Corrective surgery of double outlet right ventricle: an analysis of 27 cases

양대동맥 우심실 기시증 [Double Outlet Right Ventricle] 의 전교정술 -27례 분석-

Twenty-seven patients with double-outlet right ventricle underwent complete intracardiac repair between 1978 and 1983, June, at Seoul National University Hospital . Although definite aorto-mitral discontinuity was discovered in 20 patients, both great arteries arose wholly or mostly from the right ventricle in all cases. There were 17 cases with subaortic VSD, 6 with subpulmonic, 2 with doubly-committed, and 2 with non-committed VSD. Pulmonary stenosis was present in 21 patients. Intraventricular baffle repair was applied in 23 patients. Three patients required extracardiac conduit to establish continuity between right ventricle and pulmonary artery, and modified Fontan operation was performed in one patient. Over-all mortality rate was 37.0%, but recently 4 of 15 died [26.7%]. One late death occurred from infective endocarditis. Incremental risk factors were small patient size, subpulmonic or non-committed VSD, presence of PS, coronary artery anomalies, associated valvular lesion and other complicated anomalies. However, great artery relationship, restrictive VSD and transannular patch were not risk factors. No instances of complete heart block occurred. Of the survivors, all showed complete or in complete right bundle branch block and in one patient intermittent ventricular tachycardia had developed. The important cause of death was low cardiac output syndrome.