Surgical Options for Marfan Syndrome
Marfan syndrome is an inherited disorder of the connective tissue (fibers that provide the framework and support for the body) that is estimated to occur in at least 1 in 5,000 persons. It is seen in all races and ethnic groups, and can appear at any age. Marfan syndrome is characterized by multiple abnormalities in the connective tissue but cardiovascular complications, specifically to the aortic root and aorta, are the most serious potential complications of Marfan syndrome.
Marfan syndrome causes the aortic root and aorta to become weak and dilated, leading to aneurysm formation. Aneurysm places strain on the aortic root and aorta potentially leading to:
Aortic Root Surgery
Surgery to the aortic root and aorta requires open heart surgery to correct the various types of aneurysms and dissections. The aortic root essentially begins after the aortic valve and includes the point at which the coronary arteries (blood supply to the heart muscle) begin. The aortic root is the beginning of the aorta.
The thoracic aorta (section of the aorta in the chest—above the diaphragm) is subdivided into three sections. The thoracic aorta begins by traveling upward towards the neck (ascending thoracic aorta) then turns left and forms an arch (thoracic aortic arch) and then turns down and travels towards the feet (descending thoracic aorta). The aorta continues below the level of the diaphragm and is then called the abdominal aorta.
Aortic Root Replacement
Cardiac surgeons at the Bluhm Cardiovascular Institute repair aortic root aneurysms or dissection by replacing the aortic valve, if it is diseased, and the portion of the aorta that is diseased with a tubular Dacron graft made of synthetic (manmade) material.
Aortic Arch Repair
Aneurysms or dissections may involve more than just the aortic root. The aortic arch and the descending aorta can also be diseased. Cardiac surgeons at the Bluhm Cardiovascular Institute repair the aortic arch and the descending aorta with a tubular Dacron graft made of synthetic material.
In some cases more than one section of the aorta is diseased and must be repaired. For example, disease in the aortic root and a portion of the aortic arch may require an aortic root repair and a hemi-arch repair to be performed at the same time. In these cases, the different Dacron grafts are sewn together after each portion of the aorta is repaired.
The standard incision for aortic repairs is a full sternotomy, which involves a 6- to 8-inch incision down the middle of your chest and requires the cardiac surgeon to open your breastbone. After surgery, the breastbone is wired together and the incision is closed with sutures. Some descending aortic arch repairs may only require a left thoracotomy, which involves a small incision between the fourth and fifth rib space.
Mitral Valve Repair or Replacement Surgery
Mitral valve surgery is open-heart surgery that repairs (preserves your native valve) or replaces the diseased mitral valve. For patients with mitral valve prolapse, repair rather than replacement is the preferred operation for restoring proper valve function. During mitral valve repair surgery, a durable prosthetic ring is placed at the base of the mitral valve to provide added support to the repaired valve. If the heart valve is too damaged to repair, the valve is replaced with either a bioprosthetic (tissue) valve or mechanical valve. If a mechanical valve is used, an anticoagulation medication like Warfarin will be necessary. For patients with Marfan syndrome, a mitral valve repair or replacement may be done in conjunction with an aortic root repair or independently.
The incision options for mitral valve repair or replacement is a full sternotomy (a 6- to 8-inch incision down the middle of your chest), a mini-sternotomy incision (about 3 inches), or a thoracotomy, which involves a small incision between the ribs. After a full sternotomy or mini-sternotomy is performed, the breastbone is wired together and the incision is closed with sutures.
For more information regarding Marfan syndrome and related disorders, please contact the Bluhm Cardiovascular Institute at 312-NM-HEART (664-3278) or request a first time appointment online.
In addition, the most credible source of information about Marfan syndrome is the National Marfan Foundation (NMF). Please visit the NMF website.