From the Guidelines
Fibromuscular dysplasia (FMD) is a systemic disease that affects multiple vascular beds, leading to various cardiovascular manifestations, including renovascular hypertension, cerebrovascular symptoms, coronary artery disease, and peripheral artery involvement. The most common cardiovascular manifestation is renovascular hypertension, occurring when FMD affects the renal arteries, leading to reduced kidney perfusion and activation of the renin-angiotensin-aldosterone system 1. Patients may also develop cerebrovascular symptoms like headaches, pulsatile tinnitus, and stroke when carotid or vertebral arteries are involved. Coronary artery FMD can cause chest pain, myocardial infarction, or sudden cardiac death. Other manifestations include mesenteric ischemia causing abdominal pain after eating, and peripheral artery involvement leading to claudication or critical limb ischemia.
Diagnosis and Management
Diagnosis typically involves imaging studies such as CT angiography, MR angiography, or catheter-based angiography showing the characteristic "string of beads" appearance. Management includes:
- Blood pressure control with antihypertensive medications (particularly ACE inhibitors or ARBs) 1
- Antiplatelet therapy with aspirin 81-325mg daily 1
- In severe cases, revascularization procedures like angioplasty may be necessary 1 Regular cardiovascular monitoring is essential as FMD patients have higher rates of aneurysms and dissections throughout the arterial system.
Key Considerations
- FMD can involve multiple vascular beds, including the carotid, vertebral, renal, and coronary arteries 1
- Patients with FMD are at increased risk of ischemic events, and antiplatelet therapy and blood pressure control are recommended to prevent future events 1
- Revascularization procedures like angioplasty may be necessary in severe cases, but should be performed in experienced centers due to the high risk of restenosis 1
From the Research
Cardiovascular Manifestations of Fibromuscular Hyperplasia
The cardiovascular manifestations of fibromuscular hyperplasia, also known as fibromuscular dysplasia (FMD), can be severe and varied. Some of the key manifestations include:
- Hypertension due to renal artery involvement 2, 3, 4, 5, 6
- Transient ischemic attack or stroke due to carotid or vertebral artery involvement 2, 3, 6
- Asymptomatic cervical bruit 6
- Ischemic stroke or transient ischemic attack when the disease affects the carotid or vertebral arteries 2
Treatment Options
Treatment options for FMD vary depending on the location and severity of the disease. Some of the treatment options include:
- Percutaneous transluminal angioplasty (PTA) for patients with renal artery stenosis causing hypertension 2, 3, 4, 5, 6
- Antiplatelet agents for patients with cerebrovascular FMD 3
- Surgery for patients with severe or complex disease 4, 5
- Conservative therapy for patients with asymptomatic and extensive lesions of the carotid arteries 2
Outcomes
The outcomes of treatment for FMD can be significant, with some studies showing: