Primary Intracardiac Repair of Double Outlet Right Ventricle and Ventricular Septal Defect without Pulmonary Stenosis

Abstract

From January 1991' to May 1994, 29 patients with double outlet right ventricle with ventricular septal defect, without pulmonary stenosis underwent primary intracardiac repair at the All India Institute of Medical Sciences, New Delhi. Patients were classified into 4 groups based on location of the ventricular septal defect. The ventricular septal defect was subaortic in 11, subpulmonary in 13, doubly committed subarterial in 1, and noncommitted in 4 patients. Surgical treatment consisted of intraventricular routing of the left ventricle to the aorta (17), and the left ventricle to the pulmonary artery followed by an arterial switch operation (12). There were 4 (13.9%) early deaths. Follow-up ranged from 3 months to 3 years (mean, 1.5 years). There was no late mortality. Three patients had residual ventricular septal defect, one of whom has undergone reoperation. One patient has a gradient of 25 mmHg across the left ventricular outflow tract. Double outlet right ventricle with subpulmonic ventricular septal defect was found to be a significant risk factor for early mortality (p = 0.03). The subgroup of double outlet right ventricle with subpulmonic ventricular septal defect who had a combination of single coronary artery and post arterial switch operation was particularly prone to pulmonary hypertensive crisis and hospital death (p = 0.002).