Bicuspid Aortic Valve Disease

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Bicuspid aortic valve disease (BAVD) is a congenital heart defect in which the aortic valve is missing a flap, preventing it from closing properly. Most people with BAVD will require surgical repair or replacement of their aortic valve at some point in their life, although this typically occurs later in life.


If left untreated, BAVD can lead to:

Our Approach to Treating Bicuspid Aortic Valve Disease

What Are the Symptoms of BAVD?

Even though it’s present at birth, BAVD can be asymptomatic for many years. If and when symptoms do appear, they can include: 

  • Chest pain 
  • Fainting 
  • Heart murmurs
  • Shortness of breath and dizziness 

What Causes BAVD?

A healthy aortic valve has three flaps that open and close to control blood flow and prevent blood flowing backwards into the heart. In BAVD, the aortic valve cannot work properly because it has only two flaps. When the valve does not close properly, blood can flow back into the heart (called aortic regurgitation). Over time, the valve can also become stiff and narrow (called aortic stenosis).


The cause of BAVD is unknown, but it is thought to be caused by a connective tissue disorder, similar to the condition that causes Marfan syndrome.


How Is BAVD Diagnosed?

If your doctor suspects you have BAVD, he or she will perform a physical exam, which includes listening to the heart, and will recommend a variety of tests that can help evaluate your heart, including:


In addition, we may use an electrocardiogram (EKG) to test your heart’s electrical activity, or perform a coronary angiogram (an X-ray of the heart's blood vessels using a special dye) to help with diagnosis.


How Is BAVD treated?

Some patients diagnosed with BAVD will require surgical treatment to repair or replace the valve and part of the aorta. Surgical treatment may include a catheter-based aortic valve replacement, such as TAVR, or, in more severe cases, open surgery to repair or replace the valve.

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    A Congenital Defect Repaired with a New Heart Valve: Susan’s Story

    Born with a congenital heart defect called bicuspid aortic valve, Susan Dickson eventually needed a valve replacement. Read Susan’s story >