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Fibromuscular dysplasia

What's new

Updated 2021 AHA/ASA, 2020 CHEP, 2019 ACR and 2018 ESC/ESVS guidelines for the diagnosis and management of fibromuscular dysplasia.

Background

Overview

Definition
FMD is a noninflammatory, nonatherosclerotic arterial disease of medium-sized arteries characterized by arterial stenosis, occlusion, aneurysm, and dissection.
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Pathophysiology
The exact cause of FMD is unknown.
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Disease course
Clinical manifestations of FMD depend on the artery involved (commonly carotid, renal, vertebral) resulting in stenosis, occlusion, aneurysm, and dissection. Renal involvement presented with renovascular hypertension; carotid artery involvement symptoms included headache, pulsatile tinnitus, and dizziness; and mesenteric (celiac and mesenteric) artery involvement presented with abdominal pain, hypertension, diarrhea, nausea, vomiting, headache, hemoperitoneum, and shock.
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Prognosis and risk of recurrence
The in-hospital mortality associated with FMD is 0.74%.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of fibromuscular dysplasia are prepared by our editorial team based on guidelines from the American Heart Association (AHA/ASA 2021), the Hypertension Canada (HC 2020), the Society for Vascular Surgery (SVS 2020), the American College of Radiology (ACR 2019), the Society for Vascular Medicine (SVM/ESH 2019), the European Society of Cardiology (ESC/ESVS...
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Screening and diagnosis

Indications for testing, symptomatic patients: as per HC 2020 guidelines, Evaluate for FMD-related renal artery stenosis in patients with hypertension presenting with ≥ 1 of the following clinical clues:
significant (> 1.5 cm), unexplained asymmetry in kidney sizes
abdominal bruit without apparent atherosclerosis
FMD in another vascular territory
family history of FMD.
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  • Indications for testing (patients with RAA)

  • Indications for testing (family relatives)

  • Diagnosis

Classification and risk stratification

Classification: as per ESH/SVM 2019 guidelines, Classify arterial lesions of FMD according to angiographic appearance as focal or multifocal FMD.
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Diagnostic investigations

Renal artery imaging: as per HC 2020 guidelines, Obtain MRA or CTA to evaluate for FMD of the renal arteries.
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  • Cervicocephalic vascular imaging

  • Peripheral artery imaging

  • Imaging of other arteries

Medical management

Thromboprophylaxis: as per ESH/SVM 2019 guidelines, Consider initiating antiplatelet therapy (aspirin 75-100 mg daily) in patients with FMD, in the absence of contraindication, to prevent thrombotic and thromboembolic complications.
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Therapeutic procedures

Renal artery revascularization: as per HC 2020 guidelines, Perform renal artery angioplasty without stenting for the treatment of patients with FMD-related renal artery stenosis. Do not perform stenting unless required because of a periprocedural dissection.
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  • Carotid artery revascularization

Surgical interventions

Surgical revascularization: as per HC 2020 guidelines, Consider performing surgical revascularization in patients with complex lesions of FMD in the renal arteries less amendable to angioplasty, stenosis associated with complex aneurysm, and restenosis despite two unsuccessful attempts of angioplasty.
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Specific circumstances

Patients with ischemic stroke: as per AHA/ASA 2021 guidelines, Initiate antiplatelet therapy, control BP control, and offer lifestyle modifications for the prevention of future ischemic events in patients with a history of ischemic stroke or TIA and FMD without other attributable causes.
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  • Kidney donation

Follow-up and surveillance

Indications for referral: as per HC 2020 guidelines, Refer patients with confirmed renal FMD to a hypertension specialist.
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  • Follow-up