Bicuspid Aortic Valve

(See also Overview of Heart Defects.)

The aortic valve is the valve that opens with each heartbeat to allow blood to flow from the heart to the body. A normal aortic valve has three cusps, or leaflets.

The most common heart birth defect is bicuspid aortic valve. When the aortic valve is bicuspid, it may not open normally (a condition called aortic stenosis) or may not close normally, allowing blood to leak back into the heart instead of going to the body (called aortic regurgitation). Bicuspid aortic valve is the most common cause of aortic stenosis. People with a bicuspid aortic valve also are at risk for enlargement of the aorta and infection of the heart valves (infective endocarditis).

A bicuspid aortic valve may function normally and not cause problems in infants, older children, and adolescents, but it may cause problems in adulthood. The specific symptoms depend on the complications that develop. People with infective endocarditis may develop fever. If aortic stenosis or aortic regurgitation develops, the person may tire easily, and have shortness of breath, fainting, awareness of heartbeats (palpitations), and chest pain.

Doctors suspect a bicuspid aortic valve if they hear a heart murmur or a clicking sound during an examination with a stethoscope. Echocardiography is then done to confirm the diagnosis.

The aortic valve may need to be repaired or replaced if aortic regurgitation or stenosis occurs. Aortic stenosis can also sometimes be treated with balloon dilation during a cardiac catheterization procedure. Progressive enlargement of the aorta in some people may require surgery to replace a portion of the aorta.

Because there is a family tendency (heredity) to bicuspid aortic valves, close relatives of people with a bicuspid aortic valve should undergo screening echocardiography.