Transaortic repair of double-outlet right ventricle with situs versus, l-loop, l-malposition (I,L,L), subaortic ventricular septal defect, and associated anomalies.

Successful total correction of a rare form of double-outlet right ventricle (I.L.L.) is reported wherein transaortic construction of the ventricular baffle was used. Surgical approach to the defect was unsatisfactory through a right ventriculotomy. Excellent exposure was achieved through the aortic valve. Correction of partial anomalous venous return from the left lung, pulmonary debanding, and repair of the pulmonary artery were also done. Double superior venae cavae made special venous cannulation necessary. Cardiac catheterization at one year documented successful repair. This case illustrates the usefulness of the transaortic approach to a ventricular septal defect when repair through a ventriculotomy is difficult or impossible.