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,
the field of interventional cardiology has experienced signif-
icant growth, driven by technological improvements and
better understanding of the mechanisms and intermediate-
term results of individual procedures. Consequently, for
adults with uncorrected or previously palliated congenital
heart disease, percutaneous therapies have increasing accep-
tance as reasonable additions, alternatives, and treatments of
choice when further surgical or medical intervention is
contemplated (Table 1). Currently, interventional cardiology
of adult congenital heart disease (ACHD) is a well-
established field on its own and, programmatically, is a
fundamental component of any center providing care for
these patients.3 Although limited safety of interventional
procedures for the ACHD population has been suggested in
centers without ACHD global care programs,4 the wide
variation in clinical presentation, novelty of cardiovascular
pathologies (with similarity to as well as marked differences
from both congenital and acquired conditions), and potential
for concomitant multiple organ system pathology all contrib-
ute to situations atypical for standard adult or pediatric
laboratories. These concerns, combined with a desire to
centralize data collection to establish outcomes assessments
for ACHD patients considered for interventional catheteriza-
tion, lead to the recommendation that such procedures be
performed in centers with ACHD expertise and established
care programs.3
In this article, we review the most commonly performed
percutaneous procedures in ACHD, including valvuloplasty,
angioplasty, and device closures. We also include discussions
concerning complex ACHD patients (Table 2) as well as
future directions.
Valvuloplasty
Pulmonary Valve Stenosis
Pulmonary valve stenosis (PS) is almost always congenital in
origin and usually results from commissural fusion of thin
and pliable leaflets. Less frequently, the pulmonary valve is
dysplastic with thickened and less mobile leaflets. In patients
with complex malformations, such as tetralogy of Fallot or
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
michael.landzberg@cardio.chboston.org
(Circulation. 2007;115:1622-1633.)
© 2007 American Heart Association, Inc.
Circulation is available at http://www.circulationaha.org
1622
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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
gradients with age, and therefore most adults presenting with
moderate or severe obstruction are likely undiagnosed during
youth.
Percutaneous balloon pulmonary valvuloplasty (PPV) has
become the treatment of choice for adult patients with
isolated PS. The Second Natural History Study of Congenital
Heart Defects revealed that pulmonary valve gradients
⬎50 mm Hg were associated with poor outcomes from right
ventricular infarction, ventricular arrhythmias, and sudden
death.5 Conversely, gradients ⬍30 mm Hg in the absence of
subpulmonary ventricular systolic dysfunction or shunt-
mediated cyanosis were associated with good prognosis and
were unlikely to progress over the 2 to 3 decades to follow.
However, as the ACHD population ages, concern may be
raised regarding the even longer-term effects of mildly
increased subpulmonary ventricular afterload. Consequently,
given the low procedural risk and with the assumption that
low postprocedural gradient carries low subsequent long-term
risk, there is general agreement that PPV is indicated for
catheter-based pulmonary valve gradients ⬎40 mm Hg, irre-
spective of symptoms, or when gradients are ⱕ30 mm Hg but
in the presence of symptoms of subpulmonary ventricular
dysfunction or shunt-mediated cyanosis.6
PPV is highly technically successful and safe in the adult
population with results comparable to those of surgical
valvotomy. A significant reduction in gradient is obtained in
up to 80% of procedures (influenced by annulus size, valve
morphology, or operator technique), and most patients are
free of events in the long term after a single procedure.
Further reduction in residual gradients may be seen over time
because of resolution of residual hypertrophic subvalvular
stenosis. Long-term follow-up of adults who underwent PPV
in their youth suggests that, similar to results with surgical
valvotomy, late moderate or greater pulmonary regurgitation
is increasingly recognized,7 and preprocedural counseling and
surveillance for such are recommended. Although PPV has
been attempted in subvalvular PS and in adults with dysplas-
tic pulmonary valves, results are limited, and surgical valvot-
omy is usually indicated for these patients. The impact of
DOI: 10.1161/CIRCULATIONAHA.105.592428
 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
Tetralogy of Fallot RV dysfunction
LV dysfunction
Cyanosis
D-TGA (atrial switch)
Systemic venous congestion or subpulmonary ventricular failure
Pulmonary venous congestion or subaortic ventricular failure
Cyanosis
D-TGA (arterial switch operation)
Chest pain/ventricular failure
RV dysfunction
Semilunar valve regurgitation
Fontan Volume retention/fatigue
Cyanosis
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
intervention for adults with congenital AS, although current
practice guidelines suggest that for symptomatic adults, either
echocardiographic transvalvar Doppler velocity ⬎3.5 m/s,
mean gradient ⬎30 mm, or ECG T-wave inversion (and in
asymptomatic adults, mean gradient ⬎40 mm Hg or transval-
var Doppler velocity ⬎4 m/s) is an indication for catheter-
ization and consideration of percutaneous aortic valvulo-
plasty (PAV).6 At catheterization, a peak-to-peak gradient of
⬎60 mm Hg without severe calcification and with less than
moderate aortic insufficiency in adults without symptoms, as
well as a peak-to-peak gradient ⬎50 mm Hg in the presence
of symptoms, is considered an indication for PAV in patients
with congenital AS.
