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1. Anatomic Variability and Outcome in Prenatally
Diagnosed Absent Pulmonary Valve Syndrome
Anita Szwast, MD, Zhiyun Tian, MD, Margaret McCann, RDCS, Debbra Soffer, RDCS,
Jill Combs, RN, MSN, Denise Donaghue, RN, MSN, and Jack Rychik, MD
The Fetal Heart Program at the Cardiac Center at the Children’s Hospital of Philadelphia; and The University of Pennsylvania School of
Medicine, Department of Pediatrics, Division of Cardiology, Philadelphia, Pennsylvania
Background. We sought to describe current outcomes
and risk factors for mortality for fetuses diagnosed with
absent pulmonary valve syndrome (APV). Fetuses with
APV were divided into two cohorts, those with underly-
ing tetralogy of Fallot (TOF/APV) and those without un-
derlying TOF and either an intact ventricular septum or
small ventricular septal defect (APV/IVS).
Methods. The fetal echocardiographic database was
reviewed from January 1, 2001, until June 1, 2010, and all
subjects with a diagnosis of APV were included. Multiple
clinical and fetal echocardiographic measurements were
recorded. Statistical analysis was performed by c2
anal-
ysis and t tests. Survival analysis was performed by
Kaplan-Meier analysis. Significant relationships between
variables were explored by regression analysis. Signifi-
cance was set at p [ 0.05.
Results. The cohort consisted of 15 fetuses with
TOF/APV and 6 fetuses with APV/IVS. There were no
fetal demises in either cohort. Survival to birth was 71% in
the TOF/APV cohort and 83% in the APV/IVS cohort
(p [ 0.62). Of subjects born alive, survival was 80% for
both cohorts (p [ 0.95). However, in the APV/IVS cohort,
transplantation-free survival was only 20%. Underlying
single-ventricle physiology strongly predicted those who
underwent heart transplantation (p [ 0.003, R2
[ 0.50). For
the entire APV cohort, left ventricular dysfunction
(p [ 0.005, R2
[ 0.41) and a higher pulmonary artery valve–
to–aortic valve ratio(p[0.02,R2
[0.34)predictedmortality.
Conclusions. Postnatal outcomes continue to improve
for fetuses with APV syndrome. Left ventricular
dysfunction and higher pulmonary artery valve–to–aortic
valve ratio accurately predict postnatal mortality for fe-
tuses with APV.
(Ann Thorac Surg 2014;98:152–8)
Ó 2014 by The Society of Thoracic Surgeons
Congenital absence of the pulmonic valve (APV) is a
rare congenital heart defect and is usually associated
with tetralogy of Fallot (TOF, TOF/APV), constituting
between 3% and 6% of all postnatal cases of TOF [1]. In
TOF, there is anterior malalignment of the conal septum,
leading to subpulmonary obstruction. However, the right
ventricle and tricuspid valve are typically of normal size.
In TOF/APV syndrome, the echocardiographic hallmarks
include severe pulmonary insufficiency, right ventricular
dilation, and marked branch pulmonary artery dilation
[2–4]. The potential for respiratory failure at birth is high.
Previous case series have described the prenatal diagnosis
and subsequent outcomes in fetuses with TOF/APV leaf-
lets [1, 5–9]. Termination rates range from 30% to 43%, and
the 1-year mortality rates range from 67% to 75% [1, 5–9].
The perinatal risk factors for mortality include (1) res-
piratory distress at birth, (2) presence of a genetic syn-
drome or abnormal karyotype [5, 7–9], and (3) presence of
hydrops fetalis [10, 11]. More recent series of TOF/APV
have reported better rates of survival, ranging from 72%
to 86% [12–13]. This may be due to better anticipatory
planning at birth after prenatal diagnosis and to
improved surgical strategies for repair.
