Management and Evaluation of Suspected Fibromuscular Dysplasia (FMD)

Diagnostic Approach

The American Heart Association recommends duplex ultrasonography as an initial screening test for FMD (Class I recommendation) 1, 2
The American Heart Association recommends computed tomographic angiography and magnetic resonance angiography as screening tests for FMD (Class I recommendation) 1, 2
The American Heart Association recommends catheter angiography as the gold standard diagnostic test for FMD when clinical suspicion is high and noninvasive tests are inconclusive 1, 2
The American Heart Association does not recommend captopril renal scintigraphy, selective renal vein renin measurements, and plasma renin activity as screening tests for FMD (Class III recommendation) 2

The American Heart Association suggests considering FMD in young and middle-aged women with hypertension, particularly if difficult to control 3
The American Heart Association suggests considering FMD in patients with unexplained renal dysfunction or renal artery stenosis 4

The American College of Cardiology recommends recognizing FMD as a systemic disease requiring evaluation of multiple vascular beds, particularly renal, carotid, and vertebral arteries 1
The American College of Cardiology recommends annual noninvasive imaging of the carotid arteries to detect changes in disease extent or severity (Class IIa recommendation) 5
The American College of Cardiology recommends administration of antiplatelet medication to prevent thromboembolism (Class IIa recommendation) 5

The American College of Cardiology recommends initial management focusing on appropriate medical therapy to control blood pressure 1
The American College of Cardiology recommends RAS blockers (ACEIs/ARBs) as the drugs of choice when percutaneous intervention is not feasible, but requires careful monitoring of renal function 1
The American College of Cardiology recommends percutaneous transluminal renal angioplasty (PTRA) without stenting as the first-line revascularization technique for symptomatic renal FMD (Class IIa recommendation) 1

The American Heart Association recommends carotid angioplasty with or without stenting for patients with retinal or hemispheric cerebral ischemic symptoms related to FMD of the ipsilateral carotid artery (Class IIa recommendation) 5
The American Heart Association does not recommend revascularization for patients with asymptomatic FMD of a carotid artery, regardless of stenosis severity (Class III recommendation) 5

The American College of Cardiology recommends regular blood pressure monitoring and renal function assessment in patients with renal artery FMD 1
The American College of Cardiology recommends referring patients with FMD to specialized centers for dedicated management 1
The American Heart Association recommends avoiding treatment pitfalls such as failure to recognize FMD as a systemic disease, using stents as first-line therapy for renal FMD, and treating asymptomatic carotid FMD with revascularization 1, 5