• M Aoki, J M Forbess, R A Jonas, J E Mayer, and A R Castaneda.
• Department of Cardiovascular Surgery, Children's Hospital, Boston, MA 02115.
• J. Thorac. Cardiovasc. Surg. 1994 Feb 1; 107 (2): 338-49; discussion 349-50.

AbstractThe choice of optimal repair for many patients with double-outlet right ventricle continues to challenge the heart surgeon. We present the results of a 10-year surgical experience with the biventricular repair for double-outlet right ventricle with situs solitus and atrioventricular concordance. Preoperative anatomic findings within this population of 73 patients are detailed. These morphologic features are correlated with type of anatomic repair and clinical outcome. Patients were classified by ventricular septal defect location. Normal coronary anatomy was found in the majority of patients with subaortic and doubly-committed ventricular septal defects. Patients with subpulmonary and noncommitted ventricular septal defects had a wide variety of coronary anatomy. Patients with subpulmonary and noncommitted ventricular septal defects also had a considerably higher prevalence of aortic arch obstruction. A tricuspid-to-pulmonary annular distance equal to or greater than the diameter of the aortic annulus was found to indicate the possibility of achieving a conventional ventricular septal defect-to-aorta intraventricular tunnel repair. Tricuspid-to-pulmonary annular distance sufficient for intraventricular tunnel repair predominates in those patients with a right posterior or right side-by-side aorta. Five types of repair were used during the study period: intraventricular tunnel repair, arterial switch with ventricular septal defect-to-pulmonary artery baffle, Rastelli-type extracardiac conduit repair, Damus-Kaye-Stansel repair, and atrial inversion with ventricular septal defect-to-pulmonary artery baffle. Overall actuarial survival estimate at 8 years is 81%. The presence of multiple ventricular septal defects and patient weight lower than the median were nearly significant risk factors for early mortality (p < 0.06). Nineteen patients (26%) required 24 reoperations. Patients with subaortic ventricular septal defects were significantly reoperation free (p < 0.05). Patients with noncommitted ventricular septal defects were at significantly higher risk for reoperation during the study period (p < 0.05). The prevalence of late right or left ventricular outflow obstruction in the nonsubaortic groups is concerning. The median age at repair in this series was 0.76 years, and there was a nonsignificant trend (p = 0.13) for early mortality in patients younger than 1 year of age. These patients tended to have other serious cardiac anomalies associated with double-outlet right ventricle that necessitated early operation. On the basis of these data, we favor early repair for double-outlet right ventricle if possible.

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