Surgical results in patients with double outlet right ventricle: A 20-yearexperience

Background. The objective of this study was to review our surgical strategy in children with double outlet right ventricle and to assess risk factors for early and late mortality and reoperation. Methods. Patients (n = 124; June 1980 to January 2000; age range, 7 days to 16 years; mean, 2.8 years) who underwent repair of double outlet right ven tricle. The patients were divided into three groups. Group 1 (n = 47) had n oncomplex patients with atrioventricular concordance, a single ventricular septal defect, balanced ventricles, no straddling atrioventricular valves, and no major pulmonary artery anomalies. Group 2 (n = 39) included patients with double outlet right ventricle and a subpulmonary ventricular septal d efect (Taussig-Bing,). Group 3 (n = 38) had patients with complex anomalies including straddling atrioventricular valves, atrioventricular septal defe cts or a hypoplastic valve or ventricle, or a combination of atrioventricul ar septal defects and hypoplastic valve or ventricle. Results. Four types of definitive repairs were performed: (1) intraventricu lar tunnel repair with a baffle from the left ventricle to the aorta (n = 5 3); (2) use of a valved or nonvalved conduit (n = 20); (3) arterial switch operation with a patch committing the left ventricle to the neo-aorta (n = 16); and (4) cavopulmonary shunt and Fontan procedures (n = 33). Two patien ts with late postoperative cardiomyopathy had heart transplantation. Potent ial risk factors included location of the largest ventricular septal defect , presence of additional ventricular septal defects, ventricular outflow ob struction or hypoplasia, or both ventricular outflow obstruction and hypopl asia, previous palliation, and type of definitive operation. There were six early deaths (4.8%) and four late deaths (3.2%), and two heart transplants (1.6%). Overall 15-year survival was 95.8%, 89.7%, and 89.5% for groups 1, 2, and 3, respectively (p = 0.08). Thirteen patients (11.4%) have required 15 reoperations. Mean follow-up for survivors was 76.6 +/- 52.8 months. Up -to-date follow-ups are available on 114 surviving patients. Ninety-five of these patients (83.3%) were in New York Heart Association class I, and the remaining 19 patients (16.7%) were in New York Heart Association class II. Freedom from reoperation was 87%, 72%, and 100% at 15 years for groups 1, 2, and 3, respectively (p = 0.11). Conclusions. Survival was high for all patients with double outlet right ve ntricle undergoing intraventricular tunnel repair, arterial switch operatio n, and repair with a conduit or a modified Fontan procedure. Careful attent ion to preoperative anatomy dictates the best surgical approach and will en hance outcomes. (C) 2001 by The Society of Thoracic Surgeons.