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\begin{document}

\title{Pulmonary atresia and ventricular septal defect: How accurate is the
fetal echocardiography, and do the major aortopulmonary collateral
arteries matter?}



\author[1]{Lütfiye Uygur}%
\author[1]{Oya Demirci}%
\author[2]{Ilker Kemal Yücel}%
\affil[1]{Zeynep Kamil Kadin ve Cocuk Hastaliklari Egitim ve Arastirma Hastanesi}%
\affil[2]{Istanbul Dr Siyami Ersek Gogus Kalp ve Damar Cerrahisi Egitim ve Arastirma Hastanesi}%


\vspace{-1em}



  
  \date{May 10, 2024}


\begingroup
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\selectlanguage{english}
\begin{abstract}
Objective: To assess the accuracy of prenatal echocardiography in
defining pulmonary vasculature in pulmonary atresia with VSD (PAVSD).
The second aim is to compare the perinatal and postnatal outcomes of
different pulmonary blood supply types. Design:The cases prenatally
diagnosed with PAVSD between January 2017- October 2022 in a single
tertiary fetal medicine center were identified on the electronic
database. Fetal echocardiography reports and images were reviewed
retrospectively. Postnatal outcomes and images were acquired from the
hospital records of relevant pediatric cardiology and cardiovascular
surgery clinics. Fetal echocardiography results were compared with
postnatal results. Perinatal and postnatal outcomes were compared
between the pulmonary vascular supply types. Results: Among the 24 PAVSD
cases, six were diagnosed with major aortopulmonary collateral arteries
(MAPCA) dependent, eleven were diagnosed with ductus arteriosus (DA)
dependent pulmonary vascular supply, and seven were diagnosed with
double pulmonary supply (MAPCA + DA) on prenatal echocardiography.
Seventeen cases were live-born and have undergone postnatal
investigations. Fetal echocardiography was 88.2\% accurate about the
type of pulmonary vascular supply. The accuracy of fetal
echocardiography regarding pulmonary vascular anatomy was 82.3\%. Four
cases were demised before surgical interventions. Postoperative survival
was 69.2\%. Mortality and postoperative survival did not differ between
pulmonary supply groups. Survival was disrupted with extracardiac
anomalies. The need for early surgical interventions was significantly
higher in the DA group. Conclusion:The anatomy of pulmonary
vascularization in PAVSD can be defined precisely on fetal
echocardiography. The source of pulmonary blood supply does not impact
postnatal short-term outcomes significantly; however, it affects the
postnatal management. The associated anomalies highly contribute to
postnatal mortality. Therefore, MAPCAs, the anatomy of the pulmonary
arteries, and accompanying abnormalities should be intensely searched on
fetal ultrasonography.%
\end{abstract}%



\sloppy


\textbf{INTRODUCTION}

PAVSD is a rare congenital cardiac defect with an incidence of 0.4:10000
live births\textsuperscript{1}. It is characterized by a large
perimembranous septal defect with an overriding aorta and an atretic
pulmonary valve leading to the absence of antegrade flow from the right
ventricle to the pulmonary artery (Figure 1). The infundibulum of the
pulmonary artery (PA) is often poorly formed along with the atretic
valve. The pulmonary trunk may be severely hypoplasic; confluent or
non-confluent branches of the pulmonary arteries may present as
hypoplasic vasculature. In this case, their blood supply and size depend
on either DA or the systemic arterial collateral connections, or both
(double supply). The pulmonary trunk may completely be atretic in severe
cases. In this case, the alternate source of pulmonary blood supply is
only major aortopulmonary collateral arteries (MAPCAs). MAPCAs are the
non-regressed embryological connections between pulmonary vasculature
and the aorta or the main branches of the aorta. They usually originate
from the descending aorta and communicate with the branches of the
pulmonary or bronchial arteries. Rarely, they can arise from the
branches of the aortic arch- brachiocephalic trunk, subclavian arteries-
or even from coronary arteries\textsuperscript{2,3}. The patency of DA
and the presence of MAPCAs have important impacts on postnatal
management, survival, and prognosis of the cases with PAVSD. Therefore,
a detailed and accurate description of pulmonary vasculature provides
precise counseling for the parents and directs early postnatal
management.

This study aims to investigate the accuracy of fetal echocardiography in
the vascular anatomic assessment of PAVSD. The second aim is to compare
the postnatal prognosis in different pulmonary vascularization types.

\textbf{METHODS:}

This was a retrospective study conducted in a single tertiary fetal
medicine center. The cases diagnosed with PAVSD between January 2017 and
December 2022 were identified on the electronic database of our fetal
echocardiography clinic. All prenatal echocardiographies were performed
using Voluson E6 (GE Healthcare Ultrasound, Milwaukee, Wisconsin)
equipped with a curved transabdominal probe by the same expert fetal
medicine consultant (OD) in a systematic manner, usually twice a month
for each pregnancy. The cases with additional complex cardiac defects
were not included. Long and short-axis views were used to assess
pulmonary vasculature and comprehensive cardiac anatomy. Axial,
sagittal, and coronal views of the aorta were used to detect MAPCAs. The
vessels which supply blood to the pulmonary parenchyma were also
identified, beginning from the parenchymal tissue and following towards
the systemic origin on color Doppler. Vascular blood flow was evaluated
using high-definition (HD) Doppler, applying a pulse repetition
frequency of 0.3--0.6 kHz. As a routine clinical protocol, an informed
consent was taken from each patient approving the publication of the
ultrasound images and clinical data in case of a clinical research,
provided that their identities and personal information were kept
confidential. The study protocol was approved by the institutional
ethical committee (22.03.2023, No. 46), and it was carried out following
the principles of the Declaration of Helsinki.

