ORBi: Defraigne Jean-Olivier - Médianécrose aortique dans la maladie de Marfan chez l'adulte jeune. A propos de 5 cas opérés, avec 3 survies

Reference : Médianécrose aortique dans la maladie de Marfan chez l'adulte jeune. A propos de 5 cas o...


	Document type :	Scientific journals : Article
	Discipline(s) :	Human health sciences : Surgery Human health sciences : Cardiovascular & respiratory systems
	To cite this reference:	http://hdl.handle.net/2268/128159

Title :	Médianécrose aortique dans la maladie de Marfan chez l'adulte jeune. A propos de 5 cas opérés, avec 3 survies
Language :	French
Alternative title :	[en] Aortic Media Necrosis in Marfan's Disease in Young Adults. Apropos of 5 Surgically Treated Cases, with 3 Survivals
Author, co-author :	Defraigne, Jean-Olivier [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 [Centre Hospitalier Universitaire de Liège - CHU > > Chirurgie cardio-vasculaire >]
	Limet, Raymond [Université de Liège - ULg > Département des sciences cliniques > Chirurgie cardio-vasculaire et thoracique]
Publication date :	Aug-1988
Journal title :	Archives des Maladies du Coeur et des Vaisseaux
Publisher :	J. B. Bailliere et Fils
Volume :	81
Issue/season :	8
Pages :	991-5
Audience :	International
ISSN :	0003-9683
City :	Paris
Country :	France
Abstract :	[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.
Target :	Researchers ; Professionals ; Students
Permalink :	http://hdl.handle.net/2268/128159

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