Successful biventricular repair of double-outlet right ventricle with transposition of the great arteries, pulmonary stenosis, and straddling mitral valve.

A 9-month-old boy with polysplenia, double-outlet right ventricle (DORV), transposition of the great arteries (TGA), a large ventricular septal defect (VSD), straddling mitral valve, pulmonary stenosis (PS), and a normal-sized pulmonary annulus underwent ventricular septation at the right side of the straddling mitral chord as well as an en bloc truncal switch procedure to minimize reduction of right ventricular (RV) volume from the intraventricular conduit. Echocardiography 5 months postoperatively showed laminar flow without obstruction through both ventricular outflow tracts, normal ventricular function, trivial aortic regurgitation, and mild pulmonary regurgitation (PR). This procedure is a good option for biventricular repair in the presence of a straddling mitral valve.