Marfan or Farfan (?) Syndrome

  I am a 36 yr old male with diag of 2+ aortic insufficiency and MVP. I had an aortic coartaction corrected 14 years ago. I was told that I have the Marfan or Farfan (not sure how to spell it) Syndrome, and that my aortic valve might have to be replaced if the LV continues to enlarge. Now it is 6.3 cms. I feel well, do some exercise and am on a vegan diet
  1. Could you give me info on this Syndrome? Papers/links?
  2. What would be the best lifestyle for a person with this Syndrome?
  Thank you very much for your help.
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Dear Ignacio,
Thank you for your question.  Marfan's Syndrome is named after the doctor who first described it and is a connective tissue disorder.  Connective tissues are those parts of the body that provide structure and support (i.e. tendons, blood vessel walls, cartilage, heart valves, etc.) In the Marfan syndrome, the chemical
makeup of the connective tissue isn't normal. As a result, many of these structures aren't as stiff as they should be.  Persons with this syndrome are usually tall, have long fingers, can have dislocation of the lens in the eye and have a propensity to develop aortic aneurysms.  
The Marfan syndrome is inherited and affects many parts of the body. There's no single conclusive test for diagnosing it, but people who have it often have many similar traits. Besides perhaps having heart problems, people with the Marfan syndrome are often tall and thin. They also may have slender, tapering fingers, long arms and legs, curvature of the spine and eye problems. Sometimes the Marfan syndrome is so mild that few (if any) symptoms exist. In the most severe cases, which are rare, life-threatening problems may occur at any age.
In the Marfan syndrome, the walls of the major arteries are weakened. The aorta , the major artery that leaves the heart, often is affected. When this occurs, it gets bigger (dilates), which can weaken the inner aortic wall. Under strenuous exertion, the aorta wall can tear. Blood can leak through these tears into the aortic wall, separating its layers - a process called aortic dissection . Another problem that may occur if the aortic wall weakens is that an aneurysm may form.
What are the cardiac valve problems?
The Marfan syndrome can involve any of the four heart valves. The two most important ones, however, are the mitral and aortic valves. Blood may leak backward through a valve, increasing the heart's workload. As a result, over time the heart may enlarge. Mitral valve prolapse (M.V.P.) is a common but usually less significant valve problem. People without the Marfan syndrome can have mitral valve prolapse, but those with the Marfan syndrome often have an oversized mitral valve. As it closes, the valve billows (prolapses) back into the left atrium . This causes abnormal heart sounds, which a doctor can hear with a stethoscope .
How do you treat the Marfan syndrome?
Once the Marfan syndrome has been diagnosed, regular visits with a cardiologist are needed. Chest X-rays and Doppler echo tests are usually performed. Other tests also may be necessary. Different people may need different types of treatment. Some people may not need treatment, but others may need drugs that lower heart rate, blood pressure or both. When the aorta or aortic valve develop major problems, surgery is
usually advised.
Anyone with Marfan syndrome who has an abnormal heart or who's had heart surgery runs the risk of
developing an infection of the heart wall or heart valve (endocarditis) . This happens when bacteria enter
the bloodstream. To prevent this, antibiotics are given before certain dental or surgical procedures.
What about lifestyle changes?
     Physical activities - Because the Marfan syndrome appears in many forms, recommendations about exercise vary widely. For example, people with dilation of the aorta may be asked to avoid the usual team sports. Isometric exercises (such as weight lifting or rowing) and contact sports in which a blow to the chest could occur (such as football or hockey) also may be off-limits. Many people with the Marfan syndrome can participate in modified physical and recreational activities. A cardiologist can give advice about this.
    
Pregnancy - During pregnancy - and especially during delivery - stress to the aorta's walls greatly increases the risk of dissection or rupture. That's why pregnancy isn't advised for women with the Marfan syndrome who  have a dilated aorta. There's also a good chance that people with the disease may pass it on to their children.
I hope you find this information useful.  Information provided in the heart forum is for general purposes only.  Only your physician can provide specific diagnoses and therapies.  Please feel free to write back with additional questions.
If you would like to make an appointment at the Cleveland Clinic Heart Center, please call 1-800-CCF-CARE or inquire online by using the Heart Center website at www.ccf.org/heartcenter.  The Heart Center website contains a directory of the cardiology staff that can be used to select the physician best suited to address your cardiac problem.