Unicuspid Aortic Valve

What is a unicuspid aortic valve?

A unicuspid aortic valve is a rare heart malformation some people have at birth. Unicuspid means you have one leaflet instead of the typical three on your aortic valve. Leaflets are the flaps that open and close to let oxygen-rich blood move from your left ventricle (lower heart chamber) to your aorta. This blood flows out from your aorta to your whole body through a network of arteries.

People with this condition may also have other heart issues like:

• Aortopathy.
• Ventricular septal defect (VSD).
• Patent ductus arteriosus (PDA).
• Coronary artery anomalies.

Only 0.02% of the general population has a unicuspid aortic valve. It happens more often in people assigned male at birth (AMAB).

Types of unicuspid aortic valve

Unicuspid aortic valve disease has two types. The difference is in their commissure, or the space between where leaflets next to each other attach to the valve wall. You can think of it like your lips that are permanently attached to your face, but they can come together and separate.

The types of unicuspid aortic valve are:

• Acommissural. This is the more severe type. It has no commissures and doesn’t attach to your aorta. The leaflets fuse together, leaving a pinhole opening in the middle for blood to go through.
• Unicommissural. This type is less severe but more common. It has one commissure and attaches to your aorta. This type has a slit-like opening that some compare to a fish’s mouth.

What are the symptoms?

Unicuspid aortic valve symptoms in adults include:

• Shortness of breath.
• Dizziness.
• Angina.
• Fainting.

Children with this condition usually have left-sided heart failure and aren’t gaining weight like they should.

Symptoms of the acommissural type appear in babies. People with the unicommissural type don’t have symptoms until they’re adults in their 20s or 30s.

What causes a unicuspid aortic valve?

With unicuspid aortic valve disease, the typical three leaflets start to develop but then fuse together instead of staying separate. This happens before birth.

Researchers suspect but haven’t proven that people inherit this condition.

What are the complications of a unicuspid aortic valve?

People with a unicuspid aortic valve can have:

• Stenosis (narrowing) of their aortic valve.
• Regurgitation (leakiness) of their aortic valve.
• Infective endocarditis.
• A higher risk of aortic dissection.

How is a unicuspid aortic valve diagnosed?

A healthcare provider will listen to your heart during a physical exam. If they hear a heart murmur where your aorta is, they may want to order tests.

What tests will be done to diagnose a unicuspid aortic valve?

Tests to diagnose a unicuspid aortic valve include:

• Transthoracic echocardiogram (TTE).
• Transesophageal echocardiogram (TEE).
• Cardiac computed tomography (CT).
• Heart MRI (magnetic resonance imaging).

How is it treated?

Unicuspid aortic valve treatment options for children include:

• Surgical valvotomy (cutting the valve tissue to make more leaflets).
• Valvuloplasty.
• Commissurotomy.
• Ross procedure.

Adults usually have surgery for unicuspid aortic valve repair or replacement.

Complications/side effects of the treatment

Complications of treatment may include:

• Infection.
• Blood clots.
• Bleeding.
• Endocarditis.
• Issues with a patch a provider uses in a valve repair.
• Complete heart block.
• Heart attack.
• Valve leak that allows blood to flow the wrong way.
• Abnormal heart rhythm.

How long does it take to recover from this treatment?

It depends on the procedure you have. You may need anywhere from one week to several months to recover.

What can I expect if I have a unicuspid aortic valve?

If you have unicuspid aortic valve disease, you’ll need surgery. In fact, a healthcare provider may need to repair or replace your valve more than once in your lifetime. Also, you may have other issues with your heart. And you may need to take blood thinners (anticoagulants) after surgery, depending on the type of valve you get.

How long a unicuspid aortic valve lasts

A unicuspid aortic valve doesn’t correct itself. It lasts until you have surgery to fix it.

Outlook for unicuspid aortic valve

A study found that 97% of people with unicuspid aortic valve who had aortic valve surgery were alive 10 years later.

For people under 65 with a unicuspid aortic valve, getting a new prosthetic aortic valve might reduce your life expectancy by more than 10 years. This is why researchers prefer the Ross procedure for young, active people. With the Ross procedure, a provider uses your own pulmonary valve to replace your diseased aortic valve. Then they insert a pulmonary homograft (valve from a donated human heart) into the old pulmonary valve position.

How do I take care of myself?

After surgery, follow your provider’s instructions for taking care of your incisions (cuts). They should also tell you how long you need to rest before driving or taking on other daily activities.

When should I see my healthcare provider?

Even if you think you’re too young for heart issues, you should see a provider if you have chest pain or trouble breathing. Once you’ve had surgery for a unicuspid aortic valve, you’ll need follow-up appointments with your provider. You may need emergency care if you have complications (like an infection) after surgery.

What questions should I ask my healthcare provider?

Questions to ask your healthcare provider may include:

• What’s the best treatment for me?
• What are the differences between the types of valve replacement?
• How soon do I need to have surgery?
• How long will a replacement valve last?

A note from Cleveland Clinic

Hearing that something isn’t working right in your heart isn’t easy. But over time, providers have developed treatments for a unicuspid aortic valve. Talk with your provider about what’s best for you. Because a unicuspid aortic valve is a rare condition, it’s in your best interest to find a provider who has solid experience with it. For example, researchers say a surgeon should perform at least 15 Ross procedures a year.