Congenital Heart Disease for the Adult Cardiologist

Interventional Catheterization in Adult Congenital

Heart Disease
Ignacio Inglessis, MD; Michael J. Landzberg, MD

I mproved medical and surgical therapies for children with

congenital heart disease have resulted in a growing popu-
lation of patients reaching adulthood.1,2 Over this same time,
transposition of the great arteries, there may be additional
obstruction at the infundibular or supravalvular level. Of
note, mild PS does not appear to be associated with worsened
the field of interventional cardiology has experienced signif- gradients with age, and therefore most adults presenting with
icant growth, driven by technological improvements and moderate or severe obstruction are likely undiagnosed during
better understanding of the mechanisms and intermediate- youth.
term results of individual procedures. Consequently, for Percutaneous balloon pulmonary valvuloplasty (PPV) has
adults with uncorrected or previously palliated congenital become the treatment of choice for adult patients with
heart disease, percutaneous therapies have increasing accep- isolated PS. The Second Natural History Study of Congenital
tance as reasonable additions, alternatives, and treatments of Heart Defects revealed that pulmonary valve gradients
choice when further surgical or medical intervention is ⬎50 mm Hg were associated with poor outcomes from right
contemplated (Table 1). Currently, interventional cardiology ventricular infarction, ventricular arrhythmias, and sudden
of adult congenital heart disease (ACHD) is a well- death.5 Conversely, gradients ⬍30 mm Hg in the absence of
established field on its own and, programmatically, is a subpulmonary ventricular systolic dysfunction or shunt-
fundamental component of any center providing care for mediated cyanosis were associated with good prognosis and
these patients.3 Although limited safety of interventional were unlikely to progress over the 2 to 3 decades to follow.
procedures for the ACHD population has been suggested in However, as the ACHD population ages, concern may be
centers without ACHD global care programs,4 the wide raised regarding the even longer-term effects of mildly
variation in clinical presentation, novelty of cardiovascular increased subpulmonary ventricular afterload. Consequently,
pathologies (with similarity to as well as marked differences given the low procedural risk and with the assumption that
from both congenital and acquired conditions), and potential low postprocedural gradient carries low subsequent long-term
for concomitant multiple organ system pathology all contrib- risk, there is general agreement that PPV is indicated for
ute to situations atypical for standard adult or pediatric catheter-based pulmonary valve gradients ⬎40 mm Hg, irre-
laboratories. These concerns, combined with a desire to spective of symptoms, or when gradients are ⱕ30 mm Hg but
centralize data collection to establish outcomes assessments in the presence of symptoms of subpulmonary ventricular
for ACHD patients considered for interventional catheteriza- dysfunction or shunt-mediated cyanosis.6
tion, lead to the recommendation that such procedures be PPV is highly technically successful and safe in the adult
performed in centers with ACHD expertise and established population with results comparable to those of surgical
care programs.3 valvotomy. A significant reduction in gradient is obtained in
In this article, we review the most commonly performed up to 80% of procedures (influenced by annulus size, valve
percutaneous procedures in ACHD, including valvuloplasty, morphology, or operator technique), and most patients are
angioplasty, and device closures. We also include discussions free of events in the long term after a single procedure.
concerning complex ACHD patients (Table 2) as well as Further reduction in residual gradients may be seen over time
future directions. because of resolution of residual hypertrophic subvalvular
stenosis. Long-term follow-up of adults who underwent PPV
Valvuloplasty in their youth suggests that, similar to results with surgical
Pulmonary Valve Stenosis valvotomy, late moderate or greater pulmonary regurgitation
Pulmonary valve stenosis (PS) is almost always congenital in is increasingly recognized,7 and preprocedural counseling and
origin and usually results from commissural fusion of thin surveillance for such are recommended. Although PPV has
and pliable leaflets. Less frequently, the pulmonary valve is been attempted in subvalvular PS and in adults with dysplas-
dysplastic with thickened and less mobile leaflets. In patients tic pulmonary valves, results are limited, and surgical valvot-
with complex malformations, such as tetralogy of Fallot or omy is usually indicated for these patients. The impact of

From the Boston Adult Congenital Heart Group (I.I., M.J.L.), Massachusetts General Hospital (I.I.), Boston Children’s Hospital (I.I., M.J.L.), Brigham
and Women’s Hospital (M.J.L.), and Beth Israel Deaconess Hospital (M.J.L.), Harvard Medical School, Boston, Mass.
Correspondence and reprint requests to Michael J. Landzberg, MD, Boston Children’s Hospital, 300 Longwood Ave, Boston, MA 02115. E-mail
[email protected]
(Circulation. 2007;115:1622-1633.)
© 2007 American Heart Association, Inc.
Circulation is available at http://www.circulationaha.org DOI: 10.1161/CIRCULATIONAHA.105.592428

1622
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 Inglessis and Landzberg Interventional Catheterization in the Adult 1623

