Perloff 2012 Chapter Pda

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CLINICAL RECOGNITION OF CONGENITAL HEART DISEASE ISBN: 978-1-4377-1618-4
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Library of Congress Cataloging-in-Publication Data

Perloff, Joseph K., 1924-
Clinical recognition of congenital heart disease / Joseph K. Perloff, Ariane J.
Marelli. – 6th ed.
p. ; cm.
ISBN 978-1-4377-1618-4 (hardcover : alk. paper)
I. Marelli, Ariane. J. II. Title.
[DNLM: 1. Heart Defects, Congenital–diagnosis. WG 220]
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Chapter 20
Patent Ductus Arteriosus
Aortopulmonary Window
In 1593, Giambattista Carcano, Professor of Anatomy in on neonatal ductal patency. Ductal dependent circulations
Pavia, an ancient town in northern Italy, described the duc- include malformations in which a patent ductus is the
tus arteriosus in his book on the great cardiac vessels of the only source of pulmonary arterial blood flow (pulmonary
fetus.1 However, Leo Bottali came to be associated with atresia with intact ventricular septum), the only source of
the arterial duct, the duktus arteriosus persisten, even systemic arterial blood flow (aortic atresia or complete
though he misapplied the term to the foramen ovale.1 It interruption of the aortic arch), or the only source of bi-
was not until Karl von Rokitansky’s handbook of 1844 directional blood flow (simple complete transposition of
and his beautifully illustrated monograph of 1852 that pat- the great arteries; see relevant chapters).
ent ductus arteriosus was recognized as a specific congen- The ductus arteriosus is derived from the sixth aortic
ital malformation.2 The first section of this chapter is arch. By the fourth month of gestation, ductal tissue has
concerned with persistent patency of the ductus arteriosus. become distinctive, differing histologically from pulmo-
The second section is devoted to aortopulmonary window, nary arterial and aortic tissue.19 At 16 weeks of gestation,
often called aortopulmonary or aorticopulmonary septal the ductus consists of a muscular arterial channel with an
defect, an anomaly that is embryologically unrelated to pat- endothelium separated by an internal elastic lamina and a
ent ductus but that is physiologically and clinically similar. thin subendothelial layer.19 The media differs at the aor-
The incidence rate of isolated persistent patency of the tic and pulmonary ends, so ductal media can be aortic,
ductus has been estimated at 1:2000 to 1:5000 births, or pulmonary, or mixed.4 As gestation continues, the intima
about 10% to 12% of all varieties of congenital heart dis- thickens, and the subendothelial layer is invaded by cells
ease.3 The pulmonary orifice of the ductus is located im- from the media that disrupt the internal elastic lamina. At
mediately to the left of the bifurcation of the pulmonary term, the mature ductus harbors conspicuous intimal
trunk near the origin of its left branch (Figures 20-1 and cushions that protrude into the lumen. The ductus is then
20-2). The aortic orifice is located immediately distal to is capable of contraction, functional closure, which is fol-
the origin of the left subclavian artery (see Figures 20-1 lowed by anatomic closure that uniformally begins at
and 20-2). A patent ductus can be long and narrow or short the pulmonary arterial end (see previous).4 Anatomic
and wide, with all gradations in between (Figures 20-3, closure follows a sequence of immunohistochemical
20-4, and 20-5). Closure consistently begins at the pulmo- and ultrastructural changes, namely4,19: (1) separation
nary arterial end, so the ductus assumes the shape of a trun- of endothelium from internal elastic lamina; (2) enfolding
cated cone that is larger at its aortic end (see Figures 20-3 and ingrowth of endothelial cells; (3) migration of
and 20-4).4,5 A widely patent aortic end with a sealed undifferentiated medial smooth muscle cells into the
pulmonary end is the substrate for a ductal aneurysm subendothelium; (4) fragmentation of the internal elastic
(Figure 20-6).6–9 Patency confined to the pulmonary end lamina; (5) sealing of the lumen by endothelial cell appo-
is exceptional.10 Anatomic variations include bilateral sition; (6) accumulation of lipid droplets; and (7) intimal
patent ductus,11,12 left-sided patent ductus with right and subendothelial degenerative changes that spread
aortic arch,13 right-sided patent ductus with right aortic centrally and peripherally and result in disappearance of
arch,14 patent ductus or ligamentum arteriosum as a com- endothelial cells at luminal apposition lines.4 The normal
ponent of a vascular ring (Figure 20-7),15 and dissection process of functional closure begins within 10 to 15 hours
of the aorta with extension into a patent ductus.16 after birth and is virtually complete (probe patent) by
Despite its seeming anatomic simplicity, the ductus the second week of extrauterine life. The ductus is
arteriosus is a complex structure. The fetal ductus is a ma- an anatomically closed ligamentum arteriosum 2 to
jor anatomic component of a contiguous intrauterine 3 weeks after birth.5,17,20 When a ductus is destined to
great arterial system that consists of pulmonary trunk/ remain patent, the intrauterine subendothelial internal
ductus/aortic continuity that delivers 85% of right ven- elastic lamina lies adjacent to the intimal cushions, endo-
tricular output into the descending aorta.17 Persistent fe- thelial cells adhere to the elastic lamina, and subendothe-
tal circulation is a designation applied to an intrauterine lial edema with enfolding of endothelial cells does not
right-to-left ductal shunt that persists after birth (see occur.4,19 A ductus that remains patent in full-term
Chapter 14).18 Persistent patency of the ductus arterio- infants after 3 months of extrauterine life harbors the
sus is abnormal and therefore undesirable, although cer- histologic features of persistent patency just described.
tain forms of congenital heart disease depend for survival Spontaneous closure is then unlikely.19,21,22
368
C ha p ter 20 Patent Ductus Arteriosus Aortopulmonary Window 369
Left-to-right shunt
Ao PDA
PDA
PT
Aorta
Patent ductus
Pulmonary trunk
Lig. art. Left subclavian

Ao artery
Right-to-left shunt
APW
PDA
PT
Aortopulmonary window
FIGURE 20-1 Illustrations of patent ductus arteriosus (PDA) and

aortopulmonary window (APW). The aortic orifice of a patent
ductus arteriosus inserts immediately distal to the origin of the
left subclavian artery. (Ao ¼ aorta.) The pulmonary orifice in-
serts immediately to the left of the bifurcation of the pulmonary
trunk (PT). An aortopulmonary window is a communication
between adjacent walls of the ascending aorta (Ao) and the
pulmonary trunk (PT) proximal to its bifurcation. (Lig. art. ¼ liga-
FIGURE 20-2 Illustrations of two major flow patterns in patent
mentum arteriosum.)
ductus arteriosus (PDA). The upper drawing illustrates a left-to-
right shunt through a patent ductus in which pulmonary vascu-
lar resistance is lower than systemic vascular resistance. Shunt
flow is from aorta into the pulmonary trunk. The lower drawing
In utero ductal tone is determined by an interplay be- illustrates a right-to-left shunt through a patent ductus in which
tween the constricting effect of oxygen (relatively weak pulmonary vascular resistance is suprasystemic. Shunt flow is
because of low fetal pO2) and the dilating effect of endo- from pulmonary trunk into the aorta immediately distal to the
genous prostaglandin E2.23,24 Prostaglandin synthetase left subclavian artery.
inhibitors administered to mammalian fetuses or to preg-
nant ewes constrict the fetal ductus and deprive the fetal
right ventricle of its only outlet.24 As term approaches, and smooth muscle cell proliferation have been assigned
the ductus becomes less responsive to prostaglandin E2 a role in anatomic closure.29
and more responsive to oxygen, setting the stage for con- In healthy preterm infants, delayed closure of the duc-
striction that begins a few hours after birth in full term tus arteriosus is common.30–33 Premature neonates with a
infants.25 Functional closure is closely coupled to the gestational age of 30 weeks or more usually experience
increase in extrauterine ambient oxygen tension that spontaneous ductal closure within a time frame that
exerts a direct constricting effect on the ductal wall (see corresponds to the closure time in full-term infants.31,32
previous). Oxygen-induced constriction has been related In full-term infants, spontaneous closure is unlikely after
to inhibition of voltage-gated potassium channels.26 3 months of age,21,22 and in premature infants, it is
Flow through the closing ductus is transiently unlikely after 1 year of age (Figure 20-8).22 Exceptional
bidirectional. Left-to-right flow25 then decreases rapidly examples of spontaneous closure have been documented
during the next 12 hours and cannot be detected at between 5 and 6 years of age, between 7 and 14 years,34
48 hours.27 Anatomic closure is the culmination of after 17 years,35 and at age 19 years.
morphologic changes accrued during intrauterine ductal Persistent patency of the ductus in premature infants
maturation (see previous).4,19,28 In addition, apoptosis sometimes coincides with respiratory distress, but the
370 Ch apt er 20 Patent Ductus Arteriosus Aortopulmonary Window
LSA
AO
PDA
PT
PA
Ao
A DA
FIGURE 20-3 Lateral aortogram from a 14-year-old girl with a AO

