Early Diagnosis, Surgery Can Help Those With Marfan’s Syndrome

Surgery to correct a life-threatening heart defect in people with Marfan’s syndrome is far safer if done before symptoms develop, researchers report in today’s New England Journal of Medicine. Marfan’s is an inherited disorder of the connective tissue. Those who inherit it are often unusually tall and are likely to have weakened aortas that can rupture disastrously, causing death.

In the early 1970s, the average life expectancy of people with the disorder was about 45. But life expectancy is now in the 70s, in part because of the use of open-heart surgery to repair weakened aortas. New research at Johns Hopkins University found that the surgical death rate was only 2% when the surgery was performed at diagnosis, compared with 12% when performed on an emergency basis.

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Compiled by Times medical writer Thomas H. Maugh II