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. Circulation 72, No. 4, 825-832, 1985. ADVANCES in ultrasound technology have made it possible to examine human fetal cardiac development and function in utero'-13 and have even permitted the study of early embryogenesis in experimental animals. 14 Structural and functional heart disease in utero has been examined by a number of investigators applying M mode and cross-sectional echocardiographic imaging techniques to the examination of the human fetus during the second and third trimesters of pregnanCy. [1][2][3][4][5][6][7][8][9][10][11][12][13] This information has been used to counsel prospective parents and, in several instances, to formulate management for the remainder of pregnancy, during delivery, and in the neonatal period. '5-23 Recently scending aortic and/or umbilical blood flow by measuring mean flow velocity and cross-sectional vessel area.11 12. 2t28 This method has also been used to provide qualitative information about intracardiac blood flow. '' 12. 24. 25, 28 We used the combination of cross-sectional echocardiography and pulsed Doppler ultrasound to evaluate 466 fetuses referred to our centers for evaluation of the presence of congenital heart disease. We detected atrioventricular valve insufficiency in 23 of these 466 fetuses in the second and third trimesters of pregnancy. Of these fetuses, 16 had associated nonimmune hydrops. Four of the pregnancies were terminated. Eleven of the 12 fetuses with structural heart disease either died in utero or within the first week after birth. Seven fetuses with atrioventricular valve insufficiency and structural heart disease but without nonimmune hydrops survived pregnancy and the neonatal period.
MethodsFour hundred sixty-six fetuses, ranging from 16 to 40 weeks gestational age, whose mothers had been referred to the fetal echocardiographic laboratories