Can bicuspid aortic valve disease cause complications?

Yes, about 30% of people with bicuspid aortic valve disease develop complications. They can be very serious, even life-threatening. This is why people diagnosed with BAVD should be under the ongoing care of a specialist in heart valve disease who can monitor changes in the heart, valves and aorta over time.

The major complications are:

Heart Failure

Over time, a stenotic valve will cause the left ventricle to thicken due to the extra pumping required to force blood through the valve. This enlarged ventricle and in inefficient pumping can lead to heart failure. Smoking or high cholesterol can accelerate the process.

Aortic Aneurysm and Dissection

The underlying connective tissue disorder that causes BAVD also affects the tissue layers that form the walls of the aorta. As the tissue layers degenerate, the aorta walls lose their strength and stretch out of shape. Eventually, this can cause the formation of an aneurysm, a bulging out area of the aorta where the walls are thin and over-stretched.

The aneurysm can rupture or the inner and outer tissue layers of the aorta can shred apart (aortic dissection). Rupture and dissection are life-threatening emergencies. Cleveland Clinic heart and valve specialists are experienced at monitoring the aorta in patients with BAVD to plan surgical intervention before rupture or dissection occurs.

In the Cleveland Clinic experience, with over 5,000 patients with BAVD, 20 % had associated aneurysms. At Cleveland Clinic, we have developed a special mathematical formula we use to determine when surgery is necessary, based on the patient’s height and weight and the size of the aorta. The formula described by Dr. Svensson is the ratio between the cross sectional area of the aorta in centimeters and the height in meters. If the measurement exceeds 10, surgery is usually considered.

What is the treatment for bicuspid aortic valve disease?

In the most severe cases, when symptoms are present at birth or in early infancy, surgical repair of the valve must be performed immediately. In other cases, people can go their whole lives without knowing they have BAVD.

About 80 percent of people with BAVD will require surgical treatment to repair or replace the valve and part of the aorta, usually when they are in their 30s or 40s. Cleveland Clinic specialists can determine the best time to do the surgical repair and whether multiple repairs to the valve and aorta can be done at the same time if needed. As of December 2010, they have operated on 4,700 patients with BAVD, the largest experience in the world in treating this disease.

For the patient who has stenosis and symptoms, the preferred treatment is to replace the valve with a mechanical or biological valve. The mortality rate for this procedure at Cleveland Clinic is less than 1%.

When regurgitation is present, the aortic valve can be repaired successfully. Cleveland Clinic is one of only a handful of centers in the world to repair bicuspid aortic valves, and 91% of patients who undergo the procedure here do not need another valve operation for at least 10 years. After repair, if failure does occur, it will take place in the first year to 18 months after surgery - but thereafter the risks of re-operation are low. However, because the valve is abnormal, it is likely that it will need to be replaced after a few decades.

An aorta that is enlarged and forming an aneurysm can be repaired, but at Cleveland Clinic we more often replace the damaged section with a section of plastic tubing that is sewn into position. This is usually done with a minimally invasive “J” incision. The timing for repair of an aortic aneurysm is critical. When the valve is faulty and the aorta has enlarged to greater than 4.5 cm, we repair the aorta at the same time we repair or replace the valve. If the aorta has enlarged but the valve is still good, the optimal time to repair the aorta varies. At Cleveland Clinic, we have developed a special mathematical formula we use to determine this, based on the patient’s height and weight and the size of the aorta - surgery is usually recommended when the aortic cross sectional area in square centimeters, divided by the patient's height in meters is more than 10.

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