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Medical Management of Marfan syndrome

Marfan syndrome is an inherited disorder of the connective tissue (fibers that provide the framework and support for the body) that is estimated to occur in at least 1 in 5,000 persons. It is seen in all races and ethnic groups, and can appear at any age. Marfan syndrome is characterized by multiple abnormalities in the connective tissue primarily affecting the skeleton system (bones and ligaments), the cardiovascular system (heart and blood vessels), the eyes, the lungs, and the skin.

At the Bluhm Cardiovascular Institute, the multidisciplinary team at the Marfan syndrome and Related Disorders Clinic offer a variety of treatment options in order to best fit the specific needs of each patient. The medical treatments offered at the Marfan syndrome and Related Disorders Clinic for cardiovascular abnormalities include:

• Evaluation of Marfan syndrome with a cardiologist, scheduled follow-up visits and routine echocardiograms to assess changes in the aorta, aortic root and mitral valve.

• Medications: Beta blockers (slow the heart rate and reduce the heart's force of contraction) are useful to reduce the growth rate of the aorta and aortic root. Medications are usually started at low doses, and increased by the cardiologist to achieve optimal blood pressure and heart rate.

• Counseling on exercise restriction which includes avoidance of competitive sports and certain high-intensity activities.

Testing
An evaluation for Marfan syndrome at the Marfan syndrome and Related Disorders Clinic may include the following tests for diagnosis and follow-up:

• An Echocardiogram to screen for the presence of aortic disease and mitral valve disease.

• A Transesophageal Echocardiogram also screens for abnormal heart structure, but differs from the normal echocardiogram because the probe is inserted in the esophagus and may show clearer images.

• An Electrocardiogram which will assess for an abnormal rhythm of the heart.

• A Cardiac magnetic resonance imaging (MRI) or ECG-gated computed tomograghy (CT) angiogram which accurately shows the size of the aortic root and detailed images of the heart chambers and valves.

Contact
For more information regarding Marfan syndrome and related disorders, please contact the Bluhm Cardiovascular Institute at (866) 662-8467, or request an appointment online.

In addition, the most credible source of information about Marfan syndrome is the National Marfan Foundation (NMF). Please visit the NMF Web site.

Visit the Northwestern Memorial Hospital's Health Learning Center and Alberto Culver Women's Health Learning Center. These state-of-the-art health information libraries are among the largest hospital-based learning facilities of their kind. For more information call (312) 926-5465.

Review Date: 05/08