Bicuspid aortic valve disease (BAVD) is a heart defect you're born with.
A bicuspid aortic valve has two flaps, called leaflets, instead of the normal three. This can cause problems with blood flow from the heart to the aorta, the body's main artery.
The UPMC Center for Aortic Disease provides expert care for people living with BAVD.
We offer the latest valve repair and replacement surgeries to treat your heart problem.
To request an appointment, contact the UPMC Heart and Vascular Institute:
Arteries carry blood away from the heart.
The aortic valve is a one-way valve that connects the heart and the aorta. Blood flows from the heart to the aorta, the main artery that carries oxygen-rich blood throughout the body.
A healthy aortic valve has three flaps, called leaflets. The flaps open and close to allow blood to flow into the body.
A bicuspid aortic valve only has two flaps, so it may not close as it should. This causes blood to flow backward into the heart.
About 2 percent of all people in the U.S. have BAVD. It's far more common in males than in females, with twice as many men having the disease.
BAVD is an inherited birth defect. Parents pass it down to their children.
It's not fully clear what causes the defect, but some studies suggest a connective tissue disorder plays a part.
The two-leaflet valve starts in the early stages of a woman's pregnancy, when the heart forms. A child has the defect from birth.
Some people go years without knowing they have BAVD.
People with a bicuspid aortic valve may get aortic valve stenosis earlier than those with a tricuspid valve.
The disease can cause regurgitation (leakage) or stenosis (narrowing) of the valve.
In some cases, people with a BAVD aren't aware they have it.
In rare yet severe cases, a child born with the disease needs surgery soon after birth.
A doctor may find the problem when they listen to the heartbeat and hear a murmur, or an abnormal heart sound.
If the two valves don't fully close, blood may flow backward slightly, into the heart. Doctors call this aortic valve insufficiency. The heart has to work hard to get rid of that blood, which strains and weakens its lower left chamber.
This extra strain can cause the left chamber to expand over time and lead to heart failure or an aortic aneurysm. Both of these conditions can result from stress to the heart or aorta due to damage from a weakened muscle.
Weak connective tissue within the vascular system — worsened by age and hardening of the arteries — can also cause aortic valve insufficiency.
A bicuspid aortic valve is a congenital abnormality of the aortic valve, occuring in 1-2% of the population from birth. The aortic valve typically has 3 leaflets. In bicuspid aortic valves, 2 of the leaflets fuse in the womb, resulting in only 2 leaflets at birth.
Although people are born with a bicuspid aortic valve, it often goes undiagnosed until adulthood. One exception is in cases when a newborn has a pronounced valve defect and needs treatment right away.
In most cases, the valve works normally until middle age.
Many people never have symptoms.
But if the valve doesn't work right, it places extra strain on your heart experiences can cause symptoms, such as:
BAVD may get worse over time, causing more of these symptoms.
People with aortic aneurysms may also never have symptoms.
Talk to your doctor if you have a family history of bicuspid aortic valve.
A doctor may order further tests if they hear a heart murmur.
To diagnose BAVD, your doctor may order tests such as:
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Most people with bicuspid aortic valves need surgery in adulthood. Some children with severe BAVD may need surgery right away at birth.
About 20% of people who have BAVD will not need surgery.
Based on how complex your case is, you may need a valve repair or valve replacement.
Your doctor can advise you which type is right for you.
If you have regurgitation — that is, blood flowing back to the heart — you may need valve repair surgery.
Your surgeon makes small incisions in the chest and repairs the valve so that the flaps close properly.
With this technique, your surgeon:
During this procedure, your surgeon:
Some people who can't have open-heart surgery may be a good fit for TAVR.
This minimally invasive technique accesses the heart through a catheter inserted in a vein in the leg or groin. The surgeon inserts a new valve within the existing damaged valve.
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