Journal of Rare Cardiovascular Diseases

Fibromuscular dysplasia (FMD) is a  nonatherosclerotic, noninflammatory arterial disease of unknown etiology. Histologically, FMD is classified on the basis of arterial layer involved into intimal, medial and perimedial types. The medial form represents approximately 80% of the cases of FMD, perimedial and intimal forms are rare and account for 10% and 5% of the cases respectively. Clinically, FMD can manifest as arterial stenosis, occlusion, aneurysm or dissection. Coronary artery manifestations of FMD are rare but potentially fatal. Most cases present as a dissection of the involved coronary artery or its branch, which clinically leads to unstable angina, myocardial infarction, left ventricular dysfunction, or potentially sudden cardiac death. Various recent studies have established FMD as the leading risk factor for Spontaneous Coronary Artery Dissection (SCAD). We present autopsy findings of a 71 year old woman who suffered a spontaneous dissection of the posterior descending artery secondary to FMD‑intimal type. The optimal diagnostic and treatment guidelines for SCAD secondary to FMD have not been established due to the lack of evidence. Further research into the pathogenesis, molecular and cellular biology, epidemiology and clinical management of FMD and its coronary artery manifestations is required. JRCD 2017; 3 (2): 56–58

rare cardiovascular disease; coronary artery disease; pathology; posterior descending artery; chest pain

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