A bicuspid aortic valve is a congenital heart condition that can sometimes make it harder for the heart to pump blood to meet the body’s needs.

The aortic valve is one of the four valves in the heart. It allows blood to move from the left ventricle up into the aorta, which is the large artery that delivers blood to most of the body.

Usually, the aortic valve has three leaflets or flaps that open and close as the heart beats to allow for the proper flow of blood out of the heart. A bicuspid valve has two flaps, which can lead to cardiac complications.

However, treatment is possible for a bicuspid aortic valve. Options include medications, a heart-healthy lifestyle, or even surgery if needed. Aortic valve repair and replacement operations are common procedures that often result in positive long-term outcomes.

When your heart beats (or contracts), the flaps of the aortic valve open up and blood is pushed into the aorta.

Blood moves through the aorta and down through the chest and abdomen. The blood then travels through a network of smaller arteries and capillaries to nourish organs, muscles, and other tissue.

When the heart rests, the flaps close tightly so that no blood flows back into the heart. A tricuspid valve with three leaflets can usually open easily and close securely.

With a bicuspid valve, the leaflets may be thicker than usual, making them harder to open properly. This condition, called aortic valve stenosis, can cause the heart to work harder to pump blood out to the aorta.

Sometimes, the leaflets become thicker over time due to blood flowing unevenly across the valve. This means that some people with a bicuspid aortic valve will start out with regular function, but it will deteriorate over time.

In other cases, the bicuspid valve can’t close tightly. When blood flows back from the aorta through the leaky valve and into the heart, the condition is called aortic regurgitation. This can force the heart to work harder than usual.

Illustration of healthy tricuspid aortic valve and blood flow vs. bicuspid aortic valveShare on Pinterest
A bicuspid aortic valve can cause problems with blood flow from the heart to the body.

A bicuspid aortic valve is present at birth, but it’s unclear why some babies are born with it. Researchers are looking for specific gene mutations that may be associated with bicuspid aortic valve disease since the condition tends to run in families.

A birthing parent’s exposure to serious infection or certain powerful medications, like antiepileptic drugs, may also raise the risk of congenital valve disease, according to 2014 research.

A 2019 report suggests that bicuspid aortic valve disease is the most common congenital heart valve problem, affecting between 0.5 and 2 percent of the general population. Males are about twice as likely as females to develop the condition.

Bicuspid aortic valve can run in families. If you’re diagnosed with a bicuspid aortic valve, any of your first degree family members (parents, siblings, or children) should be screened with an echocardiogram once to assess how their valve looks.

If the valve can open and close properly initially, there may be no symptoms for a long time. Sometimes, symptoms don’t develop until adulthood. But if a baby is born with a very narrow aortic valve, serious symptoms may appear soon after birth.

These symptoms can include:

The challenge for doctors and parents is that these symptoms can be signs of other conditions, such as heart failure.

A bicuspid aortic valve causes the heart to have to pump harder to make sure enough blood reaches the entire body. This can cause complications.

A 2019 review of studies suggests that most people with a bicuspid aortic valve will develop a related complication.

Some of the more common complications include:

  • aortic stenosis
  • aortic regurgitation
  • heart failure, where a weakened heart can no longer pump blood well enough for the body’s demands
  • aortic aneurysm, where a bulge forms in the aorta, raising the risk of it rupturing
  • endocarditis, an infection of the heart
  • irregular heart rhythms (arrhythmias), where the heart beats irregularly instead of in the usual synchronized rhythm

People with a bicuspid aortic valve should be screened for aortopathies, a group of diseases that affect the aorta. Congenital heart defects can often lead to an aorta that is enlarged (aneurysm) or at risk for rupture, according to 2015 research.

Your doctor will review your symptoms and medical history. A physical exam that includes using a stethoscope to listen carefully to the heart should also be part of the appointment.

If your doctor detects a heart murmur, it may be an indication that more tests are needed. This is often how a bicuspid aortic valve is first diagnosed, especially in people without symptoms.

The main test to diagnose a bicuspid aortic valve is an echocardiogram. Echocardiography uses sound waves to produce moving images of the heart on a computer screen.

This type of screening shows how blood flows through the heart and can often reveal problems with the heart’s valves or blood vessels.

For a more detailed view, medical staff may use a transesophageal echocardiogram. They’ll numb your throat before guiding a transducer down your esophagus. They can then see some chambers of the heart that don’t usually show up on a regular echocardiogram.

