberg (initially 4 min followed by 2 min every
would be the resection of the appendage and direct 20–25 min of cardioplegic arrest). In addition, the
closure with a running suture. There are also devices patient’s body temperature is lowered to 32 °C, and
for epicardial exclusion of the left atrial appendage in most cases topic epicardial cooling is achieved
on the market. However, during mitral valve surgery by instillation of ice-cold saline solution.
direct closure under vision seems to be the most A particular focus has to be directed to the com-
valuable approach. It has to be kept in mind that ini- plete de-airing of the heart before opening of the
tially there is a risk for thromboembolism from the aortic cross-clamp following every mitral valve pro-
inner surface even if sinus rhythm is present. cedure because opening the left atrium results in air
distribution into every structure of the left heart
including the pulmonary veins. We feel two mea-
25.4 Extracorporeal Circulation, sures to be most important in order to minimize the
Cannulation Technique, risk of air embolism: insufflation of carbon dioxide
Myocardial Protection, into the pericardium and filling the left atrium with
De-airing saline before definite closure of the atriotomy.
Before releasing the aortic cross-clamp, a
Establishment of extracorporeal circulation gen- de-airing maneuver is routinely applied in coopera-
erally follows the same principles as of other tion with anesthesia and perfusion. A cannula is
cardiac surgery procedures. However, some
placed in the ascending aorta just below the
aspects need to be discussed in more detail. cross-
clamp to allow evacuation of trapped air.
802 P.B. Rahmanian and T.C.W. Wahlers
Alternatively, the entry site of an antegrade cardio- safely without leading to loss of coaptation. During
plegia cannula can be used. While moving the table the cardiac cycle, the motion of the anterior leaflet
in a head-up position pulmonary backflow into the also defines an important boundary between the
left atrium is increased by high-pressure ventilation inflow (during diastole) and outflow (during sys-
hold by anesthesia and temporary volume shift by tole) tracts of the left ventricle (David et al. 1997).
stopping venous drainage by perfusion. Careful The posterior leaflet of the mitral valve has a
25 manipulation of the ventricle and digital invagina- quadrangular shape and comprises 60 % of the
tion of the left atrial appendage (if not closed or anular circumference. Carpentier introduced a
resected) helps to release trapped air in the trabecu- surgical classification of the mitral leaflets by divid-
lar structures of the left heart. Aortic cross-clamp is ing them into three segments. In opposite to the
then removed in Trendelenburg position. However, anterior leaflet, the posterior leaflet typically has
manipulation, particularly elevation of the ventricle two well-defined indentations at its free margin,
should be done carefully because the rigid struc- which divide this leaflet into three scallops referred
tures of the valve prosthesis or anuloplasty rings to as P1 (anterior or lateral scallop), P2 (middle
may serve as a hypomochlion leading to rupture of scallop), and P3 (posterior or medial scallop). The
the atrioventricular plane, which is a life-threaten- three corresponding segments of the anterior leaf-
ing complication on its own (Rodriguez et al. 2001). let are accordingly described as A1 (anterior seg-
Weaning off cardiopulmonary bypass is initi- ment), A2 (middle segment), and A3 (posterior
ated once the patient is rewarmed. In patients segment) (Carpentier 1983). This nomenclature is
with impaired left ventricular function, we rou- particularly important for describing mitral valve
tinely place a left atrial pressure measurement pathology between echocardiographer and cardiac
catheter to control left ventricular preload. In surgeon as well as for planning the operative strat-
addition, all patients are under TEE surveillance, egy. In contrast to the anterior leaflet, the posterior
which allows control of mitral valve repair results leaflet comprises redundant tissue, particularly in
as well as left ventricular function during weaning the setting of prolapse due to excess tissue, and
of cardiopulmonary bypass. therefore tolerates more aggressive resection.
The commissures of the mitral valve define the
region where anterior and posterior leaflet come
25.5 Mitral Valve Surgery together at their origin at the anulus. However, this
area is not always well defined, and it is sometimes
25.5.1 Functional Anatomy necessary to use the corresponding papillary mus-
of the Mitral Valve cles as landmarks to identify the commissures.
The zone of coaptation plays another impor-
The mitral valve has a complex structure, and com- tant role for the function of the mitral valve which
petent function is only achieved by interaction of represents the area of contact between anterior
leaflets, commissures, chordae, papillary muscles, and posterior leaflets during systole. There are
and the left ventricle. Understanding these five ana- two zones on the atrial surface of the leaflet, the
tomical structures helps defining the pathophysio- peripheral smooth body zone and the central
logic basis of mitral valve disease and is crucial to rough zone. From the surgical view (atrial view),
achieve successful surgical repair (Adams et al. 2006). the two zones are separated by a gently curved
The mitral valve has two leaflets. The anterior coaptation line. The rough zone represents the
leaflet is of semilunar shape and comprises 40 % of coaptation surface of the valve and is crucial for
the anular circumference. There is continuity valve competency and the depth and length of
between the anterior leaflet of the mitral valve and coaptation is an important marker for the assess-
the left and noncoronary cusp of the aortic valve. ment of mitral valve function.
This area is referred to as the aortic-mitral curtain The mitral valve is an atrioventricular valve and
and is particularly fragile. The free edge of the ante- therefore separates the left atrial chamber from the
rior leaflet is slightly tented towards the cave of the left ventricle. The anulus of the mitral valve repre-
left ventricle by primary chordae and is usually con- sents the junction between the left atrium and the
tinuous without indentations. The lack of redun- left ventricle and also serves as insertion site for the
dant tissue at the edge of the anterior leaflet is of mitral leaflets. The mitral anulus is saddle shaped
importance in patients with anterior leaflet prolapse and changes its structure during the cardiac cycle.
as only little amount of the leaflet can be resected During systole, the commissures move towards the
Chapter 25 · Surgery for Acquired AV-Valve Diseases
803 25
apex of the ventricle while the anular circumfer- The complex structure of the mitral valve and
ence narrows. Both mechanisms support an opti- its connection to the left ventricle is of great impor-
mal coaptation and may be impaired by anular tance for left ventricular function. This fact is now-
dilatation or calcification. Furthermore, the mitral adays addressed by preservation of at least the
anulus is embedded into several important struc- posterior portion of the subvalvular apparatus dur-
tures, such as the coronary portion of the aortic ing mitral valve replacement either with mechani-
valve, the coronary sinus, the atrioventricular node, cal or biological prostheses, and long-term studies
and the circumflex artery, which need to be taken have shown an improvement of outcome com-
into consideration during mitral valve surgery. pared to complete resection of the subvalvular
Finally, the chordae and the two papillary mus- apparatus (Chen et al. 2003; Yilmaz et al. 2005).
cles need to be mentioned. The chordae are of
importance for the end-systolic position of both
leaflets. They have their origin at the papillary mus- 25.5.2 Mitral Valve Replacement
cles and are classified according to their insertion
sites at the leaflets. Primary chordae (marginal chor- The marked decrease of rheumatic mitral stenosis
dae) are attached to the free margin of the leaflet and in industrial countries, which represented the main
prevent prolapse of the margin during systole. indication for mitral surgery in the 1970s and
Secondary chordae (intermediate chordae) are 1980s, resulted in lower rates of mitral valve replace-
attached to the ventricular surface of the leaflets and ment compared to mitral valve repair. Today, the
relieve excess tension from the leaflet tissue. vast majority of patients undergoing mitral valve
Secondary chordae may also play a role in preserv- surgery suffer from mitral regurgitation due to
ing ventricular shape and function (Rodriguez et al. degenerative or ischemic disease, which is more
2004). Several studies, beginning with Lillehei, have likely reparable than mitral stenosis. Nevertheless,
shown that preservation of the posterior part of the mitral valve replacement is still a standard proce-
subvalvular apparatus improves ventricular func- dure in cardiac surgery and plays an important role
tion following mitral valve replacement (Lillehei in the armamentarium of every cardiac surgeon.
et al. 1964). Techniques of preservation of the sub- Since the first successful prosthetic mitral valve
valvular apparatus are described later in this chapter. replacement by Starr in 1961, many improvements
Tertiary chordae (basal chordae) are only found at were introduced into clinical practice, particularly
the posterior leaflet connecting the base of the leaflet regarding the type of prostheses. The burden of
and the mitral anulus to the papillary muscles. The thromboembolic complications following pros-
kinetics of chordae and papillary muscles with their thetic valve replacement is even more important in
connection to the left ventricle directly interlinks mitral position than after aortic valve replacement.
mitral valve function with ventricular function.
The papillary muscles connect the mitral valve 25.5.2.1 Choice of Prosthesis
and its chordae to the left ventricle. The two papil- During the recent decades, several prostheses for
lary muscles have their origin in the area between mitral valve replacement were introduced; how-
the middle and apical third of the left ventricular ever, only a few of them were further developed
wall. The anterior papillary muscle usually has one and are now established in clinical practice. The
head, whereas the posterior papillary muscle regu- ideal mitral valve prosthesis unites optimal hemo-
larly has two heads. Each papillary muscle serves as dynamic characteristics like low gradient, with
origin of chordae tendineae to both leaflets. The long-term durability and low thrombogenicity.
blood supply, particularly of the posterior papillary However, as the ideal prosthesis is not developed,
muscle is of great importance. The anterior papillary yet the choice has to be made between durability
muscle is usually supplied by the left anterior (i.e., mechanical prostheses) and low thromboge-
descending and the diagonal or a marginal branch nicity (i.e., biological prostheses). Given the fact
of the circumflex artery. The posterior papillary that patients with mitral valve disease more often
muscle is supplied by the left circumflex or the right suffer from atrial fibrillation per se requiring anti-
coronary artery, depending on the dominance of coagulation therapy, one argument for biological
the left or right coronary system. This single-vessel prosthesis is diminished. It has, however, to be
blood supply of the posterior papillary muscle leads mentioned that patients with atrial fibrillation and
to vulnerability against ischemic injury in case of a biological prosthesis require anticoagulation with
myocardial infarction. a lower target international normalized ratio (INR)
804 P.B. Rahmanian and T.C.W. Wahlers
compared to patients with a mechanical valve in In cases that require complete resection of the
mitral position. Nevertheless, the high rate of native valve, first the anterior leaflet is grasped
structural deterioration of tissue valves requiring and stabilized with a sharp clamp at its free mar-
reoperative valve replacement led to a decrease in gin. Alternatively, we place a 3-0 Prolene suture in
the preference of tissue valves since the 1980s the area of the A2 segment in order to pull the
favoring mechanical prostheses particularly in anterior leaflet towards the left atrium. Starting
25 younger patients. In our practice, we choose a bio- from a stitch incision in the A2 segment approxi-
logical prosthesis in all patients with a life expec- mately 2–4 mm from the anulus, the anterior leaf-
tancy not exceeding 10–15 years and those with let can be detached from the anulus towards both
contraindications for permanent anticoagulation. commissures using scissors or a scalpel. To excise
Mitral replacement in children and young ado- the whole valve, the chordae are then cut at their
lescents remains a problem, and therefore this patient origin at the papillary muscles, and the posterior
population preferentially undergoes mitral repair leaflet is excised in the same fashion.
even if the expected result is not ideal. Homograft However, in our practice, we always try to pre-
replacement, which is a valuable option in aortic serve at least a part or if possible the whole subval-
valve disease, has not been established in mitral valve vular apparatus as several studies have shown
disease due to suboptimal long-term results. improved outcome of patients with retained chor-
Most mechanical mitral valve prostheses used dae tendineae after mitral replacement. Moreover,
in clinical praxis today are bileaflet prostheses and there is evidence that complete chordal-sparing
have replaced the tilting disc prostheses. Modern mitral valve replacement is superior to partial
bileaflet prostheses are made of polycarbon and chordal-sparing procedures (Yun et al. 2002;
are reliable with a very low rate of mechanical fail- Chowdhury et al. 2005). We usually resect the
ure. They further have an acceptable low anterior leaflet in the fashion described above pre-
thrombogenicity but still require permanent anti- serving the posterior leaflet and the chordae to
coagulation. In our opinion, the best choice of a both papillary muscles (. Fig. 25.6). In cases of
mechanical prosthesis in mitral valve position is a severe calcification, the posterior leaflet can be
bileaflet prosthesis with a low profile and a broad decalcified using a rongeur leaving the fibrous
suture ring (Wippermann et al. 2005). The low parts of the leaflet as well as the chordae intact.
profile is important to avoid impairment of leaflet This is important to avoid destruction of the poste-
motion due to the subvalvular apparatus, which rior portion of the anulus with all its consequences
should be preserved in all cases. Generally, we such as injury of the circumflex artery or ventricu-
attempt to implant large prostheses (29 mm). In lar rupture at the atrioventricular junction (Cassel-
cases with a small anulus and a small body surface man et al. 1999; Sasaki and Ihashi 2003).
area, a 27 mm prosthesis is also acceptable. We In cases of severe mitral valve stenosis, small
never noticed complications or problems in terms left ventricles, and particularly narrow anular
of too large prostheses. However, asymmetric or diameter, a radial incision into the remaining leaf-
incomplete opening of bileaflet prostheses might let towards both trigones and to the middle por-
occur due to slow flow across a very large prosthe- tion of the posterior leaflet between both groups
sis orifice particular in patients with atrial fibrilla- of chordae creates enough space for the implanta-
tion potentially increasing the risk of tion of an adequately sized prosthesis.
thromboembolic complications. Reoperative procedures following mitral valve
replacement may become necessary due to deterio-
25.5.2.2 Excision of the Native Valve ration of biological prostheses, endocarditis,
During the last decades, the question whether to thrombosis, or paravalvular leakage. In cases of
excise or to preserve the subvalvular apparatus has mitral valve re-replacement, we prefer a transseptal
been the subject of a long debate, and there is mean- or the classic interatrial approach to the left atrium,
while clear evidence that preservation of at least a which allows the best exposure even if mobilization
part of the subvalvular apparatus improves outcome. of the heart is limited (Murashita et al. 2004). Small
However, excision of the native mitral valve is deter- noninfective paravalvular leaks can be closed by
mined by the underlying pathology of mitral valve epianular U-sutures passed through the atrial wall,
disease and necessarily needs to be waged between mitral anulus, and sewing ring of the prosthesis.
«how many tissue needs to be resected?» and «which Larger leaks require complete removal of the pros-
parts of the subvalvular apparatus can be preserved?» thesis in order to place the sutures for the new pros-
Chapter 25 · Surgery for Acquired AV-Valve Diseases
805 25
a b
..Fig. 25.6 a, b Excision of the mitral valve preserving the posterior portion of the subvalvular apparatus. Following
stitch incision in the A2 segment, the anterior leaflet is detached from the anulus towards both commissures, leaving
approximately a 2–4 mm rim of leaflet tissue at the anterior anulus. The chordae of the anterior leaflet are cut at the top
of the papillary muscle
thesis safely under direct vision. Particularly if a larly important in patients with thin and fragile
deteriorated prosthesis or valve thrombosis is pres- anular tissue. Alternatively, for example, in patients
ent, the prosthesis is often healed in very firmly and with active endocarditis, autologous or
covered by a fibrous layer. In these cases, we incise glutaraldehyde-preserved bovine pericardium can
the fibrous layer circularly at the sewing ring close be used. Usually, twelve to maximal 15 such
to the knots and cut all sutures separately. In most U-sutures are placed depending on the valve size,
instances, the prosthesis can then be removed ensuring that no gap remains between neighboring
bluntly from the pannus. In case of severe adhe- sutures. It is also important to avoid stitches cover-
sions, it might be easier to dissect the prosthesis out ing a too large anular distance that may result in
of the sewing ring, take the prosthesis out, and to plication of the anulus bearing the risk of paraval-
remove the remaining outer layer of the sewing ring vular leakage. Additional 4-0-polypropylene
and the sutures hereafter. It is particularly impor- stitches can be placed from the atrial side to the
tant to completely remove all pannus to ensure that sewing ring if there is suspicion of such plication.
the new prosthesis can be sutured to the anulus. The epianular technique provides a good adap-
More problems are created by prosthetic valve tation of the subvalvular apparatus at the base of
endocarditis with involvement of the anulus requir- the prosthesis improving the functional
ing debridement of anular tissue. In this case, the ventriculo-anular connection and therefore lead-
remaining anular tissue may not be firm enough to ing to better long-term results. In patients with
sufficiently anchor the new U-sutures requiring preservation of the posterior leaflet, the sutures can
deeper stitches and pericardium-supported sutures be placed from the anular insertion of the leaflet
bearing a particular risk for compromising struc- through the anulus leading to a horizontal plica-
tures like the circumflex artery and the AV node. tion that tightens the subvalvular apparatus. It is,
The sutures for mitral valve replacement can be however, particularly important to involve only a
placed either from the ventricular side (for epi-/ narrow portion of the leaflet because too much pli-
supraanular prosthesis placement) or from the cation may lead to protrusion of the remaining
atrial side (for intraanular placement) (. Fig. 25.7). leaflet tissue into the orifice of the prosthesis poten-
The epi-/supraanular technique results in lower tially impairing leaflet motion of mechanical pros-
stress for the anular tissue and has become the theses. This plication technique can also be applied
favorite technique. Commonly, 2-0 braided Teflon- in patients with fragile anterior leaflet tissue.
pledgeted U-sutures are passed through the anulus In our practice, we usually do not place the
from the ventricular side towards the left atrium. U-stitches from the atrial side through the anulus,
The utilization of small Teflon pledgets is particu- because this intraanular technique leads to an
806 P.B. Rahmanian and T.C.W. Wahlers
a b c
25
..Fig. 25.7 a–c Different suture techniques for mitral prosthesis implantation. a Single-stitch technique (left) and
double U-stitch technique (right). b In counterclockwise direction: pledgeted epianular U-suture; intraanular suture
from the atrium; intraanular suture from the ventricle. c Implantation of a mitral prosthesis with a running suture
a b
..Fig. 25.8 a, b Two common orientations of mechanical bileaflet prostheses in mitral position
eversion of the atrial tissue, which may become such as resection of the left atrial tissue are described
problematic in cases with extensive calcification of in the literature (Isomura et al. 1993).
the atrial wall or the anulus itself.
Several techniques for reducing the size of the 25.5.2.3 Implantation of Mechanical
left atrium are described in the literature. Most tech- Prostheses
niques base on the plication of the posterior portion Generally, the implantation of both biological
of the left atrial wall. An established approach con- or mechanical prostheses follows the suture
sists of placing a 3-0 Prolene mattress suture starting techniques described above. However, some
from the origin of the pulmonary veins towards the special considerations need to be discussed
right-sided margin of the incision in the interarte- regarding the orientation of the recent genera-
rial groove, which leads to eversion of two-times tion of bileaflet mechanical valves. There are
3–15 mm tissue depending on the stitching tech- two ways of implantation: the axis of the leaflets
nique. An additional overcasting suture smoothens can be directed horizontally or vertically to the
the endocardial atrial surface. Other techniques intercommissural line (. Fig. 25.8). The ques-
Chapter 25 · Surgery for Acquired AV-Valve Diseases
807 25
tion which position is best is not definitely During valve implantation, it is sometimes
solved yet; however, there is evidence that an useful to remove the handle of the prosthesis after
antianatomic implantation less likely leads to a tying down the three commissural sutures. This
left ventricular outflow tract obstruction (Laub helps to avoid that one or more struts jam into the
et al. 1992). Another issue may be the restric- atrial wall or into the subvalvular apparatus while
tion of leaflet motion due to remaining tissue, the prosthesis is tied down.
particularly if the posterior leaflet with the sub-
valvular apparatus is preserved. However, in 25.5.2.5 Technical Problems of Mitral
our own practice we have never observed such Valve Replacement
dysfunction. The design of some recent pros- Mitral valve replacement bears certain procedural
theses with a particular high anular cylinder risks, particularly during valve excision, anular
reduces the risk of alteration of leaflet function debridement, and implantation of the prosthesis.
from obstructing tissue. It is nevertheless Anular debridement during resection of the
important to ensure unimpaired leaflet motion native valve should not be too aggressive in order
once the valve is tied down and to make sure to leave enough tissue for safely placing the valve
that no redundant tissue protrudes into the sutures and neither damage the aortic valve, nor
valve orifice. On the other hand, with ventricu- the conduction tissue, or the circumflex artery.
lar relaxation during diastole subvalvular struc- Therefore, it is important to leave a margin of
tures are moved laterally away from the 3–5 mm leaflet tissue when excising the native
prosthesis, and valve closure happens when the valve whenever possible. This may, however, not
ventricle is maximally filled, and therefore be possible in cases of severe mitral stenosis with
impairment of prosthesis function is unlikely heavily calcified mitral leaflets and anulus where
(Wipperman et al. 2005). the left ventricular muscle is involved also (Iida
et al. 2005).
25.5.2.4 Implantation of Biological Aggressive decalcification may not leave enough
Prostheses tissue as solid skewback for the valve sutures
The commercially available biological prostheses (. Fig. 25.10), which results in a rare but particularly
for mitral replacement are made from porcine dangerous situation. Then, sutures for valve implan-
aortic valves or constructed with bovine pericar- tation sometimes need to be placed through the
dium and usually include a sewing ring and a ventricular muscle, particularly in the posterior por-
stent with three struts. During implantation, two tion of the anulus. These sutures in face of an exten-
struts are directed towards both trigones, whereas sively thinned out anulus are prone to rupture or
the third strut is positioned in the middle of the dissection of the posterior atrioventricular junction.
posterior anulus (. Fig. 25.9). This orientation This rupture may take place immediately with ven-
keeps the struts away from the left ventricular tricular contraction resuming after opening cross-
outflow tract and thus avoids obstruction during clamp but may also be delayed for several hours
systole (Chitwood 1998). Particular caution has to after the operative procedure when the sutures cut
be directed towards the U-sutures, which can through the ventricular muscle. Other causes for
potentially get entangled with the struts leading to ventricular rupture can be extensive tension on the
impaired function of the cusps of the prosthesis or anulus during valve excision, particularly during
even prosthesis destruction. Particularly bovine reoperation, and also inadequate pressure while
pericardial valves with their narrow struts are pushing the prosthesis in place.
prone to this complication, which can be seen eas-
ily and becomes evident during echocardiography
control after weaning from bypass. Therefore, The most dangerous complication of mitral
most manufacturers deliver their prostheses with valve replacement is rupture of the posterior
a temporary holding suture that connects the atrioventricular junction or the posterior left
three struts and avoids this entanglement. This ventricular wall. This commonly is caused by
securing suture is removed eventually after the too aggressive decalcification of the anular tis-
valve is tied down. In our practice, we routinely sue and/or luxation of the heart after prosthe-
ensure unimpaired leaflet motion and regular sis implantation.
coaptation using a small dentist mirror.
808 P.B. Rahmanian and T.C.W. Wahlers
a b
25
c d
..Fig. 25.9 a–d Orientation of biological prostheses. a Two of the three struts are directed towards the trigones (right
and left white point); the third strut is placed at the middle of the posterior anulus (middle posterior white point). This
orientation allows sufficient opening of the left ventricular outflow tract during systole. b Particular caution is required
when tying the U-sutures to avoid entangling the struts. c Entangling a strut results in distortion of the neighboring
leaflets which may be only discrete in bovine pericardial prostheses (above) and more obvious in porcine valves (below).
d A dentists mirror is a helpful tool to visualize correct implantation of the prosthesis after implantation
The only chance to treat atrioventricular dis- ately to unload the left ventricle. Attempts to con-
ruption is to stabilize the atrioventricular junction trol the disruption from outside should be avoided,
with pledgeted (Teflon or pericardium) sutures because this inevitably involves luxation of the
placed from inside the ventricle after temporary heart potentially worsening the life-threatening
removal of the prosthesis. It is further important situation, which is per se associated with a mortal-
to reestablish extracorporeal circulation immedi- ity of 30–50 % (David 1987). However, in some
Chapter 25 · Surgery for Acquired AV-Valve Diseases
809 25
..Fig. 25.10 In case of extensive resection of tissue and/or decalcification in the area of the posterior anulus,
dissection of the AV sulcus may result (the arrows mark the channel of disruption). In these cases, reconstruction of
the AV continuity can be achieved with pledgeted sutures
cases of atrioventricular
disruption, successful complication is serious and potentially life-
pasting of a pericardial patch onto the ruptured threatening. Particularly biological prosthesis
area has been described. Preservation of the pos- with their higher profile and the three struts of
terior leaflet minimizes the risk of atrioventricular which one is located in the middle of the posterior
disruption (Deniz et al. 2008). anulus are associated with this complication,
Resection of papillary muscle under tension is whereas it is rarely seen in patients receiving
also dangerous and bears the risk of ventricular modern low-profile mechanical prostheses.
rupture if the resection line is placed too deep at Generally, displacement of the heart should be
the base of the papillary muscle. avoided during mitral valve replacement once the
Another rare but typical complication of prosthesis is in place.
mitral valve replacement is rupture of the poste-
rior ventricular wall caused by the prosthesis
itself. This complication is seen in patients with 25.5.3 Valve Sparing Procedures
small ventricles and in cases where the prosthesis
reaches deep into the ventricular cavity. If the The decreasing incidence of mitral stenosis due to
heart is displaced for de-airing purposes, the rheumatic disease and the increasing proportion
prosthesis may cause perforation of the ventricu- of patients presenting with mitral regurgitation
lar wall. Likewise atrioventricular disruption, this due to degenerative or ischemic etiologies led to a
810 P.B. Rahmanian and T.C.W. Wahlers
a b
..Fig. 25.11 a–c Technique of open mitral valve commissurotomy. a After exposure of the line of fusion a stitch
incision is placed approximately 5 mm from the anulus within this line. b An angled clamp is then placed into the
incision and opened towards the valve orifice. The commissure is then dissected sharply. c Finally, the commissurotomy
is extended towards the central fibrous body
Chapter 25 · Surgery for Acquired AV-Valve Diseases
811 25
chordae are also conjoint, then sharp longitudi- Every exploration of a diseased mitral valve
nally separation is necessary. It should be avoided should start with functional analysis. Saline is
to extend the commissurotomy all the way to the instilled into the left ventricle. The saline leakage
anulus because the lack of tissue in this area will through the valve helps in identifying the area
result in inadequate coaptation and thus mitral and pathology of regurgitation. After saline test-
regurgitation. Some amount of superficial calcium ing, careful exploration of the valve using valve
may be carefully removed with a rongeur while hooks is performed. Following Carpentier’s ana-
keeping the continuity of the leaflet intact. In cases tomical classification, the height of coaptation of
of heavily calcified leaflets, commissurotomy may the corresponding segments of the anterior (A1,
not fix the problem, and valve replacement should A2, A3) and posterior (P1, P2, P3) leaflets is ana-
be considered instead. Mitral replacement has also lyzed (. Fig. 25.12).
to be considered if a significant regurgitation is Carpentier’s systematic classification of mitral
noticed following commissurotomy. valve regurgitation is generally based on leaflet
motion (. Fig. 25.13):
25.5.3.2 Mitral Valve Reconstruction 55 Type I, normal leaflet motion. This type of mitral
(Leaflet and Anulus) regurgitation is commonly caused by anular dil-
Mitral valve reconstruction requires a deep atation resulting in a loss of coaptation area
understanding of underlying pathology and while the leaflets retain their normal mobility.
involves all parts of the mitral apparatus. In this case, the left ventricle is usually dilated.
Carpentier’s pathophysiologic triad describes the Leaflet perforation is also referred to as type I
relationship between the cause of the disease (eti- mitral regurgitation. Isolated type I regurgita-
ology), the result of the disease (lesion), and the tion is found in about 10 % of cases.
functional impairment resulting from the lesion 55 Type II, extended leaflet motion. Extended leaf-
(leaflet dysfunction) (Carpentier 1983). Following let motion is commonly found in patients with
Carpentier’s principles, the vast majority of mitral degenerative mitral valve disease (Barlow’s dis-
pathologies –except heavily calcified lesions or ease, fibroelastic deficiency). Barlow’s disease is
extensively destroyed endocarditic valves – are typically associated with excess leaflet tissue,
potentially suitable for a reparative approach. chordal elongation, and anular dilatation lead-
However, it should be kept in mind that mitral ing to complex mitral regurgitation. Chordal
replacement with modern prostheses results in rupture and papillary muscle rupture are also
acceptable long-term outcome, and therefore a referred to as type II lesions. With 60 % of cases,
critical assessment of long-term durability in type II is the most common lesion in patients
patient with complex pathologies is required. with mitral regurgitation.
Following establishment of cardiopulmonary 55 Type III, restricted leaflet motion. Restricted leaf-
bypass and exposure of the mitral valve, the deci- let motion during systole and diastole (type IIIa)
sion whether to repair or to replace the mitral is caused by leaflet thickening and retraction,
valve has to be made by the surgeon. This judg- chordal thickening and shortening, and com-
ment depends on three important factors: missural fusion frequently observed in patients
55 Experience of the surgeon in the application with rheumatic disease. The mechanism of type
of different reconstructive techniques IIIb dysfunction is restricted leaflet motion dur-
55 Type and extension of mitral valve pathology ing systole due to left ventricular enlargement
Patient-related factors such as age, comorbidities, and papillary muscle displacement as seen in
previous cardiac operations, contraindication patients with congestive heart failure and in
against anticoagulation, and others ischemic mitral regurgitation.
To date, repair rates up to 90 % depending on
pathology are feasible, particularly in tertiary cen- The most common cause of mitral regurgita-
ters with high caseload. tion in industrialized countries is degenerative
Feasibility of repair is first of all determined by disease with leaflet dysfunction type II (excess
the valve pathology and its extension. Most con- motion of the margin of the leaflet in relation to
venient are mitral valve pathologies with pre- the anular plane) due to chordae elongation or
served leaflet mobility and enough leaflet tissue to rupture. Anular dilatation (type I) is almost
allow remodeling a competent valve. always an associated finding.
812 P.B. Rahmanian and T.C.W. Wahlers
a b
25
n
tio
n
tio
pta
ta
ap
oa
co
c
No
d
oa
Br
..Fig. 25.12 a, b Principle of mitral valve reconstruction for mitral regurgitation. One goal of reconstruction is the
restoration of a broad coaptation surface b. A small coaptation line a results in high tension on the chordae potentially
leading to chordal rupture and leaflet prolapse
In clinical practice, there is a broad spectrum patients with longstanding prolapse, secondary
of degenerative disease ranging from fibroelastic changes may occur in the prolapsing segment,
deficiency (FED) to Barlow’s disease. The most resulting in myxomatous leaflet thickening and
complex form, Barlow’s disease, is characterized expansion (FED+). Forme fruste defines an
by excess leaflet tissue with large billowing, thick- intermediate form of degenerative disease with
ened leaflets, and anular dilatation. Chordal elon- excess tissue with myxomatous changes in usually
gation is the most common cause of prolapse, and more than one leaflet segment, but usually does
multiple leaflet segments are usually involved. It not involve the whole valve, differentiating it from
generally occurs in younger patients (aged Barlow’s disease (Adams et al. 2010). The underly-
<60 years), who have a long history of a heart ing pathology defines the technical approach in
murmur. In contrast, fibroelastic deficiency is a reconstructive mitral valve surgery for degenera-
degenerative disease of older individuals (usually tive mitral regurgitation.
>60 years of age), with a shorter history of valve Mitral valve reconstruction follows three gen-
regurgitation. Rupture, often of a single chord, is eral principles: preservation of leaflet motion, res-
the most common cause of leaflet dysfunction in toration of a large surface of leaflet coaptation,
fibroelastic deficiency, and in most cases the only and remodeling of the anulus of the mitral valve
abnormal leaflet tissue is found in the prolapsing (Carpentier et al. 1971). A large area of coaptation
segment. The other leaflet segments are often thin in early systole minimizes leaflet shear stress and
and translucent and of normal height. The poste- the smaller the coaptation area, the higher the
rior anulus may be dilated, but the size of the tension on the primary cords increasing the risk
anterior leaflet and valve is most often normal. In of chordal elongation or rupture (see . Fig. 25.12).
Chapter 25 · Surgery for Acquired AV-Valve Diseases
813 25
a b c
..Fig. 25.13 a–c Carpentier’s classification of mitral valve regurgitation. a Type I dysfunction (normal leaflet motion)
results from anular dilatation, leaflet perforation, or a combination of both. Leaflet perforations may be congenital (as
part of a broad spectrum of anterior leaflet clefts with or without concomitant defects of the atrioventricular channel) or
achieved following mitral valve endocarditis. b Type II dysfunction (excessive leaflet motion) is caused by elongation or
rupture of the chordae tendineae or the papillary muscles. Papillary muscles pathology can be degenerative or ischemic.
Type II dysfunction commonly affects the posterior leaflet but may also be found in the anterior leaflet or both leaflets.
From the surgical view, degenerative type II lesions of the posterior leaflet can be repaired easily and with good long-
term durability. c Type III dysfunction (restricted leaflet motion) affects the posterior as well as the anterior leaflet and is
commonly associated with rheumatic heart disease. Due to the underlying pathology, rheumatic type III dysfunction is
less likely to be repaired as compared to type II dysfunction. In patients with concomitant mitral valve stenosis,
percutaneous valvulotomy has become a valuable approach. Open valvulotomy still plays a role; however, in most
instances mitral valve replacement with preservation of the subvalvular apparatus provides good long-term results.
Type III dysfunction may also be caused by ventricular dilatation in patients with dilated cardiomyopathy or by papillary
muscle displacement in patients with ischemic cardiomyopathy
Enlargement of the coaptation area can usually be identified during initial valve analysis in most
achieved by narrowing the mitral anulus using cases, as the free prolapsing margin including
anuloplasty devices. Due to the relationship of the the ruptured chord is flushed back into the left
anterior portion of the anulus to the fibrous atrium (Carpentier 1983). In Barlow’s disease,
trigones of the heart, significant narrowing of the not only the prolapsing segment is affected. In
anulus can only be achieved at the posterior por- these patients, excess tissue is also seen in the
tion. The normal ratio between the septolateral neighboring valve segments and needs to be
and transverse diameter of the mitral anulus is addressed to achieve successful repair. Our
approximately 3:4 during systole. A remodeling preferred approach for prolapsing posterior leaf-
ring anuloplasty and basically also all historic let segments is a quadrangular resection with
remodeling techniques, as described by Paneth, sliding plasty (. Fig. 25.14). This technique
Whooler, and others, attempt to restore this ratio. together with mitral valve anuloplasty repre-
An anuloplasty ring prevents further anular dila- sents the most common reparative measure and
tation, preserves leaflet mobility, and relieves ten- has proven long-term durability. First, it is
sion to the leaflets (stabilizing the repair) by important to assess the extension of prolapsing
optimizing the coaptation area. segment (most likely involving the P2 segment)
in order to define the amount of tissue that
kQuadrangular resection of the posterior needs to be resected. We then identify the pri-
leaflet and sliding plasty mary chordae of the prolapsing segment and
Posterior leaflet prolapse due to fibroelastic defi- mark them with a 5-0 Prolene suture. Depending
ciency is usually caused by rupture of a single on valve size, the length of the segment to be
chord and therefore often affects an isolated resected is 1–3 cm. Following the definition of
portion of the leaflet. The ruptured chord is the resection borders, it is recommended to
814 P.B. Rahmanian and T.C.W. Wahlers
a
Left fibrous trigone
Incision for
Posterior leaflet
quadrangular
resection
Direction of sliding plasty
b c
Compression suture
Compression suture
..Fig. 25.14 a–c Mitral valve reconstruction for degenerative mitral regurgitation due to chordal rupture in the P2
segment. a After careful functional and anatomic valve analysis, a quadrangular resection of the prolapsing segment of
the posterior leaflet is performed. The excision lines are directed vertically to the anulus avoiding convex or concave
orientation. For an additional sliding plasty, the incision can be extended parallel to the anulus towards the P1 and P3
segment. b Following the quadrangular resection, valve reconstruction is performed in two steps. The anulus is
narrowed at the base of the resected segment by compression sutures placed in figures of 8 or as mattress sutures with
or without pledgets at the atrial as well as the ventricular aspect of the anulus. c The remaining parts of the posterior
leaflet are then readapted by a sliding plasty at the base. The remaining cleft between the P1 and P3 segment is then
closed by single figure-of-eight sutures or by a running suture
Chapter 25 · Surgery for Acquired AV-Valve Diseases
815 25
P3 segments at the top with single Prolene 5-0 a triangular resection of the prolapsed part fol-
stitches. In cases of excess tissue as seen in lowed by direct closure with interrupted sutures
Barlow’s disease, following quadrangular resec- (. Fig. 25.15d, e). The triangular resection should
tion of the prolapsing segment, a sliding leaflet be limited to the rough zone to avoid compro-
plasty is performed to shorten the height of mising the body of the leaflet (Adams et al.
remaining leaflet segments. The residual P1 and 2006).
P3 segments are detached from the anulus
approximately one third in the direction to the kAnuloplasty
corresponding commissures. The next steps After addressing the prolapsing segments of the
attempt to gather the posterior portion of the anterior and/or posterior leaflet, an anuloplasty
anulus as much as possible. To achieve this, up should be performed. The use of anuloplasty
to three compression stitches are placed tangen- devices in the setting of mitral valve repair
tially through the posterior segment of the anu- has several reasons. Most important are the
lus. Finally, a «sliding plasty» of the residual P1 restoration of a normal anular shape and dimen-
and P3 segments is performed and the gap sion. The normal ratio between the septolateral
between the residual leaflets is closed with and transverse diameter of the mitral anulus is
sutures. approximately 3:4 during systole. This ratio
The reconstruction of the posterior leaflet inverts in patients with chronic mitral regurgita-
should be already sufficient before the additional tion leading to a loss of leaflet coaptation area,
anuloplasty is performed. To test the result, saline even in nonprolapsing segments. A remodeling
is instilled into the left ventricle as described ring anuloplasty restores the physiologic ratio of
before. In case of residual regurgitation, addi- the normal anulus during systole, restoring not
tional adaptation stitches may be placed into the only the size but also the shape of the anulus. An
A1-P1 or A3-P3 to improve leaflet coaptation. anuloplasty ring prevents further anular dilata-
The anuloplasty, which completes mitral valve tion, preserves leaflet mobility, and relieves ten-
reconstruction, stabilizes the repair but does sion on the leaflets by optimizing the coaptation
usually not improve the leaflet reconstruction zone. There are several different anuloplasty
(Lim et al. 2002). devices available including flexible or semirigid
In some patients, particularly in the case of rings and bands. In our practice, we preferably
Barlow’s disease, excessive posterior leaflet tissue use semirigid closed anuloplasty rings in addition
is present, and it is necessary to reduce the height to every mitral valve repair in order to normalize
of the posterior leaflet sufficiently to avoid post- the mitral geometry and furthermore to stabilize
operative systolic anterior motion with resultant the reconstruction by relieving tension from the
left ventricular outflow tract obstruction and sutured leaflets.
mitral regurgitation. Systolic anterior leaflet First, appropriate ring sizing is performed fol-
motion can also be a result of too small anulo- lowing Carpentier’s principles. The measurement
plasty ring sizes. Postrepair echocardiography is is based on the intercommissural distance and
an important tool to detect this complication. also the surface area of the anterior leaflet. The
height of the anterior leaflet is defined by pulling
kTriangular resection of the anterior leaflet it downwards to the posterior leaflet and in com-
As mentioned before, in contrast to the posterior parison with the sizer’s surface (. Fig. 25.15a). The
leaflet, aggressive resection of the anterior leaflet intercommissural distance is measured by the dis-
is not possible due to anatomic limitations. tance between the two corner points of each indi-
Resection of large portions of the anterior leaflet vidual sizer.
(>10 %) distorts leaflet geometry and reduces the The anuloplasty is then performed by first
coaptation area. Furthermore, extensive resec- placing horizontal mattress stitches circumfer-
tion of the anterior leaflet compromises leaflet entially through the anulus (. Fig. 25.15b). In
mobility and increases the risk of repair failure opposite to mitral valve replacement, we do not
(Filsoufi and Carpentier 2007). Therefore, repair use Teflon-pledgeted sutures for mitral anulo-
strategies for anterior leaflet prolapse are plasty. In case of leaflet reconstruction, it is
designed to correct prolapse without resection important to place the sutures outside the area
of significant areas of leaflet tissue. A limited of reconstruction. Particular attention needs to
prolapse of the anterior leaflet can be treated by be addressed to the conduction system and the
816 P.B. Rahmanian and T.C.W. Wahlers
a b
Valve sizer
Commissural
marks
25
Anterior
leaflet Repaired
posterior
leaflet
Chapter 25 · Surgery for Acquired AV-Valve Diseases
817 25
course of the circumflex artery. The anular chord belongs to. A continuous horizontal mat-
sutures are then equally spaced in the area tress suture is placed vertically to the anulus pli-
between the two commissures and the corre- cating the prolapsing segment and shortening the
sponding segment of the prosthetic ring. In the anular circumference by the length of the prolaps-
remaining portion of the anulus, the spacing is ing segment. Consequently, the suture line needs
set to conform the anulus to the shape and size to be extended into the atrial wall. An alternative
of the prosthetic ring. When the ring sutures approach would be the resection of the prolapsing
are tied, the ring reshapes the anulus to a nor- segment in a quadrangular or triangular shape
mal systolic geometry (. Fig. 25.15c). and direct readaptation of the remaining seg-
ments with intact chords. Chordal ruptures of the
25.5.3.3 Mitral Valve Reconstruction anterior leaflet chords, particularly of strut chords
(Chordae) (principle chords), represent a special challenge
Mitral regurgitation may be caused by isolated and were deemed not repairable with satisfying
chordal pathologies, but more often chordal long-term results and therefore led to valve
pathologies are accompanying lesions in patients replacement. However, a feasible approach in our
with degenerative mitral disease. In this section, practice is the utilization of the chordal transfer
reconstruction techniques for mitral regurgita- technique. A small segment of the corresponding
tion due to chordal pathologies are described. posterior leaflet segment with its marginal chord
in place is dissected and sutured to the ventricular
kPlication or resection of the posterior surface of the anterior leaflet close to the insertion
leaflet with or without chordal transfer of the ruptured chord. The resulting defect in the
Chordal rupture is most commonly located at the posterior leaflet is closed by leaflet plication. The
posterior leaflet, particularly at the P2 segment, midterm results of this technique are promising;
probably because the chordae of the P2 segment however, long-term data is required to determine
are prone to the highest tension in patients with the durability of this approach (Anyanwu and
loss of coaptation area. Chordal rupture is a typi- Adams 2007).
cal complication of Barlow’s syndrome which is
characterized by excess leaflet tissue, chordal kChordal plasty
elongation, and anular enlargement. Chordal Chordal plasty presents an alternative approach
pathologies are also seen in patients with Marfan’s for the treatment of leaflet prolapse instead of
syndrome. leaflet resection (. Fig. 25.16). In our practice, we
An isolated rupture of a posterior leaflet chord utilize 4-0 or 5-0 polytetrafluoroethylene sutures
can be treated by leaflet plication. Alternatively, (PTFE) as neo-chordae. The required length of
particularly in the setting of anterior leaflet pro- the neo-chordae is determined by lowering the
lapse due to chordal rupture, a triangular resec- free margin of the anterior leaflet to the anulus
tion of the prolapsing segment can be performed. and measuring the distance between the margin
The triangular resection technique equals the of the leaflet from this position and the head of
quadrangular resection without sliding plasty as the papillary muscle. This distance is usually
described above. Herein, the leaflet plication will 1.5–2 cm. Determining the correct length is par-
be described in detail. ticularly important – a too short neo-chord results
The leaflet plication technique involves invag- in restricted leaflet motion where as a too long
ination of the portion of the leaflet the ruptured neo-chord does not effectively reduces the leaflet
..Fig. 25.15 a–e Mitral valve anuloplasty. a Different anuloplasty rings are utilized for mitral valve reconstruction.
Sizing is performed by measuring the height of the anterior leaflet and the intercommissural distance. This can be
achieved by grasping the anterior leaflet with a hook. b Horizontal mattress sutures are placed circumferentially
through the anulus and the corresponding segments of the prosthetic ring. c When the sutures are tied down, the
anuloplasty ring reshapes the mitral anulus to a normal geometry. Usually, 10–12 sutures are required to sufficiently
secure the ring. d, e In patients with anterior leaflet prolapse due to chordal rupture, a triangular resection of the
prolapsing segment (here shown for segment A2) can be performed followed by direct closure of the defect with single
sutures. e Note the triangular resection is not extended to the base of the anterior leaflet. A stabilizing ring anuloplasty
should be added in any instance
818 P.B. Rahmanian and T.C.W. Wahlers
25
c d
Papillary muscle
Chapter 25 · Surgery for Acquired AV-Valve Diseases
819 25
prolapse (Sarsam 2002). The neo-chord is attached with anuloplasty which creates a good area of
to the corresponding papillary muscle using coaptation and therefore limits the tension on the
pledgeted sutures stitched through the fibrous
elongated chords. The valve is, moreover, mold-
portion of the muscle. Meanwhile, there are also ered towards a funnel shape which prevents pro-
commercial sutures with integrated Teflon pled- lapsing of the leaflet segment with elongated
gets available. At the leaflet margin, the suture chords (Smedira et al. 1996).
should be stitched twice to avoid tearing the often
fragile and thin leaflet tissue (Risteski et al. 2007). 25.5.3.4 Patch Reconstruction
To ensure the correct length before definitely The most common cause of leaflet perforation
tying the neo-chord, a clip can be used to fixate leading to type I regurgitation is mitral valve
the neo-chord at the estimated length. If saline endocarditis. Surgical treatment with the inten-
testing shows a sufficient result, the neo-chord tion to restore the native valve requires removal of
can be tied down to its final length. all infective material while preserving intact leaf-
let margins. The resulting defect can be closed uti-
kChordal shortening lizing a patch of autologous pericardium or
If leaflet prolapse is caused by chordal elongation, glutaraldehyde-treated bovine pericardium with a
shortening of the affected chord can be a valuable 5-0 running polypropylene suture. It is important
option. Following Carpentier, the relative amount to size the patch approximately 20 % larger than
of chordal elongation is estimated by pulling the the defect to avoid tension on the leaflet due to the
free margins of the leaflets with two valve hooks. suture line (Sternik et al. 2002; Zegdi et al. 2005).
The body of the papillary muscle originating the
elongated chordae is then spliced longitudinally. 25.5.3.5 Alfieri-Technique
The portion with the insertion of the elongated The simplest maneuver for mitral valve repair of
chordae is then slid downwards and reattached to type I insufficiency with central regurgitation is the
the remaining muscle (. Fig. 25.17). Alternatively, so-called Alfieri stitch (edge-to-edge repair)
the elongated chord can be folded downwards and (. Fig. 25.19). After exploration of the valve, the
tied to the base of the papillary muscle (. Fig. central margin of the A2 segment is identified and
25.18). Both techniques result in a shortening of pulled towards the surgeon. Accordingly, the cen-
the elongated chord thus improving valve geom- tral margin of the P2 segment is grasped, and both
etry. However, in our opinion, this technique may segments are tied together with a pledgeted (Teflon
harm the papillary muscle, and therefore we pre- or pericardium) 4-0 polypropylene mattress suture
fer the utilization of artificial chordae. Another 3–5 mm from the free margin (Maisano et al. 1998).
alternative is described by Kay (1992), who also The long-term results of this technique are
folds the elongated chord but fixates the resulting controversial. Alfieri himself recommends always
loop at the fibrous head of the papillary muscle adding an anuloplasty device. With the advent of
and not at the body. minimal-invasive and catheter-based procedures,
Regardless of these techniques described, it the edge-to-edge repair has become more popu-
needs to be taken into consideration that limited lar. These techniques are described in detail in
leaflet prolapse due to chordal elongation might 7 Chapter «Minimally Invasive Cardiac Surgery», Sect.
be resolved by correcting the posterior anulus 26.3.5.1; Sect. 26.3.5.2.
..Fig. 25.16 a–e Artificial chordoplasty. a, b The use of 5-0 Polytetrafluoroethylene (PTFE) sutures as artificial chords
has replaced common techniques for chordal shortening and «flip-over» techniques in most instances of mitral valve
reconstruction. The figures show an anterior leaflet prolapse with ruptured chordae. The remnants of the ruptured
chordae are excised. In addition, secondary chordae may be released. c, d Double-armed 5-0 PTFE sutures are passed
through the papillary muscle in a figure-of-eight manner approximately 1 cm from the tip. Both ends of the suture are
then passed through the margin of the prolapsing leaflet segment. e A temporary holding suture is passed through the
prolapsing leaflet and the corresponding nonprolapsing segment of the opposite leaflet. Pulling the holding suture
allows determination of the correct height of the artificial chordae before tying the PTFE chord
820 P.B. Rahmanian and T.C.W. Wahlers
a b
25
c d
..Fig. 25.17 a–d Carpentier’s technique of chordal shortening for elongated chords. The corresponding papillary
muscle can be incised longitudinally a, moved downwards by the length of elongation and reattached with pledget-
armed sutures thus tightening the elongated chords b. An alternative technique consists of gathering the elongated
chord and attaching it to the incised papillary muscle c. d The incision of papillary muscle is finally closed covering the
gathered chord. An additional anuloplasty is strongly recommended to stabilize the repair
..Fig. 25.19 a, b «Edge-to-edge» repair for ischemic mitral regurgitation. a Pericardial sutures or pledget-armed
horizontal mattress sutures are passed through the margins of the anterior and posterior leaflet and tied creating a
double orifice. This fixation results in a better coaptation during systole thus reducing mitral regurgitation. b «Edge-to-
edge» repair of an anterior leaflet prolapse in the area of A3 segment
to papillary muscle rupture. In the latter patient tured papillary muscle with sutures. In cases of pap-
group, acute mitral regurgitation and accompany- illary muscle elongation due to fibrotic degeneration
ing pulmonary edema often require emergency and elongation of papillary muscles following myo-
surgery. cardial infarction, Teflon-pledgeted PTFE sutures
Surgical outcome of patients with acute ischemic are stitched through the base of the papillary muscle
mitral regurgitation often is inferior; however, on one side and through the head of the papillary
immediate and consequent surgery remains the only muscle on the other side in a U-fashion. This leads to
option. In this setting, mitral valve replacement is a shortening of the elongated papillary muscle and
usually preferred over reconstructive measures. avoids cutting through the muscle (Fasol et al. 2000).
However, some authors suggest refixating the rup- If this reconstructive approach does not significantly
822 P.B. Rahmanian and T.C.W. Wahlers
reduce mitral regurgitation, mitral valve replace- recurrence or residual mitral regurgitation has
ment has to be performed in the same session. In been observed, and the choice of the right device is
this patient population in particular, it is important still under debate. The trend goes to completely
to preserve as much of the subvalvular apparatus as closed semirigid rings and some authors advocate
possible to avoid further re(de-)modeling of the disease-specific rings that help in addressing the
already altered ventricle (Jouan et al. 2004). asymmetric tenting and leaflet restriction seen in
25 Chronic ischemic mitral regurgitation in ischemic mitral regurgitation (Daimon et al.
patients with ischemic cardiomyopathy following 2006). The inabilities to overcorrect the septolat-
myocardial infarction requires a more differenti- eral dimension and to address anterior or asym-
ated approach. In most instances, chronic isch- metric anular dilatation are some major concerns
emic mitral regurgitation develops following current percutaneous approaches to correct isch-
occlusion of a dominant right coronary artery or emic mitral regurgitation are faced with (Anyanwu
a concomitant occlusion of branches of the right et al. 2006).
and circumflex artery. This leads to several
pathoanatomic and pathophysiologic changes
affecting the components of mitral valve function. 25.5.5 Mitral Valve Endocarditis
The left ventricle loses its elliptical shape and
becomes more spherical which leads to displace- Infective endocarditis commonly includes the aor-
ment of the papillary muscles, especially the pos- tic valve while involvement of the mitral valve is
teromedial papillary muscle. This papillary seen less frequently. In many instances, mitral valve
muscle displacement leads to a tethering of the endocarditis occurs secondary to aortic valve
mitral leaflets significantly reducing the coapta- endocarditis. Destruction of the infected aortic
tion area resulting in type III b mitral regurgita- valve leads to aortic regurgitation and prolapse of
tion (Filsoufi et al. 2006). This restricted leaflet the aortic cusps into the left ventricular outflow
motion is mainly seen after posterior infarction tract during diastole and can produce «jet lesions»
and affects the posteromedial scallop of the poste- of the mitral valve. These lesions are typically
rior leaflet (P3 segment) adjacent to the posterior located in the central portion of the anterior leaflet
commissure area. and range between minimal vegetation and
Surgical treatment of chronic ischemic mitral extended leaflet perforation (Piper et al. 2002).
regurgitation usually includes coronary artery Primary mitral valve endocarditis is a rare entity
revascularization, as most patients present with and usually leads to chordal rupture and/or large
multivessel disease. However, coronary revascular- vegetations. Mitral anular abscess formation is rare
ization alone, either surgically or interventionally, and presents a challenge for surgical treatment.
does not correct ischemic mitral regurgitation in The surgical principle of repairing the valve is
most patients. Furthermore, several studies have to completely remove all infected tissue (Alexiou
shown a survival benefit of patients with ischemic et al. 2000; Mylonakis and Calderwood 2001)
cardiomyopathy and sufficient mitral valve func- (. Fig. 25.20). This often leads to resection of large
tion compared to those with untreated mitral parts of the mitral valve requiring mitral valve
regurgitation. Therefore, if more than mild mitral replacement. The technique of replacement follows
valve regurgitation is present, bypass surgery the same principles as described above. We usually
should be accompanied by mitral valve recon- preserve the subvalvular apparatus – if not infected –
struction. Mitral valve anuloplasty is the appropri- and implant the prosthetic valve in an epianular
ate surgical procedure in most cases of ischemic position with Teflon-pledgeted U-sutures.
mitral regurgitation. The goal is to restore a large In some patients, resection of infected tissue is
coaptation area and to remodel the anulus while limited to smaller portions of the valve and allows
preserving leaflet mobility (Carpentier 1983). This native valve reconstruction. We particularly try to
can be achieved by downsizing the anulus using an achieve preservation of the native valve in chil-
undersized anuloplasty ring. This downsizing cor- dren, adolescents, and young adults to avoid neg-
rects the septolateral displacement and thus ative side effects of prosthetic valve replacement
reduces restricted leaflet motion and restores leaf- even if mitral valve reconstruction results in mild
let coaptation. For this approach, several devices to moderate mitral regurgitation. Reconstruction
(rings and bands) have been utilized. However, techniques depend on the localization of the
Chapter 25 · Surgery for Acquired AV-Valve Diseases
823 25
..Fig. 25.20 a–d Mitral valve reconstruction for
a
infective endocarditis. In cases of local destruction a of
the mitral valve, complete resection of the infected tissue
may be possible while preserving enough tissue for
sufficient reconstruction. In the case shown here, parts of
the anterior and posterior leaflet in the area of the
anterior commissure had to be resected b. The defect was
closed by reattaching the remaining anterior and
posterior leaflets at the commissure c resulting in a
competent and nonstenotic mitral valve d
b c
resulting defect. Smaller defects can be closed by to a very fragile tissue in this area often not allow-
direct sutures, whereas larger defects require ing a firm fixation of the prosthesis leading to para-
readaptation similar to a commissuroplasty. This valvular leakage or complete tear out of the
is only possible as long as the remaining valve ori- prosthesis. Some authors have therefore suggested
fice – measured with a sizer intraoperatively – is placing the prosthesis not in an anular position but
large enough. Even very large defects of the ante- above the anulus directly in the atrial wall (Dreyfus
rior leaflet can be handled with patches from et al. 1990; de Kerchove et al. 2007).
autologous pericardium.
A surgical challenge is patients with abscess
formations involving the mitral anulus or the 25.5.6 Testing of Mitral Valve
neighboring myocardium. In these cases, resection Reconstruction
of infected tissue involves an extended debride-
ment of anular or ventricular wall tissue and leaves Successful and reliable mitral valve repair
large defects requiring patch reconstruction. The requires intraoperative evaluation of mitral valve
patch, consisting of autologous pericardium, needs function. Several methods have been described
to be sutured carefully to the left ventricular wall (. Fig. 25.21).
and – passing the anulus – the left atrial wall The easiest and most common method to test
(Alexiou et al. 2000) before a valve prosthesis can the repair utilizes a large syringe to inject saline
be implanted. Prognosis of the patients, however, into the left ventricular cavity. Once the ventricle
remains poor, as the inflammatory processes lead is filled, the mitral leaflets are distended and
824 P.B. Rahmanian and T.C.W. Wahlers
a b
25
..Fig. 25.21 a, b Intraoperative testing of mitral valve repair by filling of the left ventricle with cardioplegic solution.
a Following isolated mitral valve reconstruction a thin perfusion catheter (12 Ch) is passed through the aortic valve into
the left ventricle using the cardioplegia insertion site at the ascending aorta. Cardioplegic solution is then administered
under pressure control. Mitral valve closure and potential leakage can be visualized from the left atrium then. In cases of
concomitant aortic valve replacement with complete excision of the aortic valve, a Foley catheter is placed into the
aortic valve orifice b. After inflating the balloon, cardioplegic solution can be administered via the catheter in a similar
fashion
mimic a systolic position. At this point, the valve testing methods may fail because they are static
should be competent along the whole coaptation and do not evaluate valve function during regular
line. According to Carpentier, the coaptation line cardiac cycle.
looks like «a smiling mouth». Therefore, definitive testing of repair can only
Hetzer has described a reliable and reproduc- be achieved with a beating heart employing trans-
ible technique to test mitral valve competency by esophageal echocardiography. This method, how-
placing a small catheter (12 Ch) through the inser- ever, requires termination of cardioplegic arrest
tion of the antegrade cardioplegia line and through and rewarming of the patient to allow at least par-
the aortic valve into the left ventricle. Cardioplegic tial weaning off bypass. Nevertheless, echocar-
solution is then administered into the ventricle diography can be performed with the cannulas in
with a maximum pressure of 40–50 mmHg to place and allows immediate reassessment for fur-
avoid distension of the myocardium. With this ther reconstruction or valve replacement in the
technique, valve closure and leaflet coaptation can absence of satisfactory repair. With consequent
be controlled easily. In cases of concomitant aortic utilization of intraoperative echocardiography, we
valve replacement, this methodology has been have not experienced relevant postoperative
modified by placing a urine catheter (Foley cathe- mitral regurgitation in our practice.
ter) in the left ventricular outflow tract through
the aortotomy and blocking the catheter at the
level of the aortic anulus after excising the cusps. 25.6 Tricuspid Valve Procedures
Saline can then be instilled into the left ventricle
accordingly (Borst et al. 1991). These methods The tricuspid valve separates the right atrium
may work in most instances. In patients with func- from the right ventricle. As the tricuspid valve is
tional mitral regurgitation such as restricted leaf- only prone to low mechanical stress, isolated
lets in ischemic cardiomyopathy, however, these acquired diseases of the tricuspid valve are rela-
Chapter 25 · Surgery for Acquired AV-Valve Diseases
825 25
tively rare. Most surgical procedures of the tricus- tion of the Eustachian valve and the Thebesian
pid valve are necessary for secondary tricuspid valve), the septal leaflet of the tricuspid valve,
valve regurgitation in the setting of chronic mitral and the orifice of the coronary sinus. The AV
valve disease and pulmonary hypertension. In nodal bundle is located near the apex of the tri-
these cases, the native tricuspid valve and its sub- angle of Koch in the region of the membranous
valvular apparatus are commonly intact, while the septum, and thus suture placement in this area
tricuspid anulus is severely dilated. After years of should be avoided.
debate whether to address tricuspid valve regurgi-
tation during mitral surgery or not, there is mean-
while clear evidence that simultaneous repair is 25.6.2 Tricuspid Valve Replacement
beneficial for patients with combined mitral and
tricuspid regurgitation (Dreyfus et al. 1990). As a As mentioned before, organic tricuspid patholo-
rapid and relatively simple procedure, we liberally gies are rare, and therefore tricuspid valve replace-
perform additional tricuspid anuloplasty in ment is rarely necessary. In cases of tricuspid
patients undergoing mitral valve surgery, when stenosis due to rheumatic disease or infective
more than moderate tricuspid regurgitation or a endocarditis with valve destruction without repair
dilated tricuspid anulus is present. This approach option, we replace the tricuspid valve following the
is based on the observation that tricuspid regurgi- same principles as described for mitral valve
tation does not recover even many years following replacement including preservation of the subval-
successful mitral valve repair or replacement vular apparatus. Even in cases of severe stenosis,
(Bianchi et al. 2009). mobilization of the subvalvular apparatus can be
Nowadays, at least in industrialized countries, achieved by commissurotomy allowing implanta-
organic tricuspid valve stenosis or combined tion of a biological prosthesis or a low-profile
pathologies due to rheumatic disease have become mechanical valve.
a rare entity. However, reconstruction can often It is important to mention that the U-sutures
be achieved by commissurotomy or anuloplasty. that are placed in the same manner than in mitral
Infective endocarditis involving the tricuspid valve replacement in the area of the anterior and
valve on the other hand is more common and posterior portion of the anulus have to be placed
increasingly seen in patients with immunosup- through the base of the septal leaflet instead of the
pression and more important chronic i.v. drug septal portion of the anulus to avoid impairment
abuse (Shatapathy et al. 2000). of the conduction system. When the leaflets need
to be excised, at least a margin of the septal leaflet
should stay in place. Nevertheless, complete AV
25.6.1 Functional Anatomy block is frequently associated with tricuspid valve
replacement.
The tricuspid valve consists of three leaflets, the If a mechanical prosthesis needs to be
anterior, posterior, and septal leaflet. The anterior implanted, one should keep in mind that thereaf-
leaflet has the largest surface area, whereas the ter placement of a Swan-Ganz catheter or more
posterior leaflet is the smallest. The septal leaflet is important implantation of transvenous pace-
attached to the fibrous part of the heart. The leaf- maker electrodes is not possible. If a tissue valve is
lets are separated by three commissures (antero- chosen, the prosthesis should be orientated in the
posterior, posteroseptal, anteroseptal). A large way that the struts are not directed towards the
anterolateral papillary muscle originates from the anterior portion of the anulus because this may
free right ventricular wall and sends chordae to cause partial outflow tract occlusion. In our opin-
the anterior and posterior leaflets, whereas a ion and as advocated by other groups, in most
smaller posteromedial papillary muscle originates cases tricuspid valve replacement is performed
from the septum and is attached to the septal leaf- with a commercially available biological mitral
let. The septal leaflet has also chordae that are valve prosthesis (Filsoufi et al. 2005). The lower
directly attached to the septal wall. mechanical burden in tricuspid position leads to a
The triangle of Koch is an important ana- significantly lower rate and later onset of
tomical landmark defined by the tendon of prosthetic biological valve degeneration com-
Todaro (a fibrous structure formed by the junc- pared to mitral or aortic position.
826 P.B. Rahmanian and T.C.W. Wahlers
25.6.3 Treatment of Tricuspid posteroseptal commissure, the sutures are stitched
Anular Dilation through the outside of the atrial wall and secured
with another Teflon pledget. When tying the
The different approaches for tricuspid valve sutures, the remaining orifice should allow for two
repair follow the same rules described for mitral of the surgeon’s fingers to be introduced. A better
valve repair. The goal is to downsize the tricuspid estimation of the remaining orifice can be achieved
25 anulus in the anterior and posterior portion while with a valve sizer; a number 27 (mm) sizer of any
keeping the geometry of the septal part of the manufacturer is recommended. With these mea-
anulus intact to avoid compromise of the conduc- sures, the anular diameter is reduced to a third,
tion system. The orifice area is commonly reduced and the orifice is downsized to about 4 cm2 (De
to 3–4 cm2. Paulis et al. 1990). Alternatively, the atrium can be
closed first. The suture lines are then tied from out-
25.6.3.1 De Vega Anulorrhaphy side the atrium after weaning off cardiopulmonary
The de Vega anuloplasty shortens the anular cir- bypass under echocardiographic control until the
cumference at the base of the anterior and poste- tricuspid regurgitation disappears.
rior leaflets (. Fig. 25.22). We usually perform this One major drawback of this simple technique
technique with a double row of a Teflon-pledgeted is the potential risk of a suture tear out due to high
semicircular 4-0 polypropylene suture beginning tension on the anulus. This can be avoided by
at the anterior trigone and running along the anu- stitching the second suture line in an overcast
lus of the base of the anterior and posterior leaflets. fashion with a supporting pericardial strip. Also
The two suture lines are placed parallel but in an the long-term results are not comparable to ring
alternative sequence to ensure homogeneous nar- reduction plasties, as it was shown in the later
rowing of the tricuspid anulus. In the area of the years.
..Fig. 25.22 Tricuspid valve anuloplasty for tricuspid regurgitation according to de Vega. The technique employs a
double-lined suture in the anular segments of the anterior and posterior leaflet running from the posteroseptal
commissure to the anteroseptal commissure and back with a pledget at the turning point at the anteroseptal
commissure. The suture is then passed through a second pledget at the posteroseptal commissure. The suture is
tightened, producing a purse-string effect resulting in a length reduction of the anterior and posterior anulus segments
providing a broader leaflet coaptation. If the suture is passed through the atrial wall at the posteroseptal commissure,
final tightening can be performed after closure of the atriotomy and weaning off bypass under digital and/or
echocardiographic control
Chapter 25 · Surgery for Acquired AV-Valve Diseases
827 25
25.6.3.2 Tricuspid Anuloplasty also tend to pick a smaller ring if a measurement
Today, the de Vega plasty has been replaced by was in between sizes available. Ring implanta-
ring anuloplasty in most cases. As for mitral tion is performed by placing a series of eight to
anuloplasty, several rings and bands especially ten simple 2-0 Polyester mattress sutures through
designed for the tricuspid valve are available. We the tricuspid anulus around the orifice. As previ-
prefer a three-dimensional ring that mimics the ously mentioned, particular attention needs to
shape of the tricuspid anulus. Alternatively, con- be directed to avoid injury of the intracardiac
ventional semirigid rings can be used. In these conduction system. As described above, the
cases, we bend the septal portion slightly inward atrioventricular node is located in the area of the
to create a three-dimensional shape which mim- membranous septum at the apex of the triangle
ics the anular direction along the membranous of Koch. This area is marked by placing the first
septum. Sizing of the tricuspid valve is based on two sutures on either side of this area. The
two measurements: the intercommissural dis- remaining sutures are then placed circumferen-
tance of the septal leaflet and the surface area of tially around the anulus. The atrio-anular junc-
the anterior and posterior leaflets. First the tion is identified by pulling down the anterior
intercommissural (intertrigonal) distance of the and posterior leaflets, respectively. The stitches
septal leaflet is measured. Using a valve hook, are placed with wide bites in a 1 mm distance
primary chordae of the anterior and posterior from this atrio-anular junction and directed
leaflets arising from the anterior papillary mus- towards the ventricle (. Fig. 25.23). Overlapping
cle are secured at the margin of the leaflets and of the sutures and smaller corresponding bites
pulled down to expose the surface area of these through the ring particularly at the posterior
two leaflets. The surface area of the anterior and leaflet produces a measured anular plication in
posterior leaflet is measured using the plane of this region of maximal dilatation. Each arm of
the sizer. If the intercommissural distance and the sutures is then passed through the sewing
surface area measurement result in different ring of the prosthesis. Next, the ring is lowered
sizes, the ring size is chosen in favor of the mea- into position and tied securely to the anulus. A
sured surface area of the leaflets. In general, we final intraoperative test by injecting normal
..Fig. 25.23 Tricuspid valve anuloplasty with an open ring. The ring is fixated with horizontal single mattress sutures
avoiding injury of the bundle of His in the septal portion of the anulus
828 P.B. Rahmanian and T.C.W. Wahlers
saline into the right ventricle should show a Chitwood WR Jr (1998) Mitral valve repair: an odyssey to
competent valve with a good amount of coapta- save the valves! J Heart Valve Dis 7(3):255–261
Chowdhury UK, Kumar AS et al. (2005) Mitral valve replace-
tion in between all three leaflets. ment with and without chordal preservation in a rheu-
matic population: serial echocardiographic assessment
25.6.3.3 Bicuspidalization of left ventricular size and function. Ann Thorac Surg
79(6):1926–1933
25 The bicuspidalization technique as described by Cohn LH (1994) Elliot Carr Cutler mitral valve surgery at
Kay et al. lowers the dimension of the posterior Peter Bent Brigham Hospital 1923. J Card Surg 9(2
anulus by plicating the posterior leaflet towards Suppl):137–138
the ventricle (Kay 1992). In our opinion, this Comas GM, Widmann WD et al. (2006) The legacy of Sir
technique should only be used occasionally in Henry Souttar: pioneer of the first mitral valvulotomy.
Curr Surg 63(6):476–481
patients with mild tricuspid regurgitation or anu- Daimon M, Fukuda S et al. (2006) Mitral valve repair with
lar dilatation or in cases of endocarditis, when Carpentier-McCarthy-Adams IMR ETlogix annulo-
mainly the posterior leaflet is affected. plasty ring for ischemic mitral regurgitation: early
echocardiographic results from a multi-center study.
Circulation 114(1 Suppl):I588–I593
David TE (1987) Left ventricular rupture after mitral valve
References replacement: endocardial repair with pericardial
patch. J Thorac Cardiovasc Surg 93(6):935–936
Adams DH, Anyanwu AC et al. (2006) Large annuloplasty David TE, Kuo J et al. (1997) Aortic and mitral valve replace-
rings facilitate mitral valve repair in Barlow’s disease. ment with reconstruction of the intervalvular fibrous
Ann Thorac Surg 82(6):2096–2100, discussion 2101 body. J Thorac Cardiovasc Surg 114(5):766–771, discus-
Adams DH, Rosenhek R et al. (2010) Degenerative mitral sion 771–762
valve regurgitation: best practice revolution. Eur Heart de Kerchove L, Vanoverschelde JL et al. (2007) Reconstructive
J 31(16):1958–1966 surgery in active mitral valve endocarditis: feasibility,
Alexiou C, Langley SM et al. (2000) Surgical treatment of safety and durability. Eur J Cardiothorac Surg: Off J Eur
infective mitral valve endocarditis: predictors of early Assoc Cardiothorac Surg 31(4):592–599
and late outcome. J Heart Valve Dis 9(3):327–334 De Paulis R, Bobbio M et al. (1990) The De Vega tricuspid
Anyanwu AC, Adams DH (2007) Etiologic classification of annuloplasty. Perioperative mortality and long term
degenerative mitral valve disease: Barlow’s disease and follow-up. J Cardiothorac Surg (Torino) 31(4):
fibroelastic deficiency. Semin Thorac Cardiovasc Surg 512–517
19(2):90–96 Deloche A, Acar C et al. (1990) Biatrial transseptal approach
Anyanwu A, Rahmanian PB et al. (2006) The pathophysiol- in case of difficult exposure to the mitral valve. Ann
ogy of ischemic mitral regurgitation: implications for Thorac Surg 50(2):318–319
surgical and percutaneous intervention. J Interv Deniz H, Sokullu O et al. (2008) Risk factors for posterior
Cardiol 19(s5):S78–S86 ventricular rupture after mitral valve replacement:
Bianchi G, Solinas M et al. (2009) Which patient undergoing results of 2560 patients. Eur J Cardiothorac Surg
mitral valve surgery should also have the tricuspid 34(4):780–784
repair? Interact Cardiovasc Thorac Surg 9(6):1009–1020 Dreyfus G, Serraf A et al. (1990) Valve repair in acute endo-
Borst HG, Klinner W et al. (1991) Herzchirurgie: Die Eingriffe am carditis. Ann Thorac Surg 49(5):706–711; discussion
Herzen und an den herznahen Gefäße. Springer, Berlin 712–713
Brawley RK (1980) Improved exposure of the mitral valve in Dubost C, Guilmet D et al. (1966) New technic of opening
patients with a small left atrium. Ann Thorac Surg of the left auricle in open-heart surgery: the trans-
29(2):179–181 septal bi-auricular approach. Presse Med 74(30):
Carpentier A (1977) Mitral valve reconstructive surgery. In: 1607–1608
Jamieson SW, Shumway NE (eds) Operative surgery. Fasol R, Lakew F et al. (2000) Papillary muscle repair sur-
Butterworth, London gery in ischemic mitral valve patients. Ann Thorac
Carpentier A (1983) Cardiac valve surgery – the “French Surg 70(3):771–776; discussion 776–777
correction”. J Thorac Cardiovasc Surg 86(3):323–337 Filsoufi F, Carpentier A (2007) Principles of reconstructive
Carpentier A, Deloche A et al. (1971) A new reconstructive surgery in degenerative mitral valve disease. Semin
operation for correction of mitral and tricuspid insuf- Thorac Cardiovasc Surg 19(2):103–110
ficiency. J Thorac Cardiovasc Surg 61(1):1–13 Filsoufi F, Anyanwu AC et al. (2005) Long-term outcomes of
Casselman FP, Gillinov AM et al. (1999) Use of the anterior tricuspid valve replacement in the current era. Ann
mitral leaflet to reinforce the posterior mitral anulus after Thorac Surg 80(3):845–850
debridement of calcium. Ann Thorac Surg 68(1):261–262 Filsoufi F, Rahmanian PB et al. (2006) Physiologic basis for
Chen SX, Xi EP et al. (2003) Preservation of the entire mitral the surgical treatment of ischemic mitral regurgita-
subvalvular apparatus during mitral valve replace- tion. Am Heart Hosp J 4(4):261–268
ment in patients with mitral stenosis. Hunan Yi Ke Da Gao G, Wu Y et al. (2004) Forty-year survival with the Starr-
Xue Xue Bao Hunan Yike Daxue Xuebao Bull Hunan Edwards heart valve prosthesis. J Heart Valve Dis
Med Univ 28(6):615–618 13(1):91–96; discussion 96
Chapter 25 · Surgery for Acquired AV-Valve Diseases
829 25
Gillinov AM, Cosgrove DM 3rd (2001) Modified quadrangu- Risteski PS, Aybek T et al. (2007) Artificial chordae for mitral
lar resection for mitral valve repair. Ann Thorac Surg valve repair: mid-term clinical and echocardiographic
72(6):2153–2154 results. Thorac Cardiovasc Surg 55(4):239–244
Guiraudon GM, Ofiesh JG et al. (1991) Extended vertical Rodriguez RA, Cornel G et al. (2001) Effect of trendelen-
transatrial septal approach to the mitral valve. Ann burg head position during cardiac deairing on cere-
Thorac Surg 52(5):1058–1060; discussion 1060–1062 bral microemboli in children: a randomized controlled
Iida H, Mochizuki Y et al. (2005) A valve replacement tech- trial. J Thorac Cardiovasc Surg 121(1):3–9
nique for heavily calcified mitral valve and anulus. Rodriguez F, Langer F et al. (2004) Importance of mitral
J Heart Valve Dis 14(2):209–211 valve second-order chordae for left ventricular geom-
Isomura T, Hisatomi K et al. (1993) Left atrial plication and etry, wall thickening mechanics, and global systolic
mitral valve replacement for giant left atrium accom- function. Circulation 110(11 Suppl 1):II115–II122
panying mitral lesion. J Card Surg 8(3):365–370 Sarsam MA (2002) Simplified technique for determining
Jouan J, Tapia M et al. (2004) Ischemic mitral valve pro- the length of artificial chordae in mitral valve repair.
lapse: mechanisms and implications for valve repair. Ann Thorac Surg 73(5):1659–1660
Eur J Cardiothorac Surg: Off J Eur Assoc Cardiothorac Sasaki H, Ihashi K (2003) Chordal-sparing mitral valve
Surg 26(6):1112–1117 replacement: pitfalls and techniques to prevent com-
Kay JH (1992) Surgical treatment of tricuspid regurgita- plications. Eur J Cardiothorac Surg Off J Eur Assoc
tion. Ann Thorac Surg 53(6):1132–1133 Cardiothorac Surg 24(4):650–652
Laub GW, Muralidharan S et al. (1992) The experimental Shatapathy P, Aggarwal BK et al. (2000) Tricuspid valve repair:
relationship between leaflet clearance and orientation a rational alternative. J Heart Valve Dis 9(2):276–282
of the St. Jude Medical valve in the mitral position. Smedira NG, Selman R et al. (1996) Repair of anterior leaflet
J Thorac Cardiovasc Surg 103(4):638–641 prolapse: chordal transfer is superior to chordal short-
Lillehei CW, Levy MJ et al. (1964) Mitral valve replacement ening. J Thorac Cardiovasc Surg 112(2):287–291, dis-
with preservation of papillary muscles and chordae cussion 291–282
tendineae. J Thorac Cardiovasc Surg 47:532–543 Sondergaard T, Gotzsche H et al. (1955) Surgical closure of
Lim E, Ali ZA et al. (2002) Determinants and assessment of interatrial septal defects by circumclusion. Acta Chir
regurgitation after mitral valve repair. J Thorac Scand 109(3–4):188–196
Cardiovasc Surg 124(5):911–917 Sternik L, Zehr KJ et al. (2002) The advantage of repair of
Maisano F, Torracca L et al. (1998) The edge-to-edge mitral valve in acute endocarditis. J Heart Valve Dis
technique: a simplified method to correct mitral
11(1):91–97; discussion 97–98
insufficiency. Eur J Cardiothorac Surg Off J Eur Wippermann J, Albes JM et al. (2005) Three years’ experi-
Assoc Cardiothorac Surg 13(3):240–245; discussion ence with the on-x conform-X bileaflet prosthesis for
245–246 ‘atrialized’ mitral valve replacement: a preliminary
Murashita T, Sugiki H et al. (2004) Surgical results for report. J Heart Valve Dis 14(5):637–643
active endocarditis with prosthetic valve replacement: Yilmaz AT, Oz BS et al. (2005) Preservation of anterior leaf-
impact of culture-negative endocarditis on early and let chordae with modified miki technique: how to do
late outcomes. Eur J Cardiothorac Surg: Off J Eur Assoc it. J Card Surg 20(1):70–72
Cardiothorac Surg 26(6):1104–1111 Yun KL, Sintek CF et al. (2002) Randomized trial comparing
Mylonakis E, Calderwood SB (2001) Infective endocarditis partial versus complete chordal-sparing mitral valve
in adults. N Engl J Med 345(18):1318–1330 replacement: effects on left ventricular volume and
Piper C, Hetzer R et al. (2002) The importance of secondary function. J Thorac Cardiovasc Surg 123(4):707–714
mitral valve involvement in primary aortic valve endo- Zegdi R, Debieche M et al. (2005) Long-term results of
carditis; the mitral kissing vegetation. Eur Heart mitral valve repair in active endocarditis. Circulation
J 23(1):79–86 111(19):2532–2536
831 26
Minimally Invasive
Cardiac Surgery
Jens Garbade, Sreekumar Subramanian,
and Friedrich-Wilhelm Mohr
References – 859
Chapter 26 · Minimally Invasive Cardiac Surgery
833 26
26.1 Introduction 3. An increase in combined procedures (i.e.,
multivalve, CABG procedures, etc.).
The development of minimally invasive and alterna- 4. The proportion of patients requiring a
tive operative techniques has gained significant reoperation is rising dramatically.
importance in modern cardiac surgery. The goal is
not only to establish better tolerated and more effec- The following chapter should provide an
tive surgical techniques but also more efficient man- verview of minimally invasive and alternative
o
agement algorithms. Central aims in modern operative methods and concepts, illustrate the
minimally invasive cardiac surgery are to minimize advantages and disadvantages of different
size of thoracic surgical access incisions, reduce techniques, depict the use of video-assisted proce-
immunologic trauma, prevent myocardial ischemia, dures, and finally present perspectives on
and maintain pulsatile systemic blood flow (i.e., myo- telemanipulator-assisted surgery.
cardial and cerebral). To achieve these aims, interdis-
ciplinary communication and the development of
common multidisciplinary therapeutic strategies are 26.2 The Development of Minimally
mandatory. This strategic approach represents an Invasive Procedures in Cardiac
important part of modern cardiac surgery. Surgery
Fundamentally, the term minimally invasive
cardiac surgery refers to the combined effort to 26.2.1 Requirements, Problems,
reduce trauma, operative risk, and thereby mor- and Challenges
bidity and mortality of conventional cardiac sur-
gical procedures. Overall, these patients should With the goal of optimal exposure, taking ana-
expect a quicker convalescence and rehabilitation. tomical factors and pathological complexity into
Factors which result in more efficient pre- and account, most cardiac surgical procedures are car-
postoperative in patient and postoperative ambu- ried out through the conventional approach of
latory care ultimately lead to a shorter hospital complete median sternotomy.
stay and reduce hospital costs, thereby achieving To reduce the invasiveness of this procedure,
more optimal resource utilization. These are ancil- various aspects of the conventional approach may
lary expectations of alternative operative strate- be altered:
gies. While cosmetic outcomes may be considered 55 Minimize the operative approach.
in the planning of cardiac surgical procedures, 55 Reduce the length of the incision.
they should come second to the central aims of 55 Avoid cardiopulmonary bypass and operate
minimal invasive cardiac surgery as outlined on the beating heart.
above. Due to more effective medical and inter- 55 Combine surgical and interventional
ventional therapies, conventional cardiac surgery strategies (hybrid procedures).
is being confronted by progressively increasing
cardiovascular disease in an aging population. We In order to do so, various strategies and tech-
must advance to meet these challenges. niques have been described:
In summary, today’s demands on cardiac sur- 55 Partial sternotomy in different fashions
gery relate to an increasing risk profile of the 55 Parasternal and thoracoscopic approaches
entire patient population. These demands are sig- 55 Lateral thoracotomy
nificantly determined by the following factors: 55 Mini-thoracotomy
1. The age of the patients who are undergoing a 55 Subxiphoid and subdiaphragmatic accesses
primary or secondary cardiac operation is
rising (2011, 13.4 % of patients were older than
80 compared to 2010, 12.4 %; 2006, 9.6 %; and The combination of a limited surgical
2005, 8.4 %) (Funkat et al. 2012; Gummert et al. approach with catheter-based intervention
2007). (hybrid strategy) represents a valuable
2. The severity of the basic cardiac illness is alternative option in highly selected patients
more complex, primarily because of comorbid and can lead to a significant further reduction
illnesses, often due to age, and prior in invasiveness (Shannon et al. 2012).
interventional procedures.
834 J. Garbade et al.
However, problems inherent to minimization including the endoclamp technique, was carried
of surgical access may occur in the presence of a out using a left anterior mini-thoracotomy on the
rigid bony thorax leading to limited exposure. Or arrested heart (Reichenspurner et al. 1998a, b.
the necessity of multiple smaller incisions to place However, for the correct positioning of
additional instruments may lead to an increase in the HeartPort system, a sufficient transesophageal
postoperative pain. echocardiography imaging is absolutely
Challenges in surgery on the beating heart, mandatory.
besides the moving target, include the hemody- As a result of a variety of reported complica-
26 namic imbalances resulting from manipulation tions, this technique is used almost exclusively now
and luxation of the blood-filled heart. As a result, in minimally invasive mitral valve surgery, telema-
an array of techniques and systems, such as video- nipulator-assisted coronary artery bypass surgery
assisted presentation, CO2 insufflation, flexible or in rare cases of conventional cardiac reopera-
mechanical stabilizers, mister-blowers, and coro- tions, or porcelain aorta (see 7 Sect. 26.2.3,
nary shunts, have been developed and represent «HeartPort Technique»).
part of the routine practice of minimally invasive With the introduction of direct coronary artery
cardiac surgery. revascularization through a limited thoracotomy
The first clinically relevant developments access such as the left anterolateral thoracotomy or
began in the early 1990s on the basis of laparo- the subxiphoid approach using video-assisted IMA
scopic techniques from general surgery. preparation on the beating heart and the deploy-
Therapeutic thoracoscopy or video-assisted tho- ment of specially developed stabilizers and instru-
racic surgery (VATS) became more commonplace ments (Reichenspurner et al. 1998a, b), new
(Mack et al. 1992). Currently, VATS is the pre- operative techniques could be transferred into
ferred worldwide approach to a variety of surgical clinical practice. Furthermore, these techniques
procedures, including more complex thoracic could be used in patients with high operative risk,
operations such as lobectomy and esophagectomy either for single- or multivessel coronary artery
(Landreneau et al. 1992). disease in combination with interventional proce-
dures as a hybrid procedure (Holzhey et al. 2012;
Shannon et al. 2012).
26.2.2 Fundamental Concepts Beating heart coronary artery revasculariza-
of Minimally Invasive Cardiac tion via a complete or partial sternotomy or vari-
Surgery ous thoracic surgical approaches can be carried
out with (on-pump beating heart) or without
In the 1990s, three significant developments led to extracorporeal circulation («off-pump»). This
a significant reduction of invasiveness in cardiac technique first became possible through the
surgery: development of special instruments and heart sta-
55 The HeartPort system bilizers, which enabled an optimal exposure of the
55 Reduced size of the surgical approaches corresponding coronary vessels.
55 Beating heart surgery Different algorithms were developed to
achieve coronary anastomoses, such as insertion
The HeartPort system, a cannulation and cath- of a shunt or application of a special blower. The
eter system developed in Stanford, CA, was first introduction of distal and proximal anastomotic
applied to carry out a LIMA-LAD anastomosis connectors facilitates these techniques.
during a coronary bypass operation after conven- Particularly with off-pump coronary bypass
tional sternotomy Woo et al. (2006). Using this surgery, effective collaboration with cardiac anes-
system, cardioplegic arrest was achieved through thesia is indispensable. This is the only way stable
inflation of an endovascular balloon (so-called hemodynamics and patient security can be guar-
endoclamp technique) in the ascending aorta with anteed. This technique is presently applied to over
subsequent administration of cardioplegia into 25 % of all coronary artery bypass operations in
the aortic root. The balloon itself was inserted via the USA. The effectiveness and the superiority as
the femoral artery and advanced into the ascend- compared to conventional coronary artery bypass
ing aorta just above the coronary arteries. As a fur- surgery with respect to blood loss, renal failure,
ther development, the LIMA-LAD operation, intensive care unit, and hospital length of stay
Chapter 26 · Minimally Invasive Cardiac Surgery
835 26
have already been demonstrated (Puskas et al. measurement of the right radial artery are essen-
2005). The proof of superior survival has not been tial for early recognition of balloon dislocation
shown. However, the recently published data and other complications (Siegel et al. 1997). The
coming from the GOPCABE study showed that HeartPort technique was initially used and evalu-
there is no significant difference between on- ated for minimally invasive coronary artery
pump versus off-pump CABG in the older popu- bypass surgery (Grossi et al. 1999; Subramanian
lation (age >75) with regard to death, stroke, et al. 1997). Further applications have been
myocardial infarction, or repeat vascularization described in aortic valve surgery (Wheatley et al.
up to 1 year after surgery (Diegeler et al. 2013). 2004), congenital heart surgery (De Mulder and
A direct comparison to interventional strate- Vanermen 2002; Yamada et al. 2000), reopera-
gies is presently the object of multiple clinical tions, and minimally invasive mitral valve surgery
studies. The 10-year follow-up from our random- (Mohr et al. 1998; Ricci et al. 2010; Meyer et al.
ized study comparing bare-metal stenting with 2009; Casselman et al. 2007a, b).
MIDCAB observed similar outcomes regarding Due to various reported complications with
composite clinical end points. Nonetheless, the this technique, indications have been severely
MIDCAB procedure was associated with more restricted. Possible complications include injury
incompleteness of coronary revascularization to the iliac vessels, dissection of the femoral ves-
(Blazek et al. 2013). sels, injuries to the ascending aorta and aortic root
In our own analyses for patients with reduced including type A acute aortic dissection, trau-
LV function and increased operative risks, the matic aortic insufficiency, incomplete cardiople-
«beating-heart» technique is superior to conven- gia administration through displacement of one
tional bypass surgery with extracorporeal circula- or both coronary ostiae with resulting inadequate
tion avoiding cross-clamping especially in myocardial protection, migration of the catheter
emergency operations (Rastan et al. 2006). and occlusion of the brachiocephalic trunk with
resulting cerebral ischemia, and incomplete deair-
ing after opening the cardiac chambers (Muhs
26.2.3 HeartPort Technique et al. 2005; Wimmer-Greinecker et al. 1999).
Exclusion criteria for the application of the
The HeartPort system, a cannulation and catheter HeartPort technique are advanced peripheral
system developed at Stanford University, CA, was vascular disease, arteriosclerotic or aneurysmal
designed to enable minimally invasive and changes of the thoracic aorta as well as aortic
endoscopic cardiac operations (CABG and mitral insufficiency, and mitral valve calcification.
valve) by providing endoluminal occlusion of the The endoluminal clamp technique enables the
ascending aorta (Stevens et al. 1996a, b; Pompili performance of minimally invasive techniques,
et al. 1996). The catheter system can be intro- but has no significant advantages over the direct
duced percutaneously via the femoral vessels. The clamp technique developed by Chitwood for
distal end consists of a balloon, which is advanced mitral valve surgery (Dogan et al. 2005;
and placed into the ascending aorta, above the Reichenspurner et al. 2005). As a routine method
aortic valve and proximal to the brachiocephalic for elective cases, this technique has not found
trunk. After the balloon is inflated with normal broad clinical application. Several authors report
saline, a temporary endoluminal occlusion of the on its use with TECAB (totally endoscopic coro-
aorta is achieved. Finally, through the same cath- nary artery bypass) procedures (Subramanian
eter system, cardioplegia can be administered into et al. 2005; Bonatti et al. 2007) and during reop-
the aortic root. Thereafter the same catheters may erations, but definitive data is lacking.
be used for drainage also. Through the jugular
vein, an additional catheter can be placed into the
pulmonary artery for drainage also. Furthermore 26.3 Current Clinical Concepts
a transvenous catheter can be placed into the cor-
onary sinus for retrograde cardioplegia adminis- 26.3.1 General Principles
tration. To ensure secure placement and
intraoperative steering of the catheter system, Median sternotomy allows for any type of surgery
TEE and an additional invasive blood pressure for all cardiac and great vessel pathologies where
836 J. Garbade et al.
surgical treatment is indicated. The excellent Sect. 26.3.6.1, «Alternative Incisions for
exposure of the thoracic anatomical structures Revascularization»).
comes at the expense of a major surgical trauma. As innovative techniques, endoscopic and
This can lead to excessive mechanical strain and telemanipulator-assisted methods have been
produce an injury of the bordering costochondral evaluated in CABG, valve surgery, repair of
joints or thoracic musculature. Patients may expe- congenital defects, and the therapy of atrial
rience pronounced postoperative pains. This sen- fibrillation. A radically new approach is beating
sitivity to pain can result in a limitation of breathing heart percutaneous therapy of various native and
26 mechanics and limit postoperative mobilization. prosthetic heart valve pathologies ranging from
The incidence of postoperative infection and aortic, mitral, and pulmonary stenosis or
mediastinitis reported to be as high as 2–6 % in the regurgitation to prosthetic valve dysfunction.
literature, with consequent additional surgical The development of new cannula systems
procedures and wound therapies, represents com- enables safe and reliable connection for
plications that should not be underestimated extracorporeal circulation via the femoral,
(Gummert et al. 2002; Gorlitzer et al. 2013). axillary, and neck blood vessels. Furthermore,
In particular for patients with a high endoscopic harvest techniques for bypass
EuroSCORE (see also 7 Chapter «Risk Scores in conduits, in particular saphenous vein and radial
Cardiac Surgery», Sect. 2.2.2), diabetes mellitus, artery, have become increasingly important.
COPD, and an elevated body mass index (BMI), Alternative surgical access for the specific
there is an increased risk of developing such pathologies of the heart will be presented in
wound complications. more detail in the ensuing paragraphs.
Since the early days of cardiac surgery, lateral 2. A second strategy is the reduction or
thoracotomy has been a routine procedure. This elimination of extracorporeal circulation.
led to its use for mitral and tricuspid valve sur- Nowadays, many coronary surgical
gery, ASD closure or treatment of congenital procedures can be carried out on the beating
descending aortic lesions. These full-thoracotomy heart without the heart-lung machine
procedures still represent a significant trauma and (HLM). Despite a conventional sternotomy,
are often associated with rib injuries and signifi- one should still regard this approach as a
cant acute and chronic postoperative pain. minimally invasive procedure. Even
Isolated encapsulated pleural effusions and lung operations such as valvular surgery or left
adhesions are not uncommon and complicate the ventricular reconstruction can be carried out
use of the lateral thoracotomy. As a result, alterna- with a beating heart technique on
tive surgical access and techniques have been extracorporeal circulation, whereby the
evaluated, whereby each approach is concentrated avoidance of cardiac ischemia leads to a
to particular anatomic structures and a specific reduction of cellular trauma. In addition, the
operative strategy. miniaturization of the heart-lung machine in
Presently, four essential strategies have been adults, as well as children, reduces the
established as routine approaches, with validation immunologic trauma. From a systemic view,
in an array of clinical studies: adding a sternotomy to a procedure is rather
1. A reduction of the size of the surgical access less invasive than adding extracorporeal
and a corresponding reduction in the surgical circulation. Therefore, employing sternotomy
trauma are of major significance. From in order to avoid cardiopulmonary bypass is
valvular surgery to repair of congenital defects a way to minimize surgical trauma.
like atrial septal defect, as well as in coronary 3. A third building block is arterial, especially
artery bypass surgery, alternative approaches aortic endografting. In increasing
like the mini-sternotomy, the left or right proportions, chronic, as well as acute,
anterior mini-thoracotomy, or the pathologies such as dissection and
subxiphoidal thoracotomy have become estab- aneurysms (including ruptured aneurysms)
lished as possible minimally invasive surgical of the thoracic and abdominal aorta are
approaches (see 7 Sect. 26.3.2.2, «Atrial Septal treated successfully with endoluminal stent
Defect Closure»; Sect.26.3.4, «Minimally Invasive graft implantation. Even dissections in the
Aortic Valve Surgery»; Sect. 26.3.5, «Minimally region of the aortic arch are being treated
Invasive Mitral and Tricuspid Valve Surgery»; with stents that cover the supra-aortic
Chapter 26 · Minimally Invasive Cardiac Surgery
837 26
vessels, and the supra-aortic vessels are preferred approach for PDA closure (Abu Hazeem
treated with separate extra-anatomic bypass et al. 2013). Interestingly, the technique was first
from the ascending aorta. successfully carried out in 1971 by Porstmann
4. This concept leads to a fourth important and colleagues (1971). Presently, different systems
strategy in minimally invasive cardiac surgery, are available for clinical use, including a variety of
one that represents a meaningful combination coil systems or the Amplatzer occluder device.
of surgical and interventional procedures, the The efficacy and safety of this approach has been
hybrid approach. This technique places new established in a large study not only in children
demands on the entire team and requires a but also in small infants and premature babies
redefinition of the equipment and instrumenta- (Sungur et al. 2013). Importantly, this procedure
tion found in the classic operating room with is not limited utility in very small children and
that of the requirements for a hybrid operating not limited for PDA closure in premature babies
room. While this process is presently in anymore (Abu Hazeem et al. 2013).
widespread clinical evaluation, it has already
become routine practice in many centers. 26.3.2.2 Atrial Septal Defect Closure
Both the first open-heart surgery by Lewis and his
team in 1952 and Gibbon’s first successful applica-
26.3.2 Congenital Diseases tion of his heart-lung machine in 1953 were oper-
ative closures of a secundum atrial septal defect
26.3.2.1 Patent Ductus Arteriosus (ASD II) (Gibbon 1953) (see also 7 chapter
At present, there are three different operative «Surgery for Congenital Heart Defects: A Historical
techniques for closure of a persistent patent duc- Perspective», Sect. 11.2). Since then, various meth-
tus arteriosus (PDA). ods were employed for ASD closure: median ster-
Gross described in 1938 the first successful notomy, right lateral thoracotomy (with central
ligation of a PDA in a 7-year-old girl via a left connections to the heart-lung machine), the right
fourth interspace posterolateral thoracotomy parasternal thoracotomy, the mini-sternotomy,
(Gross et al. 1939). After retraction of the lung, the subxiphoidal access, and the right mini-thora-
the vagus and phrenic nerves are exposed, and the cotomy (. Fig. 26.1) with connections to the
pleura is incised longitudinally over the aortic heart-lung machine via the femoral vessels, per-
isthmus. This procedure is associated with a pro- cutaneous endovascular occlusion techniques,
nounced surgical trauma, but offers an optimal hybrid techniques, and completely endoscopic
exposure to all anatomic structures. General com- procedures (Horvath et al. 1992; Doty et al. 1998;
plications include recurrent laryngeal nerve Doll et al. 2003; Hongxin et al. 2007; Bartakian
injury and rarely phrenic paresis or chylothorax. et al. 2012; Kotowycz et al. 2013; Liu et al. 2013).
