Medical management of aortic disease in Marfan syndrome.

Authors:
Mohammad A Zafar, MBBS
Mohammad A Zafar, MBBS
Aortic Institute at Yale-New Haven Hospital
Research Director
New Haven, CT | United States

Ann Cardiothorac Surg 2017 Nov;6(6):654-661

Aortic Institute at Yale-New Haven Hospital, Yale University School of Medicine, New Haven, CT, USA.

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.

Download full-text PDF

Source
http://dx.doi.org/10.21037/acs.2017.11.09DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5721117PMC
November 2017
38 Reads

Publication Analysis

Top Keywords

medical management
12
marfan syndrome
8
mfs patients
8
management mfs
8
rupture major
4
guidelines describe
4
major concerns
4
concerns management
4
arbs emerging
4
emerging equal
4
aortic dilatation
4
care angiotensin
4
equal better
4
dilatation dissection
4
dissection rupture
4
standard care
4
patients predilection
4
predilection form
4
thoracic aortic
4
management thoracic
4

Similar Publications