MARFAN SYNDROME PATIENTS BENEFIT
FROM BETA-BLOCKERS

June 28, 1994
Media Contact: Debbie Bangledorf
Phone: (410) 223-1731
E-mail: Dbangle@welchlink.welch.jhu.edu

Johns Hopkins researchers have shown that long-term use of beta-blocker drugs, which slow the heart rate, reduces progressive widening of the aorta in people with Marfan syndrome. Left untreated, the aorta can burst and cause premature death in Marfan patients.

Previously, physicians had no option but to wait until the aorta widened to a diameter of 6 cm, about 1.5 inches, and then do open heart surgery. Beta-blockers slow the process so that patients can postpone or avoid surgery. "Because aortic dilatation is so unpredictable, some people die before the aorta widens to 6 cm. Treatment with beta-blockers will save lives," says Jennifer Shores, M.D., the lead researcher in this study.

The Hopkins study, recently published in The New England Journal of Medicine, followed 70 patients over 10 years and is the largest, most long-term study of betablockers, such as propranolol and atenolol.

Marfan syndrome is a genetic disorder of collagen, the connective tissue that normally makes blood vessels and other organs strong and elastic. In Marfan syndrome patients, the aorta's wall is less strong, less elastic, and less able to withstand stress caused by blood pumping through the vessel.

Other authors were Kenneth R. Berger, M.D., Ph.D.; Edmond A. Murphy, M.D., Sc.D.; and Reed E. Pyeritz, M.D., Ph.D. This study was funded by the National Institutes of Health, the Food and Drug Administration and the National Marfan Foundation.


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