Surgery for Marfan Syndrome

Doctors often recommend surgery for people with severe aortic problems caused by Marfan syndrome. These include aortic dissection, when the aortic wall tears; aortic aneurysm, when the aortic wall bulges or ruptures; or aortic valve disease or mitral valve disease, when the valves that control the flow of blood to, from, and within the heart don’t close properly.

Open Aortic Repair Surgery

Some people with Marfan syndrome require open-heart surgery to repair damage to the aortic wall. For an aortic dissection, your NYU Langone surgeon makes a large incision in the chest and separates the breastbone to reach the aorta. The surgeon replaces the damaged part of the ascending aorta, which extends upward from the aortic root near the heart, with a synthetic material called Dacron®, which usually lasts a lifetime. 

Prior to the procedure, a nutrient-rich solution is given through a vein with intravenous (IV) infusion to safely slow the heart until it’s nearly stopped for surgery. A heart–lung machine is used to keep blood pumping to the body during surgery.

Your surgeon repairs or replaces an aortic valve that stops closing properly. This is called aortic valve disease, in which the valve between the aorta and the left ventricle, or chamber, of the heart fails to close properly, causing blood to flow back into the heart or pressure to build up within the heart. 

For mitral valve disease, in which the valve that controls blood flow between chambers of the heart stops working properly, your surgeon removes the damaged tissue and stabilizes the valve.

All of these open-heart procedures require general anesthesia and a hospital stay of up to one week. During the four to six weeks of recuperation time, your doctor may restrict your activities. You may not be able to lift anything heavier than 10 pounds, and you may need to refrain from activities that strain your chest, such as brisk walking, which can cause pressure in the lungs.

Endovascular Stent Surgery

In rare situations, this procedure may be used to treat heart-related conditions in people with Marfan syndrome. An aortic aneurysm may occur in the chest or the abdomen. Endovascular stent surgery is typically reserved only for treatment of Marfan syndrome–related thoracic aortic aneurysm—a bulging of the upper part of the aorta in the chest—in people who are too weak for open-heart surgery.

During this procedure, a stent graft, which is a tube made of polyester fabric and supported by a metal mesh, is inserted into the femoral artery in the groin through a catheter. A doctor guides the stent graft to the aneurysm in the aorta with two types of imaging procedures: fluoroscopy uses X-rays and transesophageal echocardiography uses sound waves to create pictures of the esophagus and heart. After the stent is in place, blood then flows through it, relieving pressure on the aneurysm and eliminating the risk of rupture.

This surgery uses general anesthesia and requires one or two days in the hospital and up to two weeks of recovery time at home.

Surgeries for Other Marfan Syndrome Complications

Because Marfan syndrome can cause problems with many different organs and the bones, your NYU Langone doctor may recommend other types of surgery. You may need surgery to reduce a buildup of air in the space between the lungs and the chest wall, also called a collapsed lung, or a procedure to fix a dislocation of the eyes’ lenses. In some children, surgery to correct severe scoliosis, which is a curvature of the spine greater than 40 percent, may be necessary.

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