A bicuspid aortic valve allows blood to flow backward into the heart, increasing the risk of an aortic aneurysm.

The aortic valve, which separates the heart from the rest of the body, normally has three flaps, or leaflets, that open to let blood flow out to the heart and then close to prevent blood from leaking backward into the heart from the aorta. A bicuspid aortic valve has only two leaflets.

If you have a bicuspid aortic valve, you may develop a narrowed or leaky aortic valve, but this may not occur until adulthood. A narrowed valve may make it difficult for blood to flow through the valve, and the heart has to work harder to pump blood through a narrowed valve. In other cases, the valve may leak so that blood goes backwards from the aorta into the heart. Over time, this can cause the heart to enlarge and work harder to compensate for the leaking valve.

In many people with a bicuspid aortic valve, the aorta is also abnormal and may enlarge or stretch out over time, putting them at risk for aortic aneurysms and rupture. If left untreated, this may eventually be life-threatening.

Causes

A bicuspid aortic valve occurs during fetal development. This heart defect may occur more commonly in some families. If bicuspid aortic valve runs in your family, you may want to have genetic testing.

Symptoms

Symptoms of bicuspid aortic valve include fatigue, and the first indication you have bicuspid aortic valve may be a heart murmur.

Why choose Ohio State for bicuspid aortic valve treatment?

Ohio State’s Wexner Medical Center offers a team approach to congenital heart defects (CHDs), which means each patient is evaluated by an adult congenital heart disease (ACHD) specialist, and when appropriate, a cardiac surgeon, an imaging specialist and an interventional cardiologist with training in CHD. This integrated approach means that each patient’s disease is treated individually, with that particular patient’s needs and physical condition in mind.

Anyone who has ever been diagnosed with or treated for bicuspid aortic valve should have lifelong care from a cardiologist who specializes in congenital heart defects. We provide this care from birth to adulthood; we partner with Nationwide Children’s Hospital to provide the resources necessary for the care of adult congenital cardiac patients through the Columbus Ohio Adult Congenital Heart Disease Program (COACH).

The COACH program focuses on:
• Adults with congenital heart disease (CHD)
• Pulmonary hypertension
• Cardiovascular connective tissue disorders
• Pregnancy in women with heart disease
• Transition of adolescents with CHD into adult congenital heart disease (ACHD) care
• Training of future ACHD providers

Diagnosis

Due to improvements in technology, some congenital heart defects can be detected before a baby is born. In other cases, severe heart defects are detected shortly after birth. Some cases of less-severe disease may not be detected until childhood, late adolescence or even adulthood.

Tests used to diagnose bicuspid aortic valve include:

Electrocardiogram (EKG/ECG) – A test that records the electrical activity of the heart.

Echocardiogram (also called echo) – A test that uses sound waves to assess the function and structure of the heart muscle and valves.

Chest X-ray – A radiograph or picture of the heart and lungs including blood vessels, ribs and bones of the spine.

Cardiac MRI (cardiac magnetic resonance imaging or CMR) – A noninvasive, sophisticated imaging procedure that uses large magnets and a computer to produce detailed images of the structure and function of the heart while it is beating.

Cardiac catheterization – A procedure to measure pressures in the heart and check for problems in the coronary arteries. A long, thin tube (catheter) is inserted into an artery or vein in the groin, arm or neck, and then threaded to the heart. The physician injects a contrast solution into the artery and takes X-rays to check for blockage and other abnormalities.

Treatments

Medications

Medications cannot treat a bicuspid aortic valve. However, your physician may prescribe certain medications such as beta-blockers or angiotensin receptor blockers (ARBs) to slow the progression of aortic enlargement.

Nonsurgical procedures

Balloon valvuloplasty (valvotomy) is a nonsurgical procedure to treat some cases of bicuspid aortic valve.

Surgical procedures

Aortic valve replacement may be necessary for bicuspid aortic valves that have become severely narrowed or leak severely. Surgery may also be necessary if the aorta has gotten too large, regardless of the amount of valve narrowing or leakage.

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