Mortality is exceedingly rare. One requirement for surgical ASD closure is
A second approach, described in 1992 by the heart-lung machine. With respect to cannula-
Laborde, is the video-assisted thoracoscopic pro- tion, there are several choices. For one, the
cedure, which has been in use for many years. ascending aorta or the femoral artery may be can-
Using a left chest approach, two 5-mm ports are nulated. The venous drainage can be established
placed into the thorax, and videoscopic instru- via the superior and inferior vena cavae, the jugu-
ments are used to dissect out the anatomic struc- lar vein and the inferior vena cava, or via the fem-
tures and place a titanium clip on the PDA to oral vein. However, one requirement for
achieve surgical ligation. Laborde’s 12-year expe- transfemoral cannulation is a satisfactory vessel
rience (n = 743 patients) shows no difference in size, so this approach is dependent on the size and
efficacy compared to the standard open tech- age of the patient. Even complex septal patholo-
nique. There was no operative mortality, persis- gies, including sinus venosus defect or a partial
tent damage of the recurrent laryngeal nerve was AV canal, can be surgically repaired using a lateral
observed in 4.2 % of patients, and the average hos- approach but require more experience.
pital stay was 2 days. An important exclusion cri- For isolated ASD II, complete median ster-
teria for this approach was a PDA >8 mm (Laborde notomy is rarely used. As a surgical standard, the
et al. 1993; Villa et al. 2004 and 2006). right lateral mini-thoracotomy could be consid-
Concurrently, many centers have advanced to ered the standard approach (see . Fig. 26.1)
a percutaneous catheter-based technique as the (Gibbon 1953). Notwithstanding the very good
838 J. Garbade et al.
a b
26
..Fig. 26.1 Minimally invasive approach for atrial septal defect closure: a right lateral mini-thoracotomy and
b subxiphoid approach
operative results, this approach involves a cotomy (seldom used), the surgical access was via
smaller skin i ncision, reduced trauma, reduction the third or fourth interspace. The HLM was via
in postoperative pain, results in a quicker post- direct aortic and superior and inferior vena cava
operative convalescence and, in particular for cannulation (Cremer et al. 1999).
women, an excellent cosmetic result (Kotowycz Another operative method is the mini-
et al. 2013). sternotomy (hemisternotomy). It can be further
With this technique, as with minimally inva- subdivided into an upper or lower partial sternot-
sive mitral and tricuspid valve surgery, an infra- omy or include subxiphoidal access (Barbero-
mammary curvilinear skin incision is made, Marcial et al. 1998). The advantage of a partial
followed by a fourth intercostal space thoracot- upper sternotomy in ASD closure seems to be dif-
omy. All relevant anatomical structures can be ficult to understand, and a cosmetic advantage is
nicely exposed. Finally, the pericardium is rather questionable.
opened parallel and at least 2 cm superficial to With these techniques, the HLM connections
the phrenic nerve. The HLM connections are can be made via the femoral vessels, central or
made via the femoral vessels, and an additional combined, and the venous drainage can be per-
venous cannula is placed via the jugular vein. formed via the right atrial appendage and the
The insertion of additional port systems (videos- inferior vena cava. Irrespective of which form of
copy, CO2 insufflation), the cross clamping mini-sternotomy is used, authors report very
(Chitwood technique), and cardioplegia admin- good exposure, excellent operative results, and a
istration are similar to the approach for mini- high rate of patient acceptance. Which surgical
mally invasive mitral and tricuspid valve surgery. approach will be preferred remains to be seen, but
The superior and inferior vena cavae are clamped will likely remain variable from center to center.
or snared, and following cross clamping, and In 1976, King and Mills reported the first pos-
during cardioplegia administration, the right sible interventional catheter-based closure of a
atrium may be opened, and the ASD may be secundum ASD (King et al. 1976). They laid the
closed directly. foundation for a rapid and exciting development
As soon as the ASD is securely closed, the in this area. Since the 1990s, percutaneous ASD
cross clamp may be released, and the right atrial closure using various occluder systems has been
incision can be closed while the heart is beating. established as the first-line treatment in older
With the parasternal minimally invasive thora- children and adults. A randomized comparison of
Chapter 26 · Minimally Invasive Cardiac Surgery
839 26
right lateral mini-thoracotomy or other minimally The success rate was significantly better in the
invasive approaches is still required. In the single, surgical group (100 % vs. 95.7 %), with no mortal-
nonrandomized study, catheter-based occlusion ity in either group. The early and long-term results
was compared with conventional sternotomy (Du (12 months) were similar. However, the peripro-
et al. 2002). Initially, this technique was only used cedural complications in the surgical group were
with uncomplicated ASD, but in the meantime, significantly higher (24 % vs. 7.2 %), resulting in a
even large ASDs measuring up to 4 cm have been prolonged hospital stay. Based on the higher com-
successfully closed with occluder systems. plication rate, the authors concluded that inter-
As a result of dislocation, thrombosis, perfora- ventional therapies represented a superior
tion, infection, and embolic complications, sec- approach to surgical ASD closure (Du et al. 2002).
ondary surgical treatment after interventional However, there is no randomized comparison of
ASD closure may be required (Divekar et al. 2005; minimally invasive surgical approaches with
Walther et al. 2007a). interventional therapies for ASD closure. A cur-
A further minimally invasive approach is the rently published nonrandomized single-center
combination of surgical and interventional tech- study with 718 ASD closures (383 surgical and
niques without the HLM. On a beating heart, 335 transcatheter procedures) showed that the
using a right lateral mini-thoracotomy, the right long-term mortality of interventional procedure
atrium is exposed, and through its free wall, an was not inferior to surgery (5-year mortality,
occluder device is positioned under TEE guid- 5.3 % vs. 6.3 %; p = 1.00), and the secondary out-
ance. First results of this technique have been comes were similar in both groups. However, the
reported (Hongxin et al. 2007). reintervention rate was much higher in the trans-
Completely endoscopic procedures catheter group than in the surgical group
(telemanipulator-assisted techniques) with the da (Kotowycz et al. 2013).
Vinci System (Intuitive Surgical, Sunnyvale, CA, The early and long-term results following sur-
USA) on the hypothermic fibrillating heart repre- gical ASD closure are very good. Independent of
sent innovative measures, which are not widely the selected operative approach, there are no sig-
used because the instrumentation may be cum- nificant differences in mortality (<1 %) or efficacy.
bersome and expensive (Liu et al. 2013). Minimally invasive approaches reduce the surgi-
cal trauma and lead to a quicker convalescence
26.3.2.3 Results (Bartakian et al. 2012; Kotowycz et al. 2013). In a
The efficacy and safety of catheter-based ASD clo- randomized study, Luo and colleagues compared
sure has been demonstrated in an array of clinical median sternotomy with a partial lower hemister-
studies (Du et al. 2002; Hongxin et al. 2007; notomy. Both techniques had comparable safety
Bartakian et al. 2012; Kotowycz et al. 2013). and efficacy, but the minimally invasive approach
Divekar and colleagues analyzed 34,000 resulted in less pain, less postoperative bleeding, a
occluder implantations with respect to cardiac shorter hospital stay, and a better cosmetic result
complications. Twenty-nine patients experienced (Luo et al. 2001). In general, minimally invasive
cardiac complications, with five deaths and three approaches are better accepted by patients, par-
neurologic events. Perforations were noted in the ticularly in women, where the cosmetic aspect
region of the anterosuperior right atrial wall in plays a larger role.
close proximity to the aorta. The authors did not A further technique is the previously men-
offer an explanation for the localization of this tioned combination of a right lateral surgical
complication, but we believe that this may arise approach and direct placement of an occluder
from the abrasion of the implanted system with through the right atrial wall on the beating heart
the smooth-walled portion of the right atrial wall. under TEE guidance. Hongxin et al. evaluated
Overall, there was a higher incidence of second- this technique in a series of 100 patients (Hongxin
ary surgical revisions after interventional occluder et al. 2007). The size of the ASDs ranged from 5 to
implantation (Divekar et al. 2005). 37 mm. After 12 months, the authors reported no
In 2000, a nonrandomized, multicenter study mortality and complete ASD occlusion in all
compared 442 patients who underwent percuta- patients. They concluded that this method is a
neous ASD closure with 154 patients who under- feasible alternative to isolated endovascular and
went median sternotomy and direct ASD closure. surgical techniques.
840 J. Garbade et al.
Moreover, the safety and efficacy of the Initial studies show good results, particularly with
catheter-based and combined approach for ASD perimembranous VSDs (Pinto et al. 2006). In addi-
closure has been demonstrated even in children tion, hybrid procedure to cure VSDs is also of great
less than 1 year of age. clinical interest. The current published paper by
Zhu and colleagues showed that the combination
26.3.2.4 Conclusion of transcatheter VSD closure and percutaneous
The first-line treatment for ASD closure is percu- coronary intervention for treating VSD in acute
taneous placement of an occluder, which can treat myocardial infarction is also safe and a promising
26 even large secundum ASDs up to 40 mm in size. If alternative to open surgery (Zhu et al. 2013).
this modality fails or is complicated by infection,
thrombosis, emboli, or complex anatomy, surgical
treatment is indicated. The right lateral mini- 26.3.3 Pericardial Diseases
thoracotomy with femoral vessel cannulation
could be considered the standard approach, with The first time pericardial diseases, acute and
feasible alternatives including telemanipulator- chronic pericarditis, were described was in 1669
assisted (robotic) approaches or the various forms by Lower (1669). The primary surgical approach,
of mini-sternotomy. A significant aspect to con- particularly with constrictive pericarditis, was
sider is the HLM connection, which can be car- carried out in most cases via a median sternot-
ried out via the femoral vessels, centrally or a omy. In a study by Billa and colleagues, a left lat-
combination thereof. In slim patients of all ages, eral approach was shown to be a safe operative
suprainguinal cannulation of the iliac vessels is as approach for constrictive pericarditis, with simi-
easy as employing the femoral vessels, however, lar postoperative and long-term results. In the
thereby avoiding any potential lymph leaks. sternotomy group, there were more wound infec-
Moreover, in rather young patients, the external tions and pulmonary complications (Tiruvoipati
iliac vessels are of larger and therefore more suit- et al. 2003).
able size for cannulation than the femoral vessels. A further operative approach is the subxiphoi-
The role of hybrid approaches on the beating dal access (Mills et al. 1989; Becit et al. 2005).
heart and completely endoscopic procedures is Alternative procedures, in particular pericardial
not yet determined; further study is required. window for recurrent pericardial effusion, can be
carried out via a right or left lateral mini-
26.3.2.5 VSD Closure thoracotomy (Tiruvoipati et al. 2003). Video-
A left anterior parasternal mini-thoracotomy has assisted thoracoscopic surgery has taken an
been described as a minimally invasive approach increasing role in most centers, with thoracos-
to the treatment of a subarterial or perimembra- copy requiring the use of a double-lumen endo-
nous VSD. Direct access is achieved via a third or tracheal tube and special instrumentation
fourth left intercostal space approach. Videoscopic (Georghiou et al. 2005; Noyes et al. 2003).
assistance can be achieved directly or via insertion Pericardial cysts and tumors can also be resected
of port systems. Finally, the relevant anatomic thoracoscopically. Rees and colleagues reported
structures can be exposed, to include the left thoracoscopic partial pericardiectomy via a left-
phrenic nerve, and the pericardium can be longitu- sided approach (Rees et al. 1993). Case reports of
dinally incised. The HLM is connected via the telemanipulator-assisted resection of pericardial
femoral vessels. Cross-clamping is not absolutely cysts exist, but represent a significant increase in
required. The operation can be carried out using technical expertise that may not be widely avail-
mild hypothermia and ventricular fibrillation (Lin able (Bacchetta et al. 2003).
et al. 1998). Other approaches include the partial
lower (fourth intercostal space to the xiphoid) or
upper sternotomy (inverted T incision into the 26.3.4 inimally Invasive Aortic
M
fourth intercostal spaces bilaterally) to treat subar- Valve Surgery
terial, perimembranous, and muscular VSDs
(Kadner et al. 2004; Murashita et al. 1999). The standard approach to surgically treat diseases
Alternatively, percutaneous interventional proce- of the aortic valve, ascending aorta, aortic arch,
dures with the Amplatzer system are being applied. and bordering structures is the median sternotomy
Chapter 26 · Minimally Invasive Cardiac Surgery
841 26
with central connection for ECC. However, com- recluding their use for later coronary revascular-
p
plete sternotomy is not absolutely required, ization.
because the anatomic structures can be exposed Cosgrove described a right-sided parasternal
via a minimally invasive partial sternotomy or incision (see . Fig. 26.2d). This involves division
thoracotomy. This approach is sufficient to expose of ribs from one or three to five from their sternal
and cannulate the ascending aorta and the right attachment. However, the resulting sternal insta-
atrium. The cardiac vent catheter can typically be bility resulted in this technique being abandoned
placed into the right superior pulmonary vein, the by most surgeons (Cosgrove and Sabik 1996).
pulmonary artery, or directly through the aortic The S-forming partial sternotomy (see
valve. External defibrillation pads should be . Fig. 26.2f) enables, with preservation of both
placed routinely prior to patient preparation. internal mammary arteries, a good exposure of
A more recent completely new concept is the the aortic valve region and the neighboring pul-
catheter-based aortic valve replacement. Presently, monary artery (Autschbach et al. 1998). The
three different techniques—the transapical (via advantage of this strategy lies in its preservation of
left anterior mini-thoracotomy), transaortical the continuity of the shoulder girdle. Subsequent
(via upper mini-sternotomy), and the percutane- stabilization of the sternum with wires is challeng-
ous transfemoral or transaxillary approach are ing, and the technique is rarely used.
being intensively evaluated clinically.
Various forms and modifications of the partial 26.3.4.1 esults of Minimally Invasive
R
sternotomy for aortic valve and ascending aorta Aortic Valve Surgery
operations include (. Fig. 26.2): the J-forming Whether there is a clear advantage of partial
upper sternotomy, the transverse sternotomy, the upper sternotomy versus complete median ster-
S-forming sternotomy, and the right-sided para- notomy is heavily debated. It has been shown that
sternal thoracotomy. With the J-forming partial mini-sternotomy leads to a reduction in blood
upper sternotomy (see . Fig. 26.1c), the sternum loss, and shorter ICU and hospital length of stays
is divided from the sternal notch caudally until (Bakir et al. 2006; Khoshbin et al. 2011). A signifi-
the fourth or fifth intercostal space and curved cant advantage with respect to mortality has not
into the respective intercostal space on the right been demonstrated. Alternatively, a median ster-
side, taking care to preserve the right internal tho- notomy can be performed using a small skin inci-
racic vessels (Konertz et al. 1996). Through this sion (limited skin, full sternotomy). Despite an
slightly right-dominant approach, the aortic array of alternatives, at most centers, the partial
valve, ascending aorta, and right atrium can be upper sternotomy or the classic median sternot-
well exposed. The aortic cannula can be placed omy remains the preferred approach for aortic
directly into the ascending aorta. In case of diffi- valve surgery.
cult anatomic exposure, the femoral vessels can be
cannulated for the HLM. The venous cannula may 26.3.4.2 Transapical and Transfemoral
be advanced into the right atrium under TEE Aortic Valve Replacement
guidance or may be placed directly into the right The transapical access represents a new surgical
atrium but brought up through a subxiphoidal concept (. Fig. 26.3) (Dewey et al. 2006). With this
incision or a right chest incision. approach, entry into the thorax is achieved through
The advantage of these latter approaches lies a small left anterolateral thoracotomy at the level of
in a better exposure because the light tension on the fifth or sixth intercostal space. Via this limited
the cannula results in retraction of the right access, the pericardium is opened and a purse-
atrium away from the aortic root. Sternal closure string suture is placed on the beating heart at the left
is usually achieved with normal wires, taking care ventricular apex. Simultaneously, a specially
to achieve good bone approximation and stabili- designed biological valve mounted on a stent is
zation of the entire sternal incision, including the crimped onto a delivery catheter. Next, antegrade
portion into the right intercostal space. A trans- catheter-based implantation and expansion of this
verse division of the sternum at the level of the valve is performed under angiographic and echo-
third intercostal space (see . Fig. 26.2e) may also cardiographic guidance (Dewey et al. 2006).
be utilized. The disadvantage of this method is Additionally, during the mechanical expansion of
that both internal mammary arteries are divided, the valve, an external pacemaker is used to stimulate
842 J. Garbade et al.
a b c
26
d e f
..Fig. 26.2 Minimally invasive aortic valve replacement. Surgical approaches: a L- or T-shaped sternotomy, b L- or
T-shaped upper sternotomy, c J-shaped sternotomy, d right-sided parasternal thoracotomy, e transverse sternotomy,
and f S-shaped sternotomy
a b c
26
d e
..Fig. 26.4 Minimally invasive mitral valve surgery, surgical approaches: a, b right- and left-sided partial upper
sternotomy, c left-sided C-shaped partial sternotomy, d right-sided anterolateral thoracotomy, e T-shaped upper
sternotomy
..Fig. 26.5 Chitwood
clamp
valve plane in the direction of the right shoulder, technique is a safe alternative for reoperations,
isolated mitral or tricuspid as well as combined also, in particular for high-risk patients (Seeburger
mitral and tricuspid valve pathology can be effec- et al. 2008; Garbade et al. 2013).
tively exposed and surgically treated. Additional To perform the right anterolateral thoracot-
procedures like ablation for atrial fibrillation, omy, the patient is placed in supine position, with
closure of PFOs/ASDs, or removal of tumors
the right chest elevated to around 30°. The right
(i.e., myxomas) can be performed concomitantly arm is lightly abducted and placed on a position-
without difficulty (. Fig. 26.6). ing board. External defibrillation pads should be
At the Leipzig Heart Center, this approach is applied primarily. The first surgical step is femoral
used routinely for primary procedures. This vessel cannulation and connection to ECC circuit.
Chapter 26 · Minimally Invasive Cardiac Surgery
845 26
Videoscope approximately 1 cm in size, are necessary. In an
LA-retractor CO2 imaginary axis between the mitral valve and the
right shoulder, a stab incision is made into the
second intercostal space for introduction of a vid-
eoscopic camera. This is introduced via a special
port system into the chest, which also allows for
simultaneous CO2 insufflation (4–6 l/min). We
deem the CO2 insufflation essential and perform
it during the entire time of intracardiac manipu-
30°
lation. The videoscope can either be stabilized by
Chitwood clamp a special holder arm or can be connected to a
..Fig. 26.6 Positioning, surgical incision, and speech-responsive robotic arm (Computer
projection of the anatomic structures for the right-sided Motion, USA).
anterolateral approach for mitral and tricuspid valve A second incision in the second intercostal
surgery—Leipzig technique
space at the anterior axillary line is necessary to
place the Chitwood clamp (see . Fig. 26.5).
Therefore classically the right femoral vessels are . Figure. 26.6 gives an overview of the positioning
exposed. For elective procedures, it is of value to and the necessary incisions for minimally inva-
perform the necessary cardiac catheterization by sive mitral valve surgery, as they are routinely
avoiding the right femoral vessels, since such used at the Leipzig Heart Center. After opening of
diagnostic measures may be associated with inju- the intercostal space, the pericardium is exposed.
ries and hematomas complicating cannulation. This is opened horizontally and parallel to the
Following exposure of the femoral vessels, a 4-0 phrenic nerve, with care taken to maintain a suit-
or 5-0 Prolene purse-string suture is placed on the able distance between the pericardial incision and
anterior surface of the vessels. Next, using the nerve (at least 2 cm).
Seldinger technique, the vessels are cannulated, After the ascending aorta is exposed under
beginning with the femoral vein. It is a dvantageous direct or indirect vision (videoscopic), cross-
to use TEE to determine the correct positioning clamping of the aorta with the Chitwood clamp is
of the venous cannula. If there is concomitant tri- performed as ECC flow is reduced. Care must be
cuspid valve pathology or the body weight is taken during preparation and cross-clamping of
above 90 kg, an additional venous cannula can be the aorta to avoid injury to adjacent structures, in
placed percutaneously via the jugular vein. particular of the pulmonary artery or the left
Possible complications include vessel rupture, atrial appendage. Injury to these structures usu-
particularly of the retroperitoneal vasculature, ally requires sternotomy. Although aortic cross-
and retrograde dissection. For this reason, at our clamping is preferred, endoluminal occlusion of
center, simultaneous TEE examination is per- the aorta using the HeartPort technique may be
formed during femoral cannulation. If right fem- used alternatively. As previously discussed, due to
oral cannulation is not possible, either left femoral a variety of complications, many surgeons have
or axillary cannulation may be performed. abandoned this technique (see 7 Sect. 26.2.3,
Following cannulation, the lateral mini-thoracot- «HeartPort Technique»).
omy is performed. The length of the skin incision In order to optimally access the left atrium, a
is 4–6 cm and is carried out in a lightly convex third small incision in the parasternal portion of
course caudally to the inframammary fold. For the right fourth intercostal space (see . Fig. 26.6)
especially obese patients and for women, prior is made, to place an atrial retractor and/or retrac-
skin marking and special positioning using an tion stitches. If performed under direct visual
adhesive drape is helpful. After dissection of the control, injuries to the right internal mammary
subcutaneous layers and controlled division or artery should be avoided.
displacement of the musculature, the thoracot- Along with endoscopic performance of iso-
omy is performed in the fourth intercostal space. lated steps of the operation, special instruments
Next, a special soft tissue-spreading retractor is have been developed and are necessary (i.e.,
used to spread the thoracotomy incision atrau- Chitwood clamp, HeartPort instruments). The
matically. Additionally, three further incisions, surgeon’s headlight, as well as the light source for
846 J. Garbade et al.
the videoscope, illuminates the surgical field. valve surgery, with at least comparable results to
Using a connected documentation and video- other approaches. However, the technique may
scopic camera system, every individual step, the not gain universal acceptance primarily because
pathology, and the operative result can be digi- of the resources required.
tized and recorded. An intelligent arrangement of
the monitors allows the assistant(s) and anesthe- 26.3.5.1 esults of Minimally Invasive
R
sia and nursing personnel to follow every step of Mitral and Tricuspid Valve
the operation. Via the right anterolateral approach, Surgery
26 direct access to the left and right atrium can be A current study from our group examined the
achieved easily. For isolated or concomitant tri- short- and long-term results of minimally invasive
cuspid valve pathology, additional cannulation of right anterolateral thoracotomy (Seeburger et al.
the jugular vein is necessary to achieve complete 2008). A total of 1,339 patients were analyzed. The
caval occlusion. The placement of the jugular average bypass time was 122 ± 41 min, and the
venous cannula can be performed in the induc- cross clamp time was 70 ± 35 min. The 30-day and
tion room by the cardiac anesthesiologists. Both 1-year mortality for isolated mitral valve recon-
venous cannulae are then connected with a struction were 2.4 % and 5 %, respectively and
Y-connector for ECC. The superior and inferior 3.7 % and 11.2 % for isolated mitral valve replace-
vena cavae can be controlled with tourniquets or ment, respectively. In seven patients, conversion to
large, atraumatic clamps. sternotomy was required; causes for conversion
Using a special cannula, the cardioplegia is included uncontrollable bleeding, obscured anat-
delivered into the aorta after aortic cross clamp- omy, or dense adhesions. For 494 patients, addi-
ing. Finally, this cannula can be brought out tional procedures such as atrial fibrillation ablation
through the incision for the atrial lift retractor (n = 305), tricuspid valve repair (n = 94), and clo-
and be used for later deairing (needle vent). sure of a PFO (n = 95) were carried out. The aver-
Closure of this site is performed using the usual age follow-up time was 28.4 ± 23.5 months, with a
technique. follow-up rate of 99 % (Seeburger et al. 2008). In
Particularly for reoperations, the procedure addition, a large single-center review of more than
can be carried out on the beating or fibrillating 3,000 minimally invasive mitral valve procedures
heart. After reconstruction or replacement of the showed the safety and the very low rate of conver-
corresponding heart valve, closure of the atrium is sion to complete sternotomy (Vollroth et al. 2012).
performed with simultaneous deairing. However, there is an individual learning curve for
Continuous CO2 insufflation during the opera- minimally invasive mitral valve procedures pro-
tion is useful. Following atrial closure, deairing, viding the need for good monitoring, training,
weaning from the HLM, and decannulation, and mentoring not only in the initial phase for this
hemostasis is performed. The ribs are adapted kind of surgery (Holzhey et al. 2013).
with Vicryl sutures, and the incision site for the In contrast to this work, a study from the
video camera can be used for placement of a chest Cleveland Clinic showed that the rate of mitral valve
tube. Layered wound closure and administration repair was lower after lateral thoracotomy than with
of local anesthesia into the mini-thoracotomy median sternotomy, and the former was also associ-
incision helps reduce postoperative pain. ated with a higher risk of stroke. The authors con-
Multiple centers follow the concept of a com- cluded that in most cases, sternotomy remains the
pletely endoscopic, telemanipulator-assisted sur- preferred method (Svensson et al. 2007). The results
gery. In recent years, there is a growing body of of this study were based on a small number of
literature on this topic demonstrating its safety, patients and therefore may not be generalized.
efficacy, and durability. However, widespread Completely endoscopic, telemanipulator-
application of this technique is limited by the assisted procedures are presently the subject of
extensive team and technical training and exper- intense clinical evaluation. Fifteen years experience
tise required (Woo et al. 2006; Casselman et al. from Chitwood’s group, including reoperations,
2007a, b; Arcidi et al. 2012; Nifong et al. 2012; shows that there was a comparable 30-day mortal-
Mihaljevic et al. 2013). ity (2.2 % vs. 1.0 %) among 341 video-assisted and
In summary, there are more data available on 100 completely telemanipulator- assisted proce-
the use of telemanipulators for minimally invasive dures (da Vinci System). There were no conver-
Chapter 26 · Minimally Invasive Cardiac Surgery
847 26
sions to sternotomy or dissection. In both groups, cified valves or those with leaflet restriction (i.e.,
complex mitral valve repairs were performed. type IIIB dysfunction). The necessity for perform-
However, the authors came to the conclusion that ing simultaneous procedures like atrial fibrillation
the times of cross-clamp, cardiopulmonary bypass, ablation or reconstruction of a relevant tricuspid
and overall operation were significantly longer in valve insufficiency also limits this technique.
the da Vinci group (Arcidi et al. 2012). In a recent Potential problems include the anatomic relation-
study from Vanermen et al., it was shown that for ships of the posterior mitral anulus, the CS, and
patients requiring cardiac reoperations, the video- the circumflex coronary artery (Tops et al. 2007).
assisted minimally invasive approach for mitral An important prerequisite for the safe and effec-
and tricuspid valve surgery is not only safe but also tive implantation of the partial anuloplasty is a
associated with a lower 4-year mortality than sta- parallel relationship between the anulus and the
tistically expected (Casselman et al. 2007a, b). coronary sinus (CS), which is also a limiting fac-
tor. Additionally, the CS can no longer be used
26.3.5.2 Percutaneous Minimally later for the administration of retrograde cardio-
Invasive Strategy plegia or cardiac resynchronization therapy. The
As with the aortic valve, the mitral valve is also first clinical results still reflect an experimental
the focus of intensive research efforts for the character and are not comparable with the present
development of percutaneous strategies. Based on high standards and excellent results of the right
the cooperation of multiple professional societies anterolateral minimally invasive mitral valve sur-
(STS, AATS, SCAI, ACCF, AHA, EACTS), a posi- gery.
tion paper on percutaneous valve technology was In addition to the interventional percutaneous
produced (Thomas et al. 2005). mitral valve repair (MitraClip procedure), there is
The goal of percutaneous techniques is to use a new concept of transapical beating heart adjust-
a catheter-based approach to deliver a system that able chordae replacement for mitral valve repair
can treat mitral valve disease on the beating (NeoChord device) or transapical beating heart
heart, thereby avoiding a sternotomy and ECC. mitral valve replacement (Seeburger et al. 2010;
Historically, percutaneous mitral balloon valvot- Cheung et al. 2013). Both concepts are very prom-
omy represents an established approach for the ising, but further results and specifications are
treatment of mitral stenosis that is still widely per- needed.
formed. Limitations of this concept lie in its lim-
ited durability or efficacy of the balloon dilatation. 26.3.5.3 Conclusion
However, it may be used for certain patients as a Short- and long-term results have demonstrated
bridging therapy for later surgical valve replace- the safety, efficacy, and reproducibility of the
ment (Kim et al. 2007). right-sided anterolateral mini-thoracotomy as an
At present, two different percutaneous strate- alternative approach for the treatment of mitral
gies have been evaluated: the edge-to-edge tech- and tricuspid valve disease. Additional surgical
nique using a clip or suture (double-/triple-orifice procedures like ablation of atrial fibrillation or
technique) and the partial anuloplasty through closure of a PFO/ASD can be performed simulta-
implantation of stabilizing systems in the coro- neously. This technique also has broad applicabil-
nary sinus (CS) to reduce the circumference of ity for cardiac reoperations and especially in
the posterior mitral anulus. high-risk patients. The partial opening of the
The EVEREST trial (Endovascular Valve pericardium represents an additional protective
Edge-to-Edge REpair STudies) includes the first measure for possible later sternotomy. Based on
clinical results of this double-orifice concept present data, this technique can be regarded as a
(EVEREST Registry 2007). After implantation of «gold standard» in mitral valve surgery.
the MitraClip device (Evalve Inc, Menlo Park, Video-assisted endoscopic procedures are safe
California), a significant reduction in mitral and effective, but require a constant learning pro-
regurgitation could be demonstrated (Feldman cess and intensive training. Completely endo-
et al. 2009, 2011; Glower et al. 2012). scopic, telemanipulator-assisted procedures are
Like the first technique, the partial anuloplasty presently limited to certain centers and represent
also is limited by its ability to treat all relevant interesting, albeit elaborate, alternatives. Percuta-
mitral valve pathologies, particularly severely cal- neous strategies are still evolving. Because of the
848 J. Garbade et al.
small number of clinical studies, in contrast to trates surgical approaches for revascularization
percutaneous aortic valve replacement, these via a partial sternotomy.
methods still remain experimental. In particular, for stenoses of the LAD, the
technique of left-sided anterolateral mini-
thoracotomy (MIDCAB), described by Kolessov
26.3.6 Coronary Arterial in 1967 (Kolessov 1967), has been established as a
Revascularization standard technique in many centers (. Fig. 26.8a).
An array of different surgical approaches and
26 The four major components in minimally invasive conduit harvest techniques, such as thoracoscopic
strategies for coronary revascularization are sur- LIMA preparation (Benetti et al. 1991, Benetti
gical access, use/avoidance of ECC, beating heart and Ballester 1995), the subxiphoidal
surgery (avoidance of ischemia), and the combi- (Subramanian 1997) or transabdominal approach
nation of surgery with interventional approaches (Subramanian and Patel 2000), the extended left
(hybrid strategy). anterior mini-thoracotomy (Calafiore et al. 1996),
The goal is to obtain adequate and safe expo- or the right parasternal thoracotomy (Stanbridge
sure of the target vessels through a limited incision et al. 1997), have been described in the literature
and reduce the immunologic and operative trauma (. Figs. 26.7 and 26.8).
by avoiding ECC. In achieving these goals, the With the development of special instruments
minimally invasive approach should be at least as like rib retractors and stabilizers, MIDCAB has
safe and effective as the conventional method. become the procedure of choice for isolated
Furthermore, the use of hybrid procedures should LIMA-LAD revascularization, with excellent clin-
reduce the operative risk even further. Prerequisites ical long-term results (Thiele et al. 2005; Holzhey
for this approach include not only the develop- et al. 2012; Blazek et al. 2013; Etienne et al. 2013).
ment of corresponding instruments but also an With this technique, the skin incision is inframa-
intelligent planning and performance of the oper- mmary, lightly curved over a length of approximately
ation. 6 cm. Additionally, anastomoses in the region of
55 OPCAB (off-pump coronary artery bypass) diagonal and obtuse marginal branches can be car-
refers to operations via a partial or complete ried out, whereby an extension of the skin incision
sternotomy, in which coronary revascularization can be very helpful. For two-vessel coronary disease,
is carried out without ECC on the beating heart. a hybrid strategy can also be considered as an alter-
55 MIDCAB (minimally invasive direct coronary nate approach (Repossini et al. 2013). Particularly for
artery bypass) refers to operations via a mini- high-risk patients with multiple comorbidities, a
thoracotomy, in which coronary revascular- hybrid strategy may be valuable to reduce operative
ization is done without ECC, also on the risk. The hybrid strategy may involve either simulta-
beating heart. neous or staged PCI of a second coronary vessel after
55 TECAB (totally endoscopic coronary artery LIMA-LAD revascularization.
bypass) represents an innovative approach that A direct cross-clamping of the aorta or perfor-
is still in its infancy. The principle of this tech- mance of a central anastomosis is generally not
nique is complete endoscopic and computer- possible with this technique. However, in almost
assisted performance of the coronary bypass all cases, revascularization is performed on the
operation. In addition to perfect surgical tech- beating heart. When use of ECC is required, fem-
nique, the operator must contend with the oral cannulation can be carried out.
additional visuospatial and technical demands The MIDCAB technique is also useful for revas-
of performing the operation from a console. A cularization of the right coronary artery (RCA). Via
detailed presentation of this technique is given a right-sided anterolateral mini-thoracotomy (see
in 7 Sect. 26.4.2, «Endoscopic and Telemanipu- . Fig. 26.8b), the right internal mammary artery
lator-Assisted Cardiac Surgery». (RIMA) can be exposed, prepared, and anasto-
mosed to the RCA (Stanbridge et al. 1997).
26.3.6.1 lternative Incisions for
A Further alternate approaches include the par-
Revascularization tial lower sternotomy, the partial left-sided ster-
The classical approach of complete median ster- notomy, and a technique developed at the Heart
notomy is not always required. . Figure 26.7 illus- Center in Dresden, Germany, the upper left-sided
Chapter 26 · Minimally Invasive Cardiac Surgery
849 26
a b
c d
..Fig. 26.7 Minimally invasive surgical approaches via an incomplete sternotomy for coronary artery bypass surgery:
a left-sided C-shaped partial sternotomy, b lower left-sided L-shaped sternotomy, c lower T-shaped sternotomy, and
d upper left-sided L-shaped sternotomy
anterolateral thoracotomy (see . Fig. 26.8c) mal anastomoses on the ascending aorta. The par-
(Gulielmos et al. 1999). With the latter technique, tial lower sternotomy offers the advantage of
a left third interspace thoracotomy is performed, being able to harvest both internal mammary
and rib division is often required to achieve suffi- arteries and revascularize all coronary arteries
cient exposure. The advantage of this method is a (Walterbusch 1998).
good exposure of the ascending aorta and right The subxiphoid, transabdominal approach (see
atrium, which enables central cannulation if the . Fig. 26.8d) described by Subramanian is useful for
HLM is required and the ability to perform proxi- revascularization using both internal mammary
850 J. Garbade et al.
a b
26
c d
..Fig. 26.8 Minimally invasive surgical approaches via a thoracotomy for coronary artery bypass surgery: a left-sided
anterolateral mini-thoracotomy, b right-sided anterolateral mini-thoracotomy, c extended left-sided anterolateral
thoracotomy, and d subxiphoid and transabdominal approach
arteries and the gastroepiploic artery (Subramanian 26.3.6.2 esults of Minimally Invasive
R
et al. 1997, Subramanian and Patel 2000). Open Coronary Artery
With respect to quality assurance, it is useful Bypass Surgery
to perform an assessment of the coronary bypass Multiple reports indicate that minimally invasive
grafts. Transit-time Doppler flow measurement direct revascularization has become a standard sur-
has become established as a routine approach and gical approach that has a higher freedom from rein-
can also be used with minimally invasive tervention than catheter-based imterventional
approaches. Furthermore, intra- or postoperative approaches (Diegeler et al. 2002; Thiele et al. 2005;
angiography has also been used.
Chapter 26 · Minimally Invasive Cardiac Surgery
851 26
Holzhey et al. 2012; Blazek et al. 2013; Etienne et al. Overall, the published data show that mini-
2013). In several studies, MIDCAB has also been mally invasive, direct coronary revascularization
shown to be superior in terms of freedom from is safe and effective and can be considered a stan-
angina and clinical symptoms (Thiele et al. 2005; dard of care. The precise role of hybrid proce-
Blazek et al. 2013). dures remains to be seen. However, it clearly
When compared to sternotomy, this approach represents a valuable alternative that can reduce
also resulted in fewer wound infections and less the operative risk of patients with multiple
postoperative pain, which translated into a better comorbidities.
convalescence and shorter hospital stay. The
perioperative mortality is currently under 1 %, with 26.3.6.3 Off-pump Coronary
excellent long-term results (Boodhwani et al. 2006). Revascularization Strategies
In one of our own studies (Holzhey et al. 2012), With the introduction of ECC, an innovation that
the early postoperative mortality among 1768 launched cardiac surgery, a higher standard could
MIDCAB patients was 0.8 % (n = 15), and the peri- be achieved in coronary bypass surgery, although
operative stroke rate was 0.4 % (n = 7). A conver- the first coronary bypass operation by Kolessov
sion to median sternotomy was necessary in 1.7 % was already carried out without ECC (Kolessov
(n = 31) because of severe pleural a dhesions, intra- 1967).
myocardial course of the LAD, bypass failure, or However, cardiopulmonary bypass repre-
bleeding. For 712 patients, a routine postoperative sents a significant operative and immunologic
angiography was performed, with 95.5 % of patients trauma. As a result of the manipulation of the
having a normal configuration of the bypass graft. great vessels, activation of the inflammatory
In the early postoperative course, 11 patients cascade, and active modulation of the blood
required additional PCI, and 48 patients required temperature and laminar flow, the use of the
an additional coronary bypass operation. The 5- HLM is not without risk. Activation of the
and 10-year survival was 88.3 % and 76.6 %, respec- immune system results in release of multiple
tively. Freedom from cardiac and neurologic vasoactive substances and cytokines. This
complications (MACCE) at 5 and 10 years was response results in increased capillary permea-
85.3 % and 70.9 %, respectively. The MIDCAB bility with sequestration of neutrophils and
technique is also safe for coronary reoperations but increased microemboli. As a result, a vasoplegic
challenging (Holzhey et al. 2012). syndrome is produced (Brasil et al. 1998; Gomes
In a randomized study (MIDCAB vs. PCI) for et al. 2003).
isolated one-vessel coronary disease, Cremer’s Avoidance of the HLM reduces cytokine
group in Kiel, Germany, showed no difference in release, in particular IL-8, which reduces the
mortality. However, again in this study, there was inflammatory response and the ensuing myocar-
a significant difference in reintervention rate dial injury (Deng et al. 1996). In particular, the
favoring MIDCAB. At a mean follow-up of risk of HLM under emergency conditions and for
5 years, the reintervention rate in the PCI group patients with multiple comorbid illness is very
was 24 % (Fraund et al. 2005). In a meta-analysis high (Brasil et al. 1998; Rastan et al. 2006; Palmar
of 1,110 patients, there was a significant advan- et al. 2007; Ben-Gal et al. 2011; Lamy et al. 2012).
tage to surgical revascularization of the LAD Injuries to the ascending aorta, including aortic
when compared to PCI in terms of mortality, dissection or mobilization of plaque or calcific
MACCE rate, and freedom from angina fragments, may also occur during cannulation or
(Boodhwani et al. 2006). use of the HLM and lead to cerebrovascular com-
In a study from our group, patients with iso- plications.
lated LAD stenoses were randomized to MIDCAB The renaissance of beating heart coronary
or PCI with bare-metal stent (BMS) implantation bypass surgery came primarily through the work
(Thiele et al. 2005). At 5 years, there was no differ- of Benetti (1985) and Buffolo (1991) in the early
ence in mortality or recurrent infarction rate, but 1980s. One of the most important developments
the reintervention rate in the PCI group was sig- in modern off-pump coronary artery bypass sur-
nificantly higher, and the MIDCAB patients had gery was the technique propagated by Benetti and
fewer clinical symptoms by CCS classification Calafiore in which the LIMA-LAD anastomosis
(Thiele et al. 2005). was performed directly on the beating heart via a
852 J. Garbade et al.
left lateral mini-thoracotomy (Benetti et al. 1991; nary artery bypass surgery has been demonstrated,
Calafiore et al. 1996). with beneficial effects on neurocognitive dysfunc-
Conditions for beating heart surgery include a tion, ventilation time, blood loss, and hospital stay
good exposure and stabilization of the target ves- as well as postoperative atrial fibrillation and renal
sel, a temporary interruption of coronary blood insufficiency. All of these benefits have been associ-
flow during the anastomosis without irreversible ated with optimal resource utilization. However,
myocardial ischemia, maintenance of sufficient with respect to mortality and bypass graft function,
systemic circulation during the anastomosis, and there has been increasing benefit for OPCAB when
26 a logical sequence of revascularization. compared to conventional strategies (Rastan et al.
With the development of new instruments 2006; Palmer et al. 2007; Keeling et al. 2012; Diegeler
and surgical tools that lead to efficient immobili- et al. 2013). On the other hand, a randomized study
zation and presentation of the target vessel, beat- from 2007 showed that OPCAB had no advantage
ing heart coronary bypass surgery without ECC with respect to neurocognitive function or 5-year
can be performed with very high quality and has survival (van Dijk et al. 2007). Nevertheless, patients
become standard in many centers. Important in with an acute myocardial infarction benefit from
this context is the close cooperation with a spe- this operative strategy (Rastan et al. 2006), as do
cialty group of cardiac anaesthesiologists. older patients, high-risk patients, or patients with a
In contrast to on-pump surgery, the sequence calcified/porcelain aorta (Marui et al. 2012; Keeling
in which target vessels are revascularized is of et al. 2013). In experienced hands, OPCAB is also
great clinical significance. The surgical approach safe for coronary reoperations (Usta et al. 2013).
may be a classical median sternotomy, partial
sternotomy, lateral left- or right-sided mini- 26.3.6.5 Conclusion
thoracotomy, or subxiphoidal. Despite the very good results of conventional cor-
For optimal myocardial physiology during onary artery revascularization, it should be con-
luxation and performance of the bypasses, a reduc- cluded that modern coronary revascularization is
tion of the dp/dt, heart rate, and afterload should confronted with four demanding challenges for
be achieved. Ischemic or pharmacologic precondi- surgeons:
tioning can also be helpful. The Trendelenburg 55 Increasing comorbidity of the patients
maneuver can be used to regulate volume status. 55 Increased demand for reoperations, due to
Performance of central aortic anastomoses is an aging patient population
not absolutely necessary, especially in the setting 55 Multiple previous interventions by
of a calcified aorta. As an alternative, particularly cardiology
for complete arterial revascularization, the use of 55 A higher and more progressive/aggressive
T- and Y-anastomoses based on the left internal interventional standard
mammary artery can be conducted. Using this
technique with the RIMA or the radial artery, all As a result, procedures that reduce operative
territories can be revascularized. If central anasto- trauma, such as beating heart revascularization
moses are required, a partial cross-clamp may be with or without the ECC, minimization of the
applied to the ascending aorta. Newer connector surgical approach (MIDCAB), and all-arterial
systems also offer the possibility to perform prox- revascularization should be standard procedures
imal aortic anastomoses using a clampless tech- in modern CABG surgery.
nique, thereby reducing operative trauma and
neurologic events (Emmert et al. 2011).
26.3.7 Conduit Harvest
26.3.6.4 Results
Only a brief discussion of results is presented here In recent years, endoscopically minimally invasive
but discussed in greater detail in 7 chapter procedures for conduit harvest are increasingly
«Coronary Artery Disease», Sect. 22.5. In Germany used. The endoscopic harvesting of the greater
in 2011, 14.7 % of CABG was performed using an saphenous vein, as well as the video-assisted har-
off-pump technique, with an increasing tendency vest of the radial artery, are well-accepted and stan-
in recent years (Funkat et al. 2012). In multiple dard procedures in many centers (Connolly et al.
studies, the safety and efficacy of off-pump coro- 2002; Patel et al. 2004; Williams et al. 2012; Sastry
Chapter 26 · Minimally Invasive Cardiac Surgery
853 26
et al. 2013). In clinical studies, these techniques associated with very good clinical outcomes, with
have been shown to preserve the histological struc- a low rate of complications (O’Neill et al. 2007).
ture and endothelial function of the vessels, Currently, the hybrid strategy of combined
although there is clearly a learning curve (Fabricius endocardial and thoracoscopic epicardial ablation
et al. 2000; Aziz et al. 2005). These techniques have is of great clinical interest to overcome the short-
been shown to reduce the wound infection rate, comings of catheter-based ablation therapy (mul-
particularly in obese patients (Williams et al. tiple procedures are often required and creating
2012). For minimally invasive techniques of vein linear lines are challenging) and beating heart epi-
harvest, two different procedures have been cardial ablation (creating mitral isthmus line can
described, either harvest under direct or under be challenging). First results are very promising
endoscopic vision (Sastry et al. 2013). (Pison et al. 2012).
26.3.8.1 Results
26.3.8 Cardiac Arrhythmia Surgery Minimally invasive techniques are well estab-
lished for the treatment of atrial fibrillation.
Surgical therapy of atrial fibrillation has evolved Isolated atrial fibrillation can be treated surgically
dynamically through the evaluation of various using a minimally invasive approach as for mitral
energy sources, minimally invasive approaches, valve surgery. Alternatively, bilateral video-
and completely endoscopic techniques. Based on assisted thoracoscopic pulmonary vein ablation
the theory that existing electrical triggers for can be performed using an off-pump technique.
atrial fibrillation are located in the region of the Concurrently, surgical and interventional results
pulmonary veins and the atria, the concept of must be compared, but randomized trials are
surgical isolation of these foci was developed. lacking (see 7 chapter «Surgical Therapy for Atrial
With respect to atrial fibrillation, the MAZE pro- Fibrillation», Sect. 29.6.4).
cedure represents the most effective surgical
technique but requires entry into both the left
and right atria. Irrespective of the energy source 26.3.9 Great Vessels
used (monopolar or bipolar radiofrequency,
microwave, cryoenergy, or ultrasound), a safe Since the ascending aorta and aortic arch are
and effective therapy of isolated atrial fibrillation located in the upper mediastinum, replacement
can be performed via a right lateral mini-thora- of these aortic segments can be performed via the
cotomy with femoral cannulation. Commonly, various partial sternotomy approaches described
the therapy can be combined with other cardiac earlier for aortic valve replacement. Often, aortic
surgical procedures (valve or CABG procedures). replacement for aneurysmal disease is performed
Pulmonary vein isolation can be carried out on concomitantly with aortic valve replacement, but
the beating heart, even as an off-pump proce- may also be performed as an isolated procedure.
dure, as long as no concomitant intracardiac sur- Cannulation may be performed centrally, via
gery is required. the right axillary artery or via femoral access as for
Various authors have reported successful abla- mitral or tricuspid valve surgery. With increasing
tion of atrial fibrillation on the beating heart via a experience, partial or total aortic arch replacement
minimally invasive or completely endoscopic and even various forms of aortic root replacement
approach (right- and left-sided miniMAZE) may be performed via a partial upper sternotomy.
(Weimar et al. 2012) Surgical success rate seems As with other minimally invasive procedures, the
to also be dependent on ganglionic plexus abla- quality of the operation should not be compro-
tion (parasympathetic innervation) (Mehall et al. mised by the limited surgical approach.
2007; von Oppel et al. 2009; Pison et al. 2012). In reoperative cases, or for patients with poor
Completely telemanipulator-assisted ablation lung function, the use of the partial sternotomy
techniques have been described (Weimar et al. 2012). may be beneficial to limit the amount of cardiac
The interventional treatment of atrial fibrilla- dissection required or reduce respiratory compli-
tion represents an alternate approach. This tech- cations, respectively.
nique uses focused electrical isolation in the atria Off-pump debranching of the aortic arch ves-
and pulmonary veins and has been generally sels may also be performed via a partial upper
854 J. Garbade et al.
sternotomy, with either simultaneous or staged Miller 2009; Wilson et al. 2009; Aggarwal and
implantation of a stent graft to treat aortic arch Pagani 2010). Continuous-flow pumps appear to
pathology. For cases of acute type A aortic dis- have an advantage over the larger, bulkier pulsatile-
section, a novel approach has been recently flow assist devices, both in survival and complica-
described for inoperable patients, whereby a tion rates (Griffith et al. 2001; Gregoric et al. 2011).
transapical approach has been used to deliver a Thus, supporting the ongoing trend of further
stent graft into the ascending aorta without the miniaturization of these devices and the insuffi-
HLM (Köbel et al. 2013; Ronchey et al. 2013). cient supply of acceptable donor hearts have led to
26 These are further examples of the use of innovative thinking about less invasive mechani-
minimally invasive and hybrid approaches (see cal strategies for long-term circulatory support.
7 Sect. 26.4.1 «Hybrid Strategy») to reduce the The newly designed LV-sewing ring revolu-
operative risk for cardiac surgical patients. tionizes and facilitates the positioning of the
inflow cannula to the left ventricle and makes this
extremely versatile. The diaphragmatic approach
26.3.10 Heart Failure/Ventricular to HVAD implantation is an alternative implanta-
Assist Devices tion technique that appears to be particularly suit-
able for patients with small lateral thoracic
dimensions, e.g., pediatric patients, and/or an
26.3.10.1 Left Ventricular Assist unusually enlarged heart (Cheung et al. 2011;
Device Loforte et al. 2011). Implantation via thoracotomy
The incidence of heart failure worldwide contin- with two small incisions and with or without car-
ues to increase, so does the number of treatment diopulmonary bypass (cannulation via femoral
options to prevent or delay the onset of end- vessels) avoiding the invasive sternotomy is being
stage heart failure. The last decade has seen fun- most recently being utilized and shows promising
damental advances in the medical and surgical results (Slaughter et al. 2010; Strueber et al. 2011).
therapy for patients with severe heart failure. Regarding the small design, the device can be
While heart transplantation remains the gold placed after implantation either transapical, trans-
standard for patients with end-stage heart fail- mitral, intercostal, or subcostal.
ure, the rate of transplantation has remained Several centers have published on biventricu-
relatively steady over the past 20 years (Stehlik lar application of the HVAD pump. Hetzer and
et al. 2010; Francis et al. 2010). This has resulted associates described biventricular implantation
in a major imbalance between supply and techniques and reported on five patients implanted
demand leading to 20 % mortality for patients simultaneously with an LVAD and RVAD and an
on a waiting list for heart transplantation. Giving additional three patients implanted with an RVAD
the relative constant number of donor hearts after initial LVAD implantation. All five patients
available, with a continuously increasing num- with simultaneous LVAD and RVAD implantation
ber of heart failure patients requiring transplan- survived and were discharged home (Hetzer et al.
tation at some point, the waiting list mortality 2010). Strueber and colleagues reported on one
will increase dramatically. As a consequence of patient who received a right ventricular assist
the persistent and relatively even increasing device (RVAD) after initial LVAD implantation
donor organ shortage, there has been growing (Strueber et al. 2010). The patient was awaiting
interest for alternative strategies. These strate- transplantation at the time of publication. Loforte
gies are in particular left ventricular mechanical and colleagues described a new technique for
circulatory support or partial circulatory sup- biventricular assist device (BiVAD) implantation
port not only as bridge to transplantation but in a small (1.6 m2) patient (Loforte et al. 2010).
also as a permanent solution: destination ther- In summary, the message is clear: continuing
apy (see also 7 chapter «Cardiac Assist Devices and to shrink the size of the pump and the concept of
Total Artificial Heart» Sect. 38.4). only partial circulatory support may minimize
Recently, newer, more durable devices have the surgical trauma, thus resulting in less adverse
been developed and have further advanced the events, shorter recovery times, and enhanced
mechanical circulatory support field (Lietz and quality of life.
Chapter 26 · Minimally Invasive Cardiac Surgery
855 26
26.4 New Clinical Concepts the patient, which reduces the hospital stay. A
prerequisite for this approach is the presence of
26.4.1 Hybrid Strategy an operating room that combines the require-
ments of the surgical and interventional team
The term hybrid strategy refers to the combina- and is outfitted with a modern fluoroscopic sys-
tion of surgical and interventional methods, tem and the necessary software for online image
either in a simultaneous or staged fashion that is processing and reconstruction, the so-called
used to treat a particular pathology. The goal is to hybrid operating room (. Fig. 26.9) (Jakob et al.
combine minimally invasive surgical techniques 2011).
and/or operative «partial solutions» with catheter-
based interventional procedures to keep the risks 26.4.1.1 Results
to patients as low as possible. This new, controver- First clinical experiences with the hybrid strategy
sial therapy has already found broad clinical were made in coronary revascularization (de
applications in the realm of multivessel coronary, Canniere et al. 2001; Davidavicius et al. 2005). In
valve, and great vessel disease in adults, as well as a multicenter study, the combination of MIDCAB
in various pediatric cardiac surgical diseases. The with PCI for the treatment of multivessel CAD
hybrid approach requires close multidisciplinary was evaluated. Wittwer and colleagues reported
collaboration among various specialties and a that the method was safe and effective to achieve
strategic planning of the procedures (Byrne et al. a complete revascularization and that the
2008). approach is valuable for high-risk and older
In particular, in coronary artery disease patients, as well as for patients requiring cardiac
(CAD), the combination of MIDCAB (mortal- reoperation (Wittwer et al. 2000). The 2-year
ity <1 %) and PCI is gaining increasing popular- results from Davidavicius’ group comparing
ity, particularly for high-risk patients with 2+ hybrid and OPCAB surgery for two-vessel CAD
vessel disease (Shen et al. 2013). Still unresolved showed that the hybrid method is safe and effec-
is the question whether simultaneous or staged tive. Both groups had similar freedom from car-
therapy is preferred. Simultaneous treatment diac symptoms. The perioperative morbidity,
has the advantage of achieving a complete requirement for blood products, pain control,
revascularization, immediate assessment of the and hospital stay were reduced in the hybrid
bypass graft, and a single operative setting for group (Davidavicius et al. 2005).
..Fig. 26.9 Hybrid
operating room
856 J. Garbade et al.
a b
..Fig. 26.10 Telemanipulator. da Vinci System (Intuitive Surgical, Sunnyvale, California, USA): a steering console and
b robotic console (©2015 Intuitive Surgical, Inc)
gical manipulation on the heart. Presently, the approach can occur in a stepwise fashion (level I–
most commonly used system is the da Vinci sys- III) (Falk et al. 1996, 1998; Chitwood et al. 1997a, b)
tem (Intuitive Surgical, . Fig. 26.10). until a complete, robot-assisted closed-chest opera-
The da Vinci System consists of a surgeon con- tion can be achieved (level IV) (Falk et al. 1998).
sole, a patient side cart holding the instruments,
and a vision cart, so that the surgeon can flexibly 26.4.2.1 Results
position various arms (from surgeon console two The AESOP systems are primarily used during
or three instrument arms) with up to seven minimally invasive mitral valve surgery as speech-
degrees of freedom axis for translations and ori- guided stabilizers and instrument-holding sys-
entations, achieving controlled movements within tems. Studies have shown that these systems can
the operative site. The movements of the operator be used safely and effectively (Nifong et al. 2005;
are sensed and converted into synchronous surgi- O’Neill et al. 2007; Bonatti et al. 2012; Bonaros
cal movements through computer algorithms. et al. 2013; Mihaljevic et al. 2013).
The simultaneous control of the endoscopic In 2013, a total of 523,000 procedures had been
camera produces a high-definition 3D (1080i) pic- carried out worldwide with the da Vinci Robotic
ture, which is transmitted from the operative site to System. Essentially, four different pathologies were
the console. The insertion of robotic arms is treated: coronary one-vessel disease (TECAB)—up
achieved through various ports through the body to two-vessel disease, mitral valve repair (MVR),
wall. Advantages of telemanipulator-assisted sur- ASD closure, and atrial fibrillation ablation.
gery, in comparison to endoscopic surgery, include With the TECAB (totally endoscopic coronary
increased degrees of freedom, no transmission of artery bypass surgery) procedure, initially per-
tremors, variable speed of movement transmis- formed in 1998 in Paris and Leipzig (Loulmet
sion, indexing, linear maintenance of strength, and et al. 1999; Mohr et al. 1999), the preparation of
a direct hand-eye coordination (Falk et al. 1998). the left internal thoracic artery and the LIMA-
In accordance with the technical complexity LAD anastomosis were performed totally endo-
and the visual demands, implementation of this scopically on the arrested heart with a closed-chest
858 J. Garbade et al.
26
..Fig. 26.11 Telemanipulator-assisted cardiac surgery. Operative setup (©2015 Intuitive Surgical, Inc)
approach (using the HeartPort technique). The tomosis was carried out using the HeartPort
positioning of the patient is supine, with the left technique and not on the beating heart
hemithorax lightly elevated. Three ports are then (Argenziano et al. 2006; Bonatti et al. 2007). For
placed, forming a triangle (. Fig. 26.11). One port patients with t wo-vessel coronary artery disease,
is in the fourth left intercostal space, medially and a hybrid approach can also be considered
anteriorly. The cranial port is in the third intercos- (Bonatti et al. 2012).
tal space, lateral and more posteriorly located and Telemanipulator-assisted mitral valve repair
the caudal port lies in the sixth or seventh inter- of complex pathologies has already been demon-
costal space, lateral and more posteriorly located. strated in studies by Chitwood and colleagues
For a better exposure and overview, CO2 insuffla- (1997a, b, 2003). In an FDA study of the da Vinci
tion to achieve an intrathoracic pressure of System, various techniques such as quadrangular
6–14 mmHg has also been shown to be helpful. resection, sliding plasty, chordal transfer, chordal
The results of the first prospective multi- replacement, and implantation of anuloplasty
center study with the da Vinci System showed no bands were carried out (Chitwood et al. 2000).
difference with respect to mortality and morbid- With the introduction of new instruments, suture
ity (Argenziano et al. 2006). The angiographic techniques, neochordae and nitinol clips, mitral
patency and reintervention rate were comparable valve reconstruction, and implantation of anulo-
to conventional operation. However, in this plasty bands are possible with good clinical
study, the construction of the LIMA-LAD anas- results. Increased experience has resulted in a
Chapter 26 · Minimally Invasive Cardiac Surgery
859 26
reduction of operative, bypass, and cross clamp be critically evaluated and compared to the existing
times (Reade et al. 2005; Mihaljevic et al. 2013). high standards of modern cardiac surgery.
With respect to atrial fibrillation ablation, the
MAZE procedure represents one of the most effec-
tive surgical techniques, whereby the left and right References
atria must be opened. Independent of which energy
source is used (radiofrequency or cryotherapy), a Abu Hazeem AA, Gillespie MJ, Thun H, Munson D, Schwartz
MC, Dori Y, Rome JJ, Glatz AC (2013) Percutaneous clo-
safe and effective therapy can be performed via a
sure of patent ductus arteriosus in small infants with
right lateral mini-thoracotomy with connection to significant lung disease may offer faster recovery of
ECC via the femoral vessels. Commonly, this pro- respiratory function when compared to surgical liga-
cedure is performed in conjunction with mitral or tion. Catheter Cardiovasc Interv. doi:10.1002/
tricuspid valve surgery (Seeburger et al. 2008; ccd.25032, PMID: 23723091
Aggarwal S, Pagani FD (2010) Bridge to transplantation:
Akpinar et al. 2003). Totally endoscopic techniques
current outcomes. J Card Surg 25:455–461
for isolated pulmonary vein ablation on the beating Akintürk H, Michel-Behnke I, Valeske K et al. (2002) Stenting
heart (Saltman et al. 2003; Cheema et al. 2009), but of the arterial duct and banding of the pulmonary
also systems for ablation on the beating heart, have arteries: basis for combined Norwood stage I and II
been described (Cheema et al. 2009). repair in hypoplastic left heart. Circulation 105:1099–
1103
Garrido reported successful use of the da
Akpinar B, Guden M, Sagbas E, Sanisoglu I, Ozbek U,
Vinci System to treat atrial fibrillation (Garrido Caynak B, Bayindir O (2003) Combined radiofrequency
et al. 2004). Interventional therapy of atrial fibril- modified maze and mitral valve procedure through a
lation represents an alternate therapeutic strategy. port access approach: early and mid-term results. Eur
This approach involves focused electrical isola- J Cardiothorac Surg 24:223–230
Arcidi JM Jr, Rodriguez E, Elbeery JR, Nifong LW, Efird JT,
tion in the right and left atria, and the pulmonary
Chitwood WR Jr (2012) Fifteen-year experience with
veins, and has been associated with very good minimally invasive approach for reoperations involv-
clinical results but also serious complications ing the mitral valve. J Thorac Cardiovasc Surg
(O’Neill et al. 2007) (see also 7 chapter «Surgical 143:1062–1068
Therapy for Atrial Fibrillation», Sect. 29.6.5). Argenziano M, Katz M, Bonatti J, Srivastava S, Murphy D,
Poirier R, Loulmet D, Siwek L, Kreaden U, Ligon D,
TECAB Trial Investigators (2006) Results of the pro-
26.4.2.2 Conclusion
spective multicenter trial of robotically assisted totally
The application of computer-assisted telemanipula- endoscopic coronary artery bypass grafting. Ann
tion techniques represents a new level in cardiac Thorac Surg 81:1666–1674
surgery. As with each type of breakthrough, this Autschbach R, Walther T, Falk V, Diegeler A, Metz S, Mohr
FW (1998) S-shaped in comparison to L-shaped partial
technique also has its drawbacks and has not yet
sternotomy for less invasive aortic valve replacement.
become routine practice worldwide. Meanwhile, in Eur J Cardiothorac Surg 14(Suppl 1):S117–S121
the USA this technique has become a routine Aziz O, Athanasiou T, Panesar SS, Massey-Patel R, Warren O,
approach for isolated MVR. However, in many cen- Kinross J, Purkayastha S, Casula R, Glenville B, Darzi A
ters, the combination of endoscopic techniques has (2005) Does minimally invasive vein harvesting tech-
nique affect the quality of the conduit for coronary
become the standard for minimally invasive proce-
revascularization? Ann Thorac Surg 80:2407–2414
dures, whether this is for coronary artery bypass Bacchetta MD, Korst RJ, Altorki NK, Port JL, Isom OW, Mack
conduit harvest or complex valve surgery. The rapid CA (2003) Resection of a symptomatic pericardial cyst
technical developments, the creation of new instru- using the computer-enhanced da Vinci Surgical
ments, and the evolving areas of imaging and image System. Ann Thorac Surg 75:1953–1955
Bacha EA, Hijazi ZM, Cao QL, Abdulla R, Starr JP, Quinones
processing make telemanipulator-assisted surgery
J, Koenig P, Agarwala B (2005) Hybrid pediatric cardiac
one of the most interesting areas of minimally inva- surgery. Pediatr Cardiol 26:315–322
sive cardiac surgery. However, there are significant Bacha EA, Marshall AC, McElhinney DB, del Nido PJ (2007)
associated material and time requirements that Expanding the hybrid concept in congenital heart sur-
need to be considered. In addition, intensive train- gery. Semin Thorac Cardiovasc Surg Pediatr Card Surg
Annu 146–50
ing, simulation of potential operative situations,
Bailey CP (1949) The surgical treatment of mitral stenosis
mastery of all tools and acceptance of a new percep- (mitral commisurotomy). Dis Chest 15:377
tion of the operative field, as well as a technically Baker C, Brock RC, Campbell M (1950) Valvulotomy for
accomplished and creative surgeon are required. mitral stenosis: report of six successful cases. Br Med
Despite all the excitement, each technique should J 1(4665):1283–93
860 J. Garbade et al.
Bakir I, Casselman FP, Wellens F, Jeanmart H, De Geest R, Boodhwani M, Rubens FD, Sellke FW, Mesana TG, Ruel M
Degrieck I, Van Praet F, Vermeulen Y, Vanermen H (2006) Mortality and myocardial infarction following
(2006) Minimally invasive versus standard approach surgical versus percutaneous revascularization of iso-
aortic valve replacement: a study in 506 patients. Ann lated left anterior descending artery disease: a meta-
Thorac Surg 81:1599–1604 analysis. Eur J Cardiothorac Surg 29:65–70
Barbero-Marcial M, Tanamati C, Jatene MB, Atik E, Jatene Brasil LA, Gomes WJ, Salomao R, Buffolo E (1998)
AD (1998) Transxiphoid approach without median Inflammatory response after myocardial revasculariza-
sternotomy for the repair of atrial septal defects. Ann tion with or without cardiopulmonary bypass. Ann
Thorac Surg 65:771–774 Thorac Surg 66:56–59
Bartakian S, Fagan TE, Schaffer MS, Darst JR (2012) Device Brunton L, Edin MD (1902) Preliminary note on the possi-
26 closure of secundum atrial septal defects in children bility of treating mitral stenosis by surgical methods.
<15 kg: complication rates and indications for referral. Lancet 1:352
JACC Cardiovasc Interv 5:1178–1184 Buffolo E (1991) Coronary surgery without extracorporeal
Becit N, Unlu Y, Ceviz M, Kocogullari CU, Kocak H, Gurlertop circulation. Eur J Cardiothorac Surg 5:223
Y (2005) Subxiphoid pericardiostomy in the manage- Byrne JG, Leacche M, Vaughan DE, Zhao DX (2008) Hybrid
ment of pericardial effusions: case series analysis of cardiovascular procedures. JACC Cardiovasc Interv
368 patients. Heart 91:785–790 1:459–468
Benetti FJ (1985) Direct coronary surgery with saphenous Calafiore AM, Giammarco GD, Teodori G, Bosco G,
vein bypass without either cardiopulmonary bypass or D’Annunzio E, Barsotti A, Maddestra N, Paloscia L, Vitolla
cardiac arrest. J Cardiovasc Surg (Torino) 26:217–222 G, Sciarra A, Fino C, Contini M (1996) Left anterior
Benetti FJ, Ballester C (1995) Use of thoracoscopy and a descending coronary artery grafting via left anterior
minimal thoracotomy, in mammary-coronary bypass small thoracotomy without cardiopulmonary bypass.
to left anterior descending artery, without extracorpo- Ann Thorac Surg 61(6):1658–1663
real circulation. Experience in 2 cases. J Cardiovasc Carpentier A, Loulmet D, Carpentier A, Le Bret E, Haugades
Surg (Torino) 36:159–161 B, Dassier P, Guibourt P (1996) Open heart operation
Benetti FJ, Naselli G, Wood M, Geffner L (1991) Direct myo- under videosurgery and minithoracotomy. First case
cardial revascularization without extracorporeal cir- (mitral valvuloplasty) operated with success. C R Acad
culation. Experience in 700 patients. Chest Sci III 319:219–223
100:312–316 Casselman FP, La Meir M, Jeanmart H, Mazzarro E, Coddens
Ben-Gal Y, Stone GW, Smith CR, Williams MR, Weisz G, J, Van Praet F, Vermeulen Y, Vanermen H (2007a)
Stewart AS, Takayama H, Genereux P, Argenziano M Endoscopic mitral valve and tricuspid valve surgery
(2011) On-pump versus off-pump surgical revascular- after previous cardiac surgery. Circulation 118:I270–
ization in patients with acute coronary syndromes: I275
analysis from the Acute Catheterization and Urgent Casselman FP, La Meir M, Jeanmart H, Mazzarro E, Coddens
Intervention Triage Strategy trial. J Thorac Cardiovasc J, Van Praet F, Wellens F, Vermeulen Y, Vanermen H
Surg 142:e33–e39 (2007b) Endoscopic mitral and tricuspid valve surgery
Blazek S, Holzhey D, Jungert C, Borger MA, Fuernau G, after previous cardiac surgery. Circulation 116:I270–
Desch S, Eitel I, de Waha S, Lurz P, Schuler G, Mohr FW, I275
Thiele H (2013) Comparison of bare-metal stenting Cheema FH, Weisberg JS, Khalid I, Roberts HG Jr (2009)
with minimally invasive bypass surgery for stenosis of Warm beating heart, robotic endoscopic Cox-
the left anterior descending coronary artery: 10-year cryomaze: an approach for treating atrial fibrillation.
follow-up of a randomized trial. JACC Cardiovasc Ann Thorac Surg 87:966–968
Interv 6:20–26 Cheung A, Lamarche Y, Kaan A et al. (2011) Off-pump
Bonaros N, Schachner T, Lehr E, Kofler M, Wiedemann D, implantation of the HeartWare HVAD left ventricular
Hong P, Wehman B, Zimrin D, Vesely MK, Friedrich G, assist device through minimally invasive incisions.
Bonatti J (2013) Five hundred cases of robotic totally Ann Thorac Surg 91:1294–1296
endoscopic coronary artery bypass grafting: predic- Cheung A, Webb JG, Barbanti M, Freeman M, Binder RK,
tors of success and safety. Ann Thorac Surg 95:803– Thompson C, Wood DA, Ye J (2013) 5-year experience
812 with transcatheter transapical mitral valve-in-valve
Bonatti J, Schachner T, Bonaros N, Ohlinger A, Rutzler E, implantation for bioprosthetic valve dysfunction. J Am
Feuchtner G, Kolbitsch C, Friedrich G, Bartel T, Coll Cardiol 61:1759–1766
Pachinger O, Laufer G (2007) Robotic totally endo- Chitwood WR Jr, Elbeery JR, Chapman WH, Moran JM,
scopic double-vessel bypass grafting: a further step Lust RL, Wooden WA, Deaton DH (1997a) Video-
toward closed-chest surgical treatment of multivessel assisted minimally invasive mitral valve surgery: the
coronary artery disease. Heart Surg Forum 10:E239– “micro-mitral” operation. J Thorac Cardiovasc Surg
E242 113:413–414
Bonatti JO, Zimrin D, Lehr EJ, Vesely M, Kon ZN, Wehman B, Chitwood WR Jr, Elbeery JR, Moran J (1997b) Minimally
de Biasi AR, Hofauer B, Weidinger F, Schachner T, invasive mitral valve repair using transthoracic aortic
Bonaros N, Friedrich G (2012) Hybrid coronary revas- occlusion. Ann Thorac Surg 63:1477–1479
cularization using robotic totally endoscopic surgery: Chitwood WR Jr, Nifong LW, Elbeery JE, Chapman WH,
perioperative outcomes and 5-year results. Ann Thorac Albrecht R, Kim V, Young JA (2000) Robotic mitral valve
Surg 94:1920–1926 repair: trapezoidal resection and prosthetic annuloplasty
Chapter 26 · Minimally Invasive Cardiac Surgery
861 26
with the da Vinci surgical system. J Thorac Cardiovasc Divekar A, Gaamangwe T, Shaikh N, Raabe M, Ducas
Surg 120:1171–1172 J (2005) Cardiac perforation after device closure of
Chitwood WR Jr, Kypson AP, Nifong LW (2003) Robotic atrial septal defects with the Amplatzer septal
mitral valve surgery: a technologic and economic rev- occluder. J Am Coll Cardiol 45:1213–1218
olution for heart centers. Am Heart Hosp J 1:30–39 Dogan S, Aybek T, Risteski PS, Detho F, Rapp A, Wimmer-
Connolly MW, Torrillo LD, Stauder MJ, Patel NU, McCabe JC, Greinecker G, Moritz A (2005) Minimally invasive port
Loulmet DF, Subramanian VA (2002) Endoscopic radial access versus conventional mitral valve surgery: pro-
artery harvesting: results of first 300 patients. Ann spective randomized study. Ann Thorac Surg 79:492–498
Thorac Surg 74:502–505 Doll N, Walther T, Falk V, Binner C, Bucerius J, Borger MA,
Cosgrove DM 3rd, Sabik JF (1996) Minimally invasive Gummert JF, Mohr FW, Kostelka M (2003) Secundum
approach for aortic valve operations. Ann Thorac Surg ASD closure using a right lateral minithoracotomy:
62:596–597 five-year experience in 122 patients. Ann Thorac Surg
Cosgrove DM 3rd, Sabik JF, Navia JL (1998) Minimally inva- 75:1527–1530
sive valve operations. Ann Thorac Surg 65:1535–1538 Doty DB, DiRusso GB, Doty JR (1998) Full-spectrum cardiac
Cremer JT, Boning A, Anssar MB, Kim PY, Pethig K, Harringer surgery through a minimal incision: mini-sternotomy
W, Haverich A (1999) Different approaches for mini- (lower half ) technique. Ann Thorac Surg 65:573–577
mally invasive closure of atrial septal defects. Ann Du ZD, Hijazi ZM, Kleinman CS, Silverman NH, Larntz K,
Thorac Surg 67:1648–1652 Amplatzer Investigators (2002) Comparison between
Cutler EC, Levine SA (1923) Cardiotomy and valvulotomy transcatheter and surgical closure of secundum atrial
for mitral stenosis: experimental observations and septal defect in children and adults: results of a multi-
clinical notes concerning an operated case with recov- center nonrandomized trial. J Am Coll Cardiol
ery. Boston Med Surg J 188:1023 39:1836–1844
Davidavicius G, Van Praet F, Mansour S, Casselman F, Dubost C, Guilmet D, de Parades B, Pedeferri G (1966) New
Bartunek J, Degrieck I, Wellens F, De Geest R, Vanermen technic of opening of the left auricle in open-heart
H, Wijns W, De Bruyne B (2005) Hybrid revasculariza- surgery: the transseptal bi-auricular approach. Presse
tion strategy: a pilot study on the association of robot- Med 74:1607–1608
ically enhanced minimally invasive direct coronary Eggebrecht H, Schäfer U, Treede H, Boeckstegers P, Babin-
artery bypass surgery and fractional-flow-reserve- Ebell J, Ferrari M, Möllmann H, Baumgartner H, Carrel
guided percutaneous coronary intervention. T, Kahlert P, Lange P, Walthet T, Erbel R, Mehta RH,
Circulation 112:I317–I322 Thielmann M (2011) Transcatheter valve-in-valve
de Canniere D, Jansens JL, Goldschmidt-Clermont P, implantation for deteriorated aortic bioprosthesis: ini-
Barvais L, Decroly P, Stoupel E (2001) Combination of tial clinical results and follow-up in a series of high risk
minimally invasive coronary bypass and percutaneous patients. JACC Cardiovasc Interv 4:1218–1227
transluminal coronary angioplasty in the treatment of Eggebrecht H, Schmermund A, Voigtländer T, Kahlert P,
double-vessel coronary disease: two-year follow-up of Erbel R, Matha RH (2012) Risk of stroke after transcath-
a new hybrid procedure compared with “on-pump” eter aortic valve implantation (TAVI): a meta-analysis of
double bypass grafting. Am Heart J 142:563–570 10,037 published patients. EuroIntervention 8:129–138
De Mulder W, Vanermen H (2002) Repair of atrial septal Emmert MY, Seifert B, Wilhelm M, Grünenfelder J, Falk V,
defects via limited right anterolateral thoracotomy. Salzberg SP (2011) Aortic no-touch technique makes
Acta Chir Belg 102:450–454 the difference in off-pump coronary artery bypass
Deng MC, Dasch B, Erren M, Mollhoff T, Scheld HH, Deng grafting. J Thorac Cardiovasc Surg 142:1499–1506
MC, Dasch B, Erren M, Mollhoff T, Scheld HH (1996) Etienne PY, D’hoore W, Papadatos S, Mairy Y, El Khoury G,
Impact of left ventricular dysfunction on cytokines, Noirhomme P, Hanet C, Glineur D (2013) Five-year fol-
hemodynamics, and outcome in bypass grafting. Ann low-up of drug-eluting stents implantation vs mini-
Thorac Surg 62:184–190 mally invasive direct coronary artery bypass for left
Dewey TM, Walther T, Doss M, Brown D, Ryan WH, Svensson anterior descending artery disease: a propensity score
L, Mihaljevic T, Hambrecht R, Schuler G, Wimmer- analysis. Eur J Cardiothorac Surg 44:884–890
Greinecker G, Mohr FW, Mack MJ (2006) Transapical EVEREST Registry (2007) Significant reduction in mitral
aortic valve implantation: an animal feasibility study. regurgitation 12 months following percutaneous
Ann Thorac Surg 82:110–116 mitral valve repair: initial experience with the MitraClip
Diegeler A, Thiele H, Falk V, Hambrecht R, Spyrantis N, Sick device. Clin Cardiol 30:419
P, Diederich KW, Mohr FW, Schuler G (2002) Fabricius AM, Oser A, Diegeler A, Rauch T, Mohr FW (2000)
Comparison of stenting with minimally invasive Endothelial function of human vena saphena magna
bypass surgery for stenosis of the left anterior prepared with different minimally invasive harvesting
descending coronary artery. N Engl J Med 347:561– techniques. Eur J Cardiothorac Surg 18:400–403
566 Falk V, Walther T, Diegeler A, Wendler R, Autschbach R, van
Diegeler A, Börgermann J, Kappert U, Breuer M, Böning A, Son JA, Siegel LC, Pompilli MF, Mohr FW (1996)
Ursulescu A, Rastan A, Holzhey D, Treede H, Rieß FC, Echocardiographic monitoring of minimally invasive
Veeckmann P, Asfoor A, Reents W, Zacher M, Hilker M, mitral valve surgery using an endoaortic clamp.
GOPCABE Study Group (2013) Off-pump versus on- J Heart Valve Dis 5:630–637
pump coronary-artery bypass grafting in elderly Falk V, Walther T, Autschbach R, Diegeler A, Battellini R,
patients. N Engl J Med 368:1189–1198 Mohr FW (1998) Robot-assisted minimally invasive
862 J. Garbade et al.
solo mitral valve operation. J Thorac Cardiovasc Surg Glower D, Ailawadi G, Argenziano M, Mack M, Trento A,
15:470–471 Wang A, Lim DS, Gray W, Grayburn P, Dent J, Gillam L,
Feldman T, Kar S, Rinaldi M, Fail P, Hermiller J, Smalling R, Sethuraman B, Feldman T, Foster E, Mauri L, Kron I,
Whitlow PL, Gray W, Low R, Herrmann HC, Lim S, Foster EVEREST II Investigators (2012) EVEREST II randomized
E, Glower D, EVEREST Investigators (2009) clinical trial: predictors of mitral valve replacement in
Percutaneous mitral repair with the MitraClip system: de novo surgery or after the MitraClip procedure.
safety and midterm durability in the initial EVEREST J Thorac Cardiovasc Surg 143:S60–S63
(Endovascular Valve Edge-to-Edge REpair Study) Gomes WJ, Erlichman MR, Batista-Filho ML, Knobel M,
cohort. J Am Coll Cardiol 54:686–694 Almeida DR, Carvalho AC, Catani R, Buffolo E (2003)
Feldman T, Foster E, Glower DD, Kar S, Rinaldi MJ, Fail PS, Vasoplegic syndrome after off-pump coronary
26 Smalling RW, Siegel R, Rose GA, Engeron E, Loghin C, artery bypass surgery. Eur J Cardiothorac Surg
Trento A, Skipper ER, Fudge T, Letsou GV, Massaro JM, 23:165–169
Mauri L, EVEREST II Investigators (2011) Percutaneous Gorlitzer M, Wagner F, Pfeiffer S, Folkmann S, Meihart J,
repair or surgery for mitral regurgitation. N Engl J Med Fischlein T, Reichenspurner H, Grabenwoeger M (2013)
364:1395–1406 Prevention of sternal wound complication after ster-
Francis GS, Greenberg BH, Hsu DT et al. (2010) ACCF/AHA/ notomy: results of a large prospective randomized
ACP/HFSA/ISHLT 2010 Clinical competence statement multicentre trial. Interact Cardiovasc Thorac Surg
on management of patients with advanced heart failure 17:515–522
and cardiac transplant. J Am Coll Cardiol 56:424–453 Gregoric ID, Cohn WE, Frazier OH (2011) J Heart Lung
Franzoni I, Latib A, Maisano F, Costopoulus C, Testa L, Figin Transplant 30:467–470
F, Giannini F, Basavarajaiah S, Mussardo M, Slavich M, Griffith BP, Kormos RL, Borovetz HS et al. (2001) Heart Mate
Taramasso M, Cioni M, Longoni M, Ferrarelo S, II left ventricular assist device system: from concept to
Radinovic A, Sala S, Ajello S, Sticchi A, Giglio M, first clinical use. Ann Thorac Surg 71:S116–S120
Agricola E, Chieffo A, Montorfano M, Alferie O, Gross RE, Hubbard JP (1939) Surgical ligation of a patent
Colombo A (2013) Comparison of incidence and pre- ductus arteriosus. Report of first successful case. JAMA
dictors of left bundle branch block after trancatheter 112:729
aortic valve implantation using CoreValve versus Grossi EA, Groh MA, Lefrak EA, Ribakove GH, Albus RA,
Edwards valve. Am J Cardiol Galloway AC, Colvin SB (1999) Results of a prospective
doi:pii:S0002-9149(13)01000-x multicenter study on port-access coronary bypass
Fraund S, Herrmann G, Witzke A, Hedderich J, Lutter G, grafting. Ann Thorac Surg 68:1475–1477
Brandt M, Böning A, Cremer J (2005) Midterm follow- Grube E, Schuler G, Buellesfeld L, Gerckens U, Linke A,
up after minimally invasive direct coronary artery
Wenaweser P, Sauren B, Mohr FW, Walther T, Zickmann
bypass grafting versus percutaneous coronary inter- B, Iversen S, Felderhoff T, Cartier R, Bonan R (2007)
vention techniques. Ann Thorac Surg 79:1225–1231 Percutaneous aortic valve replacement for severe aor-
Funkat AK, Beckmann A, Lewandowski J, Frie M, Schiller W, tic stenosis in high-risk patients using the second- and
Ernst M, Hekmat K, Gummert JF, Mohr FW (2012) current third-generation self-expanding CoreValve
Cardiac surgery in Germany during 2011: a report on prosthesis: device success and 30-day clinical out-
behalf of the German Society for Thoracic and come. J Am Coll Cardiol 50:69–76
Cardiovascular Surgery. Thorac Cardiovasc Surg Guiraudon GM, Ofiesh JG, Kaushik R (1991) Extended verti-
60:371–382 cal transatrial septal approach to the mitral valve. Ann
Galantowicz M, Cheatham JP, Phillips A et al. (2008) Hybrid Thorac Surg 52:1058–1060
approach for hypoplastic left heart syndrome: Gulielmos V, Brandt M, Knaut M, Cichon R, Wagner FM,
intermediate results after the learning curve. Ann
Kappert U, Schuler S (1999) The Dresden approach for
Thorac Surg 85:2063–2071 complete multivessel revascularization. Ann Thorac
Garbade J, Seeburger J, Merk DR, Pfannmüller B, Vollroth Surg 68:1502–1505
M, Barten MJ, Borger MA, Mohr FW (2013) Mitral valve Gummert JF, Barten MJ, Hans C, Kluge M, Doll N, Walther T,
pathology in severely impaired left ventricles can be Hentschel B, Schmitt DV, Mohr FW, Diegeler A (2002)
successfully managed using a right-sided minimally Mediastinitis and cardiac surgery – an updated risk
invasive surgical approach. Eur J Cardiothorac Surg factor analysis in 10,373 consecutive adult patients.
44:e1–e7 Thorac Cardiovasc Surg 50:87–91
Garrido MJ, Williams M, Argenziano M (2004) Minimally Gummert JF, Funkat A, Beckmann A, Schiller W, Hekmat K,
invasive surgery for atrial fibrillation: toward a totally Ernst M, Krian A (2007) Cardiac surgery in Germany
endoscopic, beating heart approach. J Card Surg during 2006: a report on behalf of the German Society
19:216–220 for Thoracic and Cardiovascular Surgery. Thorac
Georghiou GP, Stamler A, Sharoni E, Fichman-Horn S, Cardiovasc Surg 55:343–350
Berman M, Vidne BA, Saute M (2005) Video-assisted Harken DW, Ellis LB, Ware PF, Norman LR (1948) The surgi-
thoracoscopic pericardial window for diagnosis and cal treatment of mitral stenosis I. Valvuloplatsy. N Eng
management of pericardial effusions. Ann Thorac Surg J Med 239:801–809
80:607–610 Hetzer R, Krabatsch T, Stepanenko A et al. (2010) Long-
Gibbon JH (1953) Application of a mechanical heart term biventricular support with the heartware
and lung apparatus to cardiac surgery. Minn Med implantable continuous flow pump. J Heart Lung
37:171 Transplant 29:822–824
Chapter 26 · Minimally Invasive Cardiac Surgery
863 26
Holzhey DM, Cornely JP, Rastan AJ, Davierwala P, Mohr FW Kim JB, Ha JW, Kim JS, Shim WH, Kang SM, Ko YG, Choi D,
(2012) Review of 13-year single-center experience Jang Y, Chung N, Cho SY, Kim SS (2007) Comparison of
with minimally invasive direct coronary artery bypass long-term outcome after mitral valve replacement or
as the primary surgical treatment of coronary artery repeated balloon mitral valvotomy in patients with
disease. Heart Surg Forum 15:E61–E68 restenosis after previous balloon valvotomy. Am
Holzhey DM, Seeburger J, Misfeld M, Borger MA, Mohr FW J Cardiol 99:1571–1574
(2013) Learning minimally invasive mitral valve sur- King TD, Thompson SL, Steiner C, Mills NL (1976) Secundum
gery: a cumulative sum sequential probability analysis atrial septal defect. Nonoperative closure during car-
of 3895 operations from a single high volume center. diac catheterization. JAMA 235:2506–2509
Circulation 128:483–491 Kölbel T, Reiter B, Schirmer J, Wipper S, Detter C, Debus ES,
Hongxin L, Wenbin G, Lijun S, Zhengjun W, Hao L, Chengwei Reichenspurner H (2013) Customized transapical tho-
Z, Liang D, Guidao Y (2007) Intraoperative device clo- racic endovascular repair for acute type A dissection.
sure of secundum atrial septal defect with a right ante- Ann Thorac Surg 95:694–696
rior minithoracotomy in 100 patients. J Thorac Kolessov VI (1967) Mammary artery-coronary artery anas-
Cardiovasc Surg 134:946–951 tomosis as method of treatment for angina pectoris.
Horvath KA, Burke RP, Collins JJ Jr, Cohn LH (1992) Surgical J Thorac Cardiovasc Surg 54:535–544
treatment of adult atrial septal defect: early and long- Konertz W, Waldenberger F, Schmutzler M, Ritter J, Liu
term results. J Am Coll Cardiol 20:1156–1159 J (1996) Minimal access valve surgery through supe-
Jakob H, Tsagakis K, Dohle DS, Kottenberg E, Konorza T, rior partial sternotomy: a preliminary study. J Heart
Janosi RA, Erbel R (2011) Hybrid room technology as a Valve Dis 5:638–640
prerequisite for the modern therapy of aortic dissec- Kotowycz MA, Therrien J, Ionescu-Ittu R, Owns CG, Pilote L,
tion. Herz 36(6):525–530 Martucci G, Tchervenkov C, Marelli AJ (2013) Long-
Kadner A, Dave H, Dodge-Khatami A, Bettex D, term outcome after surgical versus transcatheter clo-
Vasangiacomo-Buechel E, Turina MI, Prêtre R (2004) sure of atrial septal defects in adult. JACC Cardiovasc
Inferior partial sternotomy for surgical closure of iso- Interv 6:497–503
lated ventricular septal defects in children. Heart Surg Laborde F, Noirhomme P, Karam J, Batisse A, Bourel P, Saint
Forum 7:E467–E470 Maurice O (1993) A new video-assisted thoracoscopic
Kang WC, Shin EK, Park CH, Kang JM, Ko YG, Choi D, Youn surgical technique for interruption of patient ductus
YN, Shim WH (2013) Hybrid endovascular repair for arteriosus in infants and children. J Thorac Cardiovasc
aortic arch pathology: intermediate outcomes and Surg 105:278–280
complications: a retrospective analysis. Cathet Lamy A, Devereaux PJ, Prabhakaran D, Taggart DP, Hu S,
Cardiovasc Interv. doi:10.1002/ccd.24384 Paolasso E, Straka Z, Piegas LS, Akar AR, Jain AR,
Kappetein AP, Head SJ, Généreux P, Piazza N, van Mieghem Noiseux N, Padmanabhan C, Bahamondes JC, Novick
NM, Blackstone EH, Brott TG, Cohen DJ, Cutlip DE, van Es RJ, Vaijyanath P, Reddy S, Tao L, Olavegogeascoechea
GA, Hahn RT, Kirtane AJ, Krucoff MW, Kodali S, Mack MJ, PA, Airan B, Sulling TA, Whitlock RP, Ou Y, Ng J,
Mehran R, Rodés-Cabau J, Vranckx P, Webb JG, Windecker Chrolavicius S, Yusuf S, CORONARY Investigators
S, Serruys PW, Leon M, Valve Academic Research (2012) Off-pump or on-pump coronary-artery bypass
Consortium-2 (2013) Updated standardized endpoint grafting at 30 days. N Engl J Med 366:1489–1497
definitions for transcatheter aortic valve implantation: Landreneau RJ, Mack MJ, Hazelrigg SR, Dowling RD, Acuff
the Valve Academic Research Consortium-2 consensus TE, Magee MJ, Ferson PF (1992) Video-assisted tho-
document. J Thorac Cardiovasc Surg 145:6–23 racic surgery: basic technical concepts and intercostal
Keeling WB, Kilgo PD, Puskas JD, Halkos ME, Lattouf OM, approach strategies. Ann Thorac Surg 54:800–807
Guyton RA, Thourani VH (2012) Off-pump coronary Lichtenstein SV, Cheung A, Ye J, Thompson CR, Carere RG,
artery bypass grafting attenuates morbidity and mor- Pasupati S, Webb JG (2006) Transapical transcatheter
tality for patients with low and high body mass index. aortic valve implantation in humans: initial clinical
J Thorac Cardiovasc Surg doi:10.1016/j.jtcvs.2012.09.035. experience. Circulation 114:591–596
pii: S0022-5223(12)01147-6 Lietz K, Miller LW (2009) Patient selection for left-
Keeling WB, Williams ML, Slaughter MS, Zhao Y, Puskas JD ventricular assist devices. Curr Opin Cardiol 24:246–
(2013) Off-pump and on-pump coronary revascular- 251
ization in patients with low ejection fraction: a report Lillehei CW, Gott VL, Dewall RA, Varco RL (1957) Surgical
from the Society of Thoracic Surgeons National correction of pure mitral insufficiency by annuloplasty
Database. Ann Thorac Surg doi:pii: S0003- under direct vision. J Lancet 77:446–449
4975(13)00837-0 Lin PJ, Chang CH, Chu JJ, Liu HP, Tsai FC, Su WJ, Yang MW,
Kempfert J, Girrbach F, Haensig M, Subramanian S, Holzhey Tan PP (1998) Minimally invasive cardiac surgical tech-
DM, Mohr FW (2013) Transapical valve-in-valve-in- niques in the closure of ventricular septal defect: an
valve implantation as a procedural rescue option. Ann alternative approach. Ann Thorac Surg 65:165–169
Thorac Surg 95:325–328 Lin PH, Dardik A, Coselli JS (2007) A simple technique to facil-
Khoshbin E, Prayaga S, Kinsella J, Sutherland FW (2011) itate antegrade thoracic endograft deployment using a
Mini-sternotomy for aortic valve replacement reduces hybrid elephant trunk procedure under hypothermic
length of stay in the cardiac intensive care unit: meta- circulatory arrest. J Endovasc Ther 14(5):669–671
analysis of randomised controlled trials. BMJ Open Liu G, Qiao Y, Ma L, Ni L, Zeng S, Li Q (2013) Totally thoraco-
1:e000266. doi:10.1136/bmjopen-2011-000266 scopic surgery for the treatment of atrial septal defect
864 J. Garbade et al.
without the robotic Da Vinci surgical system. Murashita T, Hatta E, Miyatake T, Kubota T, Sasaki S, Yasuda
J Cardiothorac Surg 8:119 K (1999) Partial median sternotomy as a minimal
Loforte A, Montalto A, Monica PLD et al. (2010) Biventricular access for the closure of subarterial ventricular septal
support with the HeartWare implantable continuous defect. Feasibility of transpulmonary approach. Jpn
flow pump: an additional contribution. J Heart Lung J Thorac Cardiovasc Surg 47:440–444
Transplant 29:1443–1444 Navia JL, Cosgrove DM 3rd (1996) Minimally invasive mitral
Loforte A, Monica PLD, Montalto A et al. (2011) HeartWare valve operations. Ann Thorac Surg 62:1542–1544
third-generation implantable continuous flow pump Nifong LW, Chitwood WR, Pappas PS, Smith CR, Argenziano
as biventricular support: mid-term follow-up. Interact M, Starnes VA, Shah PM (2005) Robotic mitral valve
Cardiovasc Thorac Surg 12:458–460 surgery: a United States multicenter trial. J Thorac
26 Loulmet D, Carpentier A, d’Attellis N, Berrebi A, Cardon C, Cardiovasc Surg 129:1395–1404
Ponzio O, Aupecle B, Relland JY (1999) Endoscopic Nifong LW, Rodriguez E, Chitwood WR Jr (2012) 540 con-
coronary artery bypass grafting with the aid of robotic secutive robotic mitral valve repairs including con-
assisted instruments. J Thorac Cardiovasc Surg 118:4– comitant atrial fibrillation cryoablation. Ann Thorac
10 Surg 94:38–42
Lower R (1669) Tractus de corde item de moto, et colore Noyes BE, Weber T, Vogler C (2003) Pericardial cysts in chil-
sanguinis et chyli in eum trasitu. J Allestry, London, dren: surgical or conservative approach? J Pediatr
p 104 Surg 38:1263–1265
Luo W, Chang C, Chen S (2001) Ministernotomy versus full O’Neill MD, Jais P, Hocini M, Sacher F, Klein GJ, Clementy J,
sternotomy in congenital heart defects: a prospective Haissaguerre M (2007) Catheter ablation for atrial
randomized study. Ann Thorac Surg 71:473–475 fibrillation. Circulation 116:1515–1523
Mack MJ, Aronoff RJ, Acuff TE, Douthit MB, Bowmann RT, Palmer G, Herbert MA, Prince SL, Williams JL, Magee MJ,
Ryan WY (1992) Present role of thoracoscopy in the Brown P, Katz M, Mack MJ (2007) Coronary Artery
diagnosis and treatment of diseases of the chest. Ann Revascularization (CARE) registry: an observational
Thorac Surg 54:403–408 study of on-pump and off-pump coronary artery
Marui A, Okabayashi H, Komiya T, Tanaka S, Furukawa Y, revascularization. Ann Thorac Surg 83:986–992
Kita T, Kimura T, Sakata R, CREDO-Kyoto Investigators Patel AN, Henry AC, Hunnicutt C, Cockerham CA, Willey B,
(2012) Benefits of off-pump coronary artery bypass Urschel HC Jr (2004) Endoscopic radial artery harvest-
grafting in high-risk patients. Circulation 126:S151– ing is better than the open technique. Ann Thorac
S157 Surg 78:149–153
McGoon DC (1960) Repair of mitral insufficiency due to Pereira E, Ferreira N, Caeiro D, Primo J, Adäo L, Olveira M,
ruptured chordae tendinae. J Thorac Cardiovasc Surg Concalves H, Ribeiro J, Santos E, Leite D, Bettencourt
39:357 N, Braga P, Simöes L, Vouga L, Gama V (2013)
Mehall JR, Kohut RM Jr, Schneeberger EW, Taketani T, Merrill Transcatheter aortic valve implantation and require-
WH, Wolf RK (2007) Intraoperative epicardial electro- ments of pacing over time. Pacing Clin Electrophysiol
physiologic mapping and isolation of autonomic gan- 36:559–569
glionic plexi. Ann Thorac Surg 83:538–541 Pilgrim T, Kalesan B, Wenaweser P, Huber C, Stortecky S,
Meyer SR, Szeto WY, Augoustides JG, Morris RJ, Vernick WJ, Buellesfeld L, Khattab AA, Eberle B, Gloeckler S,
Paschal D, Fox J, Hargrove WC 3rd (2009) Reoperative Gsponer T, Meier B, Jüni P, Carrel T, Windecker S (2012)
mitral valve surgery by the port access minithoracot- Predictors of clinical outcome in patients with severe
omy approach is safe and effective. Ann Thorac Surg aortic stenosis undergoing TAVI: a multitaste analysis.