TABLE 1. Commonly Practiced Catheter-Based Interventions in ACHD: Indications and Level of Evidence
Level of Evidence
Intervention Indication (ACC/AHA Format)
PPV Asymptomatic: I:B
Echo mean gradient/cath PSEG ⬎40 mm Hg
Symptomatic:
Echo mean gradient/cath PSEG ⬎30 mm Hg, or less if RV dysfunction, or shunt-mediated cyanosis I:B
Balloon aortic valvuloplasty For catheterization
Asymptomatic:
Echo mean gradient ⬎40 mm Hg, or Doppler velocity ⬎4 m/s, or exercise-induced T-wave inversion IIa:B
Symptomatic:
Echo mean gradient ⬎30 mm Hg, or Doppler velocity ⬎3.5 m/s I:B
For intervention
Asymptomatic:
PSEG ⬎60 mm Hg, or AVA ⱕ0.6 cm2, with ⱕmoderate calcification and ⱕmoderate aortic IIa:B
regurgitation
PSEG ⬎50 mm Hg, or AVA ⱕ1.0 cm2, with ⱕmoderate calcification and ⱕmoderate aortic IIa:C
regurgitation, when patient activities mandate potential marked augmentation of cardiac output
(pregnancy, physical competitive sports)
Symptomatic:
PSEG ⬎50 mm Hg, or AVA ⱕ1.0 cm2, with ⱕmoderate calcification and ⱕmoderate aortic IIa:B
regurgitation
Aortic coarctation dilation Discrete, postoperative, recurrent
ⱖ20 mm Hg resting PSEG (30 mm with exercise) with radial-femoral pulse delay I:B
ⱖ10-20 mm Hg resting PSEG, LV dysfunction, radial-femoral pulse delay IIa:C
Native
ⱖ20 mm Hg resting PSEG (30 mm with exercise) with radial-femoral pulse delay IIb:B
ⱖ10-20 mm Hg resting PSEG, LV dysfunction, radial-femoral pulse delay IIb:C
Balloon pulmonary angioplasty RV pressure ⬎50% systemic levels, or RV pressure 25-50% systemic levels with either shunt-mediated IIa:B
(for peripheral PAS) cyanosis, RV dysfunction, ⬍20-25% of pulmonary flow to 1 lung, pulmonary hypertension in unaffected
segments, or severe pulmonary regurgitation
Conduit/baffle stenosis dilation RV pressure ⬎50% systemic levels, or RV pressure 25-50% systemic levels with either shunt-mediated IIa:B
cyanosis, RV dysfunction, or severe pulmonary regurgitation
ASD closure RV volume overload, without additional etiology⫾pulmonary/systemic flow ⱖ1.5 I:B
PFO closure Recurrent cryptogenic stroke despite therapeutic INR IIa:B
Prevention of recurrent cryptogenic stroke or motor TIA within constraints of RCTs IIa:B
Orthodeoxia-platypnea syndrome I:B
Refractory migraines within constraints of RCTs IIb:C
VSD closure LV volume overload, without additional etiology, with pulmonary/systemic flow ⱖ1.5 IIb:B
Pulmonary/systemic flow ⬍1.5 with progressive aortic regurgitation or heart failure IIb:B
Multiple recurrent endocarditis IIb:B
Postoperative residual VSD with above criteria IIb:B
PDA closure LV volume overload without additional etiology or with severe pulmonary hypertension I:B
Asymptomatic, audible IIb:B
ACC indicates American College of Cardiology; AHA, American Heart Association; AVA, aortic valve area; INR, international normalized ratio; LV, left ventricle; PA,
pulmonary artery; PSEG, peak systolic ejection gradient; RCT, randomized controlled trial; RV, right ventricle; and TIA, transient ischemic attack.

associated aneurysmal dilation of the main pulmonary (and becomes thickened and calcified by the fourth decade of life,
potentially aortic) trunk in patients (and their relatives) with becoming less suitable to balloon dilation.
PS remains unclear. Congenital cardiovascular practice guidelines for patients
with AS have been established on the basis of valvular
Aortic Valve Stenosis peak-to-peak gradients rather than echocardiographically es-
In contrast to PS, congenital valvar aortic stenosis (AS) timated valve areas, in large part because of concerns regard-
secondary to bicuspid aortic valve disease is a progressive ing difficulties in accurately measuring systemic cardiac
disorder in the adult. The bicuspid aortic valve typically output and the uncommon presence of low output in pediatric
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1624 Circulation March 27, 2007

TABLE 2. Potential Interventions in Complex ACHD Patients

Defect Symptomatology Intervention
Tetralogy of Fallot RV dysfunction ASD closure
RV outflow tract dilation/stent
PAS dilation/stent
Residual VSD closure
Percutaneous pulmonary valve implantation
Right coronary artery intervention
LV dysfunction Residual aorta–pulmonary artery shunt closure
Residual VSD closure
Left coronary artery intervention
Percutaneous aortic valve implantation
Cyanosis PFO closure (RV dysfunction)
D-TGA (atrial switch)
Systemic venous congestion or subpulmonary ventricular failure Systemic venous baffle obstruction dilation/stent
Baffle leak closure
Pulmonary venous congestion or subaortic ventricular failure Pulmonary venous baffle obstruction dilation/stent
Cyanosis Baffle leak closure
D-TGA (arterial switch operation)
Chest pain/ventricular failure Percutaneous coronary intervention
RV dysfunction Pulmonary artery obstruction dilation/stent
Semilunar valve regurgitation Percutaneous valve implantation
Fontan Volume retention/fatigue Baffle obstruction or thrombosis dilation/stent
Pulmonary artery obstruction dilation/stent
Pulmonary venous obstruction dilation/stent
Systemic venous–pulmonary venous connection closure
Systemic arterial–pulmonary venous connection closure
Systemic arterial–systemic venous connection closure
Cyanosis Pulmonary arteriovenous fistulae closure
Intramural/baffle leak closure
Systemic venous–pulmonary venous connection closure
D-TGA indicates (S,D,D) transposition of the great arteries; LV, left ventricle; and RV, right ventricle.

