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Medical management of aortic disease in Marfan syndrome
Syed Usman Bin Mahmood1, Camilo A Velasquez1, Mohammad A Zafar1
1Aortic Institute at Yale-New Haven Hospital, Yale University School of Medicine, New Haven, CT, USA.
Abstract:
Marfan syndrome (MFS) is a hereditary disorder with numerous pathophysiological effects, some specifically creating elastic dysfunction in cardiovascular organs. Aortic dilatation, dissection and rupture are major concerns in the management of MFS patients. Predilection to form aneurysms is an indication for prophylactic medical management of thoracic aortic aneurysm disease in these patients. The current guidelines describe β-blockers as the standard of care with angiotensin receptor blockers (ARBs) emerging as an equal, if not better alternative. We elaborate current evidence for and against different medical regimens used for the medical management of MFS patients.
Insights
Marfan syndrome patients require medical management for thoracic aortic aneurysms. Beta-blockers are standard, but angiotensin receptor blockers may be a better alternative for managing aortic disease in Marfan syndrome.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Pharmacology
Background:
- Marfan syndrome (MFS) is an inherited connective tissue disorder.
- MFS commonly causes cardiovascular complications, particularly affecting the aorta.
- Aortic dilatation, dissection, and rupture are life-threatening risks in MFS patients.
Purpose of the Study:
- To review current evidence on medical management of thoracic aortic aneurysm disease in Marfan syndrome.
- To compare the efficacy of beta-blockers and angiotensin receptor blockers (ARBs) in MFS patients.
Main Methods:
- Literature review of current evidence.
- Analysis of clinical guidelines and research studies on MFS medical management.
Main Results:
- Beta-blockers are the established standard of care for MFS-related aortic disease.
- Angiotensin receptor blockers (ARBs) show comparable or superior efficacy to beta-blockers.
- Evidence supports ARBs as a potential alternative or adjunct therapy.
Conclusions:
- Medical management is crucial for preventing aortic complications in Marfan syndrome.
- ARBs represent a promising therapeutic option for MFS patients with thoracic aortic aneurysm disease.
- Further research may refine treatment guidelines for optimal patient outcomes.
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