Spontaneous coronary artery dissection secondary to intimal fibromuscular dysplasia (RCD code: I‑1C.O)

Tariq Hamza, Francis Sharkey

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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


Slovut DP, Olin JW. Fibromuscular dysplasia. New Eng J Med 2004; 350: 1862–1871.

Saw J, Aymong E, Mancini GB, et al. Nonatherosclerotic coronary artery disease in young women. Can J Cardiol 2014; 30: 814–819.

Olin JW, Froehlich J, Gu X, et al. The United States Registry for Fibromuscular Dysplasia: Results in the first 447 patients. Circulation 2012; 125: 3182–3190.

Olin JW, Gornik HL, Bacharach JM. Fibromuscular dysplasia: State of the science and critical unanswered questions: A scientific statement from the American Heart Association. Circulation 2014; 129: 1048–1078.

Saw J, Aymong E, Sedlak T, et al. Spontaneous Coronary Artery Dissection: Association With Predisposing Arteriopathies and Precipitating Stressors and Cardiovascular Outcomes. Circ Cardiovasc Interv 2014; 7: 645–655.

Plouin PF, Perdu J, La Batide‑Alanore A, et al. Fibromuscular Dysplasia. Otphanet J Rare Dis 2007; 2: 28.

Bigazzi R, Bianchi S, Quilici N, et al. Bilateral fibromuscular dysplasia in identical twins. Am J Kidney Dis 1998; 32: 4.1–4.3.

Kadian‑Dodov D, Gornik HL, Gu X, et al. Dissection and aneurysms in patients with fibromuscular dysplasia. J Am Coll Cardiol 2016; 68: 175–185.

Saw J, Ricci D, Starovoytov A, et al. Spontaneous coronary artery dissection: prevalence of predisposing conditions including fibromuscular dysplasia in a tertiary center cohort. JACC Cardiovasc Interv 2012; 6: 44–52.

Michelis KC, Olin JW, Kadian‑Dodov D, et al. Coronary artery manifestations of fibromuscular dysplasia. J Am Coll Cardiol 2014; 64: 1033–1046.

DOI: http://dx.doi.org/10.20418%2Fjrcd.vol3no2.275


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