patients. Data are few regarding timing and indications for (averaging 50% to 60% event-free survival at 5 to 10 years of
intervention for adults with congenital AS, although current follow-up in young adults undergoing PAV).8 Risk and
practice guidelines suggest that for symptomatic adults, either success of surgical repair for the uncommon patient sustain-
echocardiographic transvalvar Doppler velocity ⬎3.5 m/s, ing avulsion of a valvular cusp during PAV do not appear
mean gradient ⬎30 mm, or ECG T-wave inversion (and in compromised by attempted PAV, with periprocedural echo-
asymptomatic adults, mean gradient ⬎40 mm Hg or transval- cardiographic surveillance and timely surgical therapy.9 Al-
var Doppler velocity ⬎4 m/s) is an indication for catheter- though recommended in national guidelines for consideration
ization and consideration of percutaneous aortic valvulo- as a first-line treatment in young and older adults with AS,
plasty (PAV).6 At catheterization, a peak-to-peak gradient of PAV is best considered a palliative procedure, potentially
⬎60 mm Hg without severe calcification and with less than delaying surgical valve replacement. Relative risks of surgi-
moderate aortic insufficiency in adults without symptoms, as cal repair or replacement strategies should be counseled when
well as a peak-to-peak gradient ⬎50 mm Hg in the presence PAV is considered. The impact of associated aneurysmal
of symptoms, is considered an indication for PAV in patients dilation of the ascending aorta (and potentially the pulmonary
with congenital AS. trunk) in patients with AS should be taken into consideration,
Although effective in reducing aortic valve gradients, PAV as well. PAV has been attempted in patients with nondiscrete
is associated with a 10% to 30% risk of significant residual subaortic stenosis with limited success, and surgical interven-
aortic insufficiency (especially when a balloon/annulus ratio tion is generally recommended. Catheter-based percutaneous
⬎1 is used). Balance should be considered at the time of balloon valvuloplasty has been offered as a shorter-termed
balloon size selection between tolerating some residual ste- alternative to surgical repair of more discrete subaortic
nosis and avoiding significant aortic insufficiency. PAV is stenosis when catheter-based gradient or mean echo gradient
associated with higher restenosis rates compared with PPV is ⬎50 mm Hg and either symptoms develop or the ability to
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 Inglessis and Landzberg Interventional Catheterization in the Adult 1625

Figure 1. Evaluation of systemic hyper-

tension led to recognition of radial-
femoral pulse delay and subsequent
angiography (A) confirming discrete aor-
tic coarctation, with 40 mm Hg peak-to-
peak gradient. After stent implantation
(B), there was no measurable residual
gradient, and pulse delay was
eliminated.

augment cardiac output is needed during pregnancy or for a rare complication and highlights the need for onsite
competitive sports competition.10 catheter-based and surgical support (see below). The inci-
dence of late aneurysm formation is higher than in postsur-
Angioplasty gical patients, with wide variance reported (from 8% to 35%)
Aortic Coarctation in different series. The suspected mechanism for late aneu-
Aortic coarctation in the adult is typically located just distal rysm formation is intimal tear at the site of cystic media
to the origin of the left subclavian artery, although various necrosis, although current literature does not support a
degrees of obstruction can also occur throughout the aortic relationship between balloon size, coarctation diameter, and
arch. The presence of cystic medial necrosis in the aortic wall the incidence of late aneurysm formation. The overall long-
adjacent to the coarctation site is believed to predispose to term clinical impact of such aneurysms is unclear because
wall rupture and aneurysm formation after surgical and most aneurysms tend to be small and have not required
percutaneous interventions. further treatment.
Indications for relief of obstruction even in the asymptom- Stent implantation may theoretically overcome some of the
atic patient have included clinically determined radial- shortcomings of balloon dilatation for aortic coarctation
femoral arterial pulse delay or catheter-derived peak-to-peak because metal scaffolding may reduce the incidence of acute
gradient of ⱖ20 mm Hg at rest or ⱖ30 mm Hg after exercise. elastic recoil as well as late restenosis due to more complete
However, relief of aortic coarctation may still be indicated in elimination of gradient in the high-velocity flow arterial
the presence of lower gradients if there is a large collateral system (Figure 1). Additionally, stents may reduce the inci-
network or subaortic ventricular systolic or diastolic dysfunc- dence of residual intimal tears and subsequent aneurysm
tion or if concomitant anatomic (aortic valve, aortic aneu- formation by allowing both the use of smaller dilation
rysm, coronary or carotid atherosclerosis) procedures are balloons (especially in the presence of mild anatomic steno-
being considered. sis) as well as graded inflations in staged procedures. Intra-
There is general agreement that percutaneous balloon vascular stent implantation may carry greater potential in
angioplasty with or without stent implantation is a preferred adult patients than in children because patient growth is less
treatment modality for recurrent postsurgical aortic coarcta- of a problem, and the aging aorta is potentially more fragile.
tion.11 The procedure is successful in reducing the gradient to Preliminary data on the use of stents for aortic coarctation
⬍20 mm Hg in ⬇80% of interventions, with a 1.5% inci- suggest a lower residual stenosis, lower restenosis rate, and
dence of late aneurysm formation (with low incidence of lower rate of late aneurysm formation (⬍5%) compared with
aneurysm formation potentially the result of either postsurgi- balloon angioplasty alone. Aortic rupture has been seen in
cal periaortic fibrosis or removal of most of the abnormal stent implantation procedures, especially when lack of com-
aortic wall tissue at the time of surgery). pliance of the aorta has not been recognized. The risk of
Percutaneous balloon angioplasty of the unoperated aortic subacute stent thrombosis is believed to be low, and although
coarctation in the adult is more controversial, especially in the periprocedural antiplatelet or anticoagulant regimens are
absence of standardization of indication for repair, technique, standardly prescribed, their use remains unsubstantiated.
end points, and postprocedural follow-up. A higher incidence Stent implantation has rapidly become a recommended pro-
of late aneurysm formation and restenosis compared with cedure for the treatment of unoperated aortic coarctation in
surgically treated patients has been suggested but not univer- many institutions, despite the lack of available multicenter
sally confirmed.12–16 The procedure is successful in reducing long-term registry data regarding risks or benefits of this
the gradient to ⬍20 mm Hg in 85% of patients, with a procedure. Covered stents should be readily available in the
restenosis rate of ⬇8%. Acute dissection has been observed cardiac catheterization laboratory for the emergency therapy
and may be related to failure to recognize aortic nondisten- of acute aortic dissection or rupture complicating endovascu-
sibility during balloon inflation. Death from aortic rupture is lar procedures. This technology has also been considered for
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1626 Circulation March 27, 2007

