title: [Diagnosis and options for reconstructive heart valve surgery in the Marfan syndrome].
abstract: 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 valve regurgitation. The multiorgan involvement of many of these syndromes requires multidisciplinary expert centers that can increase the average life expectancy of affected patients from only 32 years to over 60 years. The present article both reviews classical standards of managing cardiovascular manifestations and highlights the surgical approach for aortic and mitral valve surgery in Marfan patients.
authors.forename: Amj
authors.initials: A
authors.lastname: Bernhardt
authors.forename: H
authors.initials: H
authors.lastname: Treede
authors.forename: M
authors.initials: M
authors.lastname: Rybczynski
authors.forename: S
authors.initials: S
authors.lastname: Sheikzadeh
authors.forename: T
authors.initials: T
authors.lastname: Meinertz
authors.forename: H
authors.initials: H
authors.lastname: Reichenspurner
authors.forename: Y
authors.initials: Y
authors.lastname: Kodolitsch
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D000784:Aortic Dissection / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D001011:Aorta / Q000473:pathology* / Q000503:physiopathology / Q000601:surgery; D001014:Aortic Aneurysm / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D001019:Aortic Rupture / Q000209:etiology / Q000503:physiopathology / Q000517:prevention & control; D013504:Cardiovascular Surgical Procedures / Q000379:methods / Q000592:standards; D003131:Combined Modality Therapy; D003238:Connective Tissue / Q000473:pathology; D000071838:Fibrillin-1; D000071837:Fibrillins; D006801:Humans; D033183:Interdisciplinary Communication; D008017:Life Expectancy; D008382:Marfan Syndrome / Q000150:complications / Q000235:genetics / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D008840:Microfilament Proteins / Q000235:genetics; D008944:Mitral Valve Insufficiency / Q000209:etiology / Q000503:physiopathology / Q000601:surgery; D009154:Mutation; D018802:Patient-Centered Care / Q000592:standards / Q000639:trends*; D010818:Practice Patterns, Physicians' / Q000592:standards*; D054392:Adipokines
chemical_list: D000319:Adrenergic beta-Antagonists; D000071838:Fibrillin-1; D000071837:Fibrillins; D008840:Microfilament Proteins; C000605722:FBN1 protein, human; D054392:Adipokines