CONGENITAL ABSENCE OF AORTIC AND PULMONARY VALVE IN A FETUS WITH SEVERE HEART-FAILURE

A case of congenital absence of both aortic and pulmonary valves with severe heart failure detected prenatally by cross-sectional and pulsed and colour Doppler echocardiography is reported in small for gestatio nal age male fetus in 17th week of gestation. Additional double outlet right ventricle, hypoplastic left ventricle, and ventricular septal d efect, as well as multiple extracardiac anomalies, were found by prena tal echocardiographic investigation and confirmed by necropsy examinat ion. Retrograde diastolic Doppler waveforms retrieved from pulmonary a rtery, aorta, and umbilical arteries revealed massive insufficiency th roughout both the great arteries, which eliminated diastolic placental perfusion, documented by absent anterograde diastolic flow in the umb ilical vein. These prenatal echocardiographic findings may contribute to an understanding of the mechanism of rapid and progressive heart fa ilure and growth retardation in the fetus. Severe cardiac failure may explain why congenital aplasia of both the aortic and the pulmonary va lves has not been described postnatally, and only two fetal cases reve aled by necropsy have been published.