There is no medical treatment to reverse the fibrillin abnormality in people with Marfan's syndrome. In the future, research on a strain of mice that are born with similar fibrillin problems may lead to a successful treatment for Marfan's.
Until then, doctors try to prevent or delay the aortic changes seen in Marfan's patients by prescribing medication that decreases the pressure inside the aorta. Your doctor may advise that you take a beta-blocker and/or an angiotensin receptor blocker called losartan (Cozaar). Beta blockers, such as propranolol (Inderal), metoprolol (Lopressor) and atenolol (Tenormin), decrease the strain on the aorta's wall by slowing the heart rate and reducing the force of heart contractions, especially during exercise. Losartan and other angiotensin receptor blockers help lower blood pressure, which reduces the stress on artery walls.
If you have Marfan's, your doctor will monitor your heart health with periodic echocardiograms to check for developing problems in the aorta and mitral valve. If a serious problem is found, you may need surgery to replace the aortic valve, mitral valve or part of the aorta. Doctors become concerned when the aortic root size measured on an echocardiogram expands over time. Once it reaches 5 centimeters or more, many experts recommend surgery. Some recommend surgery with lesser aortic root enlargement.
You should follow a non-strenuous exercise program that involves non-competitive, non-contact sports (walking, bicycling, jogging), which you can do at your own pace.
If you have Marfan's-related scoliosis of 20 to 40 degrees, you can be treated with a brace and physical therapy. For scoliosis greater than 45 degrees, you will need surgery. You should have an annual eye examination to look for Marfan's-related eye problems. If you have ectopia lentis, you may be able to be treated with special lenses called aphakic lenses and special eye drops to widen the pupil, rather than surgery. If eye surgery is necessary, it should be done in an ophthalmology center that specializes in the treatment of Marfan's syndrome.
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