Bicuspid Aortic Valve

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.

Why Choose UPMC for Bicuspid Aortic Valve Repair?

The UPMC Center for Thoracic Aortic Disease provides expert care for people living with BAVD.

We offer the latest valve repair and replacement surgeries to treat your heart problem.

Contact the UPMC Heart and Vascular Institute

To request an appointment, contact the UPMC Heart and Vascular Institute:

What Is a Bicuspid Aortic Valve?

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.

How common is it to have a bicuspid aortic valve?

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.

What causes bicuspid aortic valve 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.

How serious is a bicuspid aortic valve?

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.

Bicuspid aortic valve complications

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.

Bicuspid Aortic Valve Disease Symptoms and Diagnosis

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.

Bicuspid aortic valve symptoms

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:

  • A heart murmur throughout childhood. Your doctor can detect this by listening to your heart.
  • Shortness of breath when you exercise, even mildly.
  • Dizziness or fainting.
  • Chest pain.

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.

Bicuspid aortic valve diagnosis

A doctor may order further tests if they hear a heart murmur.

To diagnose BAVD, your doctor may order tests such as:

  • Echocardiogram — a type of ultrasound your doctor may use to look for a bicuspid aortic valve.
  • Transesophageal echocardiogram — an ultrasound performed through the esophagus to get an up-close view of the heart valve.
  • CT aortogram — a scan using contrast dye that provides a view of the aorta and aortic valve structure. Doctors use this test to confirm a diagnosis of BAVD.
  • MRI — an imaging scan that tests the heart, valves, and aorta.
  • EKG — a test that measures your heart's electrical activity to diagnose problems of BAVD.
  • Angiography — an x-ray of the blood vessels to look for problems linked to a bicuspid aortic valve. 

Learn More About Heart Disease at UPMC Health Beat

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Bicuspid Aortic Valve Treatment

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.

Bicuspid aortic valve 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.

Aortic valve repair

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.

Valve-sparing aortic root replacement (David procedure)

With this technique, your surgeon:

  • Removes the ascending aorta and coronary arteries just above the aortic valve.
  • Attaches a synthetic graft with small stitches and reattaches the coronary arteries to the graft.
  • This eliminates the need to replace the aortic valve with a mechanical one. But your surgeon may suggest replacing the valve as well.

Minimally invasive valve replacement

During this procedure, your surgeon:

  • Makes small incisions through the breastbone or between the ribs.
  • Uses small robot-controlled tools to replace the damaged valve. The replacement valve often contains tissue from pigs, cows, or humans.

Transcatheter aortic valve replacement (TAVR)

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. 

Learn More About Heart Valve Disease Treatments

Visit the UPMC HealthBeat blog to read these posts: