Introduction:
Coronary artery fistula (CAF) is an abnormal communication between a coronary artery (CA) and a cardiac chamber or a major vessel. This communication may be of congenital origin, or acquired (postoperatively after repair of congenital heart defects, after myocardial biopsy, or coronary angioplasty). Congenital CAF is a relatively rare cardiac anomaly accounting for 0.2-0.4% of all congenital cardiac defects (CHD), and may be an isolated lesion or in association with other CHD such as pulmonary atresia with intact ventricular septum, tetralogy of Fallot, hypoplastic left heart syndrome, or others (1, 2, 3). Isolated congenital CAFs are usually asymptomatic in children as only 10-20% of pediatric CAF patients may complain of symptoms such as chest pain, dyspnea, palpitations, or rarely symptoms of congestive heart failure (CHF) (1, 3-5). Complications that may develop include endocarditis, formation of an aneurysm, and rarely rupture (6-10). It is recommended that symptomatic children with CAF should undergo early repair; however, there is still no consensus about the indication and the timing of repair for asymptomatic CAFs (1, 3). Herein, we present a case of a 9-year-old boy who underwent successful surgical repair of a right coronary artery fistula to the right ventricle.