Surgery for Total Anomalous Pulmonary Venous Return for Children

What is TAPVR surgery for children?

Total anomalous pulmonary venous return (TAPVR) is a condition in which the blood vessels from the lungs take an abnormal path back to the heart. TAPVR surgery is open heart surgery done to fix this problem.

The heart has 4 chambers: a right and left atrium and a right and left ventricle. Normally, blood that is low in oxygen comes in from the body to the right atrium. From there, it travels to the right ventricle and then out to the lungs. In the lungs, it picks up more oxygen and releases carbon dioxide. From the lungs, blood vessels (pulmonary veins) connect to the left atrium. From there, the blood travels to the left ventricle and out to the body, where the vital organs use the oxygen.

In TAPVR, there is a problem in the usual connection between the blood vessels (pulmonary veins) coming from the lungs to the left atrium. This problem can take a number of different forms. In some cases, the blood vessels coming from the lungs connect with the right atrium instead of the left. Or the blood vessels from the lungs might drain into another blood vessel that eventually drains into the right atrium instead of the left. In all cases, the blood that goes out to the body has less oxygen than normal. That can cause major symptoms. And too much blood flows into the lungs. In some forms of TAPVR, the blood vessels of the lungs can become compressed and blocked by surrounding structures or because of the unusual course they take. This can limit blood flow. Symptoms can become worse and can start very early in life, even shortly after work.

The type of repair recommended for TAPVR depends on the specific problems with the anatomy of the blood vessels. Typically, the surgeon will form a connection between the pulmonary veins coming from the lungs and the left atrium. He or she will also close off the blood vessel into which it was previously draining. If the blood vessels are directly draining into the right atrium instead of the left, the surgeon may rebuild the atria. The blood vessels will then drain to the left atrium.

Why might my child need TAPVR surgery?

This surgery is needed to fix the symptoms of TAPVR, which can be severe. Because of the decreased oxygen in the blood, your baby might turn a bluish color and have problems breathing. In some infants, these symptoms develop right away. If the TAPVR is less severe, symptoms might not develop until a little later. Healthcare providers recommend surgery for all cases of TAPVR. Even if your child’s symptoms are not severe right away. Untreated TAPVR often results in death.

Surgery is eventually needed in all cases of TAPVR. Some infants need treatment before surgery. These treatments help stabilize the infant so that the risks of surgery are lower. These might include:

  • Oxygen therapy
  • Mechanical ventilation
  • Extracorporeal membrane oxygenation (ECMO), which takes over the job of the heart and lungs.

In most cases, the cause of TAPVR is unknown. Sometimes TAPVR occurs with other heart defects.

What are the risks of TAPVR surgery for a child?

Many infants do well with TAPVR surgery. But complications can develop. Some infants do not survive the surgery. Risk factors may vary based on the child’s overall health and the anatomy of the heart and vessel problems present. Ask your child’s healthcare provider about the specific risks for your child. Possible risks include:

  • Excess bleeding
  • Infection
  • Blood clot, which can lead to stroke or other problems
  • Abnormal heart rhythms, which can cause death in rare cases
  • Heart block, which can make a pacemaker necessary
  • Complications from anesthesia
  • Death

Your child may also need follow-up surgeries or catheterization procedures later on. That is most likely to happen in the first year after surgery.

How do I help my child get ready for TAPVR surgery?

Ask your child’s healthcare provider how to help your child get ready for the surgery. Your child may need supportive care with extra medicine, oxygen, and sometimes ventilator care. Your child may need to stop taking medicine beforehand. Your child should not eat or drink anything after midnight before the day of the surgery.

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

  • Chest X-ray
  • Electrocardiogram, to look at heart rhythm
  • Blood tests, to check general health
  • Echocardiogram, to look at heart anatomy and blood flow through the heart
  • Heart catheterization, to better look at the coronary blood vessels
  • CT scan or MRI, if the provider needs more information about the heart or blood vessels

What happens during TAPVR surgery for a child?

Talk with your child’s healthcare provider about what to expect. The details of your child’s surgery will vary based on the kind of repair being done. In general:

  • A healthcare provider will give your child anesthesia before the surgery starts. Your child will sleep deeply and painlessly during the operation. He or she won’t remember it afterward.
  • The repair will take several hours.
  • Your child’s vital signs will be closely watched during the surgery.
  • The surgeon makes a cut (incision) down the middle of your child’s chest. He or she will separate the breastbone to reach the heart.
  • Your child will be attached to a heart-lung machine. This machine acts as your child’s heart and lungs during the procedure.
  • In some cases, the surgeon will create a connection between the vessel coming from the lung and the left atrium. The surgeon may close off the vessel where the vessel from the lung was previously joined.
  • In other cases, the surgeon will rebuild the wall between the left and right atrium. That way, the blood from the lungs flows directly into the left atrium.
  • The surgeon will make other repairs to the heart as needed.
  • Once all repairs have been done, the heart-lung machine will be removed.
  • The breastbone will be put back together with wires.
  • The surgeon will close the muscle and the skin. A bandage will be applied.

What happens after TAPVR surgery for a child?

Ask your child’s healthcare provider about what to expect. In general, after your child’s surgery for TAPVR:

  • Your child may be groggy and disoriented when he or she wakes up.
  • Your child’s vital signs, such as heart rate, breathing, blood pressure, and oxygen levels, will be carefully watched.
  • Your child will feel some soreness. But he or she shouldn’t feel severe pain. Pain medicine is available if needed.
  • Your child will still need extra support for a while after the surgery.
  • Your child will likely need to stay in the hospital for several weeks.

After your child leaves the hospital:

  • Be sure your child keeps all follow-up appointments.
  • Some children may need to temporarily take medicine to prevent blood clots.
  • Talk with your child’s healthcare provider about what sort of activity and diet will be appropriate for your child.
  • Call your child’s healthcare provider if your child has fever, increased draining from the wound, or any severe symptoms.
  • Follow all the instructions you are given for medicine, exercise, diet, and wound care.

Your child may need to take antibiotics before certain medical or dental procedures. They will help prevent an infection of the heart valves. Your child will also need careful follow-up with a cardiologist after the surgery. Many children with surgery for TAPVR do quite well. But your child will always have to see a cardiologist to watch for problems. Follow-up surgery or procedures may be needed.

Next steps

Before you agree to the test or the procedure for your child make sure you know:

  • The name of the test or procedure
  • The reason your child is having the test or procedure
  • What results to expect and what they mean
  • The risks and benefits of the test or procedure
  • When and where your child is to have the test or procedure
  • Who will do the procedure and what that person’s qualifications are
  • What would happen if your child 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 your child has problems
  • How much will you have to pay for the test or procedure

Pediatric Heart Care at Johns Hopkins

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