Although effective in reducing aortic valve gradients, PAV
is associated with a 10% to 30% risk of significant residual
aortic insufficiency (especially when a balloon/annulus ratio
⬎1 is used). Balance should be considered at the time of
balloon size selection between tolerating some residual ste-
nosis and avoiding significant aortic insufficiency. PAV is
associated with higher restenosis rates compared with PPV
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Intervention
ASD closure
RV outflow tract dilation/stent
PAS dilation/stent
Residual VSD closure
Percutaneous pulmonary valve implantation
Right coronary artery intervention
Residual aorta–pulmonary artery shunt closure
Residual VSD closure
Left coronary artery intervention
Percutaneous aortic valve implantation
PFO closure (RV dysfunction)
Systemic venous baffle obstruction dilation/stent
Baffle leak closure
Pulmonary venous baffle obstruction dilation/stent
Baffle leak closure
Percutaneous coronary intervention
Pulmonary artery obstruction dilation/stent
Percutaneous valve implantation
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
Pulmonary arteriovenous fistulae closure
Intramural/baffle leak closure
Systemic venous–pulmonary venous connection closure
(averaging 50% to 60% event-free survival at 5 to 10 years of
follow-up in young adults undergoing PAV).8 Risk and
success of surgical repair for the uncommon patient sustain-
ing avulsion of a valvular cusp during PAV do not appear
compromised by attempted PAV, with periprocedural echo-
cardiographic surveillance and timely surgical therapy.9 Al-
though recommended in national guidelines for consideration
as a first-line treatment in young and older adults with AS,
PAV is best considered a palliative procedure, potentially
delaying surgical valve replacement. Relative risks of surgi-
cal repair or replacement strategies should be counseled when
PAV is considered. The impact of associated aneurysmal
dilation of the ascending aorta (and potentially the pulmonary
trunk) in patients with AS should be taken into consideration,
as well. PAV has been attempted in patients with nondiscrete
subaortic stenosis with limited success, and surgical interven-
tion is generally recommended. Catheter-based percutaneous
balloon valvuloplasty has been offered as a shorter-termed
alternative to surgical repair of more discrete subaortic
stenosis when catheter-based gradient or mean echo gradient
is ⬎50 mm Hg and either symptoms develop or the ability to
 Inglessis and Landzberg
augment cardiac output is needed during pregnancy or for
competitive sports competition.10
Angioplasty
Aortic Coarctation
Aortic coarctation in the adult is typically located just distal
to the origin of the left subclavian artery, although various
degrees of obstruction can also occur throughout the aortic
arch. The presence of cystic medial necrosis in the aortic wall
adjacent to the coarctation site is believed to predispose to
wall rupture and aneurysm formation after surgical and
percutaneous interventions.
Indications for relief of obstruction even in the asymptom-
atic patient have included clinically determined radial-
femoral arterial pulse delay or catheter-derived peak-to-peak
gradient of ⱖ20 mm Hg at rest or ⱖ30 mm Hg after exercise.
However, relief of aortic coarctation may still be indicated in
the presence of lower gradients if there is a large collateral
network or subaortic ventricular systolic or diastolic dysfunc-
tion or if concomitant anatomic (aortic valve, aortic aneu-
rysm, coronary or carotid atherosclerosis) procedures are
being considered.
There is general agreement that percutaneous balloon
angioplasty with or without stent implantation is a preferred
treatment modality for recurrent postsurgical aortic coarcta-
tion.11 The procedure is successful in reducing the gradient to
⬍20 mm Hg in ⬇80% of interventions, with a 1.5% inci-
dence of late aneurysm formation (with low incidence of
aneurysm formation potentially the result of either postsurgi-
cal periaortic fibrosis or removal of most of the abnormal
aortic wall tissue at the time of surgery).
Percutaneous balloon angioplasty of the unoperated aortic
coarctation in the adult is more controversial, especially in the
absence of standardization of indication for repair, technique,
end points, and postprocedural follow-up. A higher incidence
of late aneurysm formation and restenosis compared with
surgically treated patients has been suggested but not univer-
sally confirmed.12–16 The procedure is successful in reducing
the gradient to ⬍20 mm Hg in 85% of patients, with a
restenosis rate of ⬇8%. Acute dissection has been observed
and may be related to failure to recognize aortic nondisten-
sibility during balloon inflation. Death from aortic rupture is
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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.
a rare complication and highlights the need for onsite
catheter-based and surgical support (see below). The inci-
dence of late aneurysm formation is higher than in postsur-
gical patients, with wide variance reported (from 8% to 35%)
in different series. The suspected mechanism for late aneu-
rysm formation is intimal tear at the site of cystic media
necrosis, although current literature does not support a
relationship between balloon size, coarctation diameter, and
the incidence of late aneurysm formation. The overall long-
term clinical impact of such aneurysms is unclear because
most aneurysms tend to be small and have not required
further treatment.
Stent implantation may theoretically overcome some of the
shortcomings of balloon dilatation for aortic coarctation
because metal scaffolding may reduce the incidence of acute
elastic recoil as well as late restenosis due to more complete
elimination of gradient in the high-velocity flow arterial
system (Figure 1). Additionally, stents may reduce the inci-
dence of residual intimal tears and subsequent aneurysm
formation by allowing both the use of smaller dilation
balloons (especially in the presence of mild anatomic steno-
sis) as well as graded inflations in staged procedures. Intra-
vascular stent implantation may carry greater potential in
adult patients than in children because patient growth is less
of a problem, and the aging aorta is potentially more fragile.
Preliminary data on the use of stents for aortic coarctation
suggest a lower residual stenosis, lower restenosis rate, and
lower rate of late aneurysm formation (⬍5%) compared with
balloon angioplasty alone. Aortic rupture has been seen in
stent implantation procedures, especially when lack of com-
pliance of the aorta has not been recognized. The risk of
subacute stent thrombosis is believed to be low, and although
periprocedural antiplatelet or anticoagulant regimens are
standardly prescribed, their use remains unsubstantiated.
