Bicuspid Aortic Valve

Basic Facts

The aortic valve acts as the exit door of the heart, and it is normally made of 3 flaps called “cusps.” These cusps open to let blood flow from the main pumping chamber (left ventricle) to the aorta, then shut to prevent flow back into the left ventricle. In this congenital heart defect, the aortic valve is made of 2 cusps instead of 3.

Bicuspid aortic valve is more likely to lead to:
- Aortic stenosis (AS) = narrowing of the aortic valve.
- Aortic regurgitation (AR) = leaking back through aortic valve
- Thoracic aortic aneurysm (TAA) = enlargement of the aorta.

Though present at birth, most do not experience any symptoms until adulthood, but tend to develop symptoms 5-10 years earlier than those with tricuspid valves.
In mild to moderate cases there may be no symptoms, as valve changes usually advance slowly over years, giving the body time to adjust.
As it advances to a more severe stenosis or regurgitation, it can lead to lightheadedness, shortness of breath, and/or chest pains with exertion resulting in decreased exercise tolerance. The left ventricle has to work harder over time, which can lead to congestive heart failure and other complications, especially if untreated.

This is a congenital heart defect, meaning it is present from birth. It occurs in about 1-2% of the population.

Your healthcare provider may hear a murmur or click when the heart is squeezing (systole).

Other tests may include:

ECG – measures the electrical activity of the heart, regularity of heartbeats, and screens for thickening of heart muscle (hypertrophy) and/or heart-muscle damage.
Echocardiogram – ultrasound to evaluate heart structure & function, including the heart valves, in real time.
Cardiac catheterization - threading a small tube (catheter) up a blood vessel into the heart chambers to measure pressures across the valve.
If you have a bicuspid valve, 1st degree relatives should also be screened

Control blood pressure, as high pressure increases wear and tear on the valve over time.

Mild to moderate valve disease and/or aortic aneurysm - “watch and wait” approach with a visit and/or echocardiogram every 1-3 years.
Severe valve disease and/or aortic aneurysm - surgery to replace a damaged aortic valve and/or aorta may be necessary. Most replacement valves nowadays are made from animal tissue (bioprosthetic valves). Some may be made from metal (mechanical valves). The type of replacement valve selected depends on the patient’s age, condition, and their specific anatomy.
Valve can be replaced via open heart surgery. Though a much less invasive procedure called TAVR is available, it is not always an option for bicuspid valves.