patent ductus arteriosus.
distress may not improve with subsequent ductal clo-

sure.31,32,36 Patent ductus in preterm infants is associated
with reduced cerebral blood flow from a steal effect
caused by the aortic-to-pulmonary shunt, rather than
by a limited capacity of the preterm left ventricle to
achieve adequate cardiac output.37,38 B
First-trimester maternal rubella with rash carries an
80% incidence rate of intrauterine viral infection39; deaf- FIGURE 20-4 A, Lateral angiogram from a 16-year-old girl.
ness and cataracts (Figure 20-9). Congenital heart disease A catheter passed from the pulmonary trunk (PT) through a
ductus arteriosus (arrow), which is distinctly larger at its aortic
affects two thirds of offspring. Patent ductus arteriosus
end. (AO ¼ aortic arch; DA ¼ descending aorta.) B, Lateral
accounts for a third of the congenital malformations22 angiogram from an 8-year-old boy with a restrictive tubular
and is characterized by maturational arrest and an imma- ductus arteriosus (arrow) that fills from the aorta (AO) and tapers
ture ductal wall of the type found at 16 weeks of gestation markedly at its pulmonary end.
(see previous).22
The physiologic consequences of persistent patency of
the ductus arteriosus depend on five variables: (1) the systemic level (see Figure 20-2, upper). When pulmonary
size of the ductus; (2) pulmonary vascular resistance; vascular resistance exceeds systemic, the shunt is re-
(3) the adaptive response of the left ventricle to volume versed (see Figure 20-2, lower). Volume overload of
overload; (4) prematurity; and (5) respiratory distress. the left ventricle is then curtailed, pressure overload of
When the ductus is restrictive, pulmonary vascular resis- the right ventricle remains at systemic level, and the
tance is normal, right ventricular afterload is normal, and pulmonary vascular bed exhibits histologic changes simi-
the hemodynamic consequences are negligible. When the lar to primary pulmonary hypertension or Eisenmenger’s
ductus is moderately restrictive and pulmonary vascular syndrome (see Chapters 14 and 17).35,41
resistance is normal or nearly so, right ventricular after-
load is not significantly affected and continuous aortic-
to-pulmonary flow imposes only a moderate volume load
on the left ventricle. About 95% of isolated patent duc- HISTORY
tuses are restrictive or moderately restrictive. When
the ductus is nonrestrictive, systolic pressure in the aorta A newborn is typically pronounced healthy and dis-
and pulmonary trunk equalize at systemic level, so the di- charged as a well baby. As neonatal pulmonary vascular
rection of blood flow depends on the relative resistances resistance falls, a left-to-right shunt is established, the
in the systemic and pulmonary vascular beds.40 If pulmo- ductus murmur emerges, and the diagnosis becomes ap-
nary resistance is lower than systemic, a left-to-right parent. Less commonly, a neonate comes to attention be-
shunt is established, imposing volume overload on the left cause of low birth weight, systemic hypoperfusion, or
ventricle while right ventricular afterload remains at congestive heart failure without an incriminating ductus
PDA PDA
Ao
PT Ao
PT
LV
A B
FIGURE 20-5 Angiocardiograms from a 3-year-old boy with a nonrestrictive patent ductus arteriosus (PDA), low pulmonary vascular
resistance, and a 3 to 1 left-to right shunt. A, The ascending aorta (Ao) is relatively small. The pulmonary trunk (PT) and left ventricle (LV)
are dilated. B, The patent ductus arteriosus (PDA) connects a relatively small ascending aorta (Ao) to a dilated pulmonary trunk (PT).
murmur (Figure 20-10).33,42 Absence of a murmur does been found between the presence of a murmur and the
not necessarily mean that the ductus has closed.17 A duc- size of the arterial duct.43 Doppler echocardiography oc-
tus can be patent but silent because of the direction of the casionally detects a tiny patent ductus in infants without
jet as it enters the pulmonary artery.43 No correlation has auscultatory signs of its presence,44 or auscultation
T
a
rt
o
A
Inn
DA
Left
Lung
Right
Lung
PT
LPA
RV
LV
DA
A 23 days old B
FIGURE 20-6 A, X-ray showing the striking convexity of a large ductal aneurysm (arrow) in a 23-day-old infant. Retention of barium in
the stomach was caused by pyloric stenosis. B, Necropsy specimen showing the ductal aneurysm (DA), which was sealed at its pulmo-
nary arterial end but patent at its aortic end. (Inn ¼ innominate artery; T ¼ trachea; PT ¼ pulmonary trunk; LPA ¼ left pulmonary artery;
RV/LV ¼ right ventricle/left ventricle.)
RCA
LCA
RSA
LSA
LAA
RAA
Ao
DAo
LV
FIGURE 20-7 Angiocardiogram with contrast material injected

into the left ventricle (LV) of a 15-year-old boy with a vascular
ring. The ascending aorta (Ao) bifurcates into a right aortic arch
(RAA) that passes anterior to the trachea and a left aortic arch
(LAA) that passes posterior to the esophagus, hence a vascular FIGURE 20-9 A 5-year-old girl whose mother had first-trimester
ring that compressed the trachea and esophagus. The two aortic rubella. The bandage followed ophthalmic surgery for cataract.
arches joined to form the descending aorta (DAo). All anatomic The child had a patent ductus arteriosus.
components of the vascular ring are visualized except the liga-
mentum arteriosum, which was surgically divided. (RSA/LSA ¼
right subclavian artery/left subclavian artery; RCA/LCA ¼ right
carotid artery/left carotid artery.)
S4
SM
S1
S2
FIGURE 20-10 Phonocardiograms at the second left intercostal