Other tests your doctor may perform include:

Depending on the severity of your bicuspid aortic valve, treatment options range from regular appointments with your cardiologist to valve repair or replacement surgery.

If there are no symptoms, doctors may monitor your heart periodically with echocardiograms. They’ll look for changes in valve function or signs that the heart is having to work harder.

You should discuss with your doctor whether you need antibiotics before any dental procedures. Some doctors recommend antibiotics for people at risk of developing endocarditis from an infection.

Although the American Heart Association’s guidelines no longer include a bicuspid aortic valve as a major risk factor, there are some cases where your doctor may still recommend antibiotics.

When symptoms are present, you may need to replace or repair the valve. In some cases, doctors perform these procedures as open heart surgery. For others, the procedure can be done with a catheter inserted into a blood vessel in the leg and guided up to the heart.

If aortic stenosis has developed, the valve may have to be replaced. The replacement will be a tricuspid valve, either mechanical or made from valve tissue harvested from a cow or pig.

Doctors may advise people who get a mechanical valve to take blood thinners for life to prevent blood clots from forming in the heart.

If aortic regurgitation is the problem, valve repair may be possible, often with a catheter. During the procedure, a surgeon reshapes the valve to have three leaflets that can open and close more effectively.

After aortic valve surgery, your doctor may advise you to participate in cardiac rehabilitation, a 12-week program that teaches diet, exercise, and lifestyle strategies to support heart health.

Managing a bicuspid aortic valve is similar to managing many other heart conditions. Medications may be helpful for some people, but adopting a heart-healthy lifestyle is necessary for everyone.

Medications

Medications to maintain a typical blood pressure can be helpful if high blood pressure is a problem. You may also be prescribed statins or other medications that help lower cholesterol.

If you develop an arrhythmia, your doctor may recommend medications that help stabilize your heart rhythm. They may also prescribe blood thinners to lower your risk of getting a blood clot. A blood clot can lead to a heart attack or stroke.

Lifestyle changes

Doctors recommend a lifestyle that supports cardiovascular health. If you need help getting started, your doctor or a cardiac rehabilitation specialist may provide you with some useful strategies.

In general, the following behaviors contribute to good heart health:

  • eating a Mediterranean diet or other heart-healthy eating plans
  • regular exercise, including at least 30 to 40 minutes of aerobic exercise daily
  • avoiding smoking
  • getting 7 to 8 hours of sleep per night
  • managing stress
  • managing blood glucose levels
  • limiting sodium to less than 1500 milligrams per day

Survival rates for aortic valve surgeries are relatively high. People who have valves repaired may need subsequent repairs made if they are young. Valve replacement procedures are usually permanent.

A 2021 study comparing survival rates among various bicuspid aortic valve patient groups suggests that low risk individuals between the ages of 60 and 64 experienced a median survival time after surgery of about 16 years.

Those age 85 and older had about a 6-year survival expectancy. Individuals in high risk groups averaged somewhat lower life expectancy outcomes.

Can a bicuspid aortic valve fix itself?

A bicuspid valve will never grow a third flap on its own. However, surgery can often repair or replace an aortic valve so that it can function properly.

How long can you live with a bicuspid aortic valve without surgery?

Some people with a bicuspid valve never experience symptoms or develop problems that lead to heart failure or other complications. Aortic stenosis is the most serious threat in people with a bicuspid aortic valve.

A 2020 study suggests that the life expectancy of someone with severe aortic stenosis is only about 2 or 3 years after diagnosis if the condition is left untreated.

Will I need a pacemaker after surgery?

Typically, heart valve surgery does not mean a pacemaker is necessary. But if you also have an arrhythmia, a pacemaker or implantable cardioverter-defibrillator (ICD) may be necessary.

Since bicuspid aortic valve disease is such a common heart condition, many cardiologists and cardiac surgeons are familiar with how to treat it successfully.

Newer developments, such as transcatheter aortic valve implantation (TAVI), are improving survival and quality of life in people with bicuspid aortic valves and other similar conditions.

A 2020 study of adults with unicuspid or bicuspid aortic valves suggests that long-term surgical outcomes, like reductions in complications and mortality, work very well in patients with low risks.

The key is to be proactive about your heart health and get multiple opinions, if appropriate, about when surgery may be the best approach to preserve aortic valve function.