Marfan's Syndrome and Other Disorders of Fibrillin
- 12 May 1994
- journal article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 330 (19) , 1384-1385
- https://doi.org/10.1056/nejm199405123301911
Abstract
Marfan's syndrome is classically defined as an autosomal dominant disorder of connective tissue with variable manifestations involving primarily the cardiovascular, ocular, and musculoskeletal systems. The most important feature that reduces life expectancy is progressive dilatation of the aortic root and ascending aorta, leading to aortic-valve incompetence and aortic dissection. Aortic dilatation and dissection, however, do not always occur together. In some families, dissection tends to occur before the aorta has reached the critical diameter of 55 mm, currently considered an indication for prophylactic surgery in adults. Repair of the aortic valve and ascending aorta with a composite graft has made . . .Keywords
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