A retrospective analysis of 21 cases of prenatal diagnosed of pulmonary atresia with intact ventricular
septum (PA-IVS) is presented. In the study of the four chambers view of the fetal heart the right ventricle was
hypoplastic in 16 (76.1 %), normal in 5 (23.8 %) cases. Tricuspid atresia identified in 8 (38 %) cases. The dysplasia of the tricuspid valve identified in 5 (23.8 %) cases. In 2 (8.7 %) cases PA-IVS were in combination with
atrioventricular defects. The changes of the tricuspid valve were not identified in 6 (28.5 %) cases. In 100 %
cases reduced pulmonary artery with retrograde flow in the pulmonary valve was registrated. Hydrops fetal is
detected in 2 (9.5 %) cases. The left aortic arch was in 17 (80.9 %) cases and right aortic arch – 4 (19.1 %).
The average gestational age at prenatal diagnosis was 22,6 weeks (range 11–32 weeks). Early diagnosis is represented by one case in 11 weeks. Cardiac defects were registered in 6 (28.5 %) cases and extracardiac defects in 5
(23.8 %) cases. Ventriculocoronary arterial communications were found in 2 (9.5 %) cases of PA-IVS. In 3 (13 %)
cases were in combination with heterotaxy syndrome. In 2 (8.7 %) cases PA-IVS were in combination with left
atrial isomerism and 1 (4.8 %) with right atrial isomerism. Outcomes in fetuses with PA-IVC: termination of
pregnancy — 13 (61.9 %), fetal death — 2 (9.5 %), neonatal death — 5 (23.8 %) cases, only 1 (4.8 %) survivor.
