Marfan Syndrome Exercise Recommendations

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athlete and blood vessels. SCIEPRO/Getty Images

Marfan syndrome is the most common inherited disorder of the connective tissue, commonly affecting the skeletal system, heart, blood vessels and eyes. People with Marfan syndrome are usually quite tall and thin, and their arms and legs are longer than normal. They also often have long fingers (arachnodactyly), an abnormal curvature of the spine (kyphoscoliosis), and dislocation of the lens of the eye.

The most life-threatening complications of Marfan syndrome are related to the heart and blood vessels; in particular, to aneurysm of the aorta. An aneurysm is a dilation (ballooning-out) of the wall of the blood vessel. This dilation greatly weakens the wall of the aorta, and makes it prone to sudden rupture (a condition which doctors call dissection). Aortic dissection is a medical emergency, and can lead to sudden death in people with Marfan syndrome.

Since this catastrophe is more likely to occur during times when the cardiovascular system is being stressed, exercise, in particular, can precipitate aortic dissection in a person with Marfan syndrome. For this reason, young people who have Marfan syndrome must often limit their participation in athletic activities.

It is never easy for young people to hear that their physical activity has to be limited. Fortunately, most people who have Marfan syndrome can stay active, but with restrictions.

It is important for these young athletes to be aware of their limits.

General Exercise Recommendations For Young Athletes With Marfan Syndrome

Formal recommendations on engaging in competitive athletic activity for athletes with Marfan syndrome were published by the 2005 Bethesda Conference on Eligibility Recommendations for Competitive Athletes with Cardiovascular Abnormalities.

Here is a summary of those recommendations:

Athletes who have Marfan syndrome should have echocardiograms every six months to look for dilation of the root of the aorta.

If there is no aortic root dilation or other serious cardiac abnormalities, and no family history of aortic dissection, they may participate in what are called low and moderate dynamic, static sport activities -- those that usually do not require "bursts" of extremely high-intensity exercise. Examples of appropriate activity include golf, bowling, baseball, volleyball, and table tennis.

If there is evidence of aortic root dilation, or a family history of aortic dissection, then only very low-intensity activity, such as golf or bowling, is appropriate.

Notably, the Bethesda Conference specifically addressed people who are engaging in organized, competitive athletics. In particular, it laid out guidelines for schools and other organizations for which athletes with Marfan syndrome may be looking to participate in their programs.

It did not specifically address the recreational athlete.

However, the Bethesda recommendations can still provide guidance for recreational athletes and their physicians. In anyone with Marfan syndrome who wishes to engage in sports, periodic echocardiograms can be used to guide an appropriate level of physical activity. If periodic echocardiograms are not practical, then simply avoiding anything more than low-intensity activity should be recommended.

Sources:

Maron, BJ, Ackerman, MJ, Nishimura, RA, et al. Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. J Am Coll Cardiol 2005; 45:1340

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