space in a 21-day-old premature male with a nonrestrictive
FIGURE 20-8 X-ray from the 21-day-old premature male with a patent ductus arteriosus (see x-ray in Figure 20-8). The low-
widely patent ductus arteriosus. The phonocardiogram is shown frequency filter (upper tracing) shows a fourth heart sound
in Figure 20-10. Pulmonary blood flow is increased, the heart is (S4). The high-frequency filter (lower tracing) shows a normal
considerably enlarged, and a thymus (arrows) obscures the first heart sound (S1) and an unimpressive early to midsystolic
base. By age 4 months, the ductus had spontaneously closed, ductal murmur (SM) that fades before a loud single second heart
the thymus had disappeared, and the x-ray was virtually normal. sound (S2).
detects a tiny ductus in adults in whom the diagnosis had ductus. Susceptibility has not been determined for a tiny
been missed (see Figure 20-40). Closure of a patent duc- clinically silent ductus detected only with Doppler
tus is occasionally the result of healed infective endocar- echocardiography (see Figure 20-40; see previous).67
ditis45,46 or thrombotic occlusion.47,48 Abnormal patterns of cerebral arterial blood flow in in-
In 1561, Vesalius described a valve or membrane in a fants, especially preterm neonates with a nonrestrictive
patent ductus arteriosus.49 A valve-like structure was patent ductus, predispose to central nervous system is-
subsequently found in stillborn human fetuses and in chemia and hemorrhage into the germinal matrix.38,68,69
newborn rabbits,49 and in 1903, a necropsy report called Increased pulse pressure and major fluctuations of blood
attention to a perforated ductal valve.50 Taussig51 con- flow velocity caused by opening and closing of a ductus
firmed the presence of a membranous valve at the pulmo- may rupture capillaries of the germinal matrix and cause
nary end of a ductus and theorized that rupture might intraventricular hemorrhage. A sharp decrease in dia-
account for the sudden appearance of a ductus murmur, stolic arterial flow velocity can act as a steal from the ce-
an event occasionally witnessed in children or young rebral circulation.68
adults. A continuous murmur intermittently appeared After the first year of life, most patients with patent
and disappeared in a patient with a veil-like valve at the ductus arteriosus are asymptomatic. Beginning with the
pulmonary end of a ductus,52 and the abrupt appearance second decade, the risk of infective endarteritis exceeds
of a loud continuous murmur was described in a 55-year- the risk of congestive heart failure.35,54,70 In the third de-
old man with a ductal membrane.53 Rarely, a closed lu- cade, more and more patients with a moderately restric-
men is reopened by spontaneous intramural dissection tive ductus experience heart failure,35,54 and those with a
of a ductal aneurysm or by propagation of aortic dissec- restrictive ductus remain asymptomatic. A 20-year-old
tion into the ductus.16 man with a patent ductus had been a cross-country run-
Patent ductus arteriosus predominates in females, ner, and an active schoolmistress died at the age of
with a gender ratio of 2 or 3 to 1.35 Female prevalence 85 years because of gastrointestinal bleeding. A number
is even greater in older patients.54 There is a tendency of reports have called attention to survival beyond age
for recurrence of patent ductus in siblings55–57 and in 60 years (see Figure 20-16)35,71–75: an elderly woman
the offspring of parents with patent ductus.55 Familial represented with biventricular failure in her 81st year,76
currence has been reported in three generations of a sin- and a patient died at 90 years of age.77
gle family.58 Identical twins may both have a patent A nonrestrictive patent ductus with Eisenmenger’s
ductus, or the ductus may be patent in only one twin. syndrome is accompanied by the multisystem systemic
Canine patent ductus is more common in females and disorders of cyanotic congenital heart disease (see
can be hereditary.59 Chapter 17).78,79 Isotonic exercise with an Eisenmenger’s
In offspring of gravida with maternal rubella, patent ductus causes leg fatigue without dyspnea because an
ductus arteriosus and pulmonary artery stenosis coexist exercise-induced increase in right-to-left shunt is
as congenital malformations (see Chapter 11).60–62 Mater- channeled into the descending aorta (Figures 20-2 and
nal rubella resulted in patent ductus arteriosus in one of a 20-11) distal to the respiratory center and the carotid
twin pair; the other twin had pulmonary artery stenosis. body, precluding hypoxia-induced stimulation.78–81
Low birth weight and failure to thrive are features of the Hypertrophic osteoarthropathy is confined to the lower
rubella syndrome, even if the ductus is restrictive. A sea- extremities.82–84 In an Eisenmenger’s ductus, left ventric-
sonal incidence of patent ductus in late winter and early ular failure is absent because volume overload of the left
spring coincided with the peak incidence of rubella.62 heart is curtailed. A dilated hypertensive pulmonary trunk
Persistent patency of the ductus arteriosus is about six may cause hoarseness by compressing the recurrent laryn-
times more prevalent in high-altitude locations than in geal nerve. Angina and syncope are not features of nonre-
sea-level locations.63 A predilection for increased pulmo- strictive patent ductus with reserved shunt because right
nary vascular resistance is a feature of high-altitude births ventricular pressure cannot exceed systemic.79 Cyanosis
with patent ductus.63 The predilection exists even when is missed if the feet are not examined (see section Physical
the ductus is restrictive.63 Appearance). A young girl came to attention because she
Congestive heart failure is the most common cause noticed that when she sat in a warm bath, her toes were
of death directly related to patent ductus.35 Rarely, death blue but her fingers were pink.
is from dissection or rupture of a ductal aneurysm64 Constriction or closure of the fetal ductus deprives the
or from rupture of a hypertensive aneurysmal pulmo- right ventricle of its only outlet, so neonates present with
nary trunk.65,66 Aneurysm of a nonpatent ductus (see massive tricuspid regurgitation and right-to-left inter-
Figure 20-6) can be complicated by rupture, by spontane- atrial shunts.24 Salicylates cause constriction of the fetal
ous intramural dissection, by systemic embolism, by ductus, so the history should include enquiries about ma-
infection, by recurrent laryngeal nerve paralysis, by ternal use of aspirin. Salicylate levels can be determined
compression of the pulmonary trunk, or by hemorrhagic on umbilical cord blood.85
erosion into the esophagus or tracheobronchial tree.8,9
Infective endarteritis occurs with a restrictive patent
ductus because of the high-velocity left-to-right shunt PHYSICAL APPEARANCE
but does not occur with a nonrestrictive ductus and re-
versed shunt.35 The infection is located at the narrow pul- Maternal rubella is characterized by low birth weight
monary arterial end of the ductus or at the site of an and failure to thrive, irrespective of ductal patency,
intimal jet lesion in the pulmonary artery opposite the ductal size, or shunt volume.86,87 An underdeveloped
PDA
Ao
PT
RV LV
A B
FIGURE 20-11 A, Photographs of a 28-year-old woman with patent ductus arteriosus, suprasystemic pulmonary vascular resistance, and
reversed shunt. The upper photograph shows the patient sitting with her hands placed on the dorsum of her feet. The right hand is acya-
notic, and the digits are not clubbed. The left hand is mildly cyanotic, and the thumb is clubbed. The toes are cyanotic and clubbed. In the
close-up (lower photograph), the right hand is acyanotic, and the thumb is not clubbed (arrow). The left hand is mildly cyanotic, and the
thumb is clubbed (arrow). B, Magnetic resonance image from a 29-year-old woman with a nonrestrictive patent ductus and reversed
shunt (curved arrow) from the pulmonary trunk (PT) through a nonrestrictive patent ductus arteriosus (PDA) into the aorta (Ao). (RV/LV ¼
right ventricle/ left ventricle.)
child with a patent ductus should therefore be examined the rare presence of bilateral patent ductuses with re-
for cataracts, deafness, and mental retardation (see versed shunt, the right arm is cyanosed because the right
Figure 20-9).86,87 Another distinctive phenotype is the subclavian artery receives desaturated blood from the
clinodactly (overlapping fingers), rocker bottom feet, pulmonary artery via the right ductus arteriosus.12
and lax skin of Trisomy 18 (Figure 20-12).88,89 Char syn- Patients who are old enough to follow instructions
drome is an inherited disorder that maps to chromosome should be examined sitting or squatting with their hands
6p12-p21 and is characterized by ptosis, a flat profile, a placed alongside their feet or on the dorsum of their feet
very short philtrum, patulous duck-bill lips, facial dys- to facilitate comparison of fingers and toes (see Figure
morphism, and abnormalities of the hands.90–93 The re- 20-11A). The right and left thumbs should be compared
currence rate in offspring of an affected parent is 50%.92 (see Figure 20-11A). Differential cyanosis is exaggerated
Differential cyanosis and clubbing are important phys- by isotonic exercise or by warming the hands and feet,
ical signs of patent ductus with reversed shunt (see which are maneuvers that increase skin blood flow and
Figures 20-2 and 20-11; see previous).79,94 The toes are exaggerate the color differences. In neonates with persis-
cyanosed and clubbed because unsaturated blood is selec- tent fetal circulation, the right-to-left ductal shunt may
tively delivered to the lower extremities. A small amount cause distinctive differential cyanosis confined to the
of unsaturated blood often enters the left subclavian ar- head, right shoulder, and right arm with a demarcation
tery, so the digits of the left hand, especially the thumb, line that runs obliquely from above the left shoulder
are mildly cyanosed and clubbed (see Figure 20-11A). to below the right axilla.18
The fingers of the right hand are normal because unsatu- Healthy individuals, especially young women, may
rated blood does not reach the right subclavian artery. In have peripheral cyanosis of the feet because of
FIGURE 20-12 A, Photographs of a female infant with

patent ductus arteriosus, ventricular septal defect, and
the physical appearance of trisomy 18 with lax skin
and overlapping fingers (clinodactly). B, Rocker-
bottom feet in A are shown. A B
vasoconstriction, a mechanism that is suspected when the When pulmonary vascular disease reverses the shunt,
feet are cold. Diagnostic error is prevented by warming the arterial pulse is usually normal. However, in the
the extremities, which abolishes peripheral cyanosis presence of pulmonary hypertensive pulmonary regurgi-
but exaggerates central cyanosis. tation, the aortic diastolic pressure is lowered and the
pulse pressure widens because diastolic flow is from
the aorta through the ductus into the pulmonary artery,
across the incompetent pulmonary valve, and into the
ARTERIAL PULSE right ventricle.
Wide systemic pulse pressure is an important physical

