Biventricular repair of double outlet right ventricle with noncommitted ventricular septal defect

Double outlet right ventricle (DORV) with noncommitted ventricular septal defect (VSD) (DORVncVSD) represents the most extreme form of DORV, raising challenging surgical difficulties for biventricular repair. The considerable distance between the VSD and the aorta is primarily because of the very abnormal location of the aorta. The definition of DORVncVSD includes: (1) a VSD distant (greater than aortic diameter) from both arterial valves; (2) both great vessels arising fully from the right ventricle; and (3) a double conus. Double outlet right ventricle with noncommitted ventricular septal defect is a primitive right ventricle, as seen during embryologic development, characterized by the presence of the entire conotruncus from the right ventricle. One surgical technique for repair of DORVncVSD is rerouting of the VSD to the aorta by a long intraventricular tunnel. This technique is limited by the presence of conal tricuspid chordae and by the distance between the tricuspid and pulmonary valves, and is associated with an important risk of subaortic obstruction. Rerouting through the pulmonary artery followed by arterial switch seems a more satisfactory surgical solution. When the VSD is distant from the aorta, it is almost always quite close to the pulmonary artery. Rerouting to the pulmonary artery creates a smaller channel, and its application is not limited by the presence of tricuspid chordae or the tricuspid-to-pulmonary valve distance. However, the arterial switch frequently involves relocating complex coronary arteries.