treatment of late aortic aneurysms after endovascular or

surgical repair of aortic coarctation and for subatretic native
aortic coarctation.17,18 Caution should be exercised, however,
in regard to differing risks attributed to covered stents,
including stent thrombosis, in-stent restenosis, and inability
to redilate such stents at future settings.

Pulmonary Artery Stenosis

Pulmonary artery stenosis (PAS) in patients with congenital
heart disease may occur anywhere in the pulmonary vascular
tree from the main pulmonary artery to the distal-most
branches. PAS is typically associated with other cardiac
defects or syndromes, such as presence of ventricular septal
defect (VSD), tetralogy of Fallot, Williams syndrome, or
Alagille syndrome or after in utero rubella infection; how-
ever, it can also occur in isolation. Additionally, PAS may
result as sequelae of surgical interventions (including
systemic-pulmonary shunts, homograft or conduit implanta-
tion, pulmonary artery banding, or pulmonary arterioplasty or
after arterial switch operation) or may be a residue of
acquired diseases (including chronic thromboembolic dis-
ease, tumor infiltration or compression, inflammatory arteri-
tis, perivascular fibrosis, or postsurgical scarring after lung
resection and reanastomosis).
Although unilateral PAS typically presents asymptomat-
ically in the otherwise normal adult, in persons with abnormal
pulmonary resistance, isolated branch obstruction can result
in pulmonary hypertension, increased subpulmonary ventric-
ular afterload, pulmonary valve insufficiency, or sufficient
perfusion-ventilation imbalance that may lead to symptoms.19
Quantitative radiographic assessment of lung perfusion and
ventilation should be part of the diagnostic algorithm and Figure 2. Dyspnea and volume retention in this patient with
should be used as a tool to evaluate the results of dilation tetralogy of Fallot/pulmonary atresia after right ventricular to pul-
procedures in such patients. Surgical access and correction of monary artery conduit repair led to nuclear lung scintigraphy,
PAS may be cumbersome and limited. Consequently, after revealing 80% flow to the left lung, and echocardiography, sug-
gesting right ventricular hypertension ⬎50% systemic levels and
joint medical-surgical discussion of goals and risks, and in moderate pulmonary regurgitation, with normal right ventricular
appropriate centers, percutaneous therapy may be a preferred volume. Angiography (A) confirmed tight proximal stenosis of
treatment method to restore pulmonary blood flow and the proximal branch right pulmonary artery at the anastomotic
site with the right ventricular to pulmonary artery conduit, which
balance and to decrease resistance for patients with PAS. was relieved (B) after high-pressure balloon angioplasty. Post-
Indication for balloon pulmonary angioplasty has included procedural right ventricular pressure was 30% systemic, and
presence of right ventricular pressure ⬎50% of systemic nuclear lung scintigraphy returned to 55% flow to the right lung.
levels or lesser pressure in the setting of symptoms, shunt-
mediated cyanosis, subpulmonary ventricular dysfunction,
struction with the use of a smaller balloon-lesion ratio, with
pulmonary flow imbalance with ⬍20% to 25% total flow to
promising results.23
a single lung, pulmonary hypertension in unaffected lung
Similar to the treatment of aortic coarctation, intravascular
arterial segments, or severe pulmonary regurgitation.
stent deployment may be more practical in the adult patient
Balloon angioplasty for PAS is successful (defined as
with PAS than in the pediatric population because patient
increase of ⬎50% of predilation vessel diameter or 20%
decrease in systolic subpulmonary ventricle to aortic pressure growth is less of an issue. Stent deployment appears to have
ratio) in ⬇75% of cases when high-pressure balloons are improved the outcomes of balloon pulmonary angioplasty
used20 (Figure 2). Complications have included arterial rup- because its use is associated with a larger postprocedure
ture, unilateral or segmental pulmonary edema, thrombosis, lumen and less occurrence of restenosis, which may be
and hemoptysis. The restenosis rate approaches 15%, and treated effectively with redilation. Certain lesions remain
there is a 3% to 4% incidence of aneurysm formation.21,22 problematic for stent therapy, such as proximal main pulmo-
Certain PAS lesions treated with balloon angioplasty alone nary stenosis close to the pulmonary valve and bifurcation
have worse outcome, such as highly elastic proximal main stenoses. Stents have been proven to be cost-effective com-
branch narrowing, postsurgical stenoses, long segments, and pared with either percutaneous angioplasty methods or sur-
external compressions. Balloon angioplasty has been per- gical intervention. Innovations in stent technology are likely
formed in patients with distal chronic thromboembolic ob- to expand the future indications of stent therapy for PAS.
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 Inglessis and Landzberg Interventional Catheterization in the Adult 1627

Figure 3. Development of peripheral

edema led to cardiac magnetic reso-
nance imaging in this patient with tricus-
pid atresia after intracardiac cavopulmo-
nary anastomosis/Fontan palliation, with
relatively diffuse left pulmonary artery
stenosis. Catheterization confirmed ste-
nosis (A) with minimal gradient, which
was relieved by stent implantation (B).
Reduction in systemic venous pressures
and marked improvement in cardiac out-
put and diuresis ensued.

