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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 complication 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:

• sudden dissection (tear within the inner wall of the aortic root and/or aorta)
  
  
• rupture of the aortic root and/or aorta which can lead to sudden death
  

In addition, the mitral valve can prolapse causing backward flow of blood within the heart. This creates a murmur (abnormal extra sound made when the heart beats) that is heard in many, but not all, patients with the Marfan syndrome. If the leaky mitral valve is left untreated, heart failure may develop.

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 to 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 - synthetic (manmade) material.

• Modified Bentall Procedure: The traditional aortic root replacement surgery consists of replacing the aortic valve and the aortic root with a composite (combined) valve-graft. Typically, the composite valve-graft consists of either a tissue valve or a mechanical valve (to replace the diseased aortic valve) attached to a tubular Dacron graft (to replace the diseased aortic root). If a mechanical valve is used an anticoagulation medication like warfarin (Coumadin®) will be necessary. If a tissue valve (made from animal or human tissue) is used anticoagulation medication is not necessary.

• David Procedure: The aortic root replacement surgery that repairs the diseased aortic root with a Dacron graft but spares the non-diseased aortic valve, meaning the original aortic valve is left in place.

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 - synthetic (manmade) material.

• Total Arch Repair: The aortic arch replacement surgery where the entire aortic arch, except for the brachiocephalic patch (area where the left inominate artery, left common carotid artery, and the left subclavian artery originate), is removed and replaced with a Dacron graft.

• Hemi-Arch Repair: The aortic arch replacement surgery where a smaller portion of the aortic arch is removed and replaced with a Dacron graft. More native aortic arch tissue is left in place surrounding the left inominate artery, left common carotid artery, and the left subclavian artery than in the total arch repair surgery.

• Descending Aortic Arch Repair: Aneurysms or dissections may also occur in the descending aorta just beyond the left inominate artery, left common carotid artery, and the left subclavian artery. Again, the diseased portion of the aorta is removed and replaced with a Dacron graft.

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 breast bone 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 (Coumadin(r)) 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 incision which involves a 6 to 8 inch incision down the middle of your chest, a mini-sternotomy incision which involves a small 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 breast bone is wired together and the incision is closed with sutures.

Contact
For more information regarding Marfan syndrome and related disorders, please contact the Bluhm Cardiovascular Institute at (866) 662-8467, or request an appointment online.

In addition, the most credible source of information about Marfan syndrome is the National Marfan Foundation (NMF). Please visit the NMF Web site.

Visit the Northwestern Memorial Hospital's Health Learning Center and Alberto Culver Women's Health Learning Center. These state-of-the-art health information libraries are among the largest hospital-based learning facilities of their kind. For more information call (312) 926-5465.

Review Date: 05/08