[Diagnosis and options for reconstructive heart valve surgery in the Marfan syndrome].

Abstract excerpt

Marfan syndrome is a disorder of the connective tissue that is inherited in an autosomal-dominant fashion and that is caused by mutations in the gene coding for fibrillin-1, FBN1. Although complications of the syndrome may involve the eye, the lung and the skeleton, the high mortality of untreated cases results almost exclusively from cardiovascular complications, including aortic dissection, rupture and mitral...

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