87:1426–1430 Circ Cardiovasc Interv 5:856–861
Mihaljevic T, Pattakos G, Gillinov AM, Bajwa G, Planinc M, Pinto RJ, Dalvi BV, Sharma S (2006) Transcatheter closure of
Williams SJ, Blackstone EH (2013) Robotic posterior perimembranous ventricular septal defects using
mitral leaflet repair: neochordal versus resectional amplatzer asymmetric ventricular septal defect
techniques. Ann Thorac Surg 95:787–794 occluder: preliminary experience with 18-month fol-
Mills SA, Julian S, Holliday RH, Vinten-Johansen J, Case LD, low up. Catheter Cardiovasc Interv 68:145–152
Hudspeth AS, Tucker WY, Cordell AR (1989) Subxiphoid Pison L, La Meir M, van Opstal J, Blaauw Y, Maessen J, Crijns
pericardial window for pericardial effusive disease. HJ (2012) Hybrid thoracoscopic surgical and transve-
J Cardiovasc Surg (Torino) 30:768–773 nous catheter ablation of atrial fibrillation. J Am Coll
Mohr FW, Falk V, Diegeler A, Walther T, van Son JA, Cardiol 60:54–61
Autschbach R (1998) Minimally invasive port-access Pompili MF, Stevens JH, Burdon TA, Siegel LC, Peters WS,
mitral valve surgery. J Thorac Cardiovasc Surg Ribakove GH, Reitz BA (1996) Port-access mitral valve
115:567–574 replacement in dogs. J Thorac Cardiovasc Surg
Mohr FW, Falk V, Diegeler A, Autschback R (1999) 112:1268–1274
Computer-enhanced coronary artery bypass surgery. Porstmann W, Wierny L, Warnke H, Gerstberger G,
J Thorac Cardiovasc Surg 117:1212–1214 Romaniuk PA (1971) Catheter closure of patent ductus
Muhs BE, Galloway AC, Lombino M, Silberstein M, Grossi arteriosus. 62 cases treated without thoracotomy.
EA, Colvin SB, Lamparello P, Jacobowitz G, Adelman Radiol Clin North Am 9:203–218
MA, Rockman C, Gagne PJ (2005) Arterial injuries from Puskas J, Cheng D, Knight J, Angelini G, DeCannier D,
femoral artery cannulation with port access cardiac Diegeler A, Dullum M, Martin J, Ochi M, Patel N, Sim E,
surgery. Vasc Endovascular Surg 39:153–158 Trehan N, Zamvar V (2005) Off-pump versus conventi-
Chapter 26 · Minimally Invasive Cardiac Surgery
865 26
onel coronary artery bypass grafting: a meta-analysis Seeburger J, Borger MA, Falk V, Kuntze T, Czesla M, Walther
and consensus statement from the 2004 ISMICS con- T, Doll N, Mohr FW (2008) Minimal invasive mitral valve
sensus conference. Innovations 1:3–27 repair for mitral regurgitation: results of 1339 consec-
Rastan AJ, Eckenstein JI, Hentschel B, Funkat AK, Gummert utive patients. Eur J Cardiothorac Surg 34:760–765
JF, Doll N, Walther T, Falk V, Mohr FW (2006) Emergency Seeburger J, Borger MA, Tschernich H, Leontjev S, Holzhey
coronary artery bypass graft surgery for acute coro- D, Noack T, Ender J, Mohr FW (2010) Transapical beat-
nary syndrome: beating heart versus conventional ing heart mitral valve repair. Circ Cardiovasc Interv
cardioplegic cardiac arrest strategies. Circulation 3:611–612
114:I477–I485 Shannon J, Colombo A, Alferie O (2012) Do hybrid proce-
Reade CC, Bower CE, Bailey BM, Maziarz DM, Masroor S, dures have proven clinical utility and are they the
Kypson AP, Nifong LW, Chitwood WR Jr (2005) Robotic wave of future? Circulation 125:2492–2503
mitral valve annuloplasty with double-arm nitinol Shen L, Hu S, Wang H, Xiong H, Zheng Z, Li L, Xu B, Yan H,
U-clips. Ann Thorac Surg 79:1372–1376 Gao R (2013) One-stop hybrid coronary revasculariza-
Rees AP, Risher W, McFadden PM, Ramee SR, White CJ tion versus coronary artery bypass grafting and percu-
(1993) Partial congenital defect of the left pericar- taneous coronary intervention for the treatment of
dium: angiographic diagnosis and treatment by thora- multivessel coronary artery disease: 3-year follow-up
coscopic pericardiectomy: case report. Cathet results from a single institution. J Am Coll Cardiol
Cardiovasc Diagn 28:231–234 61:2525–2533
Reichenspurner H, Boehm DH, Welz A, Schmitz C, Wildhirt Siegel LC, St Goar FG, Stevens JH, Pompili MF, Burdon TA,
S, Schulze C, Meiser B, Schutz A, Reichart B (1998a) Reitz BA, Peters WS (1997) Monitoring considerations
Minimally invasive coronary artery bypass grafting: for port-access cardiac surgery. Circulation 96:562–
port-access approach versus off-pump techniques. 568
Ann Thorac Surg 66:1036–1040 Slaughter MS, Ising MS, Tamez D et al. (2010) Increase in
Reichenspurner H, Gulielmos V, Wunderlich J, Dangel M, circadian variation after continuous- flow ventricular
Wagner FM, Pompili MF, Stevens JH, Ludwig J, Daniel assist device implantation. J Heart Lung Transplant
WG, Schuler S (1998b) Port-access coronary artery 29:695–697
bypass grafting with the use of cardiopulmonary Smith JM, Stein H (2008) Endoscopic placement of multi-
bypass and cardioplegic arrest. Ann Thorac Surg ple artificial chordae with robotic assistance and
65:413–419 nitinol clip fixation. J Thorac Cardiovasc Surg
Reichenspurner H, Detter C, Deuse T, Boehm DH, Treede H, 135:610–614
Reichart B (2005) Video and robotic-assisted minimally Souttar HS (1925) The surgical treatment of mitral stenosis.
invasive mitral valve surgery: a comparison of the Br Med J 2:603
Port-Access and transthoracic clamp techniques. Ann Stanbridge RD, Hadjinikolaou LK, Cohen AS, Foale RA,
Thorac Surg 79:485–490 Davies WD, Kutoubi AA (1997) Minimally invasive cor-
Repossini A, Tespili M, Saino A, Kotelnikov I, Moggi A, Di onary revascularization through parasternal incisions
Bacco L, Muneretto C (2013) Hybrid revascularization without cardiopulmonary bypass. Ann Thorac Surg
in multivessel coronary artery disease. Eur 63:S53–S56
J Cardiothorac Surg 44:288–293 Starr A, Edwards ML (1961) Mitral replacement: clinical
Ricci D, Pellegrini C, Aiello M, Alloni A, Cattadori B, experience with a ball valve prosthesis. Ann Surg
D’Armini AM, Rinaldi M, Vigano M (2010) Port-access 154:726
surgery a elective approach for mitral valve opera- Stehlik J, Edwards LB, Kucheryavaya AY et al. (2010) The
tion in re-do procedures. Eur J Cardiothorac Surg Registry of the International Society for Heart and
37:920–925 Lung Transplantation: twenty-seventh official adult
Ronchey S, Serrao E, Alberti V, Fazzini S, Trimarchi S, heart transplant report – 2010. J Heart Lung Transplant
Tolenaar JL, Mangialardi N (2013) Endovascular stent- 29:1089–1103
ing of the ascending aorta for type A aortic dissections Stevens JH, Burdon TA, Peters WS, Siegel LC, Pompili MF,
in patients at high risk for open surgery. Eur J Vasc Vierra MA, St Goar FG, Ribakove GH, Mitchell RS, Reitz
Endovasc Surg 45:475–480 BA (1996a) Port-access coronary artery bypass graft-
Saltman AE, Rosenthal LS, Francalancia NA, Lahey SJ (2003) ing: a proposed surgical method. J Thorac Cardiovasc
A completely endoscopic approach to microwave abla- Surg 111:567–573
tion for atrial fibrillation. Heart Surg Forum 6:E38–E41 Stevens JH, Burdon TA, Siegel LC, Peters WS, Pompili MF,
Sastry P, Rivinius R, Harvey R, Parker RA, Rahm AK, Thomas St Goar FG, Berry GJ, Ribakove GH, Vierra MA,
D, Nair S, Large SR (2013) The influence of endoscopic Mitchell RS, Toomasian JM, Reitz BA (1996b) Port-
vein harvesting on outcomes after coronary bypass access coronary artery bypass with cardioplegic
grafting: a meta-analysis of 267 525 patients. Eur arrest: acute and chronic canine studies. Ann Thorac
J Cardiothorac Surg 44:980–989 Surg 62:435–440
Schäfer U, Ho Y, Frerker C, Schewel D, Sanchez-Quintana D, Strueber M, Meyer AL, Malehsa D et al. (2010) Successful
Schofer J, Bijuklic K, Meincke F, Thielsen T, Kreidel F, use of the HeartWare HVAD rotary blood pump for
Kuck KH (2013) Direct percutaneous access technique biventricular support. J Thorac Cardiovasc Surg
for transaxillary transcatheter aortic valve implanta- 140:936–937
tion: “the Hamburg Sankt Georg approach”. JACC Strueber M, O’Driscoll G, Jansz P, Khaghani A, Levy WC,
Cardiovasc Interv 5:477–486 Wieselthaler GM, HeartWare Investigators (2011)
866 J. Garbade et al.
Multicenter evaluation of an intrapericardial left ven- van Dijk D, Spoor M, Hijman R, Nathoe HM, Borst C, Jansen
tricular assist system. J Am Coll Cardiol 57:1375–1382 EW, Grobbee DE, de Jaegere PP, Kalkman CJ, Octopus
Subramanian VA (1997) Less invasive arterial CABG on a Study Group (2007) Cognitive and cardiac outcomes 5
beating heart. Ann Thorac Surg 63:S68–S71 years after off-pump vs on-pump coronary artery
Subramanian VA, Patel NU (2000) Transabdominal mimially bypass graft surgery. JAMA 297:701–708
invasive direct coronary artery bypass grafting Vassiliades TA Jr, Block PC, Cohn LH, Adams DH, Borer JS,
(MIDCAB). Eur J Cardiothorac Surg 17:485–487 Feldman T, Holmes DR, Laskey WK, Lytle BW, Mack MJ,
Subramanian VA, McCabe JC, Geller CM (1997) Minimally Williams DO (2005) The clinical development of per-
invasive direct coronary artery bypass grafting: two- cutaneous heart valve technology: a position
year clinical experience. Ann Thorac Surg 64:1648– statement of the Society of Thoracic Surgeons (STS),
26 1653 the American Association for Thoracic Surgery
Subramanian VA, Patel NU, Patel NC, Loulmet DF (2005) (AATS), and the Society for Cardiovascular
Robotic assisted multivessel minimally invasive direct Angiography and Interventions (SCAI). American
coronary artery bypass with port-access stabilization College of Cardiology Foundation (ACCF) and the
and cardiac positioning: paving the way for outpatient American Heart Association (AHA). J Thorac
coronary surgery? Ann Thorac Surg 79:1590–1596 Cardiovasc Surg 129:970–976
Sungur M, Karakurt C, Ozbarlas N, Baspinar O (2013) Villa E, Vanden Eynden F, Le Bret E, Folliguet T, Laborde F
Closure of patent ductus arteriosus in children, small (2004) Paediatric video-assisted thoracoscopic clip-
infants, and premature babies with Amplatzer duct ping of patent ductus arteriosus: experience in more
occluder II additional sizes: multicenter study. Catheter than 700 cases. Eur J Cardiothorac Surg 25:387–393
Cardiovasc Interv doi. doi:10.1002/ccd.24905, PMID: Villa E, Folliguet T, Magnano D, Vanden Eynden F, Le Bret E,
23460349 Laborde F (2006) Video-assisted thoracoscopic clip-
Svensson LG, Gillinov AM, Blackstone EH, Houghtaling PL, ping of patent ductus arteriosus: close to the gold
Kim KH, Pettersson GB, Smedira NG, Banbury MK, Lytle standard and minimally invasive competitor of percu-
BW (2007) Does right thoracotomy increase the risk of taneous techniques. J Cardiovasc Med (Hagerstown)
mitral valve reoperation? J Thorac Cardiovasc Surg 7:210–215
134:677–682 Vollroth M, Seeburger J, Garbade J, Pfannmueller B,
Thiele H, Oettel S, Jacobs S, Hambrecht R, Sick P, Gummert Holzhey D, Misfeld M, Borger MA, Mohr FW (2012)
JF, Mohr FW, Schuler G, Falk V (2005) Comparison of Minimally invasive mitral valve surgery is a very safe
bare-metal stenting with minimally invasive bypass procedure with very low rates of conversion to full
surgery for stenosis of the left anterior descending sternotomy. Eur J Cardiothorac Surg 42:e13–15
coronary artery: a 5-year follow-up. Circulation von Oppell UO, Masani N, O’Callaghan P, Wheeler R,
112:3445–3450 Dimitrakakis G, Schiffelers S (2009) Mitral valve sur-
Tiruvoipati R, Naik RD, Loubani M, Billa GN (2003) Surgical gery plus concomitant atrial fibrillation ablation is
approach for pericardiectomy: a comparative study superior to mitral valve surgery alone with an inten-
between median sternotomy and left anterolateral tho- sive rhythm control strategy. Eur J Cardiothorac Surg
racotomy. Interact Cardiovasc Thorac Surg 2:322–326 35:641–650
Tops LF, Van de Veire NR, Schuijf JD, de Roos A, van der Wall Walterbusch G (1998) Partial sternotomy for cardiac opera-
EE, Schalij MJ, Bax JJ (2007) Noninvasive evaluation of tions. J Thorac Cardiovasc Surg 115:256–258
coronary sinus anatomy and its relation to the mitral Walther T, Binner C, Rastan A, Dähnert I, Doll N, Falk V, Mohr
valve anulus: implications for percutaneous mitral FW, Kostelka M (2007a) Surgical atrial septal defect
annuloplasty. Circulation 115:1426–1432 closure after interventional occluder placement: inci-
Torsello G, Can A, Umscheid T, Tessarek J (2007) Hybrid tho- dence and outcome. J Thorac Cardiovasc Surg
racoabdominal aneurysm repair with simultaneous 134:731–737
antegrade visceral revascularization and supra-aortic Walther T, Falk V, Dewey T, Kempfert J, Emrich F, Pfannmuller
debranching from the ascending aorta. J Endovasc B, Broske P, Borger MA, Schuler G, Mack M, Mohr FW
Ther 14(3):342–346 (2007b) Valve-in-a-valve concept for transcatheter
Tsagakis K, Konorza T, Dohle DS, Kottenberg E, Buck T, minimally invasive repeat xenograft implantation.
Thielmann M, Erbel R, Jakob H (2013) Hybrid operating J Am Coll Cardiol 50:56–60
room concept for combined diagnostics, intervention Walther T, Simon P, Dewey T, Wimmer-Greinecker G, Falk
and surgery in acute type A dissection. Eur V, Kasimir MT, Doss M, Borger MA, Schuler G, Glogar
J Cardiothorac Surg 43:397–404 D, Fehske W, Wolner E, Mohr FW, Mack M (2007c)
Umakanthan R, Leacche M, Petracek MR, Zhao DX, Byrne Transapical minimally invasive aortic valve implan-
JG (2009) Combined PCI and minimally invasive heart tation: multicenter experience. Circulation
valve surgery for high-risk patients. Curr Treat Options 116:I240–I245
Cardiovasc Med 11:492–498 Webb JG, Pasupati S, Humphries K, Thompson C, Altwegg
Usta E, Elkrinawi R, Ursulescu A, Nagib R, Mädge M, Salehi- L, Moss R, Sinhal A, Carere RG, Munt B, Ricci D, Ye J,
Gilani S, Franke UF (2013) Clinical outcome and quality Cheung A, Lichtenstein SV (2007) Percutaneous tran-
of life after reoperative CABG: off-pump versus on- sarterial aortic valve replacement in selected high-
pump – observational pilot study. J Cardiothorac Surg risk patients with aortic stenosis. Circulation
8:66 116:755–763
Chapter 26 · Minimally Invasive Cardiac Surgery
867 26
Weimar T, Vosseler M, Czesla M, Boscheinen M, Hemmer artery bypass grafting combined with coronary angio-
WB, Doll KN (2012) Approaching a paradigm shift: plasty: preliminary results of a multicentre study.
endoscopic ablation of lone atrial fibrillation on the Heart 83:58–63
beating heart. Ann Thorac Surg 94:1886–1892 Woo YJ, Rodriguez E, Atluri P, Chitwood WR Jr (2006)
Wheatley GH 3rd, Prince SL, Herbert MA, Ryan WH (2004) Minimally invasive, robotic, and off-pump mitral
Port-access aortic valve surgery: a technique in evolu- valve surgery. Semin Thorac Cardiovasc Surg 18:
tion. Heart Surg Forum 7:E628–E631 139–147
Williams JB, Peterson ED, Brennan JM, Sedrakyan A, Tavris Yamada T, Ochiai R, Takeda J (2000) Anesthetic manage-
D, Alexander JH, Lopes RD, Dokholyan RS, Zhao Y, ment during minimally invasive cardiac surgery with
O’Brien SM, Michler RE, Thourani VH, Edwards FH, the Port-Access system for closure of atrial septal
Duggirala H, Gross T, Marinac-Dabic D, Smith PK (2012) defect. J Anesth 14:98–101
Association between endoscopic vs open vein-graft Zahn R, Schiele R, Gerckens U, Linke A, Sievert H, Kahlert P,
harvesting and mortality, wound complications, and Hambrecht R, Sack S, Abdel-Wahab M, Hoffmann E,
cardiovascular events in patients undergoing CABG Senges J, German Transcatheter Aortic Valve
surgery. JAMA 308:475–484 Interventions Registry Investigators (2013)
Wilson SR, Mudge GH, Stewart GC et al. (2009) Evaluation Transcatheter aortic valve implantation in patients
for a ventricular assist device. Circulation 119:2225– with “porcelain” aorta (from a Multicenter Real World
2232 Registry). Am J Cardiol 111:602–608
Wimmer-Greinecker G, Matheis G, Dogan S, Aybek T, Zhu XY, Qin YW, Han YL, Zhang DZ, Wang P, Liu YF, Xu YW,
Kessler P, Westphal K, Moritz A (1999) Complications of Jing QM, Xu K, Gersh BJ, Wang XZ (2013) Long-term effi-
port-access cardiac surgery. J Card Surg 14:240–245 cacy of transcatheter closure of ventricular septal
Wittwer T, Cremer J, Boonstra P, Grandjean J, Mariani M, defect in combination with percutaneous coronary
Mugge A, Drexler H, den Heijer P, Leitner ER, Hepp A, intervention in patients with ventricular septal defect
Wehr M, Haverich A (2000) Myocardial “hybrid” revas- complicating acute myocardial inferction: a multicentre
cularisation with minimally invasive direct coronary study. EuroIntervention 8:1270–1276
869 27
Aneurysm and Dissection
of the Thoracic and
Thoracoabdominal Aorta
Matthias Karck and Klaus Kallenbach
References – 907
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
871 27
27.1 Introduction wall layer of a dissected aorta will go through the
typical transformation process and evolve into an
The surgical options for treating diseases of the aneurysm. A dissection can also develop from a
aorta have been extended in the past 15 years. preexisting aneurysm. Owing to these relation-
This development was driven, in particular, by the ships and also considering the similar type of sur-
search for means of reducing surgical trauma. For gical treatment, the procedures for proximal
example, new techniques that were introduced for thoracic aneurysms and aorta dissection are pre-
procedures at the aortic arch brought about con- sented together.
siderable improvement in intraoperative neuro- Operations for aneurysms and dissection of
protection (Hagl et al. 2003). Moreover, new the descending thoracic aorta will be discussed
methods are now available for various indications with respect to the need to replace numerous
in classic «open» surgery; while they are not aortic segments. The respective chapter also deals
suited to replace the diseased aortic segment, with interventional procedures for treating aortic
these methods nonetheless enable us to treat disease involving the aortic arch and/or the tho-
affected aortal segments by implanting endovas- racoabdominal aorta. In addition, the reader is
cular prostheses – so-called endografts (Baraki referred to the corresponding 7 Chap. 28 in this
et al. 2007; Nienaber et al. 2007). Accordingly, a textbook. By definition, an aneurysm of the
new chapter, «Endovascular Surgical Therapy of ascending aorta involves the segment between
Thoracic and Thoracoabdominal Disease of the the aortic valve and the origin of the innominate
Aorta,» is added to this book (7 Chap. 28). artery, an aortic arch aneurysm when the aneu-
The surgical treatment of diseases of the rysm runs between the branches of the brachio-
aorta, therefore, is increasingly being conducted cephalic trunk and left subclavian artery, and,
at interdisciplinary treatment centers, which is finally, an aneurysm of the descending aorta if the
sensible. The following account, however, is thoracic aorta segments beyond this artery are
being given primarily from the perspective of affected.
classic aorta surgery and deals with the inter- Today, dissection of the aorta is divided into
ventional procedures according to surgical indi- type A and type B (Miller et al. 1979), basically
cation. superseding the original classification by De
Bakey et al. (1966). The two types only differ with
regard to the segment affected – ascending or
27.1.1 Definitions and Outline descending aorta – irrespective of the site of
proximal «entry» and the peripheral extent of the
Aneurysms and dissections of the aorta result dissection process. This view is thought to best
from a weakness in the viscoelastic «belt» of the satisfy the requirements for surgical manage-
vascular media that sustains the arterial pressure. ment since a type A dissection is always accessed
This can be strictly localized – as in the case of via a sternotomy, type B in contrast via a left-lat-
rupture of the vascular wall due to trauma – or eral thoracotomy. In our opinion, it is still sensi-
can involve extensive segments of the vessel – as ble to apply DeBakey’s type II terminology for
in the case of primary medial degeneration and dissections limited to the ascending aorta – in
atherosclerotic aortopathy. The consequences are spite of their rarity – because they often engraft a
a progressive increase in the vessel radius as well preexisting anular ectasia, mainly associated with
as a thinning and – and according to Laplace’s Marfan syndrome. In addition, they are usually
law – a disproportionate increase in wall tension, easier to operate than dissections extending far
resulting in compression of neighboring organs distally.
and ultimately in rupture. Aortic dissection repre- More recent studies have shown that intramu-
sents a special case, in which intima and the ral hematomas and atherosclerotic ulcerations can
degenerated media rupture and split longitudi- be signs of a beginning aortic dissection. Thus,
nally from the adventitia, usually over long Swensson et al. (1999) suggested that pathomor-
stretches of the vessel. If an individual does not phological criteria be used to distinguish them.
die as a result of an acute rupture associated with Lansman et al. (1999) recommended classify-
cardiac tamponade, massive aortic insufficiency, ing aortic dissections with regard to the site of the
or malperfusion of vital organs, the outermost intimal tear. In some cases, a type B dissection
872 M. Karck and K. Kallenbach
extends retrogradely into the aortic arch and up to this development in detail. Indeed, arch proce-
the aortic valve. dures were considered high-risk operations for
A dissection is defined as acute if surgery is quite some time until Griepp et al. (1975), Ott
undertaken within 14 days of onset, usually et al. (1978), Thevenet (1980), and particularly
accompanied by tremendous suddenly occurring Crawford (Crawford and Saleh 1981; Crawford
chest pain. It is called subacute if that pain event et al. 1979, 1984) picked up on an older sugges-
took place more than 2 weeks but less than tion made by Borst and colleagues to operate
2 months previously, and it is chronic if a longer under conditions of circulatory arrest and deep
period has passed. From the surgeon’s standpoint, hypothermia (Borst et al. 1964). After Bachet
it is largely irrelevant to distinguish between acute et al. (1991) and more so Kazui et al. (2007) intro-
27 and subacute dissection as in both cases the walls duced scientifically well-founded techniques for
of the aorta are delicate and fragile since stabiliz- protecting the brain during surgery, excellent
ing scar tissue only develops in the outermost wall results can now be achieved for partial and total
layer later on. arch replacement. Long perfusion times and
potentially severe coagulation disorders due to
the extremely low body temperatures prompted a
27.1.2 Historical Remarks number of authors to compromise between mod-
erate hypothermia and comparably short periods
Surgical strategies for treating the proximal tho- of circulatory arrest (Bachet and Guilmet 2002;
racic segments of the aorta lagged behind those of Di Eusanio et al. 2002; Kamiya et al. 2006, 2007).
the descending aorta due to initially unresolvable Today, there is increasing evidence that neuro-
technical problems. These mainly involved intra- logical outcome after circulatory arrest of more
operative protections of vital organs and the avail- than 30 min is improved when antegrade cerebral
ability of vascular prostheses that remained blood perfusion was used in comparison to deep hypo-
tight even in a patient fully heparinized. Up to the thermia alone (Krüger et al. 2011).
middle of the 1950s, therefore, surgeons were Although true long-term follow-up data are
restricted to operate upon saccular and upon fusi- still limited, endoluminal thoracic aortic prostheses
form aneurysms by resecting the first and reduc- have been widely introduced clinically as, in the
ing the size of the latter (Cooley and De Bakey meantime, this is considered to be at least as good
1952). Cooley und De Bakey were the first to an option as classic open surgery for several indica-
implant a tubular prosthesis in the ascending tions (Nienaber et al. 2007). This applies to diseases
aorta under conditions of extracorporeal circula- of the descending aorta and, as recently reported,
tion in 1956. Later, the aortic root and the ascend- also to those of the aortic arch (Schumacher et al.
ing aorta were either replaced separately (Wheat 2006; Kotelis et al. 2011). Here, however, the supra-
et al. 1964) or jointly (Bentall and De Bono 1968; aortic branches need to be extra anatomically
Schulte and Birks 1971), with the latter technique transposed to the ascending aorta before implant-
largely becoming established. Today, hospital ing aortic endovascular prostheses.
mortality of those procedures is comparable to
that for a simple aortic valve replacement
(Kouchoukos et al. 1986). The valve-sparing pro- 27.1.3 Surgical Indications
cedures introduced by Yacoub in 1983 and by
David at the early 1990s extended the therapeutic The goal of surgical treatment of aneurysms and/
options considerably (David and Feindel 1992; or dissections of the thoracic and abdominal
Yacoub et al. 1983). aorta is to prevent rupture; less often compres-
For aortic arch replacement, surgeons initially sion of neighboring organs has to be eliminated,
used temporary or permanent extra-anatomic also. In general, an increase in the diameter of an
circulatory bypasses (Cooley and DeBakey 1956; aneurysm to >5 cm in the ascending aorta and in
Cooley et al. 1955; Creech et al. 1956; Muller et al. the arch segment and to >6 cm in the descending
1960) until De Bakey, Crawford, and Cooley suc- aorta gives the indication for elective proce-
cessfully replaced the aortic arch on extracorpo- dures. Symptoms of an impending rupture or a
real circulation for the first time in 1957 (De life-
threatening compression of intrathoracic
Bakey et al. 1957). Heberer et al. (1960) described organs require a rapid line of action. For manifest
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
873 27
rupture and for acute type A dissection, emer- 27.2 Aneurysms of the Ascending
gency surgery is always needed in order to pre- Aorta
vent cardiac tamponade and massive aortic
insufficiency. Aneurysms that extend from the aortic root to the
Further refinements concerning the indica- branches of the innominate artery may be limited
tions for operative therapy in diverse subcohorts to one of the aortic sinuses or to the supracom-
of patients with aortic aneurysms have been pub- missural segment. More often, however, they
lished recently are displayed in updated guidelines involve the entire aortic bulb and the actual
and (Erbel et al. 2014; Hiratzka et al. 2010, 2016). ascending aorta, meaning an anuloaortic ectasia
associated with aortic valve insufficiency caused
by aortic ring dilatation. How radical the treat-
27.1.4 Prostheses, Sutures, ment must be depends largely on the degree of
and Stentgrafts aneurysmal dilatation. The surgeon, however, will
(Endovascular Prostheses) attempt to restrict the procedure to the proximal
segment of the aorta.
Today, prostheses fashioned from woven double-
velour Dacron and pretreated with collagen are
used almost exclusively. Mostly, simple tubular vas- For planning the procedure with the aim of
cular prostheses are applied that, depending on possibly sparing the aortic valve, the surgeon
where they will be implanted, are supplied with must be aware about the type, extent, and
prefabricated side branches to facilitate an anasto- prognosis of any concomitant valve malfor-
mosis to the large branches of the aorta (Spielvogel mations.
et al. 2003). For treating diseases involving both the
aortic arch and the descending aorta, vascular
prostheses are available that carry a stented and a
nonstented segment (so-called hybrid prostheses; 27.2.1 Access and Extracorporeal
Karck et al. 2005). By using such prostheses that are Circulation
designed like an endograft distally, a later second
operation via a lateral access is often unnecessary. For all proximal aneurysms, the heart and aorta are
In addition, a wide variety of vascular stents accessed in the same way, via a median sternotomy.
and prostheses has emerged for endovascular If the pericardium is intact (first operation), the
treatment of many aortic diseases (Chuter 2007; aneurysm is usually protected from sawing injury.
Greenberg et al. 2006; Melissano et al. 2007). For Thus, sternotomy is performed in standard fash-
details, please see also 7 Chapter «Endovascular ion. After opening the pericardium, the surgeon
Surgical Therapy of Thoracic and Thoracoabdominal first determines whether the proximal aortic arch
Disease of the Aorta», Sect. 28.2. is suitable for arterial cannulation. If not, one of the
Human aortic homografts are particularly two common femoral arteries or the right axillary
well suited for repeat procedures of the thoracic artery can be used. In complicated situations, par-
aorta due to vascular prosthesis infection (Lesèche ticularly reoperations, the procedures outlined in
et al. 2001). 7 Sect. 27.5, «Reoperations», apply.
Sutures for aortic surgery have largely been With large aneurysms, the aorta is inevitably
standardized. We exclusively employ monofila- elongated, and thus, the heart is frequently dis-
ment, usually double-armed size 3/0 suture placed to the left and caudally, which makes it dif-
material. Extra-long threads (monofilament poly- ficult to access the right atrium. In this case, it may
propylene, 120 cm) are better for deep suturing, be easier to decompress the heart by first starting
whereas lower-sized threads and needles are pref- with one venous cannula and adding a second
erable for anastomosing aortic side branches and caval catheter later. More often, we insert a single
also for joining the coronary ostia to the aorta pros- two-staged cannula through the auricle of right
thesis. By choosing a special, hardened surgical atrium, while the assisting surgeon carefully holds
needle, it is much easier to create an anastomosis if the aneurysm to the side. Frequently, adhesions
the target vessel is calcified. have developed between the aneurysm, the right
874 M. Karck and K. Kallenbach
atrium, and the superior vena cava, which need to stumps by using continuous running sutures. In
be detached first so that the aorta can be clamped cases of valve insufficiency, a valve-sparing proce-
and opened as soon as the extracorporeal circula- dure can also be chosen and the aneurysmatic
tion has been established and ventricular fibrilla- segment of the ascending aorta replaced with a
tion has been commenced. It is also advisable to vascular prosthesis at the same time.
detach adhesions between the aneurysm and the
pericardium, but the phrenic nerve must be pre-
served under all circumstances. We always insert a
Aortic root aneurysms that definitely involve
left ventricular suction catheter, either via the
the sinus of Valsalva are repeatedly and erro-
interatrial sulcus at the level of the upper right pul-
neously termed aneurysms of sinus of Val-
27 monary vein and the mitral valve or – if access is
salva. This term, however, is generally reserved
awkward – directly through the apex of the left
for congenital saccular weakness of the ven-
chamber. After clamping the aorta, cardioplegic
tricular-aortic junction that, over time, perfo-
solution is then injected into the ascending seg-
rates most frequently into the right atrium or
ment for aneurysms not associated with aortic
ventricle. Aneurysms of the sinus of Valsalva
insufficiency. Cardioplegia may be delivered in a
do not cause aortic or aortic root dilatation to
retrograde fashion through the coronary sinus,
the extent of an aortic root aneurysm.
also. In all other cases, the aorta has been opened
prior to direct intubation of the coronary ostia if
antegrade delivery of cardioplegia is planned. The
technique for opening the ascending aorta includ-
ing its base depends on the intraoperative findings. 27.2.3 Ascending Aorta and Aortic
Valve Replacement
..Fig. 27.1 Typical incision line for replacement of the ..Fig. 27.2 Separate replacement of aortic valve and
ascending aorta ascending aorta. The valve prosthesis is implanted. The
aortic prosthesis is anchored as deeply as possible in the
sinus. Arch-shaped gaps corresponding to the coronary
ostia are cut out of the proximal end of the prosthesis. The
ostia are semi-circumferentially included into the suture
27.2.3.1 Separate Replacement line (the left ostium is illustrated here). In the noncoronary
sector, the edge of the prosthesis can be joined directly
Applying the Wheat technique (Markewitz et al. with the suture ring of the artificial valve
1986; Wheat et al. 1964; Yun et al. 1997), the aortic
valve is first replaced in the usual manner. Then,
two small arch-shaped excisions are made in the
proximal end of the ascending aorta prosthesis 27.2.3.2 Combined Replacement/
that correspond to the anatomical site of the coro- Composite Graft
nary ostia; later, the prosthetic tube needs to be Combined replacement of the ascending aorta
anchored as deeply as possible in the bulb here. and aortic valve constitutes a routine procedure
Double-armed continuous anastomotic suturing for treating anuloaortic ectasia, irrespective of
begins at the level of the noncoronary sinus and whether it is caused by aneurysm or dissection
proceeds in the direction of the left coronary (Ehrlich et al. 2001; Gott et al. 1999; Mingke
ostium, which is semi-circumferentially included et al. 1998; Sioris et al. 2004). Following the
into the suture line. Sewing is continued in a original method of Bentall and De Bono (1968),
clockwise direction until the right ostium is the coronary ostia are not excised but reim-
reached and can also be included into the anasto- planted in the composite graft in continuity with
motic suture (. Fig. 27.2). With the other end of the aortic wall.
the thread, the surgeon stitches deeply into the In the modification described here, however,
noncoronary sinus to the right, to ultimately reach the coronary ostia are first excised out of the ves-
the corresponding thread and tie the two ends sel wall with a trumpet-shaped aortic tissue cuff
together. It is important to exclude the noncoro- (button) for better mobilization. We prefer this
nary sinus as completely as possible as recur- method for both primary and redo procedures at
rences are especially likely to develop in this zone. the aortic root because it always allows reimplan-
It may be advantageous, therefore, to join the tation of the coronary ostia into the aortic pros-
ascending aorta graft directly with the suture ring thesis without producing any tension. Indeed, we
of the already implanted aortic valve prosthesis have also largely departed from employing the
(for distal prosthesis-aorta anastomosing, see original method because we occasionally observed
7 Sect. 27.2.3.3, «Distal Anastomosis»). This tech- recurrent aneurysms in the area of the coronary
nique may be also employed in ascending aortic ostia anastomoses in patients who had previously
aneurysms secondary to earlier aortic valve undergone surgery. In particular Svensson et al.
replacement revealing a well-functioning valve (1992) and Gott et al. (1995) gained experience
prosthesis, usually a mechanical one. using the modified technique already early on.
876 M. Karck and K. Kallenbach
27
..Fig. 27.4 All sutures have been placed and the
..Fig. 27.3 Combined replacement of the ascending prosthesis can be inserted into the base of the aorta
aorta and aortic valve. The prosthesis is anchored along
the right aspect of the base of the aorta using
Teflon-pledgeted mattress sutures. The valve cusps are preferred procedure is to sparingly fenestrate the
tubular segment of the prosthesis precisely at the
removed. The coronary ostia are excised out of the aortic
wall and held aside by stay sutures point at which the corresponding ostium can be
anastomosed with the least possible amount of
tension. To achieve this, the first assistant holds
Other variants are described in 7 Sect. 27.2.3.6, the ostium toward the vascular prosthesis before
«Alternative Methods of Proximal Aorta Replacement» the surgeon places the window at the site that cor-
(Bachet et al. 1996). responds most favorably to the bottom of the
The size of the prosthesis for combined replace- ostium. For this, a battery-operated electrocauter-
ment of the aortic valve and the ascending aorta izer is used. At the same time, a wide-enough rem-
depends on the diameter of the aortic anulus. It is nant of prosthetic tissue must be left between the
determined in the same way as for isolated aortic suture ring of the valve prosthesis and the respec-
valve replacement after excising the cusps. After tive ostium so that the anastomosis is not under
transverse aortotomy at the level of the sinotubular any tension later. The holes in the prosthesis
junction, the coronary ostia are readily accessible, should be compatible with the size of the ostia
and the surgeon has a good view for excising them unless the ostium is sclerosed such that one would
in a «U shape» from the aortic wall and for subse- need to leave a greater margin around the holes.
quently placing stay sutures to hold them aside. When creating the anastomosis, the entire aortic
Three additional stay sutures at the level of the wall including the adventitia needs to be passed
valve commissures are lifted under light tension. by the needle. Depending on the condition of the
In this way, the entire aortic root is lifted more into surrounding aortic wall, a 5/0 or also a 4/0 suture
the surgical field of view, which facilitates the fur- is recommended. First the left ostium is anasto-
ther procedure. The prosthesis is anchored into the mosed to the prosthesis (. Fig. 27.5). For practical
base of the aorta, usually with Teflon felt-rein- reasons, the lower part of the coronary anastomo-
forced single mattress sutures that are placed from sis is sewn by inserting the needle through the
an aortal direction (. Figs. 27.3 and 27.4). prosthesis and capturing the lower margin of the
As a time-saving alternative, the composite ostium. For the rest of the anastomosis, the direc-
graft can be implanted by using three long, tion is less important. Nonetheless, it is easier to
double-armed 2/0 polypropylene sutures and a sew toward oneself and around the anastomosis.
continuous sewing technique. They proceed from The right coronary ostium is attached to the pros-
the depth of each of the three sinuses to the cor- thesis in the same way; the anastomosis is usually
responding commissure and then inserting and technically easier (. Fig. 27.6).
anchoring the prosthesis in the anulus («block- It is wise to ensure that there is no leakage in any
and-tackle» effect). of the two coronary-prosthesis anastomoses before
After fixating the composite graft in the base finishing the distal prosthesio-ascending aortic
of the aorta, the coronary ostia are sutured to the anastomosis (Borst 1981). To control for potential
respective sides of the vascular prosthesis. Our bleeding sites, the distal end of the composite graft
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
877 27
ing aorta associated with aortic valve disease. The prosthetic neosinuses of Valsalva. The base of the
ascending aorta is initially mobilized in a circular aorta needs to be reinforced at the level of the anu-
fashion as far as possible and then opened length- lus with a ring of autologous pericardium to pre-
wise into the noncoronary sinus. The vessel cali- vent anuloaortic dilatation from recurring.
ber is reduced to normal size and the valve For our procedures, we prefer the valve-
replaced. Then, two to three valve sutures are sparing reimplantation method according to
placed over the circumference of the aorta out- David (David and Feindel 1992) for replacement
ward through the aortic wall at the noncoronary of the ascending aorta. With this technique, the
sinus. After closing the aorta by using continuous diseased aortic tissue is resected to the same
sutures, the vessel is enclosed by a synthetic pros- extent as for the Yacoub procedure; however, as
27 thesis that has been beveled proximally and the valve is reimplanted into a complete vascular
adjusted to the corresponding diameter. tube graft, secondary dilatation of the aortic anu-
Thereafter, the prosthesis is fixed by sutures placed lus and reconstructed root is prevented.
through the base of the aorta and then closed
around the ascending aorta with running sutures 27.2.4.1 Indications for Applying
under moderate tension. It is obvious that the dis- the Reimplantation Method
eased segment of an aorta reconstructed in this An important advantage of aortic valve recon-
way would largely still be in place. Thus, we no struction lies in the fact that long-term anticoagu-
longer use this technique and only perform reduc- lation is not required. The mid- and long-term
tion aortoplasty of the ascending aorta – if neces- follow-up results available so far are quite positive
sary – in very elderly patients in conjunction with (David et al. 2012; Kallenbach et al. 2005; Shrestha
aortic valve replacement surgery. More recent et al. 2012). Whereas David described the reim-
results with this technique, however, showed sat- plantation method in patients with aortic root
isfactory 5-year results with regard to the fate of aneurysms and aortic insufficiency with intact
the diameter emphasizing the preserved valve cusp morphology, today this technique is
Windkessel function of the ascending aorta (Bail also employed to treat aortic insufficiency without
et al. 2007; Walker et al. 2007). pronounced aortic ectasia, acute type A aortic
dissection, bicuspid aortic valves, mildly pro-
nounced prolapse of one or more valve leaflets,
27.2.4 Ascending Aorta and also in pediatric patient groups (David et al.
Replacement Associated 2001; Kallenbach et al. 2002, 2004; Karck and
with Aortic Valve Haverich 2005; Vricella et al. 2005).
Reconstruction The long-term prognosis of valve-sparing sur-
gical techniques in patients with Marfan syndrome
Anuloaortic ectasia of the aortic root that devel- and aortic root ectasia is still the subject of contro-
ops from lateral displacement of the commissures versial discussion and requires further clarification
may cause a central aortic valve insufficiency. This (Benedetto et al. 2011; Kallenbach et al. 2007;
pathology can frequently be treated by valve- Karck et al. 2004). Irrespective of whether Marfan
sparing reconstruction of the aortic root when the syndrome is present, we apply the reimplantation
cusps are morphologically intact. Yacoub et al. method in all patients with structurally intact
(1983) developed a method of remodeling the valve leaflets. Pronounced stress fenestration or
aortic root in patients with aneurysm. The dilated very pronounced prolapse of one or more valve
ascending aorta is resected with the sinus of cusps in Marfan syndrome suggests progressive
Valsalva sparing the aortic valve with its commis- structural changes in the aortic valve. Therefore, a
sures. A narrow rim of aortic tissue is kept at the reconstruction procedure should not be per-
edge of the valve sinuses and commissures. The formed here.
coronary ostia are excised from the aortic wall and
mobilized. Then, the proximal end of a Dacron 27.2.4.2 Reimplantation of the
graft is fashioned such that three tongue-shaped Aortic Valve
extensions of the prosthesis can be anastomosed The aorta is opened transversely at the level of the
to the residual rims of the aorta. Subsequently, the sinotubular junction and completely transected
excised coronary ostia are reimplanted into the there (Karck and Haverich 2005). Often, however,
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
881 27
this structure has not been preserved, particularly prosthesis phantom or a Hegar dilator above the
in patients with Marfan syndrome, and instead aortic root, whereby the diameter size is taken
has evolved as aortic root ectasia, which is typical when the parallel commissural sutures touch the
of that condition. In this case, the ascending aorta sizer. To that number 2–3 mm are to be added in
should be opened further distally in order to avoid order to determine the corresponding vascular
injuring the coronary ostia, which occasionally prosthesis diameter because the vascular pros-
drift surprisingly far downstream. The distal part thesis encompasses the entire heart valve from
of the aorta is kept out of the surgical field by a stay the outside.
suture. After administering cardioplegic solution To anchor the vascular prosthesis, we use up
via the coronary ostia, the aortic valve is inspected. to 12 double-armed polyester sutures to be placed
The decision for or against reconstruction transmurally in an outward direction from the
depends on whether the valve geometry can be horizontal subvalvular level of the left ventricle
repaired, the degree of sinus calcification, and (. Fig. 27.12). Other groups have used as few as
whether the heart valve macroscopically three of these sutures, that is, one for each sinus
appears to be structurally intact. At reconstruc- (Tsang et al. 2011). The vascular prosthesis is then
tion the aortic root is externally mobilized as shortened to the appropriate length. Subsequently
far proximally as possible until a virtual, almost the positioning of the commissures is marked for
horizontal plane has been achieved near the later use on the outer side of the prosthesis with a
bottom of the sinus of Valsalva. Then the coro- sterile pencil by dividing the perimeter of the
nary ostia are excised from the aortic wall in a prosthesis into three equal segments. After the
U shape and held aside with stay sutures before stay sutures at the commissures have been drawn
resecting the sinus of Valsalva up to a rim of through the lumen of the prosthesis and held
4–5 mm to the insertion line of the valve leaflets aside by small clamps, the anchor sutures are
(. Fig. 27.11). placed at each corresponding position of the
To calculate the diameter of the vascular proximal end of the prosthesis. The prosthesis is
prosthesis needed for the aortic valve reimplan- anchored at the base of the aorta with ligatures to
tation, three double-armed stay sutures are first these sutures.
placed at the commissures. Under mild tension,
they are lifted to be parallel so that coaptation of
the valve cusps can be well visualized. The assis- The ligatures should not be drawn too tightly
tant holds a commercially available heart valve as this would cause an undesired plication of
the anulus.
27
..Fig. 27.14 Classification
of acute aortic dissection by
DeBakey and by the
Stanford classification
27
in order to enable clamping the vessel as rapidly can be spared/repaired or needs to be replaced. A
and as high as possible when ventricular fibrilla- stable result can be achieved for acute dissection
tion during cooling ensues. By using wide clamps and normal aortic sinus morphology by resus-
for gentle occlusion and careful closure of the pending the commissures and gluing the dissec-
branches, breakage of the damaged aortic wall can tion membrane in the base of the aorta. Then, the
be avoided. To completely exclude this risk, the «no supracommissural ascending part of the vessel is
clamp technique» is recommended, particularly in replaced. However, reports of late postoperative
cases of acute dissection. Here, the surgeon refrains redissection of the aortic root call the value of this
altogether from cross-clamping the aorta. Instead, procedure into question, particularly in younger
the patient’s body temperature is cooled to a hypo- patients (De Paulis et al. 2005; Fukunaga et al.
thermic level, and the aorta is only opened when 1999). Nowadays, we only perform this operation
circulatory arrest has been induced (Bavaria et al. as an exceptional procedure in very elderly
1996). In this case, it is essential that the left ven- patients. Our routine approach is to resect the
tricle is sufficiently decompressed during cooling. entire dissected aortic wall except for the com-
missural segment with the valve cusps attached.
In most instances, the aortic valve may be pre-
27.3.3 Selecting a Surgical served by using the technique described by
Technique David – irrespective of the diameter of the aortic
root (Kallenbach et al. 2004).
Without pronounced anuloaortic ectasia present, The base of the aorta needs to be replaced with
the standard procedure for the ascending aorta is a composite graft if the aortic root is ectatic and
replacement associated with downstream resec- the aortic valve shows pronounced pathological
tion or reconstruction of the dissected wall layers. changes. For older patients, implants with a bio-
The surgical method chosen for the area of the logical heart valve prosthesis are available.
aortic root depends on whether the aortic valve Individualized composite grafts can also be
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
885 27
crafted intraoperatively from a biological heart
valve and a Dacron graft in relatively little time.
In patients with Marfan syndrome and in
patients with anuloaortic ectasia typical for
chronic dissection as well as in acute dissection
occurring in an ectatic aorta, combined replace-
ment of the ascending aorta and valve with a com-
posite graft is one option. Here, too, valve-sparing
reimplantation techniques, especially in younger
patients with Marfan syndrome, lead to durable
results (Karck et al. 2004).
We perform a revision of the arch (under cir-
culatory arrest and moderate hypothermia with
selective antegrade cerebral perfusion) in all aor-
tic dissections of type Stanford A and in an aneu-
rysmatic aortic arch (Hagl et al. 2002; Kamiya
..Fig. 27.15 Aortotomy for acute dissection. The
et al. 2007). The extent of revision depends on the
ascending aorta was opened with a hockey stick-shaped
vessel diameter and the dissection pathology in incision. The proximal «entry» lies in the wall of the true lumen
the aortic arch. We only deviate from this proce-
dure for those rare cases of DeBakey’s type II aor-
tic dissection if the dissection in the distal
ascending segment can be resected completely The two coronary ostia must be examined
without opening the aortic arch. carefully before intubation especially because
the right coronary artery is occasionally
involved in the dissection or even shows an
27.3.4 Acute Type A Dissection «entry.»
..Fig. 27.17 Borst
fixation clamp for adapting
the dissected wall layers
employing gelatin-
resorcin-formaldehyde
glue
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
887 27
obliterate it as it is very difficult to stop any bleed- artery in order to search for the vessel just outside
ing that develops upstream from the reconstruc- the aorta and to ligate it proximally. The (venous)
tion zone. Then the suture, which serves bypass is placed end to side with the coronary ves-
exclusively to join the wall layers together, is tied. sel just beyond the ligature. This type of procedure
It should not be pulled too tightly such that the inevitably requires an ascending aorta replacement
aortic wall breaks or a purse-string effect is pro- in order to gain a base for the bypass vessel unless
duced. For aortic valve reconstruction, the com- a mammary artery bypass is employed.
missures that have now been raised into their The distal wall is reconstructed mostly in the
normal position are each secured additionally same way as has been described for the base of the
with a transmural 4/0 mattress suture. aorta. We only deviate from this procedure in
If the dissection involves the right or more selected patients who present with dilated
rarely the left coronary ostium, the layers of the descending aortas or malperfusion caused by
wall of the respective circumference of the ostium compression of the true lumen. In these patients,
first need to be joined together by continuous it may be better to use the elephant trunk tech-
everting 5/0 stitches. If one of the coronary arteries nique and its variations – also by employing novel
has been completely torn off its ostium and the ves- implants with integrated vascular stents. This
sel lumen is large enough, the surgeon should at technique is dealt with in 7 Sect. 27.4.5, «Total
least attempt a circular reapproximation by sutur- Aortic Arch Replacement Using the Elephant Trunk
ing out through the ostium using continuous 6/0 Prosthesis».
sutures, grasping the torn coronary artery together
with the adventitia of the aorta and sewing them
together (. Fig. 27.19). If the ostium is small, a 27.3.5 Chronic Type A Dissection
coronary bypass will likely be unavoidable
(Kawahito et al. 2003; Neri et al. 2001). For this, the Repairing the base of the aorta for chronic dissec-
surgeon inserts a probe into the right coronary tion type A is only slightly different from ascend-
a b
..Fig. 27.19 a, b Reconstructing a dissected right coronary ostium. a The margin of the coronary artery that is
partially or entirely torn out of the wall of the true lumen of the aorta is grasped by a 5/0 suture sewn from inside to
outside and rejoined with the orifice in the aortic wall. b After the ostium has been reconstructed, the dissected
portions of the wall are connected by sewing around the distant circumference of the ostium
888 M. Karck and K. Kallenbach
a b c
27
..Fig. 27.20 a–c Variants for aortic arch replacement. a Proximal arch replacement, sparing the supra-aortic vessels
beyond the distal prosthesio-aortic arch anastomosis. b Subtotal arch replacement, sparing the left subclavian artery
beyond the prosthesio-aortic arch anastomosis. The right arch branches are implanted into a window at the zenith of
the prosthesis. c Total arch replacement. The prosthesio-aortic anastomosis lies distal to the branch of the left
subclavian artery, namely, distal to the arch. All arch branches are implanted in one window
Normally it is possible to insert a large-lumen median and distal arch can be repaired together
two-stage venous cannula into the right auricle to with the descending aorta via a left thoracotomy
completely drain the systemic venous return and (see 7 Sect. 27.4.6.1, «Surgical Access»).
fully bypass and take over the heart’s function. In Management of the extracorporeal circulation
most cases, bicaval cannulation is not required for needs to be closely coordinated with the anesthesi-
total extracorporeal circulation. For procedures ologist, owing to the sometimes long duration of the
involving the aortic root and in particular for aor- cooling and warming phases during surgery and
tic valve insufficiency, a left ventricular suction also especially in regard to intraoperative neuro-
catheter should be inserted. monitoring. For aortic insufficiency, the aorta is
Aneurysms of the entire arch affecting the occluded when ventricular fibrillation ensues; oth-
descending aorta also can be surgically treated via erwise, the decompressed heart is allowed to fibril-
median sternotomy if a secure prosthesio-aortic late until the target temperature has been reached.
anastomosis cranial to the left pulmonary hilus Circulatory arrest is induced after an at least 30-min
can be established, that is, if no more than the cooling period to a hypothermia level of 26 °C tem-
proximal 4 cm of the descending segment are to perature both in the urinary bladder and nasopha-
be replaced. Alternatively a vascular prosthesis ryngeal. The temperature of the heart is maintained
reinforced with a stent on the distal end can be at <15 °C by repeatedly infusing cardioplegic solu-
implanted into the descending aorta via the tion and pericardial cooling. An additional neuro-
opened aortic arch for exclusion of the distal part protective effect is achieved during circulatory
of the aneurysm («frozen elephant trunk,» hybrid arrest by applying external cool packs to the head,
prothesis; Karck et al. 2003; Orihashi et al. 2001). which should avoid rewarming by ambient temper-
Otherwise, a two-step procedure is recom- ature. After opening the aortic arch, we begin with
mended, whereby the size of the aneurysm and the selective perfusion of the brain. Here, 26 °C arterial-
relative risk of rupture determine which comes first. ized blood is infused into the innominate artery and
Combined replacement of the aortic arch and the the left carotid artery via a perfusion catheter and a
descending aorta via sternotomy is difficult because roller pump (flow volume: 10 ml/kg body weight/
of the great depth of the surgical field and the left min) (Bachet et al. 1991; Frist et al. 1986; Hagl et al.
pulmonary root that is in the way. If the surgeon 2002; Strauch et al. 2003). The left subclavian artery is
still decides for this procedure, however, we recom- clamped or intubated with an occlusion catheter
mend selecting a clamshell access (Kouchoukos (. Fig. 27.21). For selective cerebral perfusion, we
et al. 2007). Alternatively, at least aneurysms of the prefer employing self-occluding balloon catheters as
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
891 27
often adherent. The aortic arch is dissected free at
its ventral aspect as far as needed, always remain-
ing close to the aortic wall. Injury to phrenic,
vagus, and laryngeal recurrent nerves must be
avoided. During the cooling phase, the surgeon
can begin with the ascending aorta and valve pro-
cedure, which later on can be completed during
the more time-consuming rewarming phase. As
soon as the desired target temperature has been
reached, perfusion is stopped. During circulatory
arrest, the patient should be kept in a moderate
Trendelenburg position to reduce the risk of cere-
bral air embolism. The head lies below the level of
the surgical field; in this way, a certain fluid level is
maintained in the supra-aortic branches reaching
the arch. The aortic arch is incised from the ascend-
..Fig. 27.21 Selective perfusion of the brain during
ing segment as far as necessary for reconstruction,
aortic arch replacement. In order to shorten the duration
of total circulatory arrest, the innominate artery and in extreme cases even beyond the arterial ligament.
the left carotid artery can be intubated from the arch At this point, active measures are taken to pro-
lumen and perfused. The left subclavian artery is occluded tect the brain; here we apply selective cerebral
here with a Fogarty catheter perfusion via the first two aortic arch branches
(see 7 Sect. 27.4.1, «Access and Extracorporeal
used for retrograde administration of cardioplegic Circulation»). It may be helpful to first loop these
solution. We perform the entire arch procedure vessels because it is then easier to maintain the
under these conditions – irrespective of the extent of position of the perfusion catheter during lengthy
the arch replacement – with the body under circula- procedures.
tory arrest but brain circulation maintained. In our If extracorporeal circulation is established
experience and as a general rule, the entire aortic through arterial perfusion via the right axillary
arch replacement procedure takes between 40 and artery, the cerebrum can also be perfused via this
60 min, for proximal replacement only 15–25 min; route as an alternative, whereby the innominate
thus, lowering the temperature during circulatory artery is then clamped near the branch or blocked
arrest to 26–28 °C suffices. If deep hypothermic cir- with an occlusion catheter. It is advisable under
culatory arrest has to be instituted without selective these conditions to intubate the left carotid artery
cerebral perfusion for >15 min, nasopharyngeal tem- separately in order to ensure that both brain
peratures of <20 °C, better 16–18 °C, are required. hemispheres are supplied with blood.
During the rewarming phase, the perfusionist If, however, the surgeon decides for retrograde
ensures that the water temperature in the heat cerebral perfusion, the venous drainage catheters
exchanger does not exceed the arterial blood tem- in the superior vena cava should first be recon-
perature by more than 6–8 °C so as to prevent gas nected to the arterial line of the extracorporeal
bubbles from pearling from the oversaturated circulation. This technique requires bicaval can-
blood. The anesthesiologist compensates the nulation for extracorporeal circulation.
omnipresent, more or less pronounced acidosis in Now, the surgeon needs to determine the kind
a stepwise fashion. Administration of vasodilators and extent of aortic replacement. In principle, the
facilitates homogenous rewarming of the body. line of the prosthesio-aortic anastomosis needs to
lie far enough distally so that it can be sutured to
an intact segment of aortic wall. For atheroscle-
27.4.2 scending Aorta: Decision
A rotic aneurysms, this is often only possible after
Making plaques and atheromas have been completely
debrided. They must be removed by targeted
After connecting the patient to extracorporeal cir- suction and lavage of the arch including the origin
culation, the next step involves further mobilizing of the supra-aortic vessels. For aneurysms, the
and then clamping the ascending aorta, which is distal prosthesio-aortic anastomosis is placed
892 M. Karck and K. Kallenbach
There are four options for protecting the When a core body temperature of 26 °C has
brain during circulatory arrest: been reached after a least 30 min of cool-
55 Deep hypothermia (nasopharyngeal ing (measured both in the urinary bladder
temperature of 16–18 °C) and nasopharyngeally), the extracorporeal
55 Deep hypothermia combined with circulation is stopped and the aortic clamp
retrograde cerebral perfusion opened; in addition, the supra-aortic vessels
55 Deep hypothermia with selective are exposed. After intubating the innominate
antegrade cerebral perfusion artery and the left common carotid artery,
55 Moderate hypothermia of 26–28 °C the catheters are blocked carefully using
27 (depending on the duration of circulatory balloons and shifted cranially out of the
surgical field. Via a separate roller pump on
arrest) with selective antegrade cerebral
perfusion the heart-lung machine, cold, oxygenated
blood at 26 °C is infused at a rate of 10 ml/
kg body weight/min, which should produce
a perfusion pressure (measured in the right
Although the evidence is still questionable, radial artery) of 40–60 mmHg. The perfusion
we prefer selective antegrade cerebral perfu- cannulas are removed just before completing
sion under moderate hypothermia even for the aortic anastomosis. Then, extracorporeal
complete arch replacement up to 60 min and circulation is reestablished, so that the brain
more. One great advantage of this technique has only been without selective perfusion for
lies in the shorter warming period and avoid- a few minutes during circulatory arrest.With
ing very low core body temperatures, which this technique, the duration of circulatory
can promote clotting disorders and systemic arrest of >90 min can be tolerated without
inflammatory reactions. We also employ this increasing the rate of neurological complica-
technique for procedures at the proximal tions (Kazui et al. 2007).
aortic arch, which usually require shorter peri-
ods of circulatory arrest. For this, we use the
self-occluding balloon catheter for cerebral
perfusion that was developed originally for perfusion catheter. Now perfusion is resumed,
retrograde cardioplegia (RSCP MR 20, 15F, whereby the patient remains in Trendelenburg
Medtronic, Minneapolis, USA). position. While the prosthesis is filling, any resid-
ual air is expelled from the supra-aortic vessels by
careful digital or instrumental manipulation.
beyond the dilated segment of aorta where the Subsequently, the prosthesis is clamped proxi-
intima is usually intact. The surgeon can often mally and the rewarming process is initiated. Any
successfully create a beveled anastomosis in the anastomotic bleeding is stopped by placing rein-
arch so that the supra-aortic vessels do not need forced mattress sutures at the source.
to be joined to the arch prosthesis separately.
Notably, one should anastomose the bases of one,
two, or all three vessels with one window that is 27.4.3 ortic Arch Replacement
A
created at the convexity of the arch prosthesis for Aneurysms
(subtotal or total arch replacement). As an alter-
native to this technique, special vascular prosthe- 27.4.3.1 Proximal Replacement
ses with prefabricated side branches are available A proximal, partial aortic arch replacement is rec-
for attaching each individual supra-aortic vessel. ommended as a complementary step while repair-
Kazui and colleagues have gained substantial ing extensive ascending aorta aneurysms,
experience in using these implants, particularly in particularly in patients with dissections (Ohtsubo
conjunction with selective antegrade cerebral per- et al. 2002). We always do this when the ascending
fusion (Kazui et al. 2007). aorta is dilatated well beyond the level of the occlu-
After finishing the arch replacement, the vas- sion clamp that is always placed immediately prox-
cular prosthesis is cannulated with the arterial imal to the brachiocephalic trunk. After opening
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
893 27
the aortic arch and initiating selective antegrade sutures. Suturing begins directly distal of the
cerebral perfusion, a prosthesis is selected whose branch of the artery that is farthest downstream
diameter corresponds to that of the aortic lumen. and often involves the vessel itself. First, it is sewn
The tube graft is trimmed and beveled such that dorsally from out of the prosthetic orifice, later
the aneurysmatic part of the arch can be com- continued at the front, and finally tied just this
pletely replaced upstream of the selected anasto- side of the innominate artery branch (. Fig. 27.23).
motic line. Complete resection of the aneurysm During this procedure, it is sensible to insert a
often helps to create a primary, blood-tight suture suction catheter into the descending aorta through
line by anastomosing end to end for the entire
length. The anastomosis is started at the deepest
point of the surgical field in the concavity of the
arch with a row of initially open stitches (3/0
sutures, continuous everting). The stitches are then
placed along the dorsal and ventral circumference
of the aorta until they meet up directly to the right
of the innominate artery. If this vessel is also dilated
due to aneurysm, we recommend anastomosing its
distal segment to an 8-mm prosthesis end to end
before initiating hypothermic circulatory arrest
and joining this to the arch prosthesis later.
After expelling the air from the arch, the prox-
imal anastomosis is created directly above the
commissures. If the ascending aorta is being
replaced at the same time, the prosthetic stumps
are anastomosed in end-to-end fashion.
..Fig. 27.22 Total arch replacement. Distal end of the
27.4.3.2 Subtotal and Total Replacement prosthesis and orifice of the descending aorta have
already been anastomosed end to end. The arch
After opening the arch and initiating antegrade prosthesis is then sparingly fenestrated at its zenith across
cerebral perfusion, a prosthesis is selected whose from the branches of the supra-aortic vessels. A
diameter corresponds exactly to that of the aorta sufficiently great distance between the distal
at the planned level of anastomosis. If possible, prosthesio-aortic anastomosis and this window facilitates
the connection should be established at the orifice considerably anastomosing the island carrying the
supra-aortic vessels with the window of the prosthesis
of the descending aorta. Beginning at the deepest
point of the planned line of anastomosis, the pros-
thesis and aorta are joined by placing several open
turns of big bites using a running double-armed
3/0 sutures. Suturing is continued on both sides in
the aortic lumen, pulling the threads at regular
intervals, before ligating the sutures at the front.
Please note that the suture line should run rather
distal to the takeoff of the left subclavian artery,
which facilitates the anastomosis to the arch pros-
thesis considerably. If the subclavian artery take-
off has a significant distance from the left carotid
artery, it may sometimes be better to leave it out-
side the prosthetic graft, distal to the prosthesio-
aortic anastomosis.
The next step involves excising an oval piece of
prosthesis from the convexity of the arch graft that
corresponds exactly with the branches of the ves-
sels that will be anastomosed (. Fig. 27.22). Their ..Fig. 27.23 Beginning at the distal point of the
common base is then in turn anastomosed to the prosthetic window the dorsal margin of the island
arch prosthesis with continuous and deep 3/0 carrying the supra-aortic branches is anastomosed first
894 M. Karck and K. Kallenbach
27
kSurgical Technique
First extracorporeal circulation is initiated. For
proximal arch replacement, the body temperature
is cooled to 27–28 °C as measured both in the uri-
nary bladder and nasopharyngeally; for total ..Fig. 27.26 After applying the gelatin-
resorcin-formaldehyde glue components into the false
replacement, the body temperature is lowered to lumen, the resected wall layers are firmly adapted with
26 °C. These target temperatures apply under the Borst clamps
condition that the brain is still being protected by
antegrade arterial perfusion during circulatory may be reconstructed by placing suture lines sup-
arrest. Without brain perfusion, the body tem- ported with one or more Teflon felt strips (Fleck
perature should be reduced to a deep hypother- et al. 2003; Sabik et al. 2000). Here, a vascular
mic level first (16–18 °C nasopharyngeal or prosthesis that has been fashioned accordingly by
tympanal temperature). After ventricular fibrilla- beveling is anastomosed to the reconstructed aor-
tion ensues, the distal ascending aorta is occluded tic wall with continuous 3/0 everting sutures. In
by using a soft clamp, and the first steps are this case, the branches of the innominate artery,
undertaken to replace the proximal aorta. Once the left carotid artery, and the subclavian artery
the desired body temperature is reached, the aor- remain beyond this anastomosis. For total
tic clamp is removed and the arch is opened – first replacement of an acutely dissected aortic arch,
only narrowly – by making an incision from the the aortic wall layers must be stabilized enough to
ascending aorta to determine the point at which allow for a blood-tight anastomosis between the
the second (arch) prosthesis can be anastomosed. prosthesis and the distal aorta. First, we resect the
The aortic arch is then mobilized at least far dissected aortic arch in toto up to the descending
enough beyond this point to create enough space aorta, sparing one tissue cuff that is bearing the
to reinforce the site on the outside with Teflon felt supra-aortic branches. With regard to the stability
strips where the anastomosis will later be estab- of the anastomosis that will be created later, it is
lished. Likewise, the proximal segments of the particularly important to entirely spare the
innominate artery and left carotid artery, possibly adventitia of the descending aorta and the vascu-
also of the left subclavian artery, are mobilized. lar cuff carrying the arch branches. Before estab-
The goal of reconstructing an acutely dissected lishing the prosthesio-aortic anastomosis, the
aortic arch is to restore antegrade circulation of dissected aortic wall downstream is joined by glu-
the true lumen of the arch, possibly also their ing the tissue or by using a reconstruction suture
branches, by joining the wall of the false lumen that is reinforced with felt strips. Alternatively,
with that of the true lumen. For this, we use the prosthesio-aortic anastomosis can be estab-
gelatin-resorcin-formaldehyde tissue glue, which lished directly by using felt-armed sutures both
is first allowed to harden after adapting the wall on the outside and possibly also inside the aorta.
layers before the prosthesio-aortic anastomosis is Whenever possible, the aortic arch branches
established. Quite often additional reinforcement should be reconstructed with the aid of reinforced
of the suture line with a Teflon felt strip is advis- mattress sutures such that a common base is cre-
able (. Fig. 27.26). It has proven useful in such ated for the subsequent anastomoses with the
situations to completely transect the aorta proxi- aortic arch prosthesis (. Fig. 27.27). In contrast to
mal to the innominate artery branch in order to acute arch dissection, in chronic dissection, it is
then mobilize the arch and its vascular branches usually not necessary and mostly not possible to
from the dorsal side. Alternatively and instead of rejoin the aortic wall layers because scar tissue
gluing, the dissected layers of the aortic arch wall has already formed on the adventitia of the aorta
896 M. Karck and K. Kallenbach
a b
..Fig. 27.28 a, b Preparation for descending aorta replacement while replacing the arch using the «elephant trunk
technique.» a Already at the beginning of the arch replacement procedure the distal end of the aortic prosthesis is
invaginated (according to Borst). The folding edge of the invaginated prosthesis is anastomosed with the proximal end
of the descending aorta. Thereafter, the invaginated edge is pushed into the descending aorta with a long straight
clamp. This trunk-shaped prosthesis now lies freely in the aneurysmatic descending aorta. b The supra-aortic vessels are
then anastomosed to the arch prosthesis. The arterial perfusion cannula is finally transferred to the arch prosthesis
a b c
27
..Fig. 27.30 a–c Three variants of distal aortic arch replacement. a Beveled prosthesio-aortic anastomosis, which
spares the arch vessels on the native aortic wall. b Prosthesio-aortic anastomosis directly distal to the branch of the left
carotid artery; anastomosis of the left subclavian artery in the arch prosthesis. c Prosthesio-aortic anastomosis between
the innominate artery and the left common carotid artery. Implantation of the common base of the common carotid
and subclavian artery into a window of the arch prosthesis
access. More often, however, only the distal seg- gia – with the exception of emergency bleeding
ment of the aortic arch with the branches of the left from a ruptured aneurysm (Girardi et al. 2005;
carotid artery and the left subclavian artery need to Safi et al. 1998b; Schäfers et al. 1987; Schepens
be replaced via this access (. Fig. 27.30b, c). et al. 1999). Therefore, we perform these proce-
dures with the patients completely heparinized
27.4.6.2 anaging Intra- and
M and using the heart-lung machine for normo-
Extracorporeal Circulation thermic partial extracorporeal circulation. The
After sufficiently exposing the aorta, the sur- perfusion cannulas can often be placed intratho-
geon must decide whether to proceed under racically via the lateral access. Venous return
conditions of simple clamping of the aorta, may be accomplished by retrograde cannulation
under partial extracorporeal circulation (left of the right ventricle via the pulmonary artery
ventricular bypass or femorofemoral bypass), (solitary angulated catheter with a diameter of
or under deep hypothermia and circulatory 14 mm) and arterial inflow by cannulating the
arrest. distal descending aorta. Should intrathoracic
cannulation be cumbersome because of pericar-
kSimple Aortic Clamping and Extracorporeal dial adhesions or pronounced aortic sclerosis,
Circulation we prefer to access the left femoral vessels.
In the past, individual authors emphatically Usually pump flow volumes of 2.0 l/m2 body
propagated simple aortic clamping for distal surface area/minute are sufficient to achieve
aortic arch replacement, pointing to the advan- physiological pressure values in an adult patient
tages of not having to cannulate the aorta or to proximally and distally to the clamped segments
heparinize the patients. Indeed, the surgical of the aorta. The anesthesiologist, the perfusion-
mortality and risk of paraplegia did not seem to ist, and the surgeon must work closely together
be significantly higher when compared to the under continuous monitoring to maintain an
use of extracorporeal circulation (Crawford adequate equilibrium between the two circula-
et al. 1984; Hammerlijnk et al. 1989; Kay et al. tion segments.
1986). Convincing evidence has been presented As an alternative to this regimen, an isolated
that perfusion of the distal descending and tho- left ventricular bypass is often employed. The
racoabdominal aorta through active circulatory means of connecting and operating the centrifu-
bypass helps to minimize the risk of paraple- gal pumps, which can be used under mild
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
899 27
heparinization (5,000 IU), have been described (Borst et al. 1964). In 1984, Crawford et al. (1984)
frequently (DeBois et al. 2000). After administer- again reported on the utility of this technique for
ing heparin, the left common femoral artery is complex procedures of the aortic arch via a left-
cannulated. The pericardium is opened via a lon- lateral access. Today, such a procedure should
gitudinal incision dorsal to the course of the mainly be considered when the arch cannot be
phrenic nerve and a purse-string suture is placed controlled and clamped proximally from the
on the left lower or upper pulmonary vein. Then, dilated segment in large or even ruptured aneu-
the vessel is cannulated with an angulated perfu- rysms. Here, starting with moderate hypother-
sion catheter. If the lumen of the pulmonary veins mia, one should proceed according to the
is too narrow or they are not accessible for other protocol for circulatory arrest as outlined in
reasons, the left auricle represents an alternative 7 Sect. 27.4.1, «Access and Extracorporeal
site for cannulation with a straight venous can- Circulation». After arresting the circulation, the
nula. However, the catheter may not be put under aneurysm is opened longitudinally from distal to
any tension during perfusion as the auricular proximal. The patient is positioned with the head
myocardium tears easily. lowered. The ostia of the left carotid artery and
A heparin-free TDMAC shunt without a the innominate artery are then intubated with
pump (Argyle, Fa. Shervood, St. Louis, USA) can perfusion catheters and selective antegrade cere-
be applied during distal aortic arch and descend- bral perfusion is commenced. Again, the left sub-
ing aorta replacement procedures if the surgeon clavian artery is either clamped or blocked with a
abstains from active circulatory support via a Fogarty catheter. If the aortic valve is patent, we
blood pump. In this case, however, the shunt put the heart under isoelectric arrest by infusing
should originate in the ascending aorta (Verdant cardioplegic solution into the aortic root via a
et al. 1988). Another technique widely applied by blocked Foley catheter. Beforehand, the catheter
some teams involves establishing a temporary is inserted retrogradely under digital control via
vascular prosthetic bypass between the right sub- the aortic arch into the ascending aorta. The
clavian artery and the left iliac artery (Kogel prosthesis, which has a side arm, can now be
2001). anastomosed – possibly including one or more of
If surgery must be performed under simple the supra-aortic vessels – to the arch.
aortic clamping in an emergency setting, the Subsequently, the prosthesis is filled with blood,
anesthesiologist is primarily responsible for man- clamped distally, and the upper half of the body is
aging the patient, ensuring that the left ventricle is perfused via the side arm, while the lower half of
not overloaded in this phase and compensating the body continues to be perfused via the original
for any ensuing metabolic acidosis continuously site of cannulation (femoral artery or distal
and effectively. The proximally measured arterial descending aorta). The distal prosthesio-aortic
blood pressure is controlled by administering anastomosis is created during the rewarming
vasodilators such that baseline values are not phase.
exceeded to any significant degree. A Swan-Ganz
catheter provides information about the preload 27.4.6.3 Procedure at the Aorta
of the left ventricle. Induced vasodilation is termi- As already mentioned, a standard incision in the
nated right before removing the aortic clamp. bed of the fourth rib offers ideal access to the dis-
Blood gas analysis is required at 10- to 15-min tal aortic arch and to the proximal segment of the
intervals. Metabolic acidosis has to be antago- ascending aorta. Any pleural adhesions present
nized without delay before and especially after the must be carefully detached in order to prevent any
aortic clamps have been removed. At this point, bleeding into the lung – particularly under hepa-
we recommend restoring left pulmonary ventila- rinization. Adhesions of the lung with the aneu-
tion to support hemostasis. rysm are only detached as far as needed to
mobilize the aorta proximal and distal to the
kHypothermic Circulatory Arrest clamp. If a strip of aorta is free of adhesions dor-
Borst performed an operation of the aortic arch sally, the vessel lumen can later be opened in this
through a left-lateral access under deep hypo- area without having to detach the lung. The
thermia and circulatory arrest already in 1963 phrenic nerve is identified, the vagus nerve is
900 M. Karck and K. Kallenbach
protected with an elastic vessel loop, and the sion site, and the distal aorta are clamped. This
recurrent branches mobilized as far as possible. may not be required under circulatory arrest. The
The left common carotid and left subclavian arter- aneurysm is then opened along the longitudinal
ies are mobilized to enable later control. axis. In the presence of a type B dissection, one
Depending on the proximal extent of the repair, first encounters the false lumen and often also the
the aorta is mobilized between the innominate proximal «entry.» The free wall of the true lumen
artery and the left carotid artery or between that is excised immediately in order to gain an over-
and the left subclavian artery. We recommend view of any residual intercostal arterial bleeding;
first dissecting the tissue between these vessels these vessels are then oversewn with heavy
with scissors or electrocautery and then with the sutures. As soon as bleeding has ceased, the sur-
27 index finger until the front face of the trachea can geon decides upon the subsequent procedure at
be palpated. This is then followed in a caudal the proximal and distal aorta.
direction. The next step involves dissecting the
arterial ligament just at the aorta followed by care- kProximal Anastomoses for Aneurysms
ful dissection into the concavity of the aortic arch For distal arch aneurysms, the surgeon attempts
until the level of tracheal bifurcation is reached. to establish the proximal and distal anastomoses
Any tissue fibers behind the aortic arch should be at the point where the diameter of the aorta
dissected under visual control, if possible. approaches a normal size and where the condi-
Subsequently, the surgeon can navigate around tions of the wall are suitable for the anastomotic
the distal arch using a curved clamp and digital sutures – as shown by an intact intima. For aneu-
guidance. After dissecting the parietal pleura, the rysms, if possible, the proximal prosthesio-aortic
readily visible intercostal arterial branches 1–4 anastomosis at the distal arch is created such that
should be closed with metal clips right away so the supra-aortic vessels do not have to be joined to
that they do not need time-consuming sutures the prosthesis separately. The prosthesis is strongly
from inside, once the aneurysm is opened. beveled in order to sew it to the aortic wall at the
Irrespective of whether the surgeon is operat- lowest point, beginning in the concavity of the
ing under conditions of simple aortic clamping or arch (. Fig. 27.31a). Suturing is continued both at
extracorporeal circulation, the proximal aorta, the back and the front in preferably healthy aortic
the supra-aortic branches distal from the occlu- wall tissue until the level of the supra-aortic ves-
a b
..Fig. 27.31 a, b The proximal prosthesio-aortic anastomosis is created in the lumen of the open distal arch.
a The branch of the left subclavian artery remains downstream of the anastomosis line in this case. b The base of the
subclavian artery is anastomosed with the prosthesis. The distal prosthesio-aortic anastomosis is completed
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
901 27
sels is reached. The leftover prosthetic flap is then recommend anastomosing the prosthesis there so
sutured to the aortic wall first at the back and then that true and false lumens communicate.
at the front just before these vessels take off, For acute retrograde dissections extending to
whereby the ends of the thread are tied just left of the ascending segment, a beveled prosthesio-
the origin of the carotid artery or the subclavian aortic anastomosis should be avoided. Rather, the
artery. If for reasons of surgical technique these aortic arch should be circumferentially mobilized
vessels need to remain downstream of the anasto- up to the selected line of anastomosis and tran-
mosis just created, they can be anastomosed sected there. If there is not enough space and too
directly or via vascular bridges to the tubular pros- little tissue surface for gluing, the dissected wall
thesis at a later point. Two surgical steps in creat- layers are joined here, too, by placing Teflon felt
ing a prosthesio-aortic anastomosis between the strips on the outside and in between the layers
left common carotid artery and the subclavian using continuous mattress sutures so that there is
artery and subsequent implantation of the latter a firm opposing point to anastomose the prosthe-
vessel into the prosthesis are illustrated in sis. Normally, the left-lateral arch vessels, whose
. Fig. 27.31. The sutures for the distal prosthesio- branches may need to be reconstructed, are anas-
aortic anastomosis are either sewn end to end or tomosed directly to the arch prosthesis.
beveled such that large intercostal arterial branches
downstream of the seventh thoracic segment are kDistal Anastomosis
spared in order to preserve spinal perfusion. If Distal anastomoses for the combined replace-
extensive aortic replacement is required, it is usu- ment of the aortic arch and the descending aorta
ally better to implant the intercostal arterial are placed at that site where the vessel diameter
branches that come into question as an island into approaches a normal size, if possible. Often, how-
a window that has been created in the prosthesis ever, the aneurysmatic dilatation involves the
and to anastomose the end of the prosthesis with entire downstream aorta so that a compromise
the distal aorta. A prerequisite for preserving such must be made as regards which vessel segment
vessels, however, is that aortic wall is still available
should be replaced. Experience has shown that the
at the base that can be used for suturing. According aorta narrows when kinking toward the hiatus
to more recent observations, all thoracic intercos- aorticus, where it can be anastomosed quite often.
tal arterial branches can also be sacrificed under Removing the residual diseased aorta is then post-
certain conditions without increasing the risk of poned to a second procedure, which may be pre-
spinal cord injury (Etz et al. 2006). pared for using the distal elephant trunk technique
(see 7 Sect. 27.4.5, «Total Aortic Arch Replacement
kProximal Anastomosis for Dissection Using the Elephant Trunk Prosthesis»). The distal
Whenever possible, the proximal end of a type B prosthesio-aortic anastomosis must be made to
dissection extending retrograde to the aortic arch the true lumen for acute dissections. This implies
should be selected as the site for anastomosis so that the entire wall must be reconstructed by using
that sutures can be placed in a firm vascular wall. Teflon felt-reinforced mattress sutures. For chronic
If the proximal end of the dissection cannot be dissections, the distal prosthesio-aortic anastomo-
reached in acute dissections, the walling of the sis is always established with the scarred adventitia
false and true lumen proximal to the dilatation of of the aorta. However, first the floating part dissec-
the aorta, which is always present and quite exten- tion membrane should be excised so that both
sive, is joined so that the proximal prosthesio- lumina remain perfused. For this purpose, it may
aortic anastomosis can be established at this level. be necessary to release the distal aortic cross-
Gluing here is only possible in exceptional cases if clamp briefly. By using this maneuver, the dissec-
enough wall tissue is available. Thus, the tech- tion membrane can be excised under direct vision
nique of applying reinforced Teflon felt-supported over a length of 2–4 cm, and an additional incision
sutures to join the walls of a dissected aortic lumen of the membrane may even reach much farther.
is mostly utilized; this technique is described in We have made it our rule to sacrifice all inter-
7 Sect. 27.3.4, «Acute Type A Dissection». For costal arterial branches up to the level of the sixth
chronic retrograde type B dissections that have a thoracic segment, while larger vessels branching
firmer outer aortic wall as a result of scarring, we farther distally must be spared by using a beveled
902 M. Karck and K. Kallenbach
prosthesio-aortic anastomosis or implanting the segment of the ascending aorta is needed to sub-
relevant vessel-bearing islands of the aortic wall sequently anastomose the prosthesis. By extend-
in the prosthetic tube graft. For the distal anasto- ing the sternotomy to the umbilicus and excising
mosis itself, the aorta is incised to the right and the hepatic ligament, the surgeon can advance to
left of the clamp as far as necessary so that a 3-cm the aortic hiatus to mobilize the aorta. The vessel
strip of intact wall is preserved dorsally. First open is either doubly occluded or tangentially clamped
stitches are made out of this strip; subsequently, off. Then, a right-angled end-to-side anastomosis
the edge of the prosthesis and the cut edge of the is created between the prosthesis, which is at least
aorta are anastomosed on both sides and the 14-mm in diameter, and the aorta. At this point,
thread tied at the front. After carefully expelling the patient is fully heparinized. An incision is then
27 the air from the aortic arch, the vessel branches, made in the diaphragm, and the prosthetic tube,
and the prosthesis, the clamps are removed with from which the air has been expelled and then put
the patient in Trendelenburg position. The wall of under tension, is pulled along the right atrium to
the aneurysm is sewn closely around the pros- the ascending aorta in order to determine the
thetic tube graft for graft inclusion. length that is required. The graft is then tailored
The «elephant trunk» technique as described by strongly beveling and then anastomosing it to
in 7 Sect. 27.4.5 can also be employed for the dis- the right aspect of the clamped ascending aorta. In
tal anastomosis whenever it appears likely that the next step, the common bases of the cerebral
further downstream repair will become necessary and brachial bridges are joined to the aortic graft
in the future (Carrel et al. 2001). The distal end to side; after careful de-airing, these vessels
prosthesio-aortic anastomosis is then not fash- can be unclamped. The branches of the supra-aor-
ioned end to end in the standard way but rather tic vessels are ligated. Finally, the natural aortic
the descending aorta prosthesis is invaginated for blood stream is interrupted beyond the anasto-
several centimeters in order to connect the thus mosis, between the aortic prosthesis and the prox-
created edge of the prosthesis and the descending imal arch, by using a specialized plastic clip
aorta. Just before tightening the suture, the invag- developed by Carpentier et al. (1981). Alternatively
inate is manipulated into the distal aorta and the the aorta can be dissected between the anastomo-
anastomosis then completed. The prosthetic ses, cut and oversewn by placing reinforced, con-
stump that is suspended in the lumen of the tinuous mattress sutures. The affected segments of
downstream aorta serves to simplify the connec- the aorta will then thrombose up to the branches
tion of the thoracoabdominal aortic prosthesis in of the first vital arteries. Late outcome results of
a future operation. this complex procedure are not available.
The hybrid technique for aortic arch replace-
ment is in part similar to the method Carpentier
27.4.7 Alternative Techniques has described; however, it has only been used in
for Aortic Arch Replacement relatively small numbers of patients to date.
Circulatory aids are not required, and here, too,
An imaginative suggestion for managing exten- the aortic arch is first «debranched,» whereby the
sive aortic arch and descending aorta aneurysms, left subclavian artery is detached close to its
the «thrombo-occlusion» operation, was origi- branch and anastomosed end to side to the left
nated by Carpentier et al. (1981). This technique carotid artery. After establishing an end-to-side
presumes that a normal aortic base and normal anastomosis between the proximal branch of a
ascending aorta are present. The operation can be Y-prosthesis and the distal ascending aorta, the
performed without using mechanical circulatory left carotid artery is also detached close to its
support. A longitudinal sternotomy and pericar- branch and anastomosed end to end with the
diotomy are made to access the heart and large right distal branch of the Y-prosthesis.
vessels. The first segments of the ascending aorta Subsequently, this route can be unclamped and
and the supra-aortic vessels are mobilized. The perfused while the left branch of the prosthesis is
latter are anastomosed side to end with 8-mm anastomosed end to side to the innominate artery,
prosthetic bridges during short-term occlusion or which can then also be unclamped centrally. In a
clamping. The authors recommend preparing bi- second step, the entire aortic arch vessels can be
or trifurcational prostheses so that only a short excluded by transfemorally inserting an endograft
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
903 27
that has been selected to correspond to the As an alternative to this procedure, surgery
patient’s individual arch anatomy. Although the can be started by performing a right anterolateral
initially available early outcome data show a con- thoracotomy in the bed of the fourth rib. If the
siderably higher mortality than for similar tech- aneurysm can be dissected from the front part of
niques performed under extracorporeal the thorax with little difficulty, a longitudinal ster-
circulation, it is to be expected that with growing notomy is undertaken and the procedure con-
experience, this technique will also produce simi- ducted in the usual way. Otherwise, the right
larly good outcome as with «open» surgical treat- atrium (or one of the femoral veins) is cannulated
ment (Kotelis et al. 2011; Schumacher et al. 2006). with a large-lumen catheter and an arterial access
is established for extracorporeal circulation via a
peripheral artery. In presence of aortic valve
27.5 Reoperations regurgitation, the patient’s core temperature is
only cooled moderately to start with (30 °C) to
Even today reoperations after prior thoracic aortic avoid premature spontaneous atrial fibrillation. If
aneurysm surgery are considered to be extraordi- there is no regurgitation, hypothermia can be
narily difficult, high-risk procedures that pose a deepened as needed. Then the sternum is opened
great challenge to the surgical team’s imagination by using an oscillating saw. Here, like with the
and technical skill. It would be beyond the scope previous technique, if the aneurysm is damaged,
of this chapter to describe such multifaceted oper- the aorta can be mobilized and clamped after
ations at the various segments of the thoracic aorta short-term cessation of the extracorporeal perfu-
in detail. The reader is referred in this regard to the sion and induced ventricular fibrillation.
extensive experience as published by Crawford’s Instead of using a right-lateral technique, con-
group and others (Crawford et al. 1984; Yamashita trol of an aneurysm can be achieved by applying a
et al. 1998; Lombardi et al. 2003). In our own expe- bilateral anterolateral thoracotomy in the bed of
rience from a more modest number of such reop- the fourth rib («clamshell thoracotomy»), whereby
erations, the technique used for the ascending the thoracic segment that is in contact with the
aorta, the arch, and descending segments does not aneurysm is dissected horizontally. An access cre-
differ significantly from that for other difficult car- ated in this way is excellent but considerably more
diovascular reoperations – except for the fact that traumatic than a longitudinal sternotomy.
great care must be taken if a second sternotomy is
required. Essentially, therefore, we will discuss safe
opening of the chest. 27.6 Aneurysms and Dissections
An overview of the site and extent of a possibly of the Descending Aorta
present contact zone of the aneurysm with the
sternum is best gained by using contrast-enhanced 27.6.1 Treatment Strategies
computed tomography. If the aneurysm has inti-
mate contact with the anterior chest wall or if even The range of options for surgical treatment of dis-
osseous destruction has developed from pulsating eases of the descending aorta has been extended
pressure, we first establish extracorporeal circula- by the interventional methods (see 7 Chapter
tion via femorofemoral access. While continu- «Endovascular Surgical Therapy of Thoracic and
ously cooling on bypass, we separate the front Thoracoabdominal Disease of the Aorta», Sect. 28.6;
blade of the sternum. Subsequently, the incision is Sect. 28.7). This group of extra-anatomical recon-
then only completed in the inferior section, struction procedures, including what is known as
whereupon the diaphragmatic surface of the heart a «hybrid procedure», exclude the diseased sec-
is detached as far as necessary so that a vent cath- tions of the aorta by implanting one or more vas-
eter can be inserted transapically or into the lower cular endografts. By taking an endovascular
right pulmonary vein (interatrial groove) in the treatment approach, all forms of intraoperative
event that ventricular fibrillation develops in circulatory support through extracorporeal circu-
patients with aortic valve insufficiency. When lation or temporary placement of extra-anatomi-
reaching a body temperature of 25 °C, the extra- cal shunting can be avoided. These procedures
corporeal circulation is stopped briefly and full differ distinctly from conventional «open» surgi-
sternotomy is completed (Karck et al. 1996). cal procedures, in which the diseased segment of
904 M. Karck and K. Kallenbach
the aorta is not excluded but rather replaced with experience is based on this form of intraoperative
a vascular prosthesis. In open procedures, circulatory support.
circulatory support is needed in order to mini-
mize the risk of organ damage as a consequence of
inadequate perfusion as the aorta is cross-clamped 27.6.3 Indications
while vascular areas distal to the aorta are being
repaired. The indications for surgical replacement of the
descending aorta for aneurysms or dissections
depend on the etiology, the maximum vessel
27.6.2 Circulatory Support diameter, the temporal dynamics of the develop-
27 ing vascular dilatation, and the patient’s symp-
In addition to the passive circulatory support toms. For us, the indication to replace a diffuse,
techniques by temporarily placing shunts or an asymptomatic aneurysm of the descending
extra-anatomic vascular prosthetic bypass, active aorta with or without dissection is given in a
blood pumps with or without an integrated oxy- patient when the maximum vessel diameter
genator are available. Some groups prefer repair- exceeds 6 cm. If the number of risk factors
ing aneurysms not involving the arch by using a increases, for example, confirmed deficiencies in
centrifugal pump as an isolated left ventricular various organ functions, then the decision to
bypass; this reduces the risk of perioperative operate must be made on a very individual basis.
bleeding as the patient does not require full hepa- For clearly limited aneurysms that developed
rinization (Coselli and LeMaire 1999; DeBois because of a plaque rupture, the surgical indica-
et al. 2000; Schepens et al. 1999). A disadvantage tion may already be given in smaller maximum
of this technique as compared to classic extracor- vessel diameters even though the cutoff value has
poreal circulation, however, lies in the fact that not been reached yet because here the three-layer
hypothermia cannot be induced here. This is aortic walling is affected and the vascular walls
important for patients with proximal aneurysms may already be dangerously thin. The same
of the terminal aortic arch just involving the applies for suture line aneurysms that can
descending aorta. Indeed, it is not rare that the develop after post childhood patch repair of an
surgeon can only decide during the procedure aortic coarctation. Here, too, owing to the lack of
whether to open the arch in order to anastomose reliable data concerning the risk associated with
the vascular prosthesis with a healthy segment of the spontaneous course of rupture, the surgeon
the aorta. In such cases, extracorporeal circula- will likely decide for an early intervention
tion is used to induce a sufficiently deep level of (Aebert et al. 1993; Karck et al. 2002).
hypothermia – in our hands, the core body tem- Posttraumatic aneurysms of the descending
perature is cooled to 26 °C. After interrupting the aorta represent a special form for which no
extracorporeal circulation, we initiate selective proven, maximum tolerable vascular diameter
antegrade cerebral perfusion for neuroprotection can be given. In these cases, the temporal dynam-
(see 7 Sect. 27.4.6.2, «Managing Intra- and ics for increasing diameter of the affected seg-
Extracorporeal Circulation»). ment in the context of the individual surgical
We also cool the body core temperature for risk profile and age of the patient will speak for
surgeries in which circulatory arrest is not used, or against operative intervention.
for reasons of organ protection mediated by hypo-
thermia, but only by about 2–3 °C. In this regard,
our own technique differs from the method of 27.6.4 Selecting a Technique
Kouchoukos, who performed procedures of the
descending aorta under deep hypothermic car- 27.6.4.1 Thoracic Access
diocirculatory arrest (Kouchoukos et al. 2002). For proximal aneurysms of the descending aorta,
We consider the option of oxygenating the patients are positioned on their right side with
blood and being able to precisely regulate the the left arm in abduction. The pelvis is rotated
blood volume distribution as additional advan- slightly in a dorsal direction as this facilitates
tages of using extracorporeal circulation for pro- better access to the left inguinal vessels needed
cedures on the descending aorta. Thus, our recent for establishing the extracorporeal circulation.
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
905 27
To separately ventilate the right and left lung, a For this purpose, the aorta needs to be mobilized for
double-lumen endotracheal tube is required. For a short distance between the Th3 and Th5 segments,
access to the proximal aneurysm of the descend- whereby it is occasionally necessary to detach one
ing aorta, we choose standard thoracotomy in or two segmental arteries. In a medial direction, the
the fourth intercostal space (see 7 Sect. 27.4.6.1, esophagus, which is frequently adherent to the
«Surgical Access»). Via this access, the most prox- aneurysm, should be safely mobilized before clamp-
imally located diseased segments can be reached, ing and kept out of the level of clamping.
and, moreover, it is possible to prepare for clamp- The aorta is now double clamped, proximally
ing the aortic arch between the left common at a healthy, more or less normal site where the
carotid artery and the left subclavian artery. If proximal anastomosis with the vascular prosthesis
the arch is definitely not involved and instead is to be performed and distally at some distance,
the entire descending aorta up to the passage most often still within the diseased segment of the
into the diaphragm is to be replaced, it may be aorta. The vascular segment lying in- between
helpful to place the thoracotomy one intercostal these clamps is opened longitudinally and pre-
space deeper, partially resect the fifth rib, or cre- pared for anastomosing. Any intercostal vessels
ate an additional second access through the sev- that are still bleeding are oversewn. Thereafter, the
enth intercostal space (see 7 Sect. 27.4.6.1, aorta is cut subtotally or over the whole circum-
«Surgical Access»). ference, depending on which variant provides the
most secure anastomosis, and leaving an approx.
27.6.4.2 Thoracic Aorta Replacement 1-cm-wide tissue cuff for anastomosis. Continuous
The prophylactic effect of lumbar puncture in pro- suturing with a vascular prosthesis of equal diam-
tecting patients from spinal injury is disputed; eter can now be performed in a routine fashion,
nonetheless, however, we preoperatively drain the adding Teflon felt strips to the suture line for frag-
cerebrospinal fluid before carrying out any exten- ile tissue. With the vascular prosthesis clamped,
sive thoracic and thoracoabdominal aorta proce- the proximal anastomosis then allowed to be per-
dures (Coselli et al. 2002; Schepens et al. 2004). The fused and tested for any blood leakage.
catheter is inserted for this procedure on the day The next level for distal clamping is chosen as
before surgery in order to exclude any acute, punc- far downstream as needed to create the prosthesio-
ture-induced spinal bleeding before full heparin- aortic anastomosis with a segment of the aorta
ization for extracorporeal circulation is initiated. that shows only slight pathological changes. For
At sites that have not been operated upon pre- aneurysms that extend to the takeoff of the celiac
viously and when the distal descending aorta is trunk, an indentation needs to be made in the dia-
not affected, extracorporeal circulation can be phragm before clamping the aortic hiatus. If
established via intrathoracic cannulation of the clamping cannot be safely done at this site with-
pulmonary artery trunk and the distal descending out damaging the branch of the celiac trunk, an
aorta (see 7 Sect. 27.4.6.2, «Managing Intra- and occlusion catheter can be inserted into the distal
Extracorporeal Circulation»). This form of cannula- aorta under visual control. For this, however, the
tion spares exposing additional peripheral vessels extracorporeal circulation needs to be stopped for
like femoral artery and vein. a few seconds in order to open the aorta.
