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Although an <jats:italic>FBN-1<\/jats:italic> mutation does not guarantee the diagnosis of Marfan syndrome it is clear however that <jats:italic>FBN-1<\/jats:italic> mutations independently confer additional risk for many of the cardiovascular complications classically associated with the disease. Furthermore, treatment with an angiotensin receptor blocker has proven effective in reducing rates of thoracic aortic root dilatation in preliminary studies of Marfan syndrome patients. Awareness of an <jats:italic>FBN-1<\/jats:italic> mutation then highlights the need for increased vigilance for the associated cardiovascular phenotypes. 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