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Few patients with Marfan syndrome live out a normal life span. However, data from 417 patients at four referral centers suggest that the prognosis may be improving for this autosomal dominant connective-tissue disorder, which is associated with aortic dissection and other cardiovascular abnormalities.
Total mortality in this cohort since 1970 has been 11%, and the mean age at death was 41 years. By comparison, a classic 1972 paper on the natural history of Marfan syndrome reported a mean age at death of 32 years. Compared with the 1972 analysis, the age at which half of patients are expected still to be alive has risen from 49 to 74 years for women and from 41 to 70 years for men. Of 112 patients who underwent surgery (most for aortic root r…