Early Heart Repair For Marfan Syndrome Patients Critical To Survival

Risk Of Death Eight Times As High When Aneurysms Require Emergency Surgery

People with Marfan syndrome should be carefully monitored for development of an aortic aneurysm -- a ballooning of the large blood vessel that leads away from the heart -- and should be treated early, according to a large, international study led by physicians at Johns Hopkins. The risk of death for these patients is eight times higher when they wait until they require emergency surgery for the aneurysms.

Results of the study, the largest to look at Marfan syndrome, were published in the April 29 issue of The New England Journal of Medicine. They showed that death rates from surgical repair of the aneurysm were only 1.5 percent when done early, compared to 12 percent among patients who underwent emergency repair.

Marfan syndrome and related connective tissue disorders affect some 200,000 people in the United States. Marfan syndrome is a disorder of the connective tissue that normally makes blood vessels and other organs strong and elastic. Usually inherited, it affects the skeleton, eyes, heart and blood vessels and is often characterized by excessive height and abnormally long and slender fingers and toes.

In Marfan patients, the aorta, or main blood vessel, is weakened and prone to enlargement. Without proper monitoring and medications to reduce the stress on the aorta, it could tear, resulting in sudden death. However, with an early diagnosis, proper treatment and a modified lifestyle, most patients with the disorder can live a normal life span. Aneurysms are treated by surgery, during which the diseased portion of the aorta is replaced, usually with a Dacron fabric tube containing a mechanical valve.

Surgery to repair an aortic aneurysm is no longer a high-risk procedure when done early, says lead author Vincent L. Gott, M.D., professor of surgery at Hopkins. Results are similar to those from bypass op

Contact: Karen Infeld
Johns Hopkins Medical Institutions

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