[A case of coronary artery-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCA)].

UNLABELLED: A case of four year old girl with tetralogy of Fallot, pulmonary atresia, major aortopulmonary collateral arteries and coronary artery to pulmonary artery fistula is reported. The patient was misdiagnosed as truncus arteriosus, pulmonary stenosis and major aortopulmonary collateral arteries (MAPCA) preoperatively. The ligation of MAPCAs was carried out through left thoracotomy as an initial procedure, because we believed that the area fed by MAPCAs had dual blood supply. However, the patient demonstrated pulmonary infarction post operatively. This complication forced us to postpone an intracardiac repair. At the time of intracardiac repair, after mentioned diagnosis was established and a Rastelli type operation and ligation of coronary-pulmonary artery fistula were carried out. A 18 mm Carpentier-Edwards valved conduit was used as an extracardiac conduit.

IN CONCLUSION: 1) We experienced a rare association of coronary artery fistula in TOF with PA. 2) The MAPCAs functioning as a sole source of blood-supply to the lung should be precisely diagnosed preoperatively and uniforcalized before an intracardiac repair.