Coronary artery involvement in fibromuscular dysplasia (FMD): a review

This report reviews the current progress in understanding of clinical manifestation, epidemiology, diagnosis and treatment strategies in fibromuscular dysplasia (FMD) involving coronary arteries. FMD involving the coronary arteries is believed to be rare but it may lead to serious, life-threating consequences including acute myocardial infarction or sudden cardiac death. The most common coronary manifestations are presented by irregular stenosis, arterial enlargement, dissection, distal tapering, smooth narrowings, and excessive coronary tortuosity with additional markers of tortuosity leading to spontaneous coronary dissection in some cases. The etiology of the disease is poorly understood but presumably the pathogenesis of FMD is associated with combination of genetic and environmental factors. There is limited data on FMD with coronary involvement, management and treatment of the disease, but many acute vascular lesions may heal spontaneously and conservative approach is preferred. The pharmacological management with antihypertensive drugs, especially in patients with concomitant hypertension, and antiplatelet therapy should be considered in patients with confirmed FMD.