Bicuspid aortic valve (BAV)

When your baby has a bicuspid aortic valve, it may not be diagnosed right away because symptoms often don’t appear until they’re older – sometimes not until adulthood. Their abnormal valve may function by itself for a long time. Or their heart may compensate by working harder.

Whenever your child is diagnosed, count on the pediatric cardiology team at Advocate Children’s Hospital to provide access to the best heart care available. Most people born with a bicuspid aortic valve live normal lives with treatment and monitoring.

What is bicuspid aortic valve (BAV)?

The human heart has four valves that control how blood flows through the heart and lungs called the pulmonary, mitral, aortic and tricuspid valves. Each valve has strong flaps of tissue called cusps that open and close to allow blood to flow in the right direction and pause when needed.

Bicuspid aortic valve disease is the most common congenital heart disease. It affects about 1% of the population. A normal aortic valve has three cusps. A bicuspid aortic valve forms abnormally and has two cusps.

Another congenital heart condition that sometimes occurs along with bicuspid aortic valve is aortic coarctation – narrowing of the aorta (main artery of the body).

The aortic valve manages the flow of oxygen-rich blood from the left bottom chamber of the heart to the aorta. When your baby or child has narrowing of the aortic artery, they may also have an enlarged heart because their heart has to work harder to make enough blood move through the aortic valve.

Bicuspid aortic valve symptoms

If your baby has a bicuspid aortic valve, they’re unlikely to have symptoms at birth. Some common bicuspid aortic valve symptoms are:

• Fatigue
• Shortness of breath
• Chest pain
• Swelling (edema) in legs, ankles or feet

If your baby or child seems to have serious chest pain or you think they might be having a heart attack, call 911 or take them to the emergency room immediately.

Complications of bicuspid aortic valve

A bicuspid aortic valve often doesn’t open as wide or close completely compared to a normal aortic valve. It can lead to:

• Aortic stenosis: Narrowing of the aortic valve that makes it hard for it to open wide enough so blood can pass through.
• Aortic regurgitation: When blood flows backward through the aortic valve.

Other complications of bicuspid aortic valve disease can include heart failure, infective endocarditis, high blood pressure in the lungs and sudden cardiac arrest.

Bicuspid aortic valve diagnosis

When your baby has signs of a heart defect, their doctor will be sure to listen for a heart murmur, which can be a sign of BAV. They’ll check the strength of their pulse at the wrists and ankles, and perform pulse oximetry to measure the amount of oxygen in your baby’s blood. Their doctor will also ask about potential bicuspid aortic valve symptoms.

They’ll also order cardiac imaging to check for signs of aortic regurgitation, aortic stenosis or an enlarged heart. Your baby may go through an echocardiogram, MRI, chest X-ray or EKG.

Bicuspid aortic valve treatment

When your child has serious symptoms from bicuspid aortic valve disease, their doctor will consult with a team of cardiologists, cardiovascular surgeons and other specialists to develop a treatment plan with you.

Balloon valvuloplasty is a nonsurgical procedure that’s used to increase the opening size of the valve when there is aortic stenosis. A balloon is inserted through a catheter, placed in the valve and inflated to widen the opening.

When is surgery needed for a bicuspid aortic valve?

Heart surgery for a bicuspid aortic valve is needed when symptoms can’t be managed well enough with other treatments. Valve repair or replacement may be recommended.

When valves are replaced surgically, your child’s surgeon may use mechanical valves or biological valves made of animal tissue. Mechanical valves are often longer lasting.

Sometimes, human heart valves are used, but the supply is limited. Minimally invasive techniques will be used when possible, but some conditions require open heart surgery.

Learn more about our success with pediatric heart surgery.

After bicuspid aortic valve treatment

Maintaining a healthy weight and exercising regularly will help keep your child’s heart healthy. People who’ve had heart surgery should avoid tobacco.

When a mechanical valve has been used for a replacement, your child will need to take blood thinners for the rest of their life.

Your child will need to come in for checkups regularly so they can be monitored for changes and complications. At Advocate Children’s Hospital, we offer ongoing support groups and other resources to support your child and your family.

Contact us with your questions about heart care for your child.

Find an Advocate Children's heart center location near you.