Form 1980 until January 1991, 76 patients with double outlet ventricle, aged from 1 year 7 months to 23.5 years (mean age 7.5± 5.0 years) underwent surgey at the A.N. Bakoulev Inste for Cardivascular Surgery. Associated heart defects were found in 77.6% patints. In 57 patients (75%), the left ventricle opened into the subaortic infundibulum; into the subpulmonary infudibulum in four (the Tassisg-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 mno-committed ventricular septact. In one case (1.3%)a non-committed defect was diagnosed as coexisting with a non-commited defect was dignosed as coexisting with a Taussig-Bing anomaly. There were eight operative deaths (10.9%) among the patients undergoing radical surgery.Another theree patients with the Taussig-Bing anomaly and obstructive pulmomnary vascular disease underwent a successful palliative Mustard operation. All deaths were associated with acute heart failure and were seen only among patiens 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 defecrs and techmical mistakes during surgical intervention, the latter due to inadequate surgical experience. During the last five years, mortality decreased significatly (one death among 34 patients undergoing surgery from 1986 through January 1991)