A 23-year-old patient with neurofibromatosis associated with acute myocardial infarction, vasospasm and a coronary artery ectasis

Abstract

We treated a 23-year-old male with neurofibromatosis with acute myocardial infarction. Cardiac catheterization revealed severe organic stenosis in the left anterior descending artery, an ectasic left circumflex artery and a small right coronary artery. Percutaneous transluminal coronary angioplasty (PTCA) was performed on the stenosis. In follow-up catheterizations, intracoronary administration of ergometrine induced vasospasm of the left coronary artery including at the PTCA site. An I123 metaiodobenzylguanidine (MIBG) scintigram showed defects in the septum, inferior wall and apex. These findings suggest abnormality of the cardiac sympathetic nerve in neurofibromatosis.