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Complications

By Mayo Clinic staff

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Illustration showing aortic dissection Aortic dissection

Because Marfan syndrome can affect almost any part of your body, it may cause a wide variety of complications.

Cardiovascular complications
The most dangerous complications of Marfan syndrome involve the heart and blood vessels. Faulty connective tissue can weaken the aorta, the large artery that curves over your heart and supplies blood to the body, then splits in your pelvis to supply blood to your legs.

  • Aortic aneurysm. The pressure of blood leaving your heart can cause the wall of your artery to bulge out, like a weak spot in a tire. In people who have Marfan syndrome, this is most likely to happen at the aortic root — where the artery leaves your heart. The bulge can spread along the entire length of the aorta, into your abdomen. If it ruptures or tears, you may die.
  • Aortic dissection. The wall of the aorta is made up of thin layers. Dissection occurs when a small tear in the innermost layer of the aorta's wall allows blood to squeeze in between the inner and outer layers of the wall. This can cause severe pain in the chest or back. An aortic dissection weakens the vessel's structure and often results in a rupture, which can be fatal.
  • Valve malformations. People who have Marfan syndrome are also more likely to have problems with their heart valves, which may be malformed or overly elastic. When heart valves don't work properly, your heart muscle often has to work harder to compensate. This can eventually lead to heart failure.

Eye complications
Eye complications may include:

  • Dislocation of the lens in one or both eyes because of weakness in the ligaments that hold the lens in place.
  • Glaucoma, a condition in which abnormally high pressure within your eyes damages the optic nerve. Symptoms may range from sensitivity to light and glare to severe eye pain, blurred vision and blindness.
  • A cataract, which clouds the eye's normally clear lens.
  • Detachment or tear in the retina, the light-sensitive tissue that lines the back wall of your eye.

Lung complications
Marfan syndrome can cause breathing difficulties, either from defective connective tissue or from chest wall abnormalities. Severe spinal curvature or a concave chest, for instance, may restrict your breathing and cause you to feel short of breath during mild or moderate activity. People with Marfan syndrome are also at higher risk of:

  • Emphysema
  • Chronic obstructive pulmonary disease
  • Collapsed lung
  • Sleep apnea

Complications of pregnancy
Women with Marfan syndrome face possible complications during pregnancy. The main threat is a rapid increase in the size of your aorta, leading to life-threatening aortic dissection or rupture. Although the risk of dissection during pregnancy is unpredictable, it's generally low if your aorta isn't enlarged before you become pregnant. But if your aorta is even slightly enlarged, the risk increases greatly. Pregnancy isn't recommended if your aorta is enlarged.

DS00540

Aug. 1, 2008

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