ANATOMIC FEATURES AND SURGICAL STRATEGIES IN DOUBLE-OUTLET RIGHT VENTRICLE

Background The objective of this study was to review anatomic features and surgical strategies in children with double-outlet right ventricl e (DORV) and to assess risk factors for early mortality. Methods and R esults Records were reviewed of all children with DORV undergoing surg ery between 1978 and 1993. Noncomplex patients (group 1) had atriovent ricular (AV) concordance, a single ventricular septal defect (VSD), ba lanced ventricles, no straddling AV valves, and no major pulmonary art ery anomaly. Group 2 (complex) comprised all remaining patients. Indep endent risk factors analyzed included location of the main VSD, presen ce of additional VSDs, coarctation, ventricular outflow obstruction, v entricular hypoplasia, age at operation, operation before 1985, previo us palliation, and type of definitive operation. Of 193 patients, 117 were in group 1 and 76 in group 2. In 148 patients, biventricular repa ir was undertaken, including 111 of 117 group 1 patients and 37 of 76 group 2 patients. Early mortality was higher among group 2 patients un dergoing biventricular repair than among group 1 patients (8 of 37 ver sus 4 of 111, P<.005) and higher than group 2 patients undergoing a Fo ntan procedure (none of 29, P<.01). Aortic arch obstruction, operation before 1985, and multiple VSDs were significant risk factors for mort ality. Age <1 month (P<.05) and multiple VSDs (P<.005) were independen t risk factors after definitive repair. Up-to-date follow-up is availa ble on 144 surviving patients, with 127 (88%) in New York Heart Associ ation class I and the remaining 17 in class II. Overall 10-year surviv al probability was 81%, whereas probability of survival, free from reo peration (after definitive surgery), was 65% at 10 years. Conclusions Biventricular repair can be achieved in most patients with DORV with l ow risk. In complex DORV, a Fontan procedure is associated with a lowe r surgical mortality.