FREE NEJM E-TOC    HOME   |   SUBSCRIBE   |   CURRENT ISSUE   |   PAST ISSUES   |   COLLECTIONS   |   Search Term  Advanced Search

Sign in | Get NEJM's E-Mail Table of Contents — Free | Subscribe

Previous Volume 330:1384-1385 May 12, 1994 Number 19 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

	Full Text Purchase this article Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

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 . . . [Full Text of this Article]

References

This article has been cited by other articles:

• Chowdhury, U. K., Mishra, A. K., Balakrishnan, P., Sharma, S., Kabra, M., Ray, R., Kalaivani, M., Gupta, R., Govindappa, R. M., Subramaniam, G. K. (2008). Role of fibrillin-1 genetic mutations and polymorphism in aortic dilatation in patients undergoing intracardiac repair of tetralogy of Fallot. J. Thorac. Cardiovasc. Surg. 136: 757-766 [Abstract] [Full Text]  
• Place, H. M., Enzenauer, R. J. (2006). Cervical Spine Subluxation in Marfan Syndrome. A Case Report. JBJS 88: 2479-2482 [Full Text]  
• De Paulis, R., Cetrano, E., Moscarelli, M., Ando, G., Bertoldo, F., Scaffa, R., Tomai, F., Chiariello, L. (2005). Effects of ascending aorta replacement on aortic root dilatation. Eur. J. Cardiothorac. Surg. 27: 86-89 [Abstract] [Full Text]  
• Giusti, B., Porciani, M. C., Brunelli, T., Evangelisti, L., Fedi, S., Gensini, G. F., Abbate, R., Sani, G., Yacoub, M., Pepe, G. (2003). Phenotypic variability of cardiovascular manifestations in Marfan Syndrome: Possible role of hyperhomocysteinemia and C677T MTHFR gene polymorphism. Eur Heart J 24: 2038-2045 [Abstract] [Full Text]  
• Immer, F. F., Bansi, A. G., Immer-Bansi, A. S., McDougall, J., Zehr, K. J., Schaff, H. V., Carrel, T. P. (2003). Aortic dissection in pregnancy: analysis of risk factors and outcome. Ann. Thorac. Surg. 76: 309-314 [Abstract] [Full Text]  
• Treasure, T. (2000). SURGERY: Cardiovascular surgery for Marfan syndrome. Heart 84: 674-678 [Full Text]  
• Detter, C., Mair, H., Klein, H.-G., Georgescu, C., Welz, A., Reichart, B. (1999). Long-term prognosis of surgically-treated aortic aneurysms and dissections in patients with and without Marfan syndrome. Eur. J. Cardiothorac. Surg. 13: 416-423 [Abstract] [Full Text]  
• Sutherland, G. R., Richards, R. I. (1994). DNA Repeats -- A Treasury of Human Variation. NEJM 331: 191-193 [Full Text]