||Marfan's Syndrome and the Adult Patient
This inherited condition usually results in early death unless carefully monitored and treated. Early death is often the result of the rupture (dissection) of the root of the aorta, which typically enlarges over time in the Marfan's patient.
Besides the enlargement of the aorta and/or aortic root, mitral valve prolapse (MVP) occurs in about 75% of Marfan's patients, and other so-called "elastic" vessels, such as the pulmonary artery, may become enlarged.
As the aorta becomes distended, its ability to withstand internal blood pressure is reduced. Therefore, beta-blockers are often prescribed to reduce blood pressure. Also, activities that might put extra stress on the aorta or expose it to external pressures (such as contact sports) are to be avoided.
Diagnosis is a complex affair because there are many ways in which Marfan's may be expressed, and there are other conditions that may be confused with it. If the symptoms or physical traits that are characteristic of this disorder are present, the patient will be examined through the use of electrocardiography (producing an ECG, or electrocardiogram), chest x-ray, MRI (Magnetic Resonance Imaging), ultrasound, CT (or CAT) Scan, and/or echocardiography (producing an echocardiogram). In some cases, a cardiac catheterization procedure may be useful.
If the aortic root becomes enlarged beyond a certain limit (55 mm is the current standard, 44 mm if pregnancy is anticipated), or if there are other indications, then an aortic root replacement may be performed. In some cases, the aortic valve will need to be replaced. If mitral valve prolapse is present, it may also require surgery. If possible, the mitral valve will be repaired, though valve replacement is sometimes necessary.
The postoperative prognosis depends on whether or not there had been an aortic dissection prior to surgery and whether or not a valve repair or replacement operation was performed. With or without surgery, it is important that the Marfan's patient's aorta and aortic root be monitored regularly to ensure that dangerous enlargement has not occurred.