Bicuspid aortic valve (BAV)

A bicuspid aortic valve (BAV) is an aortic valve that contains 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 BAV 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.

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

BAV is often associated with other conditions and complications that may develop over time, including:

• Aortic abnormalities: BAV increases the risk of thoracic aortic aneurysm, aortic dissection, and aortic coarctation.
• Aortic valve conditions: A bicuspid valve is more likely to develop aortic stenosis and aortic regurgitation.
• Arrhythmia: Some people with BAV may also experience an irregular heart rhythm like atrial fibrillation (AFib).
• Infective endocarditis: People with BAV have a higher risk of endocarditis, a bacterial infection of the heart valves.
• Genetic disorders: BAV is often associated with connective tissue disorders, such as Marfan syndrome and Ehlers-Danlos syndrome.

It is not known what causes bicuspid aortic valve. Because it is a hereditary congenital heart defect, some researchers believe it may result from a genetic mutation or abnormality. Research is ongoing to learn more about bicuspid aortic valve.

Even though a bicuspid valve is present at birth, doctors may not diagnose it until you become symptomatic. Though some infants born with BAV may have narrow valves and experience symptoms at a young age, the condition is often not discovered until 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

Approximately two percent of the population has BAV, 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 this heart valve disease.

If you’ve been diagnosed with BAV, Penn Medicine’s surgical team will monitor 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 BAV eventually require repair or replacement of the aortic valve. Our cardiac surgeons repair the aortic valve for patients with BAV and regurgitation. Valve replacement is recommended for bicuspid aortic valve with stenosis.

Penn Medicine surgeons and cardiologists treat BAV-associated conditions with procedures including:

• Aortic valve repair and replacement surgery, which repairs or replaces the bicuspid valve through traditional open-chest surgery.
• Transcatheter aortic valve replacement (TAVR), which is an innovative, nonsurgical valve replacement procedure used for select patients with bicuspid aortic valve and aortic stenosis.
• Valve-sparing aortic root replacement, which is used to repair the aortic root and valve in patients with BAV and associated thoracic aortic aneurysm.