Catheterization and Cardiovascular Interventions 67:679–686 (2006)

Core Curriculum

Management Algorithm in Pulmonary Atresia With

Intact Ventricular Septum
Mazeni Alwi,* MBBS, MRCP

Pulmonary atresia with intact ventricular septum (PAIVS) is a disease with remarkable
morphologic variability, affecting not only the pulmonary valve but also the tricuspid
valve, the RV cavity and coronary arteries. With advances in interventional techniques
and congenital heart surgery, the management of PAIVS continues to evolve. This
review is an attempt at providing a practical approach to the management of this dis-
ease. The basis of our approach is morphologic classification as derived from echocar-
diography and angiography. Group A, patients with good sized RV and membranous
atresia, the primary procedure at presentation is radiofrequency (RF) valvotomy. Often
it is the only procedure required in this group with the most favourable outcome.
Patients with severely hypoplastic RV (Group C) are managed along the lines of hearts
with single ventricle physiology. The treatment at presentation is patent ductus arterio-
sus (PDA) stenting with balloon atrial septostomy or conventional modified Blalock
Taussig (BT) shunt. Bidirectional Glenn shunt may be done 6–12 months later followed
by Fontan completion after a suitable interval. Patients in Group B, the intermediate
group, are those with borderline RV size, usually with attenuated trabecular component
but well developed infundibulum. The treatment at presentation is RF valvotomy and
PDA stenting 6 balloon atrial septostomy. Surgical re-interventions are not uncom-
monly required viz. bidirectional Glenn shunt when the RV fails to grow adequately (1½ –
ventricle repair) and right ventricular outflow tract (RVOT) reconstruction for subvalvar
obstruction or small pulmonary annulus. Catheter based interventions viz. repeat bal-
loon dilatation or device closure of patent foramen ovale (PFO) may also be required in
some patients. ' 2006 Wiley-Liss, Inc.

Key words: membranous atresia; RV hypoplasia; coronary

INTRODUCTION to decompress the hypertensive RV, or both concomi-

Pulmonary atresia with intact ventricular septum
(PAIVS) is an uncommon cyanotic congenital heart
disease characterized by varying degrees of underde-
velopment of the right ventricle (RV), ranging from a
severely hypoplastic RV where the definitive treatment
is along the line of hearts with single ventricle physiol-
ogy, to those where the RV hypoplasia is mild and an
excellent outcome with two ventricle circulation can
be expected. Apart from the RV size, other well recog-
nized abnormalities in this disease are the presence of
RV-coronary arterial connections, varying degrees of
tricuspid regurgitation with or without Ebstein’s mal-
formation of the tricuspid valve (TV), and very rarely,
aortopulmonary collateral vessels [1–5].
As this is a condition with a duct dependent pulmo-
nary blood flow, intervention is almost always required
in the neonatal period. Conventionally, this has been
in the form of constructing a systemic-pulmonary shunt
or opening the right ventricular outflow tract (RVOT)
tantly [3,6–8]. Not uncommonly re-interventions are
required later as part of the staging procedure, due to
suboptimal result of the initial definitive procedure
(inadequate RV decompression), recurrence of RVOT
obstruction, or progression of other haemodynamic prob-
lems such as tricuspid regurgitation [3,9,10].
Transcatheter therapy—radiofrequency valvotomy and
balloon dilatation (RFV), patent ductus arteriosus (PDA),
Institut Jantung Negara (National Heart Institute), Jalan Tun
Razak, Kuala Lumpur, Malaysia
*Correspondence to: Mazeni Alwi, Institut Jantung Negara (National
Heart Institute), 145 Jalan Tun Razak, 50400 Kuala Lumpur, Malaysia.
E-mail: mazeni@ijn.com.my
Received 30 December 2005; Revision accepted 4 January 2006
DOI 10.1002/cci.20672
Published online 29 March 2006 in Wiley InterScience (www.interscience.
wiley.com).

' 2006 Wiley-Liss, Inc.

