Congenital coronary artery fistulae: six new cases with a collective review.

Primary congenital coronary artery fistula is diagnosed in two of every thousand patients investigated by angiocardiography. Males and females are equally affected and most frequently present under the age of 10 years. Fistulae originate from right and left coronary arteries in almost equal proportion but over 90% drain to the right side of the heart. Over half the patients present without symptoms but with a continuous murmur; as age increases so does the incidence of complications. Cardiac failure is the commonest complication. Three-quarters of patients presenting over the age of 40 have symptoms of cardiac failure. The clinical picture closely resembles that of patient ductus arteriosus but in a proportion of cases the physical signs and chest radiograph may suggest the diagnosis. A hitherto undescribed association of cardiac failure and a fistula from the left coronary artery to the left ventricle is reported. High-quality angiocardiography, preferably with selective coronary arteriography, is essential. Surgical correction carries a low mortality (2.2%) and is curative. The incidence of complications increases with age and prophylactic surgery is advocated before symptoms develop.