sign of patent ductus arteriosus with a large left-to-
right shunt.40,94 This sign is especially useful in symp- JUGULAR VENOUS PULSE
tomatic neonates without a ductus murmur. However,
the arterial pulse may be weak in preterm infants in Congestive heart failure is accompanied by a rise in mean
whom systemic flow is reduced by the steal effect asso- jugular venous pressure with an increase in the A and V
ciated with aortopulmonary shunting (see previous).37,38 waves. When suprasystemic pulmonary vascular resistance
The typical pulse is characterized by a brisk rise, a single reverses the shunt, the A wave is relatively unimpressive
or bisferiens peak, and a rapid collapse (Figure 20-13). because the right ventricle adapts to systemic vascular
Diastolic flow from aortic root into pulmonary trunk resistance without augmented right atrial contraction.
lowers the aortic diastolic pressure, and a large left ven-
tricular stroke volume with forceful left ventricular con-
traction maintains or elevates aortic systolic pressure.
The carotid, brachial, femoral, and even dorsalis pedis PRECORDIAL MOVEMENT
pulses can be bounding.94 The superficial palmar arch AND PALPATION
as it crosses the heads of the metacarpals is sometimes
evident as a visible pulsation. In a child with a large pat- George A. Gibson96 wrote, “When the ductus arteriosus is
ent ductus, an erythematous wheal caused by a mosquito permanently patent, a very distinct thrill is to be felt—a thrill
bite blushed and blanched synchronously with the which distinctly follows the systole of the heart and persists
pulse.95 until the diastolic phase has existed for some time.”2
Normal Patent ductus murmur is rough and thrilling. It begins softly and in-
creases in intensity so as to reach its acme just about,
or immediately after the incidence of the second sound,
and from that point gradually wanes until its
termination.”
Gibson’s description cannot be improved on. Al-
BA FA Aorta though he was not the first to describe the continuous
murmur of patent ductus, he precisely characterized
the murmur and confidently established the clinical di-
agnosis based on that characterization.2 The classic mur-
mur of uncomplicated patent ductus arteriosus rises to a
peak in latter systole; continues without interruption
through the second sound, which it envelops; and then
declines in intensity during the course of diastole
(Figures 20-14, 20-15, and 20-16). The murmur can oc-
cupy the entire cardiac cycle (see Figure 20-14), or the
end of diastole or early systole can be murmur-free (see
Figure 20-15). The term continuous is best applied to the
uninterrupted progression of the murmur through the sec-
ond heart sound and not to the presence of murmur
throughout the cardiac cycle.94 The ductus murmur is
20 20 20 therefore considered continuous even when late diastole
and early systole are murmur-free.
High-velocity flow through a restrictive ductus gener-
0 0 0 ates a relatively soft high-frequency continuous murmur.
A moderately restrictive ductus generates a loud coarse
FIGURE 20-13 Femoral arterial (FA) and central aortic pulses in
two patients aged 18 months and 22 months, both with a
nonrestrictive patent ductus arteriosus and large left-to-right
shunts. Pulse pressures are wide with a brisk rate of rise, a S2
single or bisferiens (twin) peak, and a rapid collapse. A normal
brachial arterial pulse in the left panel is shown for comparison.
S1
Intracardiac
(Pulmonary artery)
A moderately restrictive patent ductus is accompanied

by volume overload of the left ventricle and a dynamic
left ventricular impulse. A right ventricular impulse is rel-
atively unimpressive because pressure overload of the
right ventricle is only moderate.
A nonrestrictive ductus with low pulmonary vascular re- Thoracic wall
sistance results in appreciable volume overload of the left (Second LICS)
ventricle and systemic systolic pressure in the right ventri- S1
cle. The left ventricular impulse is hyperdynamic, and the
right ventricular impulse is sustained. A dilated pulmonary
S2
trunk and a loud pulmonary closure sound are palpable to-
gether. If a thrill is present, it is likely to be confined to sys-
tole (see section Auscultation). When flow through the
ductus is reversed, pulmonary hypertension exists without
volume overload of the left ventricle, so palpation detects
only a right ventricular impulse. Symptomatic infants with
a nonrestrictive patent ductus and low pulmonary vascular
resistance have a hyperdynamic volume-overloaded left
ventricular impulse accompanied by the conspicuous im-
pulse of a hypertensive failing right ventricle.
FIGURE 20-14 Simultaneously recorded phonocardiograms
from within the pulmonary artery and on the thoracic wall at
the second left intercostal space of a 7-year-old girl with a mod-
AUSCULTATION erately restrictive patent ductus arteriosus and a 2 to 1 left-to-
right shunt. In Gibson’s words, the murmur “begins softly and
In 1900, Gibson96 characterized the murmur of patent increases in intensity so as to reach its acme just about, or im-
mediately after the incidence of the second heart sound, and
ductus arteriosus:“It persists through the second sound
from that point gradually wanes until its termination.”96
and dies away gradually during the long pause. The
S1 S2
MDM (Apex)
S2
2 LICS
P2
CAR
EDM
E
A2
P2
FIGURE 20-15 Schematic sequential modifications of the mur-

mur of patent ductus from a nonpulmonary hypertensive left-to-
right shunt (top) to a pulmonary hypertensive right-to-left shunt
(bottom). (MDM ¼ mid-diastolic murmur; EDM ¼ early dia-
stolic murmur; A2/P2¼ aortic and pulmonary components of FIGURE 20-16 Tracings from an 84-year-old woman with a
the second heart sound.) moderately restrictive patent ductus arteriosus (see x-ray in
Figure 20-38). The murmur in the second left intercostal
space (2 LICS) continues through the second heart sound that
was timed by the dicrotic notch of the carotid pulse (CAR).
machinery murmur punctuated with eddy sounds that are The continuous murmur then faded, rendering late diastole
randomly distributed in the second half of systole and in murmur-free.
the first half of diastole (Figure 20-17A).44,94
Intracardiac phonocardiography records the maximal in-
tensity of the ductus murmur in the pulmonary artery at
the pulmonary ostium of the ductus (see Figure 20-14). reappearance of an otherwise typical continuous ductal
Intraoperative phonocardiograms from the surface of the murmur.52,100,101 Interruption of flow has been ascribed
pulmonary trunk are in accord with this localization,97 to acute angulations of an elongated ductus100,101 or to a
and these observations coincide with the chest wall posi- valve or veil-like structure within the ductus (see previ-
tion of the ductus murmur, which is loudest in the first or ous).52 A similar mechanism has been proposed for the
second intercostal space or beneath the left clavicle as transient diastolic ductal murmur of the neonate.102
Gibson originally stated.2 Reappearance of a continuous murmur long after ductal
Occasionally, a short large ductus is devoid of mur- closure has been ascribed to reopening caused by a tear
mur despite a substantial left-to-right shunt in the valve of the ductus50 or to spontaneous intramural
(Figure 20-18). Ductal murmurs are often absent in in- dissection.
fants with a nonrestrictive patent ductus and congestive The shape, length, and timing of the murmur of patent
heart failure (see Figure 20-10) and in a significant num- ductus arteriosus depend on instantaneous differences in
ber of preterm infants with respiratory distress.98 Fail- pressure and flow between the aorta and pulmonary
ure to detect a murmur, a silent but patent ductus, trunk (see Figure 20-15).103,104 At the beginning of sys-
does not necessarily mean that the ductus has closed tole, flow into the pulmonary artery is derived from
(see section The History).17,43,99 A relatively rare aus- the right ventricle rather than the ductus. During the
cultatory variation is intermittent disappearance and course of systole, the flow contribution from the ductus
SM 4 LICS
progressively increases, and during diastole, flow into the
1 LICS
pulmonary artery is from the ductus alone.94,105 Systolic
reinforcement of the ductus murmur described by Skoda
S1 APEX
occurs because flow from aorta into pulmonary trunk is
S1 greater in systole, especially when the systemic pulse
pressure is wide, and because systolic flow from right
A2 ventricle into pulmonary artery is reinforced by simulta-
neous flow from the ductus, whereas diastolic flow is de-
MDM rived from the ductus alone. As pulmonary vascular
CAR
resistance rises, the pulmonary arterial and aortic dia-
A2 stolic pressures equalize (Figure 20-19) and diastolic duc-
tal flow diminishes and finally vanishes, so the diastolic
portion of the continuous murmur disappears, leaving
a holosystolic murmur (Figures 20-15, 20-20, and
20-21). With a further increase in pulmonary vascular re-
sistance, the systolic portion of the ductus murmur
shortens (see Figures 20-15 and 20-21) and ultimately
disappears altogether (see Figure 20-15). The ductus is
then silent because right-to-left ductal flow, a reversed
shunt, does not generate a murmur.105 The classic Gibson
murmur is then replaced by auscultatory signs of pulmo-
nary hypertension: namely, a short pulmonary midsysto-
lic murmur introduced by an ejection sound, a single or
closely split second heart sound, a loud pulmonary com-
ponent, and the Graham Steell murmur of hypertensive
A B pulmonary regurgitation (Figures 20-15 and 20-22).105
The diagnosis of patent ductus arteriosus cannot be based
FIGURE 20-17 Tracings from an 18-year-old woman with a on auscultatory signs but can confidently be based on dif-
nonrestrictive patent ductus arteriosus and increased pulmo- ferential cyanosis (see Figure 20-11; see section Physical
nary vascular resistance, but a 2.3 to 1 left-to-right shunt.
A, The ductus murmur in the first left intercostal space (1 LICS)
Appearance).
continued (paired arrows) for a short time after the aortic In the newborn, a transient soft crescendo systolic
component of the second heart sound (A2). Eddy sounds (lower murmur from left-to-right flow through the ductus is
arrows) punctuate the murmur. (CAR ¼ carotid pulse.) B, In the sometimes detected before normal physiologic clo-
fourth left intercostal space (4 LICS), the ductus murmur is sure.106–108 The murmur ends with the second sound
holosystolic (SM) and devoid of eddy sounds. The short, or continues just beyond it.106 These harmless transient
low-frequency, mid-diastolic murmur (MDM) at the apex was neonatal murmurs are physiologically analogous to the
caused by augmented flow across the mitral valve. not so harmless murmurs that appear when patent ductus
2 LICS
S1 A2
P2
Apex
FIGURE 20-18 Tracings from a 30-

year-old man with an unusually
large, short patent ductus. Despite
a 2 to 1 left-to-right shunt, a ductus
CAR murmur was neither heard nor
recorded. (2 LICS ¼ second left inter-
costal space.) Relatively wide expi-
ratory splitting of the second heart
sound was caused by delay in the
loud pulmonary component (P2) that
was transmitted to the apex. (S1 ¼ first
heart sound; A2 ¼ aortic component
of the second sound; CAR ¼ carotid
pulse.)
2 LICS
SM
S1
SM
S1
First left ICS P2