Stent implantation has rapidly become a recommended pro-
cedure for the treatment of unoperated aortic coarctation in
many institutions, despite the lack of available multicenter
long-term registry data regarding risks or benefits of this
procedure. Covered stents should be readily available in the
cardiac catheterization laboratory for the emergency therapy
of acute aortic dissection or rupture complicating endovascu-
lar procedures. This technology has also been considered for
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
To date, optimal postimplantation antiplatelet or anticoag-
ulant strategies remain undefined.
Conduit and Baffle Stenosis
Extracardiac conduits may be used to connect the subpulmo-
nary ventricle to the pulmonary arteries in patients with
complex congenital disease. These conduits can develop
obstruction secondary to severe angulation, calcification,
sternal compression, or tissue proliferation, most notably at
anastomotic sites. Balloon angioplasty procedures offer little
improvement, and restenosis is common. Stent implantation
has demonstrated better long-term outcome (predominantly
measured as freedom from reoperation), albeit with recog-
nized risk.24 Stent fracture has been observed in up to 16% of
cases. Stent implantation at anastomotic sites may limit the
ability of future surgical conduit replacement and may result
in severe regurgitation in valved conduits. Finally, the poten-
tial for coronary compression (coursing of coronary artery
sufficiently adjacent to the conduit) by stent implantation has
been reported, with catastrophic potential, and must be
assessed before intervention.
Conduit stenosis can be observed after Glenn or Fontan
operations (Figure 3). Similarly, systemic baffle obstruction
is observed in up to 15% of patients undergoing atrial switch
(Mustard or Senning) procedures. Balloon angioplasty occa-
sionally results in long-term relief; however, stent deploy-
ment has been highly successful in relieving obstruction, with
low complication rates and superior results, especially when
performed in the superior portion of the systemic venous
baffle after atrial switch operations. Systemic anticoagulation
has been recommended for at least 6 months after stenting of
the low-velocity flow baffles, without supportive data.
Device Closure
Interatrial Communications
Interatrial communications can be classified as atrial septal
defect (ASD) or patent foramen ovale (PFO), each having
different clinical and anatomic implications.
The left-to-right shunt in an ASD is driven by compliance
differences, with low to nil transatrial pressure gradients.
Morbidities related to aging may lead to decreased left
ventricular compliance, contributing to increased left-to-right
shunt.
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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.
Of all the interventions for adults with congenital heart
disease, the greatest consensus appears regarding the indica-
tion for and method of secundum ASD closure. The standard
for intervention has been promulgated as the imaging-based
presence of right ventricular volume overload (usually asso-
ciated with sustained pulmonary to systemic flow ratio
[Qp/Qs] ⬎1.5, the threshold associated with the potential for
right ventricular dysfunction, progressive pulmonary vascular
disease, and atrial arrhythmia development, even in asymp-
tomatic patients). Right-to-left shunting may be present in
such patients as evidence of streaming or elevation of
pulmonary vascular resistance, with closure still being con-
sidered provided that net Qp/Qs is ⬎1.5 (typically with
pulmonary vascular resistance ⬍8 to 10 indexed Wood units
and pulmonary/systemic resistance ⬍0.2 to 0.4), although
increased long-term risk due to residual pulmonary vascular
disease likely persists. Intracatheterization ASD test occlu-
sion with observation of resultant hemodynamics may aid in
observing the acute hemodynamic effects of closure, although
studies assessing the longer-term predictive nature of such
testing have not been performed. Complex strategies utilizing
concomitant pulmonary vascular therapies or customized
“fenestrated” closure devices have been reported in patients
with large-volume left-to-right shunting and moderate to
severe pulmonary hypertension.
Even though there are no randomized trials comparing
percutaneous versus surgical closure of ASDs, most centers
favor percutaneous closure if the anatomy is suitable.25
Percutaneous closure of ASDs has been reported in nonran-
domized uncontrolled fashion to be associated with improve-
ment in right ventricular anatomy, function, and left ventric-
ular interaction, as well as in exercise capacity as measured
by cardiopulmonary exercise testing.26,27
ASDs can be classified as ostium secundum, ostium
primum, sinus venosus, and coronary sinus septal defects,
depending on location in the interatrial septum. Occasionally,
multiple defects can be observed, especially associated with a
thin, hypermobile septum primum. Multiple devices have
been used for percutaneous ASD closure since the technique
was first attempted in the mid-1970s. The basic device design
is of a dual-disc structure joined at a waist, with each disc
deployed in either side of the septum. An atrial septum rim
⬎5 mm around the majority of the defect is required for the
safe use of any closure device, although more deficient
1628 Circulation March 27, 2007
Figure 4. Currently available devices within the United States
for percutaneous closure of atrial-level defects within random-
ized controlled trials.
anterior superior aortic rim is typical and does not preclude
percutaneous closure.
The only currently available devices for clinical use world-
wide are the Amplatzer septal occluder (AGA Medical), the
CardioSEAL device (NMT Medical), and the Helex septal
occluder (W.L. Gore and Associates) (Figure 4). At present,
2 devices (Amplatzer septal occluder and CardioSEAL de-
vice) are approved in the United States for closure of different
types of atrial defects, with the largest worldwide experience
with the use of the Amplatzer septal occluder (Figure 5).
Each of the aforementioned devices has its own advantages
and disadvantages. The Amplatzer septal occluder is a self-
centering and retrievable device up to the point of full release;
however, it has a larger profile once deployed, and rare cases
of late cardiac perforations and erosions (as late as 3 years
after implantation) causing serious or catastrophic events
have been reported and remain a source of concern and
indication for further study. The CardioSEAL device typi-
cally has a lower profile favoring rapid and more extensive
endothelialization. However, only its STARFlex modification
(NMT Medical) is a self-adjusting device. As well, retrieval
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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
under prospective study.
