SURGICAL CORRECTION OF COMBINED DOUBLE-OUTLET RIGHT VENTRICLE AND TAUSSIG-BING SYNDROME

Total surgical correction of a Taussig-Bing type double outlet right ventricle (DORV) was successfully performed in a severely cyanotic 3-year-old girl. The malformation was associated with bilateral conus, d-transposition of the great arteries, d-loop, and a subpulmonary ventricular septal defect (VSD) without significant pulmonary stenosis in situs solitus. It was impossible to create a tunnel repair by resecting the markedly hypertrophied muscular conus that separated the aortic valve from the subpulmonary ventricular septal defect. Therefore, the VSD was repaired with a Dacron patch, transforming the double outlet right ventricle into a transposition, after which total correction was achieved by means of a Mustard procedure.