Reference : Médianécrose aortique dans la maladie de Marfan chez l'adulte jeune. A propos de 5 cas o...
Scientific journals : Article
Human health sciences : Cardiovascular & respiratory systems
Human health sciences : Surgery
http://hdl.handle.net/2268/128159
Médianécrose aortique dans la maladie de Marfan chez l'adulte jeune. A propos de 5 cas opérés, avec 3 survies
French
[en] Aortic Media Necrosis in Marfan's Disease in Young Adults. Apropos of 5 Surgically Treated Cases, with 3 Survivals
Defraigne, Jean-Olivier mailto [Université de Liège - ULg > Département des sciences cliniques > Chirurgie cardio-vasculaire et thoracique >]
Grenade, Thierry [Université de Liège - ULg > Département des sciences cliniques > Chirurgie cardio-vasculaire et thoracique]
CREEMERS, Etienne mailto [Centre Hospitalier Universitaire de Liège - CHU > > Chirurgie cardio-vasculaire >]
Limet, Raymond mailto [Université de Liège - ULg > Département des sciences cliniques > Chirurgie cardio-vasculaire et thoracique]
Aug-1988
Archives des Maladies du Coeur et des Vaisseaux
J. B. Bailliere et Fils
81
8
991-5
Yes (verified by ORBi)
International
0003-9683
Paris
France
[en] Five cases of Marfan's syndrome with cardiovascular lesions are presented. Among these five patients, two with type I aortic dissection, one with true aneurysm of the ascending aorta and one with aortic regurgitation underwent composite grafting of the ascending aorta according to the Bentall technique. The fifth patient died preoperatively, due to extensive dissection of the transverse aorta. Marfan's syndrome is transmitted in an autosomal dominant manner. Fifty percent of the patients die at a mean age of 32 years, and 95 p. 100 of the deaths are caused by cardiovascular lesions. These lesions predominantly affect the left cardiac valves (aortic or mitral regurgitation) and the ascending aorta. Except in emergencies, replacement of the ascending aorta by a composite graft must be envisaged in subjects with Marfan's syndrome presenting with aortic regurgitation and dilatation of the aortic root.
Researchers ; Professionals ; Students
http://hdl.handle.net/2268/128159

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