The Amplatzer device allows for closure of ostium secun-
dum ASDs with ⬍36 to 40 mm of nominal balloon stretched
diameter, whereas the CardioSEAL can only be used in
off-label fashion for moderate-sized defects (⬍18 mm). Both
devices are successful in closing ASDs, with procedural
success (almost complete or complete closure and absence of
major complications) reported for the Amplatzer device at
⬎95% at 3 months. Although earlier nonrandomized studies
reported greater safety and efficacy with the use of the
Amplatzer device compared with the Clamshell (C.R. Brad
Inc) and CardioSEAL devices,28,29 the self-adjusting STAR-
Flex modification has been reported to have procedural
success equal to the Amplatzer septal occluder with fewer
complications.30
Although fluoroscopic guidance alone is possible and used
by some, most operators prefer echocardiographic guidance
for percutaneous ASD closure because it offers a more
complete and accurate evaluation of the interatrial septum
and surrounding structures. Both transesophageal and intra-
cardiac echocardiography have proven to be useful and
effective in guiding procedures, with most operators prefer-
ring transesophageal echocardiography for large or multiple
defects.
 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
refractory migraines.
Ventricular Septal Defects
VSDs are classified as inflow, muscular, or perimembranous
depending on location in the septum. Patients with sizable
VSD can develop cardiac left-sided volume overload, subse-
quent ventricular failure, and pulmonary hypertension, typi-
cally at younger ages than patients with atrial-level defects.
Consequently, most patients with large enough defects requir-
ing therapy are usually diagnosed and treated in childhood.
VSD closure in the adult is usually recommended either when
left ventricular volume overload, unexplained by alternative
mechanism, is present, correlating with Qp/Qs ⬎1.5, in the
presence of multiple recurrences of otherwise unexplained
bacterial endocarditis, or when accompanied by progressive
aortic regurgitation.
Percutaneous closure of congenital or acquired (post–
myocardial infarction septal rupture) muscular VSD is a
particularly attractive therapeutic option because surgical
closure of these defects may carry increased risk of morbidity
and mortality. Transcatheter VSD closure is generally tech-
nically more challenging than ASD closure, with intravascu-
lar passage through the VSD with the use of a balloon-tipped
catheter (to ensure passage via the largest lumen) from the left
ventricular side, snaring a guidewire in the pulmonary artery,
formation and externalization of a venous-arterial-venous
vascular loop, and subsequent device deployment based on a
pathway allowing maximal device arm–septal apposition,
without interference with adjacent intracardiac structures.
Although this technique may be highly successful in achiev-
ing clinical improvement as defined by risk scales designed
for this population, recent reports have highlighted the
complexity and morbidities encountered in such high-risk
patients undergoing percutaneous VSD closure with earlier
double-umbrella devices as well as the current CardioSEAL
occluder (the 1 device approved in the United States for this
indication).38 Early results with the Amplatzer muscular VSD
occluder, although encouraging, have not included outcome
data related to longer-term clinical improvement.39 Percuta-
neous closure of perimembranous VSD is even more chal-
lenging because of the proximity of the aortic and tricuspid
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valve, as well as the conduction system, with postprocedural
complete heart block observed on occasion, even with asym-
metrical devices. Increased data collection and design modi-
fications have potential to improve safety and efficacy.40
Patent Ductus Arteriosus
A patent ductus arteriosus (PDA) is an abnormally persistent
arterial connection after birth between the descending aorta
and the pulmonary artery, most commonly to the junction of
the main and left pulmonary artery branches. As is the case
for VSD, patients with large PDA may develop left-sided
volume overload and pulmonary hypertension at younger
ages, leading to diagnosis early in life. It is not uncommon
that the diagnosis of PDA is made in adulthood by means of
physical examination and presence of the continuous murmur
typical of PDA or as an incidental finding on echocardiogra-
phy. Additional problems associated with PDA include infec-
tious endarteritis, aneurysm formation, calcification, and rare
rupture. Large PDA with significant left-to-right shunt should
be closed to reduce occurrence of the sequelae of subaortic
ventricular failure or pulmonary arterial hypertension. In
adults, the treatment of small anatomic PDAs with associated
small-quantity shunting remains controversial. The standard
to advise PDA closure in an asymptomatic adult patient, with
an audible murmur but without other indication, has come
under increasing debate due to lack of supportive data for
such interventions.
Percutaneous closure of PDA has been performed for ⬎20
years with several generations of devices and is the preferred
mode of therapy worldwide given increasing surgical risk
with age because of PDA calcification and potential for
intraoperative recurrent laryngeal nerve damage. Currently,
in adults with PDA, the Amplatzer ductal occluder is the most
commonly used device (Figure 7), typically implanted during
an outpatient procedure, with coil embolization reserved for
PDA measuring ⬍2 to 3 mm or for residual leaks. Complete
closure has been reported in ⬎95% of patients at 6 months
with both techniques, with device embolization being rarely
encountered.41 Contrary to PDA closure in children, left
pulmonary artery obstruction is not a concern in adults. The
postimplantation antiplatelet regimen remains unsubstanti-
ated, although it is typically recommended for 3 to 4 months,
 Inglessis and Landzberg
and patients continue to receive antibiotic prophylaxis against
subacute bacterial endocarditis for 12 months after device
implantation.
