The surgical management of congenital coronary artery fistula.

Abstract

Congenital coronary artery fistula (CAF) is a very rare cardiovascular anomaly and only 400 cases have been reported. The purpose of this study was to analyse the experience of surgical treatments in CAF and evaluate the surgical indications, techniques and long-term results. A retrospective study reviewing 20,000 cardiovascular surgical cases from 1978 to 1993 was carried out, from which 14 patients with CAF were identified and diagnosis was confirmed by both coronary angiography (CAG) and echocardiography. Twelve patients had heart-related symptoms. CAG revealed that fistulous channels arose from the right coronary artery (RCA) in seven patients, and from the left coronary artery (LCA) in six patients. One patient had multiple fistulae, from both RCA and LCA. The surgical managements and late follow-up were assessed. Surgery was performed on 13 patients; one had died before surgery. Cardiopulmonary bypass (CPB) was performed in 11 patients. The presence of coronary aneurysm was treated by reduction plasty of the aneurysm (four patients). Other procedures were coronary artery bypass grafting (CABG; four patients), ventricular septal defect (VSD) and patent ductus arteriosus (PDA) closure (one patient), and one patient had mitral valve replacement. The mean follow-up time was 7.2 years and all symptomatic patients improved their New York Heart Association (NYHA) functional class (average 1.2 classes). Although there were no surgical deaths in our group, one late death (6 months after surgery) was caused by acute myocardial infarction; this resulted in an annual mortality rate of 1%. Surgical closure of CAF can be recommended because it is a low-risk procedure and leads to good long-term results. The problems with myocardial protection, residual fistula prevention and surgical techniques must be emphasized. For these purposes, special surgical techniques that have been introduced are described.