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Isolated congenital coronary artery fistula is a rare condition, and the surgical experience for treating this condition is limited.This was a retrospective review of 47 patients who underwent surgical repair of isolated congenital coronary artery fistula from January 2001 to March 2015.All but 1 patient presented with no symptoms. The median age at operation was 3.9 years (range, 0.4-15.2 years), and the median weight was 15.2 kg (range, 6.3-77.0 kg). Right coronary artery to right ventricle fistula was the most common pattern, which was present in 16 patients. Epicardial ligation without cardiopulmonary bypass was used to treat 4 patients, whereas cardiopulmonary bypass was used in the remaining 43 patients. Surgical techniques included transcoronary closure in 20 patients, transcameral or transpulmonary closure in 20 patients, a combination of both transcameral and transcoronary closure in 2 patients, and a conversion from transcameral closure to epicardial ligation in 1 patient. There were no deaths during our study period. Injury to the mitral valve occurred in 1 patient. Residual shunt was observed in 8 patients, 2 of which closed spontaneously during the follow-up. Extracardiac repair was associated with a higher risk of recanalization (P = .004). Freedom from recanalization was 89.4%, 85.1%, 83.0%, and 83.0% at discharge, 6 months, 1 year, and 5 years postsurgery, respectively. Two patients required transcatheter closure for residual shunts.Surgical repair of isolated congenital coronary artery fistula in pediatric patients can be performed with low mortality and morbidity. Careful evaluation after surgery is necessary to monitor the occurrence of residual shunt.