680 Alwi
stenting and closure of atrial septal defect (ASD)/patent
foramen ovale (PFO) are gaining wider acceptance as al-
ternative or to complement conventional surgery in the
armamentarium of treatment modalities for PAIVS [10–
16]. Given the wide anatomic spectrum of the disease,
ranging from mild severe RV hypoplasia with an ab-
sent infundibulum and the various permutations of as-
sociated abnormalities, a uniform management strategy
to be applied to all patients could not be recommended.
Such an approach would result in higher morbidity and
mortality as one particular mode of treatment may be
inappropriate or even deleterious for a specific subset of
patients, e.g. transcatheter valvotomy in those with se-
verely hypoplastic RV and diminutive infundibulum [17].
Treatment strategies recommended by various authors
have mainly hinged on the morphology of the RV and
pulmonary valve–infundibulum complex [3,18–20]. That
the disease is uncommon yet presents with a remarkable
morphologic heterogeneity, and that newer modes of
transcatheter therapy are increasingly being applied, the
foregoing is an outline of our management algorithm pre-
ceeded by an initial discussion on morphologic evaluation
and classification.
MORPHOLOGIC CLASSIFICATION
Patients with PAIVS can be classified into 3 groups
upon which management strategies can be based). Patients
in Group A are those with valvar or ‘‘membranous’’
atresia where the infundibulum is often well-developed
and the RV hypoplasia usually mild. In Group C are
those at the other end of the morphologic spectrum
where the RV is severely hypoplastic and the infundi-
bulum is often atretic if not diminutive and severely
muscle bound. The intermediate Group B are those in
whom the RV hypoplasia is moderately severe, often with
the trabecular component markedly attenuated but with
nevertheless reasonably developed infundibulum (this
classification is further elaborated in the following sec-
tion). It is thus essential that every patient presenting
with this diagnosis undergo a detailed evaluation for
the purpose of morphologic classification. Excluded
from this scheme are the rare patients with markedly
dilated and thin walled RV, dysplastic or Ebsteinoid,
and severely regurgitant TV. These are also the patients
who have the poorest prognosis [21].
Echocardiography is the cornerstone of this detailed
diagnosis but angiography is also essential for a com-
plete evaluation.v
Echocardiography
The TV diameter z-score on 2D echo is a fairly
objective measure of RV cavity size [3]. The ratio of
tricuspid to mitral valve diameters is also useful used
as it provides an immediate comparison between the
RV and LV sizes and gives an idea of the degree of
RV hypoplasia [22]. The RV inlet length z-score (TV
annulus to apex at ventricular end-diastole) and RV
area z-score at end-diastole with the maximal area bor-
dered by RV endocardium (all measurements in the
apical 4-chamber view) are additional measurements
that may be useful in defining the degree of RV hypo-
plasia [1,13]. Less objective but a more practical eval-
uation is the descriptive designation of the RV as tri-
partite (mild RV hypoplasia), which usually corre-
sponds to Group A, bipartite (moderate hypoplasia)
where the trabecular component is absent or markedly
attenuated, usually corresponds to Group B, and uni-
partite (severe hypoplasia) where the trabecular and
infundibular (outlet) components of RV are absent,
usually corresponds to Group C [23].
Following the earlier mentioned, 2D echo examina-
tion is directed to the pulmonary valve, annulus, and
infundibulum (subvalvar). This is to determine whether
the atresia is at valvar level (‘‘membranous’’ atresia)
where the infundibulum is well-developed (Group A)
or obliterated (‘‘muscular’’ atresia) or virtually so
(Group C). The former is amenable to RF valvotomy,
whereas in the latter RV decompression by catheter
technique would not be feasible. Cases where the atre-
sia is membranous but with concomitant severe hyper-
trophy of the subvalvar musculature giving rise to a
narrow infundibulum may also be diagnosed by 2D
echo. Angiography provides further detailed differen-
tiation of the valvar and subvalvar areas, especially
those often belonging to the intermediate RV morphol-
ogy (Group B) where the atresia is at valvar level
(membranous) and the infundibulum is reasonably well
developed but may be smaller than that in Group A
patients due to more marked muscular overgrowth.
The severity of tricuspid regurgitation and estimation
of RV systolic pressure are next determined by color
and CW Doppler. Major (>4 mm) RV–coronary arte-
rial connections can be readily seen by 2D and color
Doppler echo but minor ones are not.
Finally, branch pulmonary artery size and confluence
is assessed. More importantly description of the PDA
morphology (origin from the aorta, tortuosity, shape,
length, and its narrowest diameter) should be noted
for purposes of PDA stenting if this forms part of the
management.
Angiography
Although echocardiography provides a wealth of
information on the anatomy of PAIVS, angiography is
equally essential in the formulation of a management
strategy as the accurate measurements and assessment of