As prenatal ultrasound screening improves, an inter-
esting and relatively uncommon anomaly is being
identified with increasing frequency. Absence of the
pulmonary valve leaflets can be seen without TOF, in the
presence of an intact ventricular septum (IVS) or a small
hemodynamically insignificant muscular ventricular
septal defect (VSD). APV without TOF can occur as
“isolated APV” with a biventricular physiology in which
there is a normal tricuspid annulus and a normal or
dilated right ventricle. Alternatively, it can occur with
single-ventricle physiology with underlying tricuspid
atresia or stenosis and right ventricular hypoplasia. In
most fetal case series of APV, subjects without underlying
TOF constitute only 10% to 25% of the study cohort, with
relatively high termination rates [6, 7, 9, 13, 14]. Conse-
quently, it is difficult to draw any conclusions regarding
their outcomes. The purpose of this study was to describe
the current outcomes in fetuses with diagnoses of APV
syndrome, either associated with TOF (TOF/APV) or
the rare form not associated with TOF with an intact
ventricular septum or small VSD (APV/IVS).
Patients and Methods
The database at the Fetal Heart Program at the Children’s
Hospital of Philadelphia was reviewed, and all subjects
with a diagnosis of APV from January 1, 2001, until June 1,
2010, were included. Institutional review board approval
Accepted for publication March 4, 2014.
Address correspondence to Dr Szwast, Division of Cardiology, 8th Flr
Main Hospital, Children’s Hospital of Philadelphia, 34th and Civic Center
Blvd, Philadelphia, PA 19104; e-mail: szwast@email.chop.edu.
Ó 2014 by The Society of Thoracic Surgeons 0003-4975/$36.00
Published by Elsevier Inc http://dx.doi.org/10.1016/j.athoracsur.2014.03.002
CONGENITALHEART

2. was obtained (IRB#10-7504). All fetal echocardiograms
were performed on a Siemens Acuson Sequoia Ultra-
sound system (Mountain View, CA) coupled with a 6C2
or 8V3 transducer. Studies performed after January 2004
had images available for review. Images were acquired
in a digital format and stored on a KinetDx (Siemens,
Malvern, PA) server. Multiple tomographic views of
the fetal heart were obtained according to American
Society of Echocardiography guidelines [15]. The last
comprehensive fetal echocardiogram before delivery was
analyzed.
For each subject, the following clinical indicators were
recorded: prenatal diagnosis, pregnancy outcome, genetic
testing results, family history of congenital heart disease,
and extracardiac abnormalities. For subjects born alive,
postnatal diagnosis, Apgar scores, birth weight, need for
surgical intervention within the first hospitalization, sur-
gical palliations, days within the intensive care unit dur-
ing the first hospitalization, total days of hospitalization,
and days of follow-up were recorded. Surgical palliation
type—single ventricle versus biventricular—mortality,
and listing for heart transplantation were recorded. Res-
piratory distress was assessed by the use of supplemental
oxygen at birth, mechanical ventilation at birth, subse-
quent tracheostomy, or a combination of these factors.
To determine whether fetal echocardiographic mea-
surements were predictive of postnatal outcome, the fetal
echocardiographic measurements listed in Table 1 were
recorded. The z scores for the main, left, and right pul-
monary arteries were generated by use of a previously
published nomogram for fetal pulmonary artery di-
ameters [16]. The presence of tricuspid stenosis or atresia
was noted. For the purposes of analysis, all subjects with
either an intact ventricular septum or small, hemody-
namically insignificant VSD were considered in the APV/
IVS cohort.
Statistical analysis was performed by independent
sample t tests for continuous variables and the c2
analysis
for nonparametric variables with IBM SPSS, version 18
(IBM, Chicago, IL). The results were expressed as
means and standard deviations, medians and ranges, or
frequencies. Survival analysis was performed by Kaplan-
Meier analysis. Significant relationships between vari-
ables were explored by regression analysis. Significance
was set at p 0.05.
Results
Study Population
During the study period, 14,612 fetal echocardiograms
were performed, and 1,479 cases of congenital heart dis-
ease were identified. There were 21 subjects with APV,
constituting 1.4% of all cases of congenital heart disease.
