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Congenital Pulmonary Stenosis Balloon Valvuloplasty

What is congenital pulmonary stenosis balloon valvuloplasty?

Congenital pulmonary stenosis is a health problem that is present from birth. It’s when the pulmonary valve in your heart doesn’t fully open. Congenital pulmonary stenosis balloon valvuloplasty is a procedure to fix this problem. This procedure does not need open heart surgery.

The heart’s ventricles are the 2 lower chambers of the heart. The right ventricle pumps blood low in oxygen to the lungs. It connects to the pulmonary artery, the main blood vessel leading to the lungs. Between the right ventricle and the pulmonary artery is the pulmonary valve. It’s one of the heart’s 4 valves. These valves help the blood flow through the heart’s 4 chambers and out to the body. Normally the pulmonary valve opens fully when the right ventricle squeezes. It allows the blood to flow from the right ventricle to the pulmonary artery.

Sometimes, a person may have an abnormally thickened or fused valve. The valve might have an abnormal number of small parts, called leaflets. As a result, the valve can’t open as fully as it normally would. As pressure builds up in the right ventricle, the heart has to work harder to push the blood out to the lungs. Over time, this can damage the overworked heart muscle and lead to symptoms. Sometimes, the area around the valve also fails to form correctly.

Balloon valvuloplasty aims to fix the pulmonary valve. The procedure uses a long, thin tube called a catheter. This tube has an inflatable balloon at its tip. The healthcare provider puts this catheter through a blood vessel in the groin. The catheter then goes all the way to the pulmonary valve. The balloon is then inflated. It stretches the valve and helps open it up. Then blood can flow out to the pulmonary artery without blockage.

Why might I need congenital pulmonary stenosis balloon valvuloplasty?

This procedure is done to ease the symptoms of congenital pulmonary stenosis. Many people who have this condition will not need valvuloplasty. Mild cases may not lead to any symptoms. But if you have a moderate to severe case, you may have tiredness and shortness of breath when exercising.

You may not have any symptoms at first. But you may have them later in childhood or adulthood. People with severe symptoms often need treatment with some sort of procedure or surgery. Treatment is often advised for pregnant women, especially if the stenosis is more severe.

If you do need a procedure, healthcare providers are most likely to advise balloon valvuloplasty. It is less invasive than open heart surgery. Recovery is often shorter, too.

No one knows what causes most cases of congenital pulmonary stenosis. It sometimes happens along with Noonan syndrome, a genetic disease. Or it may happen with other heart defects.

What are the risks of congenital pulmonary stenosis valvuloplasty?

All procedures have risks. Some possible risks of this procedure include:

  • Excess bleeding

  • Infection

  • Blood clot. This can lead to stroke or other problems.

  • Abnormal heart rhythms. These can cause death in rare cases.

  • Pulmonary artery rupture

  • Tearing of the heart

  • Fluid buildup around the heart

  • Pulmonary valve problems (insufficiency)

  • Tricuspid valve problems (insufficiency)

There is also a fairly high risk that the valve will partly close again with time. You may need another valvuloplasty or surgery on the valve.

How do I get ready for a congenital pulmonary stenosis balloon valvuloplasty?

Ask your healthcare provider how to get ready for the procedure. Follow any directions for not eating or drinking before the procedure. You may also need to stop taking certain medicines beforehand.

You may need to have some extra tests before the procedure. These might include:

  • Chest X-ray. This shows the structures in and around your chest.

  • Electrocardiogram. This is done to check your heart rhythm.

  • Blood tests. These are done to check your general health.

  • Echocardiogram. This is done to see your heart anatomy and blood flow through your heart.

  • CT or MRI scans. These imaging tests provide more details about your heart.

  • Heart catheterization. This is to better look at the coronary blood vessels.

Before the procedure, the hair around the area where the catheter will be inserted may be removed.

What happens during congenital pulmonary stenosis balloon valvuloplasty?

Talk with your healthcare provider about what to expect. The procedure often happens in a cardiac catheterization lab. It is done by a cardiologist and a team of specialized nurses. During the procedure, the team will carefully watch your vital signs. In general:

  • You will be given medicine to make you sleepy. But you will be awake.

  • The procedure will take about 2 hours.

  • A numbing medicine will be injected into the area around the groin.

  • The healthcare provider will make a small cut (incision) in the groin to reach a blood vessel.

  • The provider will put a flexible, thin tube (catheter) through the incision.

  • The provider will move the tube to the pulmonary valve. They may use X-ray images to see exactly where the tube is.

  • The provider will thread a very thin wire through the tube.

  • Another tube will be passed over this wire to the pulmonary valve. This tube has a balloon that expands on its end.

  • The provider will inflate the balloon. This will stretch the valve leaflets to make the opening bigger. This may hurt a little. But you can have pain medicine.

  • Once the valve has been opened enough, the balloon will be deflated. Then the provider will remove the balloon and catheters.

  • The incision made in the groin will be closed. A dressing will be applied.

What happens after congenital pulmonary stenosis balloon valvuloplasty?

Ask your healthcare provider about what to expect. In general, after the procedure:

  • You may be sleepy and disoriented when you wake up.

  • The team will carefully watch your vital signs. These include your heart rate, breathing, and blood pressure.

  • You will feel some soreness. But you shouldn’t feel severe pain. Pain medicine is available if needed.

  • You may need to lie flat for a few hours after the procedure without bending your legs. This will help prevent bleeding.

  • You may have follow-up tests, such as an electrocardiogram or an echocardiogram.

  • You may be able to go home the day after the procedure.

After you leave the hospital:

  • Ask what medicine you need to take. Take pain medicine as advised.

  • You can go back to your normal activities fairly quickly. But don't do any strenuous activities or heavy lifting for several days.

  • Stitches or staples will be removed in a follow-up appointment. Be sure to keep all follow-up visits.

  • Follow all the instructions your healthcare provider gives you about medicine, exercise, diet, and wound care.

Most of the time, symptoms improve right away after balloon valvuloplasty. But you will need lifelong care from a cardiologist. They will watch for possible complications from the procedure. Some people will need another valvuloplasty or other procedure in the future.

When to call your healthcare provider

Call your healthcare provider if you have any of these:

  • Your limb with the puncture becomes numb, tingles, feels cold, or turns blue

  • Swelling, more bruising, bleeding, or fluid leaking around the puncture site

  • Bleeding from the puncture site that can't be stopped with firm pressure

  • Fever of 100.4°F (38°C) or above, or as advised by your provider

  • Dizziness

  • Any other symptoms

Call 911

Call 911 right away if you have any of these:

  • Chest pain

  • Shortness of breath

  • Puncture site swells up very fast

  • Bleeding from the puncture site does not slow down when you firmly press on it

  • Other severe symptoms

Next steps

Before you agree to the test or procedure, make sure you know:

  • The name of the test or procedure

  • The reason you are having the test or procedure

  • What results to expect and what they mean

  • The risks and benefits of the test or procedure

  • What the possible side effects or complications are

  • When and where you are to have the test or procedure

  • Who will do the test or procedure and what that person’s qualifications are

  • What would happen if you did not have the test or procedure

  • Any alternative tests or procedures to think about

  • When and how you will get the results

  • Who to call after the test or procedure if you have questions or problems

  • How much you will have to pay for the test or procedure

Medical Reviewers:

  • Dan Brennan MD
  • Rajadurai Samnishanth
  • Stacey Wojcik MBA BSN RN
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