Fontan Fenestrations and Atrial Switch
Baffle Leaks
Patients after Fontan operation may have spontaneous or
surgically created residual fenestrations that may lead to
right-to-left shunt and consequent systemic oxygen desatura-
tion, systemic emboli, and exercise incapacity. These fenes-
trations can be closed with the use of the CardioSEAL or
Amplatzer septal occluder devices. However, given the non-
pulsatile circulation and prothrombosis encountered in pa-
tients with Fontan palliation, we favor use of the lower-
profile CardioSEAL (the only device currently approved in
the United States for fenestration closure). Patients typically
receive long-term anticoagulation and prophylaxis against
subacute bacterial endocarditis. Transient balloon test occlu-
sion of the fenestration has been correlated with successful
longer-term outcomes after closure and, given such, should
be performed before permanent closure.42
Up to 25% of patients undergoing Mustard or Senning
operations will demonstrate late baffle leaks, likely as the
result of suture dehiscence. Although many of these shunts
are hemodynamically unapparent and do not require therapy,
closure is indicated for large defects resulting in significant
intracardiac shunting. Percutaneous closure has been reported
with the use of both CardioSEAL and Amplatzer devices as
alternatives to surgery.
Coil Embolization
Aortopulmonary Arterial Collaterals
Aortopulmonary collaterals may be observed in patients with
congenital malformations associated with decreased pulmo-
nary flow, including pulmonary atresia, as well as variations
of single-ventricle physiology or after Glenn shunt or Fontan
palliation. These collaterals can arise from any systemic
artery and connect directly to the pulmonary arteries at the
lung hilum or at the periphery. Resulting left-to-left systemic
arterial to pulmonary arterial shunting may cause pulmonary
vascular disease, elevation of systemic ventricular filling
pressure, systemic ventricular volume loading, and ultimate
failure. Residual surgically created systemic to pulmonary
arterial shunts share the same pathophysiological sequelae.
In addition to the aforementioned factors, aortopulmonary
collateral embolization is indicated before the final stage of
surgical subpulmonary ventricle to pulmonary artery connec-
tion because left-to-right shunt directly into the pulmonary
arteries complicates cardiopulmonary bypass, and dual sup-
ply to vascular branches leads to overcirculation and devel-
opment of pulmonary arterial hypertension. Embolization is
typically performed in those collaterals that share significant
flow from the main or additional unifocalized pulmonary
arteries to avoid lung infarction. Embolization is typically
performed with coils; however, the Amplatzer vascular plug
has recently become available for occlusion of larger collat-
erals, with similar success reported.
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Interventional Catheterization in the Adult 1631
Coronary Fistulae
Coronary fistulae can originate from all major epicardial
coronary arteries, and drainage usually occurs to the coronary
sinus, right atrium, right ventricle, or pulmonary artery. These
collaterals can become markedly enlarged and can lead to
significant left-to-right shunting with right-sided volume
overload and effective coronary arterial steal leading to
ischemia. Embolization has been reported predominantly
with coils; however, other devices such as ASD closure
devices or the Amplatzer vascular plug have been used
successfully, as well.43 Long-term effects of intravascular
fistula occlusion have yet to be demonstrated.
Other Forms of Collaterals
Collaterals from the systemic to the pulmonary veins
(venous-venous collaterals) can occur in patients with single-
ventricle physiology, especially after Glenn or Fontan oper-
ations, in part because of chronically elevated systemic
venous pressures. Pulmonary arteriovenous fistulae may also
present late after Glenn anastomosis, as well as in patients
with chronic liver failure or with hereditary hemorrhagic
telangiectasia (Osler-Weber-Rendu syndrome). In both in-
stances, there is right-to-left shunt with various degrees of
cyanosis. Both types of abnormal connections can be treated
successfully with coil embolization or Amplatzer closure
devices44 provided that right-to-left shunt is not of hemody-
namic benefit as potential relief of subpulmonary ventricular
afterload due to the presence of severe pulmonary arterial
hypertension. Nevertheless, recurrence of collaterals is not
uncommon unless the underlying physiology driving their
development is modified significantly.
Miscellaneous Procedures
As the ACHD population ages, epicardial coronary disease is
becoming more prevalent. Thus, coronary angiography is
indicated at the time of catheterization in all patients older
than 40 years or in younger patients with significant risk
factors for atherosclerosis (in particular, all men aged ⬎35
years). Epicardial coronary stenting should be performed, if
indicated, by operators with expertise in percutaneous coro-
nary revascularization techniques. Additionally, coronary an-
giography should be performed before any percutaneous
procedure with potential for coronary compromise, such as
stenting of a right ventricular to pulmonary conduit.
Balloon atrial septoplasty has been used to create or
enlarge existing ASDs in patients with severe pulmonary
hypertension and right ventricular failure as means for pro-
viding a “pop-off” valve to the failing right ventricle. How-
ever, this approach must be balanced against excessive
decreases in systemic oxygen saturations from increased
right-to-left shunting. Restenosis of the interatrial septum is
common, and fenestrated closure devices are becoming avail-
able with the hope of improving long-term patency.
Baffle fenestrations are performed increasingly at the time
of Fontan surgery because they reduce the incidence of
postoperative effusions. These fenestrations can become oc-
cluded in the early postoperative period, leading to rapid
hemodynamic deterioration from increased pulmonary vas-
cular resistance and low cardiac output. Balloon angioplasty
1632 Circulation March 27, 2007
of the fenestration usually restores patency; however, there is
risk of thrombus embolization to the left heart. Rarely, the
fenestration cannot be proved with a catheter, and wire and
baffle puncture with a transseptal needle is required.