Bifur. PA Bifur. PA Main PA RV
A
BA
BA
Aorta
IN PA FIGURE 20-20 Phonocardiograms from an 11-year-old girl

PA
P2 with a nonrestrictive patent ductus arteriosus and increased
pulmonary vascular resistance, but a 2 to 1 left-to-right shunt.
The upper left panel shows a holosystolic ductal murmur (SM)
in the second left intercostal space (2 LICS). A louder holosys-
tolic ductal murmur was recorded from within the pulmonary
artery at its bifurcation (Bifur. PA). The remaining panels show
the intracardiac the murmur fading then vanishing as the
catheter tip microphone was withdrawn from the bifurcation
of the pulmonary artery to the main pulmonary artery and into
right ventricle (RV).
20 20
When the left-to-right shunt is large, apical mid-

0 0 diastolic murmurs are generated by increased flow across
B the mitral valve.105 These murmurs cannot not heard un-
less the diastolic portion of the continuous murmur is at-
FIGURE 20-19 Tracings from a 15-year-old boy with a nonre- tenuated by an increase in pulmonary vascular resistance
strictive patent ductus arteriosus and increased pulmonary vas- (Figures 20-15, 20-17, and 20-23).105 In patent ductus
cular resistance, but a 2 to 1 left-to-right shunt. A, The ductus arteriosus with reversed shunt, a mid-diastolic murmur
murmur in the first left intercostal space (ICS) continued has been attributed to a right-sided Austin Flint mechanism
through the timing of the second heart sound (paired arrows) associated with pulmonary regurgitation.109 To-and-fro
but faded well before the subsequent first heart sound (S1).
murmurs over the cranium of infants with a nonrestrictive
B, An identical murmur was recorded within the pulmonary ar-
tery (IN PA) at its bifurcation. The brachial arterial (BA) and patent ductus arteriosus have been ascribed to accelerated
pulmonary arterial (PA) pulses diverge in systole but converge forward flow followed by rapid diastolic runoff.
in diastole (center panel), so the ductus murmur is maximal in The second heart sound is occasionally paradoxically
systole and minimal in diastole (left panel). The right panel split when the left-to-right shunt is large.110 Prolonged
shows relatively low diastolic pressures in the brachial artery left ventricular ejection and short right ventricular ejec-
and in the aorta with a bisferiens (twin-peaked) pulse in the tion are held responsible.40,110 A loud continuous mur-
central aorta. mur punctuated by eddy sounds obscures the second
heart sound. An increase in pulmonary vascular resistance
renders the second sound audible because the diastolic
portion of the continuous murmur softens or disappears
(see Figures 20-15, 20-17, and 20-19). When the shunt is
arteriosus is subsequently accompanied by a rise in pul- reversed, the second sound is single or closely split, and
monary vascular resistance. Even when a ductus is des- the pulmonary component is loud (see Figure 20-15).
tined to remain patent, the neonatal ductal murmur is The second sound is widely split when depressed
initially systolic and becomes continuous only after pul- right ventricular contractility and prolonged right ventric-
monary vascular resistance has fallen sufficiently to per- ular ejection delay the pulmonary component (see
mit both systolic and diastolic ductal flow. Figures 20-22 and 20-23).
SM
PDA DM
SM
S1 S2 S3
S1 P2 QRS
P
RV
FA
PA FIGURE 20-23 Phonocardiogram at the apex of a 5-year-old girl
with a nonrestrictive patent ductus and a 3 to 1 left-to-right shunt.
The systolic portion of the ductus murmur (SM) was transmitted to
the apex where a prominent middiastolic murmur (DM) was
caused by augmented flow across the mitral valve.
ELECTROCARDIOGRAM
20
A moderately restrictive patent ductus arteriosus with in-
creased pulmonary blood flow is accompanied by a pro-
longed bifid left atrial P wave in one or more limb
0 leads and in right precordial leads (Figures 20-24 and
FIGURE 20-21 Tracings from a 3-year-old girl with a nonre- 20-25). The PR interval is prolonged in 10% to 20%
strictive patent ductus and increased pulmonary vascular resis- of cases (see Figure 20-24).111 Atrial fibrillation occurs
tance but a 2.7 to 1 left-to-right shunt. The intracardiac in older patients. The QRS axis is usually normal, but
microphone recorded a decrescendo holosystolic murmur an occasional infant has right axis deviation, especially
within the lumen of the patent ductus arteriosus (PDA). The right
ventricle (RV) was silent except for the pulmonary component
of the second heart sound (P2). The femoral arterial (FA) and pul-
monary arterial (PA) pulses diverge in systole but are identical in
diastole, so the ductus murmur was confined to systole.
SM EDM 1 2 3 aVR aVL aVF
S1 A2 P2
QRS
V1 V2 V3 V4 V5 V6
FIGURE 20-24 Electrocardiogram from a 37-year-old woman

FIGURE 20-22 Phonocardiogram from the second left intercostal with a moderately restrictive patent ductus arteriosus, a 2.8 to
space of a 26-year-old woman with a nonrestrictive patent ductus, 1 left-to-right shunt, and a pulmonary arterial pressure of
suprasystemic pulmonary vascular resistance, and reversed shunt. 48/18 mm Hg (see x-ray in Figure 20-32). There is a broad bifid
A midsystolic murmur (SM) originated in the dilated hypertensive left atrial P wave in lead 2. The PR interval is 200 msec. The
pulmonary trunk. A Graham Steell murmur (EDM) issued from a QRS is prolonged, and the axis is horizontal. Left ventricular
loud delayed pulmonary component of the second heart volume overload is manifested by prominent q waves in leads
sound (P2). (S1 ¼ first heart sound; A2 ¼ aortic component of aVL and V6 and by tall R waves and ST-T wave changes in lead
the second sound.) aVL and in leads V4-6.
1 2 3 aVR aVL aVF 1 2 3 4 5 6
V1 V2 V3 V4 V5 V6
FIGURE 20-26 Electrocardiogram from a 16-year-old boy with

a moderately restrictive patent ductus, pulmonary arterial pres-
sure of 80/50 mm Hg, and a 2 to 1 left-to- right shunt. The QRS
V1 V2 V3 V4 V5 V6 axis is normal and depolarization is clockwise, so prominent
q waves and tall R waves of left ventricular volume overload ap-
FIGURE 20-25 Electrocardiogram from a 19-month-old male pear in leads 2, 3, and aVF. Volume overload of the left ventricle
with a moderately restrictive patent ductus, a 3 to 1 left-to-right is also manifested by the deep S wave in lead V1 and by prom-
shunt, and a pulmonary arterial pressure of 45/22 mm Hg (see inent q waves, tall R waves, and peaked upright T waves in leads
x-ray in Figure 20-31). There are biphasic left atrial P waves in V5-6. Biventricular hypertrophy is reflected in the large RS com-
leads V1-2. Because the QRS axis is normal, the tall R waves of left plexes in leads V2-4, which are half standardized.
ventricular volume overload appear in leads 2 and aVF. Volume
overload of the left ventricle is also manifested by tall R waves
and upright T waves in leads V5-6. Right ventricular hypertrophy
is manifested by prominent S waves in leads V5-6 and a prominent
R wave in lead V1. Leads V2-3 are half standardized and exhibit
large RS complexes of biventricular hypertrophy.
neonates with respiratory distress. Rare examples of left

