Bicuspid Aortic Valve Disease

Overview

What is a bicuspid aortic valve?

A bicuspid aortic valve is a congenital heart defect that affects the one-way valve between your heart and your aorta, known as the aortic valve. Normally, your aortic valve has three cusps that regulate blood flow from your heart to your aorta. But if you have a bicuspid aortic valve, you only have two cusps.

Your aortic valve cusps, also called leaflets or flaps, open and shut to regulate blood flow between your heart and your aorta. These flaps make sure your oxygen-rich blood flows in the right direction: Out of your heart and into your aorta. They prevent blood from flowing backward into your heart. The flaps should open widely to let blood flow out of your heart and then close securely.

If you only have two flaps, rather than three, it’s harder for your aortic valve to function in the right way. You may develop valve diseases like aortic regurgitation or aortic stenosis.

Bicuspid aortic valve disease (BAVD)

Bicuspid aortic valve disease (BAVD) refers to problems caused by your valve anatomy. When your aortic valve only has two flaps, it may have trouble closing and opening properly.

If your aortic valve doesn’t close tightly enough, it’s called aortic regurgitation. Blood leaks backward from your aorta into your heart.

If your aortic valve doesn’t open wide enough, it’s called aortic stenosis. That means less blood can flow from your heart to nourish the rest of your body.

You might have aortic regurgitation or stenosis for years and not even realize it. You may not feel any symptoms. Your aortic valve does its best to keep getting the job done. It’s just not as efficient as a valve with three flaps. Over time, though, your bicuspid aortic valve makes your heart work harder, and this can lead to serious complications.

In addition, bicuspid aortic valve disease can be associated with dilation of your aorta, particularly around the aortic root, known as an aortic aneurysm.

How serious is a bicuspid aortic valve?

About 1 in 3 people with a bicuspid aortic valve develop complications. They can be very serious or even fatal. That’s why people diagnosed with bicuspid aortic valve disease need ongoing medical checkups and testing.

You might not feel any symptoms until the disease has progressed far or you have a medical emergency. But imaging tests can catch subtle changes before you even notice symptoms. And advances in treatment can help you live a long and healthy life.

How common is bicuspid aortic valve?

As many as 1 in 50 people have a bicuspid aortic valve. It’s twice as common in men and people assigned male at birth (AMAB) compared with women and people assigned female at birth (AFAB).

People with Turner syndrome have an increased risk of heart problems including a bicuspid aortic valve.

Symptoms and Causes

What are the symptoms of a bicuspid aortic valve?

You may not have any symptoms of a bicuspid aortic valve. You could live many years without even knowing your anatomy is different. But over time, you may develop symptoms of aortic regurgitation or stenosis. If your child has a bicuspid aortic valve, you may notice symptoms soon after birth (in severe cases). But it’s more likely that you won’t notice them until later on.

Symptoms in adults include:

Symptoms in infants and children include:

  • Becoming tired easily.
  • Chest pain.
  • Fainting.
  • Pale skin.
  • Trouble breathing.
  • Trouble with feeding or gaining weight.

Many people don’t realize they have a bicuspid aortic valve until it’s diagnosed through medical testing. That’s because symptoms are easy to miss. Your family may notice a change before you do. One helpful tip is to think about how much activity you can do in a typical day. Compare it with one year ago, or even six months ago. Do you get tired more easily? Do you need more time to rest? If so, that may signal your heart is working harder to keep up.

That’s why it’s so important to keep up with your medical appointments. Talk with your healthcare provider about any changes you notice in your daily life.

What causes a bicuspid aortic valve?

Researchers don’t know what causes a bicuspid aortic valve to form. It’s the most common congenital heart defect (present at birth). It develops early in pregnancy.

Is a bicuspid aortic valve genetic?

A bicuspid aortic valve can run in families. If a close family member (parent, child or sibling) has this heart defect, talk with your healthcare provider. They may want to run some tests to check your heart anatomy and function.

If you’ve been diagnosed with a bicuspid aortic valve and are planning a pregnancy, talk with your provider. You can’t prevent this defect from happening, but you can monitor your pregnancy and try to diagnose any problems early.

Research will continue to explore the role of genetic mutations in causing bicuspid aortic valve disease. It seems to be an inherited heart defect, but researchers don’t yet fully understand the details.

Diagnosis and Tests

How is a bicuspid aortic valve diagnosed?

