Sudden death due to rupture of major aortopulmonary collateral arteries in a patient with tetralogy of Fallot and pulmonary atresia

We report a case of fatal mediastinal hemorrhage in a 20-year-old man caused by rupture of the major aortopulmonary collateral arteries (MAPCAs). The patient had an unoperated tetralogy of Fallot with pulmonary atresia. Plain chest radiograph and computed tomography showed a large hematoma in the upper mediastinum. Previous cardiac angiography, performed 6 years previously, demonstrated markedly tortuous vascular structures arising from the inferior aspect of the aortic arch. In this case, the mediastinal hemorrhage was thought to be due to rupture of the MAPCAs. If a patient presents with sudden cardiac arrest and mediastinal dilatation, and unoperated congenital heart disease is suggested by a plain chest film, the radiologist should consider the possibility, although rare, of mediastinal hemorrhage resulting from rupture of the MAPCAs.