axis deviation have been reported.112 The rubella syn-
drome may be associated with an unusually superior
QRS axis directed upward and either to the left or right.113 1 2 3 aVR aVL aVF
A nonrestrictive patent ductus with low pulmonary
vascular resistance is associated with biatrial P waves
and combined ventricular hypertrophy. Large equidi-
phasic RS complexes appear in most if not all precordial
leads, with tall R waves and prominent S waves in leads
V5-6 (Figures 20-25 and 20-26). Volume overload of
the left ventricle is responsible for tall R waves, promi-
nent q waves, and tall peaked T waves in leads V5-6
(see Figures 20-25 and 20-26). V1 V2 V3 V4 V5 V6
Patent ductus with pulmonary vascular disease and re-
versed shunt is accompanied by peaked right atrial FIGURE 20-27 Electrocardiogram from a 28-year-old woman
P waves in leads 2, 3, and V1 (Figure 20-27). Right ven- with a non-restrictive patent ductus, suprasystemic pulmonary
tricular hypertrophy is manifested by right axis deviation, vascular resistance, and reversed shunt. Peaked right atrial
tall R waves in lead V1, and inverted right precordial P waves are present in lead 2 and leads V1-2. Isolated right ven-
T waves and is prominent in left precordial leads S waves tricular hypertrophy is manifested by right axis deviation, tall
monophasic R waves, and inverted T waves in leads V1-4 and
(see Figure 20-27). R waves in leads V5-6 imply that a left-
prominent S waves in left precordial leads.
to-right shunt previously existed (see Figure 20-27).
X-RAY Figures 20-5 and 20-30). After birth, left-to-right

ductal flow recirculates through the aortic root, so the as-
The ductus itself is occasionally seen in the frontal projec- cending aorta becomes prominent (see Figure 20-3).117
tion as an inconspicuous soft convexity between the aortic In infants and children with a nonrestrictive patent ductus
knob and the pulmonary artery segment (Figures 20-5A and low pulmonary vascular resistance, pulmonary
and 20-28).114 A striking exaggeration of this inconspicu- arterial vascularity is markedly increased and all four car-
ous shadow is an aneurysm of a nonpatent ductus (see diac chambers are enlarged (Figure 20-33).
Figure 20-6). Calcium appears in the ductus of older The x-ray of a nonrestrictive patent ductus with re-
patients (Figure 20-29; see Figure 20-38A).115 versed shunt exhibits reduced pulmonary vascularity, di-
A moderately restrictive patent ductus with low pul- lation of the pulmonary trunk and its proximal branches,
monary vascular resistance results in increased pulmo- a normal or near normal left ventricle and left atrium, and
nary arterial vascularity, enlargement of the pulmonary an hypertrophied but not significantly dilated right ven-
trunk and its proximal branches, and enlargement of tricle (Figure 20-34).
the left atrium and left ventricle (Figures 20-30, 20-31
and 20-32).114 Asymmetric pulmonary vascularity is
occasionally represented by a hyperlucent left lung.116 ECHOCARDIOGRAM
In infants, the ascending aorta is inconspicuous because
intrauterine ductal flow does not traverse the aorta (see Echocardiography with color flow imaging and Doppler
interrogation establishes the size of the patent ductus
(Figure 20-35), the flow dynamics through the ductus,
and the physiologic consequences of persistent ductal
patency.103,104,118 Transesophageal echocardiography
AO improves diagnostic accuracy.119 When the shunt is
entirely left-to-right, the flow disturbance within the
ductus is continuous, with the peak velocity reinforced
in latter systole as forward flow from the right ventricle
coincides with shunt flow through the ductus
(Figure 20-36).103,104 Systolic forward flow in the
aorta distal to the orifice of the ductus is followed by re-
versed diastolic flow (Figure 20-37). In the presence
of large ductal flow, the velocities across the aortic isth-
mus and in the descending aorta are substantially
PT increased.120 The color flow pattern in the pulmonary
trunk consists of a ductal jet that adheres the lateral
wall, travels toward the pulmonary valve, and then re-
verses itself to travel up the medial wall (Figure 20-38
and Video 20-1). Alternatively, a jet directed toward
the pulmonary valve adheres to the medial wall of the
pulmonary trunk (Figure 20-39).
A Color flow imaging with Doppler interrogation iden-
tifies a clinically silent nonrestrictive patent ductus in
premature infants33,42 and detects the tiny clinically
silent ductus in adults (Figure 20-40 and Video 20-2).67
In utero ductal closure can be recognized with fetal
AO echocardiography.121,122 The reversed shunt in a nonre-
PT
strictive patent ductus with suprasystemic pulmonary
vascular resistance can be identified with Doppler
interrogation103 and contrast echocardiography.123 Right
ventricular systolic pressure and pulmonary arterial dia-
stolic pressure are estimated with continuous-wave
Doppler interrogation of the jets of tricuspid and pulmo-
B nary regurgitation and with Doppler velocities across the
ductus.
FIGURE 20-28 A, Close-up of a portion of the chest x-ray in the
14-year-old girl whose aortogram is shown in Figure 20-3. The
soft convex shadow of the patent ductus (arrow) is located be- SUMMARY
tween the aortic knuckle (AO) and pulmonary trunk (PT). The
convex shadow represents the dilated aortic end of the conical Clinical suspicion of patent ductus arteriosus is heightened
ductus shown in the aortogram of Figure 20-3. B, Aortogram in a by premature birth, maternal rubella, or birth at high alti-
5-year-old girl. The convex shadow of a conical ductus (arrow)
tude. In most patients with a moderately restrictive patent
located between the aortic knuckle (AO) and the pulmonary
trunk (PT). Compare with Figure 20-5.
ductus, the clinical signs are unmistakable. The arterial
pulse is brisk, the pulse pressure is wide, the left
A B
FIGURE 20-29 X-ray from a 63-year-old woman with a restrictive patent ductus and a left-to- right shunt of 1.3 to 1. A, Ductal calci-
fication is the comma-like density between the aortic knuckle and the main pulmonary artery segment. B, Close-up of the ductal cal-
cification (arrow). The x-ray was otherwise normal.
ventricular impulse is dynamic, and auscultation detects ventricle, left atrium, ascending aorta, and pulmonary
the distinctive continuous Gibson murmur that peaks trunk. Echocardiography with color flow imaging and
around the second heart sound and is punctuated by eddy Doppler interrogation establishes the size of the ductus
sounds. The electrocardiogram reflects volume overload of and the flow dynamics within the ductus and within the
the left ventricle, and the x-ray shows increased pulmo- contiguous aorta and pulmonary trunk and establishes
nary arterial vascularity with enlargement of the left the hemodynamic consequences of ductal patency.
PT
A B
FIGURE 20-30 X-rays from a 13-month-old female with a moderately restrictive patent ductus arteriosus, pulmonary arterial pressure of
39/15 mm Hg, and a 2.3 to 1 left-to-right shunt. A, Pulmonary vascularity is increased and the pulmonary trunk (PT) and its right branch
(white arrow) are prominent, but the ascending aorta is inconspicuous. The left cardiac border formed by a dilated left ventricle (vertical
black arrow) and the right cardiac border is formed by a dilated right atrium (horizontal black arrow). B, The femoral artery catheter
passed through the ductus (first acute bend, horizontal arrow), into the pulmonary trunk, across the pulmonary valve into the right ven-
tricle (right vertical arrow), and finally across the tricuspid valve into the right atrium where the tip lies (left vertical arrow).
PT
RA
LA
LV
A B
FIGURE 20-31 X-rays from a 19-month-old male with a nonrestrictive patent ductus and a 3 to 1 left-to-right shunt. The electrocardio-
gram is shown in Figure 20-25. A, The increased pulmonary vascularity is both arterial and venous. The pulmonary trunk (PT) and its right
branch (arrow) are dilated. The apex is formed by an enlarged left ventricle (LV), and the right cardiac border is formed by a dilated right
atrium (RA). B, The lateral barium esophagram outlines a moderately enlarged left atrium (LA).
PT
FIGURE 20-32 A, X-rays from a 37-year-old

woman with a moderately restrictive patent duc-
RA tus and a 2.8 to 1 left-to-right shunt. The electro-
cardiogram is shown in Figure 20-24. Pulmonary
vascularity is increased, the pulmonary trunk
LV (PT) is prominent, the apex is occupied by a di-
lated convex left ventricle (LV), and the right car-
diac border is formed by a dilated right atrium
(RA). B, Lateral barium esophagram outlines an
A B enlarged left atrium.
PT LPA
RPA
Ao
RA
LV
A B
FIGURE 20-33 A, X-ray from a 3-year-old boy with a nonrestrictive patent ductus, low pulmonary vascular resistance, and a 3 to 1 left-to-
right shunt (see Figure 20-5 for angiocardiograms). Pulmonary blood flow is increased, the pulmonary trunk (PT) is dilated, an enlarged left
ventricle (LV) occupies the apex, and an enlarged right atrium (RA) forms the right cardiac border. B, The right pulmonary artery (RPA) and
left pulmonary artery (LPA) are visualized through the patent ductus after contrast material was injected into the balloon-occluded descend-
ing aorta (Ao). The intrapulmonary arteries are strikingly enlarged. Compare with the increased pulmonary vascularity in A.
FIGURE 20-34 X-rays from a 14-

