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Angiotensin Receptor Blockers and Beta-Blockers in Marfan Syndrome
Quick Takes
- Angiotensin receptor blockers slowed aortic root growth rate significantly as compared to control therapy in patients with Marfan syndrome.
- Patients with Marfan syndrome showed similar aortic growth rate when treated with angiotensin receptor blockers and beta-blockers.
- Patients with Marfan syndrome may benefit from combination therapy with angiotensin receptor blockers and beta-blockers.
Study Questions:
What is the effect of angiotensin receptor blockers (ARBs) and beta-blockers (BBs) for patients with Marfan syndrome?
Methods:
The authors performed an individual patient data meta-analysis of randomized trials comparing ARBs versus control or ARBs versus BBs. The authors focused on patients who were presurgical to assess the effect of ARBs and BBs on the primary endpoint of annual rate of change of aortic root dimension, adjusted for body surface area.
Results:
The study included 1,442 patients from seven randomized trials, of which four trials (676 patients) compared ARBs with control. During a median follow-up of 3 years, ARB use was associated with half the annual rate of change in aortic root Z score size (mean annual increase 0.07 for ARB vs. 0.13 for control, p = 0.012). In a prespecified secondary analysis, the effect of ARB was large for those with fibrillin-1 pathogenic variant compared to those without the variant (heterogeneity p = 0.005). Three trials involved 766 patients to compare an ARB vs. BB. During a median follow-up of 3 years, the change in aortic size was similar between both groups (annual increase -0.08 in ARBs vs. -0.11 in BBs, p = 0.48).
Conclusions:
The authors concluded that in patients with Marfan syndrome and no prior aortic surgery, ARBs reduced the rate of aortic root size increase by about one half as compared to control. The effects of BBs were similar to those of ARBs.
Perspective:
Aortic dissection is one of the most feared cardiovascular complications for patients with Marfan syndrome. While anti-impulse therapy with BB has been proposed to reduce aortic root growth rate, ARB therapy has also been proposed to benefit patients by attenuating transforming growth factor-beta (TGF-beta) activity. This individual patient data meta-analysis pooled data from several randomized trials comparing ARB therapy to either control or BB therapy. Overall, ARB therapy (with or without concurrent BB therapy) significantly slowed aortic root growth as compared to control. When ARB therapy was compared directly to BB therapy, there was a similar rate of aortic root growth. For patients with Marfan syndrome, especially those with fibrillin-1 pathogenic variants, ARB therapy should be first-line treatment with the option of including BB therapy concurrently in patients who can tolerate combination therapy. While it is unclear if ARB and/or BB therapy benefits extend to patients with aortic dilation unrelated to Marfan syndrome, both therapies provide important blood pressure control and should be used whenever tolerated.
Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Valvular Heart Disease, Vascular Medicine, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine
Keywords: Adrenergic beta-Antagonists, Aneurysm, Dissecting, Angiotensin Receptor Antagonists, Blood Pressure, Cardiac Surgical Procedures, Diagnostic Imaging, Dilatation, Fibrillin-1, Heart Defects, Congenital, Heart Valve Diseases, Marfan Syndrome, Transforming Growth Factor beta, Vascular Diseases
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