Surgical Repair of Superoinferior Ventricles: Experience with 3 Patients

Superoinferior ventricles are a rare anomaly characterized by a horizontal ventricular septum and a hypoplastic right ventricular sinus localized anterosuperiorly to the left ventricle. This anomaly frequently is accompanied by malformation of the atrioventricular valves. A large ventricular septal defect is always present, and anomalies of the ventriculoarterial relations are common. The results of surgical repair of this complex lesion have been poor. Our recent surgical experience with 3 patients, 2 of whom are well 36 and 38 months postoperatively, suggests a hopeful outcome for the repair of this complex anomaly. The surgical approach was different in each of the 3 patients, demonstrating the need for a precise echocardiographic and angiocardiographic preoperative description of the cardiac anatomy to appropriately repair the multiple variants of this complex anomaly.