Surgical treatment of double outlet right ventricle

From 1980 until January 1991, 76 patients with double outlet right ventricle, aged from 1 year 7 months to 23.5 years (mean age 7.5 +/- 5.0 years) underwent surgery at the A. N. Bakoulev Institute for Cardiovascular Surgery. Associated heart defects were found in 77.6% patients. In 57 patients (75%), the left ventricle opened into the subaortic infundibulum; into the subpulmonary infundibulum in four (the Taussig-Bing anomaly) while, in 12 (15.8%), it opened under the outflow tracts of both great arteries. In two cases, (2.6%) there was a non-committed ventricular septal defect. In one case (1.3%) a non-committed defect was diagnosed as coexisting with a Taussig-Bing anomaly. There were eight operative deaths (10.9%) among the patients undergoing radical surgery Another three patients with the Taussig-Bing anomaly and obstructive pulmonary vascular disease underwent a successful palliative Mustard operation. All deaths were associated with acute heart failure and were seen only among patients with subaortic ventricular septal defects. The important factors, which increased the risk of correction and contributed to death were inappropriate pre-operative diagnosis of complex associated defects and technical mistakes during surgical intervention, the latter due to inadequate surgical experience. During the last five years, mortality decreased significantly (one death among 34 patients undergoing surgery from 1986 through January 1991).