Bicuspid aortic valve disease is a simple yet common congenital heart defect affecting nearly 2% of the population or roughly 1 in 50 people. In a normal heart, the aortic valve will have three cusps that regulate blood flow from the left ventricle into the aorta. When there is an abnormal development of the valve, the heart can have two cusps (bicuspid) or one cusp (unicuspid). Uneven development can present problems over time as the bicuspid aortic valve causes the aortic valve to narrow, ultimately blocking blood flow from the heart into the body.

Every seven minutes, a child in the United States is born with a bicuspid aortic valve, so it is by no means an uncommon occurrence. However, any form of heart defect can be scary and overwhelming. Below are some of the most common questions the Ochsner Health cardiology team fields about bicuspid aortic valve disease. We've also outlined some information that will be useful for both patients and family members of patients.

How is bicuspid aortic valve disease diagnosed?

To diagnose bicuspid aortic valve disease, a doctor may hear an abnormal heart sound, requiring additional heart imaging such as echocardiography imaging. Bicuspid aortic valve disease can be associated with other heart abnormalities, most commonly aneurysms.

Is it possible to be diagnosed with bicuspid aortic valve disease as an adult?

Yes, it is possible to be diagnosed with bicuspid aortic valve disease as an adult. While some severe forms are diagnosed in infancy, it is common to be diagnosed for the first time in adulthood.

What are the symptoms of bicuspid aortic valve disease?

In adults, it is common to experience no symptoms of bicuspid aortic valve disease initially. This is because symptoms are generally related to valve dysfunction, aortic dilation, tears in the aorta or infection. If the valve begins to leak (aortic regurgitation/insufficiency) or becomes narrow (stenosis), symptoms are usually shortness of breath or chest pains.

If the aorta tears (dissection), this can become life-threatening and is recognized as a medical emergency. In the case of an aorta tear, symptoms can include chest and mid-back pain. There is a risk of infection of the heart valve, and symptoms can include fever, chills and fatigue.

How is bicuspid aortic valve disease treated?

Surveillance with heart imaging is essential for the identification of significant valve abnormality or enlargement of the aorta. It is crucial to consider the size of the aorta related to the person's physical size. Good dental hygiene, blood pressure control and appropriate cholesterol control are other meaningful treatment factors.

The American College of Cardiology and the American Heart Association no longer recommend antibiotics before procedures for bicuspid aortic valve disease. This is because the risk of heart valve infection (endocarditis) is extremely low, and there is a high prevalence of antibiotic resistance.

Will I need surgery to correct the defect?

Maybe. People with bicuspid aortic valves are more likely to need surgery than the general population. However, not everybody will. You may need aortic valve replacement and replacement of the aorta at some point in your life.

How does bicuspid aortic valve disease affect my family?

There is a 9% prevalence of bicuspid aortic valve disease within immediate family members. Therefore, the American College of Cardiology and the American Heart Association recommend echocardiographic screenings for immediate family members.

Are there any limitations to exercise?

If there is no significant or only mild valve disease and normal size aorta, then exercise is encouraged. If there is severe valve disease, aerobic exercise can be performed after evaluation by a physician. If there is a significant abnormality in the aorta, aerobic exercises are well-tolerated. However, some limitations need to be discussed with your doctor about competitive sports and weight training.