Modern Concepts of Fibromuscular Dysplasia of the Coronary Arteries

Abstract

Fibromuscular dysplasia of the coronary arteries is a rare non-atherosclerotic and non-inflammatory vascular lesion that is asymptomatic until serious complications develop: stenosis, dissection, rupture, sudden cardiac death. Since there are no long-term numerous clinical observations of patients with fibromuscular dysplasia of the coronary arteries, recommendations have not been developed for diagnosing and treating the disease, which often manifests with acute coronary syndrome. In 2014, the European Consensus was published, and in 2019, the first international consensus document on the diagnosis and treatment of fibromuscular dysplasia with lesions of vessels from different regions (renal, cerebrovascular, coronary, and others). The documents state that the development of fibromuscular dysplasia of the coronary arteries considers the participation of the PHACTR1 gene mutation and the transcriptional activity of the EDN1 gene, smoking, prolonged exertion of the vascular wall, and possibly female sex hormones. In the case of acute coronary syndrome, the most informative diagnostic method is computed tomography with angiography, which reveals a smooth narrowing of the lumen in the middle or distal section in the epicardial artery, often due to intramural hematoma, and also finds dissection, spasm, and tortuous vessel. Additional diagnostic methods ‒ intravascular ultrasound and optical coherence tomography allow differentiation of fibromuscular dysplasia with atherosclerosis of the coronary artery, vasculitis, and other diseases. The choice of treatment tactics for fibromuscular dysplasia of the coronary arteries depends on the severity of the clinical manifestations ‒ conservative medical treatment and interventional methods are used.