What is a Bicuspid Aortic Valve?

The aortic valve, which connects the left ventricle to the aorta, ordinarily has three cusps or leaflets that open and close to allow the passage of blood from the heart to the body. A bicuspid valve is so named because it has two cusps instead of three, and this congenital condition can be fully benign or it may lead to problems with left heart function or valve function. Many times, presence of the bicuspid aortic valve isn’t noted until some other heart problem stemming from the double leaflet arrangement occurs, and this usually doesn’t happen until at least the second or later decades of life, unless the heart has other congenital problems that demand earlier attention.

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

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

Approximately one in three people with a bicuspid aortic valve develop complications from it, usually after the age of 10. Whether or not the condition has been diagnosed, symptoms of complications of a bicuspid valve can vary. Most commonly, people may report symptoms like paleness, tiredness, or sometimes pain in the chest. They may notice that the heartbeat is too quick or slow, and many people can feel like they are out of breath. As the valve or the left side of the heart becomes more compromised, conditions like cyanosis or blueness might be noted around the nail beds, and a person could have fainting episodes.

The highest risks are for developing aortic stenosis (narrowing) so that less blood is able to pass to the aorta, or aortic insufficiency (regurgitation), where blood leaks back from the valve into the left ventricle. Depending on severity, doctors may address these conditions with medications that help improve heart function and reduce water retention. More severe damage could require surgery or catheter intervention that expands the valve, repairs the leaflets to function more effectively, or replaces the valve with an artificial or cadaver valve.