The study population comprised 15 fetuses with TOF/
APV (71%) and 6 fetuses with APV/IVS (29%), as shown in
Table 2 and Figure 1. In the TOF/APV cohort, additional
cardiac anomalies were noted in 5 subjects. In the APV/
IVS cohort, there were 4 subjects with tricuspid atresia,
including 1 with a small muscular VSD and complete
heart block, 1 with tricuspid stenosis and severe biven-
tricular dysfunction, and 1 with severe tricuspid regur-
gitation and a normal tricuspid valve annulus, “isolated
APV.”
Survival Outcomes
Table 2 summarizes the subject outcomes, surgical palli-
ations, and current outcomes. As shown in Table 2 and
Figure 1, there were four terminations of pregnancy (27%)
in the TOF/APV group and 1 termination (17%) in the
APV/IVS group (p ¼ 0.63). There were no fetal deaths in
either cohort. Hydrops fetalis occurred in no fetuses with
TOF/APV but did occur in 2 of 6 fetuses with APV/IVS
(p ¼ 0.02). One subject in the TOF/APV group received
postnatal care at another institution and was therefore
lost to follow-up. Consequently, survival to birth was 10
of 14 in the TOF/APV cohort and 5 of 6 in the APV/IVS
cohort (p ¼ 0.45). The cumulative survival was 8 of 14 for
the TOF/APV cohort and 4 of 6 for the APV/IVS cohort
(p ¼ 0.69). Figure 2 illustrates the current survival for
subjects born alive: 8 of 10 for the TOF/APV cohort and 4
of 5 for the APV/IVS cohort. However, in the APV/IVS
Table 1. Prenatal Echocardiographic Variables for the TOF/APV Cohort and the APV/IVS Cohort
Variables TOF/APV APV/IVS p Value
Cardiothoracic area ratio 0.44 Æ 0.44 (n ¼ 12) 0.54 Æ 0.07 (n ¼ 6) 0.039
Pulmonary artery valve / aortic valve annular ratio 0.95 Æ 0.27 (n ¼ 12) 0.65 Æ 0.15 (n ¼ 6) 0.008
MPA z score 8.82 Æ 6.02 (n ¼ 12) 3.09 Æ 4.42 (n ¼ 5) 0.053
LPA z score 7.25 Æ 7.45 (n ¼ 13) 0.43 Æ 1.84 (n ¼ 5) 0.008
RPA z score 7.38 Æ 5.44 (n ¼ 12) 1.84 Æ 2.84 (n ¼ 5) 0.016
Severe LV dysfunction 2/15 2 /6 0.29
Severe RV dysfunction 3/15 6/6 0.001
LV enlargement 6/14 3/6 0.77
RV enlargement 12/14 2/6 0.019
Patent ductus arteriosus 2/15 6/6 <0.001
Hydrops fetalis 0/15 2/6 0.019
APV ¼ absent pulmonary valve; IVS ¼ intact ventricular septum or small ventricular septal defect; LPA ¼ left pulmonary artery; LV ¼ left
ventricle; MPA ¼ main pulmonary artery; RPA ¼ right pulmonary artery; RV ¼ right ventricle; TOF ¼ tetralogy of Fallot.
153Ann Thorac Surg SZWAST ET AL
2014;98:152–8 ABSENT PULMONARY VALVE SYNDROME OUTCOMES
CONGENITALHEART

3. Table 2. Cardiac Diagnoses, Outcome of Pregnancy, Surgical Palliations, and Current Follow-Up
Cardiac Diagnosis Outcome of Pregnancy Surgical Palliations Follow-up
1 TOF/APV Term birth at 39 weeks Complete repair with VSD closure, valved RV to PA conduit, and MPA
plication at 2 days
Alive at 10 years
2 TOF/APV Term birth at 39 weeks 1. Complete repair with VSD closure, valved RV to PA conduit using aortic
homograft, and MPA plication at 3 days
2. Tracheostomy at 1 month
Alive at 10 years
3 TOF/APV Termination Termination at 22 weeks
4 TOF/APV Term birth at 38 weeks Complete repair with VSD patch closure, homograft patch augmentation of
RVOT in a transannular fashion, partial resection of dysplastic leaflet,
resection of small muscle bundle in RVOT, partial ASD closure at 3.5
months
Lost to follow-up at 3.5
months
5 TOF/APV, Discontinuous LPA Term birth at 40 weeks Complete repair with VSD closure, unifocalization of LPA, RVOT
reconstruction with a 14-mm pulmonary homograft, PDA ligation at 5 days
Alive at 7 years
6 APV/IVS, Tricuspid Stenosis,
Severe Biventricular
Dysfunction
Preterm birth
at 36 weeks
None Neonatal death at 1 day of age
7 TOF/APV, AP window, apical
muscular VSD, PAPVR
Term birth at 38 weeks 1. AP window repair with division of AP window, primary closure of aorta,
patch closure of PA, plication of ascending aorta and MPA, PA banding at 7
days
2. Tracheostomy at 2 weeks
3. PA band takedown partial ASD closure, RV-PA conduit placement using a
14-mm aortic homograft at 22 months
4. PAPVR repair with modified Waarden procedure, ASD closure, creation of
a 4-mm atrial communication, placement of RV-to-PA conduit with a 20-
mm Hemashield graft, pulmonary valve replacement with a 19-mm
pericardial valve, anterior plication of RPA at 46 months
Alive at 5.9 years
8 TOF/APV Termination Termination at 19 weeks
9 TOF/APV, severe biventricular
dysfunction
Preterm birth at
36 weeks
None Neonatal death at 22 days of
age
10 TOF/APV Lost to follow-up at 29 weeks
gestation
11 TOF/APV, severe biventricular
dysfunction
Termination Termination at 20 weeks
12 TOF/APV Term birth at 39 weeks Complete repair with MPA plication, Dacron patch closure of VSD and
reconstruction of RVOT with a monocusp valve at 2 months
Alive at 3.5 years
13 TOF/APV Termination Termination at 19 weeks
14 APV/IVS, Tricuspid Atresia Term birth at 39 weeks Heart transplantation at 22 days Alive at 6 years
15 TOF/APV Term birth at 37 weeks Complete repair with plication of MPA, VSD closure, and placement of a
monocusp RVOT patch at 2 months
Alive at 3 years
16 APV/IVS, Severe tricuspid
regurgitation, LV
noncompaction, hydrops
fetalis
Term birth at 37 weeks PDA ligation and division and RVOT reconstruction with transannular patch
and monocusp valve at 15 days
Alive at 3 years
17 APV/IVS, Tricuspid Atresia Term birth at 37 weeks 1. 4-mm right modified BT shunt and division of PDA at 14 days
2. Heart transplantation at 5 months
Alive at 1 year
(Continued)
154SZWASTETALAnnThoracSurg
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CONGENITALHEART

4. cohort, heart transplantation–free survival was only 20%
(p ¼ 0.02), as shown in Figure 3.
Postnatal Clinical Course
There were no significant differences in birth weight,
1-minute or 5-minute Apgar scores, supplemental oxy-
gen, intubation at birth, intubation days, tracheostomy,
days in intenstive care, total hospitalization days during
the initial postnatal hospitalization, surgical intervention
during the initial hospitalization, and postnatal survival
between the TOF/APV and APV/IVS cohorts. However,
3 of 5 subjects with APV/IVS and single-ventricle physi-
ology underwent heart transplantation for left ventricular
dysfunction compared with none in the TOF/APV series
(p ¼ 0.02). By contrast, one subject with biventricular
physiology underwent ligation of the ductus arteriosus
and placement of a monocuspid pulmonary valve at 15
days of age. By logistic regression analysis, the presence
of single-ventricle physiology in the APV/IVS cohort
strongly predicted the need for heart transplantation
(p ¼ 0.003, R2
¼ 0.50).
Genetics
Genetic information was available in 18 of 21 subjects,
including amniocentesis results in 8 of 15 with TOF/APV
and 5 of 6 with APV/IVS. Genetic syndromes were
identified in 3 subjects with TOF/APV (Alagille’s syn-
drome, chromosome 1p3–6 microdeletion, and chromo-
some 4p1–5 microdeletion). Subject 10 had a normal
amniocentesis result yet had multiple congenital anom-
alies identified on prenatal imaging. There were no un-
derlying genetic syndromes identified in the APV/IVS
cohort, although subject 14 had abnormalities detected on
prenatal imaging. Unlike previous series, in this cohort,
an identified genetic syndrome was not associated with
death (p ¼ 0.89), although no subjects had 22q11 deletion
syndrome. However, in three cases there was a family
history of congenital heart disease. The first family had a
fetus with TOF/APV and a prior child with TOF. The
second family had a fetus with tricuspid atresia with
APV/IVS and a previous child with atrial septal defect.