A bicuspid aortic valve may be diagnosed during pregnancy through a cardiac prenatal ultrasound. Other times, it’s diagnosed when a child has other heart problems that cause symptoms. Testing then reveals the bicuspid aortic valve.

But other people go many years without knowing they have this condition. The first sign may be a heart murmur that your provider hears through a stethoscope. After that, your provider may run imaging tests to check your heart structure and function.

Tests to diagnose a bicuspid aortic valve

Your provider may run the following tests to check your heart and diagnose a bicuspid aortic valve:

Management and Treatment

What is the treatment for a bicuspid aortic valve?

About 4 out of 5 people with bicuspid aortic valve disease need aortic valve surgery. New technology continues to improve outcomes and reduce complications. Many people can have minimally invasive techniques instead of traditional open heart surgery. Your surgeon will either repair or replace your aortic valve. Your surgeon may also fix other issues (like an aneurysm) at the same time.

Your provider will evaluate your situation, run some tests and talk with you about your treatment options. Your treatment will depend on the condition of your aortic valve and aorta. But your provider will also consider your overall heart health, age and other medical conditions.

It’s important to have surgery early enough to prevent permanent damage to your heart. Even if you don’t have symptoms, your provider may want to fix the problem now to avoid complications down the road. The best timing is different for everyone. Your medical care team will discuss your options with you.

Traditional aortic valve surgery vs. minimally invasive aortic valve surgery

Traditional (open heart) aortic valve surgery involves a 6- to 8-inch incision down the middle of your sternum (breastbone). Your sternum is divided so your surgeon can directly access your heart.

Minimally invasive aortic valve surgery involves a smaller “J” incision (2 to 4 inches). The incision will be at the top of your sternum or between your ribs. Your surgeon doesn’t need to open your whole chest. This procedure reduces blood loss and allows you to leave the hospital sooner. You may also recover more quickly.

Aortic valve repair vs. aortic valve replacement

Aortic valve repair fixes your valve without replacing it. It can be a good option for aortic regurgitation (leaky valve). But it can’t be used to treat aortic stenosis (narrowing). Aortic valve repair can often be done through a minimally invasive surgery with the “J” incision. Your surgeon will reshape your aortic valve cusps to help them open and close more completely.

Aortic valve replacement is used when a repair isn’t possible. It involves removing your valve and giving you a new one. This procedure can be done through traditional open surgery. Or, it can use the minimally invasive transcatheter aortic valve replacement (TAVR) method.

There are two main options for your new valve: biological or mechanical.

  • Biological valve. This type of valve is used 80% of the time. It’s made from pig or cow tissue. This valve is safe and durable. But after ten years, you may need another replacement surgery.
  • Mechanical valve. This type of valve is very durable and can last the rest of your life. But you need to take blood thinners for your whole life to keep it working safely.

Many people prefer a biological valve so they don’t have to take blood thinners. But it depends on your age, other medical conditions and personal preferences. Your provider will discuss your options and help you decide what’s best for you.

Another option for valve replacement is the Ross procedure (also called the switch procedure). It involves using your pulmonary valve to replace your aortic valve. This procedure can be a good option for people under age 50 who want to avoid long-term use of blood thinners.

Risks of surgery

Generally, valve surgeries have a low risk of complications. But possible risks include:

About 98% of people who have valve surgery survive and have a normal life expectancy.

Prevention

How can I prevent a bicuspid aortic valve?

There’s no way to prevent a bicuspid aortic valve. It’s congenital (something you’re born with). But you can make lifestyle changes that reduce your risk of other heart problems like atherosclerosis and coronary artery disease. If you have a bicuspid aortic valve, talk with your provider about managing heart disease risk factors including:

Outlook / Prognosis

What is the life expectancy of someone with a bicuspid aortic valve?

If you have a bicuspid aortic valve, you can live just as long as someone without this condition. But you need regular follow-ups and testing with your healthcare provider. Your provider will check your valve and make sure you get treatment when needed. Treatment is essential to avoiding complications.

What are the complications of bicuspid aortic valve disease?

Bicuspid aortic valve disease can lead to serious complications if untreated. These include:

  • Heart failure. Aortic valve stenosis forces your heart to work harder to force blood through your valve. Your heart’s main pumping chamber, your left ventricle, thickens and gets bigger. It can’t pump blood as efficiently as it should. Over time, this leads to heart failure. Other risk factors like smoking or high cholesterol can speed up this process.
  • Aortic aneurysm rupture and dissection. About 1 in 3 people who have a bicuspid aortic valve also have a dilated (enlarged) aorta above their valve. As your aorta gets bigger, its walls lose their strength and stretch out of shape. Eventually, this can cause an aortic aneurysm (bulging of the aorta) to form. If the aneurysm grows too big, it can rupture or dissect. These are life-threatening emergencies.

