Congenital coronary artery- cardiac chamber fistula. Review of operative management.

Eight patients who had surgical correction of coronary artery-cardiac chamber fistula at our center and 163 from a review of the literature are presented. The patients are usually asymptomatic, and the diagnosis is suspected by observing a continuous cardiac murmur. Electrocardiographic findings are nonspecific. Angina pectoris or electrocardiographic evidence of severe ischemia are surprisingly uncommon since coronary artery steal syndrome is also rare. Cardiac catheterization with angiocardiography is required to establish the diagnosis and identify the involved coronary artery and the cardiac chamber into which the fistula terminates. Left-to-right shunt flow is usually low (average Qp/Qs = 1.5). Indications for operation are not precise. If there should be a large shunt flow (2.0) and symptoms of heart failure are present, the decision to operate is clearly justified. This situation is unusual, and operation is nearly always performed in an asymptomatic patient in whom the fistula is closed to prevent future symptoms or complications. The operation chosen is generally interruption of the fistula by direct ligation. Sometimes cardiopulmonary bypass is required. The results are good, with low morbidity (3.6% myocardial infarction) and low mortality (2%) justifying the operation, to be carried out prophylactically even in asymptomatic patients.