Absent pulmonary valve syndrom: multicenter analysis of prenatal diagnosis cases
A retrospective analysis of 10 cases of prenatal diagnosed of аbsent pulmonary valve syndrom (APVS): 9 (90 %) cases - APVS I type, 1 (10 %) - APVS type II. In the study of the four chambers of the fetal heart cardiomegaly was detected in 3 (30 %) cases. The axis of the heart changed in 9 (90 %) cases due to the prevalence of the size of the right ventricle. 100 % fetuses with APVS had increased pulmonary artery with/without branches with flow to-and-fro in the pulmonary valve. Agenesis of the ductus arteriosus (DA) identified in 5 (50 %) cases. The left aortic arch was in 7 (70 %) cases and right aortic arch - 3 (30 %). Hydrops fetalis and/or enlargent NT detected in 2 (20 %) cases. The average gestational age at prenatal diagnosis was 24,4 weeks (range 12-33 weeks). Early diagnosis is represented by one case in 12 weeks. The basis for the enhanced echocardiography were heart - cardiomegaly, large ventricular septal defect and enlargement of the pulmonary artery. Cardiac defects were registered in 5 (50 %) cases and extracardiac defects in 2 (20 %) cases. Outcomes in fetuses with APVS: the termination of pregnancy - 5 (50 %), neonatal death - 3 (30 %) cases, only 2 (20 %) survivors (one operated at 6 months and one needs surgical treatment)