Fig. 1. (a) Antero-posterior projection of RV angiogram showing membranous atresia, well
developed infundibulum and trabecular component, and mild–moderate RV hypoplasia. Mod-
erate tricuspid regurgitation. No major or minor sinusoids. (Example of Group A). (b) 1 year
post RF valvotomy, persistent mild RV hypoplasia. Complete biventricular circulation and
unobstructed RVOT.
the nature of pulmonary atresia, the infundibulum, the
pulmonary valve annulus, and the PDA morphology,
size, and length can only be obtained by this invasive
technique. Although major RV–coronary arterial com-
munications can be readily seen by echocardiography, its
detailed course, number, and size can only be determined
by angiography. Major connections are defined as promi-
nent filling from RV angiogram of one or more, usually
dilated/ectatic, coronary arteries associated with retro-
grade filling of the aorta. Minor connections are slight
filling of non-dilated coronary arteries [1]. Significant
stenoses or obliteration of major RV-coronary arterial
connections (giving rise to RV-dependent coronary cir-
culation) and the presence of minor connections need to
be diligently sought and documented on angiography.
RV angiography generally correlates with echocardiog-
raphy in the assessment of RV size and estimation of RV
systolic pressure.
MORPHOLOGIC CLASSIFICATION AND
MANAGEMENT STRATEGIES
Group A (good RV size) are those with valvar or
‘‘membranous’’ atresia where the infundibulum is often
well-developed and the RV hypoplasia is usually mild
(TV z score > 2.5). These patients usually have no
major sinusoids. Varying degrees of tricuspid regurgi-
tation is often present (Fig. 1). This group of patients
generally respond well to RF valvotomy and balloon
dilatation (RFV) alone. The long-term outlook is excel-
lent with adequate RV decompression. Normal or near
normal RV growth can be expected and further inter-
Pulmonary Atresia 681
ventions are generally not required. However, resteno-
sis of the pulmonary valve may occur, requiring repeat
balloon dilatation. Occasionally, progressive fixed sub-
valvar stenosis may require RVOT reconstruction or
progressive tricuspid regurgitation may require tricus-
pid valve repair (Fig. 5). Uncommonly, despite seem-
ingly good RV size and good results of RV decom-
pression, the patient remains deeply cyanosed because
of persistent poor compliance of the RV. They are
likely to require prolonged Prostaglandin E2 (PGE2)
infusion for as long as a few weeks and adjunct pro-
cedure to augment the pulmonary blood flow such as
systemic-pulmonary shunt or PDA stenting to tide over
this period to enable the patient to be weaned off
PGE2 and be discharged home [11,24]. At the other
end of the spectrum (Group C) are patients with the
most severe form of disease where the RV is severely
hypoplastic (TV z score < 5.0), and the infundibulum
is obliterated, ‘‘muscular’’ atresia (Fig. 2). Not uncom-
monly, at angiography the infundibulum may in fact
be shown to be not completely atretic, although the
slit-like tract that constitutes the ‘‘RVOT’’ will not be
functional because of the severe overall RV hypoplasia
and heavily muscle-bound ‘‘outflow’’ tract. In this
group of patients, the presence of major RV-coronary
arterial connections with or without stenoses/interrup-
tions are more common. The TV is generally compe-
tent and the RV systolic pressure often suprasystemic.
This is essentially a form of univentricular heart where
clearly these patients need to be placed on the Fontan
tract once the detailed diagnosis is established. As they
have a duct dependent pulmonary circulation, a sys-
682 Alwi

Fig. 2. (a) and (b) Antero-posterior and lateral projections of RV angiogram showing severely
hypoplastic RV, absent infundibulum (muscular atresia), major coronary arterial connections
from RCA and LCA without stenoses or interruption. Contrast rapidly filling aortic root from
RV. (Example of Group C) (RCA, right coronary artery. LCA, left coronary artery).

