woman with hands on chest
woman with hands on chest
woman with hands on chest

Bicuspid Aortic Valve

What is bicuspid aortic valve?

Bicuspid aortic valve is a type of abnormality in the aortic valve in the heart. In bicuspid aortic valve, the valve has only two small parts, called leaflets, instead of the normal three. This condition is present from birth. It can occur with other heart defects.

The heart's ventricles are the two lower chambers of the heart. The left ventricle pumps oxygenated blood to the body. It connects to the aorta, the main blood vessel leading to the body. Between the left ventricle and the aorta is the aortic valve, one of the heart's four valves. These valves help the blood flow through the heart's four chambers and out to the body.

The aortic valve normally has three small parts, called leaflets. Sometimes, two leaflets develop instead of three. These leaflets may be thicker and stiffer than normal, too. That may cause aortic stenosis. In aortic stenosis, the valve won't open as easily as it should when the heart squeezes. The left side of the heart has to work much harder than it normally would to get blood out to the body. Over time, this can damage the heart muscle.

Sometimes, the bicuspid aortic valve is leaky. That allows blood to flow back into the heart when the heart relaxes, instead of flowing forward into the body the way it should. This process is called aortic regurgitation. It can also make the heart work harder.

Only a small portion of people have a bicuspid aortic valve. But it is one of the most common heart defects present from birth. It is more common in boys than in girls.

What causes bicuspid aortic valve?

No one knows exactly what causes most cases of bicuspid aortic valve. Somehow, the valve does not develop normally before birth.

Bicuspid aortic valve tends to run in families. So it may result from gene defect. Researchers are still trying to learn more about the specific genes involved. It might also happen if a mother is exposed to certain infections or drugs while pregnant.

Certain factors can make the bicuspid aortic valve more likely to have problems. Some of these are:

  • High cholesterol levels
  • High blood pressure
  • Smoking
  • Chronic kidney disease

What are the symptoms of bicuspid aortic valve?

Some infants with very narrow aortic valves might have symptoms early on. Most of these valves work well for many years, though. There might not be any symptoms. Or symptoms may not appear until adulthood. Many times, people may not even realize they have a bicuspid aortic valve until they have it diagnosed as adults. Most commonly, people start to have symptoms some time in middle age.

Over time, the valve can become harder to open. Or it can become leakier. Symptoms can gradually develop. They may include:

  • Shortness of breath with exertion
  • Chest pain
  • Lightheadedness or fainting

These symptoms may look like other health problems. Always see your healthcare provider for a diagnosis.

How is bicuspid aortic valve diagnosed?

Your healthcare provider will ask about your symptoms and past health. You will also need a physical exam. Some tests can also help with the diagnosis. These include:

  • Echocardiography, the most important test, to show the movement of blood through the valve
  • Electrocardiogram, to check the heart’s electrical rhythm
  • Chest X-ray, to view the heart anatomy and lungs
  • Cardiac CT or MRI, if more detail is needed about the valve, heart, or aorta

Sometimes, bicuspid aortic valve first appears on an echocardiogram done for other reasons. A cardiologist might first diagnose it.

How is bicuspid aortic valve treated?

Your healthcare provider may choose to monitor you with periodic echocardiograms. If your stenosis or regurgitation is more severe, he or she might need to do this testing as often as once a year.

Currently, there are no treatments known to prevent bicuspid valve from developing stenosis or regurgitation. Medicines such as statins to lower cholesterol may help some people.

If you have symptoms, your healthcare provider may recommend repairing or replacing the valve. That is the only certain treatment. Some people have this done during childhood. But other people may not need it until adulthood.

Your healthcare provider might recommend repairing the valve with a procedure using a long, thin tube (catheter). This tube is threaded through a blood vessel all the way to the aortic valve. In other cases, your healthcare provider might recommend open heart surgery. The aortic valve can then be replaced or fixed.

Healthcare providers sometimes use donor valves from cadavers for valve replacement. Other times, they use artificial valves. Some artificial valves require you to take blood thinners lifelong. Sometimes surgeons replace the bicuspid aortic valve with the person's own pulmonary valve and use a donor valve to replace the pulmonary valve. Each type of procedure and surgery has its own risks and benefits. Talk with your healthcare provider about what makes sense for you.

If you are pregnant or considering becoming pregnant, talk with your healthcare provider about treatment. You may need treatment even if you don't usually have symptoms.

What are the complications of bicuspid aortic valve?

An untreated bicuspid aortic valve can eventually lead to symptoms of heart failure. These include shortness of breath, fatigue, and swelling. Also, an aortic aneurysm might develop downstream from the aortic valve and can lead to bleeding or rupture. Healthcare providers prefer to treat bicuspid aortic valve before heart failure develops.

Endocarditis is another possible complication. It's an infection of the heart valves. Your healthcare provider may give you special instructions about how to help prevent it.

People with bicuspid aortic valve can also have abnormal heart rhythms like atrial fibrillation. In rare cases, such rhythms can cause sudden death. That may be more likely to happen after exercise.

How to manage bicuspid aortic valve

Your healthcare provider may give you other instructions for managing your bicuspid aortic valve and any other existing heart disease. These might include:

  • Eating a heart-healthy diet
  • Maintaining a healthy weight
  • Exercising regularly (ask if you have any restrictions)
  • Taking medicine for high cholesterol or high blood pressure if needed
  • Avoiding smoking
  • Practicing good dental hygiene

If you have had a valve replacement, you may also need to take antibiotics before certain medical and dental procedures. Ask your healthcare provider if this applies to you. Some people will also need to take lifelong medicine to prevent blood clots.

When should I call my healthcare provider?

If your symptoms are gradually getting worse, see your healthcare provider soon. See a healthcare provider right away if you have symptoms like severe chest pain or shortness of breath.

Key points about bicuspid aortic valve

  • Bicuspid aortic valve is a type of abnormality of the aortic valve. It makes the heart work harder than it should.
  • Some people with bicuspid aortic valve will have symptoms in early childhood. Others won't have symptoms until adulthood, if ever.
  • The valve can gradually become harder to open or leakier over time.
  • Your healthcare provider may choose to monitor your aortic valve. Eventually, you may need treatment to repair or replace the bicuspid aortic valve.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

  • Know the reason for your visit and what you want to happen.
  • Before your visit, write down questions you want answered.
  • Bring someone with you to help you ask questions and remember what your provider tells you.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.
  • Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
  • Ask if your condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if you do not take the medicine or have the test or procedure.
  • If you have a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your provider if you have questions.

Johns Hopkins Adult Congenital Heart Disease Center

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The Johns Hopkins Adult Congenital Heart Disease Center provides lifelong heart care for patients diagnosed with congenital heart diseases.

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