For aneurysms of the descending aorta that do The aorta is then incised longitudinally. For
not involve the arch, the aorta can usually be aortic dissections, the dissection membrane is
clamped directly behind the takeoff of the left sub- resected at its origin in the aortic wall. Any inter-
clavian artery. The phrenic and vagal nerves are costal vessels from the proximal aorta segment that
mobilized by creating a tissue pedicle and held are still bleeding are oversewn. Segmental arteries
aside: these nerves should not be skeletonized. As originating further distally can be reimplanted
for terminal aortic arch replacement, great care individually or in multiples with continuous
must be taken here, too, when exposing and mobi- sutures in a vascular prosthetic window corre-
lizing the recurrent nerve in order to protect it sponding to the vascular tissue island; this improves
from injury caused by clamping or suturing. spinal cord perfusion. The criteria for selecting
The second clamp is placed such that the proxi- these vessels are still the subject of scientific contro-
mal prosthesio-aortic anastomosis can be estab- versy. As described in 7 Sect. 27.4.6.3, «Procedures at
lished under the best possible technical conditions. the Aorta», we prefer to liberally reimplant most
906 M. Karck and K. Kallenbach
27
large-lumen intercostal vessel branches that origi- the seventh intercostal space needs to be per-
nate from the lower half of the thoracic aorta formed using the same skin incision in order to
because the collateral supply of the middle and gain safe access to the infradiaphragmatic aorta.
lower spinal cord is less well developed than in the In contrast, thoracotomy in the seventh intercos-
more cranial segment (Biglioli et al. 2004; Minatoya tal space is sufficient to adequately expose the tho-
et al. 2002). racic aorta segment that must be replaced for type
Finally, the distal prosthesio-aortic anastomo- III or IV aneurysms.
sis is created, depending on the wall conditions Independent of type of aneurysm, the left rib
either by using an inlay technique or an end-to- arch at the ventral end of the thoracotomy in the
end anastomosis. sixth or seventh intercostal space is dissected first.
Creating a «graft inclusion» from native aortic From here, the surgeon begins to mobilize the dia-
wall tissue can occasionally help to stop any bleed- phragm in a circular pattern up to the aortic hiatus,
ing caused by the suturing in the area of the inter- whereby a rim of 3 cm is left at the point of insertion
costal artery anastomoses. Covering the vascular of the diaphragm at the thoracic wall. Compared to
prosthesis with native tissue might also protect the alternative of radial mobilization of the dia-
the site against infection and erosion. phragm, this technique spares the phrenic nerve
and facilitates later reconstruction. With progress-
27.6.4.3 Thoracoabdominal Access ing ventrodorsal mobilization of the diaphragm,
For type I and II aneurysms of the thoracoab- the intact peritoneal sac can be mobilized carefully
dominal aorta according to Crawford (. Fig. 27.32; in a craniocaudal direction on Gerota’s fascia and
Svensson et al. 1993), we prefer performing thora- medial up to the aorta. In the next step, the left-lat-
cotomy in the sixth intercostal space and continu- eral portion of the diaphragm can be sharply dis-
ing the incision above the rib arch from pararectal sected from the aorta in the hiatus, whereby the
in a caudal direction. For aneurysms that extend branches of the celiac trunk and the superior mes-
far upstream in the direction of the aortic arch, it enteric artery can be exposed at their origin from
may be necessary to choose a thoracotomy one or the aorta. The insertion at the aorta and the course
even two intercostal spaces farther cranial. In of the left renal artery can now be identified from
these less frequent cases, a second thoracotomy in dorsal. Further distal and in the same preparation
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
907 27
step from the left, the infrarenal aorta is reached artery in an end-to-end fashion (Vascutek-
from dorsal, which can now be followed and con- Terumo, Gelweave R – thoracoabdominal pros-
trolled via this access up to the left iliac artery. thesis after Coselli).
Finally, perfusion of the visceral organs can be
27.6.4.4 Thoracoabdominal Aorta restored before completing the reconstruction by
Replacement establishing the distal prosthesio-aortic anastomo-
After exposing the diseased segment of the thora- sis, depending on the aortic pathology. This anasto-
coabdominal aorta, we begin with the vascular mosis is created before, at, or behind the iliac artery
prosthetic replacement in craniocaudal order. In bifurcation as a Y-prosthesis. Before weaning the
the same manner as described for thoracic aorta patient from the extracorporeal circulation, not
replacement, the aorta is cross-clamped proximal least for reasons of better spontaneous hemostasis,
to the lesion as well as some distance downstream. the body temperature should be normal. Like for
In between these two clamps, the aorta is incised replacement of thoracic aorta, the prosthesis should
longitudinally. After completion of the proximal be enclosed by native aorta as graft inclusion.
prosthesio-aortic anastomosis the next, farther
distal level is selected for clamping. Depending on
the extent of the aneurysm, this can lie proximal References
of the celiac trunk branch or infrarenally, yet
down to the level of the iliac arteries. Aebert H, Laas J, Bednarski P, Koch U, Prokop M, Borst HG
(1993) High incidence of aneurysm formation follow-
If the first variant is chosen, a cross-clamped ing patch plasty repair of coarctation. Eur J Cardiothorac
aorta at this site can represent a considerable Surg 7:200–204
obstacle for reimplanting deeply lying intercostal Anagnostopoulos CE, Prabhakar MJ, Kittle CF (1972) Aortic
arteries into the vascular prosthesis. In this case, dissections and dissecting aneurysms. Am J Cardiol
we prefer to clamp infrarenally to then open the 30:263–273
Aybek T, Sotiriou M, Wöhleke T et al. (2005) Valve opening
entire length of the aorta. After intubating the ori- and closing dynamics after different aortic valve-
fices of the celiac trunk, the superior mesenteric sparing operations. J Heart Valve Dis 14:114–120
artery, and the left and right renal arteries with Bachet J, Guilmet D (2002) Brain protection during surgery
perfusion catheters, organ perfusion with arterial of the aortic arch. J Card Surg 17:115–124
blood is commenced via the extracorporeal circu- Bachet J, Guilmet D, Goudot B et al. (1991) Cold cerebro-
plegia. A new technique of cerebral protection during
lation. Proceeding in this way prevents ischemia operations on the transverse aortic arch. J Thorac
of the visceral organs after this segment of the Cardiovasc Surg 102:85–93
aorta is opened and thus taken off the extracorpo- Bachet J, Termignon JL, Goudot B et al. (1996) Aortic root
real circulation. Moreover, it facilitates identifica- replacement with a composite graft. Factors influenc-
tion of the intercostal or lumbar vessels that will ing immediate and long-term results. Eur J Cardiothorac
Surg 10:207–213
be selected for reimplantation in the prosthesis: Bachet J, Goudot B, Dreyfus GD et al. (1999) Surgery for
retrograde blood can no longer reach the already acute type A aortic dissection: the hospital Foch expe-
quite deeply situated site of the aorta via the intu- rience. Ann Thorac Surg 67:2006–2009
bated ostia of the visceral arteries and obstruct the Bail DH, Walker T, Gruler M, Ziemer G (2007) Preserved
view. After reimplantation of one to three seg- Windkessel function in patients following reduction
aortoplasty of the ascending aorta. Echocardiography
mental arteries from the thoracolumbar transi- 24:457–463
tional zone, the visceral arteries are anastomosed Baraki H, Hagl C, Khaladj N et al. (2007) The frozen elephant
either separately or en bloc to the central vascular trunk technique for treatment of thoracic aortic aneu-
prosthesis. It is often possible here to reimplant en rysms. Ann Thorac Surg 83:S819–S823
bloc the branches of the right renal artery, the Bavaria JE, Woo YJ, Hall RA, Wahl PM, Acker MA, Gardner TJ
(1996) Circulatory management with retrograde cere-
superior mesenteric artery, and the celiac trunk bral perfusion for acute type A aortic dissection.
together on a tissue island. Only the left renal Circulation 94(9 Suppl):II173–II176
artery, which branches somewhat further away, Benedetto U, Melina G, Takkenberg JJ et al. (2011) Surgical
needs to be anastomosed separately. management of aortic root disease in Marfan syndrome:
a systematic review and meta-analysis. Heart 97:955–958
As an alternative to this procedure, special vas-
Bentall H, De Bono A (1968) A technique for complete
cular prostheses with anatomically adapted, pre- replacement of the ascending aorta. Thorax 23:338–339
fabricated side branches can be used to separately Biglioli P, Roberto M, Cannata A et al. (2004) Upper and
create the anastomosis for each individual visceral lower spinal cord blood supply: the continuity of the
908 M. Karck and K. Kallenbach
anterior spinal artery and the relevance of the lumbar Crawford ES, Crawford JL, Stowe CL, Safi HJ (1984) Total
arteries. J Thorac Cardiovasc Surg 127:1188–1192 aortic replacement for chronic dissection occurring in
Borst HG (1981) Replacement of ascending aorta and aor- patients with and without Marfan’s syndrome. Ann
tic valve. How to do it. Ann Thorac Surg 32:613 Surg 199:358–362
Borst HG, Schaudig A, Rudolph W (1964) Arteriovenous fis- Crawford ES, Crawford JL, Safi HJ et al. (1986)
tula of the aortic arch: repair during deep hypother- Thoracoabdominal aortic aneurysms: preoperative
mia and circulatory arrest. J Thorac Cardiovasc Surg and intraoperative factors determining immediate
48:443–447 and long-term results of operations in 605 patients.
Borst HG, Walterbusch G, Schaps D (1983) Extensive aortic J Vasc Surg 3:389–404
replacement using “elephant trunk” prosthesis. Thorac Crawford ES, Coselli JS, Safi HJ (1987) Partial cardiopulmo-
Cardiovasc Surg 31:37–40 nary bypass, hypothermic circulatory arrest, and pos-
Cabrol C, Pavie A, Gandjbakhch I et al. (1981) Complete terolateral exposure for thoracic aortic aneurysm
27 replacement of the ascending aorta with reimplanta-
tion of the coronary arteries: new surgical approach.
operation. J Thorac Cardiovasc Surg 94:824–827
Crawford ES, Coselli JS, Svensson LG, Safi HJ, Hess KR (1990)
J Thorac Cardiovasc Surg 81:309–315 Diffuse aneurysmal disease (chronic aortic dissection,
Cabrol C, Pavie A, Mesnildrey P et al. (1986) Long-term Marfan, and mega aorta syndromes) and multiple
results with total replacement of the ascending aorta aneurysm. Treatment by subtotal and total aortic
and reimplantation of the coronary arteries. J Thorac replacement emphasizing the elephant trunk opera-
Cardiovasc Surg 91:17–25 tion. Ann Surg 211(5):521–537
Carpentier A, Deloche A, Fabiani JN et al. (1981) New surgi- Creech O Jr, De Bakey ME, Mahaffey DE (1956) Total resec-
cal approach to aortic dissection: flow reversal and tion of the aortic arch. Surgery 40:817–830
thromboexclusion. J Thorac Cardiovasc Surg 81: David TE, Feindel CM (1992) An aortic valve–sparing oper-
659–668 ation for patients with aortic incompetence and aneu-
Carrel T, Berdat P, Kipfer B, Eckstein F, Schmidli J (2001) The rysm of the ascending aorta. J Thorac Cardiovasc Surg
reversed and bidirectional elephant trunk technique 103:617–622
in the treatment of complex aortic aneurysms. David TE, Armstrong S, Ivanov J, Feindel CM, Omran A,
J Thorac Cardiovasc Surg 122:587–591 Webb G (2001) Results of valve-sparing operations.
Carrel TP, Berdat P, Englberger L et al. (2003) Aortic root J Thorac Cardiovasc Surg 122:39–46
replacement with a new stentless aortic valve xeno- David TE, Feindel CM, Webb GD, Colman JM, Armstrong S,
graft conduit: preliminary hemodynamic and clinical Maganti M (2007) Aortic valve preservation in patients
results. J Heart Valve Dis 12:752–757 with aortic root aneurysm: results of the reimplanta-
Chavan A, Lotz J, Oelert F, Galanski M, Haverich A, Karck M tion technique. Ann Thorac Surg 83:S732–S735
(2003) Endoluminal treatment of aortic dissection. Eur David TE, Armstrong S, Manlhiot C, McCrindle BW, Feindel
Radiol 13:2521–2534 CM (2012) Long-term results of aortic root repair using
Chuter TA (2007) Fenestrated and branched stent-grafts the reimplantation technique. J Thorac Cardiovasc Surg.
for thoracoabdominal, pararenal and juxtarenal aortic doi:10.1016/j.jtcvs.2012.11.075 [Epub ahead of print]
aneurysm repair. Semin Vasc Surg 20:90–96 De Bakey ME, Crawford ES, Cooley DA, Morrs GC Jr (1957)
Cipriano PR, Griepp RB (1979) Acute retrograde dissection Successful resection of fusiform aneurysm of aortic
of the descending aorta. Am J Cardiol 43:520–528 arch with replacement by homograft. Surg Gynecol
Cooley DA, De Bakey ME (1952) Surgical considerations of Obstet 105:657–664
intrathoracic aneurysms of the aorta and great vessels. De Bakey ME, Beall AD, Cooley DA et al. (1966) Dissecting
Ann Surg 135:660–680 aneurysms of the aorta. Surg Clin North Am 46:
Cooley DA, De Bakey ME (1956) Resection of entire ascend- 1045–1055
ing aorta in fusiform aneurysms using cardiac bypass. De Bakey ME, McCollum CH, Crawford ES, Morris GC,
JAMA 162:1158 Howell H, Noon GP (1982) Dissection and dissecting
Cooley DA, Mahaffey DE, De Bakey ME (1955) Total excision aneurysms of the aorta: twenty-year follow-up of five
of the aortic arch for aneurysms. Surg Gynecol Obstet hundred twenty-seven patients treated surgically.
101:667–677 Surgery 92:1118–1134
Coselli JS, LeMaire SA (1999) Left heart bypass reduces De Paulis R, De Matteis GM, Nardi P, Scaffa R, Colella DF,
paraplegia rates after thoracoabdominal aortic aneu- Chiarello L (2000) A new aortic Dacron conduit for sur-
rysm repair. Ann Thorac Surg 67:1931–1934 gical treatment of aortic root pathology. Ital Heart
Coselli JS, Lemaire SA, Köksoy C, Schmittling ZC, Curling PE J 1:457–463
(2002) Cerebrospinal fluid drainage reduces paraplegia De Paulis R, De Matteis GM, Nardi P, Scaffa R, Bassano C,
after thoracoabdominal aortic aneurysm repair: results Chiariello L (2002) Analysis of valve motion after the reim-
of a randomized clinical trial. J Vasc Surg 35:631–639 plantation type of valve-sparing procedure (David I) with
Crawford ES, Saleh CA (1981) Transverse aorta and aneurysm: a new aortic root conduit. Ann Thorac Surg 74:53–57
improved results of treatment employing new modifica- De Paulis R, Cetrano E, Moscarelli M et al. (2005) Effects of
tions of aortic reconstruction and hypo-thermic cerebral ascending aorta replacement on aortic root dilatation.
circulatory arrest. Ann Thorac Surg 194:180–188 Eur J Cardiothorac Surg 27:86–89
Crawford ES, Saleh SA, Schwessler JS (1979) Treatment of DeBois WJ, Girardi LN, Lawrence S et al. (2000) Perfusion
aneurysm of transverse aortic arch. J Thorac Cardiovasc method for thoracoabdominal aneurysm repair using
Surg 78:383–393 the open distal technique. Perfusion 15:231–236
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
909 27
Demers P, Miller DC (2004) Simple modification of “T. David-V” Hagl C, Ergin MA, Galla JD et al. (2002) Neurologic outcome
valve-sparing aortic root replacement to create graft after ascending aorta-aortic arch operations: effect of
pseudosinuses. Ann Thorac Surg 78:1479–1481 brain protection technique in high-risk patients.
Di Eusanio M, Schepens MA, Morshuis WJ, Di Bartolomeo R, J Thorac Cardiovasc Surg 123:581–582
Pierangeli A, Dossche KM (2002) Antegrade selective Hagl C, Khaladj N, Karck M et al. (2003) Hypothermic circu-
cerebral perfusion during operations on the thoracic latory arrest during thoracic aortic surgery: the theo-
aorta: factors influencing survival and neurologic out- retical impact of different cerebral perfusion
come in 413 patients. J Thorac Cardiovasc Surg 124: techniques and other methods of cerebral protection.
1080–1086 Eur J Cardiothorac Surg 24:358–363
Egloff L, Rothlin M, Kugelmeier J, Senning A, Turina M Hammerlijnk R, De Geest R, Brutel de la Reviere A, Defauw
(1982) The ascending aortic aneurysms: replacement J, Knaepen P, Venneulen F (1989) Surgical correction of
or repair? Ann Thorac Surg 34:117 descending thoracic aneurysms with shunt or bypass
Ehrlich MP, Ergin MA, McCullough JN et al. (2001) Favorable techniques versus simple aortic crossclamping. Eur
outcome after composite valve-graft replacement in J Cardiothorac Surg 3:37–43
patients older than 65 years. Ann Thorac Surg 71: Heberer G, Borst HG, Grill W, Eberlein HJ (1960) Zur
1454–1459 Anwendung eines extrakorporalen Umgehungskreislaufs
Erbel R, Aboyans V, Boileau C et al. (2014) ESC guidelines für Operationen an der descendierenden thorakalen
on the diagnosis and treatment of aortic diseases. Eur Aorta. Langenbecks Arch 296:317–343
Heart J 35(4):2873–2926 Hirazka LF, Bakris GL, Beckmann JA et al. (2010) 2010 ACCF/
Etz CD, Halstead JC, Spielvogel D et al. (2006) Thoracic and AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guideline
thoracoabdominal aneurysm repair: is reimplantation for the diagnosis and management of patients with
of spinal cord arteries a waste of time? Ann Thorac Thoracic Aortic Disease. Circulation 121:e266–e369
Surg 82:1670–1677 Hiratzka LF, Creager MA, Isselbacher EM et al. (2016)
Etz CD, Homann TM, Rane N et al. (2007) Aortic root recon- Surgery for aortic dilatation in patients with bicuspid
struction with a bioprosthetic valved conduit: a con- aortic valves. A statement of clarification from the
secutive series of 275 procedures. J Thorac Cardiovasc American College of Cardiology/American Heart
Surg 133:1455–1463 Association Task Force on Clinical Practise Guidelines.
Fann JI, Smith JA, Miller DC (1995) Surgical management of Circulation 133(7):680–688
aortic dissection during a 30-year period. Circulation Kallenbach K, Karck M, Leyh RG et al. (2002) Valve sparing
92:113–121 aortic root reconstruction in patients with significant
Fleck TM, Tschernich H, Grabenwoger M et al. (2003) A insufficiency. Ann Thorac Surg 74:S1765–S1768
double patch sandwich technique for surgical repair Kallenbach K, Karck M, Oelze T et al. (2004) Evolving strate-
of acute aortic dissection type A. Ann Thorac Surg gies for treatment of acute aortic dissection type
76:499–502 A. Circulation 110(Suppl II):II-243–II-249
Frist WH, Baldwin JC, Starnes VA et al. (1986) A reconsidera- Kallenbach K, Karck M, Pak D et al. (2005) Decade of aortic
tion of cerebral perfusion in aortic arch replacement. valve sparing reimplantation: are we pushing the lim-
Ann Thorac Surg 42:273–281 its too far? Circulation 112(Suppl I):I-253–I-259
Fukunaga S, Karck M, Harringer W, Cremer J, Rhein C, Kallenbach K, Baraki H, Khaladj N et al. (2007) Aortic valve –
Haverich A (1999) The use of gelatin-resorcin-formalin sparing operation in Marfan syndrome: what do we
glue in acute aortic dissection type A. Eur know after a decade? Ann Thorac Surg 83:S764–S768
J Cardiothorac Surg 15:564–569 Kamiya H, Klima U, Hagl C et al. (2006) Short moderate
Girardi LN, Krieger KH, Mack CA, Lee LY, Tortolani AJ, Isom hypothermic circulatory arrest without any adjunctive
OW (2005) Does cross-clamping the arch increase the cerebral protection for surgical repair of the ascending
risk of descending thoracic and thoracoabdominal aorta extending into the proximal aortic arch: is it
aneurysm repair? Ann Thorac Surg 79:133–137 safe? Heart Surg Forum 9:E759–E761
Gott VL, Gillinov AM, Pyeritz RE et al. (1995) Aortic root Kamiya H, Hagl C, Kropivnitskaya I et al. (2007) The safety
replacement. Risk factor analysis of a seventeen-year of moderate hypothermic lower body circulatory
experience with 270 patients. J Thorac Cardiovasc arrest with selective cerebral perfusion: a propensity
Surg 109:536–544 score analysis. J Thorac Cardiovasc Surg 133:501–509
Gott VL, Greene PS, Alejo DE et al. (1999) Replacement of Karck M, Haverich A (2005) Aortic valve reimplantation
the aortic root in patients with Marfan’s syndrome. N according to David type I technique. Oper Tech Thorac
Engl J Med 340:1307–1313 Cardiovasc Surg 10:246–258
Greenberg RK, West K, Pfaff K et al. (2006) Beyond the aortic Karck M, Cremer J, Wawersik J, Haverich A (1996)
bifurcation: branched endovascular grafts for thoracoab- Reoperation for aneurysmal disease of the ascending
dominal and aortoiliac aneurysms. J Vasc Surg 43:879–886 aorta in patients with concomitant aortic valve incom-
Griepp RB, Stinson EB, Hollingsworth JF, Buehler D (1975) petence. J Thorac Cardiovasc Surg 112:1392–1393
Prosthetic replacement of the aortic arch. J Thorac Karck M, Leyh R, von Kodolitsch Y, Haverich A (2002)
Cardiovasc Surg 70:1051–1063 Patchwerweiterungsplastik bei Aortenisthmusstenose.
Hagan PG, Nienaber CA, Isselbacher EM et al. (2000) The Gefahr von lokalen Spätaneurysmen. Dtsch Ärztebl
International Registry of Acute Aortic Dissection 100:A416–A419
(IRAD): new insights into an old disease. JAMA 283: Karck M, Chavan A, Hagl C, Friedrich H, Galanski M,
897–903 Haverich A (2003) The frozen elephant trunk tech-
910 M. Karck and K. Kallenbach
nique: a new treatment for thoracic aortic aneurysms. patients over a sixteen-year period. J Thorac
J Thorac Cardiovasc Surg 125:1550–1553 Cardiovasc Surg 78:365
Karck M, Kallenbach K, Hagl C et al. (2004) Aortic root sur- Minatoya K, Karck M, Hagl C et al. (2002) The impact of spi-
gery in Marfan’s syndrome: comparison of aortic valve nal angiography on the neurological outcome after
sparing reimplantation versus composite grafting. surgery on the descending thoracic and thoracoab-
J Thorac Cardiovasc Surg 127:391–398 dominal aorta. Ann Thorac Surg 74:S1870–S1872
Karck M, Chavan A, Khaladj N, Friedrich H, Hagl C, Haverich A Minatoya K, Karck M, Szpakowski E, Harringer W, Haverich
(2005) The frozen elephant trunk technique for the treat- A (2003) Ascending aortic cannulation for Stanford
ment of extensive thoracic aortic aneurysms: operative type A acute aortic dissection: another option.
results and follow-up. Eur J Cardiothorac Surg 28:286–290 J Thorac Cardiovasc Surg 125:952–953
Kawahito K, Adachi H, Murata S, Yamaguchi A, Ino T (2003) Mingke D, Dresler C, Stone CD, Borst HG (1998) Composite
Coronary malperfusion due to type A aortic dissec- graft replacement of the aortic root in 335 patients
27 tion: mechanism and surgical management. Ann
Thorac Surg 76:1471–1476
with aneurysm or dissection. Thorac Cardiovasc Surg
46:12–19
Kay GL, Cooley DA, Livesay JL, Reardon MJ, Duncan JM Muller WH Jr, Waren WD, Blanton FG Jr (1960) A method for
(1986) Surgical repair of aneurysms involving the dis- resection of aortic arch aneurysms. Ann Surg 151:225
tal aortic arch. J Thorac Cardiovasc Surg 91:397–404 Neri E, Toscano T, Papalia U et al. (2001) Proximal aortic dis-
Kazui T, Yamashita K, Washiyama N et al. (2007) Aortic arch section with coronary malperfusion: presentation,
replacement using selective cerebral perfusion. Ann management, and outcome. J Thorac Cardiovasc Surg
Thorac Surg 83:S796–S798 121:552–560
Kogel H (2001) Extraanatomische Bypasschirurgie. In: Nienaber CA, Kische S, Ince H (2007) Thoracic aortic stent-
Hepp W, Kogel H (Hrsg.) Gefäßchirurgie. Urban & graft devices: problems, failure modes, and applicabil-
Fischer, München Jena, S. 475–482 ity. Semin Vasc Surg 20:81–89
Kotelis D, Geisbüsch P, Attigah N et al. (2011) Total vs hemi- Ohtsubo S, Itoh T, Takarabe K et al. (2002) Surgical results of
aortic arch transposition for hybrid aortic arch repair. hemiarch replacement for acute type A dissection.
J Vasc Surg 54:1182–1186 Ann Thorac Surg 74:S1853–S1856
Kouchoukos NT, Marshall WG, Wedige-Stecher TA (1986) Orihashi K, Sueda T, Watari M, Okada K, Ishii O, Matsuura Y
Eleven-year experience with composite graft replace- (2001) Endovascular stent-grafting via the aortic arch
ment of the ascending aorta and aortic valve. J Thorac for distal aortic arch aneurysm: an alternative to endo-
Cardiovasc Surg 92:691–705 vascular stent-grafting. Eur J Cardiothorac Surg 20:
Kouchoukos NT, Masetti P, Rokkas CK, Murphy SF (2002) 973–978
Hypothermic cardiopulmonary bypass and circulatory Orihashi K, Sueda T, Okada K, Imai K (2004) Near-infrared
arrest for operations on the descending thoracic and tho- spectroscopy for monitoring cerebral ischemia during
racoabdominal aorta. Ann Thorac Surg 74:S1885–S1887 selective cerebral perfusion. Eur J Cardiothorac Surg
Kouchoukos NT, Mauney MC, Masetti P, Castner CF (2007) 26:907–911
Optimization of aortic arch replacement with a one- Ott DA, Frazier OH, Cooley DA (1978) Resection of the aor-
stage approach. Ann Thorac Surg 83:S811–S814 tic arch using deep hypothermia and temporary circu-
Krüger T, Weigang E, Hoffmann I et al. (2011) Cerebral pro- latory arrest. Circulation 58(Suppl I):227–231
tection during surgery for acute aortic dissection type Reuthebuch O, Schurr U, Hellermann J et al. (2004)
A: results of the German registry for Acute Aortic Advantages of subclavian artery perfusion for repair of
Dissection Type A (GERRADA). Circulation 124:434–443 acute type A dissection. Eur J Cardiothorac Surg 26:
Krüger T, Conzelmann LO, Bonser RS et al. (2012) Acute 592–598
aortic dissection type A. Br J Surg 99:1331–1344 Robicsek F (1982) A new method to treat fusiform aneu-
Lansman SL, McCullough JN, Nguyen KH et al. (1999) rysms of the ascending aorta associated with aortic
Subtypes of acute aortic dissection. Ann Thorac Surg valve disease: an alternative to radical resection. Ann
67:1975–1978 Thorac Surg 34:92–94
Lesèche G, Castier Y, Petit MD et al. (2001) Long-term Robicsek F (1985) Compression of the true lumen by retro-
results of cryopreserved arterial allograft reconstruc- grade perfusion during repair of aortic dissection.
tion in infected prosthetic grafts and mycotic aneu- J Cardiovasc Surg 26:36–40
rysms of the abdominal aorta. J Vasc Surg 34:616–622 Ross DN (1967) Replacement of the aortic and mitral valves
Lombardi JV, Carpenter JP, Pochettino A, Sonnad SS, Bavaria with a pulmonary autograft. Lancet 2:956–959
JE (2003) Thoracoabdominal aortic aneurysm repair Sabik JF, Lytle BW, Blackstone EH, McCarthy PM, Loop FD,
after prior aortic surgery. J Vasc Surg 38:1185–1190 Cosgrove DM (2000) Long-term effectiveness of oper-
Markewitz A, Weinhold C, Kempkes B, Reicherts B, Hemmer ations for ascending aortic dissections. J Thorac
W, Klinner W (1986) Surgical treatment of aneurysms Cardiovasc Surg 119:946–962
of the ascending aorta: 12 years’ experience in 66 Safi HJ, Miller CC, Reardon MJ et al. (1998a) Operation for
patients. Thorac Cardiovasc Surg 34:287–291 acute and chronic aortic dissection: recent outcome
Melissano G, Bertoglio L, Civilini E et al. (2007) Results of with regard to neurologic deficit and early death. Ann
thoracic endovascular grafting in different aortic seg- Thorac Surg 66:402–411
ments. J Endovasc Ther 14:150–157 Safi HJ, Winnerkvist A, Miller CC et al. (1998b) Effect of
Miller DC, Stinson EB, Oyer PE et al. (1979) Operative treat- extended cross-clamp time during thoracoabdominal
ment of aortic dissections: experience with 125 aortic aneurysm repair. Ann Thorac Surg 66:1204–1209
Chapter 27 · Aneurysm and Dissection of the Thoracic and Thoracoabdominal Aorta
911 27
Schäfers H-J, Zeuschner J, Frimpong-Boateng K, Laas J, aorta: comparison of techniques in 348 patients. Ann
Schaps D, Haverich A (1987) Aneurysmen der Aorta Thorac Surg 54:427–437
descendens – Determinanten des operativen Risikos. Svensson LG, Crawford ES, Hess KR, Coselli JS, Safi HJ (1993)
Z Herz Thorax Gefäßchir 1:177–184 Experience with 1509 patients undergoing thoracoab-
Schepens MA, Vermeulen FE, Morshuis WJ et al. (1999) dominal aortic operations. J Vasc Surg 17:357–368
Impact of left heart bypass on the results of thora- Svensson LG, Labib SB, Eisenhauer AC, Butterly JR (1999)
coabdominal aortic aneurysm repair. Ann Thorac Surg Intimal tear without hematoma. Circulation 99:1331–
67:1963–1967 1336
Schepens M, Dossche K, Morshuis W et al. (2004) Thevenet A (1980) L’arrêt circulatoire en hypothermie
Introduction of adjuncts and their influence on chang- profonde dans la chirurgie de I’aorte thoracique et
ing results in 402 consecutive thoracoabdominal aortic ses branches. Ann Chir: Chir Thorac Cardiovasc
aneurysm repairs. Eur J Cardiothorac Surg 25:701–707 34:573–575
Schulte HD, Birks W (1971) Zur chirurgischen Behandlung Tiwari KK, Murzi M, Bevilacaua S, Glauber M (2010) Which
des Aorta ascendens-Aneurysmas mit Aortenklap- cannulation (ascending aortic cannulation or periph-
peninsuffizienz beim Marfan-Syndrom. Thoraxchir Vask eral arterial cannulation) is better for acute type A aortic
Chir 19:365–372 dissection surgery? Interact Cardiovasc Thorac Surg
Schumacher H, Böckler D, Bardenheuer H, Hansmann J, 10:797–802
Allenberg JR (2003) Endovascular aortic arch recon- Tsang VT, Cameron DE, Raja SG (2011) How to avoid crimp-
struction with supra-aortic transposition for symptom- ing during valve sparing aortic root replacement
atic contained rupture and dissection: early experience using the Valsalva graft. Eur J Cardiothorac Surg
in 8 high-risk patients. J Endovasc Ther 10:1066–1074 40:266–267
Schumacher H, Von Tengg-Kobligk H, Ostovic M et al. Verdant A, Page A, Cossette R, Dontigny L, Page P, Baillot R
(2006) Hybrid aortic procedures for endoluminal arch (1988) Surgery of the descending thoracic aorta: spi-
replacement in thoracic aneurysms and type B dissec- nal cord protection with the Gott shunt. Ann Thorac
tions. J Cardiovasc Surg (Torino) 47:509–517 Surg 46:147–154
Shiono M, Hata M, Sezai A, Niino T, Yagi S, Negishi N (2006) Vricella LA, Williams JA, Ravekes WJ et al. (2005) Early expe-
Validity of a limited ascending and hemiarch replace- rience with valve-sparing aortic root replacement in
ment for acute type A aortic dissection. Ann Thorac children. Ann Thorac Surg 80:1622–1626
Surg 82:1665–16659 Walker T, Bail DH, Gruler M, Vonthein R, Steger V, Ziemer G
Shrestha M, Baraki H, Maeding I et al. (2012) Long-term (2007) Unsupported reduction ascending aortoplasty:
results after aortic valve – sparing operation (David I). fate of diameter and of Windkessel function. Ann
Eur J Cardiothorac Surg 41:56–61 Thorac Surg 83:1047–1053
Shrestha M, Kaufeld T, Beckmann E et al. (2016) Total aortic Wheat MW, Wilson JR, Bartley TD (1964) Successful replace-
arch replacement with a novel 4-branched frozen ele- ment of the entire ascending aorta and aortic valve.
phant trunk prosthesis: single-center results of the first JAMA 188:717–719
100 patients. J Thorac Cardiovasc Surg 152:148–159 Yacoub M, Fagan A, Stassano P et al. (1983) Result of valve
Sioris T, David TE, Ivanov J, Armstrong S, Feindel CM (2004) conserving operations for aortic regurgitation.
Clinical outcomes after separate and composite Circulation 68:311–321
replacement of the aortic valve and ascending aorta. Yamashita C, Okada M, Ataka K et al. (1998) Reoperation
J Thorac Cardiovasc Surg 128:260–265 after repair of type A and B dissecting aneurysm.
Spielvogel D, Mathur MN, Lansman SL, Griepp RB (2003) J Cardiovasc Surg (Torino) 39:721–727
Aortic arch reconstruction using a trifurcated graft. Yankah AC, Pasic M, Klose H, Siniawski H, Weng Y, Hetzer R
Ann Thorac Surg 75:1034–1036 (2005) Homograft reconstruction of the aortic root for
Strauch JT, Spielvogel D, Haldenwang PL et al. (2003) endocarditis with perianular abscess: a 17-year study.
Cerebral physiology and outcome after hypothermic Eur J Cardiothorac Surg 28:69–75
circulatory arrest followed by selective cerebral perfu- Yun KL, Miller DC, Fann JI et al. (1997) Composite valve
sion. Ann Thorac Surg 76:1972–1981 graft versus separate aortic valve and ascending aortic
Svensson LG, Crawford ES, Hess KR, Coselli JS, Safi HJ (1992) replacement: is there still a role for the separate proce-
Composite valve graft replacement of the proximal dure? Circulation 96(9 Suppl):II-368–II-375
913 28
Endovascular Surgical
Therapy of Thoracic
and Thoracoabdominal
Disease of the Aorta
Ali Khoynezhad, René H. Bombien, and Aamir Shah
References – 941
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
915 28
28.1 Introduction stays (Criado et al. 2005; Greenberg et al. 2005;
Song et al. 2006). TEVAR avoids cardiopulmonary
Open repair of aortic disease of the descending bypass with the need for aortic cross-clamping and
and thoracoabdominal aortic pathologies remains deep hypothermic circulatory arrest.
the gold standard for the treatment (Coselli et al.
2004; Dillavou and Makaroun 2008; Estrera et al.
2005; Khoynezhad et al. 2005; Kouchokos et al. 28.2 Devices Approved for TEVAR
1995; Stone et al. 2006; Svensson et al. 1993). This
approach can be associated with significant 28.2.1 Gore TAG Device
morbidity and mortality, with variable results
throughout different centers. The application of The thoracic aneurysm graft (TAG) endopros-
endovascular techniques utilizing stent graft tech- thesis (WL Gore and Associates, Flagstaff, AZ)
nology has evolved rapidly and changed the treat- received FDA approval in 2005 and was the first
ment paradigm of aortic diseases. Parodi and thoracic aortic device clinically approved for
coworkers first demonstrated the feasibility of implantation in the United States (Makaroun
endovascular exclusion of abdominal aortic aneu- et al. 2005) (. Fig. 28.1). This is a modular endo-
rysms in 1991 in a small clinical series that prosthesis composed of an expanded polytetra-
described the use of stent-anchored knitted fluoroethylene (ePTFE) tube with an external
Dacron grafts to treat abdominal aortic aneurysms flexible nitinol exoskeleton and has covered flares
(Parodi et al. 1991). The development and adop- with a sealing cuff present on both ends of the
tion of endovascular therapy for the treatment of device. The exoskeleton is bonded to the graft
descending thoracic aortic aneurysms soon fol- material without sutures and is constrained by a
lowed the promising use of this revolutionary PTFE sleeve. The device profile depends on the
technology. Dake and coauthors reported the first size of the graft and requires an 18–24-F sheath
series of endovascular treatment for descending for delivery. The device deploys from the middle
thoracic aortic aneurysms in 1994 (Mitchell et al. section outward by pulling on a deployment
1996; Dake et al. 1994). Semba and colleagues cord. The approved clinical indication is for
published the first reports of the endovascular descending thoracic aortic aneurysms with prox-
treatment of traumatic thoracic aortic injury in imal and distal landing zones of 20 mm. The new
1997 (Kato et al. 1997). White and coworkers per- conformable TAG (cTAG) was approved by the
formed the first thoracic endovascular aortic FDA in 2011 with an oversizing window ranging
repair (TEVAR) for chronic type B aortic dissec- from 6 to 33 %. This device conforms to the
tion in 1999 (Khoynezhad et al. 2009a).
In 2005, the US Food and Drug Administration
(FDA) approved the first commercially available
stent graft for thoracic endovascular aortic repair
(TEVAR) for the treatment of descending tho-
racic aortic aneurysms (Gore TAG, Gore and
Associates, Inc, Flagstaff, AZ) (Bavaria et al. 2007;
Hughes et al. 2012; Makaroun et al. 2005). Two
additional devices subsequently obtained FDA
approval in 2008: Talent (Medtronic, Santa Rosa,
CA) and TX2 (Cook, Inc, Bloomington, IN)
(Fairman et al. 2008; Matsumura et al. 2008).
Since then, the feasibility and successful utiliza-
tion of TEVAR have been established in selected
patients for the treatment of various aortic patholo-
gies including traumatic aortic injury, penetrating
aortic ulcers, and aortic dissections. Potential
advantages of TEVAR versus open surgery include
shorter operative time, less blood loss, decreased ..Fig. 28.1 The Gore TAG device (From W.L.Gore &
need for general anesthesia, and shorter hospital Associates; used with permission)
916 A. Khoynezhad et al.
..Fig. 28.2 Valiant
thoracic stent graft (From
Medtronic; used with
permission)
28
geometry of the aorta with better apposition the stents which allows for greater graft flexibility
along the inner curvature of the aortic arch. This and conformability and increased number of
device is available in diameters from 21 to proximal and distal uncovered stents (8-peak
45 mm, with the intended treatment diameter of crown compared to 5-peak configuration of
the noncritically diseased aorta ranging from 16 Talent) which distributes radial force across more
to 42 mm. Tapered device configurations are also points of contact and allows for better sealing
available. characteristics. The Captivia delivery system
allows for tip capture and for more controlled
deployment.
28.2.2 Medtronic Talent/Valiant
The Talent device (Medtronic, Inc, Santa Rosa, 28.2.3 Cook Zenith TX2 Device
CA) was approved by the FDA in 2008 and is a
modular device composed of a woven polyester The TX2 device was also approved in 2008
graft sutured to a nitinol lattice with a proximal (Cook, Inc, Bloomington, IN) and is composed
bare stent to improve fixation (Fairman et al. of woven Dacron fabric sutured to self-expand-
2008). It is available in diameters of 22–46 mm, ing stainless steel Z-stents (Greenberg et al. 2005;
and the delivery system ranges from 22 to 25 Fr. Matsumura et al. 2008). There are barbed cov-
Stent graft oversizing of 10–20 % relative to the ered top stents at the proximal end and an uncov-
native aortic neck diameter is recommended to ered barbed stent at the distal end of the device.
provide the necessary outward radial force to These barbed stents are intended to provide pos-
maintain apposition against the native aortic wall. itive fixation at both ends of the device. It is
The intended treatment diameter of the noncriti- available in diameters ranging from 28 to 42 mm
cally diseased aorta ranges from 18 to 42 mm, with sheath sizes of 20–22 Fr. Recommended
with 20 mm long proximal and distal landing oversizing ranges from 10 to 25 %, with 25 mm
zones required. In 2012 the Valiant (Medtronic, long proximal and distal landing zones of non-
Inc, Santa Rosa, CA) stent graft was approved by critically diseased aorta. Zenith TX2 with Pro-
the FDA, representing an improved overall design form was approved in 2011, designed to provide
(. Fig. 28.2). The Valiant stent graft differs from better apposition to the aortic wall in cases
the Talent device in several ways including elimi- of tortuous anatomy (Melissano et al. 2010)
nation of the longitudinal connecting bar between (. Fig. 28.3).
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
917 28
a b c
28
d e f
..Fig. 28.4 a Distribution of landing zones for TEVAR. b, c Placement of a device in zones 3 or 4 does not require any
revascularization. d Revascularization of covered LSA with a left carotid–subclavian bypass. e Revascularization of
covered left carotid and subclavian arteries with a carotid–carotid and carotid subclavian bypass. f Complete revascular
ization of all aortic arch vessels with aorto-innominate and left carotid bypass as well as carotid–subclavian bypass
(This figure was published in Cirugía Cardiovascular, Vol 17, Issue 1, Azizzadeh A, Valdés JA, Estrera AL, Charlton-
Ouw KM, Safi HJ. Reparación endovascular torácica aórtica (TEVAR): un enfoque sobre complicaciones, Pages 11–23,
Copyright © 2010 Sociedad Española de Cirugía Torácica-Cardiovascular. Published by Elsevier España S.L. All rights
reserved. Published with permission of the author and the publisher)
Deployment of the endograft into zones 0 and 1 28.3.3 eft Subclavian Artery
L
requires prior revascularization of the innomi- Coverage
nate and left common carotid arteries utilizing
debranching techniques. This usually does not The current management of preoperative left sub-
require shunting or extracorporeal circulation, clavian revascularization in cases with planned
unless there is a contralateral occlusion or high- device coverage of the subclavian artery is not uni-
grade stenosis of internal carotid artery. These form as most of the data regarding morbidity with
debranching procedures include aorto-innomi- this is derived from various single institutional
nate and aorto-carotid or carotid–carotid bypass. series. The Society of Vascular Surgery (SVS)
Deployment in zone 2 with coverage of the left recently formulated clinical practice guidelines
subclavian artery may require selective subcla- based on a systematic review and meta-analysis of
vian revascularization. the current literature in order to address this issue
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
919 28
(Matsamura and Rizvi 2010). The meta-analysis (Khoynezhad et al. 2007b). Perioperative hypoten-
revealed that coverage of the LSA without revascu- sion may also lead to an inadequate spinal cord
larization was associated with a much higher risk perfusion. SCI sequelae can range from monopare-
of arm and vertebral-basilar ischemia compared sis or paraparesis to paraplegia or quadriplegia and
with patients who underwent preoperative LSA can occur immediately or in a delayed fashion.
revascularization (Rizvi et al. 2009). There was also The EUROSTAR registry (Buth et al. 2007)
a trend toward an increased risk of paraplegia and enrolled 606 patients from 2000 to 2006 undergo-
anterior circulation stroke. A review of the ing TEVAR for aneurysm or dissection in the tho-
European Collaborators on Stent/Graft Techniques racic aorta. The incidence of paraplegia or
for Aortic Aneurysm Repair (EUROSTAR) regis- paraparesis was 2.5 %, and multivariate analysis
try revealed that LSA coverage without revascular- revealed LSA coverage without revascularization,
ization resulted in a significantly higher incidence renal failure, concomitant abdominal aortic sur-
of spinal cord ischemia or stroke than in patients gery, and the use of three or more stent grafts to
with prior LSA revascularization (8.4 % vs. 0 %; be associated with spinal cord injury. Coverage
p = 0.49) (Buth et al. 2007). and occlusion of the T10 level intercostal arteries
The SVS made the following GRADE 1 level C were also more frequent in patients with SCI than
recommendations (Matsamura and Rizvi 2010) in patients without neurologic symptoms.
for preoperative LSA revascularization: The mechanism of SCI after TEVAR has not
1. Presence of a patent left internal mammary to been well investigated. The lower 10 cm located
coronary artery bypass proximal to the celiac axis is felt to be a critical area
2. Termination of the left vertebral artery into of stent graft coverage. This segment of the aorta is
the posterior inferior cerebellar artery known to be the origin of the Adamkiewicz artery,
3. Absent, atretic, or occluded right vertebral artery which contributes significant collateral blood flow
4. Patent left arm arteriovenous shunt for dialysis to the spinal cord (Hyodoh et al. 2005). In our
5. Prior infrarenal aortic operation with previously experience with 184 TEVAR procedures performed
ligated lumbar and middle sacral arteries between 1998 and 2005, aneurysmal pathology, use
6. Planned extensive (>20 cm) coverage of the of an iliac conduit, and internal iliac artery cover-
descending thoracic aorta age were highly associated with postoperative SCI
7. Internal iliac artery occlusion after TEVAR (Khoynezhad et al. 2007a). It is likely
8. Presence of early aneurysmal disease where that immediate SCI post-TEVAR may be related to
further therapy involving the distal thoracic acute ischemia of the spinal cord secondary to cov-
aorta may become necessary erage of a large segment of intercostal vessels and/
or other collaterals to the spinal cord blood supply.
For these reasons, placement of preoperative
28.3.4 Cerebrospinal Fluid Drainage CSF drainage in patients undergoing TEVAR
should be strongly considered when:
The relationship between cerebrospinal fluid 1. Prior infrarenal aortic repair is present
(CSF) pressure and the development of paraplegia 2. Extensive coverage of thoracic aorta (>20 cm)
after thoracic aortic surgery was first suggested by is planned
Sugie and colleagues in 1957 (Sugie et al. 1957). 3. Coverage of the lower segments of thoracic
There have been various reports of the benefits of aorta (T8–L2 vertebrae levels) is planned
CSF drainage during open thoracic aortic repairs 4. Coverage of LSA without revascularization is
(Cina et al. 2004; Safi et al. 1994). planned
The incidence of paraplegia or spinal cord isch- 5. Use of iliac conduit or hypogastric artery
emia (SCI) in various large published series of coverage is planned
TEVAR performed for primarily aneurysmal dis-
ease has been reported between 0.6 % and 4.3 % Estrera and associates described a protocol for
(Etezadi et al. 2012; Khoynezhad et al. 2007a; Ricco CSF drain management based on their extensive
et al. 2006; Whateley et al. 2006). Possible mecha- TAAA surgical experience (Estrera et al. 2009). The
nisms for immediate SCI after TEVAR are coverage authors reported a complication rate of 1.5 % associ-
of critical intercostal or other collateral arteries that ated with CSF drain placement with no spinal hema-
provide segmental blood flow to the spinal cord tomas being observed. The CSF drain is clamped
920 A. Khoynezhad et al.
and removed on the first postoperative day if the passage of the delivery sheath or endograft, which
patient remains neurologically intact. In patients can range from 18 to 25 F diameter. With open sur-
who develop postoperative spinal cord ischemia, the gical access, either a vertical or oblique skin inci-
CSF is drained to maintain a pressure <5 mmHg, sion can be used. The advantage of a vertical
oxygen delivery is optimized by achieving a cardiac incision is that it allows the ability to gain additional
index of >2.5 L/min/m2 and blood transfusion to exposure of the common femoral artery if there is
maintain target hemoglobin of >12 gm/dl, and a significant atherosclerotic disease that may require
relatively high mean arterial pressure of >90 mmHg an endarterectomy or reconstruction. An oblique
and spinal cord perfusion pressure of >80 mmHg incision may minimize wound complications by
are maintained to optimize spinal cord blood flow. keeping the skin incision above the femoral crease.
If the femoral or external iliac arteries are of
insufficient caliber to allow passage of the device,
28.3.5 Neuromonitoring then a conduit to the common iliac artery may be
28 constructed (Abu-Ghaida et al. 2002). The iliac
The efficacy of neuromonitoring using both artery is exposed via a retroperitoneal exposure. A
somatosensory-evoked potentials (SSEPs) and prosthetic conduit (10 mm Dacron or PTFE will
motor-evoked potentials (MEPs) during thoracic accommodate the largest required sheath) is then
and thoracoabdominal aortic repair has been pre- used to perform an end-to-side anastomosis to the
viously demonstrated by Safi and colleagues common iliac artery. The conduit is then tunneled
(Achouh et al. 2007; Keyhani et al. 2009). This percutaneously under the inguinal ligament
is not a uniformly recognized intraoperative through a femoral counter-incision and the distal
adjunct. However, Estrera and colleagues demon- segment of the conduit is then readily accessed.
strated that an alteration in surgical conduct with The utilization of iliac conduits was reported to be
reimplantation of intercostal arteries in patients 15 % in the Gore TAG trial, 9.4 % in the Zenith TX2
undergoing open TAAA repair and alterations in trial, and 21 % in the Medtronic VALOR trial
anesthesia management to optimize spinal cord (Fairman et al. 2008; Makaroun et al. 2005;
perfusion in patients who lost neurologic signals Melissano et al. 2010). Alternative access sites, such
resulted in a return of neuromonitoring signals as the subclavian/axillary and the carotid artery,
(Estrera et al. 2010). This suggested a benefit of have been used along with the infrarenal aorta, the
intercostal artery reimplantation and alteration in ascending aorta, and the transapical approach.
anesthesia conduct in order to maximize perfu-
sion to the collateral network of the spinal cord.
Neuromonitoring in patients undergoing 28.3.7 uidewires, Catheters and
G
TEVAR may be a useful intraoperative adjunct as it Sheaths
may identify patients who are at risk of SCI follow-
ing stent graft deployment and in whom intraop- The physician performing TEVAR needs familiar-
erative maneuvers may be performed to optimize ity, experience, and expertise with a wide array of
spinal cord perfusion via the collateral network. endovascular tools. This includes devices not fre-
These maneuvers as described by Estrera include: quently managed in most endovascular procedures,
1. Increasing proximal aortic mean pressure to such as large-diameter occlusion balloons and
>80 mmHg endografts. However, perhaps the most important
2. Decreasing CSF pressure by gravity drainage consideration in TEVAR lies in the use of common
3. Increasing hemoglobin levels by transfusion endovascular equipment to facilitate a remarkable
4. Perform LSA revascularization if necessary to solution to a life-threatening abnormality.
improve collateral blood flow to the spinal cord
28.3.7.1 I nitial Access and Lesion
Crossing
28.3.6 Vascular Access In TEVAR, the suggested guidewire for initial arte-
rial access using the Seldinger technique is a nonhy-
The proper planning of vascular arterial access is drophilic-coated Bentson-type wire (Bentson
critical in avoiding potential complications. The Starter, Boston Scientific Corp., Natick, MA, USA,
femoral artery must be of sufficient caliber to allow or Bentson, Cook, Inc., Bloomington, IN, USA).
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
921 28
Traditionally, soft J-tipped wires (e.g., Safe T-J, the Meier wire is intended for TEVAR. However, a
Cook, Inc.) have been used for initial access because very stiff guidewire may be needed to further
of their association with the lowest risk of dissec- straighten vessel tortuosity and provide the track-
tion or other vessel injury (Andros et al. 2006; ing capability needed when delivering large
Ayerdi and Hodgson 2005). We find the Bentson devices such as thoracic aortic endograft systems
Starter wire especially useful in that its straight (Andros et al. 2006; Deeb and Williams 2004;
floppy tip forms an atraumatic «functional J-tip» Ohki and Malas 2005; Schneider 2006). For this
when advanced through a vessel, and yet the wire is we choose either an Amplatz Super-Stiff wire
able to cross tight stenoses because the «J» is not (Boston Scientific Corp.) or a Lunderquist Extra-
fixed. The primary access guidewire in TEVAR will Stiff wire (Cook, Inc.). The Lunderquist wire is the
also be used to cross the lesion in the thoracic aorta, stiffest guidewire in our supply and so the action is
in combination with a steerable selective catheter. first attempted with the Amplatz Super-Stiff wire.
The wire is later exchanged through this catheter for The most flexible and safest guidewire that can
a stiff wire, to be used for intravascular ultrasound accommodate the intended action should be used
(IVUS) interrogation and/or device tracking. first. The Lunderquist and Amplatz stiff wires fea-
The hydrophilic-coated Glidewire manufac- ture a short, straight tip; alternatively, a stiff guide-
tured by Terumo (Terumo Medical Corporation, wire with a preformed J-tip could be used, such as
Somerset, NJ, USA) features a nitinol mandrel a Rosen wire (Cook, Inc.) (Chuter 2006). This
with a polyurethane outer coating instead of a wire is often employed for exchange of angioplasty
metal coil. The polyurethane material contains catheters. . Table 28.2 lists various guidewires,
tungsten for radiopacity, and this surface is fur- both standard and exchange length, commonly
ther coated with a hydrophilic polymer (most used in TEVAR (Khoynezhad and Kruse 2009).
manufacturers now offer similarly constructed
wires). This wire is well known for its ability to 28.3.7.3 Catheters
track in tortuous areas and stenoses (Andros et al. Diagnostic catheters allow the practitioner to
2006; Ayerdi and Hodgson 2005) and is our assess areas of the vascular system in order to
choice for gaining access to the abdominal aorta if evaluate for a possible intervention, but they also
the Starter wire fails. However, care must be taken assist in performing the intervention. Often, this
when using this or any hydrophilic-coated wire, entails the delivery of radiographic contrast for
as there is a greater risk of dissection or perfora- opacification of the vessel lumen in radiographic
tion owing to the little resistance offered by these studies. This can involve visualization of a specific
wires when advanced through vessels. branch vessel using a selective catheter or visual-
ization of a significant portion of the arterial arbo-
28.3.7.2 Guidewires rization using a nonselective catheter. Angiography
Exchange-length guidewires function to provide in TEVAR is used to visualize the thoracic and
support and tracking ability to various catheters thoracoabdominal aorta, as well as all branch ves-
introduced over them, and their characteristics sels that may be affected by endograft placement.
often reflect the task they are enlisted to help . Table 28.3 presents some useful catheter prop-
accomplish (. Table 28.2). In TEVAR, a relatively erties determined by catheter material.
stiff guidewire is needed to accommodate the Nonselective catheters are usually constructed
IVUS catheter for inspection of the thoracic aorta. of nylon and designed to rapidly infuse large vol-
The guidewire also needs a soft tip so that when it umes of contrast agent without injuring the vessel.
is «anchored» by reflecting off the aortic valve, To accomplish this, the distal portion of these cath-
injury to the valve is avoided. For this purpose we eters contains multiple side holes in addition to the
prefer the Nitrex wire (ev3, Inc., Plymouth, MN, standard end hole. This distal portion is preformed
USA), Platinum Plus wire (Boston Scientific into a shape that assists in dispersion of the contrast,
Corp.), or Meier wire (Boston Scientific Corp.). that is, Omniflush, Grollman pigtail, straight, tennis
The Nitrex wire has become a favorite because it racket, and multipurpose (Ayerdi and Hodgson
routinely reflects off the valve without any diffi- 2005; Dauterman et al. 2005; White and Silva 2007).
culty. These wires provide enough support to track Nonselective catheters are generally used only in
most large catheters or devices; the Platinum Plus large-diameter vessels such as the aorta or its pri-
is often used for peripheral stent placement, and mary branches. Aortography in TEVAR cases is
28
922
Bentson Starter 0.035″ × 180 cm; stainless steel Boston Scientific Corp. (Natick, MA, USA) Preferred wire for initial access, TFE-coated
medium rigid wire with straight floppy tip
A. Khoynezhad et al.
Safe T-J 0.035″ × 180 cm; stainless steel Cook, Inc. (Bloomingdale, IM,USA) TFE coated with soft J-tip
Wholey 0.035″ × 145 cm; stainless steel with gold tip Mallinckrodt, Inc. (St. Louis, MO, USA) TFE-coated steerable wire with floppy tip
Glidewire 0.035″ × 180 cm; nitinol core wire, Terumo Medical Corp. (Somerset, NJ, USA) Hydrophilic coated, adept at traversing
polyurethane jacket with tungsten difficult anatomy, dissection risk
Magic torque 0.035″ × 180 cm; stainless steel Boston Scientific Corp. Hydrophilic-coated, calibrated wire fair
support for catheters
Nitrex 0.035″ × 260 cm; eV3 Inc. (Plymouth, MN, USA) Preferred wire for reflection off aortic valve and
nitinol core wire, Gold–tungsten coll aortic inspection with IVUS; silicone coated
Platinum plus 0.025″ × 260 cm; stainless steel with Boston Scientific Corp. TFE coated with shapeable platinum
platinum distal coil floppy tip
Meier 0.035″ × 300 cm; stainless steel with Boston Scientific Corp. TFE-coated, flexible tip
gold-plated tungsten distal
Amplatz Super Stiff 0.035″ × 260 cm; stainless steel Boston Scientific Corp. TFE-coated stiff wire with 6-cm flexible
straight tip
Lunderquist 0.035″ × 260 cm; stainless steel Cook, Inc. TFE-coated extremely stiff wire, 4-cm flexible
tip, useful in straightening tortuous anatomy
Rosen 0.035″ × 260 cm; stainless steel Cook, Inc. TFE-coated stiff wire with flexible J-tip
Amplatz Ultra Stiff 0.035″ × 260 cm; stainless steel Cook, Inc. TFE-coated stiff wire with 7-cm flexible tip
Glidewire 0.035″ × 450 cm; nitinol core wire, Terumo Medical Corp. Long wire for brachiofemoral access/«body
polyurethane jacket with tungsten floss» technique
(Greatest) 4 3 2 1 (Least)
Coefficient of friction
(Greatest) 4 3 2 1 (Least)
Torqueability (turning the ex vivo portion of the catheter results in rotation of the distal tip)
(Greatest) 4 3 2 1 (Least)
Selective
Imager II selective 5 F × 100 cm (4 F with brachial access); Pebax Boston Scientific Corp. Berenstein tip; preferred catheter for
composite with tungsten layering for aortic access in combination with
A. Khoynezhad et al.
Glidecath 5 F × 100 cm; nylon/polyurethane with braided Terumo Medical Corp. Angled tip; hydrophilic coated
stainless steel mesh
Slip-Cath 5 F × 100 cm; nylon with braided stainless steel Cook, Inc. Angled tip; hydrophilic coated
Nonselective
Royal Flush Plus 5 F × 90 cm (4 F with brachial access); nylon Cook, Inc. Pigtail; preferred catheter for
aortography
Accu-Vu Sizing Pigtail 5 F × 100 cm; nylon Anglodynamics, Inc. (Queensbury, NY, USA) Pigtail; 1-cm radiopaque markers; soft,
radiopaque tip
Imager II Flush 5 F × 100 cm; Pebax composite with tungsten Boston Scientific Corp. Pigtail
layering for radiopaque tip
IVUS
Visions PV 8.2 F 8.2 F × 90 cm Volcano Corp. (San Diego, CA. USA) 8–10 MHz IVUS catheter; preferred
catheter for thoracic aortic pathology
Pinnacle introducer 7–11 F × 10–25 cm (4-F Terumo Medical Corp. Straight sheath with
with brachial access) cross-cut hemostatic valve
Large and extra large, 10–24 F × 25–60 cm Cook, Inc. Tapered sheath with
Check-Flo hemostatic valve
Accurate measurements using advanced imaging application of IVUS is the confirmation of the
software that allow 3D reconstructions are of para- true vessel lumen in aortic dissection.
mount importance (Criado 2011; Ferreira et al. Adequate positioning of the endovascular graft
2008; Melissano et al. 2009; Setacci et al. 2012). has been cited as the major difficulty leading to
technical failure (Fattori et al. 2003; Grabenwöger
et al. 2000). The introduction of intravascular
28.4.2 Magnetic Resonance Imaging ultrasound has proved to be an important adjunct
(MRI) used during the endovascular procedure. It allows
a more accurate length measurement of the proxi-
MRI angiography is very helpful when CT angiog- mal and distal landing zones, better evaluation of
raphy is contraindicated as in contrast intolerance aortic side branch anatomy, and confirmation of
or renal insufficiency. The dynamic contrast- the position of the wire in the true lumen in aortic
enhanced MR angiography provides stable imag- dissections (Fernandez et al. 2008; Lee and White
ing within an acceptable speed. Alternatively 2004a). After release of the endograft, IVUS per-
«time-of-flight» noncontrast MRA can provide mits confirmation of adequate stent graft deploy-
adequate imaging of the aorta. When dealing with ment, complete apposition to the vessel, and
aortic dissection, a second MR scan for the late evaluation for endoleaks and gives a good assess-
enhancement may be helpful (Rousseau et al. ment of true and false lumen diameter and flow to
2009) (. Table 28.5). the branch vessels (Lee and White 2004a). IVUS
can significantly reduce the amount of contrast
material used and fluoroscopy exposure to both
28.4.3 Intravascular Ultrasound the patient and the team of healthcare providers.
For evaluation of the larger-diameter thoracic and
Intravascular ultrasound (IVUS) catheters consti- thoracoabdominal aorta, a low-frequency catheter
tute a valuable resource for the practitioner per- is needed (e.g., Visions PV 8.2 F; Volcano Corp.,
forming endoluminal procedures. The distal San Diego, CA, USA). Familiarity with the arti-
portion of the IVUS catheter incorporates a cylin- facts produced by guidewires and catheters in the
drical ultrasound transducer that generates real- arterial lumen is essential in using this technology
time cross-sectional images of vessels. This (. Tables 28.6 and 28.7).
technology has numerous applications in
TEVAR. IVUS is the most accurate modality in
measuring the luminal diameter of vessels and 28.4.4 Transesophageal
can also be used to identify the position of branch Echocardiography (TEE)
vessels, inspect vessel wall morphology, evaluate
for the presence of plaques or thrombi, and select The use of two-dimensional (2D) TEE has some
appropriate landing zones for endografts advantages in early diagnosis of thoracic aortic
(Khoynezhad et al. 2012a; Mauri and Kinlay 2007; pathologies. The TEE should be available in nearly
White 2004, 2006). A particularly important all emergency rooms; it is semi-invasive and can be
926
A. Khoynezhad et al.
IVUS
Visions PV 8.2 F 8.2 F × 90 cm Volcano Corp. (San Diego, CA, USA) 8–10 MHz IVUS catheter; preferred
catheter for thoracic aortic
pathology
28
are described along with their clinical indications
..Table 28.7 Commonly used abbreviations and limitations in today’s endovascular practice.
28.5.2 Vasopressin
28.5 Adjunctive Pharmacology
In endovascular procedures, vasopressin or other
In order to have a successful endovascular proce- vasoactive drugs (e.g., epinephrine, norepinephrine)
dure, a series of adjunctive medications is used are used to increase systemic blood pressure in
either during or after the procedure. In the follow- patients who are at high risk of spinal cord ischemia.
ing, the most frequently used pharmacologic agents Unlike catecholamines, vasopressin does not cause
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
927 28
tachycardia, which can have deleterious effects on for the treatment of hypertension in patients with
coronary perfusion and increase in myocardial con- angina, prior myocardial infarction, or heart fail-
tractility during stent graft deployment. The blood ure (Rosendorff et al. 2007). Their use in TEVAR
supply to the spinal cord arises from the intercostal is limited to temporary anti-impulsive/antihy-
arteries (most importantly from the T7 to L3 seg- pertensive therapy during deployment and bal-
mental arteries) and from branches of the subclavian looning of the stent graft and is therefore not
and internal iliac arteries. When a significant por- addressed by the American Heart Association
tion of this network of vessels is affected by the stent guidelines.
graft, the possibility of spinal cord injury resulting in Beta-blockers are competitive inhibitors of
paraplegia or paraparesis is increased (Khoynezhad endogenous catecholamines at beta-adrenergic
et al. 2005, 2007b; Khoynezhad and Donayre 2007a; receptor sites, causing negative chronotropic and
Maeda et al. 2012; Safi et al. 2008). An increased inotropic effects that are desired during certain
blood pressure following the use of vasopressin portions of TEVAR procedure. For the same rea-
increases the spinal cord perfusion pressure and sons, short-acting beta-blockers such as esmolol
therefore reduces the possibility of postprocedural are preferred during TEVAR. The onset of action
spinal cord ischemia (Eggebrecht et al. 2006a; after intravenous administration is extremely
Khoynezhad et al. 2007a). The mean arterial pres- rapid, with steady-state concentrations achieved
sure is kept between 85 and 95 mmHg using vaso- within 5 min of a loading dose being given.
pressin or other hemodynamic stimulants. Steady-state esmolol concentrations are propor-
tional to the infusion rate. Following discontinua-
tion, esmolol effects begin to decline in 1–2 min,
28.5.3 Adenosine with beta-antagonist activity completely reversed
within approximately 20 min. Another beneficial
Adenosine bolus is utilized during percutaneous side effect is the beta-1-selectivity. Cardioselective
intervention of the aorta to induce transient asys- beta-blockers such as esmolol have less influence
tole so as to reduce the impulse of the cardiac out- on bronchial and vascular beta-2 receptors and
put during stent graft deployment. The usual are safer for patients with chronic obstructive
intravenous dose for TEVAR is 36 mg adenosine lung disease (Andrus and Loyed 2008).
(followed by 18 mg for subsequent episodes), which
will produce a cardiac asystole for a period of 4–6 s
(Lippmann et al. 2007). This transient asystole 28.5.5 Aspirin
reduces the maximum rate of pressure develop-
ment in the left ventricle and the aorta and the force Aspirin is an integral part of treatment during and
applied to the stent graft at the time of the deploy- after endovascular interventions, including intra-
ment. The reduced «windsocking» effect allows for aortic and intracoronary stent implantation
a more precise deployment of the stent graft and (Rodondi et al. 2005). Aspirin can also reduce the
reduces the chance of malposition and inadvertent size of an embolic stroke by decreasing subse-
coverage of vital brachiocephalic arteries. The quent clot formation.
«windsocking» effect also applies to ballooning of Most patients undergoing TEVAR are already
the stent graft. This is performed to expand the on aspirin for the aforementioned reasons.
stent graft to appose the aortic wall, thereby stop- However, some patients with aortic dissection,
ping type I endoleaks. Adenosine cardiac arrest is aortic transection, or aortic aneurysm might not
used at this time as well. Alternatives to adenosine be receiving an antiplatelet agent. To reduce the
include rapid right ventricular pacing which is also chance of clot formation in the proximity of the
often used for transcatheter valve replacement. proximal and distal junctions of the stent graft
and the aorta, these patients should be placed on
low-dose aspirin initiated on the first postopera-
28.5.4 Beta-Adrenergic Receptor tive day for a period of 3–6 months after original
Blockers TEVAR. This will allow for pseudo-intimal cover-
age of the stent graft, including the critical seg-
The American Heart Association has published ment of the aorta that may be prone to clot
guidelines recommending the use of beta-blockers formation and distal embolization.
928 A. Khoynezhad et al.
However, sustained benefits on long-term sur- States requiring TAAA repair, a complete endo-
vival have not yet been proven (Cheng et al. 2010), vascular solution is not commercially available.
and randomized trials will be needed to address For these patients, hybrid aortic repair may be a
this question. reasonable option.
Hughes and colleagues reported a series of 47
patients from March 2005 to June 2011 undergo-
28.6.1 pen Debranching and
O ing extra-anatomic debranching of all visceral
TEVAR (Hybrid Aortic Repair) vessels followed by endovascular aneurysm exclu-
sion (Hughes et al. 2012). The debranching and
Generally, patients with «physiological con- endovascular portions of the procedure were per-
straints,» not being suitable candidates for open formed in a single operation in the initial 33
thoracoabdominal or aortic arch repair, are usu- patients and as a staged procedure during a single
ally evaluated for TEVAR (including fenestrated hospitalization in the most recent 14 patients. The
28 and branched TEVAR), while patients with 30 day rates of death, stroke, and permanent para-
«anatomic constraints,» not having proper prox- paresis/paraplegia were 8.5 %, 0 %, and 4.3 %,
imal and distal landing zones, are considered for respectively, but were 0 % in the 14 patients
open repair. There is a cohort of patients, who undergoing staged repair. Lin and colleagues also
are poor candidates due to physiologic and ana- reported a lower rate of renal insufficiency in
tomic constraints that may benefit from open patients undergoing a staged approach versus a
debranching followed by TEVAR exclusion of single operation (Lin et al. 2010).
the branched-aortic portion (. Fig. 28.5a, b). Our midterm follow-up of debranching pro-
Alternatively, for most patients in the United cedures in the TAAA and aortic arch has been
a b
..Fig. 28.5 Extra-anatomic bypass to celiac, superior mesenteric, and both renal arteries coming off the left common
iliac artery using a custom quadfurcated graft a. Completion angiogram reveals patency of all bypassed grafts and
exclusion of the aneurysm. Same custom graft can be used for total aortic arch hybrid repair b
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
931 28
published (Khoynezhad et al. 2012b). From 342 arch debranching procedures combined with
patients between 2005 and 2011, 195 required a TEVAR for arch aneurysms involving zones 0
combined open and endovascular approach. and 1 can be performed without the need for car-
From this group, 52 patients with multiple diopulmonary bypass in patients who are at sig-
comorbidities had hybrid repair using the custom nificant risk with open surgical repair. Antoniou
quadfurcated graft for TAAA (35 %) or arch and colleagues performed an analysis in 2010 of
(65 %) repair. Forty patients underwent simulta- all published series reporting on hybrid aortic
neous repair. There were 4 % perioperative stroke, arch repair with supra-aortic branch revascular-
with no paraplegia/paraparesis, no endoleak, and ization and thoracic stent graft deployment
no new-onset renal replacement therapy. Early (Antonioua et al. 2010). A total of 18 series with
mortality was 6 %, with 4-year survival rate at 195 patients were analyzed (only series with
79 %. All surgical grafts were patent; there was one greater than 5 patients were included in analysis).
endovascular reintervention and no aortic-related Complete arch repair was performed in 63 %;
death in average 34 months follow-up. In our perioperative mortality was 9 %. The incidence of
experience hybrid aortic repair is feasible, safe, perioperative stroke was 7 %, spinal cord isch-
and effective in this selected high-risk patient emia 0.5 %, and endoleak (type I or III) 9 %.
cohort. Furthermore, appropriate patient selec- Experience with branched and fenestrated
tion for hybrid debranching is critical for optimal devices has accumulated in a few specialized
outcomes, and with increased penetration of centers throughout the world during the past
commercially available branched and fenestrated decade. Rodd et al. (2011) studied 70 consecutive
stent grafts, the role of hybrid debranching would patients with thoracic abdominal aortic aneu-
be limited (Khoynezhad et al. 2012b). rysms. Only 60 % of the patients were identified
as suitable for complete endovascular repair. In
1999, Inoue (Inoue et al. 1999) implanted sur-
28.6.2 Branched and Fenestrated geon-made stent grafts (one to three side
Grafts for TEVAR branches) into 15 patients with thoracic aortic
aneurysm with a primary success rate of 60 %, a
Conventional open surgical techniques to repair median follow-up of 12.6 months, and two late
thoracoabdominal aortic aneurysms (TAAA) have deaths. Uchida (Uchida et al. 2010) reported on a
achieved excellent results in the modern era with case of an ascending aortic rupture that was
the addition of intraoperative adjuncts including treated with endovascular therapy using a cus-
distal aortic perfusion, cerebrospinal fluid drainage, tom-made fenestrated stent graft introduced via
and passive moderate hypothermia with the overall the femoral artery. These examples demonstrate
incidence of neurologic deficits decreasing to that experience with branched endovascular aor-
approximately 2 % (Estrera et al. 2005; Safi et al. tic repair and fenestrated endovascular aortic
2008). Due to the complexity of this procedure, repair is still limited (Bungay et al. 2011; Murphy
endovascular techniques have been adopted for the et al. 2012). Chuter and coauthors (2003) devel-
treatment of TAAAs in patients who are at high risk oped branched stent grafts with a simplified
for open surgical repair. If thoracic aneurysms modular approach to make branched and fenes-
require fixation or landing zones within the aortic trated TEVAR technology ready for a more wide-
arch or visceral segment of the aorta, endovascular spread use. Dijkstra, Greenberg, and colleagues
repair requires either a hybrid two-staged operation reported on advances in imaging technologies
with open surgical debranching followed by endo- with the ability to integrate a preoperative CT
vascular graft exclusion or a single-stage endovas- scan into the operative fluoroscopic image which
cular repair using either branched or fenestrated allowed for more accurate positioning of the
stent grafts. In the United States, these devices are stent graft in relation to critical branches without
currently available only as part of a physician- the need for angiography and reduced radiation
investigator-sponsored IDE (Investigational Device and contrast medium exposure in complex
Exemption) study. branched and fenestrated endovascular stent
Open surgical aortic arch replacement graft cases (Dijkstra et al. 2011).
requires cardiopulmonary bypass with deep Greenberg and colleagues reported a series of
hypothermic circulatory arrest. Hybrid aortic 406 patients with thoracoabdominal aneurysms
932 A. Khoynezhad et al.
and 227 patients with juxtarenal aneurysms who branch vessel malperfusion, and weakening of
underwent repair with branched endografts the aortic wall (Kodama et al. 2008; Svensson
(Greenberg et al. 2010). Reinforced fenestrated et al. 2008).
branches coupled with balloon-expandable stent Estrera and colleagues reported a series of 159
grafts, and sidearm branch designs were used in patients with acute type B aortic dissection in
the study. When patients undergoing e ndovascular which the mortality associated with uncompli-
repair (ER) were matched with those undergoing cated dissection (85/159 patients) was 1.2 % with
surgical repair (SR) for anatomic extent of dis- medical management (Estrera et al. 2007).
ease, mortality at 30 days (5.7 % ER vs. 8.3 % SR), Nevertheless, long-term mortality rates remain
mortality at 12 months (15.6 % ER vs. 15.9 % SR), high and successful management remains a clini-
and paraplegia risk (4.3 % ER vs. 7.5 % SR) were cal challenge. Data from the International Registry
similar between the two groups. Reinforced fenes- of Acute Aortic Dissection (IRAD) indicates that
trated branch patency when coupled with bal- the 3-year survival of patients treated medically
28 loon-expandable stent grafts was 97.8 % with a was only 77 % and that independent predictors of
mean follow-up of 15 months. mortality include female gender, history of prior
aortic aneurysm, history of atherosclerosis, inhos-
pital renal failure, and inhospital hypotension
28.7 TEVAR for Acute Thoracic (Tsai et al. 2006).
Aortic Dissection Persistent patency of the false lumen has been
implicated as a predictor of poor long-term out-
28.7.1 Uncomplicated Type B comes. The European multicenter study showed
Dissection that late outcome was better in patients with a
thrombosed false lumen than in those with a patent
Acute descending thoracic aortic dissection false lumen (Erbel et al. 1993). In an analysis of 62
(Stanford type B) is a dramatic and potentially patients with chronic type B dissections undergoing
catastrophic condition. The current treatment of regular follow-up CT scans, Sueyoshi et al. found
type B dissection remains medical (Estrera et al. that the presence of blood flow in the false lumen
2007), and the majority of patients with uncom- was the only significant risk factor for the increase in
plicated disease can be treated conservatively diameter. The patients with blood flow in the false
with anti-impulsive and antihypertensive lumen had significantly higher mean growth rate of
therapy (Khoynezhad and Plestis 2006).
the aorta (3.3 mm/year) than the patients without
Antihypertensive therapy should include blood flow (−1.4 mm/year) (Sueyoshi et al. 2004).
ß-blockers such as labetalol, esmolol, and meto- The Investigation of Stent Grafts in Aortic
prolol. This has two goals: reduction in blood Dissection (INSTEAD) trial was undertaken to
pressure and prevention of a reflex tachycardia. determine if endovascular treatment might
Reflex tachycardia may increase the maximum improve long-term outcome in patients with type
left ventricular contraction force (dP/dt) and B dissections (Nienaber et al. 2009). This was a
must be avoided in the acute phase of the disease randomized trial in which 140 patients in stable
(Khoynezhad et al. 2009b). If ß-blockers cannot clinical condition at least 2 weeks after the index
be used, calcium channel blockers may serve as dissection underwent either elective TEVAR in
equally efficacious alternative, without inducing addition to optimal medical therapy or optimal
reflex tachycardia. The target heart rate should medical therapy alone. The investigators found no
be less than 60 bpm and the target systolic blood difference in all-cause mortality or aortic-related
pressure less than 100 mmHg. An effective pain mortality between the two groups; the 2-year sur-
control regimen using morphine is recom- vival rate was 95.6 % with optimal medical ther-
mended to tranquilize the patient and reduce apy versus 88.9 % with TEVAR (p = 0.15).
stress-induced hypertension (Garbade et al. Although there was no difference in midterm sur-
2010; Gupta et al. 2009; Khoynezhad and Plestis vival, aortic remodeling (defined as true lumen
2006; Khoynezhad et al. 2010; Svensson et al. recovery and thoracic false lumen thrombosis)
2008). Hemodynamic stabilization in the acute occurred with 91.3 % of patients with TEVAR ver-
setting is important to decrease the risk of sec- sus 19.4 % of those with optimal medical therapy.
ondary adverse events such as further dissection, The question of whether thrombosis of the false
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
933 28
lumen in the thoracic aorta has any impact on type B dissection undergoing TEVAR in whom
survival needs further reporting of long-term fol- the 30-day mortality was 4 %, and the survival at
low-up on these patients. 1, 3, and 5 years was 82 %, 79 %, and 79 %, respec-
A randomized European trial comparing tively (Zeeshan et al. 2010). Twenty patients
TEVAR with the GORE TAG device and best undergoing open surgical repair with similar
medical therapy to best medical therapy alone characteristics had 30-day mortality of 40 % with
(ADSORB) in patients with uncomplicated acute 1-, 3-, and 5-year survival of 58 %, 52 %, and 44 %
type B dissections is currently ongoing (Brunkwall (p = 0.008).
et al. 2012). The primary outcomes include false The «PETTICOAT» (Provisional ExTension
lumen thrombosis, aortic rupture, and aortic dila- to Induce COmplete Attachment) procedure
tion. The results of this trial may help further employing a covered Zenith TX2 stent graft
define the role of TEVAR in the management of placed over the proximal entry tear with self-
uncomplicated acute type B dissection. expandable bare stents placed distally has been
performed in patients undergoing TEVAR for
complicated type B dissection (Lombardi et al.
28.7.2 Complicated Type B 2012; Melissano et al. 2012a). The STABLE (Study
Dissection of Thoracic Aortic Type B Dissection Using
Endoluminal Repair trial) investigators recently
Management of complicated type B dissection published their results of a prospective single-arm
remains challenging, as open surgical repair per- multicenter study with 40 patients using a com-
formed under emergent conditions even in experi- posite TEVAR (Lombardi et al. 2012). The study
enced centers of excellence is associated with reports 5 % inhospital mortality and a 90 % sur-
significant morbidity and mortality (Crawford et al. vival after 1 year. Morbidity within 30 days
1988; Estrera et al. 2007). Estrera and colleagues included stroke 7.5 %, paraplegia 2.5 %, retro-
reported a mortality of 17 % in their series of grade progression of dissection 5 %, and renal
patients with complicated type B dissection (Estrera failure 12.5 %. Favorable aortic remodeling was
et al. 2007). For this reason, TEVAR is gaining observed during follow-up, with complete throm-
increasing acceptance as an initial treatment for bosis of the thoracic false lumen noted in 31 %
patients with complicated type B dissection (Dake noted at 12 months.
et al. 1999; Hansen et al. 2004; Nienaber et al. 1999). TEVAR for complicated type B dissection is
The goal of this therapy is to exclude the primary associated with superior early and midterm out-
entry site, obliterate the false lumen, prevent aortic come compared to open surgical repair. A large
rupture, and relieve lower body malperfusion. trial with a multicenter registry is needed to sub-
In 2009, we reported midterm results of 28 stantiate these findings and establish long-term
patients undergoing endovascular interventions (>10 years) durability and safety of stent grafts in
for complicated acute type B aortic dissection complicated acute type B aortic dissection.
(Khoynezhad et al. 2009a). Indications for emer- An intramural hematoma (IMH) without inti-
gency TEVAR were rupture in 14 %, severe lower mal disruption occurs in approximately 5–20 % of
body malperfusion in 29 %, visceral/renal malp- the patients with acute aortic syndromes and is
erfusion in 25 %, persistent chest pain despite most often located in the descending aorta
proper anti-impulsive therapy in 18 %, uncontrol- (Evangelista et al. 2003; Ganaha et al. 2002;
lable hypertension in 4 %, and acute dilation of Nienaber and Eagle 2003; Shimizu et al. 2000;
false lumen with impending rupture in 11 % of Svensson et al. 2008). Progression to aortic dissec-
patients. Overall survival was 82 % at 1 year and tion occurs in 16–36 % of patients, and symptom-
78 % at 5 years follow-up (mean follow-up atic patients can be managed in similar fashion as
36 months with complete follow-up in 100 %). patients with acute type B dissections (Evangelista
The aorta-related mortality was 10 % for the entire et al. 2003; Maraj et al. 2000; Svensson et al. 2008).
follow-up period. Complete thrombosis of the TEVAR is indicated in IMH associated with pen-
false lumen was observed in 85 % of survivors, etrating aortic ulcer (PAU) or secondary to vasa
and partial thrombosis was observed in the vasorum hemorrhage in the media, as progres-
remainder. Zeeshan, Bavaria, and colleagues sion of disease with enlargement of the aneurysm
reported a series of 45 patients with complicated and ongoing dissection has to be expected
934 A. Khoynezhad et al.
(Grabenwoger et al. 2003). PAU may appear as an systematic review included 7,768 patients (mean
«ulcerlike» projection into the media of the aorta age 39 years TEVAR, 36 years OSR). The mortal-
and is often associated with extensive atheroscle- ity rate was significantly lower in patients under-
rosis, with or without IMH, as well as pseudoan- going TEVAR compared to OSR (9 % vs. 19 %,
eurysms (Chong et al. 2012; Ledbetter 2005; p < 0.01). There was no significant difference in
Svensson et al. 2008). The aortic pathology is stroke rate between the two groups, but the risk of
regional with PAU, and therefore, endovascular spinal cord ischemia (SCI) and end-stage renal
repair may be the ideal therapy (Czerny et al. disease (ESRD) was higher with OSR compared
2004; Demers et al. 2004; Neuhauser et al. 2004), with TEVAR (SCI 9 % open vs. 3 % TEVAR,
although significant atherosclerotic burden may p = 0.01; ESRD 8 % open vs. 5 % TEVAR, p = 0.01).
make suitable sheath access and passage of guide- The percentage of patients undergoing open sur-
wires into the aortic arch challenging (Svensson gical repair utilizing adjuncts including distal aor-
et al. 2008). tic perfusion is not stated. Open repair was
28 associated with an increased risk of graft infection
and systemic infections compared with TEVAR,
28.7.3 TEVAR in Blunt Aortic Injury but there was a trend toward increased risk of a
secondary procedure in TEVAR compared with
Blunt traumatic aortic injury is the second leading open repair (p = 0.07) with a median follow-up of
cause of death from blunt trauma after head injury, 2 years.
though it accounts for less than 1 % of trauma admis- A classification system for grading and treat-
sions (Clancy et al. 2001). Following traumatic aor- ment of traumatic aortic injury has been published
tic injury, approximately 85 % of the patients die by Azizzadeh et al. and adopted by the SVS for man-
before being transferred to the hospital, and 30 % of agement of these injuries (Azizzadeh et al. 2009)
those reaching the hospital alive die within the next (. Fig. 28.6). The grading classification is as follows:
6 h (Hoffer 2008; McGwin et al. 2002). grade I (intimal tear), grade II (intramural hema-
Endovascular repair is an effective treatment toma), grade III (pseudoaneurysm), and grade IV
option in patients who may not be able to undergo (rupture). Azizzadeh and associates recommend
open surgical repair safely and may decrease the expectant management with serial imaging for
risk of paraplegia. Early endovascular experience grade I injuries, with repair recommended for
included the use of commercially available aortic grades II–IV.
cuffs to treat these injuries since there were no Based on the findings of the commissioned
commercially available thoracic stent grafts suit- party, the SVS published clinical practice guide-
able for this application (Sam et al. 2003). With lines for endovascular repair of traumatic aortic
the availability of smaller diameter thoracic stent injury in 2011 (Lee et al. 2011a, b). All of the rec-
grafts which conform better to the aortic arch in ommendations were categorized as grade 2, level
younger patients who are frequently the victims C statements:
of traumatic aortic injury, TEVAR offers the 1. Endovascular repair to be performed
potential for a durable aortic repair while avoid- preferentially over open surgical repair or
ing the morbidity of a thoracotomy, aortic cross- nonoperative management, regardless of
clamping, and extracorporeal circulation. In 2012, patients age if anatomically suitable
the US FDA approved the first commercially 2. Regarding timing: urgent (<24 h) repair when
available stent graft (Gore TAG, Gore and feasible with respect to other potential
Associates, Inc., Flagstaff, AZ) for endovascular life-threatening injuries and at the latest prior
aortic repair for the treatment of traumatic aortic to hospital discharge
injury. 3. Expectant management with serial imaging
The Society of Vascular Surgery (SVS) com- for grade I injuries
missioned a third party (Knowledge and 4. Suggest selective revascularization of LSA
Encounter Research Unit, Mayo Clinic, Rochester, 5. Routine heparinization but at a lower dose
MN) to perform a systematic review and meta- than in elective TEVAR
analysis of the available literature to address this 6. Do not suggest routine spinal drainage
issue, and published clinical practice guidelines 7. Suggest general anesthesia
based on the findings (Lee et al. 2011a, b). The 8. Suggest open femoral exposure
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
935 28
..Fig. 28.6 Grading classification of traumatic aortic injury according to Azizzadeh and colleagues (Reprinted with
permission, Azizzadeh et al. 2009; Copyright Mr Chris Akers)
a b c d
28
..Fig. 28.7 a-d Type I endoleaks communicate with the aneurysm sac from around the proximal (Ia) or distal (Ib)
landing zone. a Type II endoleaks are not connected or associated to the landing zones or the junction between various
stent grafts. b Type III endoleaks occur between the junctions of two or more stent graft components. c Type IV
endoleaks are caused by graft wall porosity and mainly a problem of first-generation stent graft materials d
endoleaks required reintervention. In the TAG 2011). Using large-caliber systems (18-F to 25-F),
trial (14,78 Makaroun et al. 2005, 2008), the rates complications include arterial dissection, rup-
of observed endoleaks decreased from 8.1 % at ture, tear or thrombosis, hematoma, pseudoaneu-
1 month to 4.3 % at 5 years. Endoleaks were not rysm, arteriovenous fistula, retroperitoneal
consistently observed in some patients at all bleeding, embolism, and wound infection. This
times, and a total of 15 % had an endoleak detected underscores the importance of using iliac con-
at some time during the first 2 years of follow-up. duits whenever access vessels are of questionable
Most were of undetermined origin on CT scan. caliber and the need to perform these procedures
A review of the etiology of endoleaks follow- in an operating room where resources and equip-
ing TEVAR in 200 patients at the Cleveland ment are readily available to facilitate treatment
Clinic reported an incidence of 19.5 % (type I of any complications.
7 %, type II 8 %, type III 3.5 %) with mean follow-
up of 30 months (Morales et al. 2008). The type I
and III endoleaks were treated with either sec- 28.8.3 Neurologic Complications
ondary endovascular intervention or conserva-
tively. Of the type II endoleaks, glue embolization Stroke is among the most feared and devastating
was used to treat those due to coverage of LSA, complications of endovascular and open repairs of
and the rest were observed. Factors associated the thoracic aorta. Perioperative stroke has an inci-
with endoleaks were presence of a carotid-LSA dence of 3.5–5.5 % with a similar rate in both open
bypass and longer aortic coverage by the stent and endovascular interventions (Ehlert et al. 2011;
graft. The authors concluded that secondary Go et al. 2007; Maeda et al. 2012). Perioperative
intervention is required for most type I and III stroke after TEVAR may be related to patient age
endoleaks, and conservative treatment is ade- and underlying cerebrovascular disease, etiology
quate for most type II endoleaks. of the treated aortic pathology and proximal extent
of disease, coverage involving the aortic arch ves-
sels, systemic factors (hypotension, hypertension,
28.8.2 Vascular Access Injury anticoagulation), intracranial causes (hemorrhage,
edema, CSF drainage), or emboli (air, atheroma,
Vascular access injury is a major issue in TEVAR thrombus) (Matsamura and Rizvi 2010). Most
with potentially fatal sequelae. Incidence of injury strokes in the anterior circulation likely have an
to access vessels occurs in 1–15 % of patients embolic etiology, and posterior circulation strokes
(Dake et al. 2011; Lee et al. 2011a, b; Tonnessen are likely ischemic in origin. Over 60 % of patients
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
937 28
have a dominant left vertebral artery with absent or stroke were obesity (body mass index >30 kg/m2),
hypoplastic right vertebral artery, and coverage of peripheral vascular embolization/thrombosis,
the LSA in these circumstances can be associated and significant blood loss.
with significant risk (Matsamura and Rizvi 2010). Feezor et al. reported on the risk factors for
Embolization may be related to the use and perioperative stroke during TEVAR for various
advancement of wires, catheters, and devices into aortic pathologies (Feezor et al. 2007). The study
a diseased atheromatous aortic arch, with dis- included 196 patients treated with TEVAR with a
lodgement of atherosclerotic plaque to the brain. 4.6 % incidence of stroke. Proximal extent of
As a result, patients with a significant atheroscle- repair was significantly associated with a higher
rotic burden and those with aneurysms close to incidence of strokes (zones 0–2 vs. 3–4, p = 0.025).
the aortic arch are inherently at higher risk. In a Fifty-five percent of patients suffering a stroke
2007 review of 171 patients undergoing TEVAR, had intraoperative hypotension (SBP <80 mmHg).
Gutsche et al. reported a 5.8 % incidence of stroke Selective LSA revascularization based on preop-
which was associated with a 33 % inhospital mor- erative cerebrovascular imaging demonstrating a
tality rate (Gutsche et al. 2007). Severe atheroma- dominant left vertebral artery or incomplete circle
tous disease involving the aortic arch (>5 mm of Willis resulted in lower rates of stroke (6.4 % vs.
protrusion into aortic lumen) was strongly associ- 2.3 %, p = 0.30) and posterior circulation infarcts
ated with stroke (P = 0.0016). Combining a his- (5.5 % vs. 1.2 %, p = 0.13). The authors concluded
tory of prior stroke with extent A coverage that proximal extent of repair might serve as a
(proximal descending thoracic aorta) carried a surrogate marker for greater severity of degenera-
60 % stroke incidence, while extent C coverage tive disease in the aortic arch. Avoidance of hypo-
(entire descending thoracic aorta) resulted in a tension and preservation of antegrade vertebral
15 % incidence. There were no perioperative perfusion may be important in prevention of pos-
strokes in patients undergoing extent B coverage terior circulation strokes (Canaud et al. 2010;
(distal descending thoracic aorta). After review- Feezor et al. 2007). Furthermore, maintaining
ing these data, three risk factors for perioperative stable hemodynamics and therapeutic anticoagu-
stroke were identified: (1) a history of preopera- lation and performing the procedure expedi-
tive stroke, (2) CT grade IV atheroma (>5 mm) in tiously may further decrease the risk of stroke
the aortic arch or proximal descending aorta, and (Khoynezhad et al. 2009b; Maeda et al. 2012;
(3) extent A or C. Melissano et al. 2012b).
In a 2007 prospective multicenter report from
the EUROSTAR registry (European Collaborators
on Stent/Graft Techniques for Aortic Aneurysm 28.9 echnical Failures and Late
T
Repair) on 606 patients with endovascular repair Complications in TEVAR
of thoracic aorta pathologies (aneurysm, dissec-
tion, traumatic injury, anastomotic pseudoaneu- 28.9.1 Device Migration
rysm, and infectious/nonspecified), Buth et al.
found a 3.1 % incidence of stroke (Buth et al. Migration is defined as greater than 10 mm move-
2007). Using multivariate regression analysis, ment of the endograft or any movement of the
female sex and duration of procedure >160 min device leading to symptoms or requiring inter-
were associated with an increased risk for stroke. vention. Outside of clinical trials, there is little
This is likely related to lengthy manipulation of published data and it is likely to be underreported.
catheter, wires, and devices within the aortic arch. In the Gore TAG trial, there were three prox-
The use of transcranial Doppler ultrasound may imal migrations (4 %) and four component
be helpful in selecting appropriate catheters, migrations (6 %) at 2 years follow-up without
wires, and maneuvers to reduce significantly the any associated clinical events (Makaroun et al.
embolization (Khoynezhad et al. 2008b). 2005). In the Cook TX2 trial, proximal or distal
In our 2007 review of 184 TEVARs for DTAA, migration was noted in three stent grafts (2.8 %)
type B aortic dissection, traumatic aortic injury, within 12 months, with two cases of caudal
and penetrating aortic ulcer, we reported a stroke migration of the proximal graft and one case of
rate of 4.3 % (Khoynezhad et al. 2007b). Using cranial migration of the distal graft (Matsumura
univariate analysis, risk factors associated with et al. 2008). None were associated with clinical
938 A. Khoynezhad et al.
events or required a secondary intervention. All surgery (28 %, p = 0.868). Causes of RAAD
three patients had oversizing of the proximal included the stent graft in 60 %, manipulation of
neck diameter of less than 10 %, and all had guidewires and sheaths in 15 %, and progression
placement of the barbed stent in either an acutely of underlying aortic disease in 15 %. The majority
angled segment or in an area of thrombus. In the of cases were associated with proximal bare
Medtronic VALOR trial, four stent graft migra- spring stent grafts, with direct evidence of device-
tions (2.1 %) were noted within 12 months related injury noted at surgery or autopsy in one-
(Fairman et al. 2008). Two migrations involved half of the patients.
the proximal end of the graft moving distally, Dong et al. reported an incidence of RAAD of
and two involved the distal end of the graft mov- 2.5 % in a series of 443 patients undergoing
ing proximally. One patient required a second- TEVAR for type B dissection (chronic 75 %, acute
ary intervention related to device migration. 25 %) (Dong et al. 2009). Three of 11 patients had
Marfan syndrome. The perioperative mortality
28 was 27.3 %, and the site of new entry was identi-
28.9.2 Retrograde Dissection fied at the tip of the bare spring of the stent graft
in 81 %.
Retrograde type A dissection (RAAD) is a rare
and catastrophic complication of TEVAR. It is
defined as an intimal tear distal to the aortic arch 28.9.3 Graft Infection
which extends retrograde proximally into the
ascending aorta. The incidence has been reported The reported incidence of graft infection ranges
to be between 1.3 % and 6.8 % (Eggebrecht et al. from 1 to 5 % (Cernohorsky et al. 2011; Chiesa
2006b, 2009a; Fattori et al. 2005; Neuhauser et al. et al. 2010a; Heyer et al. 2009). Etiology includes
2005). RAAD has been attributed to several fac- perioperative contamination, hematogenous seed-
tors in these series: (1) trauma from the TEVAR ing, local bacterial translocation, and graft erosion
procedure (caused by wire manipulation, into the aerodigestive tract (Chiesa et al. 2010b).
sheaths/large-bore delivery systems, and stent The most commonly isolated organisms include
graft balloon dilation); (2) device properties Staphylococcus, Streptococcus, Propionibacterium
(semi-rigidity and proximal bare spring stent, species, Enterobacter cloacae, Escherichia coli, and
excessive oversizing >20 % causing increased Pseudomonas aeruginosa (Cernohorsky et al.
radial force); (3) aortic wall friability (acute and 2011; Heyer et al. 2009). The median time elapsed
chronic dissection); and (4) connective tissue from the TEVAR procedure to the diagnosis of
disorders (Marfan syndrome, Ehlers–Danlos infection ranges from 115 to 244 days
syndrome). (Cernohorsky et al. 2011; Heyer et al. 2009).
