What is Bicuspid Aortic Valve Disease?

Bicuspid aortic valve is a genetic heart condition that many people don’t even know they have until later in life. For some people, it never causes symptoms, but for others, it can cause serious complications. It runs in families, so a doctor’s evaluation can help keep your heart healthy.

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What Is Bicuspid Aortic Valve?

The aortic valve connects the heart to the aorta. The aorta is the largest artery in the body and carries blood away from the heart to the rest of the body.

A normal aortic valve has three flaps, called leaflets, that open and close for blood to flow away from the heart. A bicuspid aortic valve only has two flaps. The two flaps may not close as they should and blood can flow backward into the heart.

Bicuspid aortic valve forms early during the development of an embryo. The condition affects about 1-2% of the population and runs in families. A specialist, such
as those at the UPMC Center for Thoracic Aortic Disease, can perform testing to look for the condition.

Usually, echocardiogram imaging can identify a bicuspid aortic valve. Your doctor may recommend other imaging tests to confirm a diagnosis if needed.

What Are the Symptoms and Complications?

When the valve doesn’t work properly, you can develop aortic valve disease. People with bicuspid aortic valve are at higher risk of heart failure or aortic aneurysm.

A malfunctioning valve puts more strain on the heart. That can cause symptoms, such as:

  • A heart murmur, which is often found during childhood.
  • Shortness of breath with exercise.
  • Dizziness or fainting.
  • Chest pain.

In rare cases, the disease may prove severe and require surgery soon after birth.

How Is Bicuspid Aortic Valve Treated?

Most people eventually need surgery to treat a bicuspid aortic valve. Surgery can repair or replace the valve depending on how severe the condition is. Surgical options include:

  • Aortic valve repair. Complex aortic valve repairs are typically performed by open heart surgery with standard or mini sternotomy. The natural valve leaflets are repaired so that the flaps close properly.
  • Valve-sparing aortic root replacement (also known as the David Procedure) is done through open heart surgery. The surgeon keeps the natural valve and resuspends it in a tubular graft. People who have this procedure (which also treats an aortic root aneurysm) do not need a mechanical or tissue valve. Their own valve is “spared”.
  • Aortic valve replacement is performed when the valve is too diseased to be repaired or spared. A surgeon will replace the valve with either a tissue valve (from pigs, cows, or humans) or a mechanical valve. Aortic valve replacements can be done a few different ways:
    • Standard aortic valve replacement. This traditional approach is performed through open heart surgery by dividing the breastbone in half.
    • Minimally invasive aortic valve replacement. A surgeon can perform an aortic valve replacement through a “mini sternotomy” in which only the top 1/3 of the sternum is divided in half. For some patients, minimally invasive aortic valve replacement can be performed through a small 3 inch incision on the right side of the chest between the ribs. Both of these procedures allows for faster recovery time, shorter hospital stay, and less pain postoperatively.
  • Transcatheter aortic valve replacement (TAVR). During this surgery, the surgeon reaches the heart through a catheter inserted into an artery in the groin. The surgeon places a new valve within the existing valve. This procedure may be an option for older people who cannot undergo open-heart surgery.

Appropriate treatment depends on your age, overall health, and the severity of your valve disease. These surgeries are complex and require skilled surgeons. The surgeons at UPMC have extensive experience treating bicuspid aortic valve disease.

The UPMC Heart and Vascular Institute provides assessment, diagnosis, and treatment at one location. You have a team that’s connected and communicates to ensure you receive excellent care.

If someone in your family has a bicuspid aortic valve, you should talk to a doctor. Monitoring the valve’s function and your heart health allows you to detect issues early.

About Heart and Vascular Institute

The UPMC Heart and Vascular Institute has long been a leader in cardiovascular care, with a rich history in clinical research and innovation. As one of the first heart transplant centers in the country and as the developer of one of the first heart-assist devices, UPMC has contributed to advancing the field of cardiovascular medicine. We strive to provide the most advanced, cutting-edge care for our patients, treating both common and complex conditions. We also offer services that seek to improve the health of our communities, including heart screenings, free clinics, and heart health education. Find an expert near you.