Marfan Syndrome Exercise Recommendations

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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 (a condition doctors call 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 activities that usually do not require "bursts" of extremely high-intensity exercise. Examples of appropriate activities include golf, bowling, hikingl, 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.

People with Marfan syndrome are advised to avoid high-risk activities — contact sports and activities that require isometric exercise that produce a Valsalva maneuver (such as weight lifting).

Some people with Marfan syndrome may be individually cleared by their doctors (if their risk is judged to be quite low), to participate in intermediate risk sports such as basketball, baseball, touch football, and bicycling.

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.

A Word From Verywell

People with Marfan syndrome have a lifelong increased risk for serious cardiovascular events, and require regular medical follow-up.

Exercise restrictions are advised for anyone with Marfan syndrome. However, the degree of restriction will vary from person to person, and many are able to (and are encouraged to) enjoy active lifestyles with appropriate precautions.

Sources:

Hiratzka LF, Bakris GL, Beckman JA, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines For The Diagnosis And Management Of Patients With Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation 2010; 121:e266.

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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