Biventricular Repair of Double Outlet Right Ventricle with Complete Atrioventricular Septal Defect

Abstract

The combination of double outlet right ventricle and complete atrioventricular septal defect (DORV-AVSD) is a rare congenital heart malformation. Double outlet right ventricle with complete atrioventricular septal defect is commonly associated with heterotaxy but is distinct from DORV as associated with tetralogy of Fallot. Biventricular repair of DORV-AVSD is challenging because it requires not only correction of the complete AVSD and DORV, but often also involves resection of the outlet portion of the ventricular septum to create an unobstructed left ventricular outflow tract as well as repair of the frequently associated systemic and pulmonary venous connection anomalies and right ventricular outflow tract obstruction. Because of this high degree of complexity, functional single ventricle palliation has been recommended as a treatment option for DORV-AVSD by some groups. However, biventricular repair in these patients, despite its complexity, can be accomplished with excellent results. Reoperation is common in this difficult group of patients, most commonly for conduit replacement. Reintervention for recurrent left ventricular outflow tract obstruction or atrioventricular valve stenosis or regurgitation is uncommon. We feel that biventricular repair of DORV-AVSD is the preferred treatment option, although further studies are needed to define long-term outcomes.