Fig. 3. (a) and (b) Antero-posterior projection of RV angiograms showing membranous atre-
sia with fairly well developed infundibulum, small pulmonary valve annulus. Moderate–severe
RV hypoplasia with absent or markedly attenuated trabecular component. Major RV-coronary
arterial connection from RCA with stenosis (white arrow) (b) and numerous minor connec-
tions (a and b). Intermediate or Group B (RCA, right coronary artery).

temic-pulmonary shunt is required as first stage pallia-
tion in the neonatal period. The less invasive PDA
stenting is an attractive alternative to the conventional
modified Blalock Taussig (BT) shunt, and this is our
preferred mode of first stage palliation (Fig. 4). Bal-
loon atrial septostomy is also performed at the same
time. We observe that the problems of haemodynamic
instability especially in patients with major RV-coronary
sinusoidal communications or overshunting are encoun-
tered much less commonly with PDA stenting com-
pared to conventional BT shunt, leading to reduced
post procedure morbidity, ICU and hospital stay [25].
However, PDA stenting provides a less durable pallia-
tion because of rapid neo-intimal proliferation, necessi-
tating bidirectional Glenn shunt (first stage Fontan) to
be done as early as 4–6 months. Otherwise this may
be performed at 12–18 months and the completion of
the Fontan procedure a year later. Mild proximal left
 Pulmonary Atresia 683

Fig. 4. (a) Aortogram in lateral projection showing elongated and curved PDA in PAIVS with
severely hypoplastic RV (Group C). (b) 4.0 mm diameter, 16 mm long coronary stent implanted
via femoral artery as first stage palliation as alternative to systemic-pulmonary shunt. Balloon
atrial septostomy was performed at the same time. The patient underwent creation of bidir-
ectional Glenn’s anastomosis 6 months later.

pulmonary artery (LPA) stenosis may occur in some
patients after PDA stenting and this may require aug-
mentation at the time of the Glenn shunt. The long-
term outcome of this group of patients is expected to
be not dissimilar to patients with tricuspid atresia
where the main ventricle is the LV but the presence
of major RV-coronary arterial connections especially
those with interruptions or stenoses would be a major
negative prognostic factor (Fig. 5).
Patients classified into the ‘‘intermediate’’ or Group
B (Fig. 3) are those with borderline RV size (TV z
score between 2.5 and 4.5). Although the atresia is
generally of the valvar or membranous type, the infun-
dibulum or outlet component may be smaller than
those in Group A due to muscular overgrowth. Not
uncommonly this is associated with small pulmonary
valve annulus and discreet subvalvar muscular over-
growth, giving rise to a fixed RVOT obstruction and
suboptimal RV decompression after a successful RFV.
Unlike patients in Group C, major RV-coronary arte-
rial connections are less common, but minor sinusoids
are. As in Group A, varying degree of tricuspid regur-
gitation is expected.
In this group of patients, RFV is recommended as
the principal mode of treatment concomitant with PDA
stenting as the small RV is unlikely to be adequate to
take the load of the right heart circulation independ-
ently even after adequate decompression of the RV,
leading to persistent hypoxemia due to continued right
to left shunting at the atrial level. In addition, the pres-
ence of fixed RVOT obstruction due to small pulmonary
valve annulus or discreet subvalvar muscular overgrowth
causing suboptimal RV decompression is also a cause
of persistent hypoxemia and haemodynamic instability
after RFV. Concomittant PDA stenting at the time of
RFV avoids the need for prolonged PGE2 therapy or
the emergent construction of a BT shunt. Balloon atrial
septostomy may be helpful in reducing venous conges-
tion.
Following the combined initial intervention of RFV,
PDA stenting and balloon atrial septostomy, the pa-
tients are usually pink or mildly cyanosed and the haemo-
dynamics stable, allowing discharge from hospital within
a few days.
But this is also the group of patients where re-inter-
ventions are common, in the form of a bidirectional
Glenn shunt in cases where the RV fails to grow
adequately (1½ ventricle repair) or RVOT reconstruc-
tion in those with fixed obstruction from subvalvar
muscular overgrowth or small pulmonary annulus
[10,20,21,26]. Both the bidirectional Glenn shunt and
RVOT reconstruction may be required where the RV
remains small and fixed RVOT obstruction is also
present.
In those without fixed RVOT obstruction, it is rea-
sonable to wait 3–4 years in anticipation of RV growth
before deciding that a bidirectional Glenn shunt and
closure of PFO/ASD (1½ ventricle repair) be per-
formed. Some patients attain reasonable RV growth
but remain mildly cyanosed. Complete biventricular
circulation can be affected by device closure of the
ASD/PFO (Fig. 5).
684 Alwi

Fig. 5. Morphologic classification and treatment strategies. PAIVS with markedly dilated RV,
RVOT, and RA with dysplastic/Ebstein’s malformation of the tricuspid valve and severe tricus-
pid regurgitation is a rare morphologic subtype which is excluded from this classification
scheme.