To date, optimal postimplantation antiplatelet or anticoag- Of all the interventions for adults with congenital heart
ulant strategies remain undefined. disease, the greatest consensus appears regarding the indica-
tion for and method of secundum ASD closure. The standard
Conduit and Baffle Stenosis for intervention has been promulgated as the imaging-based
Extracardiac conduits may be used to connect the subpulmo- presence of right ventricular volume overload (usually asso-
nary ventricle to the pulmonary arteries in patients with ciated with sustained pulmonary to systemic flow ratio
complex congenital disease. These conduits can develop [Qp/Qs] ⬎1.5, the threshold associated with the potential for
obstruction secondary to severe angulation, calcification, right ventricular dysfunction, progressive pulmonary vascular
sternal compression, or tissue proliferation, most notably at disease, and atrial arrhythmia development, even in asymp-
anastomotic sites. Balloon angioplasty procedures offer little tomatic patients). Right-to-left shunting may be present in
improvement, and restenosis is common. Stent implantation such patients as evidence of streaming or elevation of
has demonstrated better long-term outcome (predominantly pulmonary vascular resistance, with closure still being con-
measured as freedom from reoperation), albeit with recog- sidered provided that net Qp/Qs is ⬎1.5 (typically with
nized risk.24 Stent fracture has been observed in up to 16% of pulmonary vascular resistance ⬍8 to 10 indexed Wood units
cases. Stent implantation at anastomotic sites may limit the and pulmonary/systemic resistance ⬍0.2 to 0.4), although
ability of future surgical conduit replacement and may result increased long-term risk due to residual pulmonary vascular
in severe regurgitation in valved conduits. Finally, the poten- disease likely persists. Intracatheterization ASD test occlu-
tial for coronary compression (coursing of coronary artery sion with observation of resultant hemodynamics may aid in
sufficiently adjacent to the conduit) by stent implantation has observing the acute hemodynamic effects of closure, although
been reported, with catastrophic potential, and must be studies assessing the longer-term predictive nature of such
assessed before intervention. testing have not been performed. Complex strategies utilizing
Conduit stenosis can be observed after Glenn or Fontan concomitant pulmonary vascular therapies or customized
operations (Figure 3). Similarly, systemic baffle obstruction “fenestrated” closure devices have been reported in patients
is observed in up to 15% of patients undergoing atrial switch with large-volume left-to-right shunting and moderate to
(Mustard or Senning) procedures. Balloon angioplasty occa- severe pulmonary hypertension.
sionally results in long-term relief; however, stent deploy- Even though there are no randomized trials comparing
ment has been highly successful in relieving obstruction, with percutaneous versus surgical closure of ASDs, most centers
low complication rates and superior results, especially when favor percutaneous closure if the anatomy is suitable.25
performed in the superior portion of the systemic venous Percutaneous closure of ASDs has been reported in nonran-
baffle after atrial switch operations. Systemic anticoagulation domized uncontrolled fashion to be associated with improve-
has been recommended for at least 6 months after stenting of ment in right ventricular anatomy, function, and left ventric-
the low-velocity flow baffles, without supportive data. ular interaction, as well as in exercise capacity as measured
by cardiopulmonary exercise testing.26,27
ASDs can be classified as ostium secundum, ostium
Device Closure
primum, sinus venosus, and coronary sinus septal defects,
Interatrial Communications depending on location in the interatrial septum. Occasionally,
Interatrial communications can be classified as atrial septal multiple defects can be observed, especially associated with a
defect (ASD) or patent foramen ovale (PFO), each having thin, hypermobile septum primum. Multiple devices have
different clinical and anatomic implications. been used for percutaneous ASD closure since the technique
The left-to-right shunt in an ASD is driven by compliance was first attempted in the mid-1970s. The basic device design
differences, with low to nil transatrial pressure gradients. is of a dual-disc structure joined at a waist, with each disc
Morbidities related to aging may lead to decreased left deployed in either side of the septum. An atrial septum rim
ventricular compliance, contributing to increased left-to-right ⬎5 mm around the majority of the defect is required for the
shunt. safe use of any closure device, although more deficient
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1628 Circulation March 27, 2007

Figure 5. This 52-year-old woman with dyspnea, volume reten-

tion, and complete right bundle-branch block underwent echo-
cardiography, confirming a 2.4-cm secundum ASD with accom-
panying right ventricular volume overload. Implantation of an
Amplatzer ASD occluder (arrow) under transesophageal guid-
ance and subsequent diuresis led to resolution of
symptomatology.

