Which of the following statements about the surgical repair of double-outlet right ventricle are true?

A. In double-outlet right ventricle with a subaortic or doubly committed VSD, a tunnel-type repair connecting a committed VSD with its respective great artery is usually employed.

B. Repair of the Taussig-Bing malformation can be accomplished using an intraventricular tunnel technique or by performing a straight patch closure of the VSD combined with an arterial switch procedure.

D. Some hearts with double-outlet right ventricle and a noncommitted VSD must be repaired using a modification of the Fontan procedure. 

DISCUSSION: When the VSD is subaortic or doubly committed, the tunnel-type repair connects the left ventricle via the VSD and tunnel to the aorta. The Taussig-Bing malformation can be repaired using an intraventricular tunnel technique described by Kawashima. This repair can best be accomplished when the great arteries are in a more or less side-by-side relationship with the aorta to the right of the pulmonary artery. The infundibular septum is generously resected and the VSD is connected to the aorta by an intraventricular tunnel that runs posterior to the pulmonary artery. The most common approach for the repair of the Taussig-Bing malformation involves patch closure of the VSD to the pulmonary artery. This creates transposition of the great arteries with an intact interventricular septum. An arterial switch procedure then establishes ventriculoarterial concordance. Of all the types of double-outlet right ventricle the hospital mortality is lowest when a subaortic or doubly committed VSD is present. Double-outlet right ventricle is associated with a noncommitted VSD in approximately 10% of patients in surgical series. The repair of this subset of patients is associated with a relatively high mortality, as compared with the results obtained after repair of other forms of double-outlet right ventricle. At times, because of the remote location of the VSD and because of other compelling anatomic features, complete repair cannot be performed. In this case, a modification of the Fontan procedure must be employed.