Surgery for Marfan syndrome

Surgery for Marfan syndrome is aimed at preventing dissection or rupture and treating valve problems.

Aorta surgery

About 40 percent of Marfan syndrome patients will die immediately if aortic dissection occurs. The risk of death is between 1 and 3 percent per hour after the dissection event. Even with emergency surgery, the risk of death is between 10 and 20 percent. The goal is to perform surgery prior to the dissection occurring because:

1. the early results are better (better than 98 % survival), and
2. the long-term life expectancy is better.

The normal aorta measures about 1 inch (2.54 centimeters). When the aorta diameter is more than 4.7 centimeters, or if the aorta is enlarging at a rapid pace, surgery is recommended. The decision to have surgery is based on size of the aorta, expected normal size of the aorta, rate of aortic growth, age, height, gender and family history of aortic dissection.

During non-emergency aorta surgery, the survival rate is greater than 98 percent.

Surgery involves a replacement of the dilated portion of the aorta with a graft. This can involve two techniques:

• traditional method: replace the aorta with a graft and the aortic valve with a mechanical valve
• valve sparing method: replace the aorta with a tube graft and re-implant the native valve

Traditional method

During surgery the heart surgeon removes the area of dissection or aneurysm. Due to the connective tissue disorder, the aorta is prone to tearing, so the heart surgeon must use great care.

A mechanical valve, attached to the end of the aortic graft, is sewn to the annulus (opening) of the aortic valve.

The coronary arteries are reattached to the aortic graft through small buttonholes cut into the graft. Then, the other end of the dacron graft is sewn to the tissue of the aorta.		Repair of entire aorta with graft
Graft with attached mechanical valve (Courtesy of St.Jude Medical™)

Valve remodeling surgery, aortic graft surgery
	Compare the aortic aneurysm to the left with the normal heart below
	The coronary arteries branch off the aorta The aorta is opened and the area of dissection is removed
	The coronary arteries are separated from the diseased aorta. You can see the patient's native aortic valve.
	The dacron graft is sewn to the annulus of the native valve
	The coronary arteries are re-attached to the graft. The other end of the graft is sewn to the aorta.
	The surgery is completed

There are two methods for replacing the aorta without replacing the aortic valve, the valve sparing re-implantation method and the valve remodeling method. Both are used for young people whose aortas are not too enlarged and the aortic valve has not been damaged.

The valve sparing re-implantation method is preferred for those with Marfan Syndrome.

Click here for actual photos of the valve sparing re-implantation method (photos may be graphic)

After aortic graft surgery, patients usually remain in the hospital 5 to 10 days. After a recovery period of six to eight weeks, return to activities is expected.

Valve repair or replacement

A leaky aortic or mitral valve (regurgitation) resulting in changes in the left ventricle (left lower chamber of the heart, heart's major pumping chamber) or heart failure, require surgery to repair or replace the valve.

The mitral valve can be repaired or replaced. This can be done with traditional or minimally invasive techniques. Click here to learn more about valve surgery.

It is recommended that people with Marfan syndrome undergo surgery by heart surgeons who are experienced in this type of surgery. Those that undergo surgery, still require lifelong follow-up and preventive measures to prevent future progression of the disease. The majority of patients who have surgery prior to dissection do not need future surgeries. However, those that are operated on after dissection are at greater risk for future surgeries to repair other sections of the aorta.