Details of ultrasonographic findings such as the presence or absence of
DA, pulmonary arteries, and the confluence of the pulmonary arteries,
the diameters of the right pulmonary artery (RPA) and left pulmonary
artery (LPA), the source of blood supply to the lungs (DA, MAPCAs or
double supply), the origin of the MAPCAs were obtained from the fetal
echocardiography reports and archived sonographic images. The pulmonary
blood supply was assumed as 'ductus arteriosus'' if retrograde flow was
displayed in DA adjacent to the aortic isthmus in 3VT plane or from the
undersurface of the aortic arch, distal to the left subclavian artery
towards the pulmonary artery without any intervening branches in
sagittal plane. The vascular supply was assumed as MAPCAs when
collaterals that fed the pulmonary parenchyma were detected to originate
from the main branches of the aortic arch, coronary arteries, or
descending aorta. Confirmation of the fetal echocardiographies was
sought from neonatal echocardiography and angiography with cardiac
catheterization performed in collaborated pediatric cardiology clinic
for all live-born neonates. Postnatal confirmation of the fetal
echocardiographies of the babies who had undergone surgery was made by
operation reports. TOP cases could not be confirmed due to the families'
refusal of an autopsy. Measures of association for categorical variables
were analyzed with Pearson Chi-square test. All analyses were performed
using SPSS version 22 (IBM Corp., Armonk, NY, USA). The Mann-Whitney U
test was used for comparing the surgical intervention time between
groups. A p-value of \textless{}0.05 was considered statistically
significant.

\textbf{RESULTS:}

We diagnosed 28 cases of PAVSD during five years in our fetal diagnosis
and therapy center. Four were excluded due to lacking postnatal data or
being lost to follow-ups. The mean maternal age was 30 \selectlanguage{ngerman}±5 years (range:
23-49 y). The mean gestational age at diagnosis was 22.4 ± 4.3 weeks
(range: 13-31 w). 45.8\% of the cases were isolated. On prenatal
ultrasound, 37.5\% of the cases (9 cases) were found to have
extracardiac anomalies other than thymus hypoplasia/ aplasia (Table 1).
Genetic studies could be employed for 17 cases. Chromosomal
abnormalities were detected in 41.1\% of these cases. 22q11.2
microdeletion syndrome constituted 71.4\% (5 cases) of the chromosomal
abnormalities (Table 1). All those cases with thymus hypoplasia were
found to have chromosomal abnormalities.

On prenatal ultrasound examinations, the four-chamber view was normal
except for left axis deviation in all patients. Overriding aorta,
hypoplastic or atretic pulmonary arteries, and absent antegrade flow
through the right ventricle outflow tract (RVOT) were the shared
characteristics of the cases. Right aortic arch (RAA) was detected in
five fetuses (20.8\%). Confluent pulmonary arteries were identified in
15 (62.5\%) fetuses when the pulmonary vasculature was assessed. The
Z-score of the dimensions of LPA and RPA were below -2 in all of the
fetuses\textsuperscript{4}. The antegrade ductal flow was seen in none,
and retrograde ductal flow was displayed in 18 (75\%) cases. Six of the
fetuses had MAPCA-dependent pulmonary vascular supply. Seven (30.4\%)
cases had pulmonary vascular supply originating both from the ductus
arteriosus and MAPCAs (double supply), according to the fetal
echocardiographies. (Table 1)

TOP was performed in 29.1\% of the pregnancies (7 cases). Additional
chromosomal or structural anomalies corroborated the decision of TOP in
all of the terminated cases. The remaining 17 fetuses were born alive.
Overall survival was 52.9\% for the live-born fetuses. Two infants were
demised after the early first-stage shunt procedures. Four cases
(including three with mortal extracardiac anomalies) were demised
without surgical interventions. Nine infants survived after the cardiac
operations. Among those, seven had complete cardiac repair, and two have
been scheduled for complete repairment surgery after the palliative
Blalock Taussig (BT) shunt (Figure 2). Follow-up time since the
completion of total repairment is 1-4 years. Two infants were demised
after the cardiac surgeries. 62.5\% of the babies who died before or
after surgery had major extracardiac anomalies.

There was no significant difference between the groups when we evaluated
the postoperative outcomes of our cases within the different pulmonary
blood supply groups based on postnatal diagnoses. However, the median
surgical intervention time was earlier in the DA group than in MAPCA
dependent group (Table 2). The DA-dependent group included eight
live-born babies. Four of these cases had serious extracardiac anomalies
leading to mortality (Table 1). Four cases in this group could undergo
surgery. Postoperative survival was 66.6\% for this group. MAPCA
dependent group had seven live-born babies. Two cases were demised
before surgery, and five infants in this group could undergo total
cardiac repair surgery. Postoperative survival was 60\%. The double
supply group had two cases, both surviving after surgeries. One of them
had a coronary artery-originated collateral, feeding the RPA. She
underwent surgery, which included the closure of the DA and collateral,
unifocalization of central PAs, and a modified BT shunt. She is
currently scheduled for total repair.

All of the live-born fetuses were confirmed to have PAVSD on postnatal
echocardiography. Pulmonary vascular supply type was defined precisely
on fetal echocardiography in 88.2\% of the cases, according to postnatal
echocardiography, angiography, or surgery. The existence or absence of
MAPCA was accurately detected in 100\% of the cases. There were only two
misdiagnosed cases who were considered to have a double supply on fetal
echocardiography, but were found to have only MAPCA-dependent pulmonary
flow in the catheter angiography (Table 3). The sources of MAPCAs were
mainly descending aorta except for one case with the prenatal diagnosis
of coronary artery-originated MAPCA (Figure 3) and two cases with MAPCAs
from both descending aorta and aortic arch (Figure 4). Fetal
echocardiography was 94.1\%, 82.3\%, and 88.2\% accurate in defining the
confluence of the PAs, RPA/LPA, and main pulmonary trunk, respectively
(Table 3).

\textbf{DISCUSSION}

Our study shows that fetal echocardiography can accurately identify the
origin of pulmonary vascular supply. Accuracy in defining the anatomy of
the pulmonary vasculature and the source of pulmonary blood supply was
82.3\% and 88.2\%, respectively. Previously published studies report
lower rates\textsuperscript{5,6}. Zhoi J et al. and Naimi I et al.
reported similar rates with us in recent studies\textsuperscript{7,8}.
The improvement seems to be related to the advanced ultrasound
technology. Besides, we have been intensely searching for MAPCAs and the
origin of pulmonary blood supply since the beginning of the study
period. We have no false negative MAPCA-dependent PAVSDs in our study.
Therefore, we suggest that cases with MAPCAs can be diagnosed with 100\%
sensitivity with a meticulous search, including sagittal and coronary
planes of the aortic arch and descendent aorta. MAPCAs mostly originate
from the descending aorta (Figure 4b,4c, Figure 5c). They can also arise
from the aortic arch (Figure 4a, Figure 5a), subclavian arteries,
brachiocephalic trunk, internal mammarian artery, and left coronary
artery\textsuperscript{9}.

We detected MAPCA originating from the coronary artery in one of the
cases (Figure 3a). MPA and the confluence of central PAs were absent on
fetal echocardiography. Postnatal angiography (Figure 3c,3d) and surgery
(Figure 3e,3f) confirmed the prenatal diagnosis. According to the
intraoperative assessment, MPA was absent. The LPA originated from
patent DA. The RPA was supplied by a collateral from the left main
coronary artery (LMCA) (Figure 3e,3f). There was no confluence of PAs.
Coronary to pulmonary collaterals are rare and can be detected in
1.3\%-10\% of the cases with PAVSD\textsuperscript{10,11}. To our
knowledge, this was the first case in the literature diagnosed
prenatally and confirmed surgically. It was expected that cardiac
ischemia could have occurred due to increased coronary to pulmonary
steal secondary to decreased pulmonary vascular resistance after birth.
Thanks to the prenatal diagnosis, the newborn was immediately referred
to the collaborated cardiovascular clinic on prostaglandin infusion and
underwent early surgery to avoid coronary stealing. Therefore, we
suggest that the aortic root should also be carefully searched in axial
and longitudinal planes in PAVSD cases to detect any collaterals arising
from coronary arteries. These newborns may not present cyanosis due to
the high flow from coronary arteries to the lungs; however, they may
develop acute cardiac failure based on cardiac ischemia.

The impact of MAPCAs on perinatal and postnatal outcomes is debatable in
the literature\textsuperscript{3,7}. MAPCA-dependent newborns can
present cyanosis in case of narrow collaterals, or those with adequate
collaterals may be acyanotic with subtle symptoms for months until
stenosis of the collaterals occurs. Multiple, dilated collaterals may
lead to pulmonary hypertension, congestive heart failure, or pulmonary
parenchymal bleeding. MAPCA-dependent cases usually have non-confluent
PAs with arborization abnormalities or no central PAs at all, while
DA-supplied cases usually have confluent PAs with complete
intrapulmonary arborization\textsuperscript{8}. Consistently, in our
study, except the one with DA arising from the
1\textsuperscript{st}branch of the aortic arch (Figure 6a, 6b), all
DA-dependent cases had unifocal, confluent PAs. On the other hand, only
28.5 \% of the MAPCA group had confluent PAs. The presence or absence of
confluent PAs transforms the method of surgery. Non-confluent PAs have
narrow RPA, LPA, and larger MAPCAs which may lead to pulmonary
parenchymal disease secondary to incomplete intrapulmonary
arborization\textsuperscript{7}. Therefore, assessment of the confluence
of PAs and the size and anatomy of PAs on fetal echocardiography is as
necessary as identifying the origin of pulmonary blood supply for proper
prenatal counseling. Considering the smaller PAs and impaired PA
arborization, worse outcomes could have been expected in the MAPCA group
compared with the DA group. However, we found that the existence of
MAPCAs did not significantly impact postnatal survival. This may be
related to the relatively higher accompanying anomalies in the DA group
in our study. Moreover, DA-dependent cases needed surgical intervention
to prevent desaturation secondary to the stenosis of DA within the first
two weeks of life. In contrast, MAPCAs were operated on in later weeks
as the stenosis occurred later.

The overall survival was too low compared with the other studies in the
literature\textsuperscript{3,7}. Survival was highly disrupted by
extracardiac anomalies, particularly in the DA group. Among the babies
who survived until the operation, postoperative survival rates for MAPCA
and DA groups were 60\% and 66.6\%, respectively. These rates are also
lower than the other studies\textsuperscript{3,9}. Both postoperative
deaths were related to extracardiac anomalies in the DA group.
Therefore, while counseling the parents, it should be emphasized that
extracardiac malformations may prohibit surgery or lead to postoperative
mortality. Postnatal surgical series report rather promising
outcomes\textsuperscript{12,13}. However, many newborns like the ones in
our study do not survive until the operation.

One of the weaknesses of our study is the small sample size. We
restricted the study period to the last five years as we have had a
dedicated fetal echocardiography clinic since 2017. Another consequence
of this short study period is that we could not compare the long-term
prognosis between the groups. The strength of the study is that it was
conducted in a single center, and the fetal echocardiographies were
performed by the same operator. All of the angiographies and the
majority of the surgeries were performed by the associate cardiovascular
surgery clinic.

\textbf{CONCLUSION}

Accurate assessment of pulmonary vascular anatomy on fetal
echocardiography is possible and crucial in the ultrasound examination
of PAVSD. Despite the origin of the pulmonary blood supply having no
impact on postnatal prognosis and postoperative survival, prenatal
identification of the pulmonary vascular anatomy may lead to decisions
about early postnatal management strategies.

\textbf{Conflict of Interest} : None declared.

REFERENCES

1. Leonard H, Derrick G, O'Sullivan J, Wren C. Natural and unnatural
history of pulmonary atresia. Heart (British Cardiac Society)
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2. Tchervenkov CI, Salasidis G, Cecere R, et al. One-stage midline
unifocalization and complete repair in infancy versus multiple-stage
unifocalization followed by repair for complex heart disease with major
aortopulmonary collaterals. J Thorac Cardiovasc Surg. 1997;114:727--737.

3. Gottschalk I, Strizek B, Jehle C, et al. Prenatal diagnosis and
postnatal outcome of fetuses with pulmonary atresia and ventricular
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4. Schneider C, McCrindle BW, Carvalho JS, Hornberger LK, McCarthy KP,
Daubeney PEF. Development of Z -scores for fetal cardiac dimensions from
echocardiography. Ultrasound Obstet Gynecol 2005;26:599--605.

5. Vesel S, Rollings S, Jones A, Callaghan N, Simpson J, Sharland GK.
Prenatally diagnosed pulmonary atresia with ventricular septal defect:
echocardiography, genetics, associated anomalies and outcome. Heart
2006;92:1501-1505.

6. Volpe P, Campobasso G, Stanziano A, et al. Novel application of 4D
sonography with B-flow imaging and spatio-temporal image correlation
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with pulmonary atresia and ventricular septal defect. Ultrasound Obstet
Gynecol 2006;28:40-46.

7. Zhou J, Zhou Q, Peng Q, Zhang R, Tang W, Zeng S. Fetal pulmonary
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8. Naimi I, Clouse M, Arya B, Conwell JA, Lewin MB, Bhat AH. Accuracy of
fetal echocardiography in defining pulmonary artery anatomy and source
of pulmonary blood flow in pulmonary atresia with ventricular septal
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9. Presnell LB, Blankenship A, Cheatham SL, Owens GE, Staveski SL. An
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10. Amin Z, McElhinney DB, Reddy VM, Moore P, Hanley FL, Teitel DF.
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11. Alkhushi N, Al-Radi OO, Ajlan A, Abdelmohsen G, Attia W.
Coronary-pulmonary arterial fistula in a neonate with pulmonary
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Echocardiography 2017;34:1536--159.

12. Soquet J, Liava'a M, Eastaugh L, et al. Achievements and limitations
of a strategy of rehabilitation of native pulmonary vessels in pulmonary
atresia, ventricular septal defect, and major aortopulmonary collateral
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13. Bauser-Heaton H, Borquez A, Han B, et al. Programmatic approach to
management of tetralogy of fallot with major aortopulmonary collateral
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2017;10:e004952.

\textbf{Figure legends:}

Figure 1: Images of Fallot tetralogy with PAVSD case. Figure 1a: Color
Doppler image of overriding aorta receiving blood from the right and
left ventricles, axial planes. Figure 1b: Atretic pulmonary valve
(arrow). Figure 1c: Confluence of hypoplastic pulmonary arteries (arrow)
Hypoplastic central pulmonary arteries on grayscale(asterisks). Figure
1d: Retrograde flow of main pulmonary artery. Figure 1e: Postnatal image
of RPA and LPA (asterisks) supplied by PDA (arrow) via selective ductus
arteriosus angiogram. Pulmonary blood supply is solely through a
vertically oriented PDA (arrow) with confluent pulmonary arteries. When
the arterial duct presents, it originates from the undersurface of the
aorta in most patients.

Figure 2: Flow diagram of prenatally diagnosed PAVSD in 28 cases

Figure 3: Case 4 presented double supply and MAPCA arising from the
coronary artery. Figure 3a: Grayscale image of origin of the left
coronary artery (asterisk) from the aortic root in supra valvular level
(double-headed arrow) in longitudinal view. The left main coronary
artery (LMCA) gives rise both to the left anterior descending artery
(double-headed, dashed arrow) and a collateral (thick arrow) towards the
RPA (thin arrow). RV: Right ventricule, LV: Left ventricule. Figure 3b:
Color Doppler image of the coronary artery (asterisk), which gives rise
to the left anterior descending coronary (thick arrow) and MAPCA (thin
arrow) supplying the RPA. Figure 3c: Axial view of the aortic root, the
origin of coronary artery (thin arrow), which gave MAPCA supplying the
RPA (thick arrows) and left anterior descending artery (dashed arrow).
Figure 3d: Color Doppler image of overriding aorta and ductus arteriosus
(arrow) supplying left pulmonary artery (asterisk) in longitudinal view.
Figure 3e: Angiographic images of the aortic root and the collateral
between the left main coronary artery (LMCA) and right pulmonary artery
(RPA). Figure 3f: Operation image displaying the insertion of coronary
to pulmonary collateral (CA-Pac) into the lumen of RPA. The collateral
arose from the left main coronary artery (LMCA).

Figure 4a: Sagittal plane, MAPCAs from descending aorta (arrow), and the
end of the aortic arch (asterisk) of case 1. The collateral arising from
the end of the aortic arch (asterisk) was considered as ductus
arteriosus on fetal echocardiography. However, since the pulmonary trunk
was completely atretic, it was diagnosed as MAPCA from the aortic arch
in the postnatal transcatheter angiography. Figure 4b: MAPCAs
originating from descending aorta at 35th gestational weeks, coronal
view of the aorta. They became dilated and visible even on grayscale
(arrows). Figure 4c: Color Doppler of MAPCAs (arrow) on the coronal
plane in case 1. Figure 4d: Angiographic images of MAPCAs in various
sizes and morphology. Desendan aortogram shows that large collateral
vessels are the sole source of pulmonary blood flow. No native central
pulmonary arteries were seen.

Figure 5: Multiple MAPCAs of a case with severe hypoplastic pulmonary
trunk (case 19). The pregnancy was terminated based on the genetic
diagnosis of Di George syndrome. Figure 5a: MAPCAs arising from the
aortic arch (arrows) in the sagittal plane. Figure 5b: MAPCAs arising
from descending aorta, sagittal plane. Figure 5c: Multiple and dilated
MAPCAs arising from descending aorta, sagittal plane

Figure 6a: Continuity of the first main branch of the aorta (thin arrow)
and the supplying vessel (dashed arrow) feeding the non-communicant
right pulmonary artery (thick arrow) in sagittal view. The supplying
vessel could have been considered as MAPCA originated from aortic arch
branches; however, the short, non-tortuous, unbranched course and the
narrowing at the attachment site to the right pulmonary artery
(asterisk) directed the diagnosis towards ductus arteriosus arising from
the 1\textsuperscript{st} branch of a left aortic arch.

Figure 6b: The probe was slightly turned following the supplying vessel
of the non-communicant right pulmonary artery (dashed arrow), and a
coronal view was obtained displaying the common origin of the right
subclavian artery (arrow) and the ductus arteriosus (dashed arrow). Tr:
trachea.

