What’s a bicuspid aortic valve?

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A bicuspid aortic valve, which has two cusps instead of three, can be benign or lead to heart problems. It is often not noticed until later in life and may require antibiotics during dental procedures. Symptoms vary and can lead to aortic stenosis or regurgitation, which may require medication or surgery.

The aortic valve, which connects the left ventricle to the aorta, normally has three cusps or flaps that open and close to allow blood to pass from the heart to the body. A bicuspid valve is so called because it has two cusps instead of three, and this congenital condition can be completely benign or can lead to problems with left heart function or valve function. Many times, the presence of the bicuspid aortic valve is not noticed until some other heart problem resulting from the double flap arrangement occurs, and this usually does not occur until at least the second or later decade of life, unless the heart has no other birth defects that require early attention.

Generally, a bicuspid aortic valve isn’t routinely suspected unless children have conditions such as Turner or Williams syndromes, but there also seems to be a strong factor of heredity. If there is a family history of bicuspid valve or aortic valve stenosis, parents may want to have a child evaluated. A typical evaluation includes an echocardiogram, which can visualize the valves and determine how the heart is functioning in response to the abnormal arrangement of the double flap.

It may make sense to diagnose even a perfectly healthy child because having a bicuspid aortic valve increases the risk of developing bacterial endocarditis. A person with a two-leaf valve may need to take antibiotics during dental procedures to reduce this risk. People with a functional bicuspid aortic valve are also encouraged to follow a low-fat diet to avoid high cholesterol levels which can cause aortic narrowing or stenosis.

About one in three people with a bicuspid aortic valve develop complications from it, usually after age 10. Whether or not the condition has been diagnosed, symptoms of complications of a bicuspid valve can vary. More commonly, people may report symptoms such as pale skin, tiredness, or sometimes chest pain. They may notice that the heartbeat is too fast or slow and many people may feel breathless. As the valve or the left side of the heart becomes more compromised, conditions such as cyanosis or blue around the nail beds may be noticed, and a person may have fainting spells.

The highest risks are for developing aortic stenosis (narrowing) so that less blood can pass to the aorta or aortic regurgitation (regurgitation), where blood backflows from the valve into the left ventricle. Depending on the severity, doctors may address these conditions with medications that help improve heart function and reduce water retention. More serious damage might require surgery or catheter work that expands the valve, repairs the flaps to work more effectively, or replaces the valve with an artificial or cadaveric valve.




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