What is it?
Marfan syndrome is a genetic disorder that affects the connective tissue of the body. Connective tissue gives strength and support to tendons, ligaments, blood vessel walls, cartilage, heart valves and many parts of the body. The connective tissue isn't normal in Marfan syndrome. As a result, many of these body parts aren't as stiff as they should be. It causes the blood vessels to get bigger and also causes problems with any of the four heart valves. The walls of the major arteries are weakened in Marfan syndrome. The aorta (the artery that carries blood from the heart to the body) is often affected. When this happens, it gets bigger, which can weaken the inner aorta wall. When the heart is working hard, the wall of the aorta can tear and blood can leak through these tears into the aorta and separate its layers. This process is called aortic dissection.
Another problem that may happen if the aortic wall weakens is an aneurysm (pronounced an–yer–iz–em), a blood-filled sac.
How is it treated?
Some children may not need treatment, but others may need medicines that lower the heart rate, blood pressure or both.
Surgery is usually needed when the aorta or aortic valve have major problems.

LI 5.09.15