To whom should patients with fibromuscular dysplasia (FMD) be referred for evaluation and management?

Referral Recommendations for Patients with Fibromuscular Dysplasia

Patients with fibromuscular dysplasia should be referred to specialized FMD centers with multidisciplinary expertise for comprehensive evaluation and management, as this rare vascular condition requires specialized care to reduce morbidity and mortality.

Initial Referral Decision Algorithm

Primary Referrals:

1. Vascular Specialists

   • Vascular surgeons or interventional radiologists with expertise in FMD management
   • Essential for evaluation of all patients with confirmed or suspected FMD, regardless of symptoms 1

2. Specialists Based on Arterial Bed Involvement:

   • Renal FMD:

     • Nephrologist (for hypertension management)
     • Interventional radiologist (for potential angioplasty in symptomatic cases) 2

   • Cerebrovascular FMD:

     • Neurologist (for symptom evaluation and management)
     • Neurosurgeon (if aneurysms or severe stenosis are present) 2

   • Other arterial beds:

     • Cardiologist (for coronary or other cardiac-related FMD)
     • Vascular medicine specialist (for peripheral artery involvement) 1

Indications for Urgent Referral

• Symptomatic patients with:

  • Cerebrovascular symptoms (TIA, stroke, Horner syndrome, cranial nerve palsies)
  • Refractory hypertension
  • Renal dysfunction
  • Arterial dissection
  • Aneurysms
  • Subarachnoid hemorrhage 2, 3

• Patients with renal artery FMD for whom medical management has failed (refractory hypertension, worsening renal function) should be urgently referred for consideration of revascularization 2

Specialized Testing Considerations

Patients should be referred to centers capable of performing:

1. Advanced Imaging:

   • Contrast-enhanced CTA
   • MRA
   • Catheter-based contrast angiography (gold standard) 2, 4
   • Duplex ultrasound (for initial screening and follow-up) 5

2. Comprehensive Vascular Assessment:

   • FMD can affect multiple vascular beds simultaneously
   • Complete vascular screening is necessary even when initially found in one location 6, 7

Follow-up Referral Recommendations

• Annual follow-up with vascular specialists for:
  • Noninvasive imaging of affected arteries (initially annual, may be less frequent once stability is confirmed) 2
  • Monitoring of disease progression
  • Assessment for new symptoms or complications 1

Special Considerations

• Pediatric FMD patients should be referred to pediatric nephrologists and pediatric interventional radiologists with FMD expertise 5

• Asymptomatic FMD patients still require specialist referral for:

  • Antiplatelet therapy initiation
  • Surveillance imaging protocol establishment
  • Risk assessment for complications 1

• Pregnant women with FMD require referral to maternal-fetal medicine specialists in addition to vascular specialists

Common Pitfalls to Avoid

• Delayed diagnosis due to nonspecific symptoms or misattribution to other conditions
• Incomplete vascular assessment focusing only on the initially identified affected artery
• Inappropriate revascularization of asymptomatic carotid FMD (not recommended regardless of stenosis severity) 2
• Failure to consider FMD in young or middle-aged women with unexplained hypertension or neurological symptoms 6

By following these referral guidelines, clinicians can ensure that patients with FMD receive appropriate specialized care to minimize complications and optimize outcomes.