is more complicated after right atrial disk deployment, and

the risk of device arm fracture (6%) and device-related
thrombosis appears higher than with the Amplatzer septal
occluder. Incidence of device-associated supraventricular
tachycardia after implantation of either of these devices is
Figure 4. Currently available devices within the United States under prospective study.
for percutaneous closure of atrial-level defects within random- The Amplatzer device allows for closure of ostium secun-
ized controlled trials.
dum ASDs with ⬍36 to 40 mm of nominal balloon stretched
diameter, whereas the CardioSEAL can only be used in
anterior superior aortic rim is typical and does not preclude
off-label fashion for moderate-sized defects (⬍18 mm). Both
percutaneous closure.
devices are successful in closing ASDs, with procedural
The only currently available devices for clinical use world-
wide are the Amplatzer septal occluder (AGA Medical), the success (almost complete or complete closure and absence of
CardioSEAL device (NMT Medical), and the Helex septal major complications) reported for the Amplatzer device at
occluder (W.L. Gore and Associates) (Figure 4). At present, ⬎95% at 3 months. Although earlier nonrandomized studies
2 devices (Amplatzer septal occluder and CardioSEAL de- reported greater safety and efficacy with the use of the
vice) are approved in the United States for closure of different Amplatzer device compared with the Clamshell (C.R. Brad
types of atrial defects, with the largest worldwide experience Inc) and CardioSEAL devices,28,29 the self-adjusting STAR-
with the use of the Amplatzer septal occluder (Figure 5). Flex modification has been reported to have procedural
Each of the aforementioned devices has its own advantages success equal to the Amplatzer septal occluder with fewer
and disadvantages. The Amplatzer septal occluder is a self- complications.30
centering and retrievable device up to the point of full release; Although fluoroscopic guidance alone is possible and used
however, it has a larger profile once deployed, and rare cases by some, most operators prefer echocardiographic guidance
of late cardiac perforations and erosions (as late as 3 years for percutaneous ASD closure because it offers a more
after implantation) causing serious or catastrophic events complete and accurate evaluation of the interatrial septum
have been reported and remain a source of concern and and surrounding structures. Both transesophageal and intra-
indication for further study. The CardioSEAL device typi- cardiac echocardiography have proven to be useful and
cally has a lower profile favoring rapid and more extensive effective in guiding procedures, with most operators prefer-
endothelialization. However, only its STARFlex modification ring transesophageal echocardiography for large or multiple
(NMT Medical) is a self-adjusting device. As well, retrieval defects.
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 Inglessis and Landzberg Interventional Catheterization in the Adult 1629

Although elevation of procoagulation markers has been

reported after percutaneous ASD closure,31 there remains no
consensus on the appropriate anticoagulation or antiplatelet
regimen after percutaneous ASD closure; most operators
recommend aspirin alone or a combination of aspirin and
clopidogrel for at least 6 months, in large part because of
regimens used in ongoing multicenter randomized trials
utilizing related devices. Antibiotic prophylaxis is generally
recommended for up to 12 months after device implantation.
PFO, present in ⬇25% of the adult population, has been
associated with various disease processes, including paradox-
ical emboli causing cryptogenic strokes or other systemic
arterial occlusion events, systemic hypoxemia from right-to-
left shunt, decompression sickness in divers, and migraine
headaches. To date, causal mechanisms for these associations
have not been well established; associations appear strongest
in younger populations, in which prevalence of additional
known risks for disease occurrence is less.
The treatment options for patients with PFO and crypto-
genic stroke currently include medical therapy (anticoagula-
tion or antiplatelet drugs) and surgical or percutaneous PFO
closure. Two PFO closure devices, the CardioSEAL occluder
(February 2000) and the Amplatzer PFO occluder (April
2002), were approved in the United States, via Humanitarian
Device Exemption (each limited to ⬍4000 implants yearly),
for implantation in patients with recurrent cryptogenic stroke
due to presumed paradoxical embolus despite use of conven-
tional drug therapy, defined as therapeutic international
normalized ratio with the use of oral anticoagulation.32,33 As
of the fall 2006, these Humanitarian Device Exemptions are
no longer applicable under Food and Drug Administration
regulations, having been replaced by specific corporate and
institutional research protocols. At the present time, for the Figure 6. Profound dyspnea on standing led to recognition of
greater population of patients with PFO and cryptogenic orthodeoxia-platypnea after 2 years of symptoms. Right-to-left
transatrial angiographic passage (A) is eliminated by Cardio-
stroke after the index event, despite promulgation of general SEAL PFO closure (B), accompanied by total relief of
care guidelines and desire for further study of this problem, symptomatology.
there is no consensus on best treatment strategies because no
randomized studies evaluating the available options have PFO closure has been performed successfully in patients
been completed.34 However, available nonrandomized, un- with positional oxygen-unresponsive systemic hypoxemia
controlled data analyzed within systematic review suggest (orthodeoxia-platypnea syndrome), resulting from right-sided
that approximately two thirds of recurrent thromboembolic compliance abnormalities leading to positional right-to-left
events may be prevented by percutaneous PFO closure shunt across the PFO37 (Figure 6). Care should be exercised
compared with medical therapy, corresponding to a 4% to exclude low pulmonary venous oxygen saturation caused
absolute reduction in annual events.35 As a result of increas-
by poor oxygen exchange, concomitant right ventricular
ingly widespread support, several large-scale multicenter
systolic dysfunction, and excessive pulmonary vascular resis-
randomized controlled trials (Evaluation of the STARFlex
tance in such patients before catheter-based PFO closure.
Septal Closure System in Patients With a Stroke or Transient
The association between PFO and migraine headaches has
Ischemic Attack due to Presumed Paradoxical Embolism
Through a PFO [CLOSURE 1], Randomized Evaluation of been suggested, especially for migraine with aura. Currently
Recurrent Stroke Comparing PFO Closure to Established there is no indication to close PFO for migraine occurrence.
Current Standard of Care Treatment [RESPECT], and a The complexity of headache assessment and recognition of
United States Randomized Clinical Trial of the Cardia Star large-scale placebo benefits achieved in past trials led to
Patient Foramen Ovale Closure System [CARDIA STAR Migraine Intervention With STARFlex Technology (MIST I),
trial]) evaluating the safety and efficacy of PFO closure the first double-blinded, randomized, sham procedure– con-
contrasted with best medical therapies in younger-aged pa- trolled trial comparing PFO closure with medical therapy in
tients for prevention of recurrent cryptogenic stroke are patients with refractory migraines performed in the United
actively recruiting patients, and trial results are expected to Kingdom. MIST I has been completed recently, trial data are
shed further light on the relative appropriateness of these pending detailed review, and results of additional larger trials
therapies.36 being performed worldwide are expected to further elucidate
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1630 Circulation March 27, 2007