Living With

Can I live a normal life with a bicuspid aortic valve?

Yes, you can live a normal life and do most of the things you love. You may need to make some lifestyle changes to help keep your heart healthy and reduce stress on your heart. These include:

You’ll need regular appointments with your provider to check your heart function. If you have valve surgery, you’ll have follow-ups after two weeks, three months and six months. You’ll then have an appointment every year. These appointments are essential for keeping you healthy and checking your valve.

When should I be concerned about a bicuspid aortic valve?

This defect is a concern when it starts interfering with your heart function. A bicuspid aortic valve can cause aortic stenosis and regurgitation. Over time, these problems can lead to permanent heart damage.

You may not know this is happening. A bicuspid aortic valve may not have any symptoms. Your provider will monitor your valve and your heart and tell you if there are major problems. Keep your appointments. Get regular testing to catch problems before they become severe.

When should I see my healthcare provider?

Your provider will let you know how often you need to come in for appointments. If you had surgery, you’ll have several follow-ups within the first year and then one each year.

If you have a family history of heart valve problems but haven’t been diagnosed, your provider may refer you to a cardiologist (heart doctor). Your cardiologist can screen you for heart problems and offer guidance.

When should I call 911?

Call 911 immediately if you have symptoms of an aneurysm rupture or dissection:

  • Sudden sharp and tearing pain in your chest or back.
  • Clammy, sweaty skin.
  • Dizziness.
  • Fainting.
  • Fast heartbeat.
  • Nausea and vomiting.
  • Shortness of breath.

What questions should I ask my doctor?

If you were diagnosed with a bicuspid aortic valve, talk with your provider about the condition of your heart and whether you need treatment. Some questions include:

  • Are there signs of aortic regurgitation (leaking)?
  • Are there signs of aortic stenosis (narrowing)?
  • Will I need surgery?
  • Do I have any other heart problems?
  • What lifestyle changes should I make to help my heart?

If your child was just diagnosed with a bicuspid aortic valve, ask your provider:

  • Are there signs of aortic leaking or narrowing?
  • Will my child need surgery?
  • What are the risks of surgery for my child?
  • What will recovery look like?
  • What symptoms or problems should I look out for at home?
  • What activities should my child avoid?
  • How can I lower my child’s risk of heart problems down the road?
  • Do you have resources to help me explain this diagnosis to my child?

A note from Cleveland Clinic

A bicuspid aortic valve is a common congenital heart defect. But knowing it’s common probably doesn’t make you worry any less. Keep in mind that doctors and surgeons see this problem all the time. They’re prepared to help you or your child stay healthy and strong. If you or your child were recently diagnosed, take some time to learn more about the condition. Talk with your provider and get the information you need to help you feel in control of the situation. And remember that we have the technology in place to catch problems early and treat them so you and your family can go on living.

Last reviewed by a Cleveland Clinic medical professional on 05/05/2022.

References

  • Borger MA, Fedak PWM, Stephens EH, et al. The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve-related aortopathy. J Thorac Cardiov Surg. 2018 Aug;156(2): E41-E74. Accessed 5/5/2022.
  • Merck Manual. Bicuspid Aortic Valve. (https://www.merckmanuals.com/home/children-s-health-issues/birth-defects-of-the-heart/bicuspid-aortic-valve) Accessed 5/5/2022.
  • National Health Service. Overview: Aortic Valve Replacement. (https://www.nhs.uk/conditions/aortic-valve-replacement/) Accessed 5/5/2022.
  • The Society of Thoracic Surgeons. Aortic Valve Disease. (https://ctsurgerypatients.org/adult-heart-disease/aortic-valve-disease) Accessed 5/5/2022.
  • U.S. Centers for Disease Control and Prevention. Valvular Heart Disease. (https://www.cdc.gov/heartdisease/valvular_disease.htm) Accessed 5/5/2022.
  • U.S. National Institutes of Health, National Library of Medicine. Bicuspid Aortic Valve. (https://medlineplus.gov/ency/article/007325.htm) Accessed 5/5/2022.

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy