Congenital heart defects in children

Bicuspid aortic valve

Bicuspid aortic valve is a heart problem present at birth. That means it is a congenital heart defect.

The aortic valve is between the left lower heart chamber and the body's main artery, called the aorta. Flaps of tissue on the valve open and close with each heartbeat. The flaps are called cusps. They make sure blood flows in the correct direction.

Usually the aortic valve has three cusps. A bicuspid valve has only two cusps. Rarely, some people are born with an aortic valve that has one cusp or four cusps. A valve with one cusp is called unicuspid. A valve with four cusps is called quadricuspid.

Changes to the aortic valve can cause health problems, including:

• Narrowing of the aortic valve, called aortic valve stenosis. The valve may not open fully. Blood flow from the heart to the body is reduced or blocked.
• Backward flow of blood, called aortic valve regurgitation. Sometimes, the bicuspid aortic valve doesn't close tightly. This causes blood to flow backward.
• Enlarged aorta, called aortopathy. An enlarged aorta increases the risk of a tear in the lining of the aorta. This tear is called an aortic dissection.

Symptoms

If the bicuspid valve causes severe aortic stenosis or severe aortic regurgitation, symptoms may include:

• Chest pain.
• Shortness of breath.
• Difficulty exercising.
• Fainting or near fainting.

Most people with bicuspid aortic valve don't have symptoms of heart valve disease until they're adults. But some infants may have severe symptoms.

Diagnosis

A bicuspid aortic valve may be found when tests are done for another health problem. The health care provider may hear a heart murmur when listening to the heart.

An echocardiogram can confirm a diagnosis of bicuspid aortic valve. This test uses sound waves to create videos of the beating heart. It shows how blood moves through the heart chambers, the heart valves and the aorta.

If you have a bicuspid aortic valve, you'll usually have a CT scan to check for changes in the aorta's size.

Treatment

If you have a bicuspid aortic valve, you are typically referred to a health care provider trained in congenital heart disease. This type of provider is called a congenital cardiologist.

Anyone with a bicuspid aortic valve needs regular health checkups and imaging tests. Echocardiograms check for a narrowed or leaking aortic valve. The test also looks for changes in the size of the aorta.

Treatment for a bicuspid aortic valve depends on the severity of heart valve disease. It may include medicines, procedures and surgery.

Medications

There are no medicines to repair a bicuspid aortic valve. But medicines may be used to treat symptoms caused by heart valve disease. For example, your provider may recommend blood pressure medicine.

Surgeries or other procedures

Surgery may be needed if a bicuspid aortic valve is causing:

• Aortic valve stenosis.
• Aortic valve regurgitation.
• An enlarged aorta.

Surgery is done to repair or replace the aortic valve. The type of surgery done depends on the specific heart valve condition and your symptoms.

• Aortic valve replacement. The surgeon removes the damaged valve. It's replaced with a mechanical valve or a valve made from cow, pig or human heart tissue. The tissue valve is called a biological tissue valve. Sometimes, the aortic valve is replaced with the person's own lung valve. The lung valve is replaced with a lung tissue valve from a deceased donor. This more complicated surgery is called the Ross procedure.

  Biological tissue valves break down over time. They may eventually need to be replaced. If you have a mechanical valve, you need to take blood thinners for life to prevent blood clots. Together, you and your health care provider discuss the benefits and risks of each valve type.

• Aortic root and ascending aorta surgery. Surgeons remove the enlarged section of the aorta located near the heart. It's replaced with a synthetic tube, called a graft, which is sewn into place. Sometimes, only the enlarged section of the aorta is removed and the aortic valve remains. The aortic valve also can be replaced or repaired during this procedure.

• Balloon valvuloplasty. This procedure can treat aortic valve stenosis in infants and children. In adults, the aortic valve tends to narrow again after the procedure. So it's usually only done if you're too ill for surgery or you're waiting for a valve replacement.

  This heart valve procedure uses a thin, flexible tube called a catheter. The catheter has a balloon on the tip. The surgeon inserts the catheter into an artery in the arm or groin. Then the catheter is guided to the aortic valve. Once in place, the balloon inflates, making the valve opening bigger. The balloon is deflated. The catheter and balloon are removed.

Lifestyle and home remedies

Anyone born with a bicuspid aortic valve needs health checkups for life. A provider trained in heart diseases, called a cardiologist, should examine you for changes in your condition.

People with a bicuspid aortic valve are more likely to develop an infection of the heart's lining. This infection is called infective endocarditis. Proper dental care can help lower your risk.

A bicuspid aortic valve can be passed down in families, meaning it's inherited. Parents, children and siblings of someone with a bicuspid aortic valve should have an echocardiogram to check for the condition.

Living with congenital heart defects in children?

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