year-old boy with a nonrestrictive
patent ductus, suprasystemic pulmo-
nary vascular resistance, and
reversed shunt. A, Pulmonary vascu-
PT
larity is decreased, the pulmonary
trunk (PT) and its proximal branches
are enlarged, the right atrium (RA) is PDA
prominent, and the hypertrophied
right ventricle (RV) forms an acute
PT
angle with the left hemidiaphragm. RA
B, A catheter from the left median
basilic vein entered the pulmonary
trunk (PT), crossed the ductus, and
came to rest in the descending aorta RV
(DAo). The course of the catheter rep- DAo
resents the pathway taken by unoxy-
genated blood through a reversed
ductal shunt (see Figure 20-2,lower,
and Figure 20-11A). A B
V .64
5
10
DA
–.64
4
PT
3
LA 2
Ao
1
RA [m/s]
–1 62
–2.0 –1.5 –1.0 –0.5 0.0 HR
100 mm/s
FIGURE 20-36 Continuous-wave Doppler scan from a 7-month-

old female with a moderately restrictive patent ductus.
clinical signs are those of pure pulmonary hypertension.

FIGURE 20-35 Echocardiogram of a moderately restrictive pat- The electrocardiogram shows right ventricular hyper-
ent ductus (three arrowheads) in a 2-month-old infant. (Ao ¼ trophy with little or no volume overload of the left ven-
aortic valve; PT ¼ pulmonary trunk; DA ¼ descending aorta;
tricle. The x-ray exhibits normal or reduced pulmonary
RA/LA ¼ right atrium and left atrium.)
vascularity, dilation of the pulmonary trunk, and an unim-
pressive cardiac silhouette. Echocardiography with color
In infants, a nonrestrictive patent ductus with low pul- flow and contrast imaging confirms the diagnosis.
monary vascular resistance presents with congestive heart
failure. An incriminating ductus murmur is often absent,
but the arterial pulses are bounding and the left and right
ventricular impulses are hyperdynamic. Echocardiogra- AORTOPULMONARY WINDOW
phy establishes the diagnosis.
In a nonrestrictive patent ductus with suprasystemic In a lecture delivered in 1830 at the St Thomas Hospital,
pulmonary vascular resistance and reversed shunt, the London, Professor John Elliotson124 described the first
ductus murmur is absent, but the diagnosis is confidently known case of aortopulmonary window, often called
made based on distinctive differential cyanosis. The toes aortopulmonary or aorticopulmonary septal defect. This
are cyanosed and clubbed, but the fingers are spared. The uncommon malformation consists of a communication,
pulmonary channels.125–127 The distal truncoaortic sac

is then divided by the aortopulmonary septum. Maldeve-
lopment of the truncal and aortopulmonary septum re-
Diast. sults in three morphologic types of aortopulmonary
window, namely125: (1) nonfusion of the embryonic aor-
topulmonary septum and the truncal septum that results
in a moderate-sized circular defect located about midway
between the great arterial valves and the bifurcation of
the pulmonary trunk; (2) malalignment of the embryonic
aortopulmonary septum and truncal septum that results
in a defect similarly located but helical-shaped; and
(3) complete absence of the embryonic aortopulmonary
septum that results in a nonrestrictive defect. The mor-
phogenesis of an aortopulmonary window is unrelated
to the morphogenesis of a patent ductus arteriosus, but
the physiologic consequences and clinical manifestations
Syst. of the two malformations are similar if not identi-
cal,128,129 so inclusion in this chapter is appropriate.
FIGURE 20-37 Pulsed Doppler scan from a 6-month-old fe-
male with a moderately restrictive patent ductus and a 2.8 to Because an aortopulmonary window tends to be
1 left-to-right shunt. Sample volume in the descending aorta dis- nonrestrictive, it is often mistaken for a nonrestrictive
tal to the ductus recorded systolic flow (Syst.) down the aorta patent ductus, which is the most frequent coexisting
and diastolic flow (Diast.) in the opposite direction. anomaly, estimated to have an incidence rate of 12%
(Figure 20-41).129 When pulmonary vascular resistance
is suprasystemic and the shunt is reversed, the clinical
picture is indistinguishable from a nonrestrictive ventric-
usually nonrestrictive, between adjacent walls of the ular septal defect with Eisenmenger’s syndrome (see
ascending aorta and pulmonary trunk. During early em- Chapter 17).79 An aortopulmonary window and a
bryogenesis, two opposing proximal truncal cushions ventricular septal defect can coexist, albeit rarely.130
rapidly enlarge and fuse to form the truncal septum that Aortopulmonary window is somewhat more frequent
partitions the truncus arteriosus into aortic and in males in contrast to patent ductus.128 Also in contrast
Ao
PDA
PT V .64
–.64
Ao
PT
10 PDA
LV LPA
A B 73
HR
FIGURE 20-38 A, X-ray from the 84-year-old woman with a moderately restrictive patent ductus arteriosus (PDA) whose phonocardio-
gram is shown in Figure 20-16. The pulmonary trunk (PT) and its right branch are dilated. A thin rim of calcium appears in the transverse
aorta (Ao). An enlarged left ventricle (LV) occupies the apex, and an enlarged right atrium occupies the lower right cardiac border.
B, Notch view of ductal flow going from aorta (Ao) to pulmonary trunk (PT) (Video 20-1). (LPA = left pulmonary artery.)
V .64 pulmonary vascular resistance (Figure 20-42). When an

aortopulmonary window is accompanied by suprasystemic
pulmonary vascular resistance and a reversed shunt, unoxy-
RVOT
5
genated blood enters the ascending aorta so differential
cyanosis does not occur. The clinical picture is then indistin-
–.64
guishable from nonrestrictive ventricular septal defect with
Ao
PT Eisenmenger’s syndrome (see Chapter 17).
10 A moderately restrictive aortopulmonary window
generates a continuous murmur indistinguishable from a
rPA
moderately restrictive patent ductus arteriosus.128,132 The
IPA
physiologic mechanisms responsible for variations in the
murmur of a nonrestrictive aortopulmonary window are
analogous to those that apply to a nonrestrictive patent
ductus arteriosus (see Figure 20-15). In 80% of patients,
73
2:2 HR the murmur is systolic rather than continuous
(seeFigure 20-42)128 and is punctuated by eddy sounds
FIGURE 20-39 Color Doppler flow in a 64-year-old man with a (see Figure 20-42). When the murmur is continuous, its
restrictive patent ductus. Ductal flow tracts along the medial
diastolic portion is likely to be shortened (Figure 20-43).
wall of the pulmonary trunk (PT) and downward toward the
pulmonary valve. (rPA ¼ right pulmonary artery; lPA ¼ left
A nonrestrictive aortopulmonary window with suprasyste-
pulmonary artery; RVOT ¼ right ventricular outflow tract; mic pulmonary vascular resistance and reversed shunt
Ao ¼ aorta.) is accompanied by auscultatory signs of pulmonary hyper-
tension. No murmur is generated across the defect.
A Graham Steell murmur may appear before the shunt is
to patent ductus is the rarity of infective endocarditis.128 reversed because of dilation of the hypertensive pulmonary
An appreciable percentage of infants with a nonrestrictive trunk.
aortopulmonary window die of congestive heart failure in Systolic or continuous murmurs generated by an aor-
infancy or early childhood.128 Only a minority reach teen- topulmonary window are typically maximal in the third
age or young adulthood, but occasional survivals have left intercostal space.131 A prominent systolic murmur
been reported in the fourth or fifth decade.128,131 Sur- at the mid to lower left sternal border invites the mis-
vival improves when the shunt is limited by a restrictive taken diagnosis of ventricular septal defect, but eddy
defect or curtailed by a rise in pulmonary vascular resis- sounds and a bounding arterial pulse should prevent error
tance. The patient referred to in Figure 20-41 lived to age (see Figure 20-42). Apical mid-diastolic murmurs
58 years with a nonrestrictive aortopulmonary window represent increased flow across the mitral valve (see
and Eisenmenger’s syndrome. Figure 20-43).
A bounding arterial pulse and a wide pulse pressure The electrocardiogram of a nonrestrictive aortopul-
are analogous to nonrestrictive patent ductus with low monary window with low pulmonary vascular resistance
V .64 LSA
RVOT
5
–.64 PDA
Ao Ao
10 PT
rPA IPA
A 73 B
HR
FIGURE 20-40 A, Color Doppler image from a 35-year-old man with patent ductus arteriosus. In the short-axis view, ductal flow is seen
entering the pulmonary trunk (PT) toward the right ventricular outflow tract (RVOT). (Ao ¼ aorta; rPA ¼ right pulmonary artery; lPA ¼ left
pulmonary artery.) B, Lateral aortogram showing a conical ductus arteriosus (PDA) that was patent at its aortic end (Ao, paired white
arrows) but was a virtual thread at its pulmonary arterial end (Video 20-2). (LSA ¼ left subclavian artery.)
Ca
PT PT
RA
RV
A B
FIGURE 20-41 A, X-ray from a 51-year-old cyanotic woman with an aortopulmonary window, suprasystemic pulmonary vascular re-
sistance, and a reversed shunt. Pulmonary vascularity is normal. The pulmonary trunk (PT) and its proximal branches are moderately
enlarged, but the cardiac size and configuration are virtually normal. B, X-ray 5 years later after the advent of atrial fibrillation. The right
ventricle (RV) and right atrium (RA) are markedly enlarged. The rim of calcium (Ca) above the dilated pulmonary trunk (PT) proved to be
in a restrictive patent ductus. The unmarked arrowhead adjacent to the pulmonary trunk identifies the cross section of a dilated intra-
pulmonary artery. The patient died in her 58th year.
2 LICS
E
SM
S1 S2
S1
2
A2 L
FA
P2 I
C
PA S
OS S
3
SM DM
S1 S2
20
A
p
e
0 x
FIGURE 20-42 Tracings from a 2-year-old boy with an aorto-

