Surgery for Total Anomalous Pulmonary Venous Return for Children

What is TAPVR surgery for children?

Total anomalous pulmonary venous return (TAPVR) is a condition where 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. There the vital organs use the oxygen.

In TAPVR, there is a problem in the normal connection between the blood vessels coming from the lungs to the left atrium. This problem can take many 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 over time 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 squeezed (compressed) and blocked by nