Fibromuscular dysplasia (FMD) involving the coronary arteries has been described pathologically as a cause of myocardial infarction but has not been described antemortem. Unlike renal artery FMD, its clinical manifestations remain poorly characterized. We describe demographic, clinical, and coronary angiographic characteristics of seven women with acute coronary syndromes and unusual coronary anatomy who also had renal artery FMD. All subjects were female caucasians, age 42-56, who presented with prolonged chest pain and positive troponin tests. Two were smokers, two had hypertension, and one had hypercholesterolemia. None was diabetic. There were distinctive angiographic features common to all seven patients. The left anterior descending artery was involved in six, the right posterior descending artery in one. In each case, the proximal vessel appeared normal but in the middle or distal segment there was a well-demarcated abrupt transition to diffuse obliterative disease. In six of the cases, this continued distally for the remainder of the epicardial vessel. In no case was revascularization feasible. Unlike severe diffuse atherosclerotic disease, all other coronary segments were angiographically normal. Ventricular dysfunction, if present, was mild. All seven patients had typical angiographic features of renal FMD, three bilaterally. We have observed a characteristic pattern of well-demarcated obliterative coronary artery disease associated with FMD of the renal arteries. All cases presented as acute coronary syndromes in patients at relatively low risk of coronary artery disease. We propose that these appearances in the epicardial arteries, previously undescribed ante-mortem represent coronary artery fibromuscular dysplasia.
2005 Wiley-Liss, Inc.