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Congenital coronary artery fistulas. Diagnostic and surgical considerations.

Between 1974 and April, 1988, 9 patients with congenital coronary artery fistulas underwent surgical repair. Of these, 4 were female and 5 male, ranging in age from 6 to 50 years (mean 28.2 +/- 18.9). Seven patients were symptomatic (congestive heart failure and/or angina), whereas in 2 patients the diagnosis was established through the investigation of an asymptomatic continuous murmur. The origin of the fistula was the left main coronary artery in 1, left anterior descending artery (LAD) in 2, circumflex artery (Cx) in 3, Cx + LAD in 2 and LAD + right coronary artery in 1 patient. The sites of termination of the fistulous tract were the pulmonary artery in 6, the right atrium in 1, the right ventricle in 1 and the left ventricle in 1 patient. In 2 cases the fistulous tracts were ligated without utilizing cardiopulmonary bypass (CPB). In 7 cases CPB was instituted and in 6 of these the fistulous communications were closed from within the termination chamber, in 1 patient the fistula was closed through a coronary arteriotomy during elective ventricular fibrillation. There was no operative mortality and long term follow-up was uneventful after a mean follow-up of 5.4 +/- 5.2 years, with 8 patients still completely asymptomatic. Surgical therapy is recommended for patients having coronary artery fistulas in order to prevent fistula-related complications. In those cases requiring CPB, closure of the fistula from the involved chamber only is suggested.