Table 1.

FMD summary

Fibromuscular dysplasia	Findings
Main characteristics
Sex	•80%-90% Female
Classification	•Arterial disease: nonatherosclerotic, not vasculitis •Focal or multifocal •Stenosis, aneurysm, dissection, or tortuosity in > 1 arterial bed
Vascular territory	•Coronary •Cerebral •Cervical (carotid or vertebral) •Renal •Other visceral (hepatic, celiac, splenic, superior, or inferior mesenteric) •Lower extremity (mainly iliofemoral) •Upper extremity
Environmental factors	•Smoking •Female hormones (theoretical, causation unestablished)
Clinical scenarios	•Uncontrolled hypertension •Migraine headaches •Pulsatile tinnitus •TIA, or stroke •Myocardial infarction (STEMI or NSTEMI) •Postprandial abdominal pain with unexplained weight loss •Bruits might be auscultated over affected arterial beds •Upper and lower extremity FMD is usually asymptomatic
Diagnosis	•Invasive angiography (gold standard) •CTA: best noninvasive modality •MRA, if CTA not available, but less accuracy for subtle luminal changes •Head-to-pelvis scanning at least once, in all patients, to screen extracardiac vascular beds (CTA of head and neck, and CTA of chest, abdomen, pelvis) •For acute coronary syndromes: limited role of coronary CTA (most distal branch-level dissection will be missed) •For patients with extracardiac FMD, routine invasive coronary angiography or coronary CTA not recommended (low yield) •Repeat scanning only for symptoms, or aneurysm surveillance •Routine screening of unaffected relatives not recommended (low yield)
Genetic screening	•Not recommended, unless family history of aortopathy or other collagen vascular disease •No specific genetic test for FMD (polygenetic inheritance suspected)
Medical therapy
Antiplatelet therapy	•Aspirin 75-100 mg daily is reasonable •DAPT for 1 year if a stent was placed for SCAD-related MI
β-Blocker therapy	•Recommended in patients with SCAD to reduce heart rate, blood pressure, and arterial sheer stress
Other antihypertensive therapies	•Recommended for management of uncontrolled hypertension
Statins	•No benefit
Heart failure therapies	•Recommended if left ventricular dysfunction or heart failure after SCAD-related MI
Migraine therapy	•Avoidance of triggers, and vasoconstrictive agents •Potential role for β-blocker therapy •Medications to abort migraine headaches, consider involving headache neurologist
Lifestyle
Diet	•No restrictions
Physical activity	•Moderate-intensity exercise •Avoid prolonged straining, heavy lifting, and high-intensity exercise in patients with SCAD •Reasonable to avoid severe neck traction and prolonged neck extension among patients with cervical FMD
Stress management	•Recommended
Smoking Cessation	•Recommended

CTA, computed tomography angiography; DAPT, dual antiplatelet therapy; FMD, fibromuscular dysplasia; MI, myocardial infarction; MRA, magnetic resonance angiography; NSTEMI, non–ST-elevation myocardial infarction; SCAD, spontaneous coronary artery dissection; STEMI, ST-elevation myocardial infarction; TIA, transient ischemic attack.