Fetal atrioventricular valve insufficiency associated with nonimmune hydrops: a two-dimensional echocardiographic and pulsed Doppler ultrasound study

Of 466 fetuses who underwent cardiac ultrasound examination with cross-sectional and M mode echocardiography and pulsed Doppler ultrasound and in whom we were able to follow the natural history, 12 were found to have atrioventricular valve insufficiency and nonimmune hydrops. Eleven fetuses (all of whom had structural heart disease) died either in utero or during the early postnatal period. In the one surviving hydropic fetus with supraventricular tachyarrhythmia and atrioventricular valve regurgitation but without structural heart disease, all the abnormalities disappeared on treatment with digoxin and verapamil. Seven fetuses who had atrioventricular valve insufficiency but did not develop nonimmune hydrops all survived pregnancy and the early neonatal period. The syndrome of atrioventricular valve insufficiency, nonimmune hydrops, and structural heart disease has a poor prognosis. The hydrops in this instance reflects fetal cardiac failure related to venous hypertension and low colloid oncotic pressure.