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.

Background: Transposition of the great arteries (TGA) is a conotruncal abnormality. It is associated with ventriculoarterial discordance with the parallel orientation of the great arteries, in which the aorta arises from the right ventricle to supply the systemic circulation, while the main pulmonary artery arises from the left ventricle to supply the pulmonary circulation. Aim: To analyze the prenatal and postnatal outcomes of fetuses with d-TGA and to determine whether prenatal echocardiography may predict postnatal urgent BAS. Methods: A retrospective study of fetuses with d-TGA, for which fetal echocardiography was performed at our tertiary hospital from January 2018 to May 2023. We assessed the appearance of the septum primum and the foramen ovale (FO) flap in the four-chamber view as to whether the FO had a restrictive appearance during measurement of the diameter of the FO at its maximal angle to the attachment point. Color Doppler was used to detect ventricular septal defects (VSD) and measure its diameter both in the four-chamber view and when visualizing the outlets of the great arteries in the sagittal section of the heart. Results: During the study period, 64 fetuses were diagnosed with d-TGA, which was also confirmed postnatally. Of these, 16 fetuses were excluded due to additional cardiac anomalies or the inability to reach the mother. In total, 48 cases were included in this series. In our study, the FO diameter was significantly decreased in the urgent BAS group, compared with the fetuses without urgent BAS (5.1 mm vs 6.3 mm, p0.05). A cut off of 6 mm for the FO diameter (sensitivity, 73.3%; specificity, 72.2%; area under the curve [AUC], 0.764) and 3.2 mm for the VSD diameter (sensitivity, 75%; specificity, 75%; AUC, 0.728) suggested urgent BAS. The FO diameter and the presence of VSD were independent variables associated with urgent BAS in fetuses with d-TGA (p0.05). Conclusion: Prenatal echocardiography in fetuses with d-TGA provides valuable information to estimate the need for postnatal urgent BAS that would prevent immediate life-threatening complications.

A document by Arif Yasin Çakmak. Click on the document to view its contents.