Angiotensin II blockade and aortic-root dilation in Marfan's syndrome by Brooke Benjamin S, Habashi Jennifer P, Judge Daniel P, Patel Nishant, Loeys Bart, Dietz Harry C in The New England journal of medicine (2008).

[PMID: 18579813] PubMed


Progressive enlargement of the aortic root, leading to dissection, is the main cause of premature death in patients with Marfan's syndrome. Recent data from mouse models of Marfan's syndrome suggest that aortic-root enlargement is caused by excessive signaling by transforming growth factor beta (TGF-beta) that can be mitigated by treatment with TGF-beta antagonists, including angiotensin II-receptor blockers (ARBs). We evaluated the clinical response to ARBs in pediatric patients with Marfan's syndrome who had severe aortic-root enlargement.

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