What are the blood vessel problems?
In the Marfan syndrome, the walls of the major arteries are weakened. The aorta, the major artery that leaves the heart, often is affected. When this occurs, it gets bigger (dilates), which can weaken the inner aortic wall. Under strenuous exertion, the aorta wall can tear. Blood can leak through these tears into the aortic wall, separating its layers " a process called aortic dissection. Another problem that may occur if the aortic wall weakens is that an aneurysm (AN'u-rizm) may form.
What are the cardiac valve problems?
The Marfan syndrome can involve any of the four heart valves. The two most important ones, however, are the mitral and aortic valves. Blood may leak backward through a valve, increasing the heart's workload. As a result, over time the heart may enlarge. Mitral valve prolapse (MVP) is a common but usually less significant valve problem. People without the Marfan syndrome can have mitral valve prolapse, but those with the Marfan syndrome often have an oversized mitral valve. As it closes, the valve billows (prolapses) back into the left atrium. This causes abnormal heart sounds, which a doctor can hear with a stethoscope.
How do you treat the Marfan syndrome?
Once the Marfan syndrome has been diagnosed, regular visits with a cardiologist are needed. Chest X-rays and Doppler echo tests are usually performed. Other tests also may be necessary.
Different people may need different types of treatment. Some people may not need treatment, but others may need drugs that lower heart rate, blood pressure or both. When the aorta or aortic valve develop major problems, surgery is usually advised.
People with the Marfan syndrome who have an abnormal heart or who've had heart surgery for their Marfan syndrome risk developing an infection of the heart wall or heart valve (endocarditis). This happens when bacteria enter the bloodstream. The American Heart Association no longer recommends taking routine antibiotics before certain dental procedures except for people at highest risk for bad outcomes if they develop endocarditis. This includes people with a prosthetic heart valve, people who have previously had endocarditis, people with certain types of congenital heart disease, and people with a heart transplant who develop a problem with a heart valve. The AHA no longer recommends routine antibiotics solely to prevent endocarditis in patients undergoing gastrointestinal or genitourinary procedures .
www.americanheart.org