Surgical outcome of double-outlet right ventricle with subpulmonary VSD

Background. Optimal management of double-outlet right ventricle with subpul monary ventricular septal defect remains controversial. We reviewed our 7-y ear experience with patients who had this anatomic configuration. Methods. Between January 1992 and January 1999, 20 patients underwent an ar terial switch operation (ASO group), and 12 underwent a bidirectional Glenn procedure followed by a modified Fontan in 10 (Glenn/Fontan). Mean follow- up was 23 +/- 18 months. Results. An initial palliative operation was done in 19 patients (9 in the ASO group, 10 in the Glenn/Fontan group). There were no deaths in the Glenn /Fontan group. Four patients in the ASO group died within 33 days postopera tively. Two of them had a single coronary artery, 1 had a straddling mitral valve, 1 had a hypoplastic aortic arch, and 1 had multiple ventricular sep tal defects. Three patients had reoperation for subaortic stenosis (n = 2) or pulmonary stenosis (n = 1) after the ASO. Four patients (3 in the ASO gr oup, 1 in the Glenn/Fontan) required a pacemaker for postoperative complete atrioventricular block. Actuarial survival at 5 years for the entire group was 87% (70% confidence interval, 81% to 93%). Conclusions. The ASO remains our preferred treatment for infants with doubl e-outlet right ventricle and subpulmonary ventricular septal defect. Howeve r, associated anatomic defects are important risk factors. (Ann Thorac Surg 2001;71:49-53) (C) 2001 by The Society of Thoracic Surgeons.