Cardiac arrhythmias are frequent in the complex ACHD
patient, resulting in unpredictable and variable degrees of
hemodynamic compromise. Electrophysiology procedures,
such as programmed stimulation and temporary pacing, are
performed frequently in conjunction with simultaneous he-
modynamic measurements, allowing for accurate diagnosis
and development of treatment strategies.
Future Directions
Percutaneous Valve Replacement
Percutaneous valve replacement has substantive potential for
ACHD patients who have been subjected to multiple opera-
tions and may have premature aging of multiple organ
systems, raising the risk of recurrent surgeries. The largest
group to potentially benefit from such advances, at present,
includes patients with tetralogy of Fallot with postoperative
residual pulmonary insufficiency. Early reports of percutane-
ous pulmonary valve replacement in this population are
encouraging. Nevertheless, unresolved issues remain, includ-
ing valve durability, stent fracture, device embolization,
adjacent structure impingement, limited valve size, recurrent
regurgitation and stenosis, and the need for large catheters for
delivery, although most initial results are encouraging in
limited centers.45 Larger-scale multicenter trials with percu-
taneous pulmonary valve stent implantation are expected,
and, if found to be of benefit, it is hoped that they will extend
the potential for more widespread applications.
Hybrid Procedures
As collaboration between congenital cardiovascular surgeons
and interventional cardiologists expands, hybrid procedures,
involving both teams simultaneously or in temporally staged
fashion, may increase clinical application of technological
applications, with the potential to extend the clinical well-
being of ACHD patients. Examples of such hybrid interven-
tions include intraoperative pulmonary artery dilation in
preparation for pulmonary valve or homograft placement,
percutaneous closure of apical or muscular VSDs under direct
vision in the operating department, and percutaneous closure
of aortopulmonary collaterals or completion of conduit place-
ment as Fontan palliation.46
Conclusion
The ACHD patient, at baseline or in need of intervention,
presents unique challenges to caregivers. Life demands (in-
cluding sudden needs for augmentation in cardiac output,
such as with exercise or pregnancy) and altered anatomy
coupled with increasing potential for exercise incapacity and
progressive extracardiac comorbidity contribute to physiolog-
ical occurrences, risks, and complications encountered less
frequently in typical pediatric or adult catheterization labora-
tories. As witnessed in the advancement of pediatric congen-
ital cardiovascular care, it is highly likely that catheteriza-
tion-based interventions planned and performed in
conjunction with appropriate surgical and medical caregivers
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in centers expert in such care, with centralized data collection
and outcome assessments, will likewise assist in the further
advancement of adults with congenital heart disease and its
sequelae.3
Disclosures
Dr Inglessis is on the Speakers’ Bureau for Boston Scientific and is
a consultant for Medtronic. Dr Landzberg has received research
grants from and served as a consultant for NMT Medical, AGA
Medical, Actelion, and Nitrox. He has also received a grant from
Myogen.
References
1. Warnes CA, Liberthson R, Danielson GJ, Dore A, Harris L, Hoffman JL,
Somerville J, Williams RG, Webb GD. Task Force 1: the changing profile
of congenital heart disease in adult life. J Am Coll Cardiol. 2001;37:
1170 –1175.
2. Mackie AS, Pilote L, Ionescu-Itu R, Rahme E, Marelli AJ. Healthcare
resource utilization in adults with congenital heart disease: a population
based study. J Am Coll Cardiol. 2006;47(suppl):235A. Abstract.
3. Landzberg MJ, Murphy DJ Jr., Davidson WR Jr., Jarcho JA, Krumholz
HM, Mayer JE, Mee RB, Sahn DJ, Van Hare GF, Webb GD, Williams
RG. Task Force 4: organization of delivery systems for adults with
congenital heart disease. J Am Coll Cardiol. 2001;37:1187–1193.
4. Garekar S, Paules MM, Reddy SV, Turner DR, Sanjeev S, Wynne J,
Epstein ML, Karpawich PP, Ross RD, Forbes TJ. Is it safe to perform
cardiac catheterizations in adults with congenital heart disease in a
pediatric catheterization laboratory? Catheter Cardiovasc Interv. 2005;
66:414 – 419.
5. Hayes CJ, Gersony WM, Driscoll DJ, Keane JF, Kidd L, O’Falloon WM,
Pieroni DR, Wolfe RR, Weidman WH. Second natural history study of
congenital heart defects: results of treatment of patients with pulmonary
valvar stenosis. Circulation. 1993;87:I28 –I37.
6. American College of Cardiology; American Heart Association Task
Force on Practice Guidelines (Writing Committee to Revise the 1998
Guidelines for the Management Of Patients With Valvular Heart
Disease); Society of Cardiovascular Anesthesiologists; Society for Car-
diovascular Angiography and Interventions; Society of Thoracic Sur-
geons; Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr, Faxon
DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O’Gara PT,
O’Rourke RA, Otto CM, Shah PM, Shanewise JS, Smith SC Jr, Jacobs
AK, Adams CD, Anderson JL, Antman EM, Fuster V, Halperin JL,
Hiratzka LF, Hunt SA, Lytle BW, Nishimura R, Page RL, Riegel B.
American College of Cardiology/American Heart Association 2006
guidelines for the management of patients with valvular heart disease: a
report of the American College of Cardiology/American Heart Asso-
ciation Task Force on Practice Guidelines (Writing Committee to Revise
the 1998 Guidelines for the Management of Patients with Valvular Heart
Disease). J Am Coll Cardiol. 2006;48:e1– e148.
7. Garty Y, Veldtman G, Lee K, Benson L. Late outcomes after pulmonary
valve balloon dilation in neonates, infants and children. J Invasive
Cardiol. 2005;17:318 –322.
8. Rosenfeld HM, Landzberg MJ, Perry SB, Colan SD, Keane JF, Lock JE.
Balloon aortic valvuloplasty in the young adult with congenital aortic
stenosis. Am J Cardiol. 1994;73:1112–1117.
9. Minich LL, Tani LY, Hawkins JA, McGough EC, Shaddy RE. Use of
echocardiography for detecting aortic valve leaflet avulsion and pre-
dicting repair potential after balloon valvuloplasty. Am J Cardiol. 1995;
75:533–535.
10. Richartz BM, Figulla HR, Ferrari M. KutheF, Bulow HJ, Kehrer G,
Werner GS. Percutaneous balloon dilatation of discrete subaortic stenosis.
Z Kardiol. 2002;91:581–583.
11. Hellebrand WE, Allen HD, Golinko RJ, Lutin W, Kan J. Balloon angio-
plasty for aortic recoarctation: results of Valvuloplasty and Angioplasty
of Congenital Anomalies Registry. Am J Cardiol. 1990;65:793–797.
12. Shaddy RE, Boucek MM, Sturtevant JE, Ruttenberg HD, Jaffe RB, Tani
LY, Judd VE, Veasy LG, McGough EC, Orsmond GS. Comparison of
angioplasty and surgery for unoperated coarctation of the aorta. Circu-
lation. 1993;87:793–799.
13. Fawsy ME, Awad M, Hassan W, Al Kadhi Y, Shoukri M, Fadley F.
Long-term outcome (up to 15 years) of balloon angioplasty of discrete
native coarctation of the aorta in adolescent and adults. J Am Coll
Cardiol. 2004;43:1062–1067.
 Inglessis and Landzberg
14. Oliver JM, Gallego P, Gonzalez A, Aroca A, Bret M, Mesa JM. Risk
factors for aortic complications in adults with coarctation of the aorta.
J Am Coll Cardiol. 2004;44:1641–1647.
15. Cowley CG, Orsmond GS, Feola P, McQuillan L, Shaddy R. Long-term,
randomized comparison of balloon angioplasty and surgery for native
coarctation of the aorta in childhood. Circulation. 2005;111:3453–3456.
16. Carr JA. The results of catheter-based therapy compared with surgical
repair of adult aortic coarctation. J Am Coll Cardiol. 2006;47:1101–1107.
17. Ince H, Petzsch M, Rehders T, Kische S, Korber T, Weber F, Nienaber C.
Percutaneous endovascular repair of aneurysm after previous coarctation
surgery. Circulation. 2003;108:2967–2970.
18. Butera G, Piazza L, Chessa M, Abella R, Bussadori C, Negura D,
Carminati M. Covered stents in patients with congenital heart defects.
Catheter Cardiovasc Interv. 2006;67:466 – 472.
19. Kreutzer J, Landzberg MJ, Preminger TJ, Mandell VS, Treves ST, Reid
LM, Lock JE. Isolated peripheral pulmonary artery stenoses in the adult.
Circulation. 1996;93:1417–1423.
20. Gentles TL, Lock JE, Perry SB. High pressure balloon angioplasty for
branch pulmonary artery stenosis: early experience. J Am Coll Cardiol.
1993;22:867– 872.
21. Bush DM, Hoffman TM, Del Rosario J, Eiriksson H, Rome JJ. Frequency
of restenosis after balloon pulmonary arterioplasty and its causes.
Am J Cardiol. 2000;86:1205–1209.
22. Baker CM, Mcgowan FX Jr, Keane JF, Lock JE. Pulmonary artery trauma
due to balloon dilation: recognition, avoidance and management. J Am
Coll Cardiol. 2000;36:1684 –1690.
23. Feinstein JA, Goldhaber SZ, Lock JE, Fernandes SM, Landzberg MJ.
Balloon pulmonary angioplasty for treatment of chronic thromboembolic
pulmonary hypertension. Circulation. 2001;103:10 –13.
24. Peng LF, McElhinney DB, Nugent AW, Powel AJ, Marshal AC, Bacha
EA, Lock JE. Endovascular stenting of obstructed right ventricle-to-pul-
monary artery conduits: a 15-year experience. Circulation. 2006;113:
2598 –2605.
25. Du ZD, Hijazi ZM, Kleinman CS, Silverman NH, Larntz K, Amplatzer
Investigators. Comparison between transcatheter and surgical closure of
secundum atrial septal defect in children and adults: results of a multi-
center nonrandomized trial. J Am Coll Cardiol. 2002;39:1836 –1844.
26. Veldtman GR, Razack V, Siu S, El-Hajj H, Walker F, Webb GD, Benson
LN, McLaughlin PR. Right ventricular form and function after percuta-
neous atrial septal defect closure. J Am Coll Cardiol. 2001;37:
2108 –2113.
27. Brochu MC, Baril JF, Dore A, Juneau M, De Guise P, Mercier LA.
Improvement in exercise capacity in asymptomatic and mildly symptom-
atic adults after atrial septal defect percutaneous closure. Circulation.
2002;106:1821–1826.
28. Butera G, Carminati M, Chessa M, Delogu A, Drago M, Piazza L,
Giamberti A, Frigiola A. CardioSEAL/STARFlex versus Amplatzer
devices for percutaneous closure of small to moderate (up to 18 mm)
atrial septa defects. Am Heart J. 2004;148:507–510.
29. Post M, Suttorp M, Jaarsma W, Plokker T. Comparison of outcome and
complications using different types of devices for percutaneous closure of
a secundum atrial septal defect in adults: a single center experience.
