In a series of more than 3500 pregnancies referred for fetal echocardiography, 29 cases of atrioventricular septal defect were detected in the fetus. There was a chromosomal anomaly in 14 of these cases, left atrial isomerism in 12, and right atrial isomerism in two. Complete heart block was found in 11 of the cases with left atrial isomerism. Many associated cardiac abnormalities were found, particularly in the fetuses with atrial isomerism; the most common were double outlet right ventricle or aortic arch anomalies. The prognosis was poor in all patients with atrioventricular septal defect detected prenatally. Fifteen pregnancies went to term but there are only four survivors. Two of those have trisomy 21, a further patient has inoperable defects, and only one remains well and is awaiting corrective surgery for a partial atrioventricular septal defect.