Table 1: Prenatal sonographic findings and outcomes of PAVSD cases\selectlanguage{english}
\begin{longtable}[]{@{}llllllll@{}}
\toprule
\begin{minipage}[b]{0.10\columnwidth}\raggedright\strut
Case\strut
\end{minipage} & \begin{minipage}[b]{0.10\columnwidth}\raggedright\strut
GA at first appeal\strut
\end{minipage} & \begin{minipage}[b]{0.10\columnwidth}\raggedright\strut
Prenatal defined source of pulmonary blood supply\strut
\end{minipage} & \begin{minipage}[b]{0.10\columnwidth}\raggedright\strut
MPA\strut
\end{minipage} & \begin{minipage}[b]{0.10\columnwidth}\raggedright\strut
Confluence of PAs\strut
\end{minipage} & \begin{minipage}[b]{0.10\columnwidth}\raggedright\strut
Genetic studies (karyotype/ CMA)\strut
\end{minipage} & \begin{minipage}[b]{0.10\columnwidth}\raggedright\strut
Extracardiac anomalies\strut
\end{minipage} & \begin{minipage}[b]{0.10\columnwidth}\raggedright\strut
Outcome\strut
\end{minipage}\tabularnewline
\midrule
\endhead
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
1.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
28\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Double\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
non-confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
normal/normal\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative survival\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
2.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
23\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Double\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
normal/normal\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative survival\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
3.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
30\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
MAPCAs\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
normal/normal\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative survival\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
4.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
31\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Double\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
non-confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
normal/normal\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative survival, SCR\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
5.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
19\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DA\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Tr13\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Multiple anomalies, thymus hypoplasia\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Neonatal death\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
6.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
26\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DA\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
normal/normal\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative survival\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
7.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
18\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
MAPCAs\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
non-confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Tr18\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
MCDK, anhydramnios\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Neonatal death\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
8.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
20\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Double\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
normal/normal\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative death\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
9.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
22\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
MAPCAs\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
non-confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Not performed\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative survival\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
10.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
20\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
MAPCAs\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
non-confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Not performed\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative death\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
11.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
21\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DA\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Di George\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Thymus hypoplasia\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative death\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
12.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
24\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
MAPCAs\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
non-confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Not performed\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Infant death before surgery/intervention\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
13.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
13\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DA\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
normal/normal\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Omphalocele\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Neonatal death\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
14.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
26\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DA\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Not performed\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Anal atresia with urinary fistula\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative survival\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
15.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
24\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DA\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Not performed\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DWM\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative death\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
16.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
22\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DA\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Not performed\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative survival\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
17.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
22\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DA\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
normal/normal\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Postoperative survival, SCR\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
18.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
32\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Double\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Di George\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Thymus hypoplasia\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
TOP\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.12\columnwidth}\raggedright\strut
19.\strut
\end{minipage} & \begin{minipage}[t]{0.12\columnwidth}\raggedright\strut
21\strut
\end{minipage} & \begin{minipage}[t]{0.12\columnwidth}\raggedright\strut
MAPCAs (from aortic arch and descendent aorta)\strut
\end{minipage} & \begin{minipage}[t]{0.12\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.12\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.12\columnwidth}\raggedright\strut
Di George\strut
\end{minipage} & \begin{minipage}[t]{0.12\columnwidth}\raggedright\strut
Thymus hypoplasia\strut
\end{minipage} & \begin{minipage}[t]{0.12\columnwidth}\raggedright\strut
TOP\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
20.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
22\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Double\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Di George\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Thymus hypoplasia\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
TOP\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
21.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
20\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Double\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
non-confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
normal/normal\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Cerebellar hypoplasia, FGR\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
TOP\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
22.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
18\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DA\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
+/\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
normal/normal\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
CH, CDH, PEV\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
TOP\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
23.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
16\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DA\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
NPI/NPI\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Not performed\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
MCDK, anhydramnios\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
TOP\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
24.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
21\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
DA\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
non-confluent\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Di George\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
Cleft palate, thymus hypoplasia\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
TOP\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
GA: gestational age, MAPCAs: major aortopulmonary collaterals, DA:
ductus arteriosus, MPA: main pulmonary artery, NPI: not possible to
identify, CMA: chromosomal microarray, MCDK: multicystic dysplastic
kidneys, DWM: Dandy-Walker malformation, FGR: fetal growth restriction,
CH: cystic hygroma, CDH: congenital diaphragmatic hernia, PEV: pes
equinovarus, SCR: scheduled for total cardiac repair, TOP: termination
of pregnancy.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
GA: gestational age, MAPCAs: major aortopulmonary collaterals, DA:
ductus arteriosus, MPA: main pulmonary artery, NPI: not possible to
identify, CMA: chromosomal microarray, MCDK: multicystic dysplastic
kidneys, DWM: Dandy-Walker malformation, FGR: fetal growth restriction,
CH: cystic hygroma, CDH: congenital diaphragmatic hernia, PEV: pes
equinovarus, SCR: scheduled for total cardiac repair, TOP: termination
of pregnancy.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
GA: gestational age, MAPCAs: major aortopulmonary collaterals, DA:
ductus arteriosus, MPA: main pulmonary artery, NPI: not possible to
identify, CMA: chromosomal microarray, MCDK: multicystic dysplastic
kidneys, DWM: Dandy-Walker malformation, FGR: fetal growth restriction,
CH: cystic hygroma, CDH: congenital diaphragmatic hernia, PEV: pes
equinovarus, SCR: scheduled for total cardiac repair, TOP: termination
of pregnancy.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
GA: gestational age, MAPCAs: major aortopulmonary collaterals, DA:
ductus arteriosus, MPA: main pulmonary artery, NPI: not possible to
identify, CMA: chromosomal microarray, MCDK: multicystic dysplastic
kidneys, DWM: Dandy-Walker malformation, FGR: fetal growth restriction,
CH: cystic hygroma, CDH: congenital diaphragmatic hernia, PEV: pes
equinovarus, SCR: scheduled for total cardiac repair, TOP: termination
of pregnancy.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
GA: gestational age, MAPCAs: major aortopulmonary collaterals, DA:
ductus arteriosus, MPA: main pulmonary artery, NPI: not possible to
identify, CMA: chromosomal microarray, MCDK: multicystic dysplastic
kidneys, DWM: Dandy-Walker malformation, FGR: fetal growth restriction,
CH: cystic hygroma, CDH: congenital diaphragmatic hernia, PEV: pes
equinovarus, SCR: scheduled for total cardiac repair, TOP: termination
of pregnancy.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
GA: gestational age, MAPCAs: major aortopulmonary collaterals, DA:
ductus arteriosus, MPA: main pulmonary artery, NPI: not possible to
identify, CMA: chromosomal microarray, MCDK: multicystic dysplastic
kidneys, DWM: Dandy-Walker malformation, FGR: fetal growth restriction,
CH: cystic hygroma, CDH: congenital diaphragmatic hernia, PEV: pes
equinovarus, SCR: scheduled for total cardiac repair, TOP: termination
of pregnancy.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
GA: gestational age, MAPCAs: major aortopulmonary collaterals, DA:
ductus arteriosus, MPA: main pulmonary artery, NPI: not possible to
identify, CMA: chromosomal microarray, MCDK: multicystic dysplastic
kidneys, DWM: Dandy-Walker malformation, FGR: fetal growth restriction,
CH: cystic hygroma, CDH: congenital diaphragmatic hernia, PEV: pes
equinovarus, SCR: scheduled for total cardiac repair, TOP: termination
of pregnancy.\strut
\end{minipage} & \begin{minipage}[t]{0.10\columnwidth}\raggedright\strut
GA: gestational age, MAPCAs: major aortopulmonary collaterals, DA:
ductus arteriosus, MPA: main pulmonary artery, NPI: not possible to
identify, CMA: chromosomal microarray, MCDK: multicystic dysplastic
kidneys, DWM: Dandy-Walker malformation, FGR: fetal growth restriction,
CH: cystic hygroma, CDH: congenital diaphragmatic hernia, PEV: pes
equinovarus, SCR: scheduled for total cardiac repair, TOP: termination
of pregnancy.\strut
\end{minipage}\tabularnewline
\bottomrule
\end{longtable}

Table 2 Postnatal outcomes based on the type of pulmonary vascular
supply\selectlanguage{english}
\begin{longtable}[]{@{}lllll@{}}
\toprule
\begin{minipage}[b]{0.17\columnwidth}\raggedright\strut
Pulmonary vascular supply (based on postnatal diagnosis)\strut
\end{minipage} & \begin{minipage}[b]{0.17\columnwidth}\raggedright\strut
DA (n=8)\strut
\end{minipage} & \begin{minipage}[b]{0.17\columnwidth}\raggedright\strut
MAPCA (n=7)\strut
\end{minipage} & \begin{minipage}[b]{0.17\columnwidth}\raggedright\strut
Double supply (n=2)\strut
\end{minipage} & \begin{minipage}[b]{0.17\columnwidth}\raggedright\strut
p-value (DA/MAPCA)\strut
\end{minipage}\tabularnewline
\midrule
\endhead
\begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
Neonatal/infant death without surgery\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
2/8\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
2/7\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
0\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
0.66\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.19\columnwidth}\raggedright\strut
Surgical intervention time (days)\strut
\end{minipage} & \begin{minipage}[t]{0.19\columnwidth}\raggedright\strut
7.75 (\selectlanguage{ngerman}±2,76) 7.5 (4-12)\strut
\end{minipage} & \begin{minipage}[t]{0.19\columnwidth}\raggedright\strut
179 (\selectlanguage{ngerman}±60) 150 (120-270)\strut
\end{minipage} & \begin{minipage}[t]{0.19\columnwidth}\raggedright\strut
12 (11-13)\strut
\end{minipage} & \begin{minipage}[t]{0.19\columnwidth}\raggedright\strut
\textless{}0.05\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
Postoperative mortality\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
2/8\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
2/7\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
0\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
0.55\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
Survival*\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
4/8\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
3/7\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
2 /2\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
0.59\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
Survival excluding extracardiac abnormalities\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
4/5\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
3/6\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
2/2\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
0.18\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
Including two cases that are scheduled for complete repair, one case
from DA, one case from the double supply group\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
*Including two cases that are scheduled for complete repair, one case
from DA, one case from the double supply group\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
*Including two cases that are scheduled for complete repair, one case
from DA, one case from the double supply group\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
*Including two cases that are scheduled for complete repair, one case
from DA, one case from the double supply group\strut
\end{minipage} & \begin{minipage}[t]{0.17\columnwidth}\raggedright\strut
*Including two cases that are scheduled for complete repair, one case
from DA, one case from the double supply group\strut
\end{minipage}\tabularnewline
\bottomrule
\end{longtable}

