Myocardial infarction due to coronary abnormalities in pulmonary atresia with intact ventricular septum.

We describe the clinical course, angiography, and histopathology of a newborn male with pulmonary atresia and intact ventricular septum who succumbed to a myocardial infarction. Angiography demonstrated right ventricular-dependent coronary circulation and focal areas of coronary narrowing. His clinical course was characterized by attacks of sudden irritability, consistent with ischemia. Histology demonstrated significant coronary artery narrowing secondary to fibromuscular dysplasia as well as evidence of new and old infarction. This case illustrates the severity of coronary lesions in pulmonary atresia and the potential for progression of coronary obstruction and insufficiency, and it provides correlation between angiography, ischemic symptoms, and pathology.