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.