Angulation of the inferior vena cava-right atrial junction in children with repaired omphalocele.

Three children with repaired omphalocele underwent diagnostic cardiac catheterization. In addition to intracardiac shunts, each was found to have marked angulation at the junction of the inferior vena cava and the right atrium. After review of our autopsy material following omphalocele repair, it is suggested that the inferior vena cava - right atrial angulation is due to gradual abnormal fixation of this junction either in utero or related to the surgical repair. A change in current surgical therapy is NOT recommended. Angulation of the inferior vena cava - right atrial junction is potentially important because a) it may interfere with venous cardiac catheterization from "below", b) it poses a danger to inferior vena caval cannulation for cardio-pulmonary by-pass, and c) it may become obstructive with time producing portal hypertension.