Cardiovascular surgery in Marfan syndrome: implications of new molecular concepts in thoracic aortic disease

Schoenhoff, Florian S; Cameron, Duke E; Matyas, Gabor; Carrel, Thierry P (2011). Cardiovascular surgery in Marfan syndrome: implications of new molecular concepts in thoracic aortic disease. Future cardiology, 7(4), pp. 557-69. London: Future Medicine Ltd 10.2217/fca.11.37

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Acute dissection and rupture of aortic aneurysms comprise for 1-2% of all deaths in industrialized countries. Dilation of the aorta is caused by a multitude of mechanisms including inherited connective tissue disorders such as Marfan syndrome (MFS). MFS is one of the most common inherited connective tissue disorders affecting 1 in 5000 individuals. Although the phenotype of MFS can be quite variable, aneurysmal dilation of the aortic root and consecutive acute aortic dissection is the leading cause of death in this patient population. Over the past years it has been shown that a comprehensive understanding of this disorder provides greater understanding of vascular wall biology and identifies pathways relevant to aortic aneurysms and dissection in general. The current review discusses the surgical management of patients with MFS with a special emphasis on indications for surgery in this complex group of patients.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Department of Cardiovascular Disorders (DHGE) > Clinic of Cardiovascular Surgery

UniBE Contributor:

Carrel, Thierry

ISSN:

1744-8298

Publisher:

Future Medicine Ltd

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:20

Last Modified:

06 Dec 2013 13:27

Publisher DOI:

10.2217/fca.11.37

PubMed ID:

21797750

URI:

https://boris.unibe.ch/id/eprint/6603 (FactScience: 211599)

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