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Friday, November 21, 2008

Marfan syndrome

Marfan's syndrome
Marfan's syndrome
Pectus excavatum
Pectus excavatum
Treatment

There is not a single, curative treatment for this condition.

Visual defects should be corrected where possible. Care should be taken to prevent the development of scoliosis, especially during adolescence.

The cardiovascular abnormalities are the most worrisome. The goal is primarily to prevent dilatation of the aortic root. Dilatation can lead to dissection or rupture of the aorta, both serious medical conditions that can be fatal. Medication to slow the heart rate (beta blockers) may help prevent stress on the aorta. Competitive athletics and contact sports should be discouraged as well. An echocardiogram to assess the aortic root should be performed every year. In some cases, surgical replacement of the aortic root and valve is needed.

People with Marfan should have preventive antibiotics before dental procedures in order to prevent endocarditis. Pregnancy in individuals with Marfan needs to be monitored very closely because of the increased stress on the heart and aorta.


Support Groups
www.marfan.org
Expectations (prognosis)

Lifespan is shortened somewhat because of cardiovascular complications. The survival is into the 60s, but may be extended with good care and heart surgery.


Complications
  • Dissecting aortic aneurysm
  • Aortic rupture
  • Bacterial endocarditis
  • Heart failure

Calling your health care provider

If you have a family history of Marfan syndrome and are considering having children, you are advised to call your provider. Also, call if your child has symptoms suggestive of this syndrome.



Review Date: 04/20/2005
Reviewed By: Neal Sondheimer, M.D., PhD., Division of Genetics and Metabolism, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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