pulmonary window and a 3.4 to 1 left-to-right shunt. The pho-
nocardiogram in the second left intercostal space (2 LICS) shows FIGURE 20-43 Phonocardiograms from a 14-year-old boy with
a decrescendo holosystolic murmur (SM) punctuated by eddy an aortopulmonary window and a 3 to 1 left-to-right shunt. The
sounds (paired arrows). The single second heart sound is loud systolic murmur (SM) in the second left intercostal space (2
because the pulmonary component (P2) was increased. The LICS) continues just beyond the second heart sound (S2; see
femoral arterial (FA) and pulmonary arterial (PA) pressure pulses paired arrows). (E ¼ pulmonary ejection sound.) At the apex,
diverge in systole but are identical in diastole, so the murmur is a soft third heart sound (S3) and a mid-diastolic murmur (DM)
confined to systole. (S1 ¼ first heart sound; A2 ¼ aortic compo- were the result of increased flow across the mitral valve. There
nent of the second sound.) was a faint mitral opening sound (OS).
PT
RA
LA
LV
A B
FIGURE 20-44 X-rays from a 5-month-old female with an aortopulmonary window and a 3.5 to 1 left-to-right shunt. A, Pulmonary
vascularity is increased, pulmonary venous congestion is evident at the right hilus (arrow), the pulmonary trunk (PT) is prominent,
and a dilated left ventricle (LV) occupies the apex. B, Lateral view shows an enlarged left atrium (LA).
PT
LV
A B
FIGURE 20-45 X-rays from a 6-month-old male with an aortopulmonary window and a 3.5 to 1 left-to-right shunt. A, Pulmonary arterial
and pulmonary venous vascularity are markedly increased. A dilated left ventricle (LV) occupies the apex. The course of the femoral
venous catheter is into the right atrium (lower left arrow), across the right ventricular outflow tract into the pulmonary trunk (PT), through
the aortopulmonary window into the ascending aorta (smaller oblique arrow), and into the right subclavian artery. B, The lateral pro-
jection shows the catheter passing from the pulmonary trunk (curved arrow) across the aortopulmonary window into the aorta and into
the right subclavian artery (upper arrow).
reflects combined ventricular hypertrophy in response to shunt (Figures 20-44 and 20-45). When the shunt is
volume overload of the left ventricle and pressure over- reversed (see Figure 20-41), the x-ray resembles a non-
load of the right ventricle, analogous to a nonrestrictive restrictive patent ductus with Eisenmenger’s syndrome
patent ductus with low pulmonary vascular resistance (seeFigure 20-34).
(see Figure 20-25). When pulmonary vascular resistance Echocardiography localizes the aortopulmonary
is suprasystemic and the shunt is reversed, the electrocar- window between the ascending aorta and pulmonary
diogram is analogous to nonrestrictive patent ductus with trunk just proximal to the bifurcation (Figure 20-46
Eisenmenger’s syndrome (see Figure 20-27). and Video 20-3)129,133,134 and determines whether a pat-
The x-ray cannot distinguish a nonrestrictive aortopul- ent ductus coexists. Color flow imaging identifies the
monary window with low pulmonary vascular resistance shunt as left-to-right, right-to-left (Figure 20-47 and
from a nonrestrictive patent ductus with large left-to-right Video 20-3), or bidirectional.
Ao
rp PT
FIGURE 20-46 Echocardiogram (parasternal short-axis) from a 5-month-old female with an aortopulmonary window (arrow) between
the ascending aorta (Ao) and the pulmonary trunk (PT) (Video 20-3). (rp ¼ right pulmonary artery.)
APW
Ao
AV
LA
A B
FIGURE 20-47 A, Echocardiogram (parasternal long-axis) from the 51-year-old patient whose x-rays are shown in Figure 20-41. Paired
arrows identify the aortopulmonary window (APW). (Ao ¼ aorta; AV ¼ aortic valve; LA ¼ left atrium.) B, Black-and-white print of a
parasternal long-axis color flow image. Large white arrowheads bracket a right-to-left shunt (black arrow) through the aortopulmonary
window (Video 20-3).
SUMMARY error. A moderately restrictive aortopulmonary window

is accompanied by a continuous murmur and clinical signs
Aortopulmonary window is an uncommon malformation indistinguishable from a moderately restrictive patent
that should be considered in acyanotic patients with clin- ductus except for the relatively low precordial location
ical evidence of a nonrestrictive patent ductus and a large of the murmur. An aortopulmonary window with supra-
left-to-right shunt. The murmur is usually systolic rather systemic pulmonary vascular resistance and reversed
than continuous and is maximal in the third left intercos- shunt is clinically indistinguishable from a nonrestrictive
tal space. The relatively low location of the murmur ventricular septal defect with Eisenmenger’s syndrome.
arouses suspicion of ventricular septal defect, but eddy Echocardiography with color flow imaging and Doppler
sounds and a bounding arterial pulse should prevent this interrogation make the distinction.
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1600 John F. Kennedy Blvd.

CLINICAL RECOGNITION OF CONGENITAL HEART DISEASE ISBN: 978-1-4377-1618-4

Library of Congress Cataloging-in-Publication Data

Executive Content Strategist: Dolores Meloni

Last digit is the print number: 9 8 7 6 5 4 3 2 1

Lig. art. Left subclavian

FIGURE 20-1 Illustrations of patent ductus arteriosus (PDA) and

FIGURE 20-3 Lateral aortogram from a 14-year-old girl with a AO

distress may not improve with subsequent ductal clo-

FIGURE 20-7 Angiocardiogram with contrast material injected

FIGURE 20-10 Phonocardiograms at the second left intercostal

FIGURE 20-12 A, Photographs of a female infant with

Wide systemic pulse pressure is an important physical

A moderately restrictive patent ductus is accompanied

FIGURE 20-15 Schematic sequential modifications of the mur-

FIGURE 20-18 Tracings from a 30-

First left ICS P2

IN PA FIGURE 20-20 Phonocardiograms from an 11-year-old girl

When the left-to-right shunt is large, apical mid-

SM EDM 1 2 3 aVR aVL aVF

FIGURE 20-24 Electrocardiogram from a 37-year-old woman

1 2 3 aVR aVL aVF 1 2 3 4 5 6

FIGURE 20-26 Electrocardiogram from a 16-year-old boy with

neonates with respiratory distress. Rare examples of left

X-RAY Figures 20-5 and 20-30). After birth, left-to-right

FIGURE 20-32 A, X-rays from a 37-year-old

FIGURE 20-34 X-rays from a 14-

FIGURE 20-36 Continuous-wave Doppler scan from a 7-month-

clinical signs are those of pure pulmonary hypertension.

pulmonary channels.125–127 The distal truncoaortic sac

V .64 pulmonary vascular resistance (Figure 20-42). When an

FIGURE 20-42 Tracings from a 2-year-old boy with an aorto-

SUMMARY error. A moderately restrictive aortopulmonary window

REFERENCES 26. Michelakis E, Rebeyka I, Bateson J, Olley P, Puttagunta L,

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