Coronary Artery Manifestations of Fibromuscular Dysplasia

Abstract

Fibromuscular dysplasia (FMD) involving the coronary arteries is an uncommon but important condition that can present as acute coronary syndrome, left ventricular dysfunction, or potentially sudden cardiac death. Although the classic angiographic “string of beads” that may be observed in renal artery FMD does not occur in coronary arteries, potential manifestations include spontaneous coronary artery dissection, distal tapering or long, smooth narrowing that may represent dissection, intramural hematoma, spasm, or tortuosity. Importantly, FMD must be identified in at least one other noncoronary arterial territory to attribute any coronary findings to FMD. Although there is limited evidence to guide treatment, many lesions heal spontaneously; thus, a conservative approach is generally preferred. The etiology is poorly understood, but there are ongoing efforts to better characterize FMD and define its genetic and molecular basis. This report reviews the clinical course of FMD involving the coronary arteries and provides guidance for diagnosis and treatment strategies.

Fibromuscular Dysplasia Presenting as Spontaneous Coronary Artery Dissection

“…Some studies reported that FMD has autosomal dominant trait with variable penetrance but with poorly understood underlying genetics. Patients with coronary involvement of FMD usually present with dissection of an epicardial artery or major branch, which clinically leads to acute coronary syndrome (ACS) or potential sudden cardiac death [8]. Initial ECG findings might reflect acute ischemia with ST deviation and sometimes with elevated cardiac biomarkers [3].…”

“…FMD should be considered in any patient without cardiac risk factors presenting with ACS or new left ventricular dysfunction, especially in middle-aged women. Symptoms depend on the arterial bed affected with FMD and this is most often hypertension if the renal artery is affected, headache, or pulsatile tinnitus if cervical vessel involvement [8].…”

“…Currently, only catheter-based angiography is validated for diagnosing coronary artery involvement as no studies documented the utility of other noninvasive imaging modalities for diagnosis CFMD [8]. Intravascular Ultrasound (IVUS) and Optical Coherence Tomography (OCT) are excellent adjunctive imaging modalities for use in combination with angiography but they are not used that often unless there was difficulty in diagnosing CFMD.…”

“…Therefore, treatment based on case reports and limited case series [8]. In stable patients with no MI, conservative approach is preferred, as SCAD will typically heal spontaneously.…”

“…Statins were not proven yet to be beneficial in patients with FMD and/or dissection as per published guidelines thus their use should be with the goal of treating dyslipidemia if present [5,15]. Avoidance of strenuous physical activity and heavy weights lifting is generally advised given the rare association with SCAD [8].…”