Bicuspid Aortic Valve | The Marfan Foundation

Bicuspid Aortic Valve

Bicuspid aortic valve is a defect in the heart’s aortic valve that is present at birth (congenital). The exact cause of bicuspid aortic valve is not known, but some medical experts suggest it may be caused by a connective tissue disorder similar to that which causes the heart and blood vessel problems in Marfan syndrome.

Complications of bicuspid aortic valve include aortic aneurysm (bulge) and dissection (tear), which can be life-threatening, making it important for the condition to be diagnosed early and accurately. People who have been diagnosed with bicuspid aortic valve should be under the ongoing care of a heart valve disease specialist who can monitor changes in the heart, valves, and aorta over time.

What is bicuspid aortic valve?

The aorta is the major blood vessel that carries blood from the heart to the rest of the body. The aortic valve allows oxygen-rich blood to flow from the heart to the aorta (the major blood vessel that carries blood from the heart), and prevents blood from leaking back into the heart (aortic regurgitation).

Normally, the aortic valve has three flaps controlling this blood flow. In a person with Bicuspid Aortic Valve, there are only two flaps and the valve may not be able to completely stop blood from leaking back into the heart.  In addition, the aortic valve may become stiff (aortic stenosis), making the heart pump harder to get past the valve. The aorta may also become enlarged.

What are the signs and symptoms of bicuspid aortic valve?

People with bicuspid aortic valve may have abnormal coronary arteries (blood vessels that branch off from the aorta near the heart), an aortic aneurysm, an abnormal thoracic aorta (the portion of the aorta that passes through the upper chest), and unstable high blood pressure.

Other signs of bicuspid aortic valve include:

  • Enlarged heart
  • Heart murmur
  • Weak pulse in the wrists and ankles
  • Aortic stenosis
  • Aortic regurgitation

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Bicuspid Aortic Valve