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