Congenital coronary artery-cardiac chamber fistulae: a study of fourteen cases.

ABSTRACT

BACKGROUND: Congenital coronary artery fistula is a rare heart defect. Symptoms of the patient and characteristics of the fistula were investigated and the role of surgical treatment was evaluated. METHODS: Fourteen patients with congenital coronary artery fistulae were diagnosed here between January, 1983, and December, 1991. Only one patient was asymptomatic, but diagnosis was suspected from clinical presentation of a continuous murmur. In the 13 symptomatic patients, angina and exertional dyspnea were the most common symptoms. Congestive heart failure and subacute bacterial endocarditis were rare complications. Cardiac catheterization with selective coronary angiography was performed in all of these patients. The left anterior descending coronary artery was most commonly involved in this series. Findings, different from those in other literature, were the right coronary artery has more usually been reported. Fistulous communication was most often found in the pulmonary artery, right ventricle and right atrium. The mean ratio of pulmonary-to-systemic flow was 1.67 in 10 patients, and the shunt flow of another 4 accidentally diagnosed patients was trivial. Six patients, including all 4 patients, age < 20 years at diagnosis, underwent surgical correction; only 1 patient was not given an aid of cardiopulmonary bypass. RESULTS: The six patients who received surgical treatment, one died of pneumonia and respiratory failure and the other five patients had no evidence of residual fistula. All five were asymptomatic during a mean follow-up period of five years. The symptoms were slowly progressive, and functional status worsened in two who refused operation and in two high risk patients. In four patients with accidentally diagnosed small fistulae, the symptoms remained unchanged during six years of follow-up. CONCLUSIONS: The objective of surgical treatment was closure of the fistula tract without compromise of normal flow through the coronary artery. Surgical intervention was recommended for all symptomatic patients and asymptomatic young patients as well, to prevent the development of fistula-related complications.