Article Abstract

Medical management of aortic disease in Marfan syndrome

Authors: Syed Usman Bin Mahmood, Camilo A. Velasquez, Mohammad A. Zafar, Ayman A. Saeyeldin, Adam J. Brownstein, Bulat A. Ziganshin, John A. Elefteriades, Sandip K. Mukherjee

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.

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