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Emphasizes lethal aortic complications driving early mortality in Marfan syndrome; leaves open whether targeted therapies can extend survival.
For most of the century after Marfan first described the skeletal features of the condition that bears his name,1 progress with regard to Marfan's syndrome was marked by incremental increases in the understanding of its skeletal, ocular, and cardiovascular features but no reduction in its toll of premature death. Most deaths were due to dissection or rupture of the aorta or to heart failure resulting from severe aortic regurgitation. In 1972, the median life expectancy of patients with Marfan's syndrome was reported to be about 45 years,2 with women surviving approximately 10 years longer than men. Beginning around that time, . . .
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Devereux et al. (1999) studied this question.
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