Surgery for Marfan Syndrome

Some people with Marfan syndrome require open heart surgery to repair an aortic aneurysm that puts them at increased risk of acute aortic rupture or aortic dissection. The larger the aneurysm, or bulge in the vessel, is, the greater the risk of rupture.

Elective open heart surgery, sometimes done before any symptoms occur, is often recommended for people who have aneurysmal dilation of the aortic root that is at least 4.5 centimeters wide. Before surgery, a nutrient-rich solution is given through a vein via an intravenous (IV) infusion to safely slow the heart. A heart–lung machine is used to keep blood pumping to the body during surgery.

To repair the aneurysm, the surgeon makes an 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. For some people with aortic root aneurysm, the aortic valve also needs to be repaired or replaced.

Emergency surgery is almost always needed for severe aortic conditions caused by Marfan syndrome. They include an acute aortic dissection, when the aortic wall tears; aortic valve disease; and mitral valve disease, when the valves that control the flow of blood to, from, and within the heart don’t close properly.

People with Marfan syndrome who have previously had surgery or have had their aortic dissection repaired may need additional surgery later to repair dissection or aneurysms of the thoracic or thoracoabdominal aorta, which both extend into the abdomen.

Recovery from Open Heart Surgery

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.

Surgeries for Other Marfan Syndrome Complications