Partial biventricular repair for double-outlet right ventricle with left ventricular hypoplasia.

The presence of left ventricular hypoplasia in double-outlet right ventricle may increase the risk of biventricular repair and Fontan procedures. The hypoplastic left ventricle of an 11-year-old girl with complex double-outlet right ventricle was successfully incorporated in a modified biventricular repair by Dacron patch closure of the ventricular septal defect, diversion of venous return of the inferior vena cava to the mitral valve with an intraatrial baffle, insertion of a left ventricular apex to pulmonary artery valved aortic homograft, and diversion of residual systemic venous return directly to the lungs with a bidirectional superior vena cava to pulmonary artery shunt. Postoperatively, the systemic venous atrial pressure was 6 mm Hg and the pulmonary artery pressure was 14 mm Hg. This method is proposed as a partial biventricular repair hemodynamically superior to the Fontan procedure, although aortic homograft revision may be required in the future.