The European Registry on Endovascular Treatment requires surgical explantation of the
Aortic Repair Complications reports an inci- infected endograft, in situ replacement with either
dence of RAAD of 1.3 % (Eggebrecht et al. 2009b) homograft or antibiotic-impregnated Dacron graft
with a total of 63 cases being reported from 28 with possible coverage of the new graft with an
centers between 1995 and 2008. Of these, 81 % omental flap, and lifelong antibiotic therapy. The
underwent TEVAR for type B dissection (acute mortality from this complication ranges from 25
54 %, chronic 27 %), and 83 % had a bare proxi- to 60 % (Cernohorsky et al. 2011; Heyer et al.
mal spring stent graft placed. RAAD occurred 2009).
before the TEVAR procedure in 21 %, during the
procedure in 15 %, and during follow-up after
discharge in 65 %. Management consisted of 28.9.4 Device Malfunction
emergent surgical repair in 64 %, elective repair
in 13 %, and nonoperative treatment with anti- Several factors including larger hemodynamic
impulse therapy in 23 %. Overall mortality was forces, more tortuous anatomy, and larger devices
42 %, which included 19 % of patients who pre- delivered over a longer distance increase the com-
sented with sudden death. The mortality rates plexity of device delivery and deployment in the
were similar for conservative management thoracic aorta relative to the abdominal aorta. Lee
(33 %), elective surgery (20 %), and emergency described the failure modes of thoracic stent
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
939 28
grafts and prevention and management strategies 10 years, increased risk of AEF/ABF was found to
(Lee 2009). Intraoperative factors leading to mal- be associated with aortic pseudoaneurysm as an
deployment include inability to advance the deliv- indication for TEVAR and emergent and compli-
ery system, unintended device movement, and cated procedures (Chiesa et al. 2010b). Perioperative
inability to conform to local tortuous anatomy mortality of patients undergoing surgical treatment
(Lee et al. 2007a, 2009). A transbrachiofemoral was 64 %, and all patients treated nonoperatively
wire (body floss) may be used to overcome severe died within 30 days. The overall survival was 16 %
aortic tortuosity by providing two points of wire at a mean follow-up of 17.7 months.
fixation that can provide a stiff rail to facilitate
device delivery (Lee 2009). Device infolding is
related to excessive oversizing and/or lack of 28.10 Trends and Future Directions
apposition of the device to the inner curve of the in TEVAR
aortic arch, almost exclusively being associated
with Gore TAG stent graft (Bandorski et al. 2010). 28.10.1 Total Percutaneous TEVAR
The newer generation of Gore TAG has been
designed to have greater conformability to the The total percutaneous TEVAR without the need
lesser curve of the aortic arch in patients who of inguinal dissection is an important tool, mini-
have a tight radius. mizing postoperative complications, such as lym-
In the postoperative period, device failures can phocele or wound healing complications.
manifest as endograft collapse, component sepa- Percutaneous closure devices are designed to place
rations, and metallic fractures and fabric tears. sutures into the arterial wall prior to large sheath
Additional endografts and bare stents can be introduction. After the procedure and removal of
placed to increase the radial force of the stent graft the large-bore deployment devices, the previously
and prevent proximal collapse of the device (Rodd placed sutures can be approximated and the entry
et al. 2007; Steinbauer et al. 2006). A meta-analysis deficit of the arterial wall can be closed. The
(Jonker et al. 2010) of all published cases of endo- Perclose ProGlide (Abbott Vascular, Redwood
graft collapse in 2010 identified 60 reported cases City, California) device has a success rate ranging
with a median time interval from TEVAR to diag- from 88 to 100 %. Shorter overall procedural times
nosis of endograft collapse of 15 days (range 1 day and reduced complications, such as wound healing
to 79 months). On average, the collapsed endo- problems, have been achieved (Dosluoglu et al.
grafts were oversized by 26.7 ± 12.0 %. Excessive 2007; Lee et al. 2007b; Ni et al. 2011; Shafique et al.
oversizing was reported as the primary cause of 2009). However, the deployment of these devices
endograft collapse in 20 %, and a small radius of should be done with caution. Known contraindi-
curvature of the aortic arch was responsible for cations are obesity, especially morbid obesity,
48 % of the cases. The 30-day mortality was 8.3 %. severe atherosclerosis, multiple previous proce-
Treatment entails either a redo-TEVAR with a dures in the target area, and an anatomically high
high radial force device if feasible or a conversion femoral bifurcation (Criado et al. 2009). The ben-
to an open repair (Tadros et al. 2011). All devices efit of these devices with very low short- and mid-
are subject to material fatigue, which underscores term complications is apparent in selected patients
the need for lifelong surveillance in all patients (Bent et al. 2009), but in the event problems with
undergoing endovascular repair. percutaneous closure occur, a conversion to open
surgical access arterial repair is necessary to avoid
late complications such as pseudoaneurysms or
28.9.5 Aortobronchial or limb ischemia (Lee et al. 2011a, b).
Aortoesophageal Fistula
Erosion of the thoracic endograft into adjacent tis- 28.10.2 Low Profile TEVAR Devices
sue can manifest as aortobronchial (ABF) or aorto-
esophageal fistula (AEF). The incidence of ABF or Major limiting factors in TEVAR are compromised
AEF has been reported to be 1.7–2 % (Chiesa et al. vascular access and arterial tortuosity. Commonly
2010a; Eggebrecht et al. 2009a). In a retrospective used delivery sheath sizes are 20–25-F with
review of 1,113 TEVAR procedures performed over required vascular diameters from 7.3 to 9.2 mm.
940 A. Khoynezhad et al.
Low-profile devices with increased flexibility will ity of ascending aortic stent grafting in selected
expand TEVAR availability to patients with subop- patients.
timal vascular access, as well (Pavčnik et al. 2001; In another series of 11 patients with ascending
Strecker et al. 2004). Future generation of thoracic aortic intramural hematoma, floating thrombus
stent grafts will focus on lower profile to reduce after chronic type A aortic dissection, and pene-
vascular injury in this patient cohort. trating aortic ulcer, Kolvenbach et al. (2011)
reported their experience with endovascular
treatment of these pathologies. Patients included
were high risk according to the American Society
28.10.3 evices for Ascending
D of Anesthesiologists (ASA) classification
TEVAR ≥IV. Patients with acute type A aortic dissection
were excluded due to a missing ethical board
The various pathologies of the ascending aorta approval. The combined mortality and morbidity
28 such as dissections, aneurysms, or pseudoaneu- was 18 %. Bavaria et al. (2010) treated 27 patients
rysms are treated with open ascending aortic with distal arch aneurysms with a hybrid proce-
replacement (see 7 Chapter «Aneurysm and dure consisting of debranching followed by
Dissection of the Thoracic and Thoracoabdominal ascending aortic stenting. They report a success-
Aorta», Sect. 27.1.1). Currently TEVAR for patients ful stent deployment rate of 100 % and a 30-day
with ascending aortic pathology is not indicated mortality of 11 %. These data support hybrid
in patients with acceptable surgical risk (Lin et al. repair for ascending or arch aneurysms in selected
2007). The stent grafts that have been used for this high-risk patients.
purpose were commercially available descending In order to perform TEVAR for ascending
thoracic aorta stent grafts. Taking the aortic aortic pathology such as type A aortic dissection,
pathology into account, antegrade access may the available devices must be adapted accordingly
offer better success to enter the true lumen. with shorter distal tips and elimination of bare
Retrograde deployment is more difficult and springs. Problems and technical pitfalls such as
could put the entire supra-aortic trunk perfusion penetrating bare springs, risk of cerebral and
at risk or trigger a rupture of the false lumen. myocardial emboli, a very short landing zone, and
Only a few case reports and small series have the risk of aortic rupture have to be considered
been published. Rayan et al. described in 2004 the during planning of the procedure.
endovascular repair of a mycotic ascending aortic Development of TEVAR specifically designed
pseudoaneurysm in a 54-year-old man using 28.5- to address ascending aortic pathologies would be
mm × 3.3-cm Gore Excluder aortic cuff that was advantageous compared to the open surgery
deployed via the left subclavian artery by cutdown without the need for extracorporeal circulation,
(Rayan et al. 2004). Three years later, Lin et al. deep hypothermia, and circulatory arrest.
(2007) reported the same procedure in 78-year- Currently, all authors concur that ascending
old man suffering from an ascending aortic pseu- TEVAR should only be reserved for high-risk
doaneurysm after cannulation of the aorta for patients. But if the future brings a broad special
coronary artery bypass. Its dimension was 8 × 12 device portfolio for ascending aortic EVAR, the
cm, located in the midsegment of the ascending number of treated patients will grow.
aorta compressing the pulmonary arteries. Due to There are current attempts to use permeable
his comorbidities, the patient was not suitable for multilayer stent grafts that can cover aortic side
open surgery. Under general anesthesia, using branches, maintain perfusion of branch vessels,
fluoroscopy and TEE, a Zenith aortic cuff device and simultaneously decrease blood flow within
(32 × 36-cm) was delivered via the left common the aneurysm sac. These stents are called multi-
carotid artery with transient cardiac arrest using layer aortic repair stents (MARS) (Carrafiello
intravenous adenosine. No complications et al. 2011). Potential applications of this device
occurred, and the 1-month follow-up CT scan include dissections or aneurysms involving aortic
identified a successful procedure without segments with multiple side branches, such as the
endoleak. Similar case reports support the feasibil- arch or the abdominal aorta.
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
941 28
28.11 Conclusions cal repair of descending thoracic aortic aneurysms in
low-risk patients: a multicenter comparative trial.
J Thorac Cardiovasc Surg 133(2):369–377
TEVAR is a promising alternative approach to open Bavaria J, Milewski RK, Baker J, Moeller P, Szeto W et al.
surgery in properly selected patients for treatment (2010) Classic hybrid evolving approach to distal arch
of acute aortic disease with lower early mortality aneurysms: toward the zone zero solution. J Thorac
and lower complication rates including paraplegia, Cardiovasc Surg 140(6):S77–S80; discussion S86–91
Bent CL, Fotiadis N, Renfrew I, Walsh M, Brohi K et al. (2009)
especially in high-risk patients. The only FDA- Total percutaneous aortic repair: midterm outcomes.
approved indications at this time are DTAA and Cardiovasc Intervent Radiol 32(3):449–454
traumatic disruptions. Patient selection is impor- Brunkwall J, Lammer J, Verhoeven E, Taylor P (2012)
tant, and TEVAR should be offered to those patients ADSORB: a study on the efficacy of endovascular graft-
who will benefit the most from this endovascular ing in uncomplicated acute dissection of the descend-
ing aorta. Eur J Vasc Endovasc Surg 44(1):31–36
procedure. Long-term survival, durability, and cost-
Bungay PM, Burfitt N, Sritharan K, Muir L, Khan SL et al.
effectiveness remain to be determined. Appropriate (2011) Initial experience with a new fenestrated stent
recognition and management of TEVAR complica- graft. J Vasc Surg 54(6):1832–1838
tions can improve long-term outcomes. Buth J, Harris PL, Hobo R, van Eps R, Cuypers P et al. (2007)
Neurologic complications associated with endovas-
cular repair of thoracic aortic pathology: incidence
and risk factors. A study from the European
References Collaborators on Stent/Graft Techniques for Aortic
Aneurysm Repair (EUROSTAR) registry. J Vasc Surg
Abu-Ghaida AM, Clair DG, Greenberg RK, Srivasta S, O’Hara 46(6):1103–1111
PJ et al. (2002) Broadening the applicability of endo- Canaud L, Hireche K, Berthet JP, Branchereau P, Marty-Ané C
vascular aneurysm repair: the use of iliac conduits. et al. (2010) Endovascular repair of aortic arch lesions in
J Vasc Surg 36(1):111–117 high-risk patients or after previous aortic surgery: mid-
Achouh PE, Estrera AL, Miller CC III, Azizzadeh A, Irani A term results. J Thorac Cardiovasc Surg 140(1):52–58
et al. (2007) Role of somatosensory evoked potentials Carrafiello G, Rivolta N, Annoni M, Fontana F, Piffaretti G
in predicting outcome during thoracoabdominal aor- (2011) Endovascular repair of a celiac trunk aneurysm
tic repair. Ann Thorac Surg 84(3):782–788 with a new multilayer stent. J Vasc Surg 54(4):1148–1150
Andros G, Silva MB Jr, Haser PB, Coogan SM, Schneider PA Cernohorsky P, Reijnen MM, Tielliu IF, Sterkenburg SM, van
(2006) Arterial access; guidewires, catheters, and den Dungen JJ et al. (2011) The relevance of aortic
sheaths; and balloon angioplasty catheters. In: Moore endograft prosthetic infection. J Vasc Surg 54(2):
WS (ed) Vascular and endovascular surgery: a compre- 327–333
hensive review, 7th edn. Elsevier, Philadelphia, Chaikof EL, Blankensteijn JD, Harris PL, White GH, Zarins
pp 303–324 CK, Ad Hoc Committee for Standardized Reporting
Andrus MR, Loyed JV (2008) Use of beta-adrenoceptor Practices in Vascular Surgery of The Society for Vascular
antagonists in older patients with chronic obstructive Surgery/American Association for Vascular Surgery
pulmonary disease and cardiovascular co-morbidity: et al. (2002) Reporting standards for endovascular aor-
safety issues. Drugs Aging 25(2):131–144 tic aneurysm repair. J Vasc Surg 35(5):1048–1060
Antoniou GA, Sakka KE, Hamady M, Wolfe JHN (2010) Cheng D, Martin J, Shennib H, Dunning J, Muneretto C
Hybrid treatment of complex aortic arch disease with et al. (2010) Endovascular aortic repair versus open
supraaortic debranching and endovascular stent graft surgical repair for descending thoracic aortic disease;
repair. Eur J Endovasc Surg 39(6):683–690 a systematic review and meta-analysis of comparative
Ayerdi JA, Hodgson KJ (2005) Fundamental techniques in studies. J Am Coll Cardiol 55(10):986–1001
endovascular surgery. In: Rutherford RB (ed) Chiesa R, Melissano G, Marone EM, Marrocco-Trischitta
Rutherford vascular surgery, 6th edn. Elsevier, MM, Kahlberg A (2010a) Aorto-oesophageal and aor-
Philadelphia, pp 747–784 tobronchial fistulae following thoracic endovascular
Azizzadeh A, Keyhani K, Miller CC 3rd, Coogan SM, Safi HJ aortic repair: a national survey. Eur J Vasc Endovasc
et al. (2009) Blunt traumatic aortic injury: initial expe- Surg 39(3):273–279
rience with endovascular repair. J Vasc Surg 49(6): Chiesa R, Tshomba Y, Kahlberg A, Marone EM, Civilini E
1403–1408 et al. (2010b) Management of thoracic endograft
Azizzadeh A, Valdes JA, Estrera AL, Charlton-Ouw KM, Safi infection. J Cardiovasc Surg (Torino) 51(1):15–31
HJ (2010) Thoracic endovascular repair (TEVAR): a focus Chong F, Winter M, Puckridge P, Penhall A, Joseph M (2012)
on complications. Cir Cardiovasc 17(10):25–32 A rare case of penetrating atherosclerotic ulcer of the
Bandorski D, Brück M, Günther HU, Manke C (2010) aorta. Heart Lung Circ 21(2):105–107
Endograft collapse after endovascular treatment for Chuter T (2006) Endovascular treatment of thoracoabdomi-
thoracic aortic disease. Cardiovasc Intervent Radiol nal and pararenal aortic aneurysms. In: Zelenock GB,
33(3):492–497 Huber TS, Messina LM, Lumsden AB, Moneta GL (eds)
Bavaria JE, Appoo JJ, Makaroun MS, Verter J, Yu ZF et al. Mastery of vascular and endovascular surgery.
(2007) Endovascular stent grafting versus open surgi- Lippincott Williams & Wilkins, Philadelphia, pp 117–121
942 A. Khoynezhad et al.
Chuter TA, Buck DG, Schneider DB, Reilly LM, Messina LM rotic ulcers in the descending thoracic aorta: mid-term
(2003) Development of a branched stent-graft for results,”. Ann Thorac Surg 77(1):81–86
endovascular repair of aortic arch aneurysms. Dijkstra ML, Eagleton MJ, Greenberg RK, Mastracci T,
J Endovasc Ther 10(5):940–945 Hernandez A (2011) Intraoperative C-arm cone-beam
Cina CS, Abouzahr L, Arena GO, Lagana A, Devereaux PJ computed tomography in fenestrated/branched aor-
et al. (2004) Cerebrospinal fluid drainage to prevent tic endografting. J Vasc Surg 53(3):583–590
paraplegia during thoracic and thoracoabdominal Dillavou ED, Makaroun MS (2008) Predictors of morbidity
aortic aneurysm surgery: a systematic review and and mortality with endovascular and open thoracic
meta-analysis. J Vasc Surg 40(1):36–44 aneurysm repair. J Vasc Surg 48(5):1114–1120
Clancy TV, Gary MJ, Covington DL, Brinker CC, Blackman D Dong ZH, Fu WG, Wang YQ, Guo da Q, Xu X et al. (2009)
(2001) A statewide analysis of level I and II trauma centers Retrograde type A aortic dissection after endovascular
for patients with major injuries. J Trauma 51(2):346–351 stent graft placement for treatment of type B dissec-
Coselli JS, LeMaire SA, Conklin LD, Adams GJ (2004) Left tion. Circulation 119(5):735–741
heart bypass during descending thoracic aortic aneu- Dosluoglu HH, Cherr GS, Harris LM, Dryjski ML (2007) Total
rysm repair does not reduce the incidence of paraple- percutaneous endovascular repair of abdominal aortic
28 gia. Ann Thorac Surg 77(4):1298–1303
Crawford ES, Svensson LG, Coselli JS, Safi HJ, Hess KR (1988)
aneurysms using Perclose ProGlide closure devices.
J Endovasc Ther 14(2):184–188
Aortic dissection and dissecting aortic aneurysms. Eggebrecht H, Breuckmann F, Martini S, Baumgart D,
Ann Surg 208(3):254–273 Herold U et al. (2006a) Frequency and outcomes of
Criado FJ (2011) Debranching the aorta to facilitate TEVAR: acute renal failure following thoracic aortic stent-graft
evolving techniques and strategies. In: Ruckert RI, placement. Am J Cardiol 98(4):458–463
Hepp W, Luther B (eds) Chirurgie der abdominalen Eggebrecht H, Nienaber CA, Neuhäuser M, Baumgart D,
und thorakalen Aorta, Berliner Gefäßchirurgische Kische S et al. (2006b) Endovascular stent-graft place-
Reihe. Springer Berlin Heidelberg, Berlin, Heidelberg; ment in aortic dissection: a meta-analysis. Eur Heart
11(15): 121–133 J 27(4):489–498
Criado FJ, Abul-Khoudoud OR, Domer GS, McKendrick C, Eggebrecht H, Mehta RH, Dechene A, Tsagakis K, Kühl H
Zuzga M et al. (2005) Endovascular repair of the tho- et al. (2009a) Aortoesophageal fistula after thoracic
racic aorta: lessons learned. Ann Thorac Surg aortic stent-graft placement: a rare but catastrophic
80(3):857–863 complication of a novel emerging technique. JACC
Criado FJ, McKendrick C, Criado FR (2009) Technical solu- Cardiovasc Interv 2(6):570–576
tions for common problems in TEVAR. J Endovasc Ther Eggebrecht H, Thompson M, Rousseau H, Czerny M, Lonn L
16(Suppl 1):I63–I79 et al. (2009b) Retrograde ascending aortic dissection dur-
Czerny M, Cejna M, Hutschala D, Fleck T, Holzenbein T et al. ing or after thoracic aortic stent graft placement: insight
(2004) Stent-graft placement in atherosclerotic from the European registry on endovascular aortic repair
descending thoracic aortic aneurysms: midterm complications. Circulation 120(11 Suppl):S276–S281
results. J Endovasc Ther 11(1):26–32 Ehlert BA, Durham CA, Parker FM, Bogey WM, Powell CS
Dake MD, Miller DC, Semba CP, Mitchell RS, Walker PJ et al. (2011) Impact of operative indication and surgical
et al. (1994) Transluminal placement of endovascu- complexity on outcomes after thoracic endovascular
lar stent- grafts for the treatment of descending aortic repair at National Surgical Quality Improvement
thoracic aortic aneurysms. N Engl J Med 331(26): Program Centers. J Vasc Surg 54(6):1629–1636
1729–1734 Erbel R, Oelert H, Meyer J, Puth M, Mohr-Katoly S et al. (1993)
Dake MD, Kato N, Mitchell RS, Semba CP, Razavi MK et al. Effect of medical and surgical therapy on aortic dissec-
(1999) Endovascular stent-graft placement for the tion evaluated by transesophageal echocardiography.
treatment of acute aortic dissection. N Engl J Med Implications for prognosis and therapy. The European
340(20):1546–1552 Cooperative Study Group on Echocardiography.
Dake MD, White RA, Diethrich EB, Greenberg RK, Criado FJ, Circulation 87(5):1604–1615
Society for Vascular Surgery Outcomes Committee Estrera AL, Miller CC 3rd, Chen EP, Meada R, Torres RH et al.
et al. (2011) Report on endograft management of trau- (2005) Descending thoracic aortic aneurysm repair:
matic thoracic aortic transections at 30 days and 1 12-year experience using distal aortic perfusion and cere-
year from a multidisciplinary subcommittee of the brospinal fluid drainage. Ann Thorac Surg 80(4):1290–1296
Society for Vascular Surgery Outcomes Committee. Estrera AL, Miller CC, Goodrick J, Porat EE, Achouh PE et al.
J Vasc Surg 53(4):1091–1096 (2007) Update on outcomes of acute type B aortic dis-
Dauterman KW, Casserly IP, Bajzer CJ (2005) General angio- section. Ann Thorac Surg 83(2):S842–S845
graphic and interventional principals. In: Casserly IP, Estrera AL, Sheinbaum R, Miller CC, Azizzadeh A, Walkes JC
Sacher R, Yadav JS (eds) Manual of peripheral vascular et al. (2009) Cerebrospinal fluid drainage during tho-
intervention. Lippincott Williams & Wilkins, racic aortic repair: safety and current management.
Philadelphia, pp 36–55 Ann Thorac Surg 88(1):9–15
Deeb GM, Williams DM (2004) Thoracic aortic aneurysm Estrera AL, Sheinbaum R, Miller CC, Harrison R, Safi HJ
including dissection. In: Gardner TJ, Spray TL (eds) (2010) Neuromonitor-guided repair of thoracoabdom-
Operative cardiac surgery, 5th edn. Arnold, London, inal aortic aneurysms. J Thorac Cardiovasc Surg 140(6
pp 467–482 Suppl):S131–S135
Demers P, Miller DC, Mitchell RS, Kee ST, Chagonjian L et al. Etezadi V, Schiro B, Pena CS, Kovacs M, Benenati JF et al.
(2004) Stent-graft repair of penetrating atheroscle- (2012) Endovascular treatment of descending thoracic
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
943 28
aortic disease: single-center, 15-year experience. with the Zenith TX1 and TX2 thoracic grafts:
J Vasc Interv Radiol 23(4):468–475 intermediate-term results. J Vasc Surg 41(4):589–596
Evangelista A, Dominguez R, Sebastia C, Salas A, Greenberg R, Eagleton M, Mastracci T (2010) Branched
Permanyer-Miralda G et al. (2003) Long-term follow- endografts for thoracoabdominal aneurysms. J Thorac
up of aortic intramural hematoma: predictors of out- Cardiovasc Surg 140(6 Suppl):S171–S178
come. Circulation 108(5):583–589 Gupta PK, Gupta H, Khoynezhad A (2009) Hypertensive
Fairman RM, Criado F, Farber M, Kwolek C, Mehta M, VALOR emergency in aortic dissection and thoracic aortic
Investigators et al. (2008) Pivotal results of the aneurysm-a review of management. Pharmaceuticals
medtronic vascular talent thoracic stent graft system: 2(3):66–76
the VALOR trial. J Vasc Surg 48(3):546–554 Gutsche JT, Cheung AT, McGarvey ML, Moser WG, Szeto W
Fattori R, Napoli G, Lovato L, Grazia C, Piva T et al. (2003) et al. (2007) Risk factors for perioperative stroke after
Descending thoracic aortic diseases: stent-graft repair. thoracic endovascular aortic repair. Ann Thorac Surg
Radiology 229(1):176–183 84(4):1195–1200
Fattori R, Lovato L, Buttazzi K, Di Bartolomeo R, Gavelli G Hansen CJ, Bui H, Donayre CE, Aziz I, Kim B et al. (2004)
(2005) Extension of dissection in stent-graft treat- Complications of endovascular repair of high-risk and
ment of type B aortic dissection: lessons learned emergent descending thoracic aortic aneurysms and
from endovascular experience. J Endovasc Ther 12(3): dissections. J Vasc Surg 40(2):228–234
306–311 Heyer KS, Modi P, Morasch MD, Matsumura JS, Kibbe MR
Feezor RJ, Martin TD, Hess PJ, Klodell CT, Beaver TM et al. et al. (2009) Secondary infections of thoracic and
(2007) Risk factors for perioperative stroke during tho- abdominal aortic endografts. J Vasc Interv Radiol
racic endovascular aortic repairs (TEVAR). J Endovasc 20(2):173–179
Ther 14(4):568–573 Hoffer EK (2008) Endovascular intervention in thoracic
Fernandez J, Donovan S, Garrett H Jr, Burgar S (2008) arterial trauma. Injury 39(11):1257–1274
Endovascular thoracic aortic aneurysm repair: evaluat- Hughes GC, Lee SM, Daneshmand MA, Bhattacharya SD,
ing the utility of intravascular ultrasound measure- Williams JB et al. (2010) Endovascular repair of
ments. J Endovasc Ther 15(1):68–72 descending thoracic aneurysms: results with ‘on-label’
Ferrario F, Barone MT, Landoni G, Genderini A, application in the post Food and Drug Administration
Heidemperger M et al. (2009) Acetylcysteine and non- approval era. Ann Thorac Surg 90(1):83–89
ionic isosmolar contrast-induced nephropathy – a ran- Hughes GC, Barfield ME, Shah AA, Williams JB, Kuchibhatla
domized controlled study. Nephrol Dial Transplant M et al. (2012) Staged total abdominal debranching and
24(10):3103–3107 thoracic endovascular aortic repair for thoracoabdomi-
Ferreira M, Lanziotti L, Monteiro M (2008) Branched devices nal aneurysm. J Vasc Surg 56:621, Epub ahead of print
for thoracoabdominal aneurysm repair: early experi- Hyodoh H, Kawaharada N, Akiba H, Tamakawa M, Hyodoh K
ence. J Vasc Surg 48(6):30–36 et al. (2005) Usefulness of preoperative detection of
Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H et al. artery of Adamkiewicz with dynamic contrast-enhanced
(2002) Prognosis of aortic intramural hematoma with MR angiography. Radiology 236(3):1004–1009
and without penetrating atherosclerotic ulcer: a clinical Inoue K, Hosokawa H, Iwase T, Sato M (1999) Aortic arch
and radiological analysis. Circulation 106(3):328–348 reconstruction by transluminally placed endovascular
Garbade J, Jenniches M, Borger MA, Barten MJ, Scheinert D branched stent graft. Circulation 100(19 Suppl):II316–
et al. (2010) Outcome of patients suffering from acute II321
type B aortic dissection: a retrospective single-centre Jonker FH, Schlosser FJ, Geirsson A, Sumpio BE, Moll FL et al.
analysis of 135 consecutive patients. Eur J Cardiothorac (2010) Endograft collapse after thoracic endovascular
Surg 38(3):285–292 aortic repair. J Endovasc Ther 17(6):725–734
Go MR, Cho JS, Makaroun MS (2007) Mid-term results of a Kato N, Dake MD, Miller DC, Semba CP, Mitchell RS et al.
multicenter study of thoracic endovascular aneurysm (1997) Traumatic thoracic aortic aneurysm: treatment
repair versus open repair. Perspect Vasc Surg Endovasc with endovascular stent-grafts. Radiology 205(3):
Ther 19(2):124–130 657–662
Goodney PP, Travis L, Lucas FL, Fillinger MF, Goodman DC Keyhani K, Miller CC III, Estrera AL, Wegryn T, Sheinbaum R,
et al. (2011) Survival after open versus endovascular Safi HJ (2009) Analysis of motor and somatosensory
thoracic aortic aneurysm repair in an observational evoked potentials during thoracic and thoracoab-
study of the medicare population. Circulation dominal aortic aneurysm repair. J Vasc Surg 49(1):
124(24):2661–2669 36–41
Grabenwöger M, Hutschala D, Ehrlich MP, Cartes-Zumelzu Khoynezhad A, Kruse MJ (2009) Guidewires, catheters, and
F, Thurnher S et al. (2000) Thoracic aortic aneurysms: sheaths used for thoracic endografting procedures.
treatment with endovascular self-expandable stent J Card Surg 24(2):113–119
grafts. Ann Thorac Surg 69(2):441–445 Khoynezhad A, Plestis KA (2006) Managing emergency
Grabenwoger M, Fleck T, Czerny M, Hutschala D, Ehrlich M hypertension in aortic dissection and aortic aneurysm
et al. (2003) Endovascular stent graft placement in surgery. J Card Surg 21(1):S3–S7
patients with acute thoracic aortic syndromes. Eur Khoynezhad A, Bello R, Smego DR, Nwakanma L, Plestis KA
J Cardiothorac Surg 23(5):788–793 (2005) Improved outcome after repair of descending
Greenberg RK, O’Neill S, Walker E, Haddad F, Lyden SP et al. and thoracoabdominal aortic aneurysms using modern
(2005) Endovascular repair of thoracic aortic lesions adjuncts. Interact Cardiovasc Thorac Surg 4(6):574–576
944 A. Khoynezhad et al.
Khoynezhad A, Donayre CE, Bui H, Kopchok GE, Walot I, repair (‘Preclose’ technique). J Vasc Surg 45(6):
White RA (2007a) Risk factors of neurologic deficit 1095–1101
after thoracic aortic endografting. Ann Thorac Surg Lee WA, Martin TD, Hess PJ, Beaver TM, Huber TS (2007b)
83(2):S882–S889 Maldeployment of the TAG thoracic endograft. J Vasc
Khoynezhad A, Upadhyaya P, Kruse MJ (2007b) Spinal cord Surg 46(5):1032–1035
injury and stroke following thoracic endovascular aortic Lee WA, Daniels MJ, Beaver TM, Klodell CT, Raghinaru DE
repair: a risk analysis and review of the literature. Ital et al. (2011a) Late outcomes of a single-center experi-
J Vasc Endovasc Surg 14(3):221–229 ence of 400 consecutive thoracic endovascular aortic
Khoynezhad A, Donayre CE, Smith J, Kopchok GE, Walot I repairs. Circulation 123(5):2938–2945
et al. (2008a) Risk factors for early and late mortality Lee WA, Matsumura JS, Mitchell SM, Farber MA,
after thoracic endovascular aortic repair. J Thorac Greengerg RK et al. (2011b) Endovascular repair of
Cardiovasc Surg 135(5):1103–1109 traumatic thoracic aortic injury: clinical practice
Khoynezhad A, Kruse MJ, Donayre CE, White RA (2008b) guidelines of the Society for Vascular Surgery. J Vasc
Use of transcranial Doppler ultrasound in endovascu- Surg 53(1):187–192
lar repair of a type B aortic dissection. Ann Thorac Surg Levey AS, Coresh J, Balk E, Kausz AT, Levin A, National
28 86(1):289–291
Khoynezhad A, Donayre CE, Omari BO, Kopchok GE, Walot I
Kidney Foundation et al. (2003) National Kidney
Foundation practice guidelines for chronic kidney dis-
et al. (2009a) Midterm results of endovascular treat- ease: evaluation, classification, and stratification. Ann
ment of complicated acute type B aortic dissection. Intern Med 139(2):137–147
J Thorac Cardiovasc Surg 138(3):625–631 Lin PH, Kougias P, Huynh TT, Huh J, Coselli JS (2007)
Khoynezhad A, Gupta PK, Donayre CE, White RA (2009b) Endovascular repair of ascending aortic pseudoaneu-
Current status of endovascular management of com- rysm: technical considerations of a common carotid
plicated acute type B aortic dissection. Future Cardiol artery approach using the Zenith aortic cuff endo-
5(6):581–588 graft. J Endovasc Ther 14(6):794–798
Khoynezhad A, Celis R, Hashemzadeh M, Movahed MR, Lin PH, Kougias P, Bechara CF, Weakley SM, Bakaeen FG
Jalali Z (2010) Current status of thoracic endografting et al. (2010) Clinical outcome of staged versus com-
and its adjunctive pharmacology. Expert Opin bined treatment approach of hybrid repair of thora-
Pharmacother 10(17):2871–2881 coabdominal aortic aneurysm with visceral vessel
Khoynezhad A, Donayre CE, Kopchok G, Eugene J, White RA debranching and aortic endograft exclusion. Perspect
(2012a) Use of intravascular ultrasound in e ndovascular Vasc Surg Endovasc Ther 24(1):5–13
stenting of traumatic rupture of the descending tho- Lippmann M, Kakazu C, Fang TD, Bui H, Donayre C et al.
racic aorta. The CTSNet Clinical Cases Page. Available at: (2007) Adenosine’s usefulness in vascular surgery. Tex
7 http://www.ctsnet.org/sections/clinicalresources/ Heart Inst J 34(2):258–259
clinicalcases/article-12.html. Accessed 18 Jul 2012 Lombardi JV, Cambria RP, Nienaber CA, Chiesa R, Teebken
Khoynezhad A, Donayre CE, White RA (2012b) Mid-term O et al. (2012) Prospective multicenter clinical trial
results of hybrid endovascular aortic epair using a cus- (STABLE) on the endovascular treatment of compli-
tom quadfurcated graft. In: American Association for cated type B aortic dissection using a composite
thoracic surgery aortic symposium 2012. New York, device design. J Vasc Surg 55(3):629–640
26–27 MacDonald S, Cheung A, Sidhu R, Rheaume P, Grenon SM
Kodama K, Nishigami K, Sakamoto T, Sawamura T, Hirayama et al. (2009) Endovascular aortic aneurysm repair via
T et al. (2008) Tight heart rate control reduces second- the left ventricular apex of a beating heart. J Vasc Surg
ary adverse events in patients with type B acute aortic 49(3):759–762
dissection. Circulation 118(14):S167–S170 Maeda T, Yoshitani K, Sato S, Matsuda H, Inatomi Y, et al. (2012)
Kolvenbach RR, Karmeli R, Pinter LS, Zhu Y, Lin F et al. Spinal cord ischemia after endovascular aortic repair ver-
(2011) Endovascular management of ascending aortic sus open surgical repair for descending thoracic and tho-
pathology. J Vasc Surg 53(5):1431–1437 racoabdominal aortic aneurism. J Anesth 26(6):805–811
Kouchokos NT, Daily BB, Rokkas CK, Murphy SF, Bauer S Maeder M, Klein M, Fehr T, Rickli H (2004) Contrast
et al. (1995) Hypothermic bypass and circulatory arrest nephropathy: review focusing on prevention. J Am
for operations on the descending thoracic and thora- Coll Cardiol 44(9):1763–1771
coabdominal aorta. Ann Thorac Surg 60(1):67–76 Makaroun MS, Dillavou ED, Kee ST, Sicard G, Chaikof E et al.
Ledbetter S (2005) Helical (spiral) CT in the evaluation of (2005) Endovascular treatment of thoracic aortic aneu-
emergent thoracic aortic syndromes. Radiol Clin North rysms: results of the phase II multicenter trial of the GORE
Am 37(3):1–15 TAG thoracic endoprosthesis. J Vasc Surg 41(1):1–9
Lee WA (2009) Failure modes of thoracic endografts: pre- Makaroun MS, Dillavou ED, Wheatley GH, Cambria RP, TAG
vention and management. J Vasc Surg 49(3):792–799 Gore Investigators (2008) Five-year results of endovas-
Lee JT, White R (2004a) Basics of intravascular ultrasound: cular treatment with the Gore TAG device compared
an essential tool for the endovascular surgeon. Semin with open repair of thoracic aortic aneurysms. J Vasc
Vasc Surg 17(2):110–118 Surg 47(5):912–918
Lee JT, White R (2004b) Current status of thoracic aortic Maraj R, Rerkpattanapipat P, Jacobs LE, Makornwattana P,
endograft repair. Surg Clin North Am 84(5):1295–1318 Kotler MN (2000) Meta-analysis of 143 reported cases
Lee WA, Brown MP, Nelson PR, Huber TS (2007a) Total per- of aortic intramural hematoma. Am J Cardiol
cutaneous access for endovascular aortic aneurysm 68(6):664–668
Chapter 28 · Endovascular Surgical Therapy of Thoracic and Thoracoabdominal Disease
945 28
Matsumura JS, Cambria RP, Dake MD, Moore RD, Svensson dissection by stent-graft placement. N Engl J Med
LG et al. (2008) TX2 Clinical Trial Investigators. 340(20):1539–1545
International controlled clinical trial of thoracic endo- Nienaber CA, Rousseau H, Eggebrecht H, Kische S, Fattori R
vascular aneurysm repair with the Zenith TX2 endo- et al. (2009) Randomized comparison of strategies for
vascular graft: 1-year results. J Vasc Surg 47(2):247–257 type B aortic dissection; the investigation of STEnt
Matsamura JS, Rizvi AZ (2010) Left subclavian artery revas- grafts in aortic dissection (INSTEAD) trial. Circulation
cularization: Society for Vascular Surgery Practice 120(25):2519–2528
Guidelines. J Vasc Surg 52(4 Suppl):65S–70S Ohki T, Malas MB (2005) Endovascular treatment of
Mauri L, Kinlay S (2007) Adjunctive devices: atherectomy, abdominal and thoracic aortic aneurysms. In: Casserly
thrombectomy, embolic protection, IVUS, Doppler IP, Sacher R, Yadav JS (eds) Manual of peripheral vascu-
and pressure waves. In: King SB III, Yeung AC (eds) lar intervention. Lippincott Williams & Wilkins,
Interventional cardiology. McGraw-Hill Companies, Philadelphia, pp 183–213
New York, pp 93–106 Parodi JC, Palmaz JC, Barone HD (1991) Transfemoral intra-
McGwin G, Reiff DA, Moran SG, Rue LW (2002) Incidence luminal graft implantation for abdominal aortic aneu-
and characteristics of motor vehicle collision-related rysms. Ann Vasc Surg 5(6):491–499
blunt thoracic aortic injury according to age. J Trauma Pavčnik D, Uchida BT, Timmermans H, Petersen B, Loriaux
52(5):859–866 M et al. (2001) Bifurcated drum occluder endograft for
Melissano G, Bertoglio L, Civelli V, Amato ACM, Coppi G treatment of abdominal aortic aneurysm: an experi-
et al. (2009) Demonstration of the Adamkiewicz artery mental study in dogs. J Vasc Interv Radiol 12(3):
by multidetector computed tomography angiography 359–364
analysed with the open-source software OsiriX. Eur Pisimisis GT, Khoynezhad A, Bashir K, Kruse MJ, Donayre CE
J Vasc Endovasc Surg 37(4):395–400 et al. (2010) Incidence and risk factors of renal dys-
Melissano G, Civilini E, Bertoglio L, Logoldo D, Chiesa R function after thoracic endovascular aortic repair.
(2010) Initial clinical experience with the modified J Thorac Cardiovasc Surg 140(6):S161–S167
Zenith “Pro-Form” TX2 thoracic endograft. J Endovasc Rayan SS, Vega JD, Shanewise JS, Kong LS, Chaikof EL et al.
Ther 17(4):463–470 (2004) Repair of mycotic aortic pseudoaneurysm with
Melissano G, Bertoglio L, Rinaldi E, Civilini E, Tshomba Y a stent graft using transesophageal echocardiogra-
et al. (2012a) Volume changes in aortic true and false phy. J Vasc Surg 40(3):567–570
lumen after the ‘PETTICOAT’ procedure for type B aor- Ricco JB, Cau J, Marchand C, Marty M, Rodde-Dunet MH
tic dissection. J Vasc Surg 55(3):641–651 et al. (2006) Stent-graft repair for thoracic aortic dis-
Melissano G, Tshomba Y, Bertoglio L, Rinaldi E, Chiesa R ease: results of an independent nationwide study in
(2012b) Analysis of stroke after TEVAR involving the France from 1999 to 2001. J Thorac Cardiovasc Surg
aortic arch. Eur J Vasc Endovasc Surg 43(3):269–275 131(1):131–137
Mitchell RS, Dake MD, Semba CP, Fogarty TJ, Zarins CK et al. Rizvi AZ, Murad MH, Fairman RM, Erwin PJ, Montvori VM
(1996) Endovascular stent-graft repair of thoracic aor- (2009) The effect of left subclavian artery coverage
tic aneurysms. J Thorac Cardiovasc Surg 111(5): on morbidity and mortality in patients undergoing
1054–1062 endovascular thoracic aortic interventions: a system-
Morales JP, Greenberg RK, Lu Q, Cury M, Hernandez AV atic review and meta-analysis. J Vasc Surg 50(5):1155–
et al. (2008) Endoleaks following endovascular repair 1158
of thoracic aortic aneurysm: etiology and outcomes. Rodd CD, Desigan S, Hamady MS, Gibbs RG, Jenkins MP
J Endovasc Ther 15(6):631–638 (2007) Salvage options after stent collapse in the tho-
Murphy EH, Stanley GA, Ilves M, Knowles M, Dimaio JM racic aorta. J Vasc Surg 46(4):780–785
et al. (2012) Thoracic endovascular repair (TEVAR) in Rodd CD, Desigan S, Cheshire NJ, Jenkins MP, Hamady M
the management of aortic arch pathology. Ann Vasc (2011) The suitability of thoraco-abdominal aortic
Surg 26(1):55–66 aneurysms for branched or fenestrated stent grafts –
Neuhauser B, Perkmann R, Greiner A, Steingruber I, Tausche and the development of a new scoring method to aid
T et al. (2004) Mid-term results after endovascular repair case assessment. Eur J Vacs Endovasc Surg 41(2):
of the atherosclerotic descending thoracic aortic aneu- 175–185
rysm. Eur J Vasc Endovasc Surg 28(2):146–153 Rodondi N, Cornuz J, Bauer DC (2005) Aspirin for the pri-
Neuhauser B, Czermak BV, Fish J, Perkmann R, Jaschke W mary prevention of cardiovascular disease: a compre-
et al. (2005) Type A dissection following endovascular hensive review. Compr Ther 31(3):186–193
thoracic aortic stent graft repair. J Endovasc Ther Rosendorff C, Black HR, Cannon CP, Gersh BJ, Gore J,
12(1):74–81 American Heart Association Council for High Blood
Ni ZH, Luo JF, Huang WH, Liu Y, Xue L et al. (2011) Totally Pressure Research, American Heart Association
percutaneous thoracic endovascular aortic repair with Council on Clinical Cardiology, American Heart
the preclosing technique: a case-control study. Chin Association Council on Epidemiology and Prevention
Med J 124(6):851–855 et al. (2007) Treatment of hypertension in the preven-
Nienaber CA, Eagle KA (2003) Aortic dissection: new fron- tion and management of ischemic heart disease: a sci-
tiers in diagnosis and management: part I: from etiol- entific statement from the American Heart Association
ogy to diagnostic strategies. Circulation 108(5):628–635 Council for High Blood Pressure Research and the
Nienaber CA, Fattori R, Lund G, Dieckmann C, Wolf W et al. Councils on Clinical Cardiology and Epidemiology and
(1999) Nonsurgical reconstruction of thoracic aortic Prevention. Circulation 115(2):2761–2788
946 A. Khoynezhad et al.
Rousseau H, Chabbert V, Maracher MA, El Aassar O, Auriol Sugie S, Nakajima M, Ueno A et al. (1957) Studies on occlu-
J et al. (2009) The importance of imaging assessment sion and replacement of the thoracic aorta. Nippon
before endovascular repair of thoracic aorta. Eur J Vasc J Angiocardiology 20:120
Endovasc Surg 38(4):408–421 Svensson LG, Crawford ES, Hess KR, Coselli JS, Safi HJ (1993)
Safi HJ, Bartoli S, Hess KR, Shenaq SS, Viets JR et al. (1994) Variables predictive of outcome in 832 patients under-
Neurologic deficit in patients at high risk with thora- going repairs of the descending thoracic aorta. Chest
coabdominal aortic aneurysms: the role of cerebral 104(4):1248–1253
spinal fluid drainage and distal aortic perfusion. J Vasc Svensson LG, Kouchoukos NT, Miller DC, Bavaria JE, Coselli JS
Surg 20(3):434–444 et al. (2008) Expert consensus document on the treat-
Safi HJ, Estrera AL, Azizzadeh A, Coogan S, Miller CC (2008) ment of descending thoracic aortic disease using endo-
Progress and future challenges in thoracoabdominal vascular stent-grafts. Ann Thorac Surg 85(1):S1–S41
aortic aneurysm management. World J Surg 32(3): Tadros RO, Lipsitz EC, Chaer RA, Faries PL, Marin ML et al.
355–360 (2011) A multicenter experience of the management
Sam A 2nd, Kibbe M, Matsumura J, Eskandari MK (2003) of collapsed thoracic endografts. J Vasc Surg
Blunt traumatic aortic transection: endoluminal repair 53(5):1217–1222
28 with commercially available aortic cuffs. J Vasc Surg
38(5):1132–1135
Tonnessen BH (2011) Iatrogenic injury from vascular access
and endovascular procedures. Perspect Vasc Surg
Schneider DB (2006) Endovascular treatment of descend- Endovasc Ther 23(2):128–135
ing thoracic aortic aneurysms. In: Zelenock GB, Huber Tsai TT, Fattori R, Trimarchi S, Isselbacher E, Myrmel T et al.
TS, Messina LM, Lumsden AB, Moneta GL (eds) Mastery (2006) Long-term survival in patients presenting with
of vascular and endovascular surgery. Lippincott type B acute aortic dissection: insights from the
Williams & Wilkins, Philadelphia, pp 85–93 International Registry of Acute Aortic Dissection.
Setacci F, Sirignano P, Cappelli A, Setacci C (2012) The won- Circulation 114(21):2226–2231
ders of a newly available post-analysis CT software in Uchida K, Imoto K, Yanagi H, Machida D, Okiyama M et al.
the hands of vascular surgeons. Eur J Vasc Endovasc (2010) Endovascular repair of ascending aortic rup-
Surg 43(4):404–406 ture: effectiveness of a fenestrated stent-graft.
Shafique S, Murphy MP, Sawchuk AP, Cikrit D, Dalsing MC J Endovasc Ther 17(3):395–398
(2009) Femoral arterial access management for Vicenzi MN, Meislitzer T, Heitzinger B, Halaj M, Fleisher LA
endovascular aortic aneurysm repair: evolution and et al. (2006) Coronary artery stenting and non-cardiac
outcome. Perspect Vasc Surg Endovasc Ther surgery – a prospective outcome study. Br J Anaesth
21(4):29–33 96(6):686–693
Shimizu H, Yoshino H, Udagawa H, Watanuki A, Yano K et al. Wheatley GH 3rd, Gurbuz AT, Rodriguez-Lopez JA, Ramaiah
(2000) Prognosis of aortic intramural hemorrhage VG, Olsen D et al. (2006) Midterm outcome in 158 con-
compared with classic aortic dissection. Am J Cardiol secutive Gore TAG thoracic endoprostheses: single cen-
85(6):792–795, A10 ter experience. Ann Thorac Surg 81(5):1570–1577
Song TK, Donayre CE, Walot I, Kopchok GE, Litwinski RA White RA (2004) Intravascular ultrasound imaging. In:
et al. (2006) Endograft exclusion of acute and chronic Ascher E (ed) Haimovici’s vascular surgery, 5th edn.
descending thoracic aortic dissections. J Vasc Surg Blackwell Publishing, Malden, pp 50–60
43(2):247–258 White CJ, Silva JA (2007) Diagnostic procedures: peripheral angi-
Steinbauer MG, Stehr A, Pfister K, Herold T, Zorger N et al. ography. In: King SB III, Yeung AC (eds) Interventional cardi-
(2006) Endovascular repair of proximal endograft col- ology. McGraw-Hill Companies, New York, pp 277–289
lapse after treatment for thoracic aortic disease. J Vasc White GH, May J, Waugh RC, Chafour X, Yu W (1998) Type III
Surg 43(3):609–612 and type IV endoleak: toward a complete definition of
Stone DH, Brewster DC, Kwolek CJ, Lamuraglia GM, Conrad blood flow in the sac after endoluminal AAA repair.
MF et al. (2006) Stent-graft versus open-surgical repair J Endovasc Surg 5:305–309
of the thoracic aorta: mid-term results. J Vasc Surg White RA, Donayre CE, Walot I, Kopchok GE (2006)
44(6):1188–1197 Intraprocedural imaging: thoracic aortography tech-
Strecker EP, Haberstroh J, Boos I, Metz S, Langer M et al. (2004) niques, intravascular ultrasound, and special equip-
Development of a new intravascular low-profile device ment. J Vasc Surg 43(Suppl A):53A–61A
for exclusion of aortic aneurysm: an experimental pilot Zeeshan A, Woo EY, Bavaria JE, Fairman RM, Desai ND
study. Cardiovasc Intervent Radiol 27(3):243–250 et al. (2010) Thoracic endovascular aortic repair for
Sueyoshi E, Sakamoto I, Hayashi K, Yamaguchi T, Imada T acute complicated type B aortic dissection: superior-
(2004) Growth rate of aortic diameter in patients with ity relative to conventional open surgical and medi-
type B aortic dissection during the chronic phase. cal therapy. J Thorac Cardiovasc Surg 140(6 Suppl):
Circulation 110(Suppl II):256–261 S109–S115
947 29
References – 962
29
refractory period both decreased (Wijffels et al. the sinus impulse throughout both atria. Thus,
1995). This electrical remodeling led to an most of the atrial myocardium could still be
increased susceptibility of AF («AF begets AF»). activated, resulting in preservation of atrial
However, structural changes of the atrium con- transport function in most patients. Although
tribute to this remodeling process as well. Age, the possible underlying pathomechanism was
arterial hypertension, congestive heart failure, or not all known at that time, this principle
heart valve diseases are all strong predictors for accounted for all theories we consider to be pos-
the development of AF. The pathomechanism sibly responsible for initiating and sustaining AF
includes increasing atrial wall stress and some today: it isolated anatomical structures and
degree of atrial dilatation due to enhanced vol- obstacles and reduced the critical mass, thus iso-
ume or pressure load. All these conditions can be lating triggers and preventing or terminating
the cause of the development of fibrosis and myo- reentrant circuits and rotors. In general, the
cyte hypertrophy, both of which may result in atria are not capable to fibrillate anymore after a
slow conduction or even conduction block in Cox-Maze procedure (CMP).
some areas of the atrium. The final iteration of the Maze procedure,
termed the Cox-Maze III procedure (CMP III),
became the gold standard in the surgical treat-
29.3 The Development of Surgical ment of AF with excellent long-term freedom
Therapy of AF from symptomatic AF of 95 % with a median fol-
low-up of 5.9 years and a low postoperative risk of
29.3.1 The Maze Principle stroke (Weimar et al. 2012a). There is no differ-
ence in success rates between patients undergoing
Unlike other historical attempts to surgically a stand-alone CMP and those undergoing con-
address AF, such as the left atrial isolation proce- comitant procedures, and it is also equally
dure, His-bundle ablation, the corridor proce- efficacious in paroxysmal and persistent or long-
dure, or the atrial transection procedure, the standing persistent AF (Prasad et al. 2003a;
Maze procedure, introduced by Jim Cox in 1987, Weimar et al. 2012a, b).
was designed to block the multiple Despite its proven efficacy, the CMP III was
macro-reentrant circuits which were believed to technically challenging and invasive, and it was
be the sole cause of AF at that time while suc- not widely adopted in cardiac surgery. With the
cessfully restoring both atrioventricular syn- development of ablation technologies, the surgi-
chrony and sinus rhythm (Cox et al. 1991) cal incisions have been replaced with linear lines
(. Fig. 29.3). The operation consisted of creating of ablation using a variety of different energy
a myriad of surgical incisions in both the right sources like radiofrequency (RF), cryothermy,
and left atrium while allowing propagation of or high-intensity focused ultrasound (HIFU).
Chapter 29 · Surgical Therapy of Atrial Fibrillation
951 29
a RAA b
SAN LAA
PVs
RAA AVN
..Fig. 29.3 a Propagation of the sinus impulse throughout both atria in the original Cox-Maze procedure. b The
original cut-and-sew Cox-Maze procedure III (From Cox et al. 1991; used with permission)
These ablation-assisted procedures have greatly anatomy provide a challenge to any unidirectional
expanded the field of AF surgery in the past device attempting to achieve transmural lesions.
decade. With present ablation technology, sur- The chosen ablation technology and devices to
gery can be performed with low mortality and efficaciously replace surgical incisions must meet
morbidity as well as limited access incisions three important criteria:
while preserving the high success rates of the 1. It must reliably produce bidirectional
original «cut-and-sew» procedure. conduction block since this is the mechanism
by which incisions either block macro- and
micro-reentrant circuits or isolate trigger
29.3.2 Ablation Technologies foci. Even small gaps as narrow as 1.5 mm
within ablation lines can conduct fibrillatory
Different alternative energy sources were intro- impulses.
duced to simplify and shorten the procedure, 2. It must be safe. This requires a precise definition
reduce morbidity, and allow for a minimally inva- of dose-response curves to limit excessive or
sive or endoscopic approach. The heterogenic inadequate ablation. The surgeon must also
morphology of the atria has to be kept in mind have knowledge of the effects of the specific
when using any ablation device. While the mean ablation technology on surrounding vital car-
posterior left atrial wall ranges from 2.1 ± 0.9 mm diac structures, such as the coronary sinus, cor-
to 2.5 ± 1.3 mm in patients with a history of AF, onary arteries, and valvular structures. The risk
structures as the Bachmann’s bundle as a preferen- of collateral damage to surrounding extracardial
tial conduction pathway between the right and structures like the esophagus or the phrenic
left atrium crossing across the roof of the atria in nerve should be minimized.
the transverse sinus might be much thicker with 3. It must be simple to use and allow for a
4.6 ± 1.1 mm (range 1.7–9.3 mm). In general, minimally invasive or endoscopic approach.
patients with structural heart diseases reflect
more closely patients referred for surgical abla- This would require features such as rapid lesion
tion, who frequently present with a significant formation, simplicity of use, adequate length and
larger atrial wall thickness. Moreover, epicardial flexibility, and the ability to reliably create trans-
fat is a barrier to depth of penetration for most mural lesions epicardially on the beating heart.
ablation technologies. Even in normal individu- Currently, no device has perfectly met all of these
als, the fat layer at the posterior mitral anulus can criteria yet. It is imperative for surgeons to develop a
be 10 mm thick. In addition, there are free run- complete understanding of the effects of each surgi-
ning pectinate muscles in both the right and left cal ablation technology and carefully consider varia-
atrium that are not continuous with the epicardial tions in atrial anatomy and electrophysiology. This
surface. This highly variable wall thickness and will allow for more appropriate use of devices in the
952 T. Weimar and K.-N. Doll
operating room for each individual patient. While while argon can reach temperature of
most of the devices have shown to be efficacious in −185.7 °C. Cryoablation has a well-defined effi-
the arrested heart, few have shown the capability of cacy and safety profile and is generally safe
creating reliable transmural lesions on the beating except around the coronary arteries. The rela-
heart. This has to be considered in the era of the tively long time period of 1–3 min required to
development of endoscopic and hybrid approaches reliably create transmural lesions is one poten-
in an attempt to evoke less invasive surgical ablation tial disadvantage of cryoablation. There is also
techniques difficulty in creating lesions on the beating heart
Three different energy sources are currently due to the «heat sink» of the circulating blood.
available for the surgical endocardial or epicardial Furthermore, if blood is frozen during epicar-
ablation of AF. dial ablation on the beating heart, it coagulates,
creating a potential thromboembolic risk.
29.3.2.1 Cryo-energy However, it preserves fibrous tissue and collagen
Cryoablation has an excellent safety profile and and can therefore safely be used close to valvular
has been used for ablation of arrhythmia for tissue or the fibrous skeleton of the heart with a
29 more than three decades. As opposed to other low arrhythmogenic potential.
alternative energy sources, cryoablation creates
direct physical injury, cumulative sublethal cel- 29.3.2.2 Radiofrequency Energy
lular stress response, and molecular-based cell Radiofrequency (RF) energy has been used in the
death by freezing instead of heating. The forma- electrophysiology laboratory for many years and
tion of intracellular and extracellular ice crystals was one of the first energy sources to be applied in
disrupts the cell membrane and causes cell the operating room. RF energy uses alternating
death. A homogenous surface contact is essen- current in the range of 100–1,000 kilohertz (kHz).
tial for good ablation performance. Any fluid or This frequency is high enough to prevent rapid
vapor trapped between the probe and the tar- myocardial depolarization and the induction of
geted tissue freezes to form ice balls. Ice has a ventricular fibrillation, yet low enough to prevent
low thermal conductivity coefficient and tissue vaporization and perforation. The lesion
increases thermal resistance. Thus, ice ball for- size created by thermal coagulation injury
mation within the valleys of a nonuniform sur- depends on electrode-tissue contact area, the
face-probe contact acts as an insulator, retarding interface temperature, the current and voltage
heat loss. As heat is removed by various cryo- (power), as well as the duration of delivery. On
gens such as nitrous oxide, argon, or liquid oxy- histologic examination of radiofrequency lesions,
gen; extracellular fluid freezes at −20 °C, creating a focal coagulation necrosis predominates. This
a hyperosmotic environment that causes cell correlates with the irreversible nature of the
shrinkage and ultimately cell death. Rapid freez- injury, which occurs at temperatures >50 °C. There
ing to −40 °C induces expansion of intracellular is destruction of the myocardial collagen matrix
ice formation that disrupts organelles and cell and replacement with fibrin and collagen. At high
membranes even before osmotic imbalance temperatures greater than 100 ° C, char formation
occurs. A fast rate of cooling will increase cell predominates. Char presents as an impediment to
death, while slowly thawing the tissue is also heat transduction and has been associated with
effective in prolonging the mechanisms of cell asymmetrical ablations. The depth of the lesion
destruction (Mazur 1970). Currently, two com- can be limited by char formation, epicardial fat,
mercially available sources of cryothermal myocardial and endocavity blood flow, as well as
energy are being used in cardiac surgery. One is tissue thickness.
manufactured by AtriCure (Cincinnati, Ohio) RF energy can be delivered by either unipolar
and uses a disposable malleable linear probe or or bipolar electrodes.
reusable rigid probes with nitrous oxide as cool-
ing agent. More recently, Medtronic kUnipolar RF
(Minneapolis, Minnesota) has distributed a Numerous devices are currently on the market
malleable linear probe and a clamp device based with popular devices distributed by Estech (San
on argon. At 1 atmosphere of pressure, nitrous Ramon, California), nContact (Raleigh, NC),
oxide is capable of cooling tissue to −89.5 °C, and Medtronic (Minneapolis, Minnesota). These
Chapter 29 · Surgical Therapy of Atrial Fibrillation
953 29
include both dry and irrigated devices as well as nated most of the collateral damage that occurred
devices which incorporate suction to improve with the unipolar devices likely due to the focused
tissue surface contact. Resistive heating occurs delivery of energy within the jaws of the clamp.
only within a narrow rim of tissue in direct con- However, the devices with side-by-side bipolar
tact with the electrode, usually less than 1 mm. electrodes have not been extensively evaluated for
The deeper tissue heating occurs via passive con- safety yet and could have the same potential prob-
duction. To prevent char formation at the tissue- lems as unipolar devices.
electrode interface, irrigated catheters were
developed keeping temperatures cooler at the 29.3.2.3 High-Intensity Focused
tissue-probe interface. While in animals, unipo- Ultrasound
lar RF has been shown to create transmural High-intensity focused ultrasound (HIFU) is an
lesions on the arrested heart with sufficiently ablation energy marketed by St. Jude Medical (St.
long ablation times of 60–120 s, this has been Paul, MN) and effectively ablates tissue via
more difficult in clinical use. After 2-min endo- mechanical hyperthermia. It utilizes ultrasound
cardial ablations during mitral valve surgery, beams in the frequency range of 1–5 megahertz
only 20 % of the in vivo lesions were transmural. (MHz) or higher causing compression, refraction,
Epicardial ablation has been even more difficult. and particle movement and thus creating focused
Animal studies have consistently shown that uni- lesions by rapidly raising the temperature of the
polar RF is incapable of creating transmural targeted tissue to above 80 °C. This translates into
lesions epicardially on the beating heart. A recent kinetic energy and coagulative tissue necrosis.
clinical study confirmed this problem. Epicardial HIFU produces rapid, high-concentration energy
RF ablation resulted in only 7 % of lesions being in a focused area and is reportedly able to create
transmural despite electrode temperatures of up transmural epicardial lesions even through epi-
to 90 °C. One main reason for failure has been cardial fat in a short time.
felt to be the heat sink (Santiago et al. 2003). The HIFU is unique in that it is able to create non-
complications of unipolar RF devices have been contact focal ablation in three-dimensional vol-
described after extensive clinical use and include ume without affecting intervening and surrounding
coronary artery injuries, cerebrovascular acci- tissue. Its ability to focus the target of ablation at
dents, and the devastating creation of esophageal specific depths is an advantage over other energy
perforation leading to atrial-esophageal fistula modalities. Unlike all other energy sources that
(Doll et al. 2003). heat or cool tissue by thermal conduction, HIFU
ablates tissue by directly heating the tissue in the
kBipolar RF acoustic focal volume and is therefore much less
This technology is either applied between elec- affected by the «heat sink» of the circulating endo-
trodes embedded in the jaws of a clamp or cardial blood pool. The clinical studies utilizing
between electrodes arranged in parallel in linear HIFU, however, have had mixed results (Ninet
devices. By using a clamp, the electrodes are et al. 2005). There has been little independent
shielded from the circulating blood pool, which experimental verification of the efficacy of HIFU
allows for faster ablation times and limits collat- devices to reliably create transmural lesions, and
eral injury to surrounding structures. Bipolar RF long-term efficacy seems to be rather disappoint-
ablation clamps are the most reliable devices for ing. The fixed depth of penetration of these devices
creating transmural lesions on the beating heart may be a major problem in the wide range of thick-
both in animals and humans with short ablation ness of pathologically altered atrial tissue.
times (Gaynor et al. 2004; Prasad et al. 2002,
2003b). The linear devices developed for epicar-
dial ablation on the beating heart have not been as 29.4 Preoperative Modalities
reliable and seem to be more affected by the heat
sink affect and variable wall thickness. Three 29.4.1 Classification of Atrial
companies (AtriCure, West Chester, OH; Fibrillation
Medtronic, Minneapolis, MN; and Estech, San
Ramon, CA) currently market bipolar RF devices. As per recent guidelines, AF can be defined as fol-
Use of the bipolar RF clamp devices has elimi- lows (Calkins et al. 2012):
954 T. Weimar and K.-N. Doll
..Table 29.1 Indications for concomitant surgical ..Table 29.2 Indications for stand-alone surgical
ablation of atrial fibrillation (AF) (Calkins et al. 2012) ablation of atrial fibrillation (AF) (Calkins et al. 2012)
Paroxysmal: Surgical ablation is IIa C
Paroxysmal: Stand-alone IIb C
reasonable for patients surgical ablation may be
undergoing surgery for other considered for patients who
indications have not failed catheter
ablation but prefer a surgical
Persistent: Surgical ablation is IIa C approach
reasonable for patients
undergoing surgery for other
Paroxysmal: Stand-alone IIb C
indications surgical ablation may be
considered for patients who
Long-standing persistent: IIa C have failed one or more
Surgical ablation is reasonable attempts at catheter ablation
for patients undergoing
surgery for other indications
Persistent: Stand-alone surgical IIb C
ablation may be considered for
Symptomatic AF prior to initiation of antiarrhythmic patients who have not failed
drug therapy with a class 1 or 3 antiarrhythmic agent catheter ablation but prefer a
Paroxysmal: Surgical ablation is IIa C surgical approach
reasonable for patients
Persistent: Stand-alone surgical IIb C
undergoing surgery for other ablation may be considered for
indications patients who have failed one or
Persistent: Surgical ablation is IIa C more attempts at catheter
reasonable for patients ablation
undergoing surgery for other
Long-standing persistent: Stand- IIb C
indications alone surgical ablation may be
Long-standing persistent: IIb C considered for patients who
Surgical ablation may be have not failed catheter
considered for patients ablation but prefer a surgical
undergoing surgery for other approach
indications
Long-standing persistent: IIb C
Stand-alone surgical ablation
may be considered for patients
who have failed one or more
attempts at catheter ablation
usually not amendable to surgical ablation, and
atypical atrial flutter usually necessitates a full
biatrial Cox-Maze lesion set.
..Table 29.3 CHADS2 score
There are still conflicting data whether an
extended left-atrial lesion set is sufficient to treat Condition Points
the underlying pathomechanism of AF. Future
diagnostic tools to identify the patient subgroup C Congestive heart failure 1
benefiting from a biatrial ablation might further H Hypertension: blood pressure 1
improve results. Electrocardiographic imaging consistently above 140/90 mmHg
(ECGI) can be used to compute epicardial poten- (or treated hypertension on
tials noninvasively and reconstruct a three- medication)
dimensional anatomical map of atrial electrical A Age ≥75 years 1
activity using multiple surface electrodes and
D Diabetes mellitus 1
anatomic data obtained through computerized
tomography, thus potentially providing a map- S2 Prior stroke or TIA or 2
directed approach in the future. thromboembolism
956 T. Weimar and K.-N. Doll
a b
..Fig. 29.4 a Right atrial lesion set of the Cox-Maze procedure IV. b Left-atrial lesion set of the Cox-Maze procedure IV
(From Damiano et al. 2011; used with permission)
in a significantly higher drug-free freedom from antiarrhythmic medication of 84 % at 2 years with
AF at 6 and 12 months. Cryoablation is used to no intraoperative mortality and no postoperative
connect the lesion to the mitral anulus and com- strokes were reported (Weimar et al. 2011).
plete the left-trial isthmus line. It is important to Moreover, 84 % of patients were free from antico-
remember that the coronary sinus needs to be agulative therapy with warfarin. In CMP IV series
ablated epicardially in line with the endocardial including concomitant cardiac procedures, the
ablation in order to create the left-atrial isthmus freedom from AF was 89 %, and the freedom from
line. This is usually done with either the bipolar AF off antiarrhythmic drugs was 78 % at one year
RF clamp or a linear cryoprobe placed epicardi- (Damiano et al. 2011). There is no difference in
ally over the coronary sinus. The left atrial success rates for patients with paroxysmal com-
appendage is amputated, and a final ablation is pared to persistent or long-standing persistent AF
performed through the amputated left atrial with this procedure (Damiano et al. 2011; Weimar
appendage into one of the left pulmonary veins. et al. 2011). An enlarged left-atrial diameter, failure
In patients undergoing a right minithoracotomy, to isolate the entire posterior atrium by a complete
cryoablation is more extensively applied to com- box lesion set, and early atrial tachyarrhythmias
plete the posterior left-atrial isolation, and the left are predictors identified for late AF recurrence
atrial appendage is oversewn from the inside. (Damiano et al. 2011) (. Figs. 29.5 and 29.6).
29.6.1.1 Outcomes
A propensity analysis has shown that there was no 29.6.2 Left-Atrial Lesion Sets
significant difference in the freedom from AF at 3,
6, or 12 months between the cut-and-sew Cox- Over the past decade, numerous new procedures
Maze III and the ablation-assisted CMP IV (Lall have been introduced to surgically treat
et al. 2007). However, the CMP IV has significantly AF. Especially as a concomitant procedure to
shortened operative time and lowered complica- mitral valve surgery where the left atrium is opened
tion rates while maintaining the high success rate already, this lesion set has gained popularity. These
of the original Cox-Maze III procedure (Lall et al. procedures generally involved subsets of the left-
2007; Melby et al. 2006). A Kaplan-Meier estimate atrial lesion set of the Cox-Maze procedure. This
of freedom from symptomatic AF for the CMP concept is supported by the fact that the majority
(III + IV) was 85 % at 10 years (Weimar et al. of paroxysmal AF appears to originate around the
2012a). In 100 patients undergoing a stand-alone PVs and the posterior left atrium. A left-atrial
CMP IV for lone AF (31 % paroxysmal and 69 % lesion set typically involves pulmonary vein isola-
persistent or long-standing persistent AF), a free- tion with an isthmus lesion to the mitral anulus as
dom from AF of 90 % and freedom from AF off well as removal of the left atrial appendage.
958 T. Weimar and K.-N. Doll
Freedom from AF
0.6
0.4
K-M probability
0.2
Lower limit
Upper limit
0.0
0 1 2 3 4 5 6 7 8 9 10
Time (years)
..Fig. 29.6 Freedom
from atrial tachyarrhyth- 100
mias (ATAs) and freedom
from ATA off antiarrhyth- 90
mic drugs following a
stand-alone Cox-Maze IV
80
procedure (From Weimar
et al. 2011; used with
permission)
70
60
Percentage
50
40
30
20
10
0
3 months 6 months 12 months 24 months
29.6.4.1 Outcomes
Early results with an extended left-atrial lesion set
in addition to PVI have shown improved success
especially in patients with persistent AF com-
pared to PVI alone. Sirak and colleagues reported
a success rate of 87 % off antiarrhythmic drugs
after 6 months in 32 patients with persistent
..Fig. 29.7 Port positioning for thoracoscopic approach AF. In a larger series of almost 90 patients, the
(From Weimar et al. 2012b; used with permission) authors reported success rates of about 90 %
29 freedom from AF and 85 % freedom from AF off
drugs at 12 and 24 months follow-up indepen-
2008). With concomitant procedures, the success dently from the type of AF with no conversion to
rate of PVI is even worse. This is not surprising sternotomy (. Fig. 29.9) (Weimar et al. 2012b).
because those patients present with different Moreover, quality of life improved significantly
underlying mechanisms for AF, and a choice for postoperatively (. Fig. 29.10). A recently pub-
PVI as treatment option just based on the type of lished randomized trial comparing endoscopic
AF seems to be insufficient in most of those surgical ablation to catheter ablation revealed
patients. superior success rates of 66 % vs. 37 % at one year
in favor of surgery (Boersma et al. 2012). However,
it is important to point out that it is technically
29.6.4 Endoscopic Left-Atrial challenging and more time-consuming to per-
Lesion Set form these extended lesions thoracoscopically
and that proof of conductance block is mandatory
There is wide consent that the goal of develop- for the box lesion set as well. This necessity to
ing less invasive procedures should not be at the prove lesion integrity is due to the fact that there
cost of success rates. To address the shortcom- are still no devices on the market that reliably cre-
ings of PVI alone in many patients, new ablation ate linear transmural lesion on the beating heart
devices have been developed allowing for an except for the bipolar clamps. Also it is important
extension of the applied lesion set that can be to note that acute demonstration of conduction
performed thoracoscopically. Using a new gen- delay or block does not guarantee a chronic block
eration of bipolar and unipolar RF devices, it is or transmural lesion. Moreover the demonstra-
possible to perform a more extensive left-atrial tion of conduction delay can be difficult and may
box lesion set epicardially via port access. In lead to misinterpretation even by experienced
addition to the PVI technique described above, surgeons and electrophysiologists.
the transverse and oblique sinuses are dissected.
This allows for a box lesion set to be performed
isolating not just the PVs but the entire poste- 29.6.5 The Hybrid Approach
rior left atrium. It is crucial that attention is paid
to overlapping the lines of ablation from the The goal of the hybrid procedure is to combine
right and the left side to avoid gaps and to com- the benefits of epicardially performed surgical
plete a transmural roof lesion. An inferior line is ablation and endocardially performed catheter
created to connect the encircling ablation of the ablation in a dual approach. While thoracoscopic
inferior right PV with that of the inferior left surgery is effective in isolating the PVs, address-
PV. Because it is impossible to perform a left- ing the ligament of Marshall and managing the
atrial isthmus line epicardially, it is recom- left atrial appendage, it has its limitations in
Chapter 29 · Surgical Therapy of Atrial Fibrillation
961 29
effectively ablating atrial tissue, particularly the . Fig. 29.8). Ideally, this procedure should be per-
right and left-atrial isthmus and the coronary formed in a hybrid operating room. Because the
sinus. Those areas can be addressed by catheter underlying pathomechanism are more complex
ablation if intraoperative mapping performed by in patients with persistent AF, this team approach
the electrophysiologist shows gaps and conduc- may allow for a more patient-tailored lesion set
tion in one or more of this regions. Also the suc- that more effectively prevents AF recurrence. A
cess of creating transmural ablation lines can be recent study from Allessie and colleagues has
confirmed by instantaneous mapping and by formed what they call the «Double- Layer
demonstrating conduction block using pacing. Hypothesis», where narrow wavelets in the endo-
Possible gaps can then be closed by endocardial cardial and epicardial layers of the heart con-
catheter ablation in the same setting (see stantly feed each other in persistent AF (Eckstein
et al. 2011). Although this has to be confirmed by
further studies, it could add further support as to
why hybrid ablation might be a promising
approach for persistent AF.
29.6.5.1 Outcomes
Only a few centers have reported on hybrid pro-
cedures. In many institutions it has been challeng-
ing to set up a team approach involving both
electrophysiologists and surgeons. In a combined
series of the University of Virginia and University
of Maastricht, 12 patients with persistent AF
received hybrid ablation. At 12 months of follow-
up, 83 % of patients were free from atrial fibrilla-
tion and off antiarrhythmic drugs (Edgerton et al.
2008). A larger series of 63 patients reported by La
Meir’s group revealed significant better success
rates for a hybrid approach compared to sole
endoscopic ablation (100 % vs. 87.5 %, p = 0.04)
(La Meir et al. 2012). However, it is too early to
comment on the efficacy or safety of the hybrid
..Fig. 29.8 Endoscopically performed extended left- approach. Scientifically conducted trials are
atrial lesion set (From Weimar et al. 2012b; used with needed to evaluate any advantage over other abla-
permission) tion strategies.
..Fig. 29.9 Freedom
from recurrent AF after Freedom from atrial fibrillation
endoscopic left-atrial
100
ablation on the beating Free of AF
90
heart (From Weimar et al. Free of AF off drugs
80
2012b; used with
70
Percentage
permission) 60
50
40
30
20
10
0
6 months 12 months 24 months
Pts. at risk: 89 51 19
962 T. Weimar and K.-N. Doll
SF-36 score
60
40
20
0
PF RP BP GH VT SF RE MH
References – 992
Chapter 30 · Device Therapy of Rhythm Disorders
967 30
30.1 Introduction 30.1.2 Nomenclature and Modes
of Cardiac Pacing
In general, cardiac rhythm device implantation is
a standardized and safe procedure. The overall The majority of pacing devices are versatile and
number of complications decreased over the past capable of different pacing modes. In conduction
years. Nevertheless, serious and life-threatening disorders requiring temporary or permanent pac-
complications may still occur. The possible com- ing, the most appropriate pacing mode for each
plications and their management are elucidated. patient must be selected to accommodate his spe-
The last part of this chapter is dedicated to cific clinical needs. In order to standardize and
lead extractions, a part of CIED therapy reserved clearly arrange the terminology, an international
for advanced surgeons. The indications, tech- ICHD code (ICHD, Intersociety Commission for
niques, and available extracting tools are summa- Heart Disease Resources) was implemented and
rized for both percutaneous and lead extractions periodically updated.
via open-heart surgery. At present, a five-letter position code is valid
The development of modern cardiac implant- to describe pacemaker function based on the
able electronic devices is based on more than generic pacemaker code (NBG) of the North
50 years of clinical research and experience. American Society of Pacing and Electrophysiology
Nowadays, the treatment of heart rhythm disor- (NASPE) and British Pacing and Electrophysiology
ders with cardiac implantable electronic devices Group (BPEG). See . Table 30.1.
represents a well-recognized standardized ther- According to established pacemaker code,
apy with low-risk, low-morbidity, and reliable ICD functions are also defined by the North
results. American Society of Pacing and Electrophysiology
(NASPE) and British Pacing and Electrophysiology
Group (BPEG) in Generic Defibrillator (NBD)
30.1.1 ilestones of Rhythm Device
M code (. Table 30.2).
History The purpose of cardiac pacing is to treat patients
who have AV conduction block, bradyarrhythmias,
or tachyarrhythmias. Temporary pacing is utilized
1958 The first clinical implantation of a fully
implantable pacemaker at the Karolinska
when arrhythmia is transient or as a temporary
Institute in Solna, Sweden, performed by Åke measure until definite long-term therapy can be
Senning via thoracotomy (Elmqvist and initiated. Permanent pacing is absolutely indicated
Senning 1960). The first patient, Arne Larsson, when long-term pacing is necessary to avoid or to
received 26 pacemakers during his lifetime. prevent life-threatening arrhythmias, especially in
He died in 2001, at the age of 86.
1959 Temporary transvenous pacing with the
higher-grade AV conduction block. For all other
catheter electrode inserted via the patient’s indications, the clinical condition and symptom-
basilic vein (Furman and Robinson 1959). atology of the patient are the decisive factors.
1962 Permanent transvenous pacing in
conjunction with an implanted pacemaker
(Parsonnet et al. 1962; Lagergren and 30.2 Indications for Pacing
Johansson 1963).
1964 Principle of demand-function (Castellanos
et al. 1964). 30.2.1 Temporary Pacing
1969 Principe of bifocal sequential stimulation
(Berkovits et al. 1991). The first group of indications for temporary pac-
1971 Introduction of lithium-iodide cell ing includes pacing for a reversible condition.
(Greatbatch et al. 1971).
1980 Implantation of the first implantable
This may be a result of injury to the AV node or
cardioverter/defibrillator (ICD) in human the His-Purkinje system after heart surgery.
(Watkins et al. 1980). Empirically, it may improve over time, making
1981 Programming of pacemaker via telemetry permanent pacing unnecessary.
(Kirk 2001). Intraoperative or perioperative overdrive pac-
1999 Implantation of the first cardiac
resynchronization therapy (CRT) device
ing might be used to prevent atrial fibrillation and
(Sutton et al. 2007). See also 7 Chapter «The atrial flutter, although beta-blockers are usually the
History of Cardiac Surgery», 7 Sect. 1.11. treatment of choice (Blommaert et al. 2000).
968 H. Burger et al.
..Table 30.1 North American Society of Pacing and Electrophysiology (NASPE)/British Pacing and
Electrophysiology Group (BPEG) Generic Pacemaker Code (NBG)
First letter Second letter Third letter Fourth letter Fifth letter
30
..Table 30.2 North American Society of Pacing and Electrophysiology (NASPE)/British Pacing and
Electrophysiology Group (BPEG) Generic Defibrillator Code (NBD)
Similarly, rapid burst pacing can terminate AV causes for bradycardia (e.g., digoxin, beta-blocker,
nodal and AV reentry and sustained monomorphic antiarrhythmic drug therapy, etc.) may require
ventricular tachycardias. Some polymorphic ven- temporary pacing as well.
tricular tachyarrhythmias can be prevented by rapid Temporary bradycardia may occur in the set-
pacing as well. In case of polymorphic ventricular tings of acute myocardial infarction. Permanent
tachycardia associated with prolongation of QT pacing is mostly not required since bradycardia
interval, like torsades de pointes, rapid pacing can and conduction abnormalities usually will
prevent its initiation by shortening the QT interval resolve. There may, however, be a need for tem-
and by preventing ventricular premature beats. porary pacing like in case of asystole, unstable
Prophylactic pacing prior to surgery is rarely hemodynamics (hypotension, signs of hypoper-
indicated. However, temporary pacing might be a fusion, congestive heart failure), and/or com-
part of surgical or nonsurgical intervention like plete AV block with very slow ventricular rate.
rapid pacing in a case of transcatheter aortic valve Finally, temporary pacing is indicated in
implantation (TAVI) or catheter ablation proce- pacemaker-dependent patients when a lead revi-
dures in patients who undergo AV nodal modifi- sion or pacemaker generator change is necessary
cation to slow conduction in the AV node during or if the permanent pacemaker needs to be
rapid rates in atrial fibrillation. explanted because of underlying infection.
Inflammatory entities like bacterial endocar- If necessary, temporary pacing can be per-
ditis or Lyme carditis may also harm the electrical formed via endocardial leads, epicardial electrodes
conduction system of the heart. Toxic, metabolic, placed at the time of heart surgery, external trans-
electrolyte (hyperkalemia), and drug-induced thoracic patches, and an esophageal electrode.
Chapter 30 · Device Therapy of Rhythm Disorders
969 30
30.2.2 Permanent Pacing 30.3.1 Transvenous Leads
Permanent pacing is absolutely indicated in patients Since the introduction of transvenous leads into
with life-threatening arrhythmias. Relative indica- clinical practice in the early 1960s, lead technol-
tions include preservation, restoration, or improve- ogy and design have improved substantially.
ment of patients’ clinical performance. Guidelines Bipolar leads mostly replaced the original unipo-
for permanent pacing have been established by a lar leads. The current bipolar leads are preferred
task force of the American College of Cardiology, because of greater signal-to-noise ratio, less
the American Heart Association, and the Heart interference with external signals (e.g., skeletal
Rhythm Society (ACC/AHA/HRS) in cooperation myopotentials), better prevention of skeletal
with the American Association for Thoracic muscle stimulation seen at the pulse generator
Surgery (AATS) and the Society of Thoracic site, and less frequent cross talk between the
Surgeons (Epstein et al. 2008). Concordant guide- atrial and ventricle electrodes. Another improve-
lines have also been established by the European ment in lead design is related to the improvement
Society of Cardiology (ESC) (Vardas et al. 2007). of lead fixation mechanisms. The leads utilize
Indications for permanent pacemaker implan- either passive fixation via tines or fins entangle-
tation are as follows: ment in the trabeculae of the ventricle and atrium
55 Symptomatic sinus bradycardia (. Fig. 30.1a) or active fixation via a grasping
55 Sinus node blockade/arrest screw (. Fig. 30.1b). Although both lead types
55 AV block grade IIb/III and AV block IIa with reveal equally good performances, the screw-in
complementary symptoms leads are preferred because of more versatility in
55 Trifascicular block fixation site (not dependent on the presence of
55 Sick sinus syndrome trabeculae). Especially the current technique of
55 Carotid sinus syndrome septal positioning of the ventricular lead would
55 Bradyarrhythmia absoluta and be almost impossible without an active fixation
bradyarrhythmia under indicated process. Another advantage of screw-in leads is
antiarrhythmic medication their easier removal when needed.
Steroid-eluting leads represent one of the most
For other indications, the clinical condition important landmark developments in lead tech-
and presence of symptoms of the patient are the nology. Contemporary electrodes have a reservoir
determining factors. within the tip of the electrode that elutes steroids
in order to decrease local reactive tissue fibrosis at
the screw-in site.
30.3 Pacing Device Implantation
M. deltoideus A. thoracoacromialis
V. cephalica
M. pectoralis
major
N. pectoralis
V. cephalica
a b
1
2
3
1
4
2
5
3
4
5 6
6
1
2
..Fig. 30.5 Intraoperative
view on a right atrial placed
30 epicardial suture-on lead
(Medtronic CapSure Epi
4968)
should be left in the course of the lead in order to they became very popular not only because of
allow to follow heart movement without tension. easier and faster placement in a less-incriminat-
ing implantation procedure but also because of
reliable and satisfactory function. In addition,
30.3.3 Epicardial Leads further development of steroid-coated transve-
nous leads and ongoing miniaturization in the
In the early days of pacemaker therapy, epicar- late 1980s increased its reliability and popularity
dial leads were the only option to connect pace- (Sutton et al. 2007). Therefore, epicardial leads
maker with the myocardium. The first went out of fashion. Original epicardial leads
implantable pacemaker in 1958 stimulated the were uncoated unipolar myocardial screw-in
heart via an epicardial lead (Elmqvist and leads. They acquired a bad reputation because of
Senning 1960). Unfortunately, the initial epicar- frequent and steep increase of pacing threshold
dial leads which all had to be placed by means of and therefore need for frequent replacement.
open-chest cardiac surgery were quite unreli- Another breakthrough has been the introduc-
able, and they had to be frequently replaced. tion of suture-on epicardial leads containing ste-
Transvenous leads for endocardial pacing roids (Medtronic CapSure Epi 4965, 4968) into
became available during the late 1960s. Soon, the clinical daily routine (. Fig. 30.5).
Chapter 30 · Device Therapy of Rhythm Disorders
973 30
..Fig. 30.6 Modern epicardial 3. Epicardial LV lead implantation for
screw-in lead (St. Jude Medical permanent CRT pacing
1084 T). Screw-in lead tip
1. Alternative if transvenous LV lead
approach fails
2. Chronic phrenic nerve stimulation or
inacceptable pacing threshold of
transvenous LV lead if alternative vein
for transvenous lead positioning is
missing
4. Simultaneous epicardial pacing lead
implantation concomitant to open-heart surgery
1. In patients undergoing CABG or valve
surgery if additional CRT indication
exists
5. Tricuspid valve prosthesis
1. Mechanical tricuspid valve prosthesis
represents a contraindication for
The latest step has been the development of transvenous RV lead implantation
epicardial bipolar steroid-coated screw-in leads 2. Biological valve prostheses depict a
(e.g., St. Jude/1084 T) (. Fig. 30.6). Except for reli- relative contraindication for transvenous
ability and excellent electrical performance, the RV leads
reduction in size and development of introducer 6. Pediatric heart surgery
(implant tool) enabled their implantation via min- 1. Small vessel size for transvenous access
imally invasive lateral thoracotomy or even by and children’s growth make epicardial
means of an endoscopic approach. leads a useful solution.
Current epicardial pacing leads gained nowa- 2. For ICD treatment in small children, a
days again good standing due to their excellent defibrillation coil can be placed in the
electrical performance and durability as recently pericardium or sinus transversus.
reported (Burger et al. 2012).
30.3.3.1 Surgical Approach
Indications for epicardial pacing lead implan- Standard access for epicardial LV lead implanta-
tation include: tion is left-sided anterolateral or lateral thoracot-
1. Pacemaker/defibrillator infection, sepsis, or omy. Full-lateral approach allows better view and
valve endocarditis in pacemaker-dependent more comfortable manipulations, but the patient
patients needs to be positioned on table in 60–80° right-
1. Complete removal of the cardiac sided position. Therefore, many surgeons prefer
implantable electronic device (CIED) the anterolateral approach.
system in case of pacemaker lead infection The implantation is performed under general
in pacemaker-dependent patients anesthesia. Double-lumen intubation might be
2. Systemic infection or valve endocarditis helpful for easier implantation but is not mandatory.
requiring open-heart surgery and Incision is preferentially performed in the fourth or
concomitant pacemaker indication fifth intercostal space, the subcutaneous tissue is
2. Impossible venous access divided, and the intercostal muscles are separated
1. Due to the rising number of patients close to the upper side of the rib. It might be useful
requiring cardiac electronic devices or to infiltrate local anesthetics into the intercostal
system upgrades, venous occlusion space and periostium to prevent severe postopera-
appears increasingly. Epicardial lead tive pain. After retractor placement, the pericardium
implantation represents an option for will be directly visible (. Fig. 30.7a). If present, peri-
upgrades or new implantations if cardial fatty tissue can be carefully resected while
contralateral lead implantation or paying attention to phrenic nerve. Pericardiotomy is
extraction of functional or nonfunctional performed via an L-incision. Pericardial stay sutures
leads to regain venous access fails. are placed and coronary arteries are identified. The
974 H. Burger et al.
a b
..Fig. 30.7 Implantation of epicardial lead via left sided mini-thoracotomy a exposure of pericardium after
thoracotomy, b exposure of targeted myocardium and implantation of screw-in lead after pericardiotomy
left ventricular epicardial lead should be placed dor- approaches are also practicable, and even robotic-
30 sal to the first diagonal branch. based implantations have been reported.
Electrical mapping before lead fixation is pos-
sible, especially by using suture-on leads. Mapping
procedures while using screw-in lead implement 30.3.4 Generator Pocket
high risk for bleeding complications with low
risk/benefit ratio. Epicardial leads should be fixed The implantation side does not matter. Some phy-
directly on the myocardial tissue and areas of fatty sicians prefer the right side to preserve the left
tissue should be avoided. Suture-on leads are side for a possible later defibrillator implantation;
fixed with 5–0 Prolene suture (. Fig. 30.7b). the others prefer the patient’s nondominant
The initial pacing threshold usually decreases side—mostly the left side. This would be our rec-
after a few minutes and initial values about ommended access site as well.
1.5–2 V are acceptable. After lead fixation, an ade- With the size of current generators, the pocket
quate lead loop should be left in the pericardium for pacemaker is usually formed subcutaneously.
and might be fixed with a fine suture. The pericar- After the skin incision, the subcutaneous tissue
dium should be closed with single sutures. has to be divided until the pectoralis fascia
Afterward, the lead will be positioned across the appears. Once in the correct plane, using two fin-
intercostal space and then subcutaneously tun- gers to gently spread the tissue creates the pocket.
neled to the pocket and connected with device. Alternatively, and especially in young patients
The thoracotomy closure is performed in its ana- with tight tissue, an electrocautery or sharp dis-
tomical layers. section might be used. Some surgeons prefer to
Another option for epicardial lead implanta- place a sponge soaked in antibiotic solution into
tion is the simultaneous approach during con- the pocket until final generator placement.
comitant cardiac surgery (e.g., CABG, valve In patients with a very slim habitus and pref-
surgery, etc.). Both screw-in and suture-on leads erentially in all ICD patients, a submuscular
can be implanted. They can be subsequently (subpectoral) pocket should be the preferred
connected with device or alternatively tempo- approach. In order to create a submuscular
rarily be placed subcutaneously and connected pocket, the pectoralis major muscle is divided by
in a subsequent second intervention. blunt dissection parallel to the clavicle just down
In addition, anterior or subxiphoid thoracot- to the pectoralis minor. By doing so, some fatty
omy can be performed for RV lead and occasional tissue and vessel-nerve bundle running medially
right-atrial-lead implantation, and right-sided and laterally can be found. This bundle may be
thoracotomy might be useful for atrial epicardial clipped to reach an adequate pocket size.
lead implantation. For LV lead implantation, After the pocket was prepared, the leads are
minithoracotomy approaches or thoracoscopic secured with nonabsorbable suture material and
Chapter 30 · Device Therapy of Rhythm Disorders
975 30
connected with the device. The device will be reported in several randomized multicenter clini-
placed in the pocket and the redundant length of cal studies. According to them, a significant
the leads forming coils preferably placed behind improvement in patient symptoms, quality of life,
the generator. The generator itself should be fixed exercise tolerance, and reduction of hospitaliza-
with nonabsorbable suture in the pocket in order to tions was observed (Abraham et al. 2002; Bristow
avoid its migration. Wound drains might be placed et al. 2004).
in selected cases of diffuse bleeding or in patients In the Cardiac Resynchronization-Heart
with high bleeding risk. Afterward, the wound Failure (CARE-HF) study (Cleland et al. 2005), a
closure is usually performed with two layers of 52 % reduction of heart insufficiency-based hospi-
absorbable sutures and intracutaneous suture. talizations and a 39 % reduction of severe cardio-
vascular event-dependent hospitalization in CRT-P
patients were observed. Furthermore, a 36 %
30.4 ardiac Rhythm Device
C reduction of mortality within a period of 29 months
Therapy in Severe Heart could be demonstrated. Consistent results have
Failure been observed in the Comparison of Medical
Therapy, Pacing, and Defibrillation in Chronic
30.4.1 Cardiac Resynchronization Heart Failure (COMPANION) study (Bristow et al.
Therapy (CRT) 2004) with 36 % reduction of mortality in CRT-D
patient. The Multicenter InSync ICD Randomized
For almost 20 years now, cardiac resynchroniza- Clinical Evaluation (MIRACLE- ICD) study
tion therapy (CRT) is a well-established and effec- (Abraham et al. 2002) pointed out advantages of
tive therapy in patients suffering advanced heart using CRT-D in patients suffering NYHA class III/
failure when optimal medical treatment fails IV, LVEF ≤35 %, QRS width ≥120 ms, and com-
(Auricchio et al. 1993; Auricchio and Abraham mon ICD indication. In addition, several studies
2004). Especially, patients with severe heart fail- illustrated reduction of left ventricular end-dia-
ure based on abnormally prolonged ventricular stolic diameters and an increase of left ventricular
activation (left bundle block), systolic ventricular ejection fraction (EF) within six months, illustrat-
dysfunction, prolonged QRS duration (>120 ms), ing the effect of «reverse remodeling» in more than
New York Heart Association (NYHA) functional 70 % of patients (Yu et al. 2005).
class III or IV symptoms, left ventricular ejection . Tables 30.3, 30.4, 30.5, 30.6, 30.7, and 30.8
fraction less than 35 %, and optional mitral valve give an overview of current CRT indications
insufficiency benefit from CRT therapy. The posi- according to the European Society of Cardiology
tive impact of biventricular pacing (CRT-P (pace- (ESC) and German Society of Cardiology (DGK)
maker) and CRT-D (defibrillator)) on ventricular guidelines (see current guidelines; Dickstein et al.
strength and clinical outcome, even in long-term 2010; Vardas et al. 2007; Vlay 2009; Zipes et al.
follow-up, has been thoroughly investigated and 2006).
Class II Conflicting evidence and/or a divergence of opinion about the Should be considered
usefulness/efficacy of the given treatment or procedure
Class III Evidence or general agreement that the given treatment or Is not recommended
procedure is not useful/effective and in some cases may be
harmful
976 H. Burger et al.
Level of evidence A Data derived from multiple randomized clinical trials or meta-analyses
Level of evidence B Data derived from a single randomized clinical trials or large nonrandomized studies
Level of evidence C Consensus of opinion of the experts and/or small studies, retrospective studies, registries
..Table 30.5 Recommendation for patient with severe heart failure – New York Heart Association (NYHA) class
III or IV
CRT cardiac resynchronization therapy, CRT-D CRT with additional defibrillator function, CRT-P CRT with only pace-
maker function, LVEF left ventricular ejection fraction, NYHA New York Heart Association, QRS QRS complex
..Table 30.6 Recommendation for patient with advanced heart failure – New York Heart Association (NYHA)
class II
Class of Level of
Recommendation Patient recommendations evidence
CRT cardiac resynchronization therapy, CRT-D CRT with additional defibrillator function, CRT-P CRT with only pace-
maker function, LVEF left ventricular ejection fraction, NYHA New York Heart Association, and QRS QRS complex
..Table 30.7 Recommendation for patient with advanced heart failure and permanent atrial fibrillation (AF)
Class of
Recommendation Patient recommendations Level of evidence
CRT cardiac resynchronization therapy, CRT-D CRT with additional defibrillator function, CRT-P CRT with only pace-
maker function, LVEF left ventricular ejection fraction, NYHA New York Heart Association, QRS QRS complex
Chapter 30 · Device Therapy of Rhythm Disorders
977 30
..Table 30.8 Recommendation for patient with advanced heart failure and class I indication for pacemaker
implantation
Class of
Recommendation Patient recommendations Level of evidence
CRT cardiac resynchronization therapy, CRT-D CRT with additional defibrillator function, CRT-P CRT with only pace-
maker function, LVEF left ventricular ejection fraction, NYHA New York Heart Association, QRS QRS complex
30.4.1.1 Implantation of Transvenous and its course might be sometimes a very challeng-
Left Ventricular (CRT-) Lead ing procedure. For this purpose, many specific tools
For implantation of a transvenous left ventricular have been developed: different curved delivery
lead, we recommend to use a left-sided approach. sheaths, EP catheters, assessable EP catheters, inner
This simplifies coronary sinus intubation because sheath catheters, different guide wires, and the LV
of the more homogeneously curved course of the lead itself together with a special stylet are available.
lead as compared to a double-angled course on the Description of all variants of implantation
right side when accessing via the subclavian vein. techniques is beyond the scope, but the most com-
It is reasonable to start the procedure by mon approach for LV lead implantation should be
implanting a right ventricular lead. This gives the described herein: When reaching the right atrium
option for external pacing in case of acute AV block with a delivery sheath, a little bulk of contrast agent
or even asystole, which is a possible complication can be injected close to the tricuspid valve in a dor-
when manipulating with firm guiding sheaths close sal direction in order to locate the coronary sinus
to the tricuspid valve while trying to intubate the ostium. Coronary sinus location can be hindered
coronary sinus. Moreover, the presence of an RV by the eustachian ridge, which is mostly prominent
lead indicates the position of the tricuspid valve by close to the inferior vena cava, or Thebesian valve
the course of the lead’s loop and makes it easier to located at an inferior boundary close to the coro-
locate the ostium of the coronary sinus. An argu- nary sinus ostium or a large valve flap occluding
ment against this approach would be the possibility the ostium. Furthermore, other anatomic variants
of RV lead dislocation and sometimes reduced can seriously aggravate coronary sinus cannula-
space for the delivery catheters, especially when the tion. If coronary sinus cannulation fails, one pos-
right atrium is small. Our first choice, however, is sibility might be to place the delivery sheath into
to always start with the RV lead placement first. the right ventricle, torque it dorsally, and pull the
The procedure begins usually with subclavian catheter carefully backward with simultaneous
vein puncture and insertion of a peelable lead intro- administration of contrast fluid. When passing the
ducer valve sheath. Cephalic vein access can also be tricuspid valve, the catheter then quite often slips
used but is surgically more challenging, and the vein into the coronary sinus tube. Specific landmarks
caliber is mostly not suitable for insertion of three on the triangle of Koch are shown in . Fig. 30.8.
leads. Afterward, a delivery catheter is inserted. After successful cannulation of the coronary
Good knowledge of coronary sinus anatomy and its sinus, a target vein for LV lead placement has to
variants is indispensable for the implanting physi- be identified. For this purpose, a venography can
cian and cannulation of the coronary sinus orifice, be useful (. Fig. 30.9). Contrast agent may be
978 H. Burger et al.
Tricuspid valve
anulus
30 Triangle
of Koch
Inferior
vena cava Thebesian valve
EustachianEustachian
valve ridge
Ostium of coronary sinus
a b
..Fig. 30.10 Guide wire introduced into anterolateral target vein a followed by LV lead positioning via over-the-wire
technique b
should be fixed on the pectoralis muscle by the use (CCM). Several clinical trials could demonstrate
of permanent suture and the lead sleeve provided. the efficacy of CCM therapy in patients with
Unfortunately, not every patient can be treated severe heart failure, ejection fraction fewer than
with a transvenous LV lead. In some cases, insur- 30 % but without left bundle block, and no pro-
mountable anatomical variants, coronary sinus longed QRS complex. This patient group is not
thrombosis, coronary sinus dissection, unacceptable indicated for and will not benefit from cardiac
thresholds, no LV target vessel available, or ongoing resynchronization therapy (CRT). Therefore,
phrenic nerve stimulation represent major obstacles CCM seems to be an additional device therapy in
for successful transvenous LV lead placement. In the battle against advanced heart failure, offering
some of these cases, a second attempt is reasonable also the possibility of left ventricular «reverse
(e.g., coronary sinus dissection); however some- remodeling» in long-term therapy.
times, the only alternative for efficient LV pacing is In contrast to CRT system, CCM does not ini-
transthoracic implantation of epicardial LV leads. tiate an action potential and causes myocardial
contraction like cardiac pacing in order to resyn-
30.4.1.2 LV Lead Types chronize the failing ventricle. The biphasic CCM
In consideration of the great variance of coronary impulses are delivered during the absolute ven-
venous system, various caliber and curvatures of tricular refractory period of the contractile cycle.
target veins and many different LV leads are avail- This impulse, delivered 30 ms after QRS onset
able. Besides unipolar and bipolar leads, multipolar with high voltage of 5–7 V for duration of about
leads have been developed offering numerous (up to 20 ms, can enhance ventricular inotropy in the
fourteen) possible stimulating vectors. Furthermore, failing myocardium (. Fig. 30.11).
different lead sizes are available ranging from 6 Several clinical trails have been performed in
French (Fr) down to 4 Fr size. The majority of intra- order to get knowledge about the regional and global,
venous LV leads are being passively fixed (curved as well as the acute and long-term effects of CCM
lead tips, leads with little tines). Nevertheless, an stimulation on myocardial interactions and func-
active fixation in very large veins is also possible, tions (Winter et al. 2011). The rapid and acute posi-
although this may lead to difficulties whenever lead tive inotropic effect on myocardial contraction
extraction should be required at a later stage. appears only a few minutes after CCM stimula-
tion gets started. It depends on the increase in phos-
phorylation of phospholamban. Phospholamban is a
30.4.2 Cardiac Contractility key protein that modulates the activity of sarco-
Modulation (CCM) endoplasmatic reticulum calcium ATPase type 2a
(SERCA2a) that modulates the sarcoplasmatic retic-
A novel device therapy for advanced heart failure ulum calcium turnover (Imai et al. 2007). In chronic
represents the cardiac contractility modulation heart failure, it was found that SERCA2a expression
980 H. Burger et al.
..Fig. 30.11 CCM
biphasic impulse
n
tio
Du y
la
ra
De
Amplitude
decreases and increases again by ongoing CCM has been inserted into the left ventricle by femoral
stimulation. Moreover, it was observed that this artery access and connected with a special monitor-
effect in long-term CCM-stimulated hearts was not ing system (Millar box). With increased experience
limited only close to the stimulation area SERCA2a nowadays, the use of Millar catheter is not required.
