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What Is Bicuspid Aortic Valve Disease?

A bicuspid aortic valve is an aortic valve with only two cusps (or flaps) instead of three. The aortic valve controls the flow of blood from the left ventricle (chamber) to the aorta, the main artery delivering blood to your body. Bicuspid valves may eventually leak (aortic regurgitation) and/or narrow (aortic stenosis).

It can be years before bicuspid aortic valve disease (BAVD) causes an issue. Once your valve shows signs of regurgitation or stenosis, your heart is likely working harder to pump blood to the body. The extra work can lead to heart failure. It’s important to monitor a bicuspid valve so that any changes can be caught early and treated.

Approximately 2 percent of the population has BAVD, making it the most common congenital heart condition. While there is no known cause, it’s possible to inherit a bicuspid aortic valve. This happens in up to 25 percent of first-degree relatives of people with the condition. Men are twice as likely as women to be born with a bicuspid valve.

Bicuspid Aortic Valve Symptoms

A bicuspid aortic valve is often not symptomatic. As the valve ages and develops conditions such as stenosis or regurgitation, you may begin to feel the symptoms associated with those conditions. Those symptoms may include:

  • Chest pain: Discomfort and tightness are often associated with aortic stenosis.
  • Fatigue: Increased activity may leave you feeling tired and weak.
  • Shortness of breath: Sudden trouble breathing during physical exertion is the main symptom of aortic regurgitation.
  • Fainting or lightheadedness: You may feel dizzy or even lose consciousness during physical activity.
  • Heart murmur: As symptoms worsen, your doctor may hear this abnormal heart sound.

BAVD is often associated with other conditions and complications, including:

Diagnosing a Bicuspid Valve

Even though a bicuspid valve is present at birth, doctors may not diagnose it until you become symptomatic, often in adulthood. If your doctor suspects a bicuspid aortic valve, they will use echocardiography and cardiovascular imaging techniques including:

  • Transthoracic echocardiogram (TTE)
  • Cardiac magnetic resonance imaging (MRI)
  • Transesophageal echocardiogram (TEE)
  • CT scan
  • Coronary angiography

Bicuspid Aortic Valve Treatment at Penn Medicine

If you’ve been diagnosed with BAVD, the surgical team at The Center for Bicuspid Aortic Valve Diseases will monitor you and evaluate your valve and aorta for changes. If changes occur or symptoms arise, your cardiology team will develop a treatment plan.

Approximately 80 percent of people with BAVD eventually require repair or replacement of the aortic valve. For patients with BAVD and regurgitation, our cardiac surgeons repair the aortic valve. Valve replacement is recommended for bicuspid aortic valve with stenosis.

Penn’s surgeons and cardiologists treat BAVD-associated conditions with procedures including:

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