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Published on: January 15, 2022
Coronary artery manifestations of fibromuscular dysplasia
Katherine C Michelis1, Jeffrey W Olin1, Daniella Kadian-Dodov1
1Zena and Michael A. Wiener Cardiovascular Institute, and Cardiovascular Research Center, Icahn School of Medicine at Mount Sinai, New York, New York.
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.
Insights
Fibromuscular dysplasia (FMD) affecting coronary arteries is rare but serious, potentially causing heart attacks or sudden death. Diagnosis requires identifying FMD in other arteries, with conservative treatment often preferred due to spontaneous healing.
Area of Science:
- Cardiology
- Vascular Medicine
- Genetics
Background:
- Fibromuscular dysplasia (FMD) is an uncommon condition affecting arteries.
- Coronary artery involvement in FMD can lead to severe cardiac events.
- Diagnosis requires identifying FMD in non-coronary arterial territories.
Purpose of the Study:
- To review the clinical course of coronary artery FMD.
- To provide guidance on diagnosis and treatment strategies for coronary FMD.
Main Methods:
- Review of clinical cases and existing literature on coronary artery FMD.
- Analysis of diagnostic criteria and treatment outcomes.
Main Results:
- Coronary FMD manifestations differ from other arteries, lacking the "string of beads" sign.
- Potential presentations include spontaneous coronary artery dissection, smooth narrowing, intramural hematoma, spasm, or tortuosity.
- Many FMD lesions demonstrate spontaneous healing, supporting a conservative management approach.
Conclusions:
- Coronary artery FMD is an important diagnosis associated with significant cardiac risks.
- Diagnosis is confirmed by FMD in other arterial beds.
- A conservative treatment strategy is generally recommended due to the potential for spontaneous lesion healing.
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