Table 3: Comparison of fetal echocardiographic findings and postnatal
catheter angiography/ operation findings for the live-born cases\selectlanguage{english}
\begin{longtable}[]{@{}llllllllllll@{}}
\toprule
Case & Source of pulmonary blood flow & Source of pulmonary blood flow &
Origin of MAPCAs & Origin of MAPCAs & MPA & MPA & Confluence of PA &
Confluence of PA & RPA/LPA & RPA/LPA & Postnatal outcome\tabularnewline
\midrule
\endhead
& Fetal & PN & Fetal & PN & Fetal & PN & Fetal & PN & Fetal & PN
&\tabularnewline
1. & Double & MAPCA & Descending aorta & Descending aorta + aortic arch
& - & - & +* & - & +*/+* & -/- & Complete cardiac repair,
alive\tabularnewline
2. & Double & Double & Descending aorta & Descending aorta & + & + & + &
+ & +/+ & +/+ & Complete cardiac repair, alive\tabularnewline
3. & MAPCA & MAPCA & Descending aorta & Descending aorta & + & + & + & +
& +/+ & +/+ & Complete cardiac repair, alive\tabularnewline
4. & Double & Double & Coronary artery & Coronary artery & - & - & - & -
& +/+ & +/+ & Stepped surgery, scheduled for total repair,
alive\tabularnewline
5. & DA & DA & 0 & 0 & + & + & + & + & +/+ & +/+ & Mortality without
surgery (Tr13)\tabularnewline
6. & DA & DA & 0 & 0 & + & + & + & + & +/+ & +/+ & Complete cardiac
repair, alive\tabularnewline
7. & MAPCAs & MAPCA & Descending aorta & Descending aorta & - & - & - &
- & -/- & -/- & Mortality without surgery (Tr18, MCDK)\tabularnewline
8. & Double & MAPCA & Descending aorta & Descending aorta + aortic arch
& + & - & + & + & +/+ & +/+ & Postoperative mortality\tabularnewline
9. & MAPCAs & MAPCA & Descending aorta & Descending aorta & - & + & - &
- & -/- & +*/+* & Complete cardiac repair, alive\tabularnewline
10. & MAPCAs & MAPCA & Descending aorta & Descending aorta & - & - & - &
- & -/- & +*/+* & Postoperative mortality\tabularnewline
11. & DA & DA & 0 & 0 & +* & + & + & + & +*/+* & +*/+* & Postoperative
mortality (Di George)\tabularnewline
12. & MAPCA & MAPCA & Descending aorta & Descending aorta & - & - & - &
- & -/- & -/- & Mortality without surgery\tabularnewline
13. & DA & DA & 0 & 0 & + & + & + & + & +/+ & +/+ & Mortality without
surgery (Giant omphalocele)\tabularnewline
14. & DA & DA & 0 & 0 & + & + & + & + & +/+ & +/+ & Complete cardiac
repair, alive\tabularnewline
15. & DA & DA & 0 & 0 & + & + & + & + & +/+ & +/+ & Postoperative
mortality after the first stage (DWM)\tabularnewline
16. & DA & DA & 0 & 0 & +* & + & + & + & +*/+* & +*/+* & Complete
cardiac repair, alive\tabularnewline
17. & DA & DA & 0 & 0 & + & + & + & + & +/+ & +/+ & Stepped surgery,
scheduled for total repair, alive\tabularnewline
PN: postnatal, DA: ductus arteriosus, RPA: right pulmonary artery, LPA:
left pulmonary artery, MCDK: multicystic dysplastic kidneys, DWM:
Dandy-Walker malformation, * severely hypoplastic/ not possible to
measure & PN: postnatal, DA: ductus arteriosus, RPA: right pulmonary
artery, LPA: left pulmonary artery, MCDK: multicystic dysplastic
kidneys, DWM: Dandy-Walker malformation, * severely hypoplastic/ not
possible to measure & PN: postnatal, DA: ductus arteriosus, RPA: right
pulmonary artery, LPA: left pulmonary artery, MCDK: multicystic
dysplastic kidneys, DWM: Dandy-Walker malformation, * severely
hypoplastic/ not possible to measure & PN: postnatal, DA: ductus
arteriosus, RPA: right pulmonary artery, LPA: left pulmonary artery,
MCDK: multicystic dysplastic kidneys, DWM: Dandy-Walker malformation, *
severely hypoplastic/ not possible to measure & PN: postnatal, DA:
ductus arteriosus, RPA: right pulmonary artery, LPA: left pulmonary
artery, MCDK: multicystic dysplastic kidneys, DWM: Dandy-Walker
malformation, * severely hypoplastic/ not possible to measure & PN:
postnatal, DA: ductus arteriosus, RPA: right pulmonary artery, LPA: left
pulmonary artery, MCDK: multicystic dysplastic kidneys, DWM:
Dandy-Walker malformation, * severely hypoplastic/ not possible to
measure & PN: postnatal, DA: ductus arteriosus, RPA: right pulmonary
artery, LPA: left pulmonary artery, MCDK: multicystic dysplastic
kidneys, DWM: Dandy-Walker malformation, * severely hypoplastic/ not
possible to measure & PN: postnatal, DA: ductus arteriosus, RPA: right
pulmonary artery, LPA: left pulmonary artery, MCDK: multicystic
dysplastic kidneys, DWM: Dandy-Walker malformation, * severely
hypoplastic/ not possible to measure & PN: postnatal, DA: ductus
arteriosus, RPA: right pulmonary artery, LPA: left pulmonary artery,
MCDK: multicystic dysplastic kidneys, DWM: Dandy-Walker malformation, *
severely hypoplastic/ not possible to measure & PN: postnatal, DA:
ductus arteriosus, RPA: right pulmonary artery, LPA: left pulmonary
artery, MCDK: multicystic dysplastic kidneys, DWM: Dandy-Walker
malformation, * severely hypoplastic/ not possible to measure & PN:
postnatal, DA: ductus arteriosus, RPA: right pulmonary artery, LPA: left
pulmonary artery, MCDK: multicystic dysplastic kidneys, DWM:
Dandy-Walker malformation, * severely hypoplastic/ not possible to
measure & PN: postnatal, DA: ductus arteriosus, RPA: right pulmonary
artery, LPA: left pulmonary artery, MCDK: multicystic dysplastic
kidneys, DWM: Dandy-Walker malformation, * severely hypoplastic/ not
possible to measure\tabularnewline
\bottomrule
\end{longtable}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Figure-1/Figure-1}

\end{center}
\end{figure}

\textbf{Hosted file}

\verb`Figure 2.docx`  available at \url{https://authorea.com/users/630274/articles/650078-pulmonary-atresia-and-ventricular-septal-defect-how-accurate-is-the-fetal-echocardiography-and-do-the-major-aortopulmonary-collateral-arteries-matter}\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/Figure-3/Figure-3}

\end{center}
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\includegraphics[width=0.70\columnwidth]{figures/Figure4/Figure4}

\end{center}
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\includegraphics[width=0.70\columnwidth]{figures/Figure-5/Figure-5}

\end{center}
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\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Figure-6/Figure-6}

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\textbf{Hosted file}

\verb`Table 1-PAVSD.docx`  available at \url{https://authorea.com/users/630274/articles/650078-pulmonary-atresia-and-ventricular-septal-defect-how-accurate-is-the-fetal-echocardiography-and-do-the-major-aortopulmonary-collateral-arteries-matter}

\textbf{Hosted file}

\verb`Table 2.docx`  available at \url{https://authorea.com/users/630274/articles/650078-pulmonary-atresia-and-ventricular-septal-defect-how-accurate-is-the-fetal-echocardiography-and-do-the-major-aortopulmonary-collateral-arteries-matter}

\textbf{Hosted file}

\verb`Table 3-PAVSD.docx`  available at \url{https://authorea.com/users/630274/articles/650078-pulmonary-atresia-and-ventricular-septal-defect-how-accurate-is-the-fetal-echocardiography-and-do-the-major-aortopulmonary-collateral-arteries-matter}

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