Fibromuscular dysplasia (FMD) is an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease of the musculature of arterial walls, leading to stenosis of small and medium-sized arteries, mostly involving renal and cervical arteries. As a result of better and more systematic screening, it appears that involvement of the splanchnic vascular bed is more frequent than originally assumed. We review epidemiology, pathogenesis, clinical picture as well as diagnosis and treatment of visceral artery (VA) FMD. The clinical picture is very diverse, and diagnosis is based on CT-, MR- or conventional catheter-based angiography. Involvement of VAs generally occurs among patients with multi-vessel FMD. Therefore, screening for VA FMD is advised especially in renal artery (RA) FMD and in case of aneurysms and/or dissections. Treatment depends on the clinical picture. However, the level of evidence is low, and much of the common practice is extrapolated from visceral atherosclerotic disease.
Key Points
Question
What is the prevalence of fibromuscular dysplasia, aneurysms, dissection, and tortuosity in extracoronary arteries of patients who developed a spontaneous coronary artery dissection (SCAD)?
Findings
In this case series including 173 patients with SCAD, using magnetic resonance angiography with blinded interpretation of the findings, 32% of the patients had fibromuscular dysplasia, 8% had aneurysms, and 2% had dissections; the prevalence of arterial tortuosity was similar in cases and controls. Extracoronary vascular events over a median 5-year follow-up were rare.
Meaning
The findings of this blinded analysis suggest that, in patients with SCAD, severe multivessel fibromuscular dysplasia, aneurysms, and dissections are infrequent and seldom associated with clinically evident vascular events.
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