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Congenital Pulmonary Stenosis Surgery

What is congenital pulmonary stenosis surgery?

Congenital pulmonary stenosis is when the pulmonary valve doesn’t fully open. This health problem is present from birth. Congenital pulmonary stenosis surgery aims to fix this problem. It is a type of 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 lies the pulmonary valve. It’s 1 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 helps the blood flow from the right ventricle to the pulmonary artery.

Sometimes, a person will have an abnormally thickened or fused valve at birth. 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 harm the overworked heart muscle and lead to symptoms. Sometimes, the area around the valve also doesn’t form in the correct way.

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

Several types of surgery can help fix congenital pulmonary stenosis. In some cases, the surgeon may do a valvectomy. That’s when the surgeon removes the old pulmonary valve and replaces it with a new valve. The new valve may be artificial or from a cadaver donor. The surgeon might also use a special patch to help rebuild the area around the valve.

Another choice is valvotomy. The surgeon makes an incision in the pulmonary valve to make the opening to the pulmonary artery larger. It helps the blood flow more easily into the pulmonary artery.

Why might I need congenital pulmonary stenosis surgery?

This surgery helps ease the symptoms of congenital pulmonary stenosis. Most people who have this health problem will not need surgery. Mild cases may not cause any symptoms. People with more moderate pulmonary stenosis may have symptoms such as fatigue and shortness of breath with exercise.

You might not have any symptoms at first. But you may develop them later in childhood or adulthood. People with severe symptoms often need treatment. It is sometimes advised for pregnant people, especially if the stenosis is more severe.

Your healthcare provider may also advise surgery if you have other anatomy problems along with the stenosis. You might need it if you have an underdeveloped area around the valve or have severe regurgitation of the valve. Another reason may be if you have narrowing (stenosis) that happens before or after the valve. You may also need surgery if you need other heart repairs.

What are the risks for congenital pulmonary stenosis surgery?

Most people do well with congenital pulmonary stenosis surgery. But complications sometimes happen. Risk factors may vary based on your overall health, the anatomy of the heart problems present, or other health problems. Ask your healthcare provider about your specific risks. Possible risks include:

  • Excess bleeding

  • Infection

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

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

  • Heart block. This may need a pacemaker.

  • Complications from anesthesia

  • Pulmonary valve insufficiency

You also may need follow-up surgeries or other procedures later on.

How do I get ready for a congenital pulmonary stenosis surgery?

Ask your healthcare provider about how to get ready for the surgery. Follow all instructions you are given about eating or drinking before the surgery. You may also need to stop taking certain medicines beforehand.

The healthcare provider may want some extra tests before the surgery. These might include:

  • Chest X-ray

  • Electrocardiogram, to check the heart rhythm

  • Blood tests, to check general health

  • Echocardiogram, to look at heart anatomy and blood flow through the heart

  • CT scan or MRI, for more details about your heart

  • Heart catheterization, to better look at the coronary blood vessels

Before the surgery, the hair around the area of operation may be clipped. You may also be given medicine to help you relax.

What happens during congenital pulmonary stenosis surgery?

Talk with your healthcare provider about what to expect. The details of your surgery will vary based on the kind of repair you need. During the repair, the surgery team will carefully watch your vital signs. In general:

  • You will be given anesthesia before the surgery starts. You will sleep deeply and painlessly during the operation. You won’t remember it afterward. The repair will take several hours.

  • The surgeon will make a cut (incision) down the middle of your chest. To reach the heart, the surgeon will separate the breastbone.

  • You will be attached to a heart-lung machine. This machine will act as your heart and lungs during the procedure.

  • If doing a valvectomy, the surgeon will remove the old pulmonary valve. A donor valve or a prosthetic valve will be attached between the right ventricle and the pulmonary artery.

  • If needed, the surgeon might use a special patch to reconstruct the area around the valve and the pulmonary artery.

  • If doing a valvotomy, the surgeon will make an incision in the pulmonary valve to open it.

  • The surgeon will do other repairs to the heart if needed.

  • Once all the repairs are done and your heart is working well on its own, the heart-lung machine will be removed.

  • The breastbone will be put back together with wire.

  • The muscle and the skin incisions will be closed and covered with a sterile dressing.

  • One or more tubes may be placed in your chest to help drain blood and fluids. These tubes will be removed in a day or so.

  • A tube may be inserted through your mouth into your stomach to drain stomach fluids. This tube will be removed once you are awake enough to have the breathing tube removed.

What happens after congenital pulmonary stenosis surgery?

Ask your healthcare provider about what to expect. In general, afterward:

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

  • Medical staff will carefully watch your vital signs. These include your heart rate, blood pressure, breathing, and oxygen levels.

  • You will feel some soreness. But you shouldn’t feel severe pain. Pain medicines will be available if you need them.

  • You will probably be able to drink the day after surgery. You can have regular foods as soon as you can tolerate them.

  • You will probably need to stay in the hospital several days.

After you leave the hospital:

  • You will have your stitches or staples removed in a follow-up appointment. Be sure to keep all checkups.

  • You should be able to resume normal activities relatively soon. But you may be a little more tired for a while after the surgery.

  • Ask your healthcare provider if you have any exercise limits. Don't do any strenuous activities or heavy lifting.

  • Call your healthcare provider if you have fever, difficulty breathing, or chest pain. Also call if you have increased redness, swelling or pus draining from the wound.

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

Most of the time, symptoms get better right away after surgery. But you will need lifelong follow-up care from a cardiologist. They will watch for possible complications from the procedure. You may need to take medicine to help prevent blood clots. In most cases, you will only need to take these medicines for a short time. You may also need to take antibiotics before certain medical or dental procedures to prevent an infection of the heart valves.

Next steps

Before you agree to the test or the 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 will you get the results

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

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

Medical Reviewers:

  • Dan Brennan MD
  • Rita Sather RN
  • Tracy C. Garrett RNC-NIC BSN
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