30 expression increases even in remote regions. Most often, surgical implantation starts by
Therefore, two reasons may explain the global effect. right-sided skin incision in sulcus deltoideopec-
First, the benefit is a secondary effect based on the toralis. Afterward, a generator pocket must be
benefits in global hemodynamics, and it is responsi- prepared anterior to the pectoralis fascia. Because
ble for a change of gene expression in remote areas, of substantial energy need, the CCM impulse
and second, the change of gene expression in remote generator (Optimizer IV®, Impulse Dynamics
areas is a direct effect transmitted via gap junctions. Inc., Orangeburg, NY, USA) has to be recharged
In summary, not all details are known, yet, but regularly. In order to guaranty a safe recharging
current literature could demonstrate that CCM contact, the device cannot be overlaid by tissue
impulse may have a direct impact on cellular phys- more than 2 cm thick and so a subpectoral device
iology besides the acute effect on calcium turnover placement is not reasonable. It is also important
by increasing the intracellular calcium level. Signal that the recharging coil be placed toward an out-
can impact protein-protein interaction, modulate side direction (. Fig. 30.12).
gene expression (including reversal of fetal gene
program expressed in heart failure), and increase
local and global myocardial contractility without
the increase of myocardial oxygen consumption.
Moreover, in long-term follow-up, CCM stimula-
tion induces reverse ventricular remodeling simi-
lar to the observed ventricular remodeling after
CRT system implantation (Zhang et al. 2013).
..Fig. 30.15 Right atrial lead dislocation. Arrow indicates ..Fig. 30.16 Formation of Twiddler syndrome. Arrow
position of lead tip indicates position of lead tip
984 H. Burger et al.
..Fig. 30.19 Subclavian
lead crash. Arrow indicates
site of wire crack and/or
deinsulation of wires due
to incorrect puncture
technique far too medial.
This results in an acute
angle between coil and
further wire course.
Additionally, compression
between clavicle and rib
lead to wear and tear of the
insulation sleeve (insert)
986 H. Burger et al.
the lead should be inserted into the subclavian superior syndrome). One positive predictor for
vein at the level of the lateral third of the clavicle. complete cava thrombosis is a high number of
implanted leads present. Fortunately, because of
numerous collateral vessels, the blood flow is usu-
30.6.7 Venous Thrombosis, Vascular ally not perilously compromised, and patients in
Stenosis After Lead chronic state often are asymptomatic.
Implantation, and Vena Cava
Superior Syndrome 30.6.8 Pocket and Lead Track
Irritation, Perforation,
Subsequent venous thrombosis directly after lead
and Infection
implantation is rarely reported, but the incidence
may be underestimated. Frequency increases by
The reasons for this group of complications are
the number of implanted leads present, by lead
inadequate implant technique, device dislocation,
insertion via subclavian vein puncture, and in
patient habits (especially when certain kinds of
the presence of less surgical experience.
sport are practiced), patient weight loss, and acci-
Historically, total venous occlusions after lead
dents with mechanical trauma of the pocket.
implantation were reported to occur as frequent
Independent of causality, the pocket has to be sur-
30 as in 30–50 % of patients, mostly localized in the
gically revised.
area of lead insertion (Da Costa et al. 2002)
Device or lead perforation as well as chronic
(. Fig. 30.20). With the introduction of modern
fistulae bears high risk of pocket infection.
rather thin transvenous leads into clinical prac-
Moreover, under a worst-case scenario, a descend-
tice, the incidence of this complication decreases.
ing lead infection with consequent endocarditis
Clinical signs and symptoms of venous throm-
may occur. In order to prevent these dramatic
bosis are pain, swelling of the subsequent extrem-
complications, once perforating the leads and the
ity, and development of collateral circuits.
device have to be removed and pocket be surgi-
Repeated vessel puncture, lead extraction, or
cally debrided. Similarly, also in a scenario of
vessel dissection can be causative for acute
already manifested pocket infection (incidence
thrombosis. Chronic thrombosis resulted usually
about 1 %), surgical treatment is the therapy of
from «vascular overload» with leads or external
choice (Parsonnet and Roelke 1999; Kiviniemi
vein compression.
et al. 1999).
Diagnostic tools for confirming the suspicion
for venous thrombosis after lead implantations
include venography, ultrasound or CT, and, in a
case of an MRI compatible system, also MRI. 30.6.9 Cardiac Arrhythmia
Very rarely, the superior vena cava can be
completely obliterated by a thrombus (vena cava Ventricular extrasystole, tachycardia, or ventricu-
lar fibrillation as well as acute AV block or atrial
fibrillation can be triggered by mechanical manip-
ulation during implant procedures. Ventricular
arrhythmia usually terminates spontaneously
after the lead is pulled out of the ventricle; how-
ever, occasionally, external defibrillation may
become necessary.
Lead manipulation close to the tricuspid
valve can provoke acute AV block. In this case,
external pacing via RV or LV lead is indispens-
able. Fortunately, most of the higher-graded AV
blocks occurring during implantation are only
temporary.
In predisposed patients, manipulation in the
..Fig. 30.20 Subclavian vein stenosis. Lead tip (arrow) right atrial appendage can cause atrial fibrillation.
Chapter 30 · Device Therapy of Rhythm Disorders
987 30
Removing lead does not necessarily terminate 30.7 Lead Extraction
arrhythmia. Antiarrhythmic drug treatment may
be less effective in narcotized patients, thus Infective endocarditis after pacemaker or ICD
requiring external cardioversion. One prerequi- implantation represents the most severe complica-
site for external electrical cardioversion is echo- tion with up to 33 % mortality in CIED. In addi-
cardiographic exclusion of the presence of tion, the presence of lead vegetations with size
intracardial thrombi. >10 mm increases the potential hazard of pulmo-
nary embolism substantially. Moreover, the tricus-
pid valve can be involved and therefore open-heart
30.6.10 Phrenic Nerve Stimulation surgery might be necessary. Infected leads have to
be removed completely, and simultaneous antibi-
Phrenic nerve stimulation after pacemaker or otic therapy has to be initiated to avert the patient
ICD implantation may occur even if intraopera- of lethal complication. In case of pocket infection,
tive testing with 10 V stimulation test was not endocarditis, device or lead perforation, lead-
able to provoke diaphragm contractions. The rea- dependent arrhythmia, or high number of
son might be a different more caudal or slightly implanted leads with lead dysfunction, lead extrac-
turned heart position after the patient gets up. tions can become necessary or urgent. The major-
Diaphragm contractions are in vast majority of ity of data for lead extractions in the current
cases caused by phrenic nerve stimulation via LV literature is based on extractions due to infection
lead. Nevertheless, although relatively rare, RV (54–73.4 %) and lead dysfunction (20.3–40 %)
and RA leads may also stimulate the phrenic (Smith et al. 1994; Jones et al. 2008; Rusanov and
nerve. Spotnitz 2010).
In some cases, phrenic nerve stimulation via Lead extractions represent the most challeng-
LV lead can be suppressed by reprogramming the ing operations within the framework of cardiac
stimulation vector or different impulse widths. In rhythm device interventions. Therefore, these
this scenario, the multipolar LV leads have a clear high-risk operations at times should be con-
advantage. If phrenic nerve stimulation cannot ducted in special centers with adequate experi-
be solved via reprogramming, a temporary ence. According to published results, complete
switch-off LV lead for a period up to 3 months lead extraction success is more than 90 % with
may lead to desired effect. The possible mecha- increase in efficiency with modern coated leads.
nism is callus formation around the tip of the The mortality varies between 0.5 and 1 % inde-
lead, causing slight electrical isolation. Failing of pendently of the extraction method (Hemmer
these alternatives makes lead replacement indis- et al. 2002).
pensable. While there is no doubt about the necessity
of lead extraction in case of infected system,
extraction of old functionless leads was for a
30.6.11 Pectoral Muscle Stimulation long time controversial. Traditionally, the func-
tionless leads were shortened but left in situ.
Unipolar pacing can cause pectoral muscle stim- New leads are usually implanted from the same
ulation, when the pacemaker contact surface is side, if impossible from the contralateral side.
very small and direct muscle contact exists. Therefore, some patients collected relevant num-
Nowadays, preferentially, bipolar pacemaker ber of leads over the years. With increasing
leads are mostly used, making a simple solution number of leads, the risk for vascular stenosis/
of pacemaker stimulation vector switch to bipo- thrombosis also increases making any new lead
lar stimulation possible. If only unipolar lead is implantation without extraction of the some-
present, the generator pocket can be revised to times-very-old leads impossible. Moreover, it is
perform a different device placement. However, known that the risk of incomplete lead extrac-
pectoral muscle stimulation is in majority of tion or extraction complication increase in par-
cases caused by lead insulation defects. In these allel to lead age.
cases, the lead replacement, rather than insula- As a consequence of the dilemma and ongo-
tion repair, represents the solution. ing discussion, guidelines for transvenous lead
988 H. Burger et al.
extraction have been renewed in May 2009 by the Evolution/Shortie®, Cook Medical or
Heart Rhythm Society in collaboration with the TightRail/Mini®,Spectranetics), or excimer
American College of Cardiology (ACC) and the laser devices (SLS II® or GlideLight®,
American Heart Association (AHA) (Wilkoff Spectranetics) are currently available. These
et al. 2009). devices can be used even in case of complete
According to them, lead removal should be subclavian vein occlusion to extract
performed in patients if a CIED implantation the leads.
would require more than four leads on one side or 2. Lead extraction by open-heart surgery
five leads through the superior vena cava (class IIa In case of massive vegetation >30 mm,
indication) are indicated. strongly scarred or calcified tissue
The number of patients treated with implant- surrounding the leads, in cases of
able cardiac rhythm devices increases rapidly. endocarditis or when additional cardiac
Therefore, despite decreasing percentages of surgery is required anyways, an open-heart
complications due to better implantation tech- access is advisable. Depending on individual
nique and material improvements, the absolute circumstances, a median thoracotomy with
number of patients requiring lead extraction all cardiac surgical options or a minimally
increases substantially. For this reason, lead invasive right-sided thoracotomy with
extractions become an important part in some slightly limited options (no concomitant
30 cardiac surgery centers. Some of the different CABG possible) could be performed.
approaches for lead extraction are described Before starting the extraction, some impor-
below in detail. tant questions need to be clarified:
In general, two different approaches for lead 55 Reason for extraction
extraction exist: 55 Lead type: anchor or screw-in lead
1. Percutaneous lead extraction 55 Lead position and number
1. Unproblematic percutaneous lead removal by 55 Pacemaker or ICD lead, single or dual coil
moderate traction: Transvenous screw-in 55 Lead age
leads may be relatively easily removed by 55 Lead size
moderate traction if implanted only 1 and 55 Pacemaker-dependent patient (AV block)
up to 3 years ago. Anchor leads (passive fixa- 55 Echocardiography with information about
tion), however, tend to adhere substantially lead vegetation and its dimension, potential
especially at the lead tip. Therefore, lead lead adhesion to myocardium or tricuspid
removal via moderate traction can even be valve, tricuspid valve function, pericardial
impossible after 6 months. effusion, and ejection fraction
2. Percutaneous lead extraction by the use of 55 Number of and reason for previous pocket or
extraction devices: Old leads or leads with lead revision
strong adhesions can be extracted by 55 Previous cardiac surgery
using various extraction devices. In differ- 55 Optionally, venography to evaluate the
ent escalation steps, special lead locking subclavian and superior cava vein
devices (e.g., Liberator®, Cook Medical or 55 Other cardiac entities (e.g., coronary or valve
LLD®, Spectranetics) can be used to lock diseases) and eventually the need for cardiac
the lead and raise traction power. surgery (CABG, valve operations, etc.)
Moreover, mechanical dilating sheaths
(e.g., Byrd Sheath®, Cook Medical or
VisiSheath®, Spectranetics) can be 30.7.1 Selection of Extraction
threaded over the lead, followed by Method
countertraction, rotation, and push
forward to chip adhesions free the lead. Pacemaker screw-in leads within the first year and
The next escalation step for strong often up to 3 years after the implantation most
adhesions is the use of active extraction often can be simply extracted by moderate traction.
devices. For this purpose, electrocautery Leads with passive fixation (e.g., anchor leads)
systems (Perfecta®, Cook Medical) tend to adhere tightly, especially at the lead tip.
mechanical rotating dilator sheaths (e.g., Therefore, lead removal via moderate traction can
Chapter 30 · Device Therapy of Rhythm Disorders
989 30
even be impossible already after 3 months. under local anesthesia. However, it may limit esca-
Nevertheless, moderate traction is the method of lation of extraction methods when needed.
first choice in these leads up to 3 years after the The percutaneous lead extraction starts with
implantation. the removal of the generator out of pocket fol-
For older leads or for patients with known lowed by lead and sleeve mobilization. Thereafter,
adhesions or vein occlusions, alternative extrac- a purse string is placed around the lead close to the
tion methods using different extraction tools have clavicula. A guide wire should be inserted into the
to be applied. lead to avoid lead damage by lead pulling. In a case
Lead extractions in the presence of lead vegeta- of screw-in lead, the screw will be released. The
tions are controversially discussed in current litera- lead is carefully pulled out by continuously
ture. In general, very large vegetations should be increased traction under fluoroscopy. Because val-
extracted by open-chest cardiac surgery. Until now, vular leaflet adhesions to the lead may exist, spe-
no consensual clear cutoff exists in order to decide cial attention should be paid to tricuspid valve
whether percutaneous or open-heart lead extraction function as visualized by transesophageal echocar-
should be performed. Most physicians make a cutoff diography. In case of leaflet adhesions, further
at 20–30 mm vegetation size. The structure, form, and traction on the lead may cause massive tricuspid
consistence of vegetations as estimated in TEE also insufficiency through valve damage. In these cases,
are of utmost importance. Therefore, the decision extraction method has to be changed and counter-
whether percutaneous or open-heart lead extraction traction technique be performed. For this purpose,
will be performed must be made individually. The passive mechanical tissue dilating outer sheaths
advantage of open-heart surgery is the safe removal of like Byrd Sheath® (Cook Medical, Bloomington,
all vegetations. On the other hand, bleeding risk and IN, USA) or SightRail®/VisiSheath® (Spectranetics,
risk of infection are much higher, and reconvales- Colorado Springs, CO, USA) can be used. In case
cence is much longer when compared to any percuta- of massive adhesion more aggressive, active outer
neous approach. The disadvantage of percutaneous sheaths can become necessary like an electrosurgi-
approach is the risk of pulmonary embolism, which cal dissection cautery sheath (Perfecta® Cook
increases with larger and with friable vegetations. Medical), sharp rotating trephines like the stiff
Selection of the extraction method is based on Evolution® (Cook Medical) or the flexible
objective lead/patient characteristics, available TightRail® (Spectranetics) or an excimer laser
devices, and last but not least, on personal experi- device (SLS II®/GlideLight®, Spectranetics).
ence. Extraction should be performed either
directly in cardiac surgery or in institutions with 30.7.2.1 Lead Locking Devices
cardiac surgery being readily available. If more traction is needed, guide wire should be
taken out and lead end be cut to remove the plug
connector. The inner diameter of the lead should
30.7.2 Percutaneous Lead be determined by using gauging tool and corre-
Extraction sponding locking stylet like LLD® (Spectranetics)
or Liberator® (Cook Medical) inserted into the
Lead extractions should be performed under gen- lead. It is recommendable to fix the lead at the end
eral anesthesia, especially if the use of extraction by robust suture or metal device like Bulldog®
devices is expected. According to estimated (Cook Medical) in order to redistribute part of the
extraction risk dependent on lead age and expected traction power on lead surface (. Fig. 30.21).
adhesions or venous occlusion, transesophageal
echocardiography should be simultaneously per- 30.7.2.2 assive Mechanical Outer
P
formed in order to evaluate the function of tricus- Sheath
pid valve and eventual pericardial effusion. In After locking stylet is introduced into the lead, an
addition, extracorporeal circulation (ECC) should outer sheath device can be threaded over the lead.
be available on standby for any emergency situa- The device should be oriented parallel to lead
tions of potential hemodynamic instability or direction. Afterward, the dilator sheath can be
severe bleedings. In the case of leads implanted rotated and pushed forward under fluoroscopy
within 1 year, without vegetations and no expected control in order to stepwise dissect the lead and
adhesions, the removal can also be performed reach the subclavian vein. In addition, a combina-
990 H. Burger et al.
30.7.2.3 Active Outer Sheath Systems kMechanical rotating trephine dilator sheath
Regrettably, very old leads often have solid adhe- Mechanical rotating dilator sheaths like the stiff
sions, which cannot be separated by use of passive Evolution® (Cook Medical) or the flexible Tight-
mechanical dilator devices or contratraction Rail® (Spectranetics) represent powerful non-
devices. Moreover, extraction of the leads under electrical extraction tools. In principle, these
these circumstances represents relatively high risk trephine sheaths distinguish by the kind of its
of tricuspid valve or vascular damage. Therefore, drill bit, the character of its tubular shank and its
active outer sheath systems should be applied to length. The short versions (Shortie®, Cook Medi-
escalate extraction method after passive extraction cal and TightRail Mini®, Spectranetics) are
devices failed. Exemplary tools are electrosurgical offered to reopen the first pathway and to per-
dissection cautery sheaths, mechanic rotating tre- form constriction from lead entrance to the sub-
phine dilator sheaths, or excimer laser sheaths. clavian vein passing the clavicle and first rib.
If an active device-based lead extraction is Especially the Shortie® device mills more side-
planned, the patients should be evaluated accord- wise, and therefore, it can even dissect a very tight
ing to expected complication risk. General anes- lead adhesions close to the bone or ligamentum
thesia, ECG and arterial pressure monitoring costoclaviculare (usually as a consequence of very
unit, simultaneous transesophageal echocardiog- median subclavian vein puncture followed by
raphy (TEE), and essential instruments for pacing inadequate lead insertion). The longer devices
and defibrillation and emergency cardiac surgical Evolution® and TightRail® can thoroughly mobi-
support including an ECC on standby should be lize adhesions all throughout intravascular lead
assured. In case of pacemaker dependency, either course. Evolution® seems to be more aggressive
a temporary pacing lead has to be inserted or, as and its blades forces mostly forward even in case
Chapter 30 · Device Therapy of Rhythm Disorders
991 30
of heavy calcification. TightRail® also includes a 30.7.3 Lead Extraction
powerful drill bit which remains shielded in the by Open-Heart Surgery
shaft until activated and therefore may course less
complication. Its greatest advantage supposed to Nowadays, open-heart surgery for lead extraction
be the flexible tubular shank that allows easily to has become rare because of high efficacy of percuta-
trail lead´s course. neous lead extraction devices. But, nevertheless, in
case of incomplete lead removal, large vegetations
kExcimer laser sheath. on the leads and/or tricuspid valve, or need of addi-
Excimer laser system (SLS II®/GlideLight®, Spec- tional heart surgery anyways, it still plays an impor-
tranetics) represents another powerful extraction tant role in absolutely indicated lead extractions.
tool. In analogy to devices described above, the
locked lead is threaded into the inner lumen of
the laser sheath. To begin with, the laser sheath 30.7.4 Lead Extraction Via Median
should be pressed gently into the obstructing tis- Sternotomy
sue, followed by moderate pressure on the laser
sheath while laser is working forward. The device Lead extraction via median sternotomy is per-
can advance into the tissue with approximately formed in standard approach, bicaval cannula-
1 mm per second. Continuous homogenous trac- tion, and total cardiopulmonary bypass. After
tion on the lead must be assured (. Fig. 30.22). right atriotomy, all leads and vegetations can be
Older laser generations have sometimes removed under excellent exposure (. Fig. 30.23).
been insufficient to deal with massive calcifica- If necessary, tricuspid valve repair or replace-
tion. Therefore, the new enhanced laser system ment and/or additional surgery like mitral valve
(GlideLight®) offers now 80 Hz instead of the ear- repair/replacement or CABG might be simultane-
lier 40 Hz (SLS II®). This new enhanced laser sys- ously performed.
tem noticeably increases the dissecting speed and
theoretically also the possibility to release calci-
fied tissue without an increase in risk for vascular 30.7.5 Lead Extraction
perforations. In case of strong calcified tissue, the via Right-Sided Minimal
laser sheath should be a little oversized. A sloped Access Thoracotomy
outer sheath can also be slid over the laser sheath
and additional mechanical dissection maneuvers Median sternotomy represents the gold standard
are possible. The laser should not be used any- in cardiac surgery for lead extraction in case of
more when the lead tip is closer than 1 cm. The tip additional cardiac surgery. If there are no addi-
of the lead should be released by countertraction
using the outer sheath and the traction device.
a
b
c
e d
tractility modulation and cardiac resynchronization mias and the prevention of sudden cardiac death: a
therapy in heart failure patients with different QRS report of the American College of Cardiology/American
durations. Int J Cardiol 167:889–893 Heart Association Task Force and the European Society
Zipes DP, Camm AJ, Borggrefe M, American College of of Cardiology Committee for Practice Guidelines (writing
Cardiology/American Heart Association Task Force, committee to develop Guidelines for Management of
European Society of Cardiology Committee for Practice Patients With Ventricular Arrhythmias and the Prevention
Guidelines, European Heart Rhythm Association, Heart of Sudden Cardiac Death): developed in collaboration
Rhythm Society (2006) ACC/AHA/ESC 2006 guidelines with the European Heart Rhythm Association and the
for management of patients with v entricular arrhyth- Heart Rhythm Society. Circulation 114:e385–e484
30
995 31
Cardiac Tumors
and Pericardial Diseases
Christof Schmid
References – 1007
31.1 Cardiac Tumors atria (Reynen 1995). However, 94 % of tumors are
solitary (Carney 1985). Myxomas are usually
31.1.1 Preliminary Remarks pedunculated. They rarely sit on a broad base.
They typically arise from the interatrial septum at
Cardiac tumors are rare and have been found in the cranial border of the fossa ovalis, but they may
large autopsy statistics with an incidence of 0.17– develop at any place of the endocardium, includ-
0.19 %, which is about 1 in 500 cases (Strauss and ing heart valves and even caval veins. It has been
Merliss 1945). This includes primary benign and assumed that the reason for the preferred localiza-
malignant cardiac tumors, as well as cardiac tion at the fossa ovalis is due to the late develop-
metastases. ment of the atrial septum from fetal mesenchymal
cells, and the content of immature tissue.
The length of the tumor pedicle determines
tumor mobility. Surgically, it is interesting that the
Seventy-five percent of primary cardiac tumors tumor growth inside the stalk only rarely reaches
are benign (Mc Allister and Fenoglio 1978). beyond the endocardial layer.
Macroscopically, two-thirds of tumors present
as an ovoid or ball-shaped structure with a smooth
Myxomas account for 50 % of primary benign or slightly lobulated surface. These myxomas are
cardiac tumors followed by rhabdomyomas with rather solid. Less often (one-third of cases), a
20 % and several nonmyxomatous cardiac tumors polyploid villus-type tumor pattern is seen, which
31 with 30 % (Silverman 1980). Primary malignant is fragile, and tends to fragmentation and emboli-
tumors are dominated by sarcomas with 75 % of zation. It has been assumed that myxomas develop
cases; however, in many instances, an exact histo- from pluripotent, subendothelial mesenchymal
logical classification is impossible (Mc Allister cells (Ferrans and Robert 1973).
and Fenoglio 1978). The most frequent sarcomas There are few reports to support the hypothe-
are angiosarcomas, rhabdomyosarcomas, malig- sis that myxomas may develop malignancy. Local
nant mesotheliomas, and fibrosarcomas (Mc aggressive tumor growth as well as distant metas-
Allister and Fenoglio 1978). Secondary malignant tases including vessel wall infiltration has been
cardiac tumors, namely, cardiac metastases are observed (Read et al. 1974; Seo et al. 1980).
also very uncommon, but they can be found in
about 10–20 % of patients dying of disseminated 31.1.2.2 Other Benign Tumors
cancer (Fine 1968; Silverman 1980). The term nonmyxomatous cardiac tumor sum-
marizes rhabdomyomas, fibromas, and lipomas,
or any type of mesenchymal tissue described as
31.1.2 Surgical Anatomy main component of a heart tumor (Mc Allister
and Fenoglio 1978). In contrast to the peduncu-
31.1.2.1 Myxomas lated, usually solitary endoluminal growth of
Myxomas are mesenchymal tumors of the heart, mxyomas, nonmyxomatous cardiac tumors pres-
which can develop at any age. Their incidence is ent with intramyocardial growth even in both
highest between the third and sixth decades of life, ventricles at multiple sites. This renders surgical
with females being affected two to three times therapy difficult and radical resection in most
more often than the males (Bulkley and Hutchins cases impossible (Reece et al. 1984). At the surface
1979). On average, myxomas are 5–6 cm large and of heart valves, papillary fibroelastomas may
have a weight of about 50–60 g, but myxomas with develop, which can obstruct coronary ostia and
a diameter exceeding 15 cm have been reported cause emboli by fragmentation, also.
(Reynen 1995; Wold and Lie 1980). While 75 % of Rhabdomyomas account for 20 % of all benign
myxoma cases are localized in the left atrium, 20 % cardiac tumors. They are the most frequent car-
are in the right atrium. One ventricle, either right diac tumors found in childhood (Bigelow et al.
or left in the same frequency, is involved in only 1954). Eighty-five percent of rhabdomyoma
5 % of cases. Multiple myxomas in different heart patients are younger than 15 years. In >90 % of
chambers have been described, most often in both cases, rhabdomyomas simultaneously develop at
Chapter 31 · Cardiac Tumors and Pericardial Diseases
997 31
multiple sites, mostly affecting the ventricles. 31.1.2.4 Secondary Malignant Tumors
Pathoanatomically, rhabdomyomas are hamarto- (Metastases)
mas. Therefore, half of the children further dem- At autopsy, myocardial or pericardial metastases
onstrate symptoms of tuberous sclerosis. of extracardiac malignant tumors are much more
Fibromas of the heart also are typical for child- often found than primary malignant cardiac
hood cardiac tumors with more than 80 % occur- tumors. However, they are usually not clinically
ring in children, especially in infants and toddlers. evident because of the limitations related to the
In contrast to other tumors, they are more often underlying disease (Fine 1968). It has been esti-
solitary and exclusively found in the ventricles. mated that 10 % of metastasizing tumors finally
There is no association with other diseases or any reach the heart and pericardium, either hematog-
known hereditary trait. enously, via lymphatics, or direct ingrowth. Such
Lipomas can develop anywhere in the heart metastases are common for malignant melanoma,
and at any age. They are well encapsulated and can bronchial carcinoma, and breast cancer, and they
reach a considerable size before clinical symptom mainly involve the ventricles and the pericar-
may develop. A nonencapsulated fat deposition in dium. A peculiar variant is the endoluminally
the interatrial septum is termed «lipomatous growing hypernephroma, which reaches the heart
hypertrophy» and can be found predominantly in via the inferior caval vein. It can almost com-
obese elderly patients. pletely occlude the right atrium and ventricle with
Papillary fibroelastomas account for about a cone-like tumor mass (Paul et al. 1975).
7 % of all cardiac tumors. They arise characteris-
tically at aortic or mitral valve leaflets, less fre-
quently at tricuspid valve leaflets, or at the 31.1.3 Clinical Presentation
ventricular septum.
Myxomas are typically sporadic and isolated,
31.1.2.3 rimary Malignant Cardiac
P which means they are not associated with other
Tumors anomalies. Only about 5 % of myxoma patients
Primary malignant tumors account for about 25 % of show a hereditary familial clustering with an
all primary cardiac tumors (Mc Allister and Fenoglio autosomal dominant transmission. The latter are
1978). Almost all of them are sarcomas. They do usu- younger, without a gender preference, and present
ally not appear clinically before adulthood and there more often with multisite tumors. One out of five
is equal distribution for gender. The preferred local- myxoma patients suffers from additional neo-
ization is the right heart, mainly the right atrium. plasms (adrenocortical nodular hyperplasia,
Histologically, different mesenchymal tissues are Sertoli cell tumor, pituitary gland tumor, multiple
involved. Most common is the angiosarcoma, myxoid fibroadenomas of the breast, cutaneous
whereas rhabdomyosarcomas, myosarcomas, and myomas, and pigment alterations). This is why
other soft tissue sarcomas are much less frequent. this clinical entity has also been termed «complex
Highly malignant and undifferentiated tumors, how- myxoma» (Carney 1985).
ever, often cannot be classified histologically. The clinical presentation of the myxomas is
Sarcomas arise from the endocardium or pericar- primarily determined by their localization and
dium and rapidly infiltrate the myocardial wall, grow texture. A characteristic feature of all cardiac
into adjacent mediastinal structures, and metastasize tumors is a (variable) symptom-free interval until
early into the liver, lung, and brain (Bjerregard and diagnosis is established (Larrieu et al. 1982). Most
Baandrup 1979). At the time of diagnosis, 80 % of commonly, a local complication such as obstruc-
patients already suffer from metastases in both lungs. tion of the left ventricular outflow tract or a syn-
Another growth pattern exhibits a more intramyo- cope as sign of an intermittent LV inflow
cardial, horizontal expansion, where the endocar- obstruction initiates first symptoms and ulti-
dium and pericardium remain intact for quite a mately leads to diagnosis.
while. Finally, sarcomas sometimes demonstrate an Myxomas of the left atrium reaching a certain
exophytic growth into the heart chambers. size may impair left ventricular filling and mimic
An extremely rare primary malignant heart mitral stenosis. If myxomas grow into the mitral
tumor is the hemangioendothelioma (Lisy et al. 2007). ostium or prolapse into the left ventricle, the
998 C. Schmid
clinical picture of mitral incompetence occasion- nated by a rapidly progressive, myocardial failure
ally may evolve, also. A short and acute medical with cardiomegaly not responding to medical ther-
history is characteristic and contrasts with the apy. Intramyocardially growing tumors may lead to
long-standing symptoms in patients with postin- conduction disturbance. The suspected diagnosis is
flammatory mitral valve disease. Ultimately, the usually cardiomyopathy. Some patients complain
pedunculated myxomas lead to dyspnea due to about thoracic pain, fever, and weight loss, suspi-
lung congestion, particularly when the patients cious of a pulmonary disease. Not infrequently,
lie in on their back and the tumor prolapses into patients are referred on an emergency basis because
the mitral valve. of hemorrhagic pericardial effusion and symptoms
It is a distinctive feature in cardiac myxomas of tamponade. There also may be severe venous
that heart failure builds up suddenly and may be congestion of the upper half of the body.
associated with supraventricular rhythm disor-
ders, which do not respond to digitalis and diuret-
ics. Furthermore, syncopes (extremely rare in In patients with an extracardial malignant
acquired mitral stenosis) and seizures can be trig- disease, a secondary involvement of the
gered by hypotensive episodes. Myxomas adher- heart has to be thought of, if intractable
ent to the atrial wall provoke intermittent dyspnea bradycardia or tachycardia, cardiomegaly,
which increases with tumor growth. Peripheral hemorrhagic pericardial effusion, or
and central emboli due to detached tumor frag- obstruction of a caval vein without a visible
ments are noted in 30–40 % of cases (Silverman mediastinal neoplasm is present.
31 1980). Pathohistology of suspicious embolic
debris removed from peripheral arteries can occa-
sionally be the first diagnostic tool for cardiac The detection of malignant cells of the pri-
myxoma (Bulkley and Hutchins 1979). The inci- mary tumor after pericardial puncture may estab-
dence of cerebral emboli is high and has been lish diagnosis of cardiac metastases. Massive
reported in several investigations to be as high as hemopericardium may lead to hospital admission
50 % (Sandok et al. 1980). Neurological symptoms on an emergency basis like in cases with primary
associated with atrial myxomas have to be seen as malignant cardiac tumors; however, an acute out-
a consequence of cerebral tumor embolism. flow tract obstruction is more often responsible
Myxomas of the right atrium can lead to tricus- for referral.
pid valve dysfunction, which resembles the clinical
presentation of tricuspid stenosis, chronic right
heart failure, or constrictive pericarditis. Recurrent 31.1.4 Diagnosis and Indication
tumor embolization into the pulmonary vascula- for Surgery
ture can cause pulmonary hypertension. Rarely,
paradoxical tumor embolism of a right atrial myx- The clinical examination is unspecific and hardly
oma through a patent foramen ovale occurs. Right productive except in cases which manifest heart
atrial myxomas not infrequently lead to a «myx- failure. The chest x-ray is usually normal, but car-
oma disease,» which includes fever, arthralgies, diac enlargement, pulmonary congestion, and
polymyositis, weight loss, and hypergammaglobu- sometimes an intracardiac calcification can give a
linemia (Fitzpatrick et al. 1986). A rather common hint. The ECG is also of little help, even if rhythm
misdiagnoses is rheumatoid disease and long-term disturbances are present. The diagnosis of an
cortisone therapy for that with secondary osteopo- atrial myxoma is established by echocardiogra-
rosis has been published (Reynen 1995). phy. It also may be an incidental finding following
Ventricular myxomas and other tumors peripheral tumor embolism (DePace et al. 1981).
become symptomatic only at a very late stage. Both the transthoracic and even better the trans-
Only the progressive expansion of the tumor leads esophageal technique allow for exact visualization
to an obstruction of the inflow or outflow tract, or of the tumor location and size. Alternative diag-
an extracardial tumor growth finally compromises nostic methods are cardiac computed tomogra-
adjacent structures such as the phrenic nerve. phy and magnetic resonance imaging. Cardiac
The symptoms of malignant tumors depend on catheterization is not necessary to establish indi-
their growth pattern. The clinical picture is domi- cation for surgery but may be necessary to rule
Chapter 31 · Cardiac Tumors and Pericardial Diseases
999 31
out concomitant coronary artery disease in elderly favorable. During institution of extracorporeal cir-
patients. Indication for surgery is always given as culation, manipulation at the heart should be
the natural course of the disease cannot be pre- reduced to a minimum to prevent tumor emboliza-
dicted. The important differential diagnosis of a tion. For the same reason, it is reasonable to encircle
thrombus has to be always considered. the caval veins not before initiation of cardiopulmo-
The diagnosis of the other benign and malig- nary bypass and cross-clamping the aorta.
nant tumors is ascertained by computed tomogra- Myocardial protection is achieved with standard
phy and magnetic resonance imaging, if the patients aortic root cardioplegia and mild hypothermia.
present with heart failure, unspecific chest pain,
and/or weight loss. Echocardiography can establish 31.1.5.1 Access to the Left Atrium
the diagnosis of a malignant cardiac tumor, too. A left atrial myxoma can be approached directly
Abnormal ventricular wall motion or displacement through the left atrium via the interatrial groove
of coronary vessels in ventriculography may be sus- (Waterston’s groove) similar to a mitral access. At
picious of intramural cardiac tumor, but an exact the arrested heart, the tumor size can be analyzed,
topographic diagnosis is not possible by catheter- and the tumor pedicle can be completely excised. It
ization. Sometimes, primary malignant cardiac is recommended to excise the pedicle with a 5 mm
tumors are only recognized during an emergency surrounding of healthy tissue in a subendocardial
explorative thoracotomy. layer or even better a local full-thickness resection
of the interatrial septum. There are advantages and
disadvantages of a direct left atrial access. In face of
An indication for surgery is given in all a large tumor, retrieval in one piece and the exci-
patients when distant metastases have been sion of the pedicle may be difficult. Moreover, clo-
excluded with an adequate staging sure of the septal defect after tumor resection may
procedures and when complete resectability become difficult with this access.
of the tumor seems to be likely. We prefer a transseptal access coming through
the right atrium. The right atriotomy is followed by
an incision of the interatrial septum along the
31.1.5 Operative Techniques upper (or lower) edge of the fossa ovalis. The septal
incision is further directed toward the right upper
Although myxomas are operated upon in a rather pulmonary vein or superior caval vein (or to the
typical fashion, a so-called gold standard tech- left atrial roof if necessary) (. Fig. 31.1a).
nique has not been defined, yet. Basically, simple Thereafter, the tumor pedicle—usually attached to
myxomas are only excised out of their endocar- the atrial septum at the cranial part of the fossa
dial basis, whereas familial myxomas may also be ovalis—is exposed caudad to the incision
treated more aggressively. All other cardiac (. Fig. 31.1b). The pedicle is then excised out of the
tumors are resected as radical as possible to lower septum with a piece of full-thickness septum. The
the rate of local recurrence and clinical impor- septum is further incised as much as necessary
tance. The limits of resectability are determined until the entire tumor can be seen and completely
by the remaining functionality of the heart. removed (. Fig. 31.1c). After tumor resection and
Usually, complete tumor resection is only pos- removal, the left atrium, the pulmonary lung ori-
sible with the use of extracorporeal circulation fices, and the left ventricular cavity have to be
(Dein et al. 1987). Closed techniques for resection meticulously inspected for tumor fragments, and
of an atrial myxoma are obsolete as tumor frag- they have to be intensively rinsed. If part of the
ments or adherent thrombi can be dislodged. The pedicle of the tumor/myxoma or other tumor rem-
standard approach for extirpation of a cardiac nants are found, further resection with an appro-
tumor/atrial myxoma is the median sternotomy. priate healthy surrounding is necessary to prevent
Extracorporeal circulation is connected with bica- recurrent disease. This is usually true when the
val cannulation, where direct cannulation of both tumor arose not from the atrial septum but the free
caval veins offers the best access to the atria. If the left atrial wall and when the tumor tears off.
tumor reaches the orifice of a caval vein, one caval Whether a subendocardial resection is sufficient or
and additional cannulation of a femoral vein or the an aggressive transmural resection is necessary has
right jugular vein respectively can be more not been adequately clarified yet. The resulting
1000
C. Schmid
a b c
..Fig. 31.1 a Access to the left atrium through the atrial septum. The right atrium is longitudinally incised; the dashed
line shows the incision in the atrial septum. b The opened atrial septum gives exposure to the tumor pedicle. The
pedicle is resected around its insertion. c The left atrial tumor is removed through the extended septal incision
(parietal layer), which is covered with a thin meso- cardiography. Typical findings during echocar-
thelium (visceral layer) at its inner side. This fibro- diography are a diastolic collapse of the right
elastic sac contains about 20 cc of serous fluid, atrium and ventricle and the free floating heart
which maintains a low frictional resistance of the within the effusion («swinging heart»). Computed
moving heart against the surrounding structures tomography and magnetic resonance imaging
(pleura, mediastinum, diaphragm) (Schollmayer may serve as alternative tools, as both diagnostic
1982). The pericardial fluid also represents a bar- techniques have a high diagnostic value, also with
rier against inflammation following contamina- regard to the composition of the pericardial fluid.
tion or infection of the surrounding tissues. A pericardial tamponade is a critical increase
The clinically most important diseases of the of the amount of pericardial fluid/effusion to the
pericardium are a pericardial effusion and a tam- point of circulatory compromise. It can be
ponade as well as constrictive pericarditis. assumed in case of a decreased systemic blood
Pericardial cysts and primary pericardial tumors pressure, an increased central venous pressure,
are extremely rare. and tachycardia in combination with an accord-
ing medical history. Diagnosis is confirmed again
with the abovementioned imaging techniques.
31.2.2 Pericardial Effusion
..Fig. 31.3 a, b a b
Pericardiocentesis with
Seldinger’s technique. a A
guidewire is forwarded into
the pericardium through
the needle. b The needle
has been removed. A
smooth catheter is inserted
into the pericardium over
the guidewire so that the
effusion can be aspirated
1004
C. Schmid
age and also allows local rinsing as well the instil- persistent fenestration of the pericardium as an
lation of drugs. internal drainage into the pleura (Effler and
Under local or general anesthesia, a 4–6 cm Proudfit 1957) or a pericardial resection is recom-
median incision starting at the base of the xiphoid mended (Levitsky et al. 1976).
caudally is followed by division of the former and A thoracoscopy requires a lateral position of
the rectus abdominis muscle. A spreader is put into the patient which sometimes is not well tolerated.
place, and with elevation of the lower sternum and It also requires a double-lumen endotracheal tube
detaching the diaphragm from the sternum, the intubation and a pleural cavity without dense
pericardium can be identified. A separation of the adhesions. In selected patients, it may be an ele-
diaphragm is seldom required. The pericardium is gant alternative to open surgery.
transversely incised over a length of 2–3 cm at the After endotracheal anesthesia and thoracot-
lower border, and a smooth drainage is placed after omy, the pericardium is incised ventrally to the
aspiration of the pericardial effusion (. Fig. 31.4). phrenic nerve, and by pericardial resection, a
window of about 4 × 6 cm is created, carefully
31.2.2.4 Pericardial Window avoiding a prolapse of the heart. The pericardial
In patients with chronically recurrent serous peri- effusion is aspirated, and a drainage is placed into
cardial effusions, a simple subxiphoid pericardi- the pericardium as well as into the pleura prior to
otomy is inadequate since the drains would have closure of the wound. This pericardial drainage is
to stay in situ rather long until the healing process deemed to be important by some to keep the
has finished which would add a considerable window open as long as the intrapericardial
31 increase in the risk of infection. In these cases, a secretion maintains. A different opinion is espe-
..Fig. 31.4 Subxiphoid
pericardiotomy. After a
median skin incision along
the xiphoid process, the
abdominal wall layers are
divided and the Sternum
pericardium is opened. A
smooth pericardial
drainage is placed which is
then exited over the skin
incision or separately
Pericardium
Catheter
Heart
M. transversus
thoracis
Diaphragm
Xiphoid
Chapter 31 · Cardiac Tumors and Pericardial Diseases
1005 31
cially not to employ a pericardial drain but only a cases, a calcified shell corresponding to the peri-
pleural drain. The idea is that, with a pericardial cardial sac is visible. More suitable for diagnosis
drain placed, the window created may lose its are computed tomography and magnetic reso-
function prematurely being not necessary as long nance imaging, as they also allow visualization of
as the pericardial drain is in place. Obviously, the pericardial thickness, cardiac size (atrial dila-
both ways work in most of the patients. tation, ventricular constriction), and the delinea-
The surgical technique of the pericardial tion of the myocardial and pericardial layers
resection is similar to the pericardiectomy in con- (Soulen et al. 1985; Sutton et al. 1985). Moreover,
strictive pericarditis (see below). an infiltration of the pericardial calcification into
the myocardium which increases the operative
risk due to myocardial lacerations can be verified.
31.2.3 Constrictive Pericarditis Echocardiography is less suitable to diagnose a
constrictive pericarditis; however, the restriction
In the second half of the last century, the incidence of diastolic filling with a simultaneously preserved
of constrictive pericarditis has steadily decreased systolic contractile function can be well demon-
and finally was reported to account for 0.2–0.5 % strated.
of all cardiac diseases (Hermann et al. 1983; The hemodynamic proof of a constriction is
McCaughan et al. 1985; Wood 1961). Whereas for- obtained by a right heart catheterization. When
merly previous tuberculosis had been responsible measuring the pressure course in the right ven-
in most cases for this disease, a tuberculous etiol- tricle, a characteristic dip and plateau phenome-
ogy is now encountered in <3 % of constrictive non is noted, which comprises an undisturbed
pericarditis (Hamelmann 1962; McCaughan et al. early diastolic filling («dip») and an abrupt stop of
1985). Nowadays, the underlying etiology is quite the ventricular filling («plateau»). Additionally,
variable. Each pericarditis—of unspecific, viral, an equalization of the right atrial and right ven-
bacterial, or fungal origin—can finally result in a tricular pressure is visible. This phenomenon is
constriction of the heart. Increasingly important typical for a restrictive disease, but does not dif-
are a mediastinal radiation, a postcardiotomy ferentiate between restrictive cardiomyopathy
inflammation, and a trauma (Bubenheimer et al. and constrictive pericarditis.
1985; Pick et al. 1984; Rice et al. 1981). A pathological electrocardiogram and unspe-
The embrace and constriction of the heart by cific ST segment and T wave alterations are com-
the scarred pericardium, and sometimes also cal- mon, but barely specific. A low voltage of the QRS
cified shells, with a thickness of up to 1 cm, leads complex or atrial fibrillation is noted in one-third
to an increasing impediment of atrial and con- of patients (McCaughan et al. 1985).
secutively ventricular filling. When the superior The indication for a pericardiectomy is estab-
or inferior caval veins are strangulated, the venous lished with the typical clinical and diagnostic
return is additionally impaired. The constrictive findings.
pericarditis also compromises myocardial con-
traction und ultimately leads to muscular atrophy. 31.2.3.2 Pericardiectomy
Cardiac ejection fraction drops and the body is A pericardiectomy is an elective surgical proce-
underperfused. Already in NYHA II, end-organ dure.
dysfunction of lung, liver, and kidney evolves
(Hermann et al. 1983). Clinically, dyspnea on
exertion, abdominal complaints with hepatomeg- The perioperative management requires
aly and ascites, and peripheral edema dominate. monitoring of the systemic arterial and both
systemic and pulmonary venous pressures
31.2.3.1 Diagnosis and Indication (right and left atrium), since the filling
for Pericardiectomy pressures can rapidly alter after release of the
The diagnosis of constrictive pericarditis can be pericardial constraint and the pump function
established with evaluation of hemodynamic/ worsens due to the lessened wall tension.
functional parameters and/or with imaging tech- Moreover, this online monitoring allows an
niques. A chest x-ray only rarely demonstrates an immediate online control of the surgical result.
increased silhouette of the heart, but in 40 % of
1006
C. Schmid
Kleinschmidt O (1945) Die Eingriffe an der Brust und in der and prognosis of lesions other than cIassical benign
Brusthöhle. Allgemeine und spezielle chirurgische myxoma in 20 patients. J Thorac Cardiovasc Surg
Operationslehre, Bd 111/3. Springer, Berlin 88:439–452
Larrieu A, Jamieson WR, Tyers GF et al. (1982) Primary car- Rehn L (1913) Zur experimentellen Pathologie des
diac tumors. Experience with 25 cases. J Thorac Herzbeutels. Verh Dtsch Ges Chir 42:339
Cardiovasc Surg 83:339–348 Reynen K (1995) Cardiac myxomas. N Engl J Med 333:
Levitsky S, Engelman R, Konchigevi HN, Wyndham CRC, 1610–1617
Roper K (1976) Total pericardiectomy for uremic peri- Rice PL, Pifarre R, Montoya A (1981) Constrictive pericardi-
carditis. Circulation 54:11–21 tis following cardiac surgery. Ann Thorac Surg 31:
Lisy M, Beierlein W, Müller H, Bültmann B, Ziemer G (2007) 450–453
Left atrial epithelioid hemangioendothelioma. Robertson JM, Mulder DG (1984) Pericardiectomy: a
J Thorac Cardiovasc Surg 133:803–804 changing scene. Am J Surg 148:86–92
Marvasti MA, Obeid AI, Potts JL, Parker FB (1984) Approach Sandok BA, von Estorff I, Giuliani ER (1980) Subsequent
in the management of atrial myxoma with long-term neurological events in patients with atrial myxoma.
follow-up. Ann Thorac Surg 38:53–58 Ann Neurol 3:305–307
Mc Allister HA, Fenoglio JJ Jr (1978) Tumors of the cardio- Sauerbruch F (1925) Chirurgie der Brustorgane. Springer,
vascular system. In: Hartmann WH, Cowan WR (eds) Berlin
Atlas of tumor pathology, series 2. Armed Forces Schollmayer P (1982) Perikarditis. In: Roskam H, ReindelI H
Institute of Pathology, Washington, DC, pp 1–20 (eds) Herzkrankheiten. Springer, BerIin, p S 1161
McCaughan, Schaff HV, Piehier JM et al. (1985) Early and Seo IS, Warner TF, Colyer RA, Winkler RF (1980)
late results of pericardiectomy for constrictive pericar- Metastasizing atrial myxoma. Am J Pathol 4:391–399
ditis. J Thorac Cardiovasc Surg 89:345–350 Shumaker HB Jr, Roshe J (1960) Pericardiectomy.
Paul O, Castleman B, White PD (1948) Chronic constrictive peri- J Cardiovasc Surg 2:65
carditis: a study of 53 cases. Am J Med Sci 216:361–380 Silverman N (1980) Primary cardiac tumors. Ann Surg
31 Paul JG, Rhodes OB, Skow JR (1975) Renal cell carcinoma 191:127–139
presenting as right atrial tumor with successful removal Sortie O, Myhre ES, Stalsberg H (1984) Angiosarcoma of
using cardiopulmonary bypass. Ann Surg 181:471–474 the heart: unusual presentation and survival after
Pick RA, Joswig BC, Bloor GM (1984) Recurrent cardiac con- treatment. Br Heart J 51:94–97
striction after pericardiectomy. Arch Intern Med Soulen RL, Stark DD, Higgins CB (1985) Magnetic reso-
144:2061–2063 nance imaging of constrictive pericardial disease. Am
Portal RW, Bestermann EM, Chambers RJ, SeIlors TH, J Cardiol 55:480–484
Somerville W (1966) Prognosis after operation for con- Strauss R, Merliss R (1945) Primary tumor of the heart. Arch
strictive pericarditis. Br Med J 1:563–569 Pathol Lab Med 39:74–80
Poule GY, Meredith JW, Breyer RH, Mills SA (1983) Surgical Sutton FJ, Whiteley NO, Applefeld MM (1985) The role of
implications in malignant cardiac diseases. Ann Thorac echocardiography and computed tomography in the
Surg 36:484–490 evaluation of constrictive pericarditis. Am Heart
Read RC, White HJ, Murphy ML, Willimas, Sum CN, Flanagan J 109:350–355
WH (1974) The malignant potentiality of left atrial myx- Wold LE, Lie JT (1980) Cardiac myxomas: a clinicopatho-
oma. J Thorac Cardiovasc Surg 68:857–862 logic profile. Am J Pathol 101:219–240
Reece IJ, Cooley DA, Frazier OH, Hallman GL, Powers PL, Wood P (1961) Chronic constrictive pericarditis. Am
Montero CG (1984) Cardiac tumors: cIinical spectrum J Cardiol 7:48–61
1009 32
Cardiac Injury
Thierry Carrel
References – 1015
..Fig. 32.1 Algorithm
for treatment of a patient
Trauma Patient
with penetrating
mediastinal trauma
Circulatory status. Stable?
Instable Stable
Surgery Diagnostics
is performed. Any posttraumatic hemopericar- and has to be treated with a patch, the injured
dium represents an indication for surgery. ventricle generally requires unloading with the
Unstable patients are treated in the emergency help of the extracorporeal circulation. If there is
room by left anterolateral thoracotomy. Stable no cardiopulmonary bypass circuit available, a
patients are treated, when necessary, in the oper- short period (preferably less than 3–5 min) of bi-
ating room by median sternotomy or left thora- caval inflow occlusion (clamping of the superior
cotomy, depending on the site of injury (Asensio vena cava and the inferior vena cava) may be per-
et al. 1998). A stab wound to the right side of the formed following hyperventilation and maximal
sternum is rather approached via a sternotomy; a oxygen delivery. The ventricles will be empty after
stab wound to the left chest via a left anterior tho- a few heartbeats and the aorta can also be clamped.
racotomy (Kaljusto and Tønnessen 2012; Sanchez The cardiac injury can be treated in the remaining
et al. 2010; Mitchell et al. 1993). 2–3 min, after which the circulation can be re-
established (if necessary with manual cardiac
massage to start).
32.3 Stab Wounds and Gunshot With less extensive injuries, the heart can be
Wounds temporarily and repeatedly stopped using intra-
venous administration of adenosine in order to
Patients who are admitted to hospital with a suture the myocardium (Kokotsakis et al. 2007).
stab wound to the heart but do not require Stabilizers used in «off-pump» bypass surgery can
resuscitation have a relatively good chance of help local immobilization of the heart. In addition
surviving the injury if they receive adequate to the pericardium, both pleural cavities should
treatment. This is in contrast to patients who be opened via median sternotomy in order to
32 require resuscitation or have borderline hemo- eliminate or treat further sources of bleeding from
dynamics, as is often the case with gunshot intercostal or mammary arteries, as well as hilar
wounds. In a hospital with a cardiac surgery vessels and lung parenchyma. In cases in which
unit, a median sternotomy is the preferred there is a transdiaphragmatic stab wound directed
option to approach the chest, because this towards the heart, the upper abdomen (stomach,
approach usually allows maximal control of the liver) also must be evaluated.
major cardiac injuries and facilitates the use of A significant rate of postdischarge complica-
extracorporeal circulation if needed. However, tions is associated with penetrating cardiac inju-
ECC use is also possible through femoro-femo- ries. In a retrospective trauma registry review
ral cannulation if thoracotomy has been chosen from a level I trauma center, (Tang et al. 2011)
as the surgical approach. However, cardiac inju- reviewed 406 patients who sustained a penetrat-
ries originating parasternally from the right ing cardiac injury during a 10-year period. Besides
should only be treated via median sternotomy. observations on the in-hospital stay, outpatient
If possible, foreign objects should only be follow-up, echocardiogram results and outcomes
removed after opening of the pericardium and were analyzed. One hundred nine (26.9 %) sur-
the placement of sutures to control bleeding. vived to hospital discharge. A significant rate of
Bleeding wounds at the atrium, the aorta or the cardiac complications was observed, and follow-
vena cava are best controlled with partial clamp- up echocardiographic evaluation was found to be
ing when possible; a localized ventricular myo- necessary prior to discharge.
cardial injury is best controlled digitally. After Cardiac chambers involved were the right
the source of bleeding has been verified, sutures ventricle (45.9 %), left ventricle (40.3 %), right
can be placed. atrium (10.1 %), left atrium (0.9 %) and combined
Atrial perforations are closed with polypro- (2.8 %). Abnormal echocardiograms demon-
pylene 4/0 or 5/0. For larger injuries, an autolo- strated pericardial effusion, abnormal wall
gous or xenopericardial patch may be used. Large motion, decreased ejection fraction (<45 %),
sutures in the ventricle are best reinforced with intramural thrombus and valve injury as the most
Teflon felt or xenopericardium to prevent further frequently observed complications following pen-
tearing of the tissue. Biological glues can provide etrating cardiac trauma. Following discharge, the
additional strengthening of the closure (Jones 1-year and 9-year survival rates were 97 % and
et al. 2012). When the heart is severely injured 88 %, respectively.
Chapter 32 · Cardiac Injury
1013 32
32.4 Blunt Cardiac Trauma sometimes only becoming so after hours or days,
potentially leading to heart failure (Asensio et al.
Blunt cardiac trauma and blunt trauma to the 1998). In this case, other injuries such as traumatic
large vessels frequently occur in association with ventricular septal defect or traumatic valvular
multiple injuries, mainly in road traffic accidents defect must be excluded first. Delayed or long-
(deceleration trauma), but also in falls, explo- term effects may include pericardial tamponade,
sions, combat actions or dangerous sports. The rupture of the injured endocardium and formation
steering wheel injury was once the most common of a ventricular aneurysm in the contusion area,
cause, but this has been significantly reduced with which are treated either surgically, endovascularly
the advent of seat belts and airbags. or using hybrid procedure (Kadner et al. 2007).
The presentation of blunt, posttraumatic car- The treatment of myocardial contusion is gen-
diac injuries ranges from a total lack of symptoms erally conservative. Antiarrhythmic medication
to impaired myocardial function, although this and, if necessary, catecholamines are initiated; if
rarely leads to symptoms of cardiogenic shock. cardiac instability persists, invasive monitoring
Blunt trauma can result in the following damages including a Swan-Ganz catheter is advisable. In
to the heart: cases of severe ventricular dysfunction, an intra-
55 Myocardial contusion aortic balloon pump or other cardiac support sys-
55 Injury to the pericardium, the atrial free wall, tems like peripheral ECMO may be used until the
the endocardium, the interventricular heart shows signs of recovery. Pericardial effusion,
septum, the valves and the coronary arteries as long as not hemorrhagic in origin, is treated with
drainage or occasionally by repeated puncture.
Cardiac trauma may be present without any vis-
ible external chest injury. Electrocardiogram is com-
monly normal. Fractures to the sternum, ribs (in 32.5 Heart Valve Injury
particular parasternal fractures) and mediastinal
enlargement as well as an atypical mediastinal shape The appropriate surgical method for managing an
are always suspicious. The most important diagnos- injury to a heart valve is selected based on the char-
tic feature is a widened mediastinum, which can be acter of the heart valve injury, e.g. direct sutures for
observed on an anterior- posterior chest X-ray. small tears, refixation for partial avulsion or other
Immediate surgery with the use of cardiopulmonary reconstructive procedures. Large valve defects occa-
bypass is only appropriate in patients with significant sionally necessitate valve replacement (Hashmi
injuries rehoming valve repair or replacement or et al. 2010). AV valve ruptures caused by tearing of
reconstruction of another initial structure. The deci- the valve leaflets or by rupture of the chordae or
sion to operate under full heparinization (as required papillary muscles occasionally occur, in particular
for cardiopulmonary bypass) must be weighed following accidents leading to rudder new are
against the risks of a life-threatening hemorrhage in increase in the chest. Regarding AV valves, the more
other organ systems (e.g. brain), especially in cases of ventrally positioned tricuspid valve is more often
associated cranial and pelvic fractures or other sites affected than the mitral valve (Thekkudan et al.
of frank bleeding. The management also involves 2012); however, tricuspid valve insufficiency usually
continuous ECG monitoring (arrhythmias, signs of becomes symptomatic only in a chronic state. Acute
myocardial infarction) and echocardiography. If mitral valve insufficiency can be followed by pul-
allowed by the hemodynamic status, coronary angi- monary hypertension with concomitant acute pul-
ography as well as radiological MRI may be indi- monary edema. The insufficient mitral valve must
cated in the presence of elevated serum parameters usually be operated on immediately. Rupture of the
for significant myocardial ischemia. aortic valve occurs less frequently than of the AV
While commotio cordis simply describes a valves. If the patient survives the acute trauma, a
functional disorder of the heart, cardiac contusion new murmur and signs of decompensation may
involves a pathological change to the myocardium later appear. Depending on the severity of the injury,
caused by trauma and can range from small, local- this can occur immediately, but may also appear
ized endo-, intra- or epicardial bleeding to trans- later. Posttraumatic heart failure carved by a valve
mural necrosis (Berg et al. 2012). A cardiac injury that cannot be controlled conservatively
contusion is not always immediately symptomatic, must be treated surgically in a timely fashion.
1014 T. Carrel
32.6 Coronary Artery Injury pigtail catheter should be inserted above the
puncture needle in order to provide further drain-
Coronary artery injury is characterized by a high age. Should the bleeding be severe or persistent, a
mortality rate (Asensio et al. 1998). In general, cor- large subxiphoidal chest tube must be inserted
onary artery injury leads to pericardial tamponade. (Thorson et al. 2012). A complete tear of the peri-
Assuming it can be promptly recognized by a tho- cardium with cardiac herniation into the pleural
racotomy, the acute treatment of small vessel lesions space with circulatory collapse due to kinking of
is best performed by ligation of the appropriate seg- the heart is extremely rare.
ment. Larger vessels (>1.5 mm) should be bypassed
(aortocoronary venous bypass or left internal tho-
racic artery bypass (see also 7 Chapter «Coronary 32.8 Myocardial Injury
Artery Disease», Sect. 22.4.1). Arteriovenous coro-
nary fistulas and arterial fistulas to the heart cavities Injury to the myocardium after blunt and penetrating
are rare complications and have to be closed surgi- trauma usually involves the right atrium, although
cally exceptionally by transcatheter method. if the other locations are possible. A complete rupture is
shunt volume is large. Occasionally, catheter- usually first identified at necropsy. If the patient does
guided interventional treatment using coils or reach the clinic, a suspected myocardial rupture
other occlusion devices may be necessary. The most should be immediately treated in the operating the-
frequent carve of coronary artery injury is catheter ater (Kumar et al. 2012). Myocardial rupture can
intervention. More recently, coronary injuries have appear immediately or after an asymptomatic inter-
been described following interventional antiar- val of several days and without clinical warnings.
rhythmic ablation procedures (Wong et al. 2011).
32
32.9 Traumatic Ventricular Septal
32.7 Pericardial Injury Defect
The most common cardiac injury involves the peri- A traumatic ventricular septal defect usually
cardium. If this results in persistent bleeding from the appears shortly after the accident and can be sus-
pericardium, hemopericardium or hemothorax may pected by evidence of a murmur and the clinical
develop. Small bleeding varices are not acutely life- situation and verified by echocardiography. A small
threatening and may sometimes only be recognized ventricular septal defect with no hemodynamic
by a pericardial friction murmur. Even small amounts impairment can initially be treated conservatively.
of pericardial fluid (100–200 ml) can cause typical Immediate surgical intervention is only required
signs and symptoms of a tamponade with jugular for large ventricular septal defects with acute pul-
venous distension with or without a drop of the arte- monary hypertension (Sugiyama et al. 2011; Ryan
rial pressure. et al. 2012). As with muscular defects, they are
closed using conventional suture technique with
patches. In case of thermodynamic stability, pros-
thetic or xenopericardial traumatic defects are ide-
Early signs of cardiogenic shock in
ally closed after 4–12 weeks once the borders have
combination with a decline of the diuresis, an
become fibrotic (Schaffer et al. 1999). In certain
enlarged cardiac silhouette in the chest X-ray
situations, an interventional percutaneous defect
or a direct sign of a pericardial effusion in
closure may be considered.
echocardiography or CT scan require
immediate pericardial puncture or
subxiphoid drainage (Adams et al. 2012). 32.10 Iatrogenic Cardiac Injury
Romaguera R, Waksman R (2011) Covered stents for coro- Tang AL, Inaba K, Branco BC et al. (2011) Postdischarge
nary perforations: is there enough evidence? Catheter complications after penetrating cardiac injury: a sur-
Cardiovasc Interv 78:246–253 vivable injury with a high postdischarge complication
Romaguera R, Sardi G, Laynez-Carnicero A et al. (2011) rate. Arch Surg 146:1061–1066
Outcomes of coronary arterial perforations during Thekkudan J, Luckraz H, Ng A, Norell M (2012) Tricuspid
percutaneous coronary intervention with bivalirudin valve chordal rupture due to airbag injury and review
anticoagulation. Am J Cardiol 108:932–935 of pathophysiological mechanisms. Interact
Ryan L, Skinner DL, Rodseth RN (2012) Ventricular septal Cardiovasc Thorac Surg 15:555–557
defect following blunt chest trauma. J Emerg Trauma Thorson CM, Namias N, Van Haren RM et al. (2012) Does
Shock 2:184–187 hemopericardium after chest trauma mandate ster-
Sanchez GP, Peng EW, Marks R, Sarkar PK (2010) ‘Scoop and notomy? J Trauma Acute Care Surg 72:1518–1525
run’ strategy for a resuscitative sternotomy following Verna E, Repetto S, Saveti C, Forgione N, Merchant S,
unstable penetrating chest injury. Interact Cardiovasc Binaghi G (1992) Myocardial dissection following suc-
Thorac Surg 10:467–468 cessful chemical ablation of ventricular tachycardia.
Schaffer RB, Berdat PA, Seiler C, Carrel TP (1999) Isolated Eur Heart J 13:844–846
fracture of the ventricular septum after blunt chest Wong KC, Lim C, Sadarmin PP et al. (2011) High incidence
trauma. Ann Thorac Surg 67:843–844 of acute sub-clinical circumflex artery ‘injury’ following
Sugiyama G, Lau C, Tak V et al. (2011) Traumatic ventricular mitral isthmus ablation. Eur Heart J 32:1881–1890
septal defect. Ann Thorac Surg 91:908–910
32
1017 33
Combined Procedures
in Cardiac and Vascular
Surgery
Heinz G. Jakob and Stephan C. Knipp
References – 1028
95
90
0
0 1 2 3 4 5
Years
..Fig. 33.1 Kaplan-Meier curves for stroke-free survival in the Asymptomatic Carotid Surgery Trial (ACST). In this trial,
33 CEA resulted in a 5.35 % ARR over 5 years. As you can see from the curves, the stroke rate in the conservative group
remained constant over time (s. ACST Halliday et al. 2004)
acetylsalicylic acid arm or the acetylsalicylic acid sus 17.9 % (ARR 4.6 %) (Halliday et al. 2010)
monotherapy arm (controls) (Executive (. Figs. 33.2 and 33.3).
Committee for the Asymptomatic Carotid The benefits of surgery for isolated carotid dis-
Atherosclerosis Study 1995). Despite a periopera- ease still strongly depend on the severity of the
tive complication rate of 2.3 %, the rate of ipsilat- carotid stenosis. Most often it is expressed as per-
eral stroke after 5 years was significantly lower in centage reduction of the lumen. Although the results
the surgery group compared with the controls obtained very much depend on the actual method of
(5.1 % vs. 11 %; relative risk reduction 54 %; NNT measurement, quite often, the latter is not stated. The
16.9; p < 0.004) (Rothwell 2004). Between 1993 and angiographic measurements of the US trials
2003, a total of 3120 patients with >60 % stenosis (NASCET) do not coincide with those of the
from 30 countries were randomized in 126 trial European studies (ECST), and only rarely are these
sites as part of the subsequent large-scale European measurements checked against duplex scans and cal-
Asymptomatic Carotid Surgery Trial (ACST) culations, which are increasingly becoming a routine
(Halliday et al. 2004). After the expected increased modality. In the case of 70 % stenosis determined by
initial risk, the Kaplan-Meier graphs of both the NASCET method, it has to be realized that this
groups already intersected 2 years after random- really corresponds to an extremely severe case of ste-
ization, and 5 years later, the rate of stroke was sig- nosis with as much as 95 % reduction in lumen.
nificantly lower for the surgery arm (6.4 % vs.
11.8 %; p < 0.0001) (. Fig. 33.1). While meta-
analysis of both trials shows a benefit with CEA, 33.2.4 Surgical and Interventional
this benefit is far less pronounced than in symp- Options
tomatic patients. Even 10 years after randomiza-
tion, the benefits of successful carotid surgery still For patients requiring coronary surgery who also
persist with a combined risk of stroke and death present with significant carotid atherosclerosis,
(perioperative and over the course) of 13.4 % ver- various combinations regarding sequence of
Chapter 33 · Combined Procedures in Cardiac and Vascular Surgery
1023 33
CEA + off-pump CABG» to 2.7 % in «staged CEA- amputation of the internal carotid artery (ICA) and
CABG,» 4.2 % in «staged CAS-CABG,» 4.6 % in subsequent anastomosis with the common carotid
«synchronous CEA + on-pump CABG,» and 6,3 % artery (CCA). The latter technique is particularly
in «reverse staged CABG-CEA» (Naylor et al. useful when the internal carotid artery is elongated.
2003a, 2009). The relative low stroke rate in the After the oblique skin incision along the anterior
«staged CEA +CABG» arm was partially offset by margin of the sternocleidomastoid muscle, dissec-
the highest rate of myocardial infarction (6.5 %). In tion is carried out first through the subcutaneous
«reverse staged surgery,» where CABG was per- tissue, then the platysma, and finally the neurovas-
formed first followed by CEA, the rate of myocar- cular bundle; both the hypoglossal nerve and the
dial infarction was lowest (0.9 %); on the other vagus nerve must be treated with meticulous
hand, this group displayed the highest risk of stroke. respect. A transverse facial vein is transected
Somewhat unexpectedly, «staged CAS-CABG» was between ligatures. Once the carotid vessels have
associated with the highest procedural mortality been exposed, vessel loops are placed around the
rate of 5.5 %. The cumulative 30-day rate of stroke, arteries, and the vessels are then clamped. The CCA
myocardial infarction, and death was comparable is opened and longitudinal arteriotomy extended
in all groups at 10–12 %, while other analyses beyond the stenosis into the ICA. The stenosing cal-
showed rates of up to 17.7 % (Roffi 2007). A large- cified cylinder is separated from the wall with a dis-
scale nationwide hospital database analysis in the sector and extracted under direct vision of its distal
USA for the period 1998–2007 compared patients end. Care must be taken not to leave any intimal
who underwent CEA before or after CABG during flap. In case of severe contralateral carotid stenosis
the same hospital stay, but not on the same day, or if there is no neuromonitoring (e.g., SSEP, NIRS,
with patients who underwent synchronous CEA + transcranial duplex scanning), shunting is recom-
CABG. Mortality (4.2 % vs. 4.5 %) and stroke rate mended, and the TEA should be performed as an
(3.5 % vs. 3.9 %) in the 6153 patients of the «staged» open procedure. Usually, the arteriotomy is closed
arm were similar to that in the 16,639 patients of with a patch plasty fashioned from autologous
33 the synchronous arm (p > 0.7 for both) (Gopaldas saphenous vein, a procedure which lends itself in
et al. 2011). However, morbidity was slightly higher case of synchronous CABG; patches tailored from
in the «staged» group (48.4 % vs. 42.6 %; OR 1.8; xenogenic bovine or Vascu-Guard pericardium or
95 % confidence interval [CI] 1.5–2.2; p < 0.001). Dacron may also be employed. In case of a large-
These results imply the necessity for prospective diameter artery (>8 mm), the arteriotomy may be
comparison trials studying sequential or synchro- closed directly. Due to intraoperative hepariniza-
nous combined treatment and also stand-alone tion, the wound should only be closed after comple-
conservative treatment without touching the tion of the CABG and heparin reversal. In order to
carotid stenosis. avoid hyperperfusion while the patient is on-pump,
In synchronous surgery, the carotid procedure it is desirable to leave the autoregulation of the brain
is usually carried out before CABG. Quite often, it intact. Thus, the theoretical advantages of alpha-stat
is performed by separate teams of vascular and blood gas management strategy should be employed
heart surgeons. In synchronous surgery, some- to avoid hypercapnia which may otherwise affect
times the carotid procedure is performed under autoregulation (Schoof et al. 2007; Selim 2007).
regional anesthesia.
Carotid revascularization is performed either as 33.2.4.1 I solated CABG or CABG
conventional thromboendarterectomy (TEA) with Followed by CEA (Reverse
dissection of the intima cylinder and patch graft or Staged)
as eversion endarterectomy (EEA) with transverse The estimated prevalence of significant carotid
stenosis (i.e., ≥50 %) in patients undergoing CABG
is 6–8 %, but the rate of synchronous combined
If surgery is performed under general anes- surgery is about 2 % in administrative data banks
thesia without routine intraluminal shunting, on CABG (Naylor and Bell 2002). This suggests
cerebral monitoring (e.g., somatosensory that quite a substantial number of cardiac opera-
evoked potentials [SSEP], near-infrared spec- tions are performed without actively addressing
troscopy [NIRS]) is recommended. concomitant carotid stenosis. Despite this com-
mon practice, there are only few data available
Chapter 33 · Combined Procedures in Cardiac and Vascular Surgery
1025 33
examining the correlation between untreated with impaired ventricular function, the percent-
severe asymptomatic carotid stenosis and the rate age of patients where staged CEA is indicated
of stroke during and after cardiac surgery. A sin- should be low. The percentage of patients eligible
gle-center case series of 50 patients with >70 % for this procedure is estimated at less than 10 %
carotid stenosis did not demonstrate an increased (Hertzer et al. 1997).
rate of stroke compared with a patient population
without carotid stenosis (Ghosh et al. 2005). In a
larger retrospective study of 878 patients with
In the overwhelming majority of cases with
documented preoperative carotid duplex scans
concomitant carotid artery stenosis and
who underwent isolated CABG between 2003 and
coronary artery disease, it is not justified to
2009, the group of 117 patients with carotid steno-
risk myocardial infarction by performing
sis >75 % did not suffer from an increased rate of
isolated CEA.
in-hospital stroke (3.4 % vs. 3.6 %) or death (3.4 %
vs. 4.2 %) when compared to patients without
(severe) carotid stenosis (Mahmoudi et al. 2011).
In contrast, the only prospective observational Once interventional treatment of carotid steno-
trial in patients with (symptomatic and asymp- sis became more prevalent, the benefits of dilating
tomatic) carotid stenosis comparing isolated and stenting the carotid stenosis before CABG were
CABG with combined CABG followed by CEA also studied. So far, experience with this regimen is
(«reverse staged CABG-CEA») found a higher still limited. Prospective trials with a small number
rate of stroke, but a lower mortality and a lower of patients have concluded that changing the surgi-
rate of myocardial infarction (Hertzer et al. 1989). cal sequence in favor of intervening on the carotid
Irrespective of the impact on the immediate artery before cardiac surgery is not justified
perioperative rate of stroke once the carotid stenosis (Randall et al. 2006; Versaci et al. 2009). Analysis of
has been eliminated as part of the combined CABG several trials including 760 patients with carotid
and CEA procedure, it is also necessary to consider angioplasty and stenting before CABG found a
the effect of CEA in the long-term prevention of combined risk of stroke, myocardial infarction, and
stroke. While previous trials on asymptomatic death in 9.4 % of the cases over a 30-day period after
carotid stenosis (ACAS, ACST) have demonstrated CABG; however, the 5.5 % rate of death was higher
a slight benefit for primary preventive CEA than in any other treatment series (van der Heyden
(Executive Committee for the Asymptomatic et al. 2007, 2008). After carotid angioplasty and
Carotid Atherosclerosis Study 1995; Halliday et al. stenting, it is highly recommended to initiate anti-
2004), this is being reexamined by several ongoing platelet therapy with acetylsalicylic acid and clopi-
trials, the nonsurgical regimen of which has been dogrel for 4 weeks, which in turn will delay cardiac
improved as compared with the past, in particular surgery due to the increased risk of bleeding. Since
with regard to consistent administration of choles- a waiting period of 5–7 days is also required after
terol synthesis inhibitors (i.e., statins), acetylsali- termination of clopidogrel, this mandates a waiting
cylic acid, and close control of blood pressure (e.g., period of at least 5 weeks before cardiac surgery can
angiotensin- converting enzyme inhibitors, AT1 be performed. Whether this is acceptable must be
receptor blockers) (Transatlantic Asymptomatic assessed for each patient individually. In order to
Carotid Intervention Trial [TACIT], Stent- avoid such waiting periods, some study groups per-
Protected Angioplasty vs.Carotid Endarterectomy-2 form carotid angioplasty and stenting under acetyl-
[SPACE-2] in asymptomatic carotid stenosis, salicylic acid and full heparinization 24 h before or
Asymptomatic Carotid Trial [ACT]). The CABACS on the same day as CABG, whereas clopidogrel is
trial will also contribute to this. started after heart surgery (Versaci et al. 2007,
2009). Due to the low number of cases and a lack of
33.2.4.2 taged CEA Followed by
S comparison trials, for the time being, this hybrid
CABG revascularization cannot be deemed superior. With
If CABG is strictly indicated only in highly symp- the growing interest of cardiologists in interven-
tomatic patients or is performed as a preventive tional carotid procedures and its enthusiastic accep-
measure in patients with a stenotic left main coro- tance by the patients, an unbiased evaluation of this
nary artery and coronary three-vessel disease treatment option is warranted.
1026 H.G. Jakob and S.C. Knipp
33.2.4.3 Synchronous CEA plus CABG chronous surgery seems to be higher than the
In synchronous combined surgery for cardiac and simple addition of the known complication rates
carotid disease, the sequence of events is not uni- for each procedure alone.
form; most often, first, CEA is performed, usually The question as to whether additional carotid
under general anesthesia but sometimes under surgery actually increases the complication rate as
regional anesthesia. Some centers prefer to revas- such or if a less favorable risk profile of the patients
cularize the carotid artery once extracorporeal undergoing synchronous procedures is responsi-
circulation is initiated, still delaying cardiac sur- ble for this result yields conflicting answers. A
gery. With this protocol, the moderate hypother- comparison of patients registered in the New York
mia on cardiopulmonary bypass may serve as a State Cardiac Surgery Database who underwent
brain-protecting measure (Minami et al. 2000). combined surgery with a group of patients having
One risk, however, is the rather prolonged on- the same risk according to the propensity score
pump period using extracorporeal circulation. A did not demonstrate any difference in the compli-
trial comparing both protocols was unable to cation rate. This implies that the additional CEA
demonstrate the presumed benefits of CEA per- does not add to the increased complication rate
formed on bypass (Bonacchi et al. 2002). (Ricotta et al. 2005). However, a study comparing
Systematic meta-analysis of a large number of patients undergoing isolated CABG with a risk-
single-center observational studies, comprising a adjusted group of patients undergoing synchro-
total of 7753 patients undergoing synchronous nous surgery demonstrated a complication rate
CEA + CABG, demonstrated a 4.6 % rate of stroke which was higher by 38 % (Dubinsky and Lai
for the 30-day period following the surgery 2007). The authors of this study blamed it on the
(Naylor et al. 2003a; Naylor 2009). The cumula- combined surgery.
tive rate of stroke or death was 8.2 % (95 % CI
7.1–9.3 %). Taking also into account the signifi-
cance of preventing perioperative myocardial 33.2.5 Treatment Rationale
33 infarction, a complication often neglected in the in Coronary Artery Disease
context of preventive CEA, the 30-day risk of and Concomitant Carotid
death, stroke, or myocardial infarction increased Stenosis
to 11.5 % (95 % CI 10.1–13.1 %). A nationwide US
register trial comprising 26,197 patients who There are no clear-cut recommendations in the
underwent surgery between 2000 and 2004, not current literature or guidelines regarding the treat-
including the systematic reviews cited above, ment rationale in patients requiring coronary sur-
found an 8.6 % risk of stroke or death after syn- gery and also exhibiting high-grade asymptomatic
chronous CABG and CEA (Timaran et al. 2008). carotid stenosis. Therefore, optimum treatment
One alternative to traditional synchronous CEA strategy for this disease entity still is open for
and on-pump CABG is the combination of CEA debate. The higher complication rates in (staged or
and off-pump CABG. Pooled data from trials on synchronous) combined surgery compared with
synchronous CEA and off-pump CABG in 324 isolated CABG (leaving the asymptomatic carotid
patients showed a relatively low risk of stroke at stenosis untouched) demand that the need for
1.1 % and a 30-day risk of death, stroke, or myo- combined surgery must be assessed in detail for
cardial infarction of only 3.6 % (95 % CI 1.6– each patient. Appropriate strategies would involve
5.5 %) (Beauford et al. 2003; Garcia-Rinaldi and assessment of each disease on its own in terms of
Cruz 2004; Mishra et al. 2004; Fareed et al. 2009). the urgency of its treatment. To this end, data are
Although this might be due to statistical error available at the highest evidence level, together
(e.g., small series, publication bias, etc.), another with guidelines derived from these data. Priority
important explanation could be the simple fact should be given to treatment of the symptomatic
that off-pump CABG avoids manipulation and disease. Recurrent transient ischemic attacks, par-
cannulation of the aorta as well as extracorporeal ticularly within a period of 14 days after onset,
circulation. Thus, more detailed research into this underline the urgency of treating the carotid
protocol is warranted. Therefore, the risk of syn- stenosis. On the other hand, unstable angina or
Chapter 33 · Combined Procedures in Cardiac and Vascular Surgery
1027 33
stenosis of the left main coronary artery is an indi- 33.3 ther Combined Cardiac
O
cation for urgent treatment of the coronary artery and Vascular Procedures
disease. Should both coronary disease and carotid
disease be symptomatic, this situation would 33.3.1 Combined Cardiac
necessitate synchronous surgery. and Abdominal Aortic
If there are no symptoms or if angina is only Aneurysm Surgery
triggered at high levels of physical exertion, the deci-
sion must be based on objective findings. Filiform Of all patients requiring abdominal aortic aneu-
stenosis of important coronary arteries should not rysm (AAA) repair, 27–46 % also presented with
be put at the risk of occlusion prior to CEA, while coronary artery disease (Kioka et al. 2002;
moderate stenosis of the left main coronary artery Garofalo et al. 2005). Coronary artery disease and
should allow synchronous surgery or even isolated its sequelae are the main causes for perioperative
CABG leaving the carotid stenosis untouched. complications and mortality in AAA repair. For
Asymptomatic carotid stenosis requires a very this reason, special emphasis is placed on presur-
precise assessment of the severity of the stenosis, gical diagnostics of coronary heart disease, in par-
primarily through diagnostic angiography as well ticular, when prophylactic surgery of
as carotid duplex scanning. It should be ensured asymptomatic abdominal aortic aneurysms is
that the degree of stenosis is >70 % according to planned. Several trials, including one with a pro-
the NASCET method or >80 % according to the spective randomized design, however, could not
ECST protocol. Here, too, there are no verified demonstrate an overall advantage of preventive
data on the necessity of synchronous surgery, and coronary revascularization preceding major vas-
the results of perspective randomized trials need cular procedures (McFalls et al. 2004). Only
to be awaited. An asymptomatic significant patients with symptomatic angina pectoris at low
carotid stenosis according to the above measure- exercise levels (Canadian Cardiovascular Society
ments, with concomitant occlusion of the contra- [CCS] classes III and IV), unstable angina, or
lateral carotid artery, however, would support the acute coronary syndrome should not undergo
decision for synchronous surgery. Furthermore, elective vascular surgery without prior coronary
the decision should also be based on the results of revascularization. There are no data on the risks
prospective trials on isolated surgery of asymp- of elective vascular procedures in patients with
tomatic carotid stenosis since long-term preven- morphological findings relevant for the prognosis
tion of stroke is to be expected (SPACE-2, TACIT). of long-term survival such as left main coronary
When it comes to surgical techniques and stenosis or left main equivalent coronary disease
sequence of treatment for coexisting carotid and or three-vessel coronary disease with impaired
coronary disease, there are insights into which left ventricular function. It has to be assumed,
treatment method may be superior. To date, the however, that these conditions will increase the
expectations for interventional treatment of surgical risk. In coronary heart disease, AAA does
carotid stenosis before CABG have fallen short. not represent a complication-prone concomitant
The results of on-pump CEA under the protection disorder. Although asymptomatic AAA with a
of hypothermia also did not yield any benefits diameter >5 cm is not infrequent in patients with
when compared with the other protocols. coronary disease (incidence 1–5.2 %), AAA rup-
There were hopes to get further insights into ture during heart surgery appears to be extremely
the best treatment strategy for concomitant coro- rare (Durham et al. 1991; Bergersen et al. 1998;
nary and carotid arteriosclerotic disease with reli- Monney et al. 2004).
able data, from the CABACS trial (A Randomised The risk of aneurysm rupture increases expo-
Comparison of Synchronous CABG and Carotid nentially with its diameter, and for aneurysms with
Endarterectomy Vs. Isolated CABG in Patients a diameter >7 cm, the risk of rupture within a year
with High-Grade Carotid Stenosis), starting in is 32.5 % (Lederle et al. 2002). Aneurysms of this
December 2010. These hopes were blasted, when size should be repaired at an early stage—approx.
the trial was stopped early by the funder because 2 weeks after cardiac surgery—or, at the latest,
of slow recruitment (Weimar et al. 2016). when they are 8 cm in diameter and simultaneously
1028 H.G. Jakob and S.C. Knipp
with cardiac surgery (Blackbourne et al. 1994). ficial femoral artery, these procedures are par-
Simultaneous surgery should preferably also be ticularly promising if there is angiographic
performed on symptomatic aneurysms (Onwudike evidence of collateral vessels connecting to a
et al. 2000). The coincidence of coronary artery dis- popliteal artery which is still patent. If this is
ease and AAA, both urgently requiring surgery, missing completely up to the level of the trifurca-
appears to be rare. tion in the lower leg and if there is also gangrene,
In terms of surgical technique, the AAA is best bypass revascularization, with its known risks, of
exposed through full-length median sternotomy one of the arteries in the lower leg cannot be
and laparotomy. This extensive access offers an avoided. It is recommended that this procedure
exceptionally good view for both procedures. be performed after successfully completed
Current publications describe the benefits and, in CABG surgery and carried out in a cardiovascu-
part, better results of off-pump CABG surgery lar context which can be predicted to be as stable
(Ascione et al. 2001; Wolff et al. 2006). The per- as possible. The bypass material available and the
ceived benefits of the on-pump technique, how- quality of the target vessels should offer a reason-
ever, include a decreased load on the heart during able expectation of successful revascularization
the aortic clamping period and the possibility of as the need for revision surgery during the post-
autotransfusion during extracorporeal circulation. operative period can thus be kept as low as pos-
In exceptionally rare cases, it may be advisable sible. In any case of peripheral vascular occlusive
to perform simultaneous carotid surgery, CABG, disease, the respective extremity should be
and AAA repair. spared from vein harvesting. With significant
peripheral vascular occlusive disease present at
the time of cardiac surgery, the patient must
33.3.2 Cardiac Surgery always be informed in detail about the possibility
and Peripheral Vascular of severe limb ischemia.
Procedures
33
Although many patients with coronary artery
disease reveal peripheral arterial occlusive dis- References
ease also, synchronous surgery is rarely required.
Ascher E, Hingorani A, Yorkovich W, Ramsey PJ, Salles-Cunha
Only when peripheral artery disease with pain at S (2001) Routine preoperative carotid duplex scanning
rest or already at the stage of necrosis is inacces- in patients undergoing open heart surgery: is it worth-
sible by interventional techniques, synchronous while? Ann Vasc Surg 15:669–678
surgery may be indicated. In order to keep the Ascione R, Iannelli G, Lim KH, Imura H, Spampinato N
(2001) One-stage coronary and abdominal aortic
surgical trauma as limited as possible, less inva-
operation with or without cardiopulmonary bypass:
sive surgical techniques for the peripheral vascu- early and midterm follow-up. Ann Thorac Surg 72:768–
lar part are preferred, even if this may mean a 774, discussion: 775
limited long-term outcome. Therefore, despite Barnett HJ, Taylor DW, Eliasziw M et al. (1998) Benefit of
the poorer long-term outcome, occlusive disease carotid endarterectomy in patients with symptomatic
moderate or severe stenosis. North American
of the abdominal aorta would be treated by
Symptomatic Carotid Endarterectomy Trial
extra-anatomical axillofemoral bypass, since this Collaborators. N Engl J Med 339:1415–1425
will avoid having to enter both the thoracic and Beauford RB, Saunders CR, Goldstein DJ (2003) Off pump
abdominal cavities, or a unilateral retroperito- concomitant coronary revascularization and carotid
neal procedure would be performed on the iliac endarterectomy. J Cardiovasc Surg (Torino) 44:407–415
Bergersen L, Kiernan MS, McFarlane G, Case TD, Ricci MA
axis, focusing on the extremity with the most
(1998) Prevalence of abdominal aortic aneurysms in
severely impaired blood flow at rest. Quite often, patients undergoing coronary artery bypass. Ann Vasc
pain at rest without necrosis due to involvement Surg 12:101–105
of the femoral arteries can be treated by isolated Bernhard VM, Johnson WD, Peterson JJ (1972) Carotid
revascularization at the level of the femoral artery stenosis. Association with surgery for coronary
artery disease. Arch Surg 105:837–840
bifurcation, e.g., by endarterectomy of the com-
Blackbourne LH, Tribble CG, Langenburg SE et al. (1994)
mon femoral artery and usually combined with Optimal timing of abdominal aortic aneurysm repair
patch grafting of the deep artery of the thigh. after coronary artery revascularization. Ann Surg
Even if there is extensive occlusion of the super- 219:693–696, discussion: 696–698
Chapter 33 · Combined Procedures in Cardiac and Vascular Surgery
1029 33
Bonacchi M, Prifti E, Frati G et al. (2002) Concomitant Fareed K, Rothwell PM, Mehta Z, Naylor AR (2009)
carotid endarterectomy and coronary bypass surgery: Synchronous carotid endarterectomy and off-pump
should cardiopulmonary bypass be used for the coronary artery bypass: An updated systematic
carotid procedure? J Cardiac Surg 17:51–59 review of early outcomes. Eur J Vasc Endovasc Surg
Brott TG, Hobson RW 2nd, Howard G et al. (2010) Stenting 37:375–378
versus endarterectomy for treatment of carotid artery Garcia-Rinaldi R, Cruz H (2004) Concomitant carotid endar-
stenosis. N Engl J Med 363:11–23 terectomy and off-pump coronary revascularization.
Brown KR, Kresowik TF, Chin MH, Kresowik RA, Grund SL, Ann Thorac Surg 78:1883
Hendel ME (2003) Multistate population based out- Garofalo M, Nardi P, Borioni R, Del Giudice C, Pellegrino A,
comes of combined carotid endarterectomy and coro- Chiariello L (2005) The impact of coronary revascular-
nary artery bypass. J Vasc Surg 37:32–39 ization on long-term outcomes after surgical repair of
Byrne J, Darling RCR, Roddy SP et al. (2006) Combined abdominal aortic aneurysm. Ital Heart J 6(Suppl):
carotid endarterectomy and coronary artery bypass 369–374
grafting in patients with asymptomatic high-grade ste- Ghosh J, Murray D, Khwaja N, Murphy MO, Walker MG
noses: an analysis of 758 procedures. J Vasc Surg (2005) The influence of asymptomatic significant
44:67–72 carotid disease on mortality and morbidity in patients
Daneault B, Kirtane AJ, Kodali SK et al. (2011) Stroke associ- undergoing coronary artery bypass surgery. EJVSF
ated with surgical and transcatheter treatment of aortic 29:88–90
stenosis. A comprehensive review. JACC 58:2143–2150 Gopaldas RR, Chu D, Dao TK et al. (2011) Staged versus syn-
Danton MH, Anikin VA, McManus KG, McGuigan JA, chronous carotid endarterectomy and coronary artery
Campalani G (1998) Simultaneous cardiac surgery with bypass grafting: analysis of 10-year nationwide out-
pulmonary resection: presentation of series and review comes. Ann Thorac Surg 91:1323–1329
of literature. Eur J Cardiothorac Surg 13:667–672 Halliday A, Mansfield A, Marro J, Peto C, Peto R, Potter J,
Das SK, Brow TD, Pepper J (2000) Continuing controversy in Thomas D (2004) Prevention of disabling and fatal
the management of concomitant coronary and carotid strokes by successful carotid endarterectomy in
disease: an overview. Int J Cardiol 74:47–65 patients without recent neurological symptoms:
DeBakey ME, Crawford ES, Cooley DA, Morris GC Jr, Garrett Randomised controlled trial. Lancet 363:1491–1502
HE, Fields WS (1965) Cerebral arterial insufficiency: one Halliday A, Harrison M, Hayter E et al. (2010) 10-year stroke
to 11-year results following arterial reconstructive oper- prevention after successful carotid endarterectomy
ation. Ann Surg 1965(161):921–945 for asymptomatic stenosis (ACST-1): a multicentre ran-
Dubinsky RM, Lai SM (2007) Mortality from combined domised trial. Lancet 376:1074–1084
carotid endarterectomy and coronary artery bypass Hertzer NR, Beven EG, O’Hara PJ, Krjewski LP (1989)
surgery in the US. Neurology 68:195–197 Surgical staging for simultaneous coronary and
Durand DJ, Perler BA, Roseborough GS et al. (2004) carotid disease: a study including prospective ran-
Mandatory versus selective preoperative carotid domisation. J Vasc Surg 9:455–463
screening: a retrospective analysis. Ann Thorac Surg Hertzer NR, O’Hara PJ, Mascha EJ, Krajewski LP, Sullivan TM,
78:159–166, discussion: 159–166 Beven EG (1997) Early outcome assessment for 2228
Durham SJ, Steed DL, Moosa HH, Makaroun MS, Webster consecutive carotid endarterectomy procedures: the
MW (1991) Probability of rupture of an abdominal aor- Cleveland Clinic experience from 1989 to 1995. J Vasc
tic aneurysm after an unrelated operative procedure: a Surg 26:1–10
prospective study. J Vasc Surg 13:248–251, discussion: Hill MD, Shrive FM, Kennedy J, Feasby TE, Ghali WA (2005)
251–252 Simultaneous carotid endarterectomy and coronary
Dyszkiewicz W, Jemielty MM, Piwkowski CT, Perek B, artery bypass surgery in Canada. Neurology 64:1435–
Kaasprzyk M (2004) Simultaneous lung resection for 1437
cancer and myocardial revascularization without car- Hill MD, Brooks W, Mackey A et al. (2012) Stroke after
diopulmonary bypass (off-pump coronary artery carotid stenting and endarterectomy in the Carotid
bypass grafting). Ann Thorac Surg 77:1023–1027 Revascularization Endarterectomy versus Stenting
Dyszkiewicz W, Jemielity M, Piwkowski C, Kasprzyk M, Perek Trial 126:3054–3061
B, Gasiorowski L, Kaczmarek E (2008) The early and late Hillen T, Nieczaj R, Munzberg H et al. (2000) Carotid athero-
results of combined off-pump coronary artery bypass sclerosis, vascular risk profile and mortality in a
grafting and pulmonary resection in patients with con- population- based sample of functionally healthy
comitant lung cancer and unstable coronary heart dis- elderly subjects: the Berlin ageing study. J Intern Med
ease. Eur J Cardiothorac Surg 34:531–535 247:679–688
European Carotid Surgery Trialists΄ Collaborative Group Hogue CW, Murphy SF, Schechtman KB, Davila-Roman VG
(1998) Randomized trial of endarterectomy for recently (1999) Risk factors for early or delayed stroke after car-
symptomatic carotid stenosis: final results of the MRC diac surgery. Circulation 100:642–647
European Carotid Surgery Trial (ECST). Lancet 351: International Carotid Stenting Study investigators, Ederle J,
1379–1387 Dobson J, Featherstone RL et al. (2010) Carotid artery
Executive Committee for the Asymptomatic Carotid stenting compared with endarterectomy in patients
Atherosclerosis Study (1995) Endarterectomy for with symptomatic carotid stenosis (International
asymptomatic carotid artery stenosis. JAMA 273: Carotid Stenting Study). an interim analysis of a ran-
1421–1428 domised controlled trial. Lancet 375:985–997
1030 H.G. Jakob and S.C. Knipp
Kioka Y, Tanabe A, Kotani Y et al. (2002) Review of coronary bypass: influence of surgical and patient variables. Eur
artery disease in patients with infrarenal abdominal J Vasc Endovasc Surg 26:230–241
aortic aneurysm. Circ J 66:1110–1112 Naylor AR, Mehta Z, Rothwell PM (2009) A systematic
Knipp S, Scherag A, Beyersdorf F et al. (2012) Randomized review and meta-analysis of 30-day outcomes follow-
comparison of synchronous CABG and carotid endar- ing staged carotid artery stenting and coronary
terectomy vs. isolated CABG in patients with asymp- bypass. Eur J Vasc Endovasc Surg 37:379–387
tomatic carotid stenosis: the CABACS trial. Int J Stroke North American Symptomatic Carotid Endarterectomy
7:354–360 Trial Collaborators (1991) Beneficial effect of carotid
Lederle FA, Johnson GR, Wilson SE et al. (2002) Rupture rate endarterectomy in symptomatic patients with high-
of large abdominal aortic aneurysms in patients refus- grade carotid stenosis. N Engl J Med 325:445–453
ing or unfit for elective repair. JAMA 287:2968–2972 Onwudike M, Barnard M, Singh-Ranger R, Raphael M,
Likosky DS, Leavitt BJ, Marrin CA et al. (2003) Intra- and Adiseshiah M (2000) For debate: concomitant critical
postoperative predictors of stroke after coronary coronary arterial disease and abdominal aortic aneu-
artery bypass grafting. Ann Thorac Surg 76:428–434, rysm—timing of corrective procedures. Cardiovasc
discussion: 435 Surg 8:333–339
Lloyd-Jones D, Adams R, Carnethon M et al. (2009) Heart Randall MS, McKevitt FM, Cleveland TJ, Gaines PA, Venables
Disease and stroke statistics 2009 update. A report GS (2006) Is there any benefit from staged carotid and
from the American Heart Association Statistics coronary revascularization using carotid stents? A
Committee and Stroke Statistics Subcommittee. single-center experience highlights the need for a ran-
Circulation 119:480–486 domized controlled trial. Stroke 37:435–439
Mahmoudi M, Hill PC, Xue Z et al. (2011) Patients with Rankin J (1957) Cerebral vascular accidents in patients over
severe asymptomatic carotid artery stenosis do not the age of 60. II. Prognosis. Scott Med J 2:200–215
have a higher risk of stroke and mortality after coro- Rao V, Todd TR, Weisel RD, Komeda M, Cohen G, Ikonomidis
nary artery bypass surgery. Stroke 42:2801–2805 JS, Christakis GT (1996) Results of combined pulmo-
McFalls EO, Ward HB, Moritz TE et al. (2004) Coronary- nary resection and cardiac operation. Ann Thorac Surg
artery revascularization before elective major vascular 62:342–347
surgery. N Engl J Med 351:2795–2804 Ricotta JJ, Faggioli GL, Castiloni A, Hassett JM (1995) Risk
McKhann GM, Grega MA, Borowicz LM Jr, Baumgartner WA, factors for stroke after cardiac surgery. Buffalo Cardiac-
Selnes OA (2006) Stroke and encephalopathy after car- Cerebral Study Group. J Vasc Surg 21:359–363
33 diac surgery: an update. Stroke 37:562–571
Minami K, Fukahara K, Boethig D, Bairaktaris A, Fritzsche D,
Ricotta JJ, Wall LP (2003) Treatment of patients with com-
bined coronary and carotid atherosclerosis.