Figure 6 summarizes our experience in the manage-
ment of 23 patients who belonged to the intermediate
group from January 2001 to June 2005. There was one
early death from low output syndrome following the
procedure. Of the 22 survivors, seven were on follow
up less than 1 year. Four had 1½ ventricle circulation,
receiving bidirectional Glenn shunt 3–4 years after the
primary procedure. The remaining 11 patients had com-
plete bi-ventricular circulation, nine of whom without
further interventions. One patient underwent RVOT re-
construction for fixed subvalvar obstruction, and the
other underwent PDA restenting and repeat balloon dil-
atation followed by RVOT reconstruction because of
small pulmonary annulus.
CONCLUSION
PAIVS is a disease with remarkable morphologic
variability particularly affecting the TV, RV, and pul-
monary valve and infundibulum. RV-coronary arterial
connections is a peculiar feature of this disease, mainly
seen in those with severe RV hypoplasia. Such tremen-
dous diversity makes prescription of a preferred surgi-
cal or catheter intervention as standard therapy unten-

Fig. 6. Summary of outcome of patients in the intermediate group following RFV as initial
primary treatment.
able. Excluding those rare cases with markedly dilated
RV, dysplastic TV and severe TR, we propose a mor-
phologic classification based on the size of the RV, the
nature of the atresia, the infundibulum, and the pulmo-
nary annulus with data derived from echocardiography
and angiography. Treatment strategies especially the
initial intervention at presentation are tailored to these
morphologic subgroups. Patients with the most favor-
able anatomy where the RV is mildly hypoplastic, the
atresia is valvar (membranous), and the infundibulum
is well developed, RFV alone is recommended and
excellent long-term outcome with complete biventricu-
lar circulation can be expected. At the extreme end of
the spectrum where the RV is severely hypoplastic
(TV z score < 5.0) and the atresia is muscular, often
associated with major RV-coronary arterial connec-
tions, the definitive treatment is the Fontan’s type of
operation, preferably staged, with PDA stenting and
balloon atrial septostomy as the recommended inter-
vention in the neonatal period.
The intermediate subgroup, those with moderate RV
hypoplasia characterized usually by an attenuated tra-
becular component, with valvar atresia but may be
small infundibulum and pulmonary annulus, RFV is of-
ten feasible. However, the RV size and compliance
with or without the presence of residual fixed obstruc-
tion at the subvalve or annulus level may cause persis-
tent severe cyanosis and haemodynamic instability.
The recommended primary treatment strategy at pre-
sentation is RFV and PDA stenting. Some of these
patients are destined for 1½ ventricle repair. Bidirec-
Pulmonary Atresia 685
tional Glenn shunt and closure of ASD/PFO may be
required if the RV fails to grow and the patient
remains cyanosed. Those where the RV is felt to have
grown sufficiently and the cyanosis is mild, the ASD/
PFO can be closed by a device, in the catheter labora-
tory. Fixed obstruction at subvalvar or annulus level
require RVOT reconstruction, but this may be delayed
to 6 months and beyond when the risk of low cardiac
output following surgery is lower.
PAIVS is a disease that continues to be of interest
to morphologists, pediatric cardiologists, and pediatric
cardiac surgeons. We recognize the limitations of mor-
phologic classification based on the earlier mentioned
diagnostic tools, given the continuum within the spec-
trum with which patients may present, such that the
assignment into either Group A or Group B may be
difficult in some. Nevertheless, our proposed guideline
is meant to be a practical approach to management,
which is within the capability of most pediatric cardiac
centers. With development in interventional techniques
and advances in congenital heart surgery, it is likely
that the management of PAIVS will continue to
evolve. This is a disease where cardiologists and car-
diac surgeons will continue to complement one anoth-
er’s role.
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