BACKGROUND: Fibromuscular dysplasia (FMD) of coronary arteries has been described pathologically as a cause of myocardial infarction but has not been identified ante-mortem. Unlike renal artery FMD, its clinical manifestations remain poorly characterized.

METHODS: We describe demographic, clinical and coronary angiographic characteristics of a case series of patients with acute coronary syndromes (ACS), distinctive coronary artery abnormalities, and classical features of renal artery FMD.

RESULTS: Seven female caucasians, age 42-56, presented with ACS; none was diabetic, only 2 were smokers, 2 had hypertension and 1 had hypercholesterolemia. There were unusual angiographic features common to all seven patients. In each case a well-demarcated, abrupt transition to diffuse obliterative disease was observed in the culprit vessel. In 6 of the cases the abnormal appearance continued distally for the remainder of the epicardial vessel. In no case was revascularization feasible. Unlike severe diffuse atherosclerotic disease, all other coronary segments including the proximal culprit vessel were angiographically normal. The left anterior descending artery was involved in 6, the right posterior descending artery in 1. Ventricular dysfunction, if present, was mild. All seven patients had typical angiographic features of renal FMD, 3 bilaterally.

CONCLUSION: 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 atherosclerosis. We propose that these appearances in the epicardial arteries, previously un-described in vivo, represent coronary artery FMD.

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