How is Marfan syndrome treated?

Marfan syndrome requires a treatment plan that is individualized to the patient's needs. Some people may not require any treatment, just regular follow-up appointments with their doctor. Others may need medications or surgery. The approach depends on the structures affected and the severity of the condition.

Lifestyle issues

  • Follow-up: Routine follow-up including cardio-vascular, eye, and skeletal exams, especially during the growing years. Your doctors will discuss the frequency of follow-up with you.
  • Activity: Activity guidelines vary, depending on the extent of the disease and symptoms. Most people with Marfan syndrome can participate in some type of physical and/or recreational activities. Those with dilation of the aorta will be asked to avoid high-intensity team sports, contact sports, and isometric exercises (such as weight lifting). Ask your cardiologist about activity guidelines for you.
  • Pregnancy: Genetic counseling should be performed prior to pregnancy as Marfan syndrome is an inherited condition. Pregnant women with Marfan syndrome are considered high risk cases. If the aorta is normal size, the risk for dissection is lower, but not absent. Those with even slight enlargement are at higher risk and the stress of pregnancy may cause more rapid dilation. Careful follow-up, with frequent blood pressure checks and monthly echocardiograms is required during pregnancy. If there is rapid enlargement or aortic regurgitation, bed rest or surgery may be required. Your doctor will discuss with you the best method of delivery with you.
  • Endocarditis prevention: People with Marfan syndrome who have also had valve surgery have an increased risk for bacterial endocarditis. This is an infection of the heart valves or tissue which occurs when bacteria enters the blood stream. To reduce the risk of endocarditis, antibiotics should be given prior to dental or surgical procedures in patients with Marfan syndrome who have had valve surgery. Check with your doctor about the type and amount of antibiotics you should take. A wallet card may be obtained from the American Heart Association (PDF) with specific antibiotic guidelines.

Medications

Medications are not used to treat Marfan syndrome, however they may be used to prevent or control complications. Medications may include:

  • A beta-blocker improves the heart’s ability to relax, decreases the forcefulness of the heartbeat and the pressure within the arteries, thereby preventing or slowing the enlargement of the aorta. Beta-blocker therapy should begin at an early age.

In people who are unable to take beta-blockers due to asthma or side-effects, a calcium channel blocker, such as verapamil, is recommended.

  • An angiotensin receptor blocker (ARB) is a type of medication that acts on a chemical pathway in the body. These agents are often used in treatment of high blood pressure as well as heart failure.

Clinical trials are currently being conducted to evaluate how these medications may prevent aortic enlargement. Early studies are encouraging.

Surgery

Surgery for Marfan syndrome is aimed at preventing aortic dissection or rupture and treating valve problems. When the aorta diameter is more than 4.7 cm (centimeters) to 5.0 cm (depending on your height), or if the aorta is enlarging at a rapid pace, surgery is recommended. Your cardiologist may also calculate your aortic root diameter to height ratio, since this may also influence whether you should have surgery. If you are contemplating a pregnancy then that would also affect the recommendations for surgery.

The recommendation for surgery is based on size of the aorta, expected normal size of the aorta, rate of aortic growth, age, height, gender and family history of aortic dissection. Surgery involves a replacement of the dilated portion of the aorta with a graft.

Valve repair or replacement surgery may be needed when Marfan syndrome causes a leaky aortic or mitral valve (regurgitation) that leads to changes in the left ventricle (left lower chamber of the heart) or heart failure.

It is recommended that people with Marfan syndrome undergo surgery by surgeons at major centers who are experienced in this type of surgery. A better understanding of Marfan syndrome combined with earlier detection, careful follow-up and safer surgical techniques have resulted in better outcomes for patients.

Get more information on Aorta Surgery for Marfan Syndrome including: Aorta surgery, valve sparing re-implantation aorta surgery, and valve repair or replacement surgery for Marfan Syndrome.

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