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

Florian S. Schoenhoff, Duke E. Cameron, Gabor Matyas, Thierry P. Carrel

Research output: Contribution to journalReview articlepeer-review

Abstract

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.

Original languageEnglish (US)
Pages (from-to)557-569
Number of pages13
JournalFuture Cardiology
Volume7
Issue number4
DOIs
StatePublished - Jul 2011

Keywords

  • Marfan syndrome
  • aortic aneurysm
  • aortic surgery
  • connective tissue disorders

ASJC Scopus subject areas

  • Molecular Medicine
  • Cardiology and Cardiovascular Medicine

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