Figure 7. This 80-year-old subject had progressive fatigue and dyspnea at walking 1 block. By echocardiography, global left ventricular
dysfunction (ejection fraction 30%) and right ventricular pressures estimated that 50% systemic levels were present, and a 4- to 5-mm
PDA was noted with left-to-right flow. Catheterization noted unobstructed epicardial coronary arteries. Lateral angiography (A) con-
firmed a long conical tunnel-type PDA with minimal diameter 5 mm (B), which was closed with an Amplatzer PDA occluder (C) on an
outpatient basis. Three months later, the patient had returned to walking 2 miles daily, with echocardiographic left ventricular ejection
fraction 45%.

the relative risks and benefits of such therapy in patients with valve, as well as the conduction system, with postprocedural
refractory migraines. complete heart block observed on occasion, even with asym-
metrical devices. Increased data collection and design modi-
Ventricular Septal Defects fications have potential to improve safety and efficacy.40
VSDs are classified as inflow, muscular, or perimembranous
depending on location in the septum. Patients with sizable Patent Ductus Arteriosus
VSD can develop cardiac left-sided volume overload, subse- A patent ductus arteriosus (PDA) is an abnormally persistent
quent ventricular failure, and pulmonary hypertension, typi- arterial connection after birth between the descending aorta
cally at younger ages than patients with atrial-level defects. and the pulmonary artery, most commonly to the junction of
Consequently, most patients with large enough defects requir- the main and left pulmonary artery branches. As is the case
ing therapy are usually diagnosed and treated in childhood. for VSD, patients with large PDA may develop left-sided
VSD closure in the adult is usually recommended either when volume overload and pulmonary hypertension at younger
left ventricular volume overload, unexplained by alternative ages, leading to diagnosis early in life. It is not uncommon
mechanism, is present, correlating with Qp/Qs ⬎1.5, in the that the diagnosis of PDA is made in adulthood by means of
presence of multiple recurrences of otherwise unexplained physical examination and presence of the continuous murmur
bacterial endocarditis, or when accompanied by progressive typical of PDA or as an incidental finding on echocardiogra-
aortic regurgitation. phy. Additional problems associated with PDA include infec-
Percutaneous closure of congenital or acquired (post– tious endarteritis, aneurysm formation, calcification, and rare
myocardial infarction septal rupture) muscular VSD is a rupture. Large PDA with significant left-to-right shunt should
particularly attractive therapeutic option because surgical be closed to reduce occurrence of the sequelae of subaortic
closure of these defects may carry increased risk of morbidity ventricular failure or pulmonary arterial hypertension. In
and mortality. Transcatheter VSD closure is generally tech- adults, the treatment of small anatomic PDAs with associated
nically more challenging than ASD closure, with intravascu- small-quantity shunting remains controversial. The standard
lar passage through the VSD with the use of a balloon-tipped to advise PDA closure in an asymptomatic adult patient, with
catheter (to ensure passage via the largest lumen) from the left an audible murmur but without other indication, has come
ventricular side, snaring a guidewire in the pulmonary artery, under increasing debate due to lack of supportive data for
formation and externalization of a venous-arterial-venous such interventions.
vascular loop, and subsequent device deployment based on a Percutaneous closure of PDA has been performed for ⬎20
pathway allowing maximal device arm–septal apposition, years with several generations of devices and is the preferred
without interference with adjacent intracardiac structures. mode of therapy worldwide given increasing surgical risk
Although this technique may be highly successful in achiev- with age because of PDA calcification and potential for
ing clinical improvement as defined by risk scales designed intraoperative recurrent laryngeal nerve damage. Currently,
for this population, recent reports have highlighted the in adults with PDA, the Amplatzer ductal occluder is the most
complexity and morbidities encountered in such high-risk commonly used device (Figure 7), typically implanted during
patients undergoing percutaneous VSD closure with earlier an outpatient procedure, with coil embolization reserved for
double-umbrella devices as well as the current CardioSEAL PDA measuring ⬍2 to 3 mm or for residual leaks. Complete
occluder (the 1 device approved in the United States for this closure has been reported in ⬎95% of patients at 6 months
indication).38 Early results with the Amplatzer muscular VSD with both techniques, with device embolization being rarely
occluder, although encouraging, have not included outcome encountered.41 Contrary to PDA closure in children, left
data related to longer-term clinical improvement.39 Percuta- pulmonary artery obstruction is not a concern in adults. The
neous closure of perimembranous VSD is even more chal- postimplantation antiplatelet regimen remains unsubstanti-
lenging because of the proximity of the aortic and tricuspid ated, although it is typically recommended for 3 to 4 months,
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 Inglessis and Landzberg Interventional Catheterization in the Adult 1631

and patients continue to receive antibiotic prophylaxis against Coronary Fistulae

subacute bacterial endocarditis for 12 months after device Coronary fistulae can originate from all major epicardial
implantation. coronary arteries, and drainage usually occurs to the coronary
sinus, right atrium, right ventricle, or pulmonary artery. These
Fontan Fenestrations and Atrial Switch collaterals can become markedly enlarged and can lead to
Baffle Leaks significant left-to-right shunting with right-sided volume
Patients after Fontan operation may have spontaneous or overload and effective coronary arterial steal leading to
surgically created residual fenestrations that may lead to ischemia. Embolization has been reported predominantly
right-to-left shunt and consequent systemic oxygen desatura- with coils; however, other devices such as ASD closure
tion, systemic emboli, and exercise incapacity. These fenes- devices or the Amplatzer vascular plug have been used
trations can be closed with the use of the CardioSEAL or successfully, as well.43 Long-term effects of intravascular
Amplatzer septal occluder devices. However, given the non- fistula occlusion have yet to be demonstrated.
pulsatile circulation and prothrombosis encountered in pa-
tients with Fontan palliation, we favor use of the lower- Other Forms of Collaterals
profile CardioSEAL (the only device currently approved in Collaterals from the systemic to the pulmonary veins
the United States for fenestration closure). Patients typically (venous-venous collaterals) can occur in patients with single-
ventricle physiology, especially after Glenn or Fontan oper-
receive long-term anticoagulation and prophylaxis against
ations, in part because of chronically elevated systemic
subacute bacterial endocarditis. Transient balloon test occlu-
venous pressures. Pulmonary arteriovenous fistulae may also
sion of the fenestration has been correlated with successful
present late after Glenn anastomosis, as well as in patients
longer-term outcomes after closure and, given such, should
with chronic liver failure or with hereditary hemorrhagic
be performed before permanent closure.42
telangiectasia (Osler-Weber-Rendu syndrome). In both in-
Up to 25% of patients undergoing Mustard or Senning
stances, there is right-to-left shunt with various degrees of
operations will demonstrate late baffle leaks, likely as the
cyanosis. Both types of abnormal connections can be treated
result of suture dehiscence. Although many of these shunts
successfully with coil embolization or Amplatzer closure
are hemodynamically unapparent and do not require therapy,
devices44 provided that right-to-left shunt is not of hemody-
closure is indicated for large defects resulting in significant
namic benefit as potential relief of subpulmonary ventricular
intracardiac shunting. Percutaneous closure has been reported
afterload due to the presence of severe pulmonary arterial
with the use of both CardioSEAL and Amplatzer devices as
hypertension. Nevertheless, recurrence of collaterals is not
alternatives to surgery.
uncommon unless the underlying physiology driving their
development is modified significantly.
Coil Embolization
Miscellaneous Procedures
Aortopulmonary Arterial Collaterals As the ACHD population ages, epicardial coronary disease is
Aortopulmonary collaterals may be observed in patients with becoming more prevalent. Thus, coronary angiography is
congenital malformations associated with decreased pulmo- indicated at the time of catheterization in all patients older
nary flow, including pulmonary atresia, as well as variations than 40 years or in younger patients with significant risk
of single-ventricle physiology or after Glenn shunt or Fontan factors for atherosclerosis (in particular, all men aged ⬎35
palliation. These collaterals can arise from any systemic years). Epicardial coronary stenting should be performed, if
artery and connect directly to the pulmonary arteries at the indicated, by operators with expertise in percutaneous coro-
lung hilum or at the periphery. Resulting left-to-left systemic nary revascularization techniques. Additionally, coronary an-
arterial to pulmonary arterial shunting may cause pulmonary giography should be performed before any percutaneous
vascular disease, elevation of systemic ventricular filling procedure with potential for coronary compromise, such as
pressure, systemic ventricular volume loading, and ultimate stenting of a right ventricular to pulmonary conduit.
failure. Residual surgically created systemic to pulmonary Balloon atrial septoplasty has been used to create or
arterial shunts share the same pathophysiological sequelae. enlarge existing ASDs in patients with severe pulmonary
In addition to the aforementioned factors, aortopulmonary hypertension and right ventricular failure as means for pro-
collateral embolization is indicated before the final stage of viding a “pop-off” valve to the failing right ventricle. How-
surgical subpulmonary ventricle to pulmonary artery connec- ever, this approach must be balanced against excessive
tion because left-to-right shunt directly into the pulmonary decreases in systemic oxygen saturations from increased
arteries complicates cardiopulmonary bypass, and dual sup- right-to-left shunting. Restenosis of the interatrial septum is
ply to vascular branches leads to overcirculation and devel- common, and fenestrated closure devices are becoming avail-
opment of pulmonary arterial hypertension. Embolization is able with the hope of improving long-term patency.
typically performed in those collaterals that share significant Baffle fenestrations are performed increasingly at the time
flow from the main or additional unifocalized pulmonary of Fontan surgery because they reduce the incidence of
arteries to avoid lung infarction. Embolization is typically postoperative effusions. These fenestrations can become oc-
performed with coils; however, the Amplatzer vascular plug cluded in the early postoperative period, leading to rapid
has recently become available for occlusion of larger collat- hemodynamic deterioration from increased pulmonary vas-
erals, with similar success reported. cular resistance and low cardiac output. Balloon angioplasty
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1632 Circulation March 27, 2007

of the fenestration usually restores patency; however, there is in centers expert in such care, with centralized data collection
risk of thrombus embolization to the left heart. Rarely, the and outcome assessments, will likewise assist in the further
fenestration cannot be proved with a catheter, and wire and advancement of adults with congenital heart disease and its
baffle puncture with a transseptal needle is required. sequelae.3
Cardiac arrhythmias are frequent in the complex ACHD
patient, resulting in unpredictable and variable degrees of Disclosures
hemodynamic compromise. Electrophysiology procedures, Dr Inglessis is on the Speakers’ Bureau for Boston Scientific and is
such as programmed stimulation and temporary pacing, are a consultant for Medtronic. Dr Landzberg has received research
grants from and served as a consultant for NMT Medical, AGA
performed frequently in conjunction with simultaneous he- Medical, Actelion, and Nitrox. He has also received a grant from
modynamic measurements, allowing for accurate diagnosis Myogen.
and development of treatment strategies.
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 Interventional Catheterization in Adult Congenital Heart Disease
Ignacio Inglessis and Michael J. Landzberg

Circulation. 2007;115:1622-1633
doi: 10.1161/CIRCULATIONAHA.105.592428
Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231
Copyright © 2007 American Heart Association, Inc. All rights reserved.
Print ISSN: 0009-7322. Online ISSN: 1524-4539

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