Artery fistula causing aortic regurgitation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

We report a case of aortic regurgitation (AR), coronary artery-to-pulmonary artery (CAPA) fistula, pulmonary atresia with ventricular septal defect (PA/VSD), and major aortopulmonary collateral arteries (MAPCAS). As a result of coronary steal and AR, myocardial ischemia and ventricular dysfunction occurred. When the patient was 2 months old with a body weight of 2.7 kg, we performed fistula ligation, aortic valvuloplasty, unifocalization of the MAPCAS, and right ventricle-to-pulmonary artery shunting. After the operation, the AR volume reduced, and the patient was scheduled for repair.