Marfan Syndrome
Marfan syndrome may be very mild or severe. There is no single test for diagnosing the disorder, but doppler ultrasound and chest X-rays are frequently used in follow-up of individuals with the condition. People who have the condition share some similar traits, including:
  • heart problems
  • tall, thin stature
  • long arms and legs
  • slender fingers
  • spine curvature
  • eye problems


How does Marfan syndrome affect the cardiovascular system?

In Marfan syndrome, the walls of arteries are weakened. The aorta (the large artery that leaves the heart) often becomes enlarged, with a weakening of the inner wall of the aorta. The aortic wall can tear and/or dissect. Marfan syndrome also increases the possibility of developing an aneurysm (a ballooning out of a blood vessel, which can cause clots or rupture).

Marfan syndrome can involve the heart valves. Blood may leak backward (regurgitate) through a valve, which increases the heart's workload. This increased workload can cause the affected area of the heart to enlarge. Mitral valve prolapse is a common but less dangerous condition associated frequently with Marfan syndrome.

How is Marfan syndrome treated?

Some people with mild symptoms might not need specific treatment beyond routine follow-up with their doctor. Medications to lower heart rate and/or blood pressure are sometimes indicated. When heart valves are significantly affected, surgery is sometimes indicated

Marfan syndrome increases the possibility of developing endocarditis, which is an infection of the endocardium (the tissue that lines the heart wall and valves). To prevent this, antibiotics are often prescribed before medical or dental procedures. Ask your doctor about this if you have any questions or think you might need antibiotic therapy for an upcoming procedure. These procedures include something as minor as getting your teeth cleaned at the dentist.

People with Marfan syndrome might need to modify their physical activity, depending on the severity of their condition. Contact sports that could result in a blow to the chest are usually to be avoided, as well as weight lifting and most team sports. Ask your doctor about your particular situation.

Pregnancy for women with a dilated aorta is not advised, because of the stresses of pregnancy and delivery.

  • palpitations
  • rapid heartbeat
  • signs and symptoms associated with congestive heart failure

How is myocarditis diagnosed?

Your doctor will take your history and perform a physical exam. If symptoms appear within 6 months of a recent infection, myocarditis might be suspected. Your doctor will look for a rapid heartbeat (tachycardia) and signs of heart failure (including congestion in the veins of the lungs and body).

Some diagnostic tests and/or imaging techniques can be helpful in diagnosing myocarditis. These tests include:

  • ECG
  • chest X-ray
  • heart biopsy
  • cultures

How is myocarditis treated?

If myocarditis is caused by a bacterial infection, your doctor will prescribe antibiotics. Myocarditis is treated with analgesics or anti-inflammatory drugs to relieve pain. You may require treatment for an arrhythmia. If this is the case, your doctor may prescribe antiarrhythmic medications. Additional oxygen is often given to assist the heart in oxygenating the blood. Activity may be restricted to bed rest to reduce cardiac workload, and the diet may be salt/sodium restricted. A heart transplant may be considered if the condition is severe and unresponsive to treatment.

 

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