What are the recommended anti-hypertensive (blood pressure lowering) medications for patients with fibromuscular dysplasia?

Antihypertensive Management in Fibromuscular Dysplasia

For patients with fibromuscular dysplasia causing hypertension, percutaneous transluminal renal angioplasty (PTRA) without stenting is the primary treatment, but when revascularization is not feasible or fails, RAS blockers (ACE inhibitors or ARBs) are the drugs of choice for medical management. 1

Primary Treatment Strategy

Revascularization should be pursued first in patients with hemodynamically significant renal artery stenosis from fibromuscular dysplasia, as PTRA without stenting can restore renal perfusion pressure, lower blood pressure, and potentially cure hypertension. 1 This approach is particularly effective in younger patients with milder hypertension of shorter duration. 2

• The 2024 ESC Guidelines give PTRA without stenting a Class IIa recommendation (Level C evidence) for fibromuscular dysplasia with hemodynamically significant stenosis. 1
• Success rates for blood pressure normalization are highest when intervention occurs early, before end-organ damage develops. 3, 2
• Angioplasty alone (without stenting) is specifically recommended for fibromuscular dysplasia, distinguishing it from atherosclerotic disease where stenting may be considered. 1

Medical Therapy: When and What to Use

RAS Blockers as First-Line Medical Therapy

When PTRA is not feasible, has failed, or while awaiting intervention, RAS blockers (ACE inhibitors or ARBs) are the drugs of choice. 1 These medications directly target the renin-angiotensin system activation that drives hypertension in renovascular disease. 4

• ACE inhibitors or ARBs block the pathophysiological mechanism of hypertension in fibromuscular dysplasia by interrupting renin-mediated vasoconstriction. 5, 4
• Even after successful PTRA, combination therapy with RAS blockers is often necessary to achieve optimal blood pressure control. 4

Critical Monitoring Requirements with RAS Blockers

Careful monitoring of renal function is mandatory when using RAS blockers in fibromuscular dysplasia patients. 1

• RAS blockers can cause acute renal failure in patients with tight bilateral stenoses or a stenosed solitary functioning kidney. 1
• Monitor serum creatinine and electrolytes within 1-2 weeks of initiating therapy and regularly thereafter. 1
• If creatinine rises >30% above baseline or hyperkalemia develops, consider dose reduction or alternative therapy. 1

Systemic Nature of Fibromuscular Dysplasia

Recognize that fibromuscular dysplasia is a systemic vascular disease affecting multiple arterial beds, not just the renal arteries. 1

• Possible involvement of carotid, coronary, and other major arteries must be considered, as uncontrolled blood pressure can lead to arterial dissection. 1
• This systemic nature makes blood pressure control critically important for preventing vascular complications beyond the kidney. 1
• Target blood pressure should generally be <130/80 mmHg to reduce cardiovascular risk, though individualized based on age and comorbidities. 1

Alternative Medical Management Strategies

When RAS Blockers Cannot Be Used

If RAS blockers are contraindicated or not tolerated, standard antihypertensive therapy should follow general hypertension guidelines. 1

• Calcium channel blockers (CCBs) are effective alternatives and do not carry the same risk of acute renal dysfunction in bilateral disease. 1
• Thiazide or thiazide-like diuretics can be added for additional blood pressure control. 1
• Beta-blockers may be considered, particularly if there are other indications such as coronary artery disease. 1

Pediatric Considerations

In very young children with fibromuscular dysplasia, medical management alone may be sufficient when blood pressure is controlled and renal function is preserved. 6

• Surgical or endovascular intervention in small children can be technically challenging with potential complications. 6
• Medical stabilization with antihypertensive medications can be effective short-term, reserving intervention for medication-refractory hypertension or compromised renal function. 6
• Multiple antihypertensive agents may be required to achieve adequate control in pediatric cases. 6

Common Pitfalls and How to Avoid Them

Do not delay revascularization in favor of prolonged medical management in appropriate candidates. 3, 2 Younger patients with recent-onset hypertension have the highest cure rates with PTRA, and delaying intervention may reduce the likelihood of cure as end-organ changes develop. 2

Do not use RAS blockers without careful renal function monitoring. 1 The risk of acute kidney injury is real, particularly in bilateral disease or solitary kidney scenarios.

Do not assume fibromuscular dysplasia only affects the renal arteries. 1 Screen for and manage involvement of other vascular territories, as dissection risk increases with uncontrolled hypertension.

Do not use stents during initial PTRA for fibromuscular dysplasia. 1 Unlike atherosclerotic disease, fibromuscular dysplasia responds well to angioplasty alone, and stenting is not indicated unless there is a complication such as dissection.