Finally, a third family had two pregnancies involving
tricuspid stenosis and APV/IVS.
Prenatal Echocardiographic Data for the APV Cohorts
Table 1 demonstrates the comparison of fetal echocar-
diographic variables for the APV/IVS and TOF/APV
cohorts. The median fetal echocardiographic measures
were made at 34 weeks 6 days (range, 17 weeks 2 days to
38 weeks 0 days). By regression analysis, the pulmonary
artery valve–to–aortic valve annular ratio, MPA, LPA, and
RPA z scores did not predict any of the indicators of
respiratory distress in the TOF/APV cohort.
Predictors of Survival in Each Cohort
Table 3 demonstrates the echocardiographic and clinical
differences between survivors and nonsurvivors in the
TOF/APV cohort. In the TOF/APVcohort, a higher pul-
monary artery valve–to–aortic valve annular ratio and
severe left ventricular dysfunction were more common in
Table2.Continued
CardiacDiagnosisOutcomeofPregnancySurgicalPalliationsFollow-up
18TOF/APV,APcollateralsPretermbirthat34
weeks
1.CompleterepairwithDacronpatchclosureofVSDandtransannular
homograftpatchaugmentationofRVOTat3months
2.Tracheostomyat5months
Deathat9months
19TOF/APVTermbirthat38weeksCompleterepairwithVSDclosure,plicationofpulmonaryarteriesand
relocationofthepulmonaryarteriesanteriortoaortawithaLecompte
maneuver,placementofa15-mmpulmonaryhomograftat1month
Aliveat1.4years
20APV/IVS,TricuspidAtresia,
hydropsfetalis
TerminationTerminationat17weeks
21APV/IVS,TricuspidAtresia,
smallmuscularVSD,complete
heartblock
Termbirthat37weeks1.Pacemakerplacementondayofbirth
2.Centralshunt,PDAligation,andMPAligationat28days
3.Tracheostomyat2months
4.BidirectionalGlennat7months
5.Hearttransplantationat3.8years
Aliveat4years
AP¼aortopulmonary;APV¼absentpulmonaryvalve;ASD¼atrialseptaldefect;BT¼Blalock-Taussig;IVS¼intactventricularseptumorsmallventricularseptaldefect;LPA¼left
pulmonaryartery;LV¼leftventricle;MPA¼mainpulmonaryartery;PA¼pulmonaryartery;PAPVR¼partialanomalouspulmonaryvenousreturn;PDA¼patentductus
arteriosus;RV¼rightventricle;RVOT¼rightventricularoutflowtract;TOF¼tetralogyofFallot;VSD¼ventricularseptaldefect.
155Ann Thorac Surg SZWAST ET AL
2014;98:152–8 ABSENT PULMONARY VALVE SYNDROME OUTCOMES
CONGENITALHEART

5. nonsurvivors than in survivors. By logistic regression
analysis, a higher pulmonary artery valve–to–aortic valve
annular ratio predicted mortality (p ¼ 0.041, R2
¼ 0.41),
with a trend toward significance for left ventricular
dysfunction (p ¼ 0.053, R2
¼ 0.31). Conversely, in the
APV/IVS cohort, only severe left ventricular dysfunction
was more common in nonsurvivors compared with sur-
vivors (p ¼ 0.025) and was also predictive of mortality by
logistic regression analysis (p ¼ 0.025, R2
¼ 0.63).
Comment
This study highlights the unique differences between
fetuses with TOF/APV and those with the more rare form
of APV/IVS. All subjects with APV/IVS had a patent
ductus arteriosus (PDA), compared with only 2 subjects in
the TOF/APV cohort. Branch pulmonary artery size was
markedly discrepant between the groups. The mean
branch pulmonary artery z score was normal for fetuses
with APV/IVS but markedly elevated for fetuses with
TOF/APV. We hypothesize that the presence of the PDA
may be protective in preventing massive dilation of the
proximal branch pulmonary arteries. Finally, subjects
with APV/IVS frequently manifested anatomic abnor-
malities of the tricuspid valve and right ventricular
structure and function.
The outcomes were also markedly discrepant between
cohorts. Excluding terminations of pregnancy, TOF/APV
conferred a better prognosis, with a transplantation-free
postnatal survival of 80%. Our outcomes are similar to
those in more recent case series, wherein survival ranged
from 72% to 86% in cases with active postnatal manage-
ment [12, 13]. However, unlike most previous case series,
in our cohort, there were no subjects with 22q11 deletion
Fig 1. Flow diagram illustrating outcomes
for the TOF/APV and APV/IVS cohorts.
(APV ¼ absent pulmonary valve; IVS ¼
intact ventricular septum or small ventricular
septal defect; TOF ¼ tetralogy of Fallot.)
Fig 2. Kaplan-Meier survival curve for the APV/IVS cohort,
illustrated in blue, and the TOF/APV cohort, illustrated in green.
Subjects at risk at each time point are displayed above the curves.
There is no difference in survival (p ¼ 0.95). (APV ¼ absent
pulmonary valve; IVS ¼ intact ventricular septum or small
ventricular septal defect; TOF ¼ tetralogy of Fallot.)
Fig 3. Kaplan-Meier transplantation-free survival curve for the
APV/IVS cohort, illustrated in blue, and the TOF/APV cohort,
illustrated in green. Subjects at risk at each time point are displayed
above the curves. There is a trend toward a difference in survival
(p ¼ 0.064). (APV ¼ absent pulmonary valve; IVS ¼ intact
ventricular septum or small ventricular septal defect; TOF ¼ tetralogy
of Fallot.)
156 SZWAST ET AL Ann Thorac Surg
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6. syndrome, which is a previously reported risk factor for
poor outcome [4, 7–9]. There may be other genetic causes
of APV [17] , inasmuch as one mother had two preg-
nancies affected by TOF and TOF/APV, yet both cases
were negative for the 22q11 deletion syndrome.
Ventricular dysfunction is an important risk factor for
mortality in the APV syndrome [1, 18]. Severe left ven-
tricular dysfunction was an independent predictor of
death for the APV/IVS cohort (p ¼ 0.025), with a trend
toward significance in the TOF/APV cohort (p ¼ 0.053).
Similarly to a prior study at this institution [19], we found
that a higher pulmonary artery valve–to–aortic valve
annular ratio was predictive of mortality for the TOF/APV
group. We hypothesize that a relatively larger pulmonary
annulus is a marker for increased pulmonary regurgitant
flow, which may lead to greater right ventricular dilation
and dysfunction, compressing the left ventricle and
compromising cardiac output.
Neonates with TOF/APV are at high risk for respiratory
distress at birth. However, we could not define any pre-
natal echocardiographic variables that would accurately
predict respiratory distress at birth or eventual trache-
ostomy. In our series, larger proximal branch pulmonary
arteries, indexed to gestational age by z score, were not
associated with poor outcome or indicators of respiratory
distress at birth. This disputes previous reports that
massive dilation of the pulmonary arteries is directly
related to bronchomalacia and poor outcomes, and it
argues against a linear relationship between size of
proximal branch pulmonary arteries and respiratory dif-
ficulties [3, 20]. Indeed, respiratory distress may be
related to abnormalities of the distal pulmonary vascu-
lature, which are poorly visualized by fetal ultrasound
technology, or to parenchymal lung disease. Magnetic
resonance imaging may prove more useful in the evalu-
ation of these fetuses in the future.
In our APV/IVS study cohort, transplantation-free
survival was only 20%. The presence of single-ventricle
physiology secondary to tricuspid atresia strongly pre-
dicted those who underwent heart transplantation
(p ¼ 0.003). In contrast to other forms of tricuspid atresia,
in tricuspid atresia with APV/IVS, the coronary arteries
may be markedly abnormal [21], which can lead to
ischemic changes, compromising left ventricular function.
