Abstract

Double outlet right ventricle (DORV) may be divided into subsets according to the position and commitment of the ventricular septal defect (VSD) to the great arteries. In DORV with subaortic VSD, an intraventricular tunnel repair is the recommended operation. The current hospital mortality is 5% with an actuarial survival of 83% at 15 years. DORV with doubly committed VSD should also be repaired with an intraventricular tunnel, and the surgical results are similar to those for DORV and subaortic VSD. In DORV with subpulmonary VSD, an intraventricular tunnel repair is advisable when the distance from the tricuspid to the pulmonary valve exceeds the distance from tricuspid to aortic valve. Otherwise, a spiral intraventricular tunnel or an arterial switch procedure should be considered. In DORV with doubly committed VSD, the results of surgical treatment have been less good, and alternative forms of surgical treatment require further evaluation. The surgical treatment of DORV with pulmonary stenosis and the surgical details of the intraventricular tunnel repair are discussed.

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