Koerfer R (2000) Long-term results of simultaneous J Cardiovasc Surg (Torino) 44:363–369
carotid endarterectomy and myocardial revasculariza- Ricotta JJ, Wall LP, Blackstone E (2005) The influence of con-
tion with cardiopulmonary bypass used for both pro- current carotid endarterectomy on coronary bypass: a
cedures. J Thorac Cardiovasc Surg 119:764–773 case-controlled study. J Vasc Surg 41:397–401, discus-
Mishra Y, Wasir H, Kohli V et al. (2004) Concomitant carotid sion: 401–402
endarterectomy and coronary bypass surgery: out- Roach GW, Kanchuger M, Mangano CM et al. (1996) Adverse
come of on-pump and off-pump techniques. Ann cerebral outcomes after coronary bypass surgery: mul-
Thorac Surg 78:2037–2042, discussion: 2042–2043 ticenter study of perioperative ischemia research
Monney P, Hayoz D, Tinguely F et al. (2004) High preva- group and the ischemia research and education foun-
lence of unsuspected abdominal aortic aneurysms in dation investigators. N Engl J Med 335:1857–1863
patients hospitalised for surgical coronary revasculari- Roffi M (2007) Management of patients with concomitant
sation. Eur J Cardiothorac Surg 25:65–68 severe coronary and carotid artery disease: is there a
Moussa ID, Rundek T, Mohr JP (2007) Asymptomatic carotid perfect solution? Circulation 116:2002–2004
artery stenosis: risk stratification and management: a Rothwell PM (2004) ACST: which subgroups will benefit most
primer on risk stratification and management. Informa from carotid endarterectomy. Lancet 364:1122–1123
UK ltd., Oxon Schoof J, Lubahn W, Baeumer M et al. (2007) Impaired cere-
Naylor AR (2009) Does the risk of post-CABG stroke merit bral autoregulation distal to carotid stenosis/occlu-
staged or synchronous reconstruction in patients with sion is associated with increased risk of stroke at
symptomatic or asymptomatic carotid disease? cardiac surgery with cardiopulmonary bypass.
J Cardiovasc Surg 50:71–81 J Thorac Cardiovasc Surg 134:690–696
Naylor AR, Bell PRF (2002) Carotid artery disease and stroke Selim M (2007) Perioperative stroke. N Engl J Med 356:
during coronary artery bypass: a critical review of the 706–713
literature. Eur J Vasc Endovasc Surg 23:283–294 Sheiman RG, d’Othee BJ (2007) Screening carotid sonog-
Naylor AR, Cuffe RL, Rothwell PM, Bell PR (2003a) A system- raphy before elective coronary artery bypass graft
atic review of outcomes following staged and syn- surgery: who needs it. Am J Roentgenol 188:
chronous carotid endarterectomy and coronary artery W475–W479
bypass. Eur J Vasc Endovasc Surg 25:380–389 Tarakji KG, Sabik JF III, Bhudia SK, Batizy LH, Blackstone EH
Naylor R, Cuffe RL, Rothwell PM, Loftus IM, Bell PR (2003b) (2011) Temporal onset, risk factors, and outcomes
A systematic review of outcome following synchro- associated with stroke after coronary artery bypass
nous carotid endarterectomy and coronary artery grafting. JAMA 305:381–390
Chapter 33 · Combined Procedures in Cardiac and Vascular Surgery
1031 33
Timaran CH, Rosero EB, Smith ST, Valentine RJ, Modrall JG, Versaci F, Del Giudice C, Scafuri A et al. (2007) Sequential
Clagert GP (2008) Trends and outcomes of concurrent hybrid carotid coronary artery revascularization: imme-
carotid revascularization and coronary bypass. J Vasc diate and mid-term results. Ann Thorac Surg 84:
Surg 48:355–361 1508–1513
Van der Heyden J, Suttorp MJ, Bal ET et al. (2007) Staged Versaci F, Reimers B, Del Giudice C et al. (2009) Simultaneous
carotid angioplasty and stenting followed by cardiac hybrid revascularization by carotid stenting and coro-
surgery in patients with severe asymptomatic carotid nary artery bypass grafting. The SHARP study. J Am
artery stenosis: early and long-term results. Circulation Coll Cardiol Intv 2:393–401
116:2036–2043 Weimar C, Bilbilis K, Rekowski J, et al. (2016) A randomized
Van der Heyden V, Lans HW, van Werkum JW, Schepens M, comparison of synchronous cabg and carotid endar-
Ackerstaff RG, Suttorp MJ (2008) Will carotid angioplasty terectomy vs isolated cabg in patients with asymp-
become the preferred alternative to staged or synchro- tomatic high-grade carotid stenosis: the cabacs trial.
nous carotid endarterectomy in patients undergoing (Abstr.) European Stroke Journal 1(1S) 718
cardiac surgery? Eur J Vasc Endovasc Surg 36:379–384 Wolff T, Baykut D, Zerkowski HR, Stierli P, Gurke L (2006)
van Swieten J, Koudstaal P, Visser M, Schouten H et al. (1988) Combined abdominal aortic aneurysm repair and coro-
Interobserver agreement for the assessment of handi- nary artery bypass: presentation of 13 cases and review
cap in stroke patients. Stroke 19:604–607 of the literature. Ann Vasc Surg 20:23–29
1033 34
Combined Cardiac
and Thoracic Surgery
Mark K. Ferguson
References – 1039
A number of concerns have been raised during the 34.2.2 Organ Dysfunction
past two decades regarding whether eliminating
an additional anesthetic and an additional inci- Organ dysfunction is common after cardiopul-
sion, both of which are required for staged cardiac monary bypass and is related to the systemic
and lung operations, is sufficient to overcome the inflammatory response that results in part from
potential adverse effects of a combined operation CPB as well as other factors such as hypoperfu-
(. Table 34.1). These issues may have limited the sion and hypoxemia. Subclinical organ dysfunc-
number of combined operations performed for tion is common and becomes clinically manifest
patients with cardiac problems and lung cancer. in patients with limited reserve or who have an
Chapter 34 · Combined Cardiac and Thoracic Surgery
1035 34
exaggerated inflammatory response to CPB. The 1997), at least one case report of systemic tumor
incidence of major organ dysfunction after iso- dissemination during such surgery has been pub-
lated coronary artery bypass grafting (CABG) is lished (Hasegawa et al. 2002). The systemic
appreciable, including renal failure in 3.6 %, respi- inflammatory response that follows CPB may
ratory failure in 9.6 %, cerebrovascular accident in increase the ability of such cells to adhere to the
1.4 %, and composite adverse outcomes in 14.4 % vascular endothelium, diapedese through the
(Shahian et al. 2009). Such perturbations in organ capillary wall, and develop into micrometastases.
function adversely affect a lung resection patient’s The mechanism for this may be an increase in cir-
ability to recover from surgery. Impaired cogni- culating adhesion molecules which has been
tion reduces a patient’s ability to cooperate with reported after CPB, whereas no such change was
postoperative pulmonary toilet exercises and identified during lung resection operations (Boldt
other physical therapies; renal dysfunction may et al. 1998). Interestingly, one study reported
precipitate fluid overload and contribute to prob- improved long-term survival in lung cancer
lems with respiratory gas exchange; cardiac patients in whom the cancer was removed prior to
dysfunction may also contribute to pulmonary institution of CPB (Brutel de la Reviere et al.
edema. Ventilation is severely impaired during 1995). Whether this was related to elimination of
the first 24–48 h after major lung resection, result- most circulating tumor cells prior to the onset of
ing in a 60–70 % decrease in forced vital capacity; bypass and the systemic inflammatory response is
other contributions to pulmonary dysfunction unknown.
related to concomitant cardiac surgery may fur- One of the primary concerns about the ade-
ther test pulmonary reserve during this critical quacy of major lung resection at the time of a car-
period. diac operation is surgical exposure and access to
the hilum and mediastinum. In general, the surgi-
cal approach is chosen to favor exposure for the
34.2.3 Oncologic Issues cardiac operation, while exposure for lung resec-
tion is a secondary or sometimes a tertiary con-
Circulating tumor cells can be identified in the sideration. Most often the cardiac and thoracic
peripheral blood of a moderate number of lung procedures are performed through a sternotomy.
cancer patients and in a large percentage of pul- Although it is technically feasible to do most stan-
monary venous blood in such patients (Okumura dard lobectomies or pneumonectomies using this
et al. 2009). The percentages vary somewhat with approach, a sternotomy is rarely the incision of
the staining techniques used for identifying the choice for a major lung resection. In addition,
cells. Such circulating cells are found in 20 % of access to performing a left lower lobectomy can
patients with stage I lung cancer (Chen et al. be challenging, particularly in patients with car-
2013). The importance of these cells is not entirely diomegaly. More technically demanding opera-
clear, although they have been shown to have tions, such as segmental resections, bronchoplasty
prognostic value, in addition to histology and or arterioplasty, and sleeve resections, can be
stage, for survival. They have also been purported quite difficult to accomplish through the exposure
to serve as a means for identifying tumor makers offered by a standard sternotomy. If combined
and for monitoring response to systemic therapy surgery is necessary, it is optimal to perform the
(O’Flaherty et al. 2012; Young et al. 2012). While more technically demanding pulmonary resec-
the lung tumor is the source of circulating tumor tions through a separate incision at the time of the
cells in patients with early stage lung cancer, the cardiac operation.
lung also serves as a filter removing such cells Of perhaps more importance is access to the
from the peripheral circulation. Placing patients mediastinum for performing systematic lymph
on cardiopulmonary bypass circulates the blood node sampling or mediastinal nodal dissection,
past this natural filter directly into the systemic which is a standard of care for lung cancer resec-
circulation, potentially exposing the peripheral tion (Lardinois et al. 2006; De Leyn et al. 2007).
circulation to a higher concentration of circulat- The regions that are most difficult to access
ing cells. Although one study demonstrated that through a sternotomy include the pulmonary lig-
most circulating tumor cells are trapped on the ament (level 9), paraesophageal (level 8), and
internal surface of arterial filters (Akchurin et al. subcarinal (level 7) nodes. Lack of adequate
1036 M.K. Ferguson
..Table 34.2 Outcomes of combined cardiac surgery and major pulmonary resection in series reporting ten or more patients
Author Year Patients Lung cancer Cardiac operations Operative mortality Major morbidity
Ulincy et al. 1992 10 5 CABG (7), AVR (1), CABG + MVR (2) 1 (10 %) 8 (80 %)
Brutel de la Riviere et al. 1995 79 79 CABG (72), AVR (5), CABG + AVR (2) 5 (6.3 %) 12 (15 %)
Patane et al. 2002 11 9 CABG (6), AVR (2), MVR (1), AVR + MVR (1), 0 0
myxoma resection (1)
Cathenis et al. 2009 27 27 CABG 922), valve (2), valve + CABG (3) 0 16 (59 %)
Chapter 34 · Combined Cardiac and Thoracic Surgery
1039 34
evaluating the potential benefit or harm have been more advanced lung cancer at the time of treat-
performed. It is likely that such combined opera- ment. Therefore, the conclusions from this iso-
tions represent much less than 5 % of all major lated study need confirmation in larger clinical
lung resections for cancer. There is a trend toward experiences.
somewhat increased operative mortality for com-
bined operations compared to cardiac surgical
procedures alone. It is not possible to reliably 34.6 Conclusions
determine whether long-term cancer-specific sur-
vival is related to whether the lung resection was Combined cardiac and pulmonary operations
performed in a staged or synchronous manner. are uncommon. A number of theoretical con-
Survival data for combined operations are scarce cerns exist regarding the adverse effects of com-
and are not reported according to clinical stage, so bining the operations, but the data regarding
direct comparisons are not possible. One retro- these concerns are limited and few clear conclu-
spective observational study reported no differ- sions can be drawn. No general agreement exists
ence in long-term survival for staged procedures as to which portion of the operation should be
compared to combined procedures (Voets et al. done first, whether the lung resection should be
1997), whereas another study from the same time performed on or off bypass, whether performing
period reported significantly better 5-year sur- the cardiac operation off pump is advantageous
vival for pathologic stage I patients who had a and whether performing the lung resection
staged resection compared to those who under- through a separate incision provides any advan-
went combined resection, 100 % vs 36.5 % (Miller tage. There appears to be an increase in both
et al. 1994). operative mortality and surgical morbidity for
It is unclear from the literature whether, when combined operations compared to isolated car-
a combined approach is used, a subspecialist in diac and thoracic operations. Staged, rather than
thoracic surgery performs the lung resection or combined, surgery may provide a cancer-specific
whether this is done by the cardiothoracic sur- survival benefit.
geon who is also performing the cardiac opera-
tion. Recent findings suggest that subspecialist
thoracic surgeons perform a more oncologically References
appropriate operation for lung cancer when per-
formed as an isolated operation compared to gen- Ahmed AA, Sarsam MA (2001) Off-pump combined coro-
eral and cardiac surgeons (Farjah et al. 2009; Ellis nary artery bypass grafting and left upper lobectomy
et al. 2011). Morbidity and mortality for major through left posterolateral thoracotomy. Ann Thorac
Surg 71(6):2016–2018
lung resection are lower for thoracic surgeons
Akchurin RS, Davidov MI, Partigulov SA, Brand JB, Shiriaev
than for cardiac surgeons and general surgeons AA, Lepilin MG, Dolgov IM (1997) Cardiopulmonary
(Schipper et al. 2009). Survival after lung cancer bypass and cell-saver technique in combined onco-
resection is also better when surgery is performed logic and cardiovascular surgery. Artif Organs
by a thoracic surgeon than a cardiothoracic or 21(7):763–765
Boldt J, Kumle B, Papsdorf M, Hempelmann G (1998) Are
general surgeon (Farjah et al. 2009). How these
circulating adhesion molecules specifically changed in
findings apply to a combined cardiac and pulmo- cardiac surgical patients? Ann Thorac Surg 65(3):
nary operation is unclear. 608–614
Despite the theoretical risk that extracorpo- Brutel de la Rivière A, Knaepen P, Van Swieten H,
real circulation will increase metastatic potential, Vanderschueren R, Ernst J, Van den Bosch J (1995)
Concomitant open heart surgery and pulmonary
this has not been borne out reliably in the litera-
resection for lung cancer. Eur J Cardiothorac Surg
ture. In fact, one retrospective observational 9(6):310–314
study demonstrated improved long-term all- Cathenis K, Hamerlijnck R, Vermassen F, Van Nooten G,
cause survival in patients undergoing combined Muysoms F (2009) Concomitant cardiac surgery and
coronary revascularization on CPB and lung can- pulmonary resection. Acta Chir Belg 109(3):306–311
Chen Q, Ge F, Cui W, Wang F, Yang Z, Guo Y, Li L, Bremner
cer resection compared to those undergoing
RM, Lin PP (2013) Lung cancer circulating tumor cells
revascularization without CPB (Schoenmakers isolated by the EpCAM-independent enrichment strat-
et al. 2007). However, the off-pump group in this egy correlate with Cytokeratin 19-derived CYFRA21–1
study was significantly older and had somewhat and pathological staging. Clin Chim Acta 419C:57–61
1040 M.K. Ferguson
D’Andrilli A, Venuta F, Rendina EA (2012) The role of lymph- Nwogu CE, Groman A, Fahey D, Yendamuri S, Dexter E,
adenectomy in lung cancer surgery. Thorac Surg Clin Demmy TL, Miller A, Reid M (2012) Number of lymph
22(2):227–237 nodes and metastatic lymph node ratio are associated
Danton MH, Anikin VA, McManus KG, McGuigan JA, with survival in lung cancer. Ann Thorac Surg
Campalani G (1998) Simultaneous cardiac surgery 93(5):1614–1620
with pulmonary resection: presentation of series and O’Flaherty JD, Gray S, Richard D, Fennell D, O’Leary JJ,
review of literature. Eur J Cardiothorac Surg 13(6):667– Blackhall FH, O’Byrne KJ (2012) Circulating tumour
672 cells, their role in metastasis and their clinical utility in
De Leyn P, Lardinois D, Van Schil P, Rami-Porta R, Passlick B, lung cancer. Lung Cancer 76(1):19–25
Zielinski M, Waller D, Lerut T, Weder W, ESTS (2007) Okumura Y, Tanaka F, Yoneda K, Hashimoto M, Takuwa T,
European trends in preoperative and intraoperative Kondo N, Hasegawa S (2009) Circulating tumor cells in
nodal staging: ESTS guidelines. J Thorac Oncol pulmonary venous blood of primary lung cancer
2(4):357–361 patients. Ann Thorac Surg 87(6):1669–1675
Dyszkiewicz W, Jemielity M, Piwkowski C, Kasprzyk M, Patanè F, Verzini A, Zingarelli E, di Summa M (2002)
Perek B, Gasiorowski L, Kaczmarek E (2008) The early Simultaneous operation for cardiac disease and lung
and late results of combined off-pump coronary cancer. Interact Cardiovasc Thorac Surg 1(2):69–71
artery bypass grafting and pulmonary resection in Pintar T, Collard CD (2003) The systemic inflammatory
patients with concomitant lung cancer and unstable response to cardiopulmonary bypass. Anesth Clin N
coronary heart disease. Eur J Cardiothorac Surg 34(3): Am 21(3):453–464
531–535 Rao V, Todd TR, Weisel RD, Komeda M, Cohen G, Ikonomidis
Ellis MC, Diggs BS, Vetto JT, Schipper PH (2011) JS, Christakis GT (1996) Results of combined pulmo-
Intraoperative oncologic staging and outcomes for nary resection and cardiac operation. Ann Thorac Surg
lung cancer resection vary by surgeon specialty. Ann 62(2):342–347
Thorac Surg 92(6):1958–1964 Rosalion A, Woodford NW, Clarke CP, Buxton B (1993)
Farjah F, Flum DR, Varghese TK Jr, Symons RG, Wood DE Concomitant coronary revascularization and resection
(2009) Surgeon specialty and long-term survival after of lung cancer. Aust N Z J Surg 63(5):336–340
pulmonary resection for lung cancer. Ann Thorac Surg Schipper PH, Diggs BS, Ungerleider RM, Welke KF (2009)
87(4):995–1006 The influence of surgeon specialty on outcomes in
Hasegawa S, Otake Y, Bando T, Cho H, Inui K, Wada H (2002) general thoracic surgery: a national sample 1996 to
Pulmonary dissemination of tumor cells after 2005. Ann Thorac Surg 88(5):1566–1573
extended resection of thyroid carcinoma with cardio- Schoenmakers MC, van Boven WJ, van den Bosch J, van
pulmonary bypass. J Thorac Cardiovasc Surg Swieten HA (2007) Comparison of on-pump or off-
34 124(3):635–636
Johnson JA, Landreneau RJ, Boley TM, Haggerty SP, Hattler
pump coronary artery revascularization with lung
resection. Ann Thorac Surg 84(2):504–509
B, Curtis JJ, Hazelrigg SR (1996) Should pulmonary Shahian DM, O’Brien SM, Filardo G, Ferraris VA, Haan CK,
lesions be resected at the time of open heart surgery? Rich JB, Normand SL, DeLong ER, Shewan CM,
Am Surg 62(4):300–303 Dokholyan RS, Peterson ED, Edwards FH, Anderson RP,
Kauffmann M, Krüger T, Aebert H (2013) Surgery on extra- Society of Thoracic Surgeons Quality Measurement
corporeal circulation in early and advanced non-small Task Force (2009) The Society of Thoracic Surgeons
cell lung cancer. Thorac Cardiovasc Surg 61(2): 2008 cardiac surgery risk models: part 1—coronary
103–108 artery bypass grafting surgery. Ann Thorac Surg 88(1
Köksal C, Sarikaya S, Zengin M, Atasalihi A (2002) Combined Suppl):S2–S22
off-pump coronary revascularization and lung resec- Ulicny KS Jr, Schmelzer V, Flege JB Jr, Todd JC, Mitts DL,
tion. Acta Med (Hradec Kralove) 45(3):119–121 Melvin DB, Wright CB (1992) Concomitant cardiac and
La Francesca S, Frazier OH, Radovancĕvić B, De Caro LF, pulmonary operation: the role of cardiopulmonary
Reul GJ, Cooley DA (1995) Concomitant cardiac and bypass. Ann Thorac Surg 54(2):289–295
pulmonary operations for lung cancer. Tex Heart Inst Vallejo R, Hord ED, Barna SA, Santiago-Palma J, Ahmed S
J 22(4):296–300 (2003) Perioperative immunosuppression in cancer
Lardinois D, De Leyn P, Van Schil P, Porta RR, Waller D, patients. J Environ Pathol Toxicol Oncol 22(2):139–146
Passlick B, Zielinski M, Lerut T, Weder W (2006) ESTS Voets AJ, Joesoef KS, van Teeffelen ME (1997) Synchronously
guidelines for intraoperative lymph node staging in occurring lung cancer (stages I–II) and coronary artery
non-small cell lung cancer. Eur J Cardiothorac Surg disease: concomitant versus staged surgical approach.
30(5):787–792 Eur J Cardiothorac Surg 12(5):713–717
Miller DL, Orszulak TA, Pairolero PC, Trastek VF, Schaff HV Yokoyama T, Derrick MJ, Lee AW (1993) Cardiac operation
(1994) Combined operation for lung cancer and car- with associated pulmonary resection. J Thorac
diac disease. Ann Thorac Surg 58(4):989–994 Cardiovasc Surg 105(5):912–917
Ng CS, Wan S (2012) Limiting inflammatory response to Young R, Pailler E, Billiot F, Drusch F, Barthelemy A, Oulhen
cardiopulmonary bypass: pharmaceutical strategies. M, Besse B, Soria JC, Farace F, Vielh P (2012) Circulating
Curr Opin Pharmacol 12(2):155–159 tumor cells in lung cancer. Acta Cytol 56(6):655–660
1041 35
Pulmonary Embolectomy
and Pulmonary
Thromboendarterectomy
Stein Iversen
References – 1057
flow imaging (CFI, also: color flow Doppler imag- high (up to 90 %). Hemodynamic and respiratory
ing - CD sonography) is concordant with the result support is required when hypotension or even
of CT venography (van Belle et al. 2006). shock are present. Aggressive volume expansion
Multi-detector computed tomography has may further impair right ventricular function by
replaced conventional angiography as «gold stan- causing additional mechanic overstretch, tricus-
dard» of radiologic imaging of the pulmonary pid regurgitation, and further loss of contractility.
vasculature in routine clinical practice. The visu- Modest fluid support, however, may improve car-
alization of the pulmonary arteries is adequate up diac index. Positive inotropic drugs should be
to the segmental level, and the detection of administered to improve contractility and main-
thrombi suffices as evidence of pulmonary embo- tain adequate coronary perfusion. No clinical
lism. The large PIOPED II (Prospective data are available on noradrenaline (norepineph-
Investigation of Pulmonary Embolism Diagnosis) rine) in pulmonary embolism; dobutamine
study observed a sensitivity of 83 % and a specific- improves cardiac index but may cause systemic
ity of 96 % (van Belle et al. 2006). Additionally, vasodilation and hypotension. Adrenaline (epi-
evaluation of right ventricular size and strain is nephrine) combines the beneficial properties of
feasible, and a CT venogram decides the status of drugs mentioned and exerts beneficial effects in
deep vein thrombosis (Goldhaber 2005; Loud shock patients (Torbicki et al. 2008). Systemic
et al. 2005; Perrier et al. 2005; Stein et al. 2004). administration of vasodilators may be detrimen-
However, due to the relatively high overall exami- tal due to systemic hypotension, but may be ben-
nation radiation, the value of CT venography is eficial when inhaled. For respiratory support by
debated in spite of diagnostic accuracy and should mechanical ventilation, it is important to avoid
be used selectively (Torbicki et al. 2008). excessive increase in intrathoracic pressure as it
Magnetic resonance imaging is noninvasive may further compromise right ventricular func-
and provides reliable diagnostic data. The method tion. Therefore, high end-inspiratory pressures
is time consuming, expensive, and not always and PEEP are avoided.
available. Especially for unstable patients with Occasionally, extracorporeal membrane oxy-
high-risk status, this diagnostic option is not genation (see 7 Chapter «Extracorporeal Circulation
suited (Madini and Jamieson 2003). and Myocardial Protection in Adult Cardiac Surgery»,
Sect. 6.3) may be necessary at times extended to
35 mechanical circulatory support (7 Chapter
In patients with suspected high-risk pulmo- «Cardiac Assist Devices and Total Artificial Heart»,
nary embolism, i.e., presenting with shock or Sect. 38.4.3), followed by thrombolysis or surgery.
hypotension, diagnostic measures must be Anticoagulant therapy is mandatory in patients
kept at a minimum, either after echocardiog- with pulmonary embolism, and immediate full
raphy alone, or, if allowed for by stabilized heparin administration should even be consid-
hemodynamics, after a pulmonary CT, imme- ered before definite diagnosis of pulmonary
diate therapeutic action (thrombolysis or sur- embolism is confirmed. Unfractionated heparin
gical embolectomy) is imperative. given intravenously and low molecular weight
heparin and fondaparinux (Factor XA Inhibitor)
administered subcutaneously are all equally effec-
tive and approved for treatment of low-risk pul-
35.1.3 Medical Treatment monary embolism and the latter two generally
and Surgical Therapy preferred due the easier application modus
(Konstantinides 2006; Kucher and Goldhaber
All patients with suspected significant pulmonary 2005). Unfractionated heparin is applied in
embolism should immediately be transferred to patients with high risk of bleeding, in renal fail-
the ICU. Acute right ventricular failure and subse- ure, and/or in high-risk pulmonary embolism.
quent shock, which is the leading cause of mortal-
ity, may be imminent, and rapid diagnosis 35.1.3.1 Thrombolytic Therapy
followed by supportive therapy is imperative. Thrombolysis is the treatment of choice in high-
About 40–50 % of patients with acute pulmonary risk patients with cardiogenic shock and persistent
embolism have right heart strain. If this leads to hypotension due to massive pulmonary embolism
circulatory instability, mortality is exorbitantly (Jerjes-Sanchez et al. 1995).
Chapter 35 · Pulmonary Embolectomy and Pulmonary Thromboendarterectomy
1045 35
Recent major trauma/surgery/head injury within Urokinase 4,400 IU/kg bolus over 10 min,
preceding 3 weeks followed by 4,400 IU/kg/h over
12–24 h
Gastrointestinal bleeding within preceding 1 month
Accelerated regimen: 3 mill IU
Active bleeding over 2 h
Pregnancy or within 1 week postpartum Heparin is infused concurrently with rtPA administra-
tion only, with urokinase and streptokinase afterward
Non-compressible punctures From: Torbicki et al. (2008); used with permission
Traumatic resuscitation
thrombolytic therapy versus heparin only revealed 55 Massive right ventricular failure and absolute
no significant difference in outcome (Konstantinides contraindication to thrombolytic therapy
et al. 2002; Wan et al. 2004; Dong et al. 2006), but 55 Hemodynamic instability and imminent
Konstantinides found a significant higher degree of circulatory failure in spite of ongoing
clinical deterioration requiring secondary (rescue) thrombolytic treatment
thrombolysis in the heparin-only group. In patients
with massive pulmonary embolism and hemody- Furthermore, patients with floating (transit)
namic instability, thrombolysis is associated with intracardial thrombi and/or patent foramen ovale
lower mortality rates than the heparin group (6.2 % should primarily be considered for surgical removal.
vs. 12.7 %) but without statistical significance (Wan In this group mortality is particularly high, 80–100 %
et al. 2004). It is accepted that randomized studies when left untreated. With thrombolytic therapy,
cannot be obtained. Therefore, although not veri- mortality is still beyond 20 % as the intracardiac
fied, thrombolytic treatment has become a guide- thrombus does not always dissolve in situ, but may
line-based standard of care. Whether thrombolytic disperse and migrate to the pulmonary vascular bed
therapy is effective for chronic thromboembolic (Chartier et al. 1999; Torbicki et al. 2003). However,
pulmonary hypertension or for preventing its aggressive interventionists see no contraindication
occurrence after acute pulmonary embolism in this constellation and have reported good results
remains unclear (Meneveau et al. 2003). with low mortality (Rose et al. 2002). In the pres-
Interventional percutaneous techniques to ence of a patent foramen ovale, hospital mortality is
reopen the central pulmonary arteries can be significantly higher (33 % vs. 14 %). Atrial shunts
applied either in adjunct to ongoing thrombolysis may be easily detected during the primary diagnos-
or as an alternative to lytic therapy or surgical tic procedures and the finding must be considered
embolectomy when these measures are absolutely in the decision-making of therapy; especially in
contraindicated. Various techniques are applied event of «paradoxical» emboli, morbidity and
either for removal (i.e., Greenfield suction cathe- mortality are increased (Chartier et al. 1999;
ter) or fragmentation and dispersion of thrombo- Konstantinides et al. 1998; Rose et al. 2002). Patients
emboli utilizing conventional or specially with subacute pulmonary embolism (older than
designed catheters. By fragmentation a greater 5–7 days), in whom the thromboembolic debri has
contact area for the lytic agent is achieved migrated into the segmental arteries and systemic
organization and break down of clot has begun, are
35 (Uflacker et al. 1996, Uflacker 2004). Its applica-
poor candidates for embolectomy, as the removal of
tion should be limited to the central arteries to
avoid perforation and terminated as soon as hemo- clot may be incomplete or impossible. Likewise,
dynamic improvement is attained. Hemodynamic patients with recurrent pulmonary embolism
improvement can be substantial and lifesaving, superimposed on long-standing history of dyspnea
and various good results have been described, but and pulmonary hypertension should not undergo
definite evaluations in larger trials are pending pulmonary embolectomy, but should be evaluated
(Greenfield et al. 1969; Kucher et al. 2005; Brady for pulmonary thromboendarterectomy.
et al. 1991; Chamsuddin et al. 2008).
Heat exchanger
CO2
O2
Oxygenator
35
..Fig. 35.1 Longitudinal incision of the pulmonary artery in the main stem. Grasping and extraction of a large
thromboembolus with a bile duct clamp. Insert: removal of peripheral embolic material by suction
absence of shock, mortality varied between 6 and nary embolectomy is set at different levels in
8 % (Aklog et al. 2002; Leacche et al. 2005; different institutions. The primary goal of the
Meneveau et al. 2006). Furthermore, patient age, operative procedure performed in an emergency
duration of symptoms, and number of episodes of situation on moribund patients is to prevent acute
pulmonary embolism influence results. The wide death from circulatory collapse. Any conventional
range of mortality rates reported reflects the inho- statistical analysis of results does not apply in
mogeneity of the investigated patients with the these situations. If the patient is already in shock,
diagnosis massive or fulminant pulmonary embo- mortality will be high. Working against long odds,
lism. It also reflects that the indication for pulmo- each single success will corroborate the method.
Chapter 35 · Pulmonary Embolectomy and Pulmonary Thromboendarterectomy
1049 35
35.2 Chronic Thromboembolic Symptoms in patients with pulmonary hyper-
Pulmonary Hypertension tension are mostly nonspecific, and the early
and Pulmonary course of the disease is insidious and correct diag-
Thromboendarterectomy nosis is often missed in the early stage. The most
ostensible symptom is progressive dyspnea upon
35.2.1 Clinical Presentation exertion—in advanced stages, signs of right heart
failure, arterial hypoxemia, and cyanosis become
Chronic pulmonary hypertension caused by pul- evident. Up to two-third of patients with chronic
monary embolism is a rare disease. Approximately thromboembolic pulmonary hypertension have
4 % of patients experiencing a pulmonary embo- no evident history of acute pulmonary embolism
lism develop pulmonary artery hypertension (Lang 2004). In the daily practice, there is much
within 2 years after the embolic event (Pengo et al. uncertainty, lack of knowledge, and controversial
2004; Tapson and Humbert 2006). opinions in regard to indication for surgery,
It has been estimated that only 0.1 % of all pul- patient selection, and timing of surgery. Much too
monary embolism patients require surgery for often, patients are referred to surgery in advanced
chronic thromboembolic hypertension (Fedullo stages as a last resort after years of misdiagnoses
et al. 2000). Left untreated, the prognosis in these and/or palliative medical treatment (Auger et al.
patients is poor and correlates with the degree of 1999; Riedel 2003; Thistlethwaite et al. 2002).
elevation in right ventricular afterload and pul-
monary pressures. An older analysis showed that
the 5-year survival rate was 30 % when the mean 35.2.2 Pathophysiology
pulmonary artery pressure exceeded 30 mmHg,
and merely 10 % at mean pressures was measured In acute pulmonary embolism, the intraluminal
beyond 50 mmHg. On the contrary, if mean pul- obstruction of the pulmonary vessels by fresh
monary artery pressures were found below thromboemboli can be easily mobilized. In
30 mmHg, the survival rate was nearly 90 % chronic pulmonary embolism, the thromboem-
(Riedel et al. 1982). bolic mass has become organized, i.e., incorpo-
In general, pulmonary artery hypertension of rated into the vascular intimal layer, altered into
various etiologies remains a chronic disease with- fibrous and elastic tissue, and endothelialized. The
out definite cure. In spite of recent advances, med- pulmonary arteries reveal characteristic features of
ical treatment is at large a palliative therapeutic intimal thickening, membranous occlusions, intra-
option (Bresser et al. 2006; Galiè et al. 2004; luminal webs and bands, and stenoses (Wagenvoort
Ghofrani et al. 2003). The mortality of patients and Wagenvooort 1977). The central arteries
treated medically—dependent upon the degree of may—but not obligatory—be filled with intralumi-
pulmonary artery pressure elevation—amounted nal appositional thrombi of various ages.
to 31.5 % at 18 months at the beginning of this Fundamental for the progression of pulmonary
century (Lewczuk et al. 2001). But modern drug hypertension once established is the development
therapy—today approximately ten different drugs of a precapillary vasculopathy mainly in the still
are in use alone and in combinations—has man- perfused vascular bed. By mechanisms not yet
aged to reduce mortality by 43 % (Galiè et al. clearly understood, probably by relative hyperper-
2009). fusion, the small vessels restrict, undergo a media
Pulmonary thromboendarterectomy is a sur- hypertrophy, and become sclerotic. In the end, the
gical desobliteration of the obstructed pulmonary vascular pathology of chronic thromboembolic
artery vessels that has been established for pulmo- pulmonary hypertension presents as a mixed pic-
nary hypertension caused by chronic persistent or ture of mechanically obstruction at the stem, lobar,
recurrent pulmonary embolism (Archibald et al. and (sub)segmental level which may be surgically
1999; Daily et al. 1999; D’Armini et al. 2000; removed and in the end stage an irreversible vascu-
Iversen 1994; Jamieson et al. 2003). Thus, chronic lopathy in the primarily nonobstructed vessels.
thromboembolic pulmonary hypertension has The degree of small vessel vasculopathy signifi-
become a surgically manageable form of pulmo- cantly determines the surgical outcome (Galiè and
nary hypertension with the potential of curative Kim 2006; Moser and Bloor 1993; Timby et al.
treatment. 1990; Yi et al. 2000). The degree of vascular
1050 S. Iversen
obstruction and the level of precapillary vasculop- sion capacity (diffusion lung capacity for carbon
athy are individual, highly variable, and time monoxide (DLCO)) is commonly found
dependent. Thus, the degree of pulmonary hyper- decreased, and 20 % of the patients present with
tension, the course of the disease, the patient’s con- reduction of vital capacity below 80 % due to
dition at time of diagnosis, and the angiographic parenchymal scarring. The pulmonary function is
findings are different in each case. The progression otherwise normal in the majority of patients
of vascular changes leads to a gradual increase of (Fischer et al. 1995; Moser et al. 1990).
right ventricular afterload which at first is compen-
sated by right ventricular hypertrophy, and cardiac 35.2.3.2 Cardiac Diagnostic
output remains normal. With time, however, right Transthoracic echocardiography will document
ventricular dysfunction and failure become evi- right ventricular strain with hypertrophy and dil-
dent. The clinical condition worsens with parallel atation and the presence of pulmonary hyperten-
progressive hypoxemia due to reduced pulmonary sion at rest. Ventricular contractility as well as
gas exchange and decreased low cardiac output valvular function of both cardiac chambers may
leading to global cardiopulmonary failure. be determined and rule out left ventricular dys-
function as a cause of pulmonary hypertension.
There will be a variable degree of right atrial and
35.2.3 Diagnostics ventricular dilation, tricuspid regurgitation, and a
leftward shift of the interventricular septum. A
All diagnostic procedures aim at establishing the progressive right and in later stages, also left ven-
correct diagnosis of thromboembolic pulmonary tricular dysfunction may be diagnosed. A pericar-
hypertension and stage of the disease, differenti- dial effusion may be seen, and in many instances
ating from other forms of pulmonary hyperten- high right atrial pressures may reopen a previ-
sion, and above all evaluating the possibility of ously closed interatrial communication and a pat-
and indication for surgical intervention. ent foramen ovale may be demonstrated by contrast
or color echocardiography (Menzel et al. 2000).
35.2.3.1 Basic Diagnostic Procedures Invasive cardiac evaluation and coronary angi-
The general diagnostic work-up in patients pre- ography are generally required in patients with
senting with dyspnea on exertion must consider chronic thromboembolic pulmonary hyperten-
35 all differential diagnoses. ECG, chest x-ray exami- sion, especially if the patient is more than 40 years
nation, arterial blood gas analysis, and pulmonary old or in the presence of risk factors. Response to
function tests will help rule out other causes of vasodilator therapy such as sildenafil or nitric
dyspnea and focus on pulmonary hypertension. oxide inhalation may be tested. Right heart cath-
ECG may show right heart strain through axis eterization is mandatory and must include record-
deviation, a p-pulmonale, and specific changes of ing of all right-sided pressures, determination of
the QRST segment. X-ray findings in chronic right and left side oxygen saturations, as well as
thromboembolic pulmonary hypertension may cardiac output and calculation of pulmonary vas-
be absent or discrete, but full and large pulmo- cular resistance to assess the hemodynamic
nary hili, right heart enlargement, and sparse, impairment. The correct measurement of pulmo-
irregular, pulmonary, vascular markings with nary wedge pressure may be difficult to achieve if
clear lung fields are suggestive. Signs of peripheral measured in a thrombotic obstructed vessel. Thus,
scarring from previous infarction may be seen. repeated measurements at different sites or inclu-
The arterial blood gas analysis may be normal or sion of LV end diastolic pressures may be neces-
show a mild hypocapnia at normal oxygen value sary to correctly obtain pulmonary vascular
also in significant pulmonary hypertension. resistance. Left ventricular catheterization and
Hypercapnia is generally associated with obstruc- coronary angiography provide evidence of valvu-
tive or interstitial lung disease. A decline in oxy- lar and/or coronary heart disease and indicate
gen level at rest occurs in advanced stages of whether concomitant surgery at time of pulmo-
pulmonary hypertension or becomes unmasked nary endarterectomy will be required.
with exertion. Pulmonary function tests must be Radioisotope ventilation and perfusion scan is
performed to rule out severe obstructive or helpful to establish the diagnosis of chronic
fibrotic lung disease. The alveolar–arterial diffu- thromboembolic pulmonary hypertension. The
Chapter 35 · Pulmonary Embolectomy and Pulmonary Thromboendarterectomy
1051 35
typical mismatch found with a normal ventilation Schwickert et al. 1994). The investigation is less
scan demonstrates segmental perfusion defects precise at the segmental level and the diagnostic
with high sensitivity and specificity, but may also value limited (Auger et al. 1999; Bergin et al.
highly underestimate its extent and localization of 1997; Pitton et al. 2002).
obstructions and therefore play only a minor role The immaculate imaging of both central and
when assessing operability. distal pulmonary vasculatures is a conditio sine qua
non because the exact location of chronic throm-
35.2.3.3 Radiologic Imaging boembolic vascular changes and the prediction of
Pulmonary angiography in the form of digital sub- possibility for surgical removal of obstructions and
traction angiography combined with multi-slice CT consecutive reduction of right ventricular after-
scan remains the «gold standard» for imaging of the load are crucial. Profound knowledge of the patho-
pulmonary arteries and definition of the pathomor- morphologic changes of the pulmonary vasculature
phologic anatomy. It will differentiate the chronic in chronic thromboembolic pulmonary hyperten-
thromboembolic pulmonary hypertension from sion is important to understand and correctly
that of other etiologies, help determine the location interprets the radiologic findings. Extensive
of obstructions, and assess the surgical accessibility thromboembolic masses and vascular changes
and thus technical operability. centrally in the major pulmonary arteries are char-
Pulmonary angiography should be performed acterized mainly by a mechanical obstruction with
biplane with the advantage of a lateral view and sparse antegrade perfusion. The secondary small
with separate contrast injections of the left and vessel disease and reactive hypertension may be
right side. The investigation is safe also in patients expected to be less pronounced and the prospect
with severe pulmonary hypertension when per- of surgical success auspicious. Solely distally
formed in an experienced setting. Characteristic located thromboembolic obstructions are associ-
angiographic findings may include various filling ated with a significantly higher operative mortality
defects, abrupt vessel narrowing or cutoff, intimal (Auger et al. 1999; McGoon et al. 2004; Oikonomou
irregularities with pouching, or intraluminal webs et al. 2004). Moreover, distal occlusions with time
and bands, or the vessels will be completely and in advanced stages of the disease coincide with
thrombosed with total occlusion at the segmental a higher degree of secondary reactive vascular
level or of the larger branches (Auger et al. 1992). changes and progressive pulmonary hypertension.
Contrast-enhanced multi-slice chest CT is In cases with long-standing thromboembolic pul-
often used primarily in the imaging of patients monary hypertension over many years, the angio-
presented for surgical evaluation. The fast acqui- graphic findings of thromboembolic obstruction
sition of images in a single-breath technique is may become discrete and do not correlate with the
less invasive, advantageous, and safe in this often advanced elevation of pulmonary vascular resis-
very sick patient group. CT findings consist of tance. With excessively elevated pulmonary vascu-
chronic thromboembolic material within the lar resistance and the presence of frank right heart
pulmonary arteries often lining the vessels failure, the operative risk will be very high and the
eccentrically, enlargement of the central arteries, patients finally become inoperable.
as well as variability in the distribution and size
of peripheral pulmonary branches. Typically, in
the parenchymal «window,» a mosaic pattern 35.2.4 Surgical Indications
reflecting the inhomogeneous perfusion (along and Operative Techniques
with underperfused areas with low attenuation)
is seen. Chest CT scan will also reveal parenchy- 35.2.4.1 Indications and Patient
mal defects from prior infarctions, concomitant Selection
fibrous or emphysematous lung disease, obstruct- Prerequisite for successful surgery is careful
ing tumors, or mediastinal fibrosis. Contrast- patient selection. All patients with the diagnosis of
enhanced thoracic CT alone will determine thromboembolic pulmonary hypertension should
thromboembolic pulmonary hypertension in all primarily be considered surgical candidates.
patients and predict technically operability with Pulmonary endarterectomy is indicated if pul-
a specificity of 77 % and sensitivity of 100 % monary vascular resistance at rest exceeds
(Bergin et al. 1996, 2000; Castañer et al. 2009; 300 dyn·s·cm−5 (Daily et al. 1999; Iversen et al. 1992;
1052 S. Iversen
Jamieson et al. 1993, 2003; more recent literature surgery—in dubio pro operatione—be offered
does not add to current knowledge). A few after extensive and detailed patient information.
patients may present with less elevated pulmo- More recently, excellent results were reported for
nary vascular resistance although they have a distal chronic thromboembolic pulmonary
complete central, but only one-sided, occlusion. hypertension patients also (D’Armini et al. 2014).
They suffer from significant dyspnea upon exer- The presence of parenchymal lung disease is a
tion due to death space ventilation and also strong contraindication for surgery, as this condi-
should be considered surgical candidates. Another tion is associated with an increased postoperative
subgroup of patients in the early course of the dis- morbidity and mortality due to respiratory failure.
ease will reveal pathologically elevated pressures Perivascular inflammatory reactions which
only during physical stress. Considering the may complicate or frankly render thromboembo-
chronic course with progression of pulmonary lectomy impossible must be expected after septic
hypertension due to reactive vasculopathy, it pulmonary emboli. In patients with subacute pul-
seems expedient to offer early surgery to these monary embolism, surgery should be postponed
patients in the absence of contraindications. A for at least 3 months to allow for completion of
complete restitutio ad integrum is only possible the organization process of the thromboembolic
with removal of the thromboembolic obstruc- material. Concomitant heart procedures such as
tions in the early stages of the disease. In advanced myocardial revascularization or valve interven-
stages, however, pulmonary thromboendarterec- tions can be performed simultaneously.
tomy may be too late because the progressive Pulmonary thromboendarterectomy is tech-
increase of pulmonary resistance is associated nically possible if thromboembolic vascular wall
with a decrease of cardiac output. This condition changes are indentified at or proximal to the lobar
has proven significant for both the surgical risk arteries. With this criterion fulfilled, further
and the long-term success of the procedure peripherally located vascular changes are irrele-
(Iversen et al. 1992; Jamieson et al. 2003; Dartevelle vant. As a prerequisite of technical operability, the
et al. 2004; Corsico et al. 2008). Decision for sur- surgical intimal dissection must start centrally, but
gical treatment is based upon the vascular will then extend into the subsegmental arteries.
obstructions deemed accessible for removal and a Solely peripheral, i.e., at the (sub)segmental level
significant (i.e., >50 %) reduction of right ventric- located, thromboembolic vascular lesions are
35 ular afterload predicted. The decision-making only marginally or non-accessible to desoblitera-
depends upon the accuracy of diagnostics and its tion. Surgery limited to these segments carries the
interpretation and is strongly influenced by the hazard of incomplete removal, inadequate
experience of the surgical team (Bergin et al. decrease in pulmonary vascular resistance, and
1996, 2000; Daily et al. 1989, 1999; Iversen et al. consecutive right heart failure postoperatively.
1993; Jamieson et al. 1993, 2003; Dartevelle et al.
2004). In patients in whom the thromboembolic 35.2.4.2 Operative Techniques
obstructions are mainly located in the central pul- The desobliteration of pulmonary arteries in
monary vessels, surgery should always be per- chronic thromboembolism is a true endarterec-
formed, because a significant reduction in tomy different from the techniques applied dur-
pulmonary vascular resistance can be expected. ing embolectomy for acute pulmonary embolism.
In turn, the solely peripheral manifestation of vas- The pathological intima must be extracted along
cular changes combined with year-long-standing with the intraluminal obstructions; the removal of
pulmonary hypertension and the presence of intraluminal thrombi alone will not clear the
frank right heart failure are associated with high underlying vascular obstructions. The operative
operative risk and deficient right ventricular techniques developed by Pat O. Daily have only
afterload reduction. Between these two extremes, been slightly modified over the last 20 years (Daily
the variety in clinical stages and pathological find- et al. 1989; Iversen et al. 1993; Jamieson et al.
ings is broad, and ultimately, the individual sur- 1993).
geon’s expertise will determine when to operate The surgical approach is via a median sternot-
which patient. In young patients, in particular, in omy with the use of extracorporeal circulation in
whom the long-term prognosis with any alternate deep hypothermia and periods of circulatory
therapy already is highly limited, should arrest. The heart is arrested with cardioplegia
Chapter 35 · Pulmonary Embolectomy and Pulmonary Thromboendarterectomy
1053 35
before the first period of circulatory arrest. Some during cooling to save operative time because,
basic principles and tricks applied during the from experience, most thrombotic material is
preparation of the procedure differ from the stan- found on the right side and the proximal sequence
dard heart operation and will facilitate the expo- of endarterectomy may here be performed with-
sure of the pulmonary arteries in the dorsal out the need of circulatory arrest. For this part of
pericardium. The pleural cavities remain closed the procedure, it is advantageous for the surgeon
during the procedure. The pericardium is incised to change to the left side of the table. The right pul-
in a wing-door fashion by mobilizing the pericar- monary artery is exposed either lateral or medial
dial flexion around the great vessels and the rims to the superior vena cava by placing a small (blunt)
fixed to the presternal tissue. The valves of the self-retaining retractor (e.g., type cone); vision is
sternal retractor are placed underneath to span facilitated using loupes and headlight. The inci-
and elevate the pericardial sac. The superior caval sion is begun proximal to the truncus anterior and
vein is mobilized entirely up to the confluence of extended into the intermediate stem carefully
the hemiazygos vein hereby dissecting the adhe- avoiding the orifice of the middle lobar artery.
sions between the right atrium and right Distally, the exposure may be improved by elevat-
pulmonary artery. This is followed by an incision ing the overlying upper pulmonary vein with a
of the pericardial flexion posterior and parallel to fine malleable (e.g., type «Marberger») vein retrac-
the phrenic nerve over the upper right pulmonary tor. After identification of the correct plane within
vein and the loose surrounding connecting tissue the pulmonary wall (i.e., lamina elastica interna),
divided. Hence, the ventral aspect of the truncus, the dissection is at first carried out circumferen-
and anterior, main, and intermediate stem of the tially in the central pulmonary artery and then
right pulmonary artery is exposed. It is recom- continued peripherally (. Fig. 35.2). The intimal
mended to perform this dissection before heparin cast of each segmental branch is mobilized and
administration; often dilated bronchial vessels, extracted, and the successful endarterectomy
lymph channels and engorged veins embedded in extends to the subsegmental level beyond the
fatty tissue are encountered, which demand subtle obstructions where the intimal layer is thin and
dissection and hemostasis. It is important to leave
the adventitia of the pulmonary arteries intact and
not dissect it, as the dilated artery wall will become Sucker tip dissector
quite thin and fragile after the endarterectomy.
For the same reason, subsequently only the adven-
titial tissue but never the artery wall or rims of
incision is grasped transmurally with forceps to
avoid lesions and bleeding. Following administra-
tion of heparin, the arterial cannulation is carried
out high in the ascending aorta or even in the arch
to improve pulmonary artery exposure. Bicaval
cannulation with tape snares around both veins is
obligatory for total venous return. Because of the
often massive collateral blood flow in the pulmo-
nary vessels via the bronchial arteries, vent cathe-
ters are placed both in the left ventricle over the
left atrium and in the pulmonary artery trunk.
Cooling of the patient is initiated immediately to a
core temperature of 18 °C with temperatures mea- V. cava superior
sured both for core (rectal or vesical) and the brain
(nasopharyngeal or tympanal). Cerebral protec- ..Fig. 35.2 Anatomic view via median sternotomy: the
tion is carried out under EEG monitoring and adventitia and the media layer of the right pulmonary
includes administration of barbiturates and pred- artery are incised from the level of the truncus anterior
into the intermediate stem at a length of 5 cm without
nisolone as well as additional topical cooling (ice- opening the intima. The intimal cast is circumferentially
packs or cooling helmet). The endarterectomy is mobilized before the dissection is continued
begun in the right pulmonary artery already peripherally
1054 S. Iversen
nonobstructive (. Fig. 35.3). This subtle anatomi- Circulatory arrest and exsanguination are rarely
cal dissection is considerably facilitated using spe- required for desobliteration of the upper lobe seg-
cially constructed, slim suction dissectors (Daily mental vessels. The incisions are closed with a
et al. 1992). double row of 6/0 Prolene running suture starting
In spite of these measures, accurate vision and distally. It’s advisable to use sutures with a small
precise endarterectomy are hindered by the pres- (CV) needle to minimize suture hole bleeding,
ence of severe collateral blood flow via the bron- and in the event of extreme fragile remnant pul-
chial arteries, and therefore, the peripheral monary wall after endarterectomy, the area may
desobliteration is best achieved in periods of be patched using autologous pericardium to
either reduced flow of extracorporeal circulation relieve strain from the suture row.
or complete exsanguination of the pulmonary The left pulmonary artery is then exposed by
vessels in deep hypothermic circulatory arrest. sharp and blunt dissection of the pericardial flex-
After initiation of circulatory arrest, the lungs are ion and mobilization of the crossing left upper
repeatedly hyperinflated by the anesthetist and pulmonary vein. The preparation is carried out
the patient’s blood drained into the reservoir via close to the vessels to avoid lesion to the phrenic
the venous and vent lines. With increased surgical nerve. The arteriotomy is made in the ventral
experience, circulatory arrest times exceeding aspect of the concavity into the pars basalis, avoid-
20 min for each lung side are rarely required. ing the take-off of the lingual artery (. Fig. 35.4).
When longer arrest times are necessary, intermit- The incision extends to the level of the crossing
tent whole body reperfusion is recommended. If upper lobe bronchus, with the intraluminal expo-
required, an additional incision may be made in sure being facilitated by stay sutures on both sides
the truncus anterior of the upper lobe when the of the incision and by retraction of the pulmonary
obstructions are located peripherally and cannot vein (e.g., Zenker retractor). The thromboembolic
be removed via the right pulmonary artery stem. accretion and intimal changes on the left side are
generally less distinct and often located in the lin-
gual and lower lobe artery. Centrally the intima
may appear normal, but may be elevated with the
use of sharp intimal dissector. Endarterectomy is
35
is well tolerated when cardiac output is increased, the reactive increase in cardiac output prevails,
but aggressive diuresis should be sustained by but it will further decline over time with regres-
maintaining a mean arterial pressure beyond sion of right ventricular hypertrophy (D’Armini
70 mmHg. Additional administration of furose- et al. 2000; Iversen et al. 1992; Madini and
mide is suggested to achieve an early negative fluid Jamieson 2003; Mayer et al. 1996; Moser et al. 1987).
balance. Hematocrit is kept at values >30 % also to In own material a reduction of pulmonary vascular
improve oxygen transport capacity. These mea- resistance >80 % (from mean 1045 to 194 dyn.s.cm−5)
sures serve to obviate the development of pulmo- was found (Iversen et al. 1992). This complies
nary reperfusion edema. Total negative fluid with data reported from others (Daily et al. 1999;
balance before extubation must consider the pre- Moser et al. 1987; Tscholl et al. 2001; Mayer et al.
operative status of the patient as well as the intra- 2011). The early relieve of right ventricular strain
operative balance and not solely depend upon may also be confirmed by echocardiography
postoperative fluid management, arterial blood gas (Dittrich et al. 1989). Normalization of right ven-
analyses, and chest x-ray examination under tricular volume, decline in tricuspid regurgita-
mechanical ventilation. tion, and paradoxical septal movement can be
seen already in the first postoperative days.
35.2.5.3 Anticoagulation and Cava In the early postoperative period, the pulmo-
Filter nary gas exchange remains impaired and lags
Patients undergoing pulmonary thromboendar- behind the hemodynamic convalescence. The
terectomy remain on lifelong anticoagulation regi- arterial blood gas analysis may even show worse
men to prevent recurrent pulmonary embolism or values compared to preoperative values and pro-
thrombus formation due to remnant obstructions longed continuous oxygen supplement be neces-
in the pulmonary vessels. In the absence of bleed- sary. However, oxygenation and gas exchange
ing, continuous intravenous heparin administra- enhance with improvement of ventilation–perfu-
tion is begun 6 h postoperatively (initially in «low» sion ratio after absorption of pulmonary edema,
dose—normal prothrombin time) and increased resolution of pulmonary infiltrates, and normal-
to therapeutic dose (PTT doubled to 60–80 s) on ization of pulmonary microcirculation (Kapitan
the first postoperative day. After mobilization on et al. 1990). Accordingly, further decrease of pul-
the normal ward, the overlapping therapy with monary vascular resistance can be expected in the
35 oral anticoagulants is affected. Although debated late postoperative course (Fadel et al. 2004; Hirsch
in the literature, as hard data are difficult to obtain et al. 1996; Iversen 1994; Moser et al. 1987). Final
in this patient group, an inferior vena cava filter statements concerning the degree of circulatory
device is inserted before discharge, if it not had and respiratory improvement after pulmonary
been placed preoperatively already. As various thromboendarterectomy can thus not be made
degrees of unresolved pulmonary artery hyper- until 6–12 months postoperatively.
tension will remain in some of the patients, the The attained operative results are generally
additional filter protection against recurrent sustained. Long-term follow-up reports reveal
embolism is prudent even in the absence of 6 years survival rate >75 %. The majority of the
thrombophlebitis residue in the lower body. At patients are classified into NYHA stage I or II
the most, the exception would be the definite ori- (Archibald et al. 1999; D’Armini et al. 2000; Mayer
gin of thromboembolus in the upper body (e.g., et al. 1996). The procedure may be curative in
pacemakers, port access devices, etc.) after exclu- individual patients, dependent upon the preoper-
sion of hereditary or acquired coagulopathies. ative status, whereas in cases with incomplete sur-
gical removal of peripherally located obstructions
residual pulmonary hypertension, persistence of
35.2.6 Results symptoms and compromised long-term progno-
sis may be expected in survivors. Also, a relapse in
The effectiveness of pulmonary thromboendar- the clinical condition and progression of pulmo-
terectomy correlates with the degree of pulmo- nary hypertension after temporarily postopera-
nary vessel recanalization and presents with an tive improvement are occasionally observed.
immediate decrease in pulmonary vascular resis- Reasons may be rethrombosis in the pulmonary
tance. The immediate reduction of pulmonary arteries due to inadequate anticoagulation or
artery pressures may be only moderate as long as presence of severe coagulopathy, but in particular
Chapter 35 · Pulmonary Embolectomy and Pulmonary Thromboendarterectomy
1057 35
due to progression of secondary vasculopathy in a major and independent risk factor for in-hospital
patients who presented to surgery at an advanced and 1-year mortality, and therefore the indication
stage of the clinical course. for surgery in patients with peripheral disease and
Repeat pulmonary thromboendarterectomy high pulmonary vascular resistance remains con-
may be possible in highly selected patients. In own troversial. Some authors propagate that all patients
experience also corresponding with literature (Mo are operable but they show some reluctance in the
et al. 1999), reoperations are nearly exclusively presence of risk factors (Jamieson et al. 2003;
limited to patients in whom the primary interven- Thistlethwaite et al. 2002). The definitely high
tion was inadequate and incomplete. Such patients operative risk in this patient subgroup must be
should better be reinvestigated and evaluated in evaluated in the light of limited alternative treat-
an experienced high-volume center, and the indi- ment modalities and life expectancy without
cation for surgery is set on a case-to-case basis. attempted thromboendarterectomy. Surely, high
Albeit it’s potential success, pulmonary throm- pulmonary vascular resistance (exceeding
boendarterectomy is associated with a high opera- 1500 dyn.s.cm−5) alone should not pose a contra-
tive mortality. Main causes of death are respiratory indication to surgery, but the individual patient’s
failure due to reperfusion edema, cardiac failure as findings rather be evaluated to predict outcome
a result of persistent pulmonary hypertension, or a (accessibility, duration of pulmonary hyperten-
combination of these two complications. sion, and thus potential operative reduction of
Perioperative morbidity is also high. In an interna- right ventricular afterload). For inoperable
tional registry (Mayer et al. 2011), nearly every sec- patients, bilateral lung transplantation may be an
ond patient suffered from one or more option when medical therapy fails. Due to limited
complications—e.g., pulmonary and other infec- donor organ availability, waiting-list pre-transplant
tions, persistent pulmonary hypertension, reperfu- mortality is as high as 30 %. Posttransplant survival
sion edema, neurologic and bleeding complications, at 3 months and 1 year is merely 76 % and 50 %,
and pericardial effusion. Yet, operative risk has respectively, in patients operated for idiopathic
decreased significantly over the past 20 years due to pulmonary hypertension. For patients surviving
increased experience in patient selection, operative the first year, half-life survival is 9.5 years (Long
techniques, and perioperative management. et al. 2011; Christie et al. 2011). Thus, transplanta-
Published results from various centers indicate an tion remains a palliative measure limited to inop-
operative mortality between 4.5 and 23.5 % erable patients, and no alternative to potentially
(D’armini et al. 2000; Doyle et al. 2004; Hartz et al. curative pulmonary thromboendarterectomy.
1996; Iversen 1994; Jamieson et al. 2003; Tscholl
et al. 2001)—in the international registry, overall
mortality amounted to 4.7 % (Mayer et al. 2011). References
This wide range in results reflects not only the vari-
able experience but rather also the diverse criteria Aklog L, Williams CS, Byrne JG et al. (2002) Acute pulmo-
in patient selection. Analyses of results reveal nary embolectomy; a contemporary approach.
patient age, level, and duration of pulmonary Circulation 105:1416–1419
hypertension at time of diagnosis as independent Archibald CJ, Auger WR, Fedullo PF (1999) Outcome after
pulmonary thromboendarterectomy. Semin Thorac
risk factors. There is a nearly linear relation between Cardiovasc Surg 11:164–171
preoperative pulmonary vascular resistance and Auger WR, Fedullo PF, Moser KM et al. (1992) Chronic
in-hospital mortality (Mayer et al. 2011). Thus, the major-vessel thromboembolic pulmonary artery
operative risk will reach levels of 40 % and above obstruction: appearance at angiography. Radiology
when patients in NYHA class IV and manifest right 183:393–398
Auger WR, Channick RN, Kerr KM et al. (1999) Evaluation of
heart failure are accepted for surgery (Hartz et al. patients with suspected chronic thromboembolic pul-
1996; Iversen 1994; Tscholl et al. 2001). monary hypertension. Semin Thorac Cardiovasc Surg
11:179–190
Bergin CJ, Rios G, King MA et al. (1996) Accuracy of high-
35.2.7 Persistent Pulmonary resolution CT in identifying chronic pulmonary throm-
boembolic disease. Am J Roentgenol 166:1371–1377
Hypertension Bergin CJ, Sirlin CB, Hauschildt JP et al. (1997) Chronic
thromboembolism: diagnosis with helical CT and MR
The insufficient relief of pulmonary vascular resis- imaging with angiographic and surgical correlation.
tance after pulmonary thromboendarterectomy is Radiology 204:695–702
1058 S. Iversen
Bergin CJ, Sirlin C, Deutsch R et al. (2000) Predictors of Dartevelle P, Fadel E, Mussot S et al. (2004) Chronic throm-
patient response to pulmonary thromboendarterec- boembolic pulmonary hypertension. Eur Respir
tomy. Am J Roentgenol 174:509–515 J 23:637–648
Bounameaux H, Cirafici P, Moerloose P et al. (1991) De Vega NG (1972) La annuloplastia selective regulable y
Measurement of D-dimer in plasma as diagnostic aid permanete: una trchnica orginal para el tratamiento
in suspected pulmonary embolism. Lancet 337:196– de la insufficiencia tricuspide. Rev Esp Cardiol 25:555–
200 562
Brady AJ, Crake T, Oakley CM (1991) Percutaneous catheter Dittrich HC, Chow LC, Nicod PH (1989) Early improvement
fragmentation and distal dispersion of proximal pul- in left ventricular diastolic function after relief of
monary embolus. Lancet 338:1186–1189 chronic right ventricular pressure overload. Circulation
Brau J, Benacerraf A, Wainer R et al. (1981) Arterial blood 80:823–830
gas analysis in acute pulmonary embolism. Arch Mal Dong B, Jirong Y, Liu G et al. (2006) Thrombolytic therapy
Cœur Vaiss 74:207–214 for pulmonary embolism. Cochrane Database Syst Rev
Bresser P, Pepke-Zaba J, Jais X et al. (2006) Medical thera- (2). Database online
pies for chronic thromboembolic pulmonary hyper- Doyle RL, McCrory D, Channick RN et al. (2004) Surgical
tension. Proc Am Thorac Soc 3:594–600 treatments/interventions for pulmonary arterial
Castañer E, Gallardo X, Ballestreros E et al. (2009) CT diag- hypertension. Chest 126:63S–71S
nosis of chronic pulmonary thromboembolism. Fadel E, Michel RP, Eddahibi S et al. (2004) Regression of
Radiographics 29:31–50 postobstructive vasculopathy after revascularization
Chamsuddin A, Nazzal L, Kang B et al. (2008) Catheter- of chronically obstructed pulmonary artery. J Thorac
directed thrombolysis with the endowave system in Cardiovasc Surg 127:1009–1017
the treatment of acute massive pulmonary embolism: Fedullo PF, Rubin LJ, Kerr KM et al. (2000) The natural his-
a retrospective multicenter case series. J Vasc Interv tory of acute and chronic thromboembolic disease:
Radiol 19:372–376 the search for the missing link. Eur Respir J 15:435–437
Chartier L, Béra J, Delomez M et al. (1999) Free floating Fischer BM, Kreiselmeyer M, Schlegel J et al. (1995)
thrombi in the right heart. Diagnosis, management, Lungenfunktion bei chronisch persistierender
and prognostic indexes in 38 consecutive patients. Lungenembolie. Pneumologie 49:273–276
Circulation 99:2779–2783 Galié N, Kim NHS (2006) Pulmonary microvascular disease
Christie JD, Edwards LB, Kucheryavaya AY et al. (2011) The reg- in chronic thromboembolic pulmonary hypertension.
istry of the International Society for Heart and Lung Proc Am Thorac Soc 3:571–576
Transplantation: 28. Adult lung and heart-lung trans- Galié N, Seeger W, Naeije R et al. (2004) Comparative analy-
plant report-2011. J Heart Lung Transplant 30:1104–1122 sis of clinical trials and evidenced-based treatment
Corsico AG, D’Armini AM, Cerveri I et al. (2008) Long-term algorithm in pulmonary arterial hypertension. JACC
outcome after pulmonary endarterectomy. Am 43:81S–88S
J Respir Crit Care Med 178:419–424 Galié N, Manes A, Negro L et al. (2009) A meta-analysis of
35 Couves GM, Nakai SS, Sterns LP et al. (1973) Hemorrhagic
lung syndrome. Hemorrhagic lung infarction following
randomized controlled trials in pulmonary artery
hypertension. Eur Heart J 30:304–403
pulmonary embolectomy. Ann Thorac Surg 15:187 Ghofrani HA, Schermuly RT, Rose F et al. (2003) Sildenafil
D’Armini AM, Cattadori B, Monterosso C et al. (2000) treatment for long-term treatment of inoperable
Pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension.
chronic thromboembolic pulmonary hypertension: Am J Resp Crit Care Med 167:1139–1141
hemodynamic characteristics and changes. Eur Goldhaber SZ (2005) Multislice computed tomography for
J Cardiothorac Surg 18:696–701 pulmonary embolism—a technological marvel. N Engl
D’Armini AM, Morsolini M, Mattiucci G et al. (2014) Pulmonary J Med 352:1812–1814
endarterectomy for distal chronic thromboembolic pul- Goldhaber SZ, Haire WD, Feldstein ML et al. (1993)
monary hypertension. J Thorac Cardiovasc Surg Alteplase versus heparin in acute pulmonary embo-
148:1005–1011 lism: randomized trial assessing right ventricular func-
Daily PO, Dembitsky WP, Iversen S (1989) Technique of pul- tion and pulmonary perfusion. Lancet 341:507–511
monary thromboendarterectomy for chronic pulmo- Goldhaber SZ, Visani L, De Rosa M et al. (1999) Acute pul-
nary embolism. J Cardiac Surg 4:10–24 monary embolism: clinical outcomes in the
Daily PO, Dembitsky WP, Daily RP (1992) Dissectors for pul- International Cooperative Embolism Registry
monary thromboendarterectomy. Ann Thorac Surg (ICOPER). Lancet 353:1386–1389
51:842–843 Greenfield LJ, Kimmell GO, McCurdy WC (1969) Transvenous
Daily PO, Dembitsky WP, Jamieson SW (1999) The evolu- removal of pulmonary emboli by vacuum-cup cathe-
tion and the current state of the art of pulmonary ter technique. J Surg Res 9:347–352
thromboendarterectomy. Semin Thorac Cardiovasc Grosser KD (1980) Lungenembolie. Erkennung und differ-
Surg 11:152–163 entialtherapeutische Probleme [Pulmonary embo-
Dalla-Volta S, Palla A, Santolicandro A, PAIMS 2 et al. (1992) lism—problems in identifying and treating the
Altelase combined with heparin versus heparin in the condition]. Internist 21:273–282
treatment of acute pulmonary embolism; Plasminogen Hartz RS, Byrne JG, Levitsky S et al. (1996) Predictors of
Activator Italian Multicenter Study 2. J Am Coll Cardiol mortality in pulmonary thromboendarterectomy. Ann
20:520–526 Thorac Surg 62:1255–1260
Chapter 35 · Pulmonary Embolectomy and Pulmonary Thromboendarterectomy
1059 35
Hirsch AM, Moser KM, Auger WR et al. (1996) Unilateral pul- results in 47 consecutive patients after rapid diagnosis
monary artery thrombotic occlusion: is distal arteriopa- and aggressive surgical approach. J Thorac Cardiovasc
thy a consequence? Am J Resp Crit Care Med 154:491–496 Surg 129:1018–1023
Iversen S (1994) Zur operativen Behandlung der thrombo- Lewczuk J, Piszko P, Jagas J et al. (2001) Prognostic factors
embolisch bedingten pulmonalen Hypertonie. Z in medically treated patients with chronic pulmonary
Kardiol 83(Suppl 6):193–199 embolism. Chest 119:818–823
Iversen S (1997) Chirurgische Aspekte der akuten und Long J, Russo MJ, Vigneswaran WT (2011) Surgical treat-
chronischen Lungenembolie. Med Welt 48:343–347 ment of pulmonary hypertension: lung transplanta-
Iversen S, Hake U, Gerharz E et al. (1992) Pulmonale tion. Pulm Circ 1:327–333
thrombendarteriektomie bei thromboembolischer Loud PA, Katz DS, Belfi L et al. (2005) Imaging of deep
pulmonaler Hypertonie. Indikationen und Ergebnisse. venous thrombosis in suspected pulmonary embo-
Dtsch Med Wochenschr 117:1087–1092 lism. Semin Roentgenol 40:33–40
Iversen S, Mayer E, Schmiedt W et al. (1993) Das chirurgische Lualdi JC, Goldhaber SZ (1995) Right ventricular dysfunc-
Konzept der pulmonalen Thrombendarteriektomie. tion after acute pulmonary embolism: pathophysio-
Z Herz-, Thorax-, Gefäßchir 7:23–28 logic factors, detection, and therapeutic implications.
Jakob H, Vahl C, Lange R et al. (1995) Modified concept for Am Heart J 130:1276–1282
fulminant pulmonary embolism. Eur J Cardiothorac Madani MM, Jamiseson SW (2003) Pulmonary thromboen-
Surg 9:557–560 darterectomy. In: Cohn LH, Edmunds LH Jr (eds)
Jamieson SW, Auger WR, Fedullo PF et al. (1993) Experience Cardiac surgery in the adult. McGraw-Hill, New York,
and results with 150 pulmonary thromboendarterec- pp 1205–1228
tomy operations over a 29-month period. J Thorac Mahfood S, Hix WR, Aaron BL et al. (1988) Reexpansion pul-
Cardiovasc Surg 106:116–126 monary edema. Ann Thorac Surg 45:340
Jamieson SW, Kapelanski DP, Sakakibara N et al. (2003) Makey AR, Bliss BP, Ikram H et al. (1971) Fatal intra-alveolar
Pulmonary endarterectomy: experience and lessons pulmonary bleeding complicating pulmonary embo-
learned in 1,500 cases. Ann Thorac Surg 76:1457–1464 lectomy. Thorax 26:466
Jerjes-Sanchez CM, Ramirez-Rivera A, Garcia ML (1995) Mayer E, Dahm M, Hake U, Schmid FX et al. (1996) Mid-
Streptokinase and heparin versus heparin alone in term results of pulmonary thromboendarterectomy
massive pulmonary embolism: a randomized con- for chronic thromboembolic pulmonary hyperten-
trolled trial. J Thromb Thrombolysis 2:227–229 sion. Ann Thorac Surg 61:1788–1792
Kapitan KS, Clausen JL, Moser KM (1990) Gas exchange in Mayer E, Jenkins D, Lindner J et al. (2011) Surgical manage-
chronic thromboembolism after pulmonary thrombo- ment and outcome of patients with chronic thrombo-
endarterectomy. Chest 98:14–19 embolic pulmonary hypertension: results from an
Kay JH, Masselli-Campagna GM, Tsuji KK (1965) Surgical treat- international prospective registry. J Thorac Cardiovasc
ment of tricuspid insufficiency. Ann Surg 162:53–58 Surg 141:702–710
Kearon C, Kahn SR, Agnelli G et al. (2008) Antithrombotic McGoon M, Guttermann D, Steen V et al. (2004) Screening,
therapy for venous thromboembolic disease. Chest early detection and diagnosis of pulmonary arterial
133:454S–545S hypertension. Chest 126:14S–34S
Konstantinides S (2006) Venöse Thromboembolie. Dtsch Melzer C, Richter C, Rogalla P et al. (2004) Tenecteplase for
Med Wochenschr 131:1389–1400 the treatment of massive and submassive pulmonary
Konstantinides S, Geibel A, Olschewski M et al. (1997) embolism. J Thromb Thrombolysis 18:47–50
Association between thrombolytic treatment and the Meneveau N, Ming LP, Seronde MF et al. (2003) In-hospital
prognosis of hemodynamically stable patients with and long-term outcome after submassive and massive
major pulmonary embolism: results of a multicenter pulmonary embolism submitted to thrombolytic ther-
registry. Circulation 96:882–888 apy. Eur Heart J 24:1447–1454
Konstantinides S, Geibel A, Kasper W et al. (1998) Patent Meneveau N, Seronde MF, Blonde MC et al. (2006)
foramen ovale is an important predictor of adverse Management of unsuccessful thrombolysis in acute
outcome in patients with major pulmonary embolism. pulmonary embolism. Chest 129:1043–1050
Circulation 97:1946–1951 Menzel T, Wagner S, Kramm et al. (2000) Pathophysiology
Konstantinides S, Geibel A, Olschewski M et al. (2002) of impaired right and left ventricular function in
Importance of cardiac troponins I and T in risk stratifi- chronic embolic pulmonary hypertension: changes
cation in patients with acute pulmonary embolism. after pulmonary thromboendarterectomy. Chest
Circulation 102:1263–1268 118:897–903
Kucher N, Goldhaber SZ (2005) Management of massive Mo M, Kapelanski DP, Mitruka SN et al. (1999) Reoperative
pulmonary embolism. Circulation 112:e28–e32 pulmonary thromboendarterectomy. Ann Thorac Surg
Kucher N, Windecker S, Banz Y et al. (2005) Percutaneous cath- 68:1770–1776
eter thrombectomy device for acute pulmonary embo- Morgenthaler TI, Ryu JH (1995) Clinical characteristics of
lism; in vitro and in vivo testing. Radiology 236:852–858 fatal pulmonary embolism in a referral hospital. Mayo
Lang IM (2004) Chronic thromboembolic pulmonary hyper- Clin Proc 70:417–424
tension: not so rare after all. N Engl J Med 350:2236–2238 Morpurgo M, Schmid C (1995) The spectrum of pulmonary
Leacche M, Unic D, Goldhaber SZ et al. (2005) Modern sur- embolism. Clinicopathological correlations. Chest
gical treatment of massive pulmonary embolism; 107(Suppl 1):185–205
1060 S. Iversen
Moser KM (1997) Lungenembolien. In: Kurt JG (ed) Tapson VF, Humbert M (2006) Incidence and prevalence of
Harrison: Innere Medizin (Deutsche Version der 13. CTEPH: from acute to chronic pulmonary embolism.
Aufl.), vol 1. Blackwell, Berlin, pp 14–26 Proc Am Thorac Soc 3:564–567
Moser KM, Bloor CM (1993) Pulmonary vascular lesions Tebbe U, Graf A, Kamke W et al. (1999) Hemodynamic effects
occurring in patients with chronic major vessel thrombo- of double bolus reteplase versus alteplase infusion in
embolic pulmonary hypertension. Chest 103:685–692 massive pulmonary embolism. Am Heart J 138:39–44
Moser KM, Daily PO, Peterson KL et al. (1987) Thistlethwaite PA, Mo M, Madani MM et al. (2002) Operative
Thromboendarterectomy for chronic, major vessel classification of thromboembolic disease determines
thromboembolic pulmonary hypertension. Immediate outcome after pulmonary endarterectomy. J Thorac
and long-term results. Ann Intern Med 107:560–565 Cardiovasc Surg 124:1203–1211
Moser KM, Auger WR, Fedullo PF (1990) Chronic major- Timby J, Reed C, Zeilender S et al. (1990) “Mechanical”
vessel thromboembolic pulmonary hypertension. causes of pulmonary edema. Chest 98:973–979
Circulation 81:1735–1743 Torbicki A, Galié N, Covezzoli A et al. (2003) Right heart
Moser KM, Fedullo PF, Littlejohn JK et al. (1994) Frequent thrombi in pulmonary embolism; results from the
asymptomatic pulmonary embolism in patients with International Cooperative Pulmonary Embolism
deep venous thrombosis. JAMA 271:223–228 Registry. J Am Coll Cardiol 41:2241–2251
Oikonomou A, Dennie CJ, Muller NL et al. (2004) Chronic Torbicki A, Perrier A, Konstantinides S et al. (2008)
thromboembolic pulmonary arterial hypertension: Guidelines on the diagnosis and management of
correlation of postoperative results of thromboendar- acute pulmonary embolism. Eur Heart J 29:2276–2315
terectomy with preoperative helical contrast-nhanced Tscholl D, Langer F, Wendler O et al. (2001) Pulmonary
computed tomography. J Thorac Imaging 19:67–73 thromboendarterectomy—risk factors for early sur-
Pengo V, Lensing AW, Prins MH et al. (2004) Incidence of vival and hemodynamic improvement. Eur
chronic thromboembolic pulmonary hypertension J Cardiothorac Surg 19:771–776
after pulmonary embolism. N Engl J Med 350: Uflacker R (2004) Treat the clot. Endovasc Today 3:23–32
2257–2264 Uflacker R, Strange CH, Vujic I (1996) Massive pulmonary
Perrier A, Roy P-M, Sanchez O et al. (2005) Multidetector- embolism: preliminary results of treatment with the
row computer tomography in suspected pulmonary Amplatz thrombectomy device. J Vasc Interv Radiol
embolism. N Engl J Med 352:1760–1768 7:519–528
Pitton MB, Herber S, Schweden F et al. (2002) Cronische van Belle A, Buller HR, Huisman MV et al. (2006)
thromboembolische pulmonale Hypertonie. Diagnos Effectiveness of managing suspected pulmonary
tische Wertigkeit von Mehrschicht-CT und selektiver embolism using an algorithm combing clinical prob-
Pulmonalis-DSA. Fortschr Röntgenstr 174:474–479 ability, D-dimer testing and computed tomography.
Riedel M (2003) Indications for pulmonary endarterec- JAMA 295:172–179
tomy. Letter to the editor. J Thorac Cardiovasc Surg Verstraete M, Miller GA, Bounameaux H et al. (1988)
126:1227–1228 Intravenous and intrapulmonary recombinant tissue-
35 Riedel M, Stanek V, Widimsky J et al. (1982) Long term fol-
low-up of patients with pulmonary embolism: late
type plasminogen activator in the treatment of acute
massive pulmonary embolism. Circulation 77:353–360
prognosis and evolution of hemodynamic and respira- Wacker P, Wacker R, Golnik R et al. (2003) Akute
tory data. Chest 81:151–158 Lungenembolie: Ein neues Score zur Quantifizierung der
Rose PS, Punjabi NM, Pearse DB (2002) Treatment of right akuten Rechtsherzinsuffizienz. Intensiv Med 40:130–137
heart thromboemboli. Chest 121:806–814 Wagenvoort CA, Wagenvooort N (1977) Pathology of pul-
Sandler DA, Martin JF (1989) Autopsy proven pulmonary monary hypertension. Wiley, New York
embolism in hospital patients: are we detecting Wan S, Quirlan DJ, Agnelli G et al. (2004) Thrombolysis
enough deep vein thrombosis. J R Soc Med 82:203–207 compared with heparin for initial treatment of pulmo-
Schwickert HC, Schweden F, Schild HH et al. (1994) nary embolism: a meta-analysis of the randomized
Pulmonary arteries and lung parenchyma in chronic controlled trials. Circulation 110:744–749
pulmonary thromboembolism: pre- and postopera- Ward BJ, Pearse DB (1988) Reperfusion pulmonary edema
tive CT findings. Radiology 191:351–357 after thrombolytic therapy of massive pulmonary
Stein PD, Kayalli F, Olson RE (2004) Trends in the use of embolism. Am Rev Respir Dis 138:1308–1311
diagnostic imaging in patients hospitalized with acute Yi ES, Kim H, Ahn H et al. (2000) Distribution of obstructive
pulmonary embolism. Am J Cardiol 93:1316–1317 intimal lesions and their cellular phenotypes in
Stulz P, Schlapfer F, Feer RM et al. (1994) Decision-making chronic pulmonary hypertension: a morphometric
in the surgical treatment of massive pulmonary embo- and immunohistochemical study. Am J Resp Crit Care
lism. Eur J Cardiothorac Surg 8:188–193 Med 162:1577–1586
1061 36
Lung Transplantation
Clemens Aigner and Walter Klepetko
References – 1075
Chapter 36 · Lung Transplantation
1063 36
36.1 Introduction 36.2 Indications for Lung
Transplantation
Lung transplantation is the established thera-
peutic option for all nonmalignant end stage Virtually all non-malignant end stage lung diseases
lung diseases. The surgical technique is stan- causing mono-organ failure are potential indications
dardized and advances in perioperative and for lung transplantation (. Table 36.1). A general pre-
long-term patient management lead to requisite is a limited life expectancy due to the end
improved results in long- term survival and stage lung disease, the failure of all other conserva-
quality of life. According to the International tive and surgical treatment options and a continuous
Society for Heart and Lung Transplantation progression of the disease (De Meester et al. 2001).
(ISHLT) registry, there is a continuous increase A specific indication is the retransplantation
in the number of procedures performed world- due to acute or chronic graft failure which will be
wide with meanwhile almost 4000 procedures discussed separately.
reported annually (. Fig. 36.1). This increase is The most frequent indication for lung trans-
due to a constant rise in bilateral lung trans- plantation still is chronic obstructive pulmonary
plants, whereas the number of single lung disease (COPD) followed by idiopathic pulmo-
transplants shows a steady state. The number of nary fibrosis (IPF) and cystic fibrosis (CF). Other
combined heart-lung transplants is decreasing. parenchymal and vascular lung diseases account
This chapter mainly deals with the surgical and for smaller percentages of transplantations per-
perioperative aspects of lung transplantation, formed worldwide. Lung transplantation for
whereas medical issues which are usually han- advanced lung cancer of a bronchioloalveolar
dled by pulmonary transplant physicians are growth pattern is not accepted by most centers
only briefly mentioned. due to the high recurrence rate (Machuca and
3500 3459
3180
Number of Transplants
3000 2841
2907
2706
2482
2500
2140
2000 1903 1938
1713
1635
1494
1500 1417 1445
1296 1300
1160
1055
1000 874
664
500 385
160
5 6 32 69
0
08
09
10
11
12
13
14
99
00
01
02
03
04
05
06
07
85
86
87
88
89
90
91
92
93
94
95
96
97
98
20
20
20
20
20
20
19
20
20
20
20
20
20
20
20
20
19
19
19
19
19
19
19
19
19
19
19
19
19
19
..Fig. 36.1 Annual number of lung transplant procedures by type (From: Yusen et al. (2016) ISHLT; used with
permission)
1064 C. Aigner and W. Klepetko
Lymphangioleiomyomatosis
Cystic fibrosis
Bronchiectasis
Keshavjee 2012; de Perrot et al. 2004). However, pressure >15 mmHg, mean pulmonary arterial
incidental nodules or even verified early stage pressure >55 mmHg (Orens et al. 2006).
non-small cell lung cancer stage I do not neces- The most commonly used age limit for lung
sarily pose a contraindication for lung transplan- transplantation is 65; however, the biological age
tation and good outcome has been reported in rather than the actual age should guide the deci-
carefully selected patients. Since the introduction sion for waiting list acceptance.
of the lung allocation score (LAS), which priori-
tizes patients with a higher risk for mortality on
the waiting list, a significant shift in the indication 36.3 Contraindications for Lung
spectrum occurred with a steady increase in the Transplantation
number of procedures performed for IPF et al.
36 (Pierson et al. 2000). There are only few absolute contraindications for
The decision for referral and listing for trans- lung transplantation; however, a number of relative
plantation is not only based on functional criteria contraindications exist, which might vary from cen-
alone, but also takes the prognosis of the specific ter to center according to different individual experi-
disease, the anticipated waiting list time and qual- ences and differences in the availability of donor
ity of life into account. organs. Absolute contraindications are: significant
The functional parameters indicating the systemic diseases, severe extrapulmonary organ dys-
necessity for lung transplantation are as follows: functions, recent malignancies, HIV infection,
COPD: FEV1 <25 % predicted, pCO2 >55 mmHg Hepatitis B or C, pan-resistant MRSA or Burkholderia
with or without subsequent pulmonary hyper- cepacia, active nicotine, alcohol or drug abuse.
tension. The large number of relative contraindications
CF: FEV1 <30 % predicted, increasing hospitaliza- must be individually judged from case to case:
tion time, cachexia, pO2 <55 mmHg, pCO2 osteoporosis, muscular or skeletal diseases,
>50 mmHg extreme cachexy or obesity, long time corticoste-
IPF: Forced vital capacity (FVC) or Diffusing roid therapy, infection with mycobacteria, coro-
capacity of the lung for carbon monoxide nary disease or left ventricular dysfunction,
(DLCO) <50 % predicted significant peripheral vascular disease, renal
Pulmonary hypertension: WHO functional class insufficiency, mechanical ventilation, severe chest
III or IV, cardiac index <2 l/min/m2, right atrial wall deformity and psychosocial instability.
Chapter 36 · Lung Transplantation
1065 36
36.4 Recipient Evaluation bilateral transplantation which is the standard
approach for pulmonary vascular disease.
Prior to accepting a patient on the waiting list, a Combined heart-lung transplantation is reserved
complete medical workup is required. A list which for complex cardiac defect with Eisenmenger’s syn-
might serve as an example for required examina- drome. In simple atrial or ventricular septal
tions is shown in . Table 36.2. Apart from these defects, intraoperative repair during double lung
objective findings, the patient’s clinical appear- transplantation on cardiopulmonary bypass (CPB)
ance and quality of life are major factors for the can usually be performed (Pielsticker 2001).
decision. If the patient is put on the waiting list, a The necessity of a ventilator support or extra-
physiotherapeutic training program is strongly corporeal bridging device has long been consid-
recommended (Schwaiblmair et al. 1999). ered a contraindication for lung transplantation.
The decision whether to list a patient for single However, with refinements in the management
(SLTX) or bilateral lung transplantation (BLTX) and the introduction of new allocation algo-
depends on various factors. In infectious diseases rithms, such as the lung allocation score (LAS),
such as CF or bronchiectasis, bilateral transplanta- significantly improved outcome has been reported
tion is required to avoid cross infection of the in the group of patients. The current strategy in
transplanted organ. In COPD and IPF, single lung patients requiring invasive support is to try to
transplantation is possible; however, most available avoid intubation and keep the patient ambulatory
data report on favorable outcome for bilateral pro- with early implantation of an extracorporeal
cedures. In pulmonary hypertension various bridging device (Lang et al. 2012). By using this
approaches exist. Due to the shortage of donor strategy the patient can be kept awake and in
organs, unilateral transplantation was suggested, some cases even maintain physiotherapy to mini-
which, however, has the significant disadvantage mise amyotrophia. There are several bridging
that almost the entire cardiac output is directed in modalities available for different indications. The
the transplanted lung. This can be overcome by arteriovenous Novalung® is used for CO2 removal
(Camboni et al. 2009; Schmid et al. 2008; Strueber
..Table 36.2 Recipient evaluation et al. 2009). Veno-venous extracorporeal mem-
brane oxygenation (ECMO), which can be
Medical history and physical examination inserted with two separate cannulas or via a single
Blood tests: routine laboratory examinations
double-lumen cannula (Avalon®, iLA activve®),
offers additional oxygenation (Hayes et al. 2012;
Blood group Garcia et al. 2011), although its main function
TSH, T4, T3, PTH, calcitonin, osteocalcin, Vit. D remains CO2 removal as first described by
Gattinoni in 1980 as ECCO2-R (Extracorporeal
HLA—typing, panel reactive antibodies
(PRA)—lymphocytotoxic antibodies
CO2 removal, Gattinoni et al. 1980, 1986).
Hemodynamic support with reduction of pulmo-
Tuberculin test, virus serology: CMV (IgG/IgM), nary arterial pressure is only provided by veno-
EBV, HIV, Hepatitis A, B, C
arterial ECMO, which is the standard bridging
Chest X-ray, chest CT, lung function test, ventilation/ tool in patients with pulmonary hypertension
perfusion scan (Fuehner et al. 2012).
Sputum culture
ECG, echocardiography
36.5 Legal Situation of Organ
Bone densitometry Donation
Abdominal sonography
There are different legal regulations of organ
Disease specific: sinus x-ray, right cardiac catheter,
donation. Three main forms of legislation can be
coronary angiography, sonography of the carotid
and vertebral arteries described:
Presumed consent (e.g. Austria, Belgium,
Age >40 years: tumor markers, gynecologic Luxemburg, Slovenia): Everyone diagnosed
examination, mammography, urologic examination
brain dead is a potential organ donor, unless
Additional examinations according to patient registration in an opting-out register has been
specific considerations performed during lifetime.
1066 C. Aigner and W. Klepetko
Informed consent (e.g. Germany, Netherlands): donors are categorized according to the Maastricht
To be a potential organ donor, an informed criteria in the controlled and uncontrolled. The
consent has to be signed during their lifetime. majority of centers are using Maastricht category
Additionally there is the possibility for the III controlled donors with result comparable to
relatives to give permission at the time of brain dead donors. One Spanish center has accu-
death, usually in the knowledge that the mulated a substantial experience with uncon-
potential donor had expressed a wish to trolled DCD. The rate of primary graft dysfunction
become a donor. is higher than in other donor groups; however,
Required request (e.g. USA): The physician in long-term results are encouraging. With the
charge of potential donors is required to ensure development of ex vivo lung perfusion (EVLP), a
that someone speaks to the relatives about powerful tool to evaluate DCD lungs has been
organ donation. introduced.
36.6.1 Brain Dead Organ Donors Living lung donation was initially reported in
1994 and has been primarily used in pediatric CF
The lung is especially susceptible to bacterial recipients to overcome donor shortage (Cohen
infection or aspiration before brain death and and Starnes 2001). Results are comparable and in
organ procurement and is furthermore exposed some series even superior to brain dead donor
to the trauma of mechanical ventilation; therefore grafts. Two adults, either the parents or close rela-
potential donors have to be carefully evaluated. tives serve as donor, each donating one lower lobe
When mechanically ventilated with a FiO2 and a bilateral lobar transplantation is performed
of 1.0 and positive end-expiratory pressure of via clamshell incision using ECMO support.
