Marfan Syndrome

Treatment

Enlarged aorta (aneurysm) before surgery.

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Valve sparing aortic root repair procedure

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Mechanical valve procedure

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The integrated team of medical specialists at Mayo Clinic works closely with each Marfan syndrome patient to provide the most appropriate treatment. Mayo Clinic cardiologists and cardiac surgeons have expertise and experience in advanced treatments and surgeries, such as aortic root surgery, to treat Marfan syndrome.

Treatment of the condition depends on the organ systems affected. Marfan syndrome treatment at Mayo Clinic could include surgery, medication, lifestyle changes or a combination of these. Genetic and reproductive counseling is also offered to patients.

Surgery

Some patients with Marfan syndrome need heart surgery. Types of heart surgery performed at Mayo Clinic include aortic aneurysm repair, aortic valve repair, aortic valve replacement and mitral valve repair or replacement.

Aortic Aneurysm Repair

Aortic aneurysm repair is the most common cardiovascular operation required in Marfan syndrome. This surgery involves replacement of a part of the aorta with an artificial graft.

Aortic Valve Repair

Mayo Clinic is a leading center in aortic root surgery, including aortic valve repair, among patients with Marfan syndrome. Mayo Clinic cardiac surgeons have performed hundreds of these surgeries.

Valve sparing aortic root repair

Valve sparing aortic root repair involves replacement of the enlarged aorta with a graft, while saving the patient's valve. This procedure avoids the potential problems of replacing the aortic valve with a new one (see illustration). Also, patients who have this surgery do not need to take blood-thinning medicines (anticoagulants). A disadvantage of valve sparing surgery is that the valve may degenerate over time.

Aortic Valve Replacement

Aortic valve replacement is often performed along with aortic root replacement. Mechanical or biological valves may be used to replace the patient's valve.

Mechanical valves are made of synthetic materials, which can last many years. Because blood tends to stick to mechanical valves and create clots, patients with these valves will need to take blood-thinning medicines (anticoagulants) (see illustration).

Biological valves are made from animal or human tissue. Patients with biological valves generally do not need to take anticoagulants. These valves are less durable than their mechanical counterparts, and may eventually need to be replaced.

Mayo Clinic participates and is one of the lead enrolling centers in a National Marfan Foundation study about aortic operations. The study will determine which type of aortic operation, aortic valve sparing or aortic valve replacement, is the most appropriate option for Marfan patients. Read about Marfan syndrome clinical trials.

Mitral Valve Repair or Replacement

The mitral valve may be affected by valve leakage in patients who have Marfan syndrome. Usually, mitral valve prolapse causes the leakage. Mitral valve repair is usually possible. Mitral valve repair is preferred, as it avoids problems related to mitral valve replacement with artificial heart valves.

Medication

Beta-blocker medications have been shown to slow enlargement of the aorta and delay aortic surgery. Current research is studying possible benefits of new medications.

Lifestyle Changes

Lifestyle changes, such as avoiding strenuous exercise and contact sports, can improve the quality of life for those who have Marfan syndrome.