• Department of Surgery, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Sciences&Peking Union Medical College, Beijing 100037, P. R. China;
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Abstract: Marfan syndrome (MFS) is a congenital and heritable autosomal dominant disorder of the connective tissue which is often passed down through families. Its clinical presentation typically involves the skeletal, cardiovascular and ocular systems with a high natural mortality. Aortic root aneurysm and consecutive acute aortic dissection represent the main cardiovascular manifestations and main causes of morbidity and mortality in MFS. At present, the predominant therapeutic method is surgery, but surgical outcomes are quite unsatisfactory. Recent studies demonstrate that losartan, a common antihypertensive agent, is useful to treat MFS, the mechanism of which may results from inhibiting overactivation of transforming growth factor β (TGF-β) signaling. This discovery will definitely promote the transition of traditional surgical treatment of MFS into pharmacotherapy. In this review, we focus on the molecular biological pathogenesis, traditional and new therapeutic strategies for MFS patients. 

Citation: GONG Bing,YANG Xiubin.. Therapy Progress for Vascular Pathology of Marfan Syndrome. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2012, 19(6): 668-670. doi: Copy

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