5 cm H2O, a suitable allograft is characterized Access for harvesting the lobe is gained by antero-
by a pO2 of at least 300 mmHg. When ventilated lateral thoracotomy. No donor mortality has been
with a FiO2 of 0.4, pO2 has to be above described and 85 % of the donors report about no
120 mmHg. Concerning the acceptable donor difference in their health status since donation.
age, different guidelines exist, though donors
above 65 years are generally not accepted. The
smoking history should be <20 pack-years. 36.6.4 x Vivo Lung Perfusion
E
36 Chest radiography should be without any patho- (EVLP)
logic intrapulmonal findings. A chest trauma or
in certain cases an infiltrate, if unilateral, does EVLP has emerged as a new option to increase the
not preclude the donation of the unimpaired donor organ pool for lung transplantation. The
lung. Bronchoscopy is performed routinely first experience with EVLP was reported by the
prior to harvesting. group in Lund, who used a blood-based perfusion
Besides those specific criteria the general pre- solution for evaluation of a DCD lung. Thereafter
clusion criteria like malignant tumors, general- EVLP was applied to repair and improve lungs
ized infections, HIV infection and, in most cases, which were not suitable for transplantation by
Hepatitis B and C apply. conventional functional criteria. Several technical
The definite decision whether the organ is refinements have been made during the develop-
suitable for transplantation or not is made after ment process of EVLP with a complete paradigm
retrieval and after inspection of the parenchyma shift from cold to normothermic perfusion. The
and hilar structures. first extended experience with EVLP was reported
by the Toronto group, who used acellular Steen
solution (®XVIVO, Gothenberg, Sweden) for pro-
36.6.2 Donation After Circulatory longed perfusion periods of up to 12 h. From the
Death (DCD) same group derives the currently largest clinical
experience, published in the NEJM in April 2011
Donation after circulatory death has led to a sig- with 23 EVLPs resulting in 20 transplantations
nificant increase in the available donor pool. DCD during a 17-months observation period (Cypel
Chapter 36 · Lung Transplantation
1067 36
et al. 2011). We evaluated EVLP in a prospective fourth or fifth intercostal space depending on the
clinical trial at 13 occasions resulting in nine individual anatomical situation (. Fig. 36.2). For
transplantations with excellent short term results double lung transplantation, access is gained
(Aigner et al. 2012). According to the current either by bilateral transsternal anterior thoracot-
experience, EVLP is a valuable tool for evaluation omy (. Fig. 36.3), the so-called ‘clamshell inci-
and functional improvement of borderline donor sion’ (. Fig. 36.4) or by two separate anterolateral
lungs. However, it remains undetermined how far
the limits for acceptance can be pushed by the use
of EVLP. Furthermore, the potential of EVLP to
recondition damaged lungs by counteracting the
functional impairments caused by brain death has
to be evaluated. Also still undetermined is
whether the quality of procurement in standard
donor lungs may be improved by routine EVLP.
..Fig. 36.5 Positioning for bilateral transplantation ..Fig. 36.6 Right hilum after preparation for
implantation
a b
Left
180° Upper
Lobe
Left
Lower
Lobe
..Fig. 36.8 a Technique of split lung transplantation and b typical postoperative chest X-ray (From Marta et al. 2005.
Used with permission)
(TLC). The lower lobe is implanted on the left side overload, infusions should be minimized and
and the upper lobe is rotated 180° along the longi- intravenous drips concentrated. Blood pressure is
tudinal axis and implanted on the right side to be supported by catecholamines at low doses
(Couetil 1997). The technique is demonstrated in rather than by volume loading.
. Fig. 36.8. On the right side, the upper lobe bron- Physiotherapy is important for secretion
chus is stapled and the intermediate bronchus is clearance and should be started as early postop-
used for the anastomosis to allow sufficient length eratively as possible. Once the patient is extu-
and improve size match. The atrial cuff is divided in bated, early mobilization is crucial.
the upper and lower lobe vein. The left donor pul- Routine bronchoscopies are performed imme-
monary artery is stapled and the fissural aspect of diately before extubation, usually on the first post-
the artery is splitted and used for the anastomosis. operative day. Surveillance bronchoscopies start
1 week postoperatively. Additional bronchosco-
pies are performed upon clinical necessity.
36.9.2 Retransplantation Usually patients can be discharged from the
36 ICU 1 or 2 days after successful extubation.
Retransplantation has been used for the treatment of Since wound healing is decelerated due to the
acute and chronic graft failure refractory to other high corticosteroid doses required, skin sutures
treatment options (Novick et al. 1992, 1998). Results and staples must not be removed before the 12th
of early retransplantation for primary graft dysfunc- postoperative day. A lung function test and a
tion are disappointing and do not justify the use of computed tomography of the chest are performed
donor organs for this indication. However, with before discharge of the patient.
retransplantation for patients with chronic lung After uneventful postoperative course,
allograft dysfunction without relevant comorbidity, patients can usually be discharged 3 weeks post-
results close to those of primary transplantation can operatively and will subsequently spend 4 weeks
be achieved (Aigner et al. 2008; Strueber 2006). in a rehabilitation facility.
virus or as primary infections mainly in the con- ing on the size of the transplant center and has
stellation of a CMV negative recipient who receives been significantly reduced since the introduction
a graft from a CMV-positive donor. Symptoms are of the lung allocation score (LAS). Calculation of
non-specific and are not infrequently accompa- the LAS is based on formulas that take into
nied by leukenia. Treatment of choice is currently account the statistical probability of a patient’s
ganciclovir or valganciclovir. Additionally patients survival in the next year without a transplant,
may receive CMV hyperimmunoglobulin (IgM) how long that survival would be, the probability
100 mg IV weekly for the first 4 weeks. All patients of survival following a transplant and the pro-
are routinely screened for CMV infection at every jected length of survival post-transplant. A raw
outpatient follow-up appointment. allocation score based on these values is calcu-
Fungal infections occur mainly during the lated and normalized to obtain the actual LAS,
first 2 months after transplantation with which has a range from 0 to 100. Recipients under
Aspergillus species and Candida species being the 12 years of age automatically receive priority in
predominant pathogens. Treatment of manifest organ allocation.
infection is performed by administration of IV Survival statistics provided by the Interna-
antifungal agents, according to the underlying tional Society for Heart and Lung Transplantation
subtype. (ISHLT) report: 1-year, 3-year and 5-year survival
Protozoan infections, especially with rates of about 79 %, 63 % and 52 %, respectively;
Pneumocystis carinii or Toxoplasma gondii are however, there has been a significant rise in sur-
usually prevented by lifelong administration of vival rates recently as shown in . Fig. 36.9. There
trimethoprim/sulfametrol (3 days per week). is a difference in the survival rates in favor of dou-
ble lung recipients versus single lung recipients,
which diverges between the third and fourth year
36.12.6 Post-transplantation postoperatively. The survival benefit of transplan-
Malignancy tation versus medical therapy differs depending
on the underlying disease.
Due to immunosuppression the incidence of some
neoplasms is higher in transplant patients than
usual. Non Hodgkin lymphomas may appear 36.13.2 Functional Outcome
3–12 months after transplantation. Post-transplant
lymphoproliferative disorder (PTLD) is associ- After successful transplantation most patients
ated with Epstein–Barr virus (EBV), with EBV- experience a major improvement in functional
36 negative recipients being at higher risk. PTLD is capacity and report by the end of the first year
commonly found in extranodal sites like the lungs, about no restriction in activity. Six-minute walking
intestine or central nervous system. Therapy con- tests are at least twice the preoperative value (Cas-
sists of a decrease in immunosuppression with tleberry et al. 2015). A progressive rise in total lung
additional chemotherapy or radiotherapy. Apart capacity, FEV1 and vital capacity is observed. Work
from PTLD the incidence of squamous cell cancer capacity, tidal volumes and peak minute ventilation
of the skin and lip, carcinoma of vulva and are not statistically different between single and
perineum, as well as the kidney and hepatobiliary double lung recipients (Schwaiblmaier et al. 1998).
tumors, are elevated. Quality of life is markedly improved after lung
transplantation. Independence from oxygen
insufflation with diminished dyspnea, improved
36.13 Results sleep, improved mobility and energy to accom-
plish activities of everyday life all collude for
36.13.1 Mortality improved quality of life. Three years after trans-
plantation according to ISHLT, more than 40 % of
Mortality on the waiting list varies widely from the patients are full- or part-time employed.
country to country depending on the individual Thereafter, with the development of bronchiolitis
legal situation. Eurotransplant reports about an obliterans syndrome, this trend reverses, though
overall mortality of 25 % on the waiting list. In most patients remain active for quite some time,
the USA the average waiting time differs depend- despite the development of obliterative bronchiol-
Chapter 36 · Lung Transplantation
1075 36
70
p < 0.0001
Survival (%)
60
50
40
30
20
Bilateral/Double Lung (N=25,041)
10
Single Lung (N=15,251)
0
0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
Years
..Fig. 36.9 Survival rates international (From: Yusen et al. (2014) ISHLT; used with permission)
itis. Another problem restricting long-term qual- systemic immunosuppression is carried out and
ity of life is the side effects of immunosuppressive might hold potential for the future.
medication.
References
36.14 Future Perspectives
Aigner C, Mazhar S, Jaksch P, Seebacher G, Taghavi S, Marta
Lung transplantation is now an established ther- G, Wisser W, Klepetko W (2004) Lobar transplantation,
apy option for advanced lung disease. Restricting split lung transplantation and peripheral segmental
resection–reliable procedures for downsizing donor
factors are the limited availability of donor organs
lungs. Eur J Cardiothorac Surg 25(2):179–183
and limited long time survival after transplanta- Aigner C, Wisser W, Taghavi S, Lang G, Jaksch P, Czyzewski
tion. Options for an increase of available donor D, Klepetko W (2007) Institutional experience with
organs are living related donations, especially for extracorporeal membrane oxygenation in lung trans-
pediatric recipients and split lung transplantations, plantation. Eur J Cardiothorac Surg 31(3):468–473
Aigner C, Jaksch P, Taghavi S et al. (2008) Pulmonary retrans-
which are already performed in increasing num-
plantation: is it worth the effort? A long-term analysis of
bers. Donation after circulatory death already had 46 cases. J Heart Lung Transplant 27(1):60–65
an impact on the number of available lung donors, Aigner C, Slama A, Hötzenecker K et al. (2012) Clinical ex vivo
and ex vivo lung perfusion holds a great potential lung perfusion—pushing the limits. Am J Transplant
for a further increase in the donation rates. 12(7):1839–1847
Camboni D, Philipp A, Arlt M et al. (2009) First experience
Refinements in organ procurement, lung pres-
with a paracorporeal artificial lung in humans. ASAIO
ervation and immunosuppression will hopefully J 55(3):304–306
contribute to increased long-term survival rates. Castleberry AW, Englum BR, Snyder LD, Worni M, Osho AA,
Research on induction of graft tolerance without Gulack BC, Palmer SM, Davis RD, Hartwig MG (2015)
1076 C. Aigner and W. Klepetko
The utility of preoperative six-minute-walk distance in Machuca TN, Keshavjee S (2012) Transplantation for lung
lung transplantation. Am J Respir Crit Care Med cancer. Curr Opin Organ Transplant 17:479–484
192(7):843–852 Marta GM Aigner C, Klepetko W (2005) Split lung trans-
Cohen RG, Starnes VA (2001) Living donor lung transplan- plantation with intraoperative extracorporeal mem-
tation. World J Surg 25(2):244–250 brane oxygenation (ECMO) support. Multimed Man
Colt HG, Janssen JP, Dumon JF et al. (1992) Endoscopic Cardiothorac Surg 2005(809):mmcts.2004.000984.
management of bronchial stenosis after double lung doi:10.1510/mmcts.2004.000984
transplantation. Chest 102(1):10 Morris PJ, Russell NK (2006) Alemtuzumab (campath-1H): a
Couetil JP, Tolan MJ, Loulmet DF, Guinvarch A, Chevalier systematic review in organ transplantation.
PG, Achkar A, Birmbaum P, Carpentier AF (1997) Transplantation 81:1361–1367
Pulmonary bipartitioning and lobar transplantation: a Novick RJ, Kaye MP, Patterson GA et al. (1992) Redo lung
new approach to donor organ shortage. J Thorac transplantation: a North-American European experi-
Cardiovasc Surg 113(3):529–537 ence. J Heart Lung Transplant 12(1pt1):5
Cypel M, Yeung JC, Liu M et al. (2011) Normothermic Novick RJ, Schäfers HJ, Stitt L, Andréassian B, Klepetko W,
ex vivo lung perfusion in clinical lung transplantation. Hardesty RL, Frost A, Patterson GA (1995) Seventy-two
N Engl J Med 364(15):1431–1440 pulmonary retransplantations for obliterative bron-
De Meester J, Smits JM, Persijn GG, Haverich A (2001) chiolitis: predictors of survival. Ann Thorac Surg
Listing for lung transplantation: life expectancy and 60(1):111–116
transplant effect, stratified by type of end-stage lung Novick RJ, Stitt LW, Al-Kattan K et al. (1998) For the
disease, the eurotransplant experience. J Heart Lung Pulmonar Retransplant Registry. Pulmonary retrans-
Transplant 20(5):518–524 plantation: predictors of graft function and survival in
de Perrot M, Chernenko S, Waddell TK, Shargall Y, Pierre AF, 230 patients. Ann Thorac Surg 65(1):227
Hutcheon M, Keshavjee S (2004) Role of lung trans- Orens JB, Estenne M, Arcasoy S, Conte JV, Corris P, Egan JJ,
plantation in the treatment of bronchogenic carcino- Egan T, Keshavjee S, Knoop C, Kotloff R, Martinez FJ,
mas for patients with end-stage pulmonary disease. Nathan S, Palmer S, Patterson A, Singer L, Snell G,
J Clin Oncol 22(21):4351–4356 Studer S, Vachiery JL, Glanville AR (2006) International
Estenne M, Maurer JR, Boehler A et al. (2002) Bronchiolitis guidelines for the selection of lung transplant candi-
obliterans syndrome 2001: an update of the diagnos- dates: 2006 update—a consensus report from the pul-
tic criteria. J Heart Lung Transplant 21(3):297–310 monary scientific council of the International Society
Fuehner T, Kuehn C, Hadem J et al. (2012) Extracorporeal for Heart and Lung Transplantation. J Heart Lung
membrane oxygenation in awake patients as bridge Transplant 25:745–755
to lung transplantation. Am J Respir Crit Care Med Pereszlenyi A, Lang G, Steltzer H, Hetz H, Kocher A,
185(7):763–768 Neuhauser P, Wisser W, Klepetko W (2002) Bilateral
Garcia JP, Kon ZN, Evans C et al. (2011) Ambulatory veno- lung transplantation with intra- and postoperatively
venous extracorporeal membrane oxygenation: inno- prolonged ECMO support in patients with pulmonary
vation and pitfalls. J Thorac Cardiovasc Surg hypertension. Eur J Cardiothorac Surg 21(5):858–863.
142(4):755–761 Pierson RN, Milstone AP, Loyd JE et al. (2000) Lung allocation
Gattinoni L, Pesenti A, Rossi GP, Vesconi S, Fox U, Kolobow in the United States, 1995–1997: an analysis of equity
36 T, Agostoni A, Pelizzola A, Langer M, Uziel L, Longoni F, and utility. J Heart Lung Transplant 19(9):846–851
Damia G (1980) Treatment of acute respiratory failure Radley-Smith RC, Burke M, Pomerance A et al. (1995) Graft
with low-frequency positive-pressure ventilation and vessel disease and obliterative bronchiolitis after
extracorporeal removal of CO2. Lancet 316(8189):292– heart/lung transplantation in children. Transplant Proc
294, 9 August 1980 27(3):2017
Gattinoni L M.D., Pesenti A M.D., Mascheroni D M.D., Sarahrudi K, Carretta A, Wisser W et al. (2002) The value of
Marcolin R M.D., Fumagalli R M.D., Rossi F M.D., switching from cyclosporine to tacrolimus in the treat-
Lapichino G M.D., Romagnoli G M.D., Uziel L M.D., ment of refractory acute rejection and obliterative
Agostoni A M.D., Kolobow T M.D., Damia G M.D. (1986) bronchiolitis after lung transplantation. Transpl Int
Low- frequency positive-pressure ventilation with 15(1):24–28
extracorporeal CO2 removal in severe acute respira- Sato M, Waddell TK, Wagnetz U et al. (2011) Restrictive
tory failure. JAMA 256(7):881–886 allograft syndrome (RAS): a novel form of chronic lung
Hayes D Jr, Kukreja J, Tobias JD et al. (2012) Ambulatory allograft dysfunction. J Heart Lung Transplant
venovenous extracorporeal respiratory support as a 30(7):735–742, Epub 2011 Mar 17
bridge for cystic fibrosis patients to emergent lung Schmid C, Philipp A, Hilker M et al. (2008) Bridge to lung trans-
transplantation. J Cyst Fibros 11(1):40–45 plantation through a pulmonary artery to left atrial oxy-
Heng D, Sharples LD, McNeil K et al. (1998) Bronchiolitis genator circuit. Ann Thorac Surg 85(4):1202–1205
obliterans syndrome: incidence, natural history, prog- Schwaiblmair M, Beinert T, Seemann M, Behr J, Reiser M,
nosis and risk factors. J Heart Lung Transplant Vogelmeier C (1998) Relations between cardiopulmonary
17(12):1255 exercise testing and quantitative high-resolution com-
Lang G, Taghavi S, Aigner C et al. (2012) Primary lung trans- puted tomography associated in patients with alpha-
plantation after bridge with extracorporeal membrane 1-antitrypsin deficiency. Eur J Med Res 3(11):527–532
oxygenation: a plea for a shift in our paradigms for indi- Schwaiblmair M, Reichenspurner H, Mueller C et al. (1999)
cations. Transplantation 93(7):729–736 Cardiopulmonary exercise testing before and after
Chapter 36 · Lung Transplantation
1077 36
lung and heart-lung transplantation. Am J Respir Crit van Loenhout KC, Groves SC, Galazka M, Sherman B, Britt E,
Care Med 159(4pt1):1277 Garcia J, Griffith B, Iacono A (2010) Early outcomes
Shyu S, Dew MA, Pilewski JM, DeVito Dabbs AJ, Zaldonis using alemtuzumab induction in lung transplantation.
DB, Studer SM, Crespo MM, Toyoda Y, Bermudez CA, Interact Cardiovasc Thorac Surg 10(2):190–194
McCurry KR (2011) Five-year outcomes with alemtu- Verleden GM, Vos R, Verleden SE et al. (2011) Survival
zumab induction after lung transplantation. J Heart determinants in lung transplant patients with chronic
Lung Transplant 30(7):743–754 allograft dysfunction. Transplantation 92(6):703–708
Strueber M, Fischer S, Gottlieb J, Simon AR, Goerler H, Yusen RD, Edwards LB, Dipchand AI et al. (2014) The Registry
Gohrbandt B, Welte T, Haverich A (2006) Long-term of the International Society for Heart and Lung
outcome after pulmonary retransplantation. J Thorac Transplantation: Thirty-first Adult Lung and Heart-Lung
Cardiovasc Surg 132(2):407–412. Transplant Report-2014; Focus Theme Retransplant. J
Strueber M, Hoeper MM, Fischer S et al. (2009) Bridge to Heart Lung Transplant 33(10):1009–1024
thoracic organ transplantation in patients with pul- Yusen RD, Edwards LB, Dipchand AI et al. (2016) The
monary arterial hypertension using a pumpless lung Registry of the International Society for Heart and
assist device. Am J Transplant 9(4):853–857 Lung Transplantation: Thirty-third Adult Lung and
Sudarshan CD, Clark SC, Dark JH (1998) Single or bilateral Heart–Lung Transplant Report—2016; Focus Theme:
lung transplantation for chronic obstructive pulmo- Primary Diagnostic Indications for Transplant. J Heart
nary disease. J Thorac Cardiovasc Surg 115(2):485 Lung Transplant 35(10):1170–1184
1079 37
Heart and Heart–Lung
Transplantation
Bruno Meiser and Bruno Reichart
References – 1100
Chapter 37 · Heart and Heart–Lung Transplantation
1081 37
37.1 Heart Transplantation first human heart transplant. Within 2 years, more
in Adults than 60 teams had transplanted hearts in some
150 patients (see also 7 Chapter «The History of
37.1.1 History and State of the Art Cardiac Surgery», Sect. 1.12).
The leading European pioneer in this field was
The basic surgical technique behind the transplan- Christian Cabrol at the Pitié-Salpêtrière Hospital
tation of organs, the suturing of blood vessels, was in Paris. He performed in 1968 the first heart
developed by Alexis Carrel, a French surgeon, at transplantation in Europe.
the beginning of the twentieth century. Together At that time, the missing experience with the
with Charles Guthrie, he described the first het- post-transplant immune reaction and the very
erotopic heart transplant in ‘The Transplantation limited variety of immunosuppressant drugs
of Veins and Organs’ (Carrel and Guthrie 1905). In available (methylprednisolone, azathioprine,
1912 Carrel received the Nobel Prize in Medicine antilymphocyte globulin) resulted in 80 % 1-year
and Physiology for his groundbreaking research. mortality. The results were discouraging, and the
The next surgeon to experiment extensively number of heart transplants worldwide went
with heart transplantation was Vladimir down to 18 in 1970 (DiBardino 1999).
Demikhov, a Russian. By the 1940s, he had already One of the few groups who continued was
developed an artificial heart and transplanted Norman Shumway’s group at Stanford University.
‘piggyback’ hearts and heart–lungs in dogs. His His team developed techniques to determine and
most far-reaching achievement was a series of quantify rejection allowing them to tailor immu-
experiments in which canine hearts were success- nosuppression and to achieve 65 % 1-year sur-
fully transplanted orthotopically, at a time when vival. In 1980, they were the first to test cyclosporin
hypothermia had not been used and the equip- A clinically and to incorporate its use into clinical
ment for successful extracorporeal circulation practice after heart transplantation.
had not been invented. In the following decade, the improved outcome
In the 1950s, Norman Shumway and Richard resulted in a second boom, reaching a peak in the
Lower, heart surgeons at Stanford University, mid-1990s with about 4,700 heart transplants per
began publishing their experiments. It proved to year. Subsequently, mainly due to the limited avail-
be the beginning of a long list of important ability of donor hearts, the annual number began to
achievements. Key to their success was the use of level off and has reached in the last few years a
topical hypothermia, which allowed the interrup- steady state of about 4,000 to 4,500 cases (Lund
tion of blood flow and adequate time to operate et al. 2015).
on the hearts. In 1960, they described their stud-
ies with orthotopic homotransplantations of
canine hearts (Lower and Shumway 1960). In the 37.1.2 Indication, Management
following years, they improved and optimized of Candidates, Listing
their technique and management for orthotopic
allotransplantation (Lower et al. 1962). The Indication for cardiac transplantation is the sig-
‘Shumway technique’ is today still the preferred nificant, irreversible functional impairment of the
method for cardiac replacement (Lower et al. heart despite maximal medical therapy.
1965). The most common causes are either idiopathic
On December 3, 1967, Christiaan Barnard dilated cardiomyopathy or ischemic cardiomyop-
used this technique to transplant the first human athy caused by coronary artery disease. Less com-
donor heart at the Groote Schuur Hospital in mon are congenital heart disease, valvular heart
Cape Town, South Africa. The patient died of disease or damage of the myocardium by condi-
pneumonia on the eighteenth postoperative day tions like amyloidosis or sarcoidosis. . Figure 37.1
(Barnard 1967). Three days after Barnard, sur- gives the distribution of the current indications
geons at the Maimonides Medical Center in for heart transplantation worldwide between 2005
Brooklyn undertook the second human heart and 2010 (Lund et al. 2015).
transplantation. Recipient was a 3-month-old An optimal management of potential cardiac
infant who died the same day. One month later, transplant candidates is mandatory. It includes
Norman Shumway and his team undertook their pharmacological and non-pharmacological
1082 B. Meiser and B. Reichart
11% 6%
35%
7%
1% 1%
2% Cardiomyopathy Congenital
2% 3%
74% Retransplant CAD 2%
Other Valvular
18–39 40–59
years years
56% 57%
51%
1% 1%
3% 2%
60–69 70+
years years
1% 38%
1% 44% 3%
0%
..Fig. 37.1 Indications for heart transplantation in adults between 2009 and 2014 (Lund et al. 2015)
measures: The retention of body fluids results in these cases, the temporary implantation of a
peripheral edema and ascites which should be mechanical circulatory assist device should be con-
controlled by the application of diuretics. Chronic sidered in order to prevent further deterioration of
heart failure activates the neuroendocrine system the clinical status and to improve renal and liver
of the body as a reaction to the malperfusion of function. In cases of intermittently disabling tachy-
the vital organs. The sympathetic nervous sys- cardia or cardiac dyssynchrony, cardiac re-synchro-
tem, the renin–angiotensin–aldosterone system is nization therapy with an implantable cardioverter
activated. Vasopressin, cytokines and endothelin defibrillator should be used (Moss et al. 2002; Bardy
are released. Therefore, neurohormonal antago- et al. 2005; Gronda et al. 2006).
nists like ACE (angiotensin-converting enzyme) Elective transplant listing should be guided by
or ARB (angiotensin receptor blockers) should evaluation of several parameters: Indication for
37 be used. Furthermore, ß–blockers or aldosterone transplantation is given if the maximal cardiopul-
antagonists can be added (Jessup et al. 2006). monary exercise test results in a peak oxygen con-
These pharmacologic measures should be sumption (Vo2) <10 ml/kg/min; a peak Vo2
accompanied by restriction of salt and fluid intake between 10 and 14 ml/kg/min in combination with
and regular exercises. Periodical examinations by a major limitation to the activities of daily life can
a medical team experienced in heart failure treat- also be considered. In addition, the Heart Failure
ment should be conducted, including semi-annual Survival Score (HFSS) may be used. The score is a
right heart catheterization to access pulmonary predictive model calculated from seven prognostic
vascular resistance and to adjust medical treat- variables including peak Vo2, left ventricular ejec-
ment (Jessup et al. 2006). tion fraction, serum sodium, mean blood pressure,
If cardiac function further deteriorates, intrave- heart rate at rest, non-specific intraventricular con-
nous vasodilators are first choice options in medical duction delay and ischemic heart failure etiology.
management; in cases of hypoperfusion short term After calculation of the score, it can be grouped in
inotropic therapy can be used (Hübner et al. 2015; risk strata: Low-risk strata are considered ≥8.10,
von Scheidt et al. 2016) If patients with cardiac fail- medium-risk strata 7.20–8.09 and high-risk strata
ure of class NYHA (New York Heart Association) <7.20 (Aaronson et al. 1997; Mehra et al. 2006).
III or IV are refractory to medical treatment, urgent Before listing for transplantation, patients
indication for heart transplantation is given. In should undergo a psychosocial assessment.
Chapter 37 · Heart and Heart–Lung Transplantation
1083 37
valuation should include their ability to comply
E countries with that type of legislation, the rate of
with instructions, including drug therapy. consent for organ donation by next of kin is rather
Possible co-morbidities must be carefully low. No more than 50 % of those US families from
evaluated. Age over 70, pre-transplant body mass whom donation is requested agree to donate.
index (BMI) >30 kg/m2 and pre-existing neo- Nevertheless, the United States still achieve annu-
plasms with a high risk of tumor recurrence are ally around 25 donors per million inhabitants.
associated with poor outcome after transplanta- The organ donation process itself is country
tion. Diabetes with end-organ damage, irrevers- specific organized by national organ procurement
ible renal dysfunction and severe symptomatic organizations. In Germany, this duty is contracted
cerebrovascular disease should also be considered to the German Foundation Organ Transplantation
as relative contraindications for transplantation (Deutsche Stiftung Organtransplantation, DSO).
(Mehra et al. 2006). In the United States, more than 50 organ procure-
Serial evaluations are essential for patients ment organizations (OPOs) provide this service
placed on the transplant waiting list. Assessment for the close to 300 transplant centers. The major-
of heart failure stability should include echocar- ity of these OPOs are private, nonprofit organiza-
diogram and electrocardiogram as well as routine tions, only a few are hospital based. Each OPO has
laboratory testing to evaluate multi-organ func- a contiguous geographical service area designated
tion. Right heart catheterization should be used to by the Federal Government for recovering organs
examine pulmonary pressures. Pulmonary hyper- in all hospitals in that region (Nathan et al. 2003).
tension should be challenged by vasodilators to Even in countries with high donation rates,
determine whether it is fixed or reversible. the shortage of post-mortem organ donors in
Irreversible pulmonary artery hypertension is a general and especially of donors for thoracic
relative contraindication to cardiac transplanta- organs represents the biggest challenge and limi-
tion when the pulmonary vascular resistance tation for heart transplantation. In addition to the
(PVR) is >5 Wood units, the pulmonary vascular lack of donors, the mean donor age is steadily
resistance index (PVRI) is >6 or the transpulmo- increasing, further reducing the acceptance of
nary gradient (TPG) exceeds 16–20 mmHg potential heart grafts. Therefore, optimal donor
(Mehra et al. 2006). management and improved evaluation and utili-
zation of cardiac grafts are mandatory.
The care of brain-dead donors should involve
37.1.3 rgan Donation, Evaluation
O cardiologists, pulmonologists, intensive care spe-
and Allocation cialists and surgeons. Their goal should be optimi-
zation of the hemodynamic management,
Organ donation rates vary significantly between maintaining a neutral acid-base balance and cor-
the countries in Western Europe. While the recting hormonal perturbations. Treatment with
Eurotransplant members Croatia, Austria and insulin, corticosteroids, triiodothyronine and
Belgium as well as Spain achieve donor rates per arginine vasopressin as well as blood transfusions
million inhabitants far above 20, Germany only in cases of severe anemia might improve cardiac
reaches an annual quota of about 10–15 per mil- function (Novitzky et al. 1984; Zaroff et al. 2002).
lion. Besides organizational variations, the major Older donors (>55 years of age) can be used
difference is how consent is regulated by law. Most particularly for higher-risk or older recipients if
countries have implemented donation rules based other risk factors (left ventricular hypertrophy,
on presumed consent while Germany still relies ischemic time) are not significant. In donors aged
on informed consent. The new German trans- 46–55 years, coronary angiography is recom-
plantation law, however, introduced several mea- mended; older donors should have it routinely.
sures to improve donation rates (e.g. in house Minor coronary plaques in angiography or ele-
coordinators and regular education and informa- vated cardiac enzymes without the presence of
tion of the population) but did not alter the other donor risk factors do not justify nonuse of a
underlying informed consent (which is now donor heart (Zaroff et al. 2002).
called decisional consent) system. Heart grafts should be declined if there is echo-
In the United States, organ donation is also cardiographic and ECG evidence of substantial
based on informed consent. Similar to other left ventricular hypertrophy, severe valvular or
1084 B. Meiser and B. Reichart
congenital cardiac abnormality or if the expected are set by OPTN/UNOS. All patients waiting for
cold ischemic time exceeds 6 h. The value of echo- an organ from a deceased donor are required to
cardiography to determine the physiological suit- be placed in the common waiting list database; a
ability of a donor heart is limited. Particularly computer algorithm for allocation of each type of
younger hearts with left ventricular dysfunction organ matches donor and recipient. The current
can recover normal function over time (Zaroff allocation algorithm prioritizes matching patients
et al. 2002). in the local OPO service area, followed by
A growing number of recipients are pre-sensi- regional and then national candidates.
tized mainly due to former blood transfusions or a
previous transplantation. The pre-existing antibod-
ies might result in antibody mediated rejection, 37.1.4 The Donor Operation
either hyeracute or chronic, associated with hemo-
dynamic compromise, increased graft loss and car- 37.1.4.1 onor Operation for Orthotopic
D
diac allograft vasculopathy. Solid-phase assays to Heart Transplantation Using
determine antibody presence in the recipient have Atrial Anastomoses (Lower et al.
allowed for the development of a calculated panel- 1965)
reactive antibody to avoid donors harboring the Before starting the surgery to explant the donor
corresponding unacceptable antigens (Kobashigawa heart, the matching blood groups of donor
et al. 2011; Chang et al. 2012). Recently a portable respectively recipient should be checked similar
warm blood perfusion system for donor hearts to procedures before blood transfusions. Due to
(organ care system) has been introduced. The the immaturity of the immune system of new-
machine maintains the heart in a functioning status borns and infants, ABO-incompatible trans-
outside of the body. The technology might allow to plantation my be an option in that age group.
increase the time between explanation and implan- Low isohemagglutinin levels are a prerequisite
tation (allowing longer transport or recipient prep- (West et al. 2001).
aration times), to assess the function more After median sternotomy the pericardium
thoroughly and to improve function or to enable opened. The heart is carefully inspected, particu-
resuscitation of the organ. Another perfusion sys- larly for possible signs of coronary artery disease.
tem developed in Lund, Sweden, also revealed Contractility of both the right and the left ventri-
promising results (Steen et al. 2016). Further evalu- cle is assessed. After the final decision to explant
ation of the benefit of the systems, also considering the organ is made, preparations begin: First, the
the substantial costs for its use, is necessary. ascending aorta is separated from the pulmonary
In Germany as well as in currently six other trunk. The superior vena cava (SVC) is exposed
European states, donor organs are allocated by the all the way up to its junction with the azygos vein
37 Eurotransplant International Foundation in and the inferior vena cava (IVC) down into the
Leiden, the Netherlands. Allocation guidelines diaphragm. The course of both the right and the
are being continuously further developed by left pulmonary veins is noted. Heparin (2.5–
national authorities. In Germany, the German 3.0 mg/kg i.v.) is administered. Next, the SCV is
Medical Association determines the rules based divided between two ligatures. The vessel should
on an organ-specific common waiting list and a be divided up high, as close as possible to the azy-
patient-specific allocation system. While alloca- gos vein (. Fig. 37.2). This minimizes possible
tion regulations should consider urgency and suc- damage to the sinus node. The ascending aorta is
cess according to the German transplantation law, cross clamped proximal to the brachiocephalic
heart allocation is currently predominantly attrib- trunk, after which the cardio-protective solution
uted to urgency. The vast majority of patients is inserted into the aortic root. The IVC is clamped
transplanted in Germany are therefore belonging directly at the diaphragmatic level and incised
to the so-called high-urgency status group. proximally in order to empty the right heart. An
In the United States, the United Network for incision into one of the pulmonary veins empties
Organ Sharing (UNOS), based in Richmond, VA, the left heart. During infusion of cold cardiople-
administers the national Organ Procurement and gic solution, further cooling is achieved by filling
Transplantation Network (OPTN), in which each the pericardium with ice-cold, normal saline
OPO is a member. The rules for organ allocation solution.
Chapter 37 · Heart and Heart–Lung Transplantation
1085 37
If one is still not able to discontinue the heart–lung level of the SVC respectively IVC extended to a
machine support—and if the anesthetist has to point approximately 2 cm anterior to the right
increase the amount of catecholamines, especially phrenic nerve. This pericardial flap is laid over
adrenaline—the quick decision for the insertion of the hilum of the right lung and serves as a cradle
an extracorporeal membrane oxygenation system for the donor heart. The transplanted organ will
is life-saving, in our experience in about half of the be located within the right chest and partially
cases. The circulation cannulas are then connected compresses the right middle and lower lobe of
to the right atrium and the ascending aorta; the lung.
heparin-coated lines should be used. The chest is After commencing extracorporeal circula-
then enclosed with a plastic patch which is attached tion, the body temperature is cooled to 30 °C, the
to the skin using running suture. ascending aorta is cross clamped and the cardio-
plegic solution is infused into the recipient’s
37.1.5.2 Bicaval Anastomoses: heart. In addition ice-cold physiologic saline
Orthotopic Heart solution is applied topically. The donor heart is
Transplantation (Dreyfus et al. now placed in the right chest, the opening within
1991) the left atrium facing anteriorly (. Fig. 37.8).
Bicaval anastomoses might generate an undisturbed This is followed by a longitudinal incision into
flow within the right atrium, when compared with the recipient’s left atrium through the interatrial
the original Lower and Shumway technique; bicaval groove, as of for mitral valve surgery. Both left
connections are essential in patients with certain atria are connected to each other by a continu-
congenital cardiovascular lesions and their correc- ous suture line of double-armed 4–0 polypropyl-
tions. The Paris group described in their paper not ene. One half of the suture is laid clockwise, the
only the end-to-end anastomosis of SVC respec- other half counterclockwise, and finally tied at
tively IVC but also the separate implantation of the
left and the right pulmonary vein pairs—like for
solitary lung transplantations.
Sarsam and coworkers modified in 1993 this
original Dreyfus technique by leaving the pulmo-
nary vein within the remnants of the left atrium as
in classic orthotopic heart transplantation
(Sarsam et al. 1993).
..Fig. 37.9 The right atrial anastomosis is performed ..Fig. 37.10 The donor aorta is anastomosed end to
with a running suture starting at the midpoint of the side to the ascending aorta of the recipient. The main
recipient incision as shown in Fig. 37.8. The pulmonary artery of the donor is then extended with a
diamond-shaped anastomosis is then finished in a circular vascular prosthesis which is finally anastomosed end to
way like for the left atrium side to the recipient’s main pulmonary artery
1090 B. Meiser and B. Reichart
the right chest) are inserted. It is also recom- adhesions, which have formed between the donor
mended that pairs of temporary epicardial pacing heart and the right lower lobe, present a great
wires are placed onto each heart. Then, the chest challenge. Multiple lung leaks are, therefore, not
is closed in a routine fashion. infrequent and pose a source of infection in an
The right-sided donor heart within the right area of blood clots and extensive dissection. In
pleural cavity impedes expansion of the middle order to make these procedures easier and safer,
and lower right lung lobes—a situation which is orthotopic replacement of the recipient’s old heart
usually well tolerated. After heterotopic proce- is recommended, leaving the heterotopic donor
dures, patients need heparin immediately fol- heart untouched. As a result, the patient is left with
lowed by long-term coumadin in order to prevent two different donor hearts—a situation which is
thrombus formation within the left ventricular well tolerated. The two other possibilities—
cavity of the recipient heart. replacement of the rejected heterotopic heart or its
removal followed by orthotopic transplantation—
37.1.5.4 Transplantation in Patients are not favored because of severe bleeding compli-
with Mechanical Circulatory cations and a high risk of infection. The latter
Support Devices technique, changing from a heterotopic to an
After implantation of a mechanical circulatory orthotopic situation, is especially unfortunate,
support device, severe adhesions must be since it creates an empty space previously filled by
expected. The adhesions are the result of foreign the donor heart. Decortication of the right lung
surfaces, in permanent non-physiological move, would then be necessary (Novitzky et al. 1987).
with heat generation and chronic inflammations/
infections. It is therefore safe to connect the
patient to extracorporeal circulation first, utilizing 37.2 Heart Transplantation
the groin vessels; a long venous cannula may be in Neonates and Infants
advanced into the IVC. Later on, when the recipi-
ent heart and the great vessels are dissected out, 37.2.1 Indications and Outcome
only the SVC must be accessed for full venous
return; venous tourniquets are snared. Terminal heart diseases necessitating transplanta-
If extremely severe adhesions are encoun- tions at a younger age may be caused by systolic
tered, the SVC cannula may be introduced percu- pump failure, most probably due to hereditary
taneously via the right jugular vein. dilated cardiomyopathies or in the follow-up of
The ascending aorta will then be cross valvular malformations at the aortic or atrioven-
clamped, allowing the surgeon to work on an tricular level (Dipchard et al. 2015). Cases of
open and empty heart, a step which greatly facili- myocarditis are a primary indication for left or
37 tates further dissections. Once the in- and outflow biventricular assist devices; transplants seem to be
tubes of the assist system and the device itself are only indicated after irreversible cardiac damage in
removed, this may take 2–3 h, the actual heart spite of a lengthy treatment period. Bland–White–
transplantation can begin. Garland syndrome or Kawasaki syndrome caus-
At the end of the operation and with good ing myocardial infarctions are rare. Patients with
control of bleeding sites, the remaining cavities of corrected transposition of the great vessels or
the previous assist system are also drained. In those after atrial switch operations (Senning or
cases of local infections, irrigation lines are advis- Mustard procedure) have two ventricular cham-
able as well as i.v. antibiotics according to bacte- bers (see 7 chapter «Congenitally Corrected
rial cultures and their resistance profile. Transposition and D-transposition of the Great
Arteries», Sect. 19.2.5.1); the systemic ventricle is,
37.1.5.5 Cardiac Re-transplantation however, the anatomical right one—and as such
Acute or chronic rejection crises may necessitate not suited for lifelong function to generate sys-
re-transplantations. After orthotopic implantation temic pressures; severe systemic heart failure may
of the heart, this operation differs little from other be the result.
reoperations that form part of a cardiac surgeon’s Morbus Ebstein and Uhl’s disease (right ven-
routine. However, following heterotopic opera- tricular dysplasia) are rare causes of isolated ter-
tions, greater difficulties are encountered. Lung minal right ventricular failure.
Chapter 37 · Heart and Heart–Lung Transplantation
1091 37
Endocardial fibroelastosis and diffuse, hyper- branches are isolated and loosely taped. Heparin
trophic cardiomyopathy cause terminal diastolic is given and the venous cannula inserted into the
pump disorders. right atrium.
Different types of univentricular circulations The arterial cannula is inserted via the distal
may necessitate transplantations mostly during main pulmonary artery through the patent ductus
adolescence. This very complex group of con- into the descending aorta and extracorporeal cir-
genital cardiac diseases comprises many lesions culation is commenced immediately. A loose tape
and includes tricuspid atresia, double inlet left placed around the patent ductus is tightened after
ventricle, mitral/aortic atresia, respectively, full bypass.
hypoplasia (see 7 Chapter «Definite Palliation of The recipient is now cooled to 18 °C. Bags of
Functional Single Ventricle», Sect. 13.3). crushed ice around the head are additionally used
The majority of patients just mentioned pres- to protect the brain. Deep hypothermic circulatory
ent after previous thoracic surgery, like bilateral arrest is instituted and the patient’s blood drained
Glenn anastomosis, various modifications of into the reservoir, both cannulas are removed.
Fontan procedures including multistage pallia- Before that, the supraaortic vessels were snared.
tions like Norwood operations (see 7 chapter The excision of the heart commences at the level
«Surgery for Aortic Atresia, Hypoplastic Left Heart of the left and right atria, just above the AV valves.
Syndrome, and Hypoplastic Left Heart Complex», The main pulmonary artery is transected at the
Sect. 17.6.2). bifurcation, the patent ductus close to the descend-
Transplant vasculopathy is also a limiting factor ing aorta. On the side of the pulmonary artery, the
in pediatric cases, with an incidence of 2, 9 and 17 % ductus is ligated or suture closed. The diminutive
(after 1, 3 and 5 years), numbers which are much ascending aorta is cut. The concavity of the aortic
lower when compared with adult transplantation arch is incised, from the level of the brachiocephalic
patients; new-onset terminal heart failure was seen trunk to a point distal of the transected ductus.
in 1, 4 and 9 % after 1, 5 and 9 years (Pahl et al. 2005). The donor heart is removed as for routine har-
Re-transplantations are possible; their long-term vest, with the exception that the full ascending aorta
results are, however, inferior, if compared with pri- and the arch are retained. All aortic arch vessels are
mary interventions (Chin et al. 2006). tied off, except for the brachiocephalic trunk
This brief overview, which by far is not a com- through which cardioplegic solution is infused. The
plete compilation of possible indications (Rosenthal donor main pulmonary artery branches are tran-
et al. 2004), proves that the rare pediatric cardiac sected separately, and one large opening is created
transplantation should be reserved for those few by cutting the tissue bridge at the bifurcation.
centers who have experience in both cardiothoracic Implantation of the donor heart is carried out
transplantation and surgical correction of congeni- with continuous 6 or 7–0 polypropylene sutures,
tal heart disease and their follow-up. Under these beginning with the left and right atria, the original
circumstances, follow-up results of pediatric car- Shumway technique. The pulmonary artery anas-
diac transplantations are good and surpass those of tomosis is facilitated by the relatively large donor
adults (Groetzner et al. 2005; Ross et al. 2006). vessel circumference created by joining the right
and left main branches. The donor aorta is cut
obliquely behind the brachiocehalic trunk in
37.2.2 rimary Orthotopic Heart
P order to match the incision made in the aortic
Transplantation Technique arch and isthmus of the recipient. After finishing
Used for Hypoplastic Left the aortic anastomoses, extracorporeal circula-
Heart Syndrome (HLHS) tion is reinstituted by reinserting a venous can-
nula into the atrium; an aortic cannula is placed
In 1985, L. Bailey and coworkers from Loma into the arch via the open brachiocephalic trunk.
Linda performed the first transplants in neonates After air is evacuated, rewarming to 37 °C starts.
with hypoplastic left heart syndrome (Bailey et al. Thereafter, bypass is discontinued and protamine
1986). given. Hemostasis is achieved, pacing wires and
A sternotomy is performed and the pericar- drains are inserted and the chest is closed.
dium opened longitudinally. The thymus is almost While in the 1980s and early 1990s, neonatal
completely removed. Thereafter, the aortic arch cardiac transplantation was a viable alternative to
1092 B. Meiser and B. Reichart
Norwood-type palliations for HLHS, nowadays In cases of TGA, the (donor) pulmonary
primary choice for HLHS patients, is conven- artery trunk must be anastomosed to the left
tional Norwood surgery. It can be expected, how- (recipient) pulmonary artery branch, the anasto-
ever, that some of the Fontan children (‘Stage III mosis augmented with an incised (autologous
Norwood’) will need transplantations in the preferable) pericardial patch.
future due to the fact that the univentricular An unrestricted venous return is difficult to
(right) ventricle has to support the systemic circu- achieve in patients with situs inversus. The
lation and may fail in addition to the shortcom- description of a case may serve as an example:
ings of a Fontan circulation. dextrocardia with a univentricular heart (with
hypoplastic left ventricle, ASD, VSD and mitral
atresia) and TGA; during the previous Fontan
37.2.3 Techniques of Heart intervention, the left-sided right atrial auricle was
Transplantation After Fontan connected to the left pulmonary artery. An azygos
Surgery for Single Ventricle drainage of the IVC complicated the situs inversus
position of the SVC; hepatic veins drained into
The ascending aorta or the femoral artery serve as the atrium directly on the left.
arterial access, IVC (or extracardiac tunnel) and The femoral artery served as arterial access;
SVC (or innominate vein) as venous return. After the lower part of the body was drained with a
commencing extracorporeal circulation, the long cannula which was advanced from the
ascending aorta is cross clamped and the venous femoral into the azygos vein. The anonymous
return snared with tourniquets. The recipient heart and hepatic veins were further drained by two
and the great vessels are freed from adhesions separate cannulas.
which are usually severe due to multiple previous Cross clamping of the ascending aorta was fol-
interventions (it would therefore be important to lowed by the removal of the dissected heart. The
always reconstruct the precardial pericardium implantation started at the left atrial level, after
with a PTFE membrane (Gore-Tex®) at the end of rotating the donor organ counterclockwise by
each complex pediatric intervention before closing 30–45°; by doing so, an end-to-end anastomosis
the chest—to facilitate future interventions). of the donor IVC and the connecting vessel of the
A Glenn-anastomosis, atrio-pulmonary con- hepatic veins became possible. Both recipient pul-
nections, respectively an extracardiac tunnel, monary artery branches were enlarged with an
both cranial and caudal of the right pulmonary adequately trimmed (and partially incised) autol-
artery branch, are taken down and their openings ogous pericardial patch, which was then con-
closed, preferably utilizing autologous pericar- nected with the donor pulmonary artery trunk.
dium. An extracardial tunnel will be removed and After the end-to-end anastomosis of both ascend-
37 the IVC transected. ing aortic stumps, the confluence of the azygos
The implantation follows the modified ortho- drainage with the SVC was connected to the right
topic technique by Sarsam and coworkers (1993) as atrium directly using a large-sized ring-stabilized
described in 7 Sect. 37.1.5.2, «Bicaval Anastomoses: Gore-Tex® prosthesis; alternatively a pulmonary
Orthotopic Heart Transplantation (Dreyfus et al. or aortic homograft may have been chosen. The
1991)». It is advisable to keep the SVC long (to a orifice of the donor SVC was finally oversown.
point where the anonymous vein merges).
Two additional vascular anomalies, may be
worthwhile mentioning: A persistent left-sided SVC 37.3 Heart–Lung Transplantation
(without anonymous vein connection to the right
side) and the transposition of the great arteries (TGA). 37.3.1 History and State of the Art
If the presence of a left-sided SVC is known in
advance, the innominate (donor) vein may be dis- The first attempts of combined heart and lung
sected and left attached to the SVC; the innomi- transplantation were reported by Cooley 1968 and
nate (donor) vein will later be anastomosed end to Lillehei in 1969, respectively. In those times immu-
end to the (recipient) left superior caval vein. nosuppressive therapy was crude, consisted of only
Hemodynamically significant left-sided SVCs corticosteroids and azathioprine, and therefore the
need an extra venous cannula for drainage. results were disappointing. It was decided that
Chapter 37 · Heart and Heart–Lung Transplantation
1093 37
heart–lung transplantation techniques render no tal heart diseases (and originally unrestricted
long-term results and have therefore no future. A pulmonary artery flow) causing Eisenmenger
decade later, however, Bruce Reitz from Stanford syndrome (extreme cases of pulmonary atresia
University proved in non-human primates the without distal vessels are another, however, rare
opposite, using cyclosporine A as primary immu- indication).
nosuppressive agent (Reitz et al. 1980). In 1981, he Under these circumstances the pulmonary
performed the first successful human heart–lung artery pressure is at systemic level or above;
transplantation, and the 45-year-old female patient concomitant severe tricuspid incompetence is
survived more than 4 years. present.
In 2014, according to the ISHLT statistics, less Echocardiography, x-ray, computer tomogra-
than 100 heart–lung transplantations were done phy, and magnet resonance techniques verify the
worldwide. Congenital cardiac lesions with severe diagnoses. Lung function tests reveal restrictive
pulmonary hypertension were the main indica- and obstructive lesions.
tion (Yusen et al. 2015; Goldfarb et al. 2015). In
our own experience with 36 patients receiving
combined heart and lung transplantations at the 37.3.3 Evaluation of Donors
University of Munich Medical Center after 1996, for Combined Heart and
71 and 63 % survived 1 and 10 years, compared Lung Transplantation
with 50 and 28 % of those treated between 1983
and 1996. Patients with congenital cardiac lesions The cardiac function should be checked as
were younger and lived longer: of those who were described in paragraph 7 Sect. 37.1.3, «Organ
under 18 years of age at the time of the operation, Donation, Evaluation and Allocation».
82 and 71 % survived 1 and 10 years. Special considerations include freedom from
any pulmonary impairment or serious lung con-
tusion. Careful attention is directed toward clear-
37.3.2 Indications ing pulmonary secretions with regular gentle
aspiration via the endotracheal tube; retrieved
During the last decade, combined heart and lung aspirate is sent for culture and direct examination.
procedures were indicated in fewer and fewer The tracheal aspirate should not show gross con-
patients—this was due to the success of bilateral tamination, particularly with candida. A bron-
lung transplantations even in patients with mod- choscopy is always indicated to rule out severe
erate to severe primary pulmonary hypertension. (sometimes hidden) bronchial infection.
Bilateral lung transplants were even successfully A size match is more important in combined
performed in patients with congenital cardiac heart–lung than in lung transplantation only. In
malformations (and Eisenmenger reaction). order to match for size, measurements of the max-
Under these preconditions concomitant correc- imal thoracic diameters and the heights of the
tions of the heart lesions are necessary—a lengthy thoracic cavities are compared (. Fig. 37.11).
operation not without risks. In straightforward Ideally, the donor chest should be slightly smaller
cases without previous interventions, it may than that of the recipient to avoid postoperative
therefore still be simpler and safer to decide for a atelectasis that would inevitably follow the inabil-
combined transplant of both lungs and the heart. ity of larger lungs to expand within a smaller chest
In combined heart and lung transplantations cavity. Mismatches of up to 10 % may be accepted.
in primary pulmonary hypertension patients, one Too big lungs may produce severe impairment of
may consider the ‘domino heart’ strategy whereby cardiac function, once the chest will be closed.
the non-malformed (right sided) hypertrophied The only solution would then be an atypical lung
(recipient) heart is transplanted into another per- resection or the removal of the left lower lobe.
son with somewhat elevated pulmonary artery Functional parameters include a peak infla-
pressure but otherwise acceptable pulmonary tion pressure on the respirator being less than
function (Yacoub et al. 1990). 30-cm H2O with a minute ventilation of 15 cc/kg
Heart–lung transplants are indicated in at eight breaths per minute. Adequate gas
NYHA class III–IV and IV patients with termi- exchange is mandatory, with an arterial oxygen
nal double organ failure, mostly due to congeni- tension of more than 100 mmHg on a FiO2 of 0.4.
1094 B. Meiser and B. Reichart
..Fig. 37.11 Thoracic
diameters allowing a
comparison between
donor and recipient
measures: 1 sternal length
(jugulum to xiphoid), 2
thoracic aperture, 3
jugulum to acromion, 4 3
thoracic diameter at the
height of the mammillae, 5
1 2
thoracic diameter at the
height of the thoracic 4
aperture
In a trial with the FiO2 being increased to 1.0, the are dissected; the arterial ligament is transected;
PO2 should rise to more than 300 mmHg. Positive on the right, the ascending aorta and SVC are
end-expiratory pressure of 3–5-cm H2O is main- freed of pulmonary artery tissue. Care must be
tained to prevent atelectasis. taken not to damage the upper lobe pulmonary
In recent years these just-describe ‘classic’ artery. Anterior traction on the aorta and the bra-
principles of pulmonary harvesting were success- chiocephalic trunk facilitates the exposure of the
fully extended following the lead of Patterson and trachea which is located just distal to, and slightly
Cooper (1988) and Aigner et al. (2005). A history to the right of the vessels. The trachea is freed,
of smoking is no more important as are small with care being taken to leave the peritracheal
lung damages, which can be excluded using sta- connective tissue intact as far as possible, espe-
37 pler devices. The donor age is being increased cially 2 cm above the carina.
continuously. The SVC is then divided in between two liga-
tures. A straight vascular clamp is used to occlude
the IVC which is then divided proximally. Once
37.3.4 Donor Operation the right heart is decompressed, the ascending
aorta is cross clamped as distal as possible. Ice-
After median sternotomy, the pericardium is cut cold cardioplegia is given (see 7 Sect. 37.1.3,
longitudinally and resected on both sides down as «Organ Donation, Evaluation and Allocation»); the
far as the pulmonary veins, while both phrenic pulmonary protection with 4,000 ml LPD solu-
nerves are divided and the thymus gland is tion (Perfadex®; Müller et al. 1999) is started with
removed. added Tris buffer (0.5 ml/l).
Heparin is now administered intravenously. It is recommended that 500-mg epoprostenol
The SVC and IVC are exposed, as well as the azy- (Flolan®) is administered before infusing the
gos vein; the latter vessel is doubly ligated and Perfadex solution in order to dilate the pulmo-
transected. Umbilical tape, passed around the nary arterioles. The tip of the left atrial appendage
ascending aorta, is used to pull the aorta away is cut to decompress the left ventricle. A few liters
from the pulmonary trunk. Then the bifurcation, of ice-cold saline solution are used for continuous
the right and the left pulmonary artery branches external cooling.
Chapter 37 · Heart and Heart–Lung Transplantation
1095 37
Finally, the ascending aorta is divided imme-
diately proximal to the brachiocephalic trunk.
The lungs are inflated to about one third when
the trachea is clamped 4 cm above the carina.
With the clamp in place, the trachea is divided
proximally. The clamp is pulled gently anterior
by the assistant. The back walls of the trachea
and of the main bronchi are dissected off the
esophagus in a cranial to caudal direction. The
remainder of the pericardium and the pleura are
divided, as well as the right and the left pulmo-
nary ligaments.
For transportation, the trachea is closed with a
stapling device and the clamp removed. Thereafter
the block of organs is secured in three plastic bags ..Fig. 37.12 Left- and right-sided pericardial pedicle
incorporating the left and right phrenic nerves. After the
filled with ice-cold saline solution.
excision of the recipient’s heart, the dorsal portions of the
left and right atrial structures are exposed. Aorta and
main pulmonary artery are transected. The excision of
37.3.5 Organ Implantation both lungs starts at the dorsal wall of the left atrium
in Combined Heart–Lung which is split in between the left respectively the right
pulmonary pair of veins.
Transplantation (Reitz et al.
1980)
rhabdomyolysis, mostly when used in higher heart transplantation. Curr Opin Organ Transplant
doses. 17:423–426
Chin C, Naftel D, Pahl E et al. (2006) Cardiac re-
Chronic immunosuppression is also a risk fac- transplantation in pediatrica: a multi-institutional
tor for the development of malignancies. Since the study. J Heart Lung Transplant 25:1420–1424
most common tumor after transplantation is skin Cooney GF, Jeevanandam V, Choudhury S et al. (1998)
cancer, transplanted patients should undergo fre- Comparative bioavailability of Neoral and
quent dermatologic surveillance. In cases of lym- Sandimmune in cardiac transplant recipients over 1
year. Transplant Proc 30:1892–1894
phomas adjuvant therapy should be minimization DiBardino DJ (1999) The history and development of car-
of immunosuppression as much as possible (Hunt diac transplantation. Tex Heart Inst J 26:198–205
et al. 2012). Dipchand AI, Rossano JW et al. (2015) The Registry of the
International Society for Heart and Lung
Transplantation: Eighteenth Official Pediatric Heart
Transplantation Report–2015; Focus Theme: Early
References Graft Failure. J Heart Lung Transplant 34:1233–1243
Dreyfus G, Jebara V, Mihaileanu S, Carpentier AF (1991) Total
Aaronson KD, Schwartz JS, Chen TM et al. (1997) orthotopic heart transplantation: an alternative to the
Development and prospective validation of a clinical standard technique. Ann Thorac Surg 52:1181–1184
index to predict survival in ambulatory patients Groetzner J, Reichart B, Roemer U et al. (2005) Cardiac trans-
referred for cardiac transplant evaluation. Circulation plantation in pediatric patients: fifteen-year experience
95:2660–2667 of a single center. Ann Thorac Surg 79:53–61
Aigner C, Winkler G, Jaksch P et al. (2005) Extended donor Goldfarb SB, Benden C, Edwards LB et al. (2015) The
criteria for lung transplantation—a clinical reality. Eur Registry of the International Society for Heart and
J Cardiothorac Surg 27:757–761 Lung Transplantation: Eighteenth Official Pediatric
Aliabadi A, Grömmer M, Zuckermann A (2011) Is induction Lung and Heart-Lung Transplantation Report–2015;
therapy still needed in heart transplantation? Curr Focus Theme: Early Graft Failure. J Heart Lung
Opin Organ Transpl 16:536–542 Transplant 34:1255–1263
Bailey L, Concepcion W, Shattuck H, Huang L (1986) Groetzner J, Kaczmarek I, Schulz U et al. (2009)
Method of heart transplantation for treatment of Mycophenolate and sirolimus as calcineurin inhibitor-
hypoplastic left heart syndrome. J Thorac Cardiovasc free immunosuppression improves renal function bet-
Surg 92:1–5 ter than calcineurin inhibitor-reduction in late cardiac
Bardy GH, Lee KL, Mark DB et al. (2005) Amiodarone or an transplant recipients with chronic renal failure.
implantable cardioverter-defibrillator for congestive Transplantation 87:726–733
heart failure. N Engl J Med 325:225–235 Gronda E, Bourge RC, Costanzo MR et al. (2006) Heart rhythm
Barnard CN (1967) The operation. A human cardiac trans- considerations in heart transplant candidates and con-
plant: an interim report of a successful operation per- siderations for ventricular assist devices: International
formed at Groote Schuur Hospital, Cape Town. S Afr Society for Heart and Lung Transplantation Guidelines
Med J 41:1271–1274 for the Care of Cardiac Transplant Candidates—2006.
Belzer FO, Southhard JH (1988) Principles of solid-organ pres- J Heart Lung Transplant 25:1043–1056
ervation by cold storage. Transplantation 21:673–686 Hölscher M, Groenewoud AF (1991) Current status of the
37 Billingham ME (1981) Diagnosis of cardiac rejection by HTK solution of Bretschneider in organ preservation.
endomyocardial biopsy. Heart Transpl 1:25–30 Transplant Proc 23:2334–2337
Bretschneider HJ, Hübner G, Knoll D et al. (1975) Myocardial Hübner T, Nickel T, Steinbeck G et al. (2015) A single
resistance and tolerance to ischemia: physiological an German center experience with intermittent inotro-
biochemical basis. J Cardiovasc Surg 16:241–260 pes for patients on the high-urgent heart transplant
Cantarovich M, Giannetti N, Barkun J et al. (2004) waiting list. Clin Res Cardiol 104:929–934
Antithymocyte globulin induction allows a prolonged Hunt SA, Burch M et al. (2012) ISHLT Guidelines for the care
delay in the initiation of cyclosporine in heart trans- of heart transplant recipients. Part 3: Longterm care.
plant patients with postoperative renal dysfunction. ISHLT Monograph Series 6:173–246
Transplantation 78:779–781 Jacobs S, Rega F, Meyns B (2010) Current preservation
Carrel A, Guthrie CC (1905) The transplantation of veins technology and future prospects of thoracic organs.
and organs. Am Med 10:1101–1102 Part 2: heart. Curr Opin Organ Transplant 15:156–
Carrier M, White M, Perrault LP et al. (1999) A 10-year expe- 159
rience with intravenous thymoglobulin in induction of Jessup M, Banner N, Brozena S et al. (2006) Optimal pharma-
immunosuppression following heart transplantation. cologic and non-pharmacologic management of car-
J Heart Lung Transplant 18:1218–1223 diac transplant candidates: approaches to be
Caves PK, Stinson EB, Billingham ME, Shumway NE (1974) considered prior to transplant evaluation: International
Serial tansvenous biopsy of the transplanted human Society for Heart and Lung Transplantation Guidelines
heart. Improved management of acute rejection epi- for the Care of Cardiac Transplant Candidates—2006.
sodes. Lancet 1:821–826 J Heart Lung Transplant 25:1003–1023
Chang D, Kobashigawa J (2012) The use of the calculated Kajihara N, Morita S, Tanoue Y et al. (2006) The UW solution
panel-reactive antibody and virtual crossmatch in has greater potential for longer preservation periods
Chapter 37 · Heart and Heart–Lung Transplantation
1101 37
than the Celsior solution: comparative study for ven- Morris RE, Meiser BM (1989) Identification of a new phar-
tricular and coronary endothelial function after 24-h macologic action for an old compound. Med Sci Res
heart preservation. Eur J Cardiothorac Surg 29: 17:609–610
784–789 Moss AJ, Zareba W, Hall WJ et al. (2002) Prophylactic
Kobashigawa J, Miller L, Renlund D et al. (1998) A random- implantation of a defibrillator in patients with myocar-
ized active controlled trial of mycophenolate mofetil in dial infarction and reduced ejection fraction. N Engl
heart transplant recipients. Transplantation 66:507–515 J Med 346:877–883
Kobashigawa J, Mehra M, West L et al. (2009) Consensus Müller C, Fürst H, Reichenspurner H, Briegel J et al. (1999)
conference: participants report from a consensus con- Lung procurement by low-potassium, dextran an the
ference on the sensitized patient awaiting heart trans- effect on preservation injury. Transplantation 68:
plantation. J Heart Lung Transplant 28:213–225 1139–1143
Kobashigawa J, Crespo-Leiro MG, Ensminger SM et al. Nathan HM, Conrad SL, Held PJ et al. (2003) Organ dona-
(2011) Report from a consensus conference on anti- tion in the United States. Am J Transplant 3(Suppl
body-mediated rejection in heart transplantation. 4):29–40
J Heart Lung Transplant 30:252–269 Novitzky D, Cooper DK, Barnard CN (1983) The surgical
Lehmkuhl H, Arizon J, Vigano M et al. (2009) Everolimus technique of heterotopic heart transplantation. Ann
with reduced cyclosporine versus MMF with standard Thorac Surg 36:476–482
cyclosporine in de novo heart transplant recipients. Novitzky D, Wicomb WN, Cooper DKC et al. (1984)
Transplantation 88:115–122 Electrocardiographic, haemodynamic an endocrine
Losman JG, Barnard CN, Barcaly TD (1977) Hemodynamic changes occurring during expertmental brain death in
evaluation of left ventricular bypass with a homologous the Chacma baboon. J Heart Transplant 4:63–69
cardiac graft. J Thorac Cardiovasc Surg 74:695–708 Novitzky D, Cooper DKG, Lanza RP, Barnard CN (1985)
Lower RR, Shumway NE (1960) Studies on the orthotopic Further cardiac transplant procedures in patients with
homotransplantations of the canine heart. Surg Forum heterotopic heart transplants. Ann Thorac Surg
11:18–19 39:149–154
Lower RR, Stofer RC, Hurley EJ et al. (1962) Successful Novitzky D, Cooper DKC, Brink JG et al. (1987) Sequential—
homotransplantations of the canine heart after anoxic second and third—transplants in patients with het-
preservation for seven hours. Am J Surg 104:302 erotopic heart allografts. Clin Transplant 1:57–62
Lower RR, Dong E, Shumway NE (1965) Long-term survival Oyer P, Stinson E, Jamieson S et al. (1983) CYA in cardiac
of cardiac homografts. Surgery 58:110–119 transplantation: a 2 1/2 year follow-up. Transplant Proc
Lund LH, Edwards LB, Kucheryavaya AY et al. (2015) The 15:2546
Registry of the International Society for Heart and Lung Pahl E, Naftel DC, Kuhn MA et al. (2005) The impact and
Transplantation: Thirty-second Official Adult Heart outcome of transplant coronary artery disease in a
Transplantation Report–2015; Focus Theme: Early Graft pediatric population: a 9-year multi-institutional
Failure. J Heart Lung Transplant 34:1244–1254 study. J Heart Lung Transplant 24:945–951
Maathuis MHJ, Leuvenink HGD, Ploeg RJ (2007) Perspectives Patterson GA, Cooper JD (1988) Status of lung transplanta-
in organ preservation. Transplantation 83:1289–1298 tion. Surg Clin North Am 68:545–558
Mehra MR, Kobashigawa J, Starling R (2006) Listing criteria Pham SM, Kormos RL, Hattler BG et al. (1996) A prospective
for heart transplantation: International Society for trial of tacrolimus (FK506) in clinical heart transplanta-
Heart and Lung Transplantation Guidelines for the tion: intermediate-term results. J Thorac Cardiovasc
Care of Cardiac Transplant Candidates—2006. J Heart Surg 111:764–772
Lung Transplant 25:1024–1042 Reichart B, Meiser BM, Viganò M et al. (1998) European
Meiser BM, Überfuhr P, Fuchs A et al. (1998) Single-center multicenter tacrolimus (FK506) heart pilot study; one-
randomized trial comparing tacrolimus (FK506) and year results—European tacrolimus multicenter heart
cyclosporine in the prevention of acute myocardial study group. J Heart Lung Transplant 17:775–781
rejection. J Heart Lung Transplant 17:782–788 Reitz BA, Burton NA, Jamieson SW et al. (1980) Heart and
Meiser BM, Pfeiffer M, Schmidt D et al. (1999) Combination lung transplantation, autotransplantation and allo-
therapy with tacrolimus and mycophenolate mofetil transplantation in primates with extended survival.
following cardiac transplantation: importance of J Thorac Cardiovasc Surg 80:360–372
mycophenolic acid therapeutic drug monitoring. Rosenthal D, Chrisant MRK, Edens E et al. (2004) ISHLT:
J Heart Lung Transplant 18:143–149 practice guidelines for management of heart failure in
Meiser BM, Kaczmarek I, Müller M et al. (2007) Low-dose children. J Heart Lung Transplant 23:1313–1333
tacrolimus/sirolimus and steroid withdrawal in heart Ross M, Kouretas P, Gamberg P et al. (2006) Ten- and
recipients is highly efficacious. J Heart Lung Transplant 20-year survivor of pediatric orthotopic heart trans-
26:598–603 plantation. J Heart Lung Transplant 25:261–270
Meiser B, Buchholz S, Kaczmarek I (2011) De-novo Sarsam MA, Campell CS, Yonan NA, Deiraniya AK,
calcineurin-inhibitor-free immunosuppression with Rahman AN (1993) An alternative surgical tech-
sirolimus and mycophenolate mofetil after heart nique in orthotopic cardiac transplantation. J Card
transplantation: 5-year results. Curr Opin Organ Surg 8:344–349
Transplant 16:522–528 Steen S, Paskevicius A, Liao Q et al. (2016) Safe orthotopic
Menasche P, Termingnon JL, Pradier F et al. (1994) transplantation of hearts harvested 24 hours after
Expertimental evaluation of Celsior, a new heart pres- brain death and preserved for 24 hours. Scand
ervation solution. Eur Cardiothorac Surg 8:207–213 Cardiovasc J 50:193–200
1102 B. Meiser and B. Reichart
Stewart S, Winters GL, Fishbein MC et al. (2005) Revision of heart transplantation: a four-year randomized trial.
the1990 working formulation for the standardization Circulation 96:1398–1402
of nomenclature in the diagnosis of heart rejection. West LJ, Pollock-Barziv SM, Dipchand AI et al. (2001) ABO-
J Heart Lung Transplant 24:1710–1720 incompatible heart transplantation in infants. N Engl J
Taylor DO, Barr ML, Radovancevic B et al. (1999) Randomized, Med 344:793–800
multicenter comparison of tacrolimus and cyclo- Yacoub MH, Banner NR, Khaghani A et al. (1990) Heart-
sporine immunosuppressive regiments in cardiac lung transplantation for cystic fibrosis and subsequent
transplantation; decreased hyperlipidemia and hyper- domino heart transplantation. J Heart Transplant
tension with tacrolimus. J Heart Lung Transplant 18: 9:459–466; discussion 466—467
336–345 Yusen RD, Edwards LB, Kucheryavaya AY et al. (2015) The
Taylor DO, Meiser BM, Webber SA, et al. (2012) ISHLT guide- Registry of the International Society for Heart and Lung
lines for the care of heart transplant recipients. Part 2: Transplantation: Thirty-second Official Adult Lung and
Immunosuppression and rejection. ISHLT Monograph Heart-Lung Transplantation Report – 2015; Focus
Series 6:97–172 Theme: Early Graft Failure. J Heart Lung Transplant
von Scheidt W, Pauschinger M, Ertl G (2016) Long-term 34:1264–1277
intravenous inotropes in low-output terminal heart Zaroff JG, Rosengard BR, Armstrong WF et al. (2002)
failure? Clin Res Cardiol 105:471–481 Maximizing use of organs recovered from the cadaver
Wenke K, Meiser B, Thiery J et al. (1997) Simvastatin donor: cardiac recommendations. J Heart Lung
reduces graft vessel disease and mortality after Transplant 21:1153–1160
37
1103 38
discharge patients from hospital with mechanical the Jarvik 7 TAH was performed in 1985 by the
circulatory support for their waiting time before same group. The patient was discharged and was
cardiac transplantation. Loisance et al. were the finally able to return to work.
first to use a portable Novacor driver in 1993 In 1991 the Jarvik 7 TAH was renamed the
(Loisance et al. 1994). We started doing this in CardioWest (SynCardia Cardiosystems, Tucson,
Bad Oeynhausen in 1994. USA). To date, more than 1,100 implants have been
In contrast to the first-generation assist performed with 79 % of all patients having been suc-
devices which operated as displacement pumps cessfully bridged to transplant (Copeland et al. 2004,
and generated pulsatile flow, the miniaturized sec- 2006; Leprince et al. 2003). A modified BerlinHeart
ond- and third-generation devices are continuous EXCOR mobile driver which makes the discharge of
flow systems. The first time a second-generation the patients possible has been available since 2004.
axial flow device (Micromed DeBakey) was CE certification for the mobile driver was issued on
implanted was in 1998 in the German Heart the basis of the results of a clinical trial carried out by
Center Berlin (Noon et al. 2001). The only second- our group in Bad Oeynhausen.
generation axial flow pump device currently The Abiocor (Abiomed, Danvers,
approved by the FDA for destination therapy in Massachusetts, USA) is a completely implantable
the USA is the HeartMate II (Thoratec Inc., TAH developed by the Texas Heart Institute and
Pleasantville, NJ, USA). Abiomed. The first implant took place in 2001 at
In third-generation assist devices, the rotor is the Jewish Hospital in Louisville, Kentucky. In a
magnetically suspended. This suspension signifi- first clinical trial, the Abiocor was implanted in
cantly decreases the degree of hemolysis and the patients not eligible for transplantation and with a
rate of thromboembolic complications (Gazzoli life expectancy of <30 days. Results have not been
et al. 2007; Nishinaka et al. 2006). We started satisfying so far. The Abiocor is still under further
implanting the CorAide (Arrow Inc., Reading, development (Copeland et al. 2006).
USA) in 2001 and the DuraHeart assist devices
(Terumo Heart Inc., Ann Arbor, USA) in 2004 in
Bad Oeynhausen. Both have been first implanta- 38.3 Definition of Mechanical
tions worldwide. The DuraHeart is the only third- Circulatory Support
generation assist device so far to receive CE
approval (CE: Conformité Européenne), the cer- The term mechanical circulatory support (MCS)
tificate to allow for widespread use in Europe. is used when a mechanical pump sustains sys-
The Arrow LionHeart LVD 2000 (Arrow temic and/or pulmonary circulation over a vari-
International) was the first fully implantable left able period of time. A distinction between
ventricular assist system specifically designed for univentricular (left or right sided ventricle) and
destination therapy. All components including the biventricular support is made. Almost always the
pump with the inlet and outlet cannulae, the native heart remains in situ.
38 motor controller, the transcutaneous energy When left ventricular support is introduced,
transmission systems and the compliance cham- blood flow is directed either from the left atrium
ber were implanted in a preperitoneal pocket, the or the left ventricle through the pump into the
subcutaneous and the right pleural space, respec- aorta. For reliable unloading of the left ventricle,
tively. The system was first implanted by our the inflow cannula is typically placed in the left
group in 1999 at Bad Oeynhausen (El-Banayosy ventricle via the apex than in the left atrium. In
et al. 2003; Mehta et al. 2001). Unfortunately, due right ventricular support, blood flow is from the
to logistical reasons, the LionHeart is currently right atrium or the right ventricle through the
not available. pump and then into the main pulmonary artery.
The pioneering research of Kolff, Olsen and In order to employ a total artificial heart
Jarvik in the field of TAH aimed to develop a (TAH), both ventricles have to be excised at the
device capable of permanently replacing the atrioventricular level. The inflow cuffs of the TAH
native heart. In the early 1980s, in four patients are sutured to the remaining native atria, and the
with end-stage heart failure, the Jarvik 7 TAH was artificial ventricles are connected to the aorta and
implanted. Two of these patients suffered a stroke. pulmonary artery via vascular outflow prostheses,
Ultimately, all four patients died due to sepsis. The respectively. The two TAH systems which are cur-
first successful bridge to transplantation case with rently available are the CardioWest (SynCardia
Chapter 38 · Cardiac Assist Devices and Total Artificial Heart
1107 38
Systems Inc., Tucson, USA) and the Abiocor 55 Myocarditis (frequently giant cell myocarditis)
(Abiomed Inc., Danvers, USA). The Carmat TAH 55 Acute myocardial infarction
has been developed by Alain Carpentier and 55 Congenital heart defects
Matra Défense. The first in-man implant is sched- 55 Post-cardiotomy cardiogenic shock
uled for 2012. The ReinHeart TAH is being
developed by our institution and the Department In chronic patients, the decision to employ
of Applied Medical Engineering in Aachen, MCS is rather based on the deteriorating clinical
Germany and is currently being tested in acute state (NYHA stage IV, AHA/ACC stage D) and
and chronic animal models. the left ventricular ejection fraction, estimated by
echocardiography (<20 %), than on the invasive
hemodynamic parameters (Cardiac Index <2.2 l/
38.4 I ndications for Mechanical qm/min, systolic blood pressure <90 mmHg, pul-
Circulatory Support monary wedge pressure >20 mmHg and maximal
oxygen uptake <12 ml/kg/min).
38.4.1 General
Mechanical circulatory support as a bridge to trans- Although it is known that in dilated cardiomy-
plantation is an established therapy for end-stage opathy assisted circulation may contribute to
heart failure in chronic patients with dilated or isch- reverse remodeling, it is still unclear in which sub-
emic cardiomyopathy demonstrating clinical dete- sets of patients this reverse remodeling once tak-
rioration with an imminent risk of death despite ing place on the cellular level actually does translate
maximal inotropic support while being on the to myocardial recovery and restoration of the basic
waiting list for transplantation. The aim is to secure cardiac functions. Successful weaning and long-
survival and to prevent multiple organ failure term cardiac stability has been reported so far in
before cardiac transplantation can be performed. less than 10 % of patients following the implanta-
Furthermore, bridge to transplantation may tion of a left ventricular assist device for dilated
be considered as a therapeutic option in patients cardiomyopathy. It has been hypothesized that the
with an end-stage heart failure resulting from: myocyte atrophy resulting from long-term ven-
55 Valvular cardiomyopathy tricular unloading may impair successful weaning
1108 R. Koerfer et al.
from assisted circulation despite the potentially patients with contraindications for cardiac trans-
beneficial reverse remodeling. Clenbuterol, a selec- plantation.
tive b2-agonist, initially approved for the treat- According to the current recommendations of
ment of bronchial asthma, has been reported to the US Centers for Medicare and Medicaid
have beneficial effects on remodeling and protec- Services, destination therapy is covered for patients
tive effects against cellular apoptosis (Birks et al. who have chronic end-stage heart failure for at
2006; Dandel et al. 2005; Mancini et al. 1998; Mann least 90 days with a life expectancy of less than
and Willerson 1998; Simon et al. 2005). 2 years, who are not candidates for heart trans-
In post-cardiotomy cardiogenic shock, recov- plantation and meet the following conditions:
ery of the ventricular function should be expected 55 The patient’s Class IV heart failure symptoms
within 7–14 days. In acute allograft rejection, pri- have failed to respond to optimal medical
mary allograft failure or isolated right ventricular management, including dietary salt restric-
failure after heart transplantation recovery should tion, diuretics, digitalis, beta-blockers and
be within 14 days, also, while in chronically ACE inhibitors (if tolerated) for at least 60 of
dilated cardiomyopathy recovery may take as long the last 90 days.
as 6 months. 55 The patient has a left ventricular ejection
fraction (LVEF) <25 %.
55 The patient has demonstrated functional
38.4.4 Bridge to Bridge (BTB) limitation with a peak oxygen consumption
<12 ml/kg/min; or the patient has a contin-
Bridge to Bridge support mostly is considered in ued need for intravenous inotropic therapy
patients in whom at the initiation of MCS the owing to symptomatic hypotension, decreas-
potential duration of support required was quite ing renal function, or worsening pulmonary
unclear. Most of these patients had been in acute congestion.
cardiogenic shock and were under resuscitation 55 The patient has the appropriate body size
before and during MCS initiation. The neurologi- (≥1.5 m2) to support the VAD implantation.
cal state and the extent of end-organ failure are
initially unclear. Extracorporeal life support sys- The REMATCH trial has been the landmark
tems allow a short-term support of up to 1 month. study for the destination therapy concept. Long-
If there are no further contraindications for heart term mechanical circulatory support resulted in a
transplantation, long-term support systems can significant risk reduction for death of any cause of
be implanted during that time frame. up to 48 % in NYHA IV patients when compared to
Common causes of cardiogenic shock are the optimal medical therapy (Rose et al. 1999, 2001).
following: The INTERMACS registry (Interagency
55 Acute myocarditis Registry for Mechanical Assisted Circulatory
55 Acute myocardial infarction Support) has the aim to define those patients who
38 55 Toxic cardiomyopathy are eligible for mechanical circulatory support and
55 Post-cardiotomy circulatory failure to optimize the clinical outcomes (Kirklin and
55 Acute heart failure Naftel 2008). Seven patient profiles (‘levels’) have
55 Postpartum cardiomyopathy been defined according to the clinical symptoms:
1. Persistent cardiogenic shock despite an esca-
Although in most of these cases after initial life- lating inotropic support.
saving short-term circulatory support the next step 2. Progressive decline despite inotropic sup-
is a BTT therapy, it also may end in a bridge to port.
recovery. However, a secondary device employed 3. Patient is stable under moderate inotropic
for BTT or BTR may require a further step, a change support.
to a device compatible with destination therapy. 4. Recurrent advanced heart failure susceptible
to medical treatment.
5. Comfortable at rest but intolerant to light
38.4.5 Destination Therapy (DT) exercise.
6. Comfortable at rest but limited to light exercise.
Destination therapy is the long-term MCS as an 7. Comfortable at rest but limited to mild exer-
alternative to cardiac transplantation for those cise.
Chapter 38 · Cardiac Assist Devices and Total Artificial Heart
1109 38
Destination therapy is currently recom- According to our experience, biventricular sup-
mended in those patients who are clinically stable port is indicated in the presence of the following:
and in whom implantation can be performed elec- 55 Biventricular heart failure refractory to
tively (levels 3–6). For these patients the results of medical treatment
the third-generation assist devices are indeed 55 Including high-dose i.v. inotropic therapy
encouraging. The 1-year survival is at 86 % and is 55 Imminent multi-organ failure (renal failure
comparable to orthotopic heart transplantation. requiring hemodialysis and hepatic failure
Destination therapy could therefore become a safe resulting in an increase of bilirubin >5 mg/dl
alternative to heart transplantation, especially for and an increase of GOT and GPT three times
elderly people (Lietz and Miller 2005; Pennington the standard value)
et al. 1999; Stevenson and Rose 2003). 55 Malignant arrhythmias
Since mandatory guidelines are not available, the Relative contraindications for mechanical cir-
indication for biventricular support is mainly based culatory support include:
upon clinical criteria. Hemodynamic parameters 55 Aortic valve regurgitation > I°
(CI <2.2 l/qm/min, CVP >18 mmHg, pulmonary 55 Mechanical prosthesis in the aortic position
vascular resistance >500 dyn/s/ym5, low pulmo- 55 Fixed pulmonary hypertension >6 Woods
nary artery pressure) in addition are helpful. units
1110 R. Koerfer et al.
..Fig. 38.1 CentriMag
1114 R. Koerfer et al.
failure. The CentriMag LVAD is ideal as an ‘extra- Thoratec PVAD. Meanwhile, more than 7,000
corporeal life support system’. The pump rotates patients have been treated with this device.
at speeds between 1,500 and 5,500 rpm and gen- The pump consists of a rigid polysulfone case
erates a flow of up to 9 L/min. Since the pump is and a soft and flexible Thoralon pumping sack.
magnetically levitated without any bearings or The Thoralon material provides a low thromboge-
seals included, the degree of hemolysis is remark- nicity and high endurance of the pump regarding
ably low. wear and tear. Mechanical valves within the inflow
Similar systems are the Rotaflow (Maquet and outflow conduits facilitate unidirectional
Cardiopulmonary AG, Rastatt, Germany) and the blood flow.
TandemHeart (CardiacAssist Inc., Pittsburgh, PA, The filling state of the pump is controlled by a
USA). sensor which triggers blood ejection. The pump
can generate a stroke volume of up to 65 ml at a
38.8.1.2 Biomedicus Centrifugal Pump stroke rate between 20 and 110/min. The resulting
The Biomedicus centrifugal pump (Medtronic, cardiac output ranges from 1.3–7.2 l/min.
Eden Prairie, MN, USA) is frequently used as a In the LVAD position, the inflow graft/can-
short-term circulatory support in patients with nula gets blood from the left atrium or the native
post-cardiotomy heart failure or in any form of LV. The outflow cannula is connected to the
cardiogenic shock. It may be employed as a ascending aorta. In order to prevent kinking, the
femoro-femoral or standard central cardiopulmo- ventricular and the arterial cannulas are wire
nary bypass as part of an extracorporeal mem- reinforced. A velvet coat minimizes the risk of
brane oxygenation (ECMO) system. It is possible ascending percutaneous mediastinitis via the
to either support one or both ventricles. tubes.
Electrical and pneumatic leads connect the
pump to the dual driver. The operating pump rate,
the vacuum level and the pressure of the system
First-generation extracorporeal systems can be adjusted. In biventricular support these
have been associated with a high rate of adjustments can be made for each pump sepa-
adverse events, including hemolysis, rately. There are two modes of operation: a fixed
excessive bleeding and thromboembolism. rate mode and a volume mode. The volume mode
For these reasons, we currently prefer the is beneficial, since the pump can respond to an
CentriMag pump. increased demand on stroke volume during exer-
cise.
Due to the paracorporeal placement of the
pumps, the Thoratec PVAD can also be used in
38.8.2 aracorporeal Assist Devices
P small patients down to a body surface area of
(PAVD) 0.73 m2.
38 38.8.2.2 BerlinHeart EXCOR
During paracorporeal assist the pump is placed
outside but close to the body. Inflow and outflow The BerlinHeart EXCOR (BerlinHeart AG, Berlin,
grafts are used to connect the heart via atrium or Germany) is a pneumatically driven system for
ventricle and also the great vessels. uni- or biventricular support. Since different sizes
of the pumps are available (10–80 ml), it can be
38.8.2.1 Thoratec PVAD used not only in adults with a small body surface
The Thoratec PVAD (Thoratec Inc., Pleasanton, area but also in children. The three leaflet valves
CA, USA) is designed for uni- and biventricular in the inflow and outflow conduits are made of
support. It was developed in the early 1980s by polyurethane. The system is operated by a porta-
William Pierce and James Donachy at ble dual driver. Like the Thoratec PVAD, the
Pennsylvania State University. In 1984 Donald BerlinHeart EXCOR is mainly used in the bridge
Hill reported the first successful BTT case with a to transplantation setting.
Chapter 38 · Cardiac Assist Devices and Total Artificial Heart
1115 38
38.8.3 artially Implantable Assist
P
Devices
The main indication for left ventricular sup- The pump is implanted through a left-sided
port with the HeartMate II LVAD is still bridge to thoracotomy (5th or 6th intercostal space) under
transplantation (BTT). According to the results of femoro-femoral bypass. The outflow graft is anas-
the bridge to transplantation pivotal trial, the tomosed to the descending aorta, the driveline is
combined survival rate for recovery, ongoing sup- tunneled in the right anterior abdominal quad-
port or transplantation was 79 % at 18 months. rant and the pump is finally positioned into the
The incidence of major complications such as left ventricle through the left ventricular apex.
neurological events and infections was remark- Until now, the Jarvik 2000 has been implanted
ably low (Pagani et al. 2009). In the post-approval in more than 200 patients of whom 75 % have
bridge to transplantation study, combined sur- received the device as a bridge to transplantation.
vival at 6 and 12 months was 90 % and 85 %, The main adverse event has been pump thrombosis.
respectively (Starling et al. 2011). The longest duration of support has been 7.5 years.
..Fig. 38.6 DuraHeart
38
..Fig. 38.7 VentrAssist
Chapter 38 · Cardiac Assist Devices and Total Artificial Heart
1121 38
Renfrewshire, Scotland, UK), driveline, external the most reliable and therefore the most frequently
controller and the external batteries. implanted TAH. The system received FDA approval
The VentrAssist LVAD was implanted for the as a bridge to transplantation therapy device in
first time in the year 2003 and was CE certified. patients with end-stage heart failure and has been
The medical device maker collapsed in 2009. already implanted more than 1,100 times. Recently
it received additional approval as a Humanitarian
Use Device designation to be used for destination
38.9.6 ully Implantable, Pulsatile
F therapy. Due to the large dimensions of the artifi-
LVAD LionHeart cial ventricles (70 cc), the SynCardia CardioWest
TAH can be currently used only in patients with a
The LionHeart LVAD 2000 (Arrow LionHeart body surface area of more than 1.7 m2. A smaller,
LVAD, Arrow International, Reading, PA, USA) 50 cc system, which is designed for patients with a
was the only fully implantable left ventricular body surface area between 1.2 and 1.7 m2, is cur-
assist device. It was developed by The Pennsylvania rently being developed.
State University (Penn State) in the USA as an Technical description: The SynCardia
alternative to transplantation (destination ther- CardioWest TAH is a pneumatically driven,
apy). It was implanted for the first time in 1999 by biventricular system used for the orthotopic
our group in Bad Oeynhausen. It was tested in the replacement of the native ventricles. The pump
CUBS trial (Clinical Utility Baseline Study (Pae consists of four flexible polyurethane diaphragms
et al. 2007a)). Although initial clinical results have housed in a semi-rigid polyurethane sack. By dis-
been satisfying, currently, the LionHeart is not placement the diaphragms generate a stroke vol-
available for clinical implantation due to high ume of up to 70 ml and a pump flow of up to 10 l/
development costs. min. Two mechanical valves, which are mounted
in the inflow (Medtronic Hall 27 mm) and the
outflow (Medtronic Hall 25 mm) conduits facili-
38.10 Total Artificial Hearts tate unidirectional blood flow. Two wire-rein-
forced, Dacron-coated drivelines connect the
Carefully selected patients with intracardiac artificial ventricles to the external console.
shunts or thrombi, structural damage to the The external console consists of two pneu-
heart due to an extended myocardial infarction matic drivers (of which one serves as a backup),
or with end-stage congenital heart disease may batteries, air reserve tanks, an alarm panel and a
benefit from implantation of a total artificial computer system which monitors the operation of
heart (TAH). the TAH. There are two different drivers available.
The Companion 2 hospital driver was approved
by the FDA in the year 2012. During the initial
38.10.1 SynCardia CardioWest TAH recovery stage, the driver is docked in the hospital
cart and after leaving the bed in the companion
The SynCardia CardioWest TAH (SynCardia caddy which enables greater mobility during hos-
Systems Inc., Tucson, AZ, USA; . Fig. 38.8) is still pital stay. Prior to discharge the patients are
switched over to the Freedom 2 portable driver.
This weighs 6 kg, is worn in the backpack or a
shoulder bag and is powered by two lithium-ion
batteries. The Freedom portable driver received
CE mark at the year 2010 and is currently under-
going an investigational device exemption clinical
study in the USA.
In order to prevent end-organ failure and in
order to minimize thrombus formation on the
blood-contact surfaces, it is essential to provide a
pump flow of 7–8 l/min at a systemic pressure of
70–90 mmHg and a perfusion pressure of
..Fig. 38.8 CardioWest TAH 55–80 mmHg. For this reason:
1122 R. Koerfer et al.
55 It is important to overcome the systemic and the tricuspid valve and a 1–2-cm wide rim of ven-
the pulmonary artery pressure by adjusting tricular muscle. The ascending aorta and the main
the drive pressure at the right side 30 mmHg pulmonary artery are then excised immediately
over the systolic pulmonary artery pressure above the valvular level. The trimmed cuffs are
and the drive pressure at the left side anastomosed to the native atria and the outflow
60 mmHg over the systemic systolic artery grafts to the aorta and the pulmonary artery. The
pressure. driveline is tunneled, and the artificial ventricles are
55 The TAH beat rate should range between 100 connected to the native atria and the outflow grafts.
and 130/min, and the systolic duration The ventricles are deaired by inflating the lungs and
should be between 50 and 60 % of the com- gradually declamping the great vessels.
plete cardiac cycle length.
55 The negative force, the vacuum in the artifi-
cial ventricles, should range between −10 and 38.10.2 AbioCor TAH
−15 mmHg.
The AbioCor (Abiomed Inc., Danvers, MA, USA)
38 Implantation procedure: the system consists is the first totally implantable, electrically driven
of the following components (. Fig. 38.9): TAH, designed as an alternative to cardiac trans-
55 Two artificial ventricles plantation in patients:
55 Two atrial cuffs 55 With end-stage biventricular heart failure
55 Two outflow grafts 55 Who are not eligible for transplantation
55 One driveline 55 Who cannot be weaned from inotropic
55 Tools for leakage testing therapy
55 Who cannot be weaned from a biventricular
Prior to median sternotomy, the outflow con- mechanical circulatory support
duits are precoated with CoSeal Surgical Sealant 55 Who are younger than 75 years of age
(Baxter, Deerfield, IL, USA) and then cut to a length
of 4–5 cm for the aortic and 5–6 cm for the pulmo- The internal part of the system consists of a
nary outflow. On total cardiopulmonary bypass, centrifugal pump, which can generate a flow of up
the native ventricles are excised at the atrioventric- to 8 l/min at a rotational speed of 4,000–8,000 rpm,
ular level while care is taken to spare the mitral and a controller, a rechargeable battery and a coil for
Chapter 38 · Cardiac Assist Devices and Total Artificial Heart
1123 38
2 5
2 1
3 4
7 3
6
5
4
..Fig. 38.10 ReinHeart TAH pump unit: (1) linear motor,
(2) left pusher plate, (3) right pusher plate, (4) linear
bearing, (5) left artificial ventricle, (6) right artificial
ventricle, (7) mechanical valve (From Pelletier et al. (2014))
55 Keep the International Normalized Ratio aim for increasing the endurance and the func-
(INR) higher than 1.5 tional independence of the patient. The final
55 Keep the platelet count above 100,000 ul target is to gradually reintegrate the patient in
55 Keep the partial thromboplastin time (PTT) his social and working environment.
around 50–60 s
The hemodynamic data should be always cor- 38.11.5.3 Heart Failure Medication
related to the data displayed by the pump controller. 55 The standard heart failure medication must be
continued according to the recommendations
of the ACCF/AHA (Jessup et al. 2009). The
38.11.3 Physical Therapy diastolic arterial pressure should not exceed
90 mmHg.
We always seek to wean the patients from
mechanical ventilation as early as possible. 38.11.5.4 Antiarrhythmic Medication
This is possible in 80 % of our MCS patients. 55 Since arrhythmias are a common event, a
Physical therapy can therefore be started pre-existent antiarrhythmic therapy must be
already on the first post-operative day. Manual continued even after the implantation of an
therapy techniques and therapeutic exercise assist device.
Chapter 38 · Cardiac Assist Devices and Total Artificial Heart
1125 38
38.11.6 Wound Management problems, were frequent. Our own experience
with positive displacement pumps, such as the
A meticulous aseptic wound management is crucial HeartMate XVE, confirmed these results. The
in order to prevent driveline and pump pocket infec- incidence of bleeding complications was 54 %, the
tions. The relatives of the patient have to be trained incidence of thromboembolism 7.4 %. Driveline
to appropriately change the driveline dressings. infections were documented in 21 % of all cases
and a pump failure in 11 %. Fifty percent of all
MCS patients underwent a heart transplantation
38.11.7 Ambulatory Care (El-Banayosy et al. 2000, 2001).
The REMATCH trial has been important for
Ambulatory care is possible when: two reasons: it proved that a long-time support is
55 The patient has fully recovered and is in possible, and it outlined the necessity to develop
NYHA stage I–II. assist devices, which would be associated with less
55 There are no signs for an end-organ failure. adverse events.
55 There are no signs for a systemic infection. Magnetically suspended pumps have several
55 The patient and his relatives are familiar with advantages: (1) they are small and can therefore
the handling of the device and the oral- be used even in pediatric patients, (2) adverse
coagulation self-management. events such as excessive hemolysis, thromboem-
55 The patient and his relatives are familiar with bolism and bleeding disorders are less frequent
the management of the driveline dressings. and finally they are longer durable than other
55 Social and familial support is granted. pump principles.
55 A contact person from the hospital (VAD Due to bleeding complications associated with
coordinator) is available at any time. an aggressive anticoagulation management, 1-year
survival rate in patients treated with the DuraHeart
A follow-up is performed regularly (every third-generation assist device was initially only
8 weeks) and includes: 70 %. Following adjustment of the anticoagulation
55 A physical examination management, employing self-management also,
55 An ECG examination 1-year survival rate went up to 90 %. An infection
55 An echocardiographic examination was documented in 15 % and an acute right heart
55 A laboratory examination failure in 31 % of all DuraHeart patients. Right
55 An inspection of the driveline heart failure was always treated medically and all
patients recovered fully. Major neurological events
in this group were documented in 27 % of patients.
38.12 Summary and Perspectives There were no cases of pump failure, excessive
hemolysis or pump thrombosis. In two cases the
Mechanical circulatory support is a well- pump had to be replaced due to a temporary flow
established option for the treatment of end-stage interruption. This resulted from a distortion of the
heart failure. The clinical experience and the results motor back electromotive force waveform which
of several multicenter trials demonstrate that it is led to a temporary interruption of the rotation of
possible to maintain survival and at the same time the motor.
provide comfort for these critically ill patients. The results of the HeartMate II second-
The REMATCH trial is considered to be the generation axial flow pump are similar to those
landmark study in the field of mechanical circula- obtained with the DuraHeart. Bleeding complica-
tory support. The results, which were published in tions were reported in 31 %, driveline infections
2001 (Rose et al. 2001), demonstrated a signifi- in 14 %, medically treatable right heart failure in
cantly higher 1-year survival in those patients, 13 %, excessive hemolysis in 3 % and major neuro-
who were treated with the pulsatile HeartMate logical events in 8 % of HeartMate II patients.
XVE LVAD when compared to medically treated There had been no cases of a pump failure.
patients (52 % vs. 25 %, respectively). MCS However, the pump had to be replaced in five
patients also experienced a superior quality of life. cases (3.7 %). This was due to pump thrombosis or
However, adverse events, such as infections, complications associated with the operative pro-
bleeding disorders and technical or mechanical cedure. There had been three device-associated
1126 R. Koerfer et al.
deaths: two patients died due to technical prob- series 1. Mechanical circulatory support. Elsevier,
lems of the external batteries and one due to kink- Amsterdam, pp 105–125