Isomerism of the atrial appendages in the fetus

Thirty hearts from fetuses with visceral heterotaxy were examined to determine the range of cardiac malformations. By examining the morphology of the atrial appendages, 20 hearts were identified as having left isomerism (67%) and 10 hearts right isomerism (33%). Fifteen of the hearts with left isomerism had biventricular atrioventricular connections and five had univentricular atrioventricular connections. All 10 hearts with right isomerism had biventricular atrioventricular connections. Flow to the pulmonary arteries was obstructed or occluded in 6 hearts with left isomerism (30%) and 7 hearts with right isomerism (70%). Obstruction to aortic flow was present in 4 hearts with left isomerism (20%). Anomalies of the systemic and pulmonary veins were common in both groups. The suprarenal portion of the inferior caval vein was interrupted in 11 hearts with left isomerism (55%) and 1 heart with right isomerism (10%). Atrioventricular septal defect was present in 13 hearts with left isomerism (65%) and 9 hearts with right isomerism (90%). The arrangement of the liver or stomach in each group was not consistent, nor was the status of the spleen. The constellations of cardiac malformations in both groups corresponded to those known in postnatal life and similar guidelines for their identification were applicable.