Catheter Cardiovasc Interv. 2006;67:438 – 443.
30. Nugent AW, Britt A, Gauvreau K, Piercey G, Lock JE, Jenkins KJ.
Device closure rates of simple atrial septal defects optimized by the
STARFlex device. J Am Coll Cardiol. 2006;48:538 –544.
31. Rodes-Cabau J, Palacios A, Palacio C, Girona J, Galve E, Evangelista A,
Casaldliga J, Albert D, Pico M, Soler-Soler J. Assessment of the markers
of platelet and coagulation activation following transcatheter closure of
atrial septal defects. Int J Cardiol. 2005;98:107–112.
Downloaded from http://circ.ahajournals.org/ by guest on May 28, 2015
Interventional Catheterization in the Adult 1633
32. US Department of Health and Human Services Food and Drug Admin-
istration Web site. Center for Devices and Radiologic Health. H990011-
CardioSEAL septal occlusion system. Available at http://www.fda.gov/
cdrh/ode/H990011sum.html. Accessed July 5, 2006.
33. US Department of Health and Human Services Food and Drug Admin-
istration Web site. Center for Devices and Radiologic Health. H000007-
Amplatzer PFO occluder. Available at: http://www.fda.gov/cdrh/ode/
H000007sum.html. Accessed July 5, 2006.
34. Sacco RL, Adams R, Albers G, Alberts MJ, Benavente O, Furie K,
Goldstein LB, Gorelick P, Halperin J, Harbaugh R, Johnston SC, Katzan
I, Kelly-Hayes M, Kenton EJ, Marks M, Schwamm LH, Tomsick T.
Guidelines for prevention of stroke in patients with ischemic stroke or
transient ischemic attack: a statement for healthcare professionals from
the American Heart Association/American Stroke Association Council on
Stroke. Circulation. 2006;113:e409 – e449.
35. Khairy P, O’Donnell CP, Landzberg MJ. Transcatheter closure versus
medical therapy of patent foramen ovale and presumed paradoxical
thromboemboli: a systematic review. Ann Intern Med. 2003;139:
753–760.
36. Maisel WH, Laskey WK. Patent foramen ovale closure devices: moving
beyond equipoise. JAMA. 2005;294:366 –369.
37. Delgado G, Inglessis I, Martin-Herrero F, Yoerger D, Liberthson R,
Buonanno F, Palacios IF. Management of platypnea-orthodeoxia
syndrome by transcatheter closure of atrial communication: hemodynam-
ic characteristics, clinical and echocardiographic outcome. J Invasive
Cardiol. 2004;16:578 –582.
38. Knauth AL, Lock JE, Perry SB, McElhinney DB, Gauvreau K, Landzberg
MJ, Rome JJ, Hellenbrand WE, Ruiz CE, Jenkins KJ. Transcatheter
device closure of congenital and postoperative residual ventricular septal
defects. Circulation. 2004;110:501–507.
39. Holzer R, Balzer D, Cao Ql, Lock K, Hijazi ZM, Amplatzer Muscular
Ventricular Septal Defect Investigators. Device closure of ventricular
septal defects using the Amplatzer muscular ventricular septal defect
occluder: immediate and mid-term results of a U.S. registry. J Am Coll
Cardiol. 2004;43:1257–1263.
40. Fu YC, Bass J, Amin Z, Radtke W, Cheatham JP, Hellebrand WE, Balzer
D, Cao Ql, Hijazi ZM. Transcatheter closure of perimembranous ventric-
ular septal defects using the new Amplatzer membranous VSD occluder:
results of the US phase 1 trial. J Am Coll Cardiol. 2006;47:319 –325.
41. Pass RH, Hijzai Z, Hsu DT, Lewis V, Hellenbrand WE. Multicenter USA
Amplatzer patent ductus arteriosus occlusion device trial: initial and
one-year results. J Am Coll Cardiol. 2004;44:513–519.
42. Goff DA, Blume Ed, Gauvreau K, Mayer JE, Lock JE, Jenkins
KJ. Clinical outcome of fenestrated Fontan patients after closure: the first
10 years. Circulation. 2000;102:2094 –2099.
43. Armsby LR, Keane JF, Sherwood MC, Forbess JM, Perry SB, Lock JE.
Management of coronary artery fistulae: patient selection and results of
transcatheter closure. J Am Coll Cardiol. 2002;39:1026 –1032.
44. Bialkowski J, Zabal C, Szkutnik M, Garcia-Montes JA, Kusa J, Zembala
M. Percutaneous interventional closure of large pulmonary arteriovenous
fistulas with the Amplatzer duct occluder. Am J Cardiol. 2005;96:
127–129.
45. Coats L, Khambadkone S, Derrick G, Sridharan S, Schievano S, Mist B,
Jones R, Deanfield JE, Pellerin D, Bonhoeffer P, Taylor AM. Physiolog-
ical and clinical consequences of relief of right ventricular outflow
obstruction late after repair of congenital heart defects. Circulation.
2006;113:2037–2044.
46. Bacha EA, Cao QL, Galantowicz ME, Cheatham JP, Fleishman CE,
Weinstein SW, Becker PA, Hill SL, Koenig P, Alborias E, Abdulla R,
Starr JP, Hijazi ZM. Multicenter experience with periventricular device
closure of muscular ventricular septal defects. Pediatr Cardiol. 2005;26:
169 –175.
 Interventional Catheterization in Adult Congenital Heart Disease
Ignacio Inglessis and Michael J. Landzberg

Circulation. 2007;115:1622-1633
doi: 10.1161/CIRCULATIONAHA.105.592428
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