Successful repair of double-outlet right ventricle with bilateral conus, 1-transposition of great arteries (S,D,L), and subpulmonary ventricular septal defect.

Surgical correction was carried out successfully in a severely cyanotic 3-year-old Japanese girl who had a very rare type of double-outlet right ventricle. The malformation was associated with bilateral conus, 1-transposition of the great arteries, and subpulmonary ventricular septal defect without significant pulmonary stenosis in situs solitus. A large amount of subaortic conal musculature which separated the aortic valve from the subpulmonary ventricular septal defect was removed, as was the anterior rim of the ventricular septal defect. A tunnel, constructed with a woven Teflon prosthesis, was inserted in such a manner as to direct blood from the left ventricle through the defect and out to the aorta. The pulmonary outflow tract was reconstructed with a Teflon patch lined with pericardium. The patient's postoperative recovery was uneventful, and she was doing well 3 months postoperatively. To our knowledge, no identical case with a similar type of surgical correction has previously been reported.