In addition, previous investigators have described
marked histologic abnormalities of the right ventricle and
prominent sinusoids in the setting of tricuspid atresia and
APV/IVS [21, 22]. Furthermore, there can be exaggerated
leftward bowing of the ventricular septum, causing left
ventricular outflow tract obstruction and compromising
cardiac output. Subject 6, who died shortly after birth,
had marked obstruction of the left ventricular outflow
and poor biventricular function. Unlike in other case se-
ries [13, 23, 24], none of our subjects with single-ventricle
physiology and APV were deemed satisfactory candidates
for Fontan palliation as a consequence of systemic ven-
tricular dysfunction and consequently were referred
for heart transplantation. In contrast, the infant with a
biventricular physiology, “isolated APV,” received me-
chanical ventilation at birth for cardiorespiratory failure,
which persisted until a large PDA was ligated surgically
and a pulmonary valve was placed. Early ligation of
the PDA has been previously reported in patients with
“isolated APV” and an intact ventricular septum to
prevent ongoing congestive heart failure [25, 26].
Limitations
This study is limited by its relatively small numbers and
retrospective design. Some subjects were lost to follow-
up. Prenatal echocardiographic images were not avail-
able for review in two cases; therefore, data were
collected from the echocardiographic reports.
Table 3. Postnatal Survivors vs Postnatal Nonsurvivors for the TOF/APV Cohort
Variables Survivors Nonsurvivors p Value
Pulmonary artery valve / aortic valve annular ratio 0.82 Æ 0.27 (n ¼ 6) 1.23 Æ 0.11 (n ¼ 2) 0.022
MPA z score 5.37 Æ 4.3 (n ¼ 6) 5.18 Æ 0.23 (n ¼ 2) 0.96
LPA z score 6.22 Æ 5.2 (n ¼ 7) 5.93 Æ 5.18 (n ¼ 2) 0.95
RPA z score 9.30 Æ 6.05 (n ¼ 6) 4.8 Æ 6.42 (n ¼ 2) 0.41
1-min Apgar score 8 (5–9) (n ¼ 6) 3.5 (1–6) (n ¼ 2) 0.14
5-min Apgar score 8.5 (8–9) (n ¼ 6) 4.5 (1–8) (n ¼ 2) 0.14
Palliation during initial hospitalization 4/8 0/2 0.20
Intubation before initial surgical palliation 6/8 1/2 0.49
Supplemental oxygen before palliation 6/8 1/2 0.49
Days of intubation before initial palliation 4 (1–28) (n ¼ 6) 21 (n ¼ 1) 0.22
ICU days before initial palliation 13.5 (3–110) (n ¼ 8) 28.0 (21–35) (n ¼ 2) 0.27
Total hospitalization days 14.5 (6–110) (n ¼ 8) 28.0 (21–35) (n ¼ 2) 0.35
Genetic syndrome 2/8 0/2 0.43
Tracheostomy 2/8 1/2 0.49
Severe LV dysfunction 0/8 1/2 0.035
Severe RV dysfunction 1/8 1/2 0.24
APV ¼ absent pulmonary valve; ICU ¼ intensive care unit; LPA ¼ left pulmonary artery; LV ¼ left ventricle; MPA ¼ main pulmonary
artery; RPA ¼ right pulmonary artery; RV ¼ right ventricle; TOF ¼ tetralogy of Fallot.
157Ann Thorac Surg SZWAST ET AL
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7. Conclusions
Congenital absence of the pulmonary valve can be found
in association with TOF or with an intact ventricular
septum. The outcomes for prenatally diagnosed APV
vary significantly depending on the underlying lesion.
The prognosis for TOF/APV has improved considerably,
with 80% postnatal survival. The risk factors for mor-
tality include a higher pulmonary artery valve–to–aortic
valve annular ratio and severe left ventricular dysfunc-
tion. In APV/IVS with single-ventricle physiology, sig-
nificant ventricular dysfunction secondary to coronary
abnormalities should lead to consideration for heart
transplantation.
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