Embolic myocardial infarction in a patient with a Fontan circulation.

Coronary artery embolism is an uncommon cause of acute myocardial infarction (MI). We present a patient with pulmonary atresia and severe right heart hypoplasia who underwent a lateral tunnel Fontan procedure in childhood and presented with an acute ST-segment elevation MI at 19 years of age. In addition to the known risk of thrombotic complications associated with a Fontan circulation, potential predisposing factors to thromboembolism in this patient included a right ventricle to left anterior descending coronary connection and a Fontan baffle leak. The patient was treated with device closure of the baffle leak and anticoagulation. This is one of the first reports of an embolic MI in a patient with a Fontan circulation. The optimal method of reducing thromboembolic risk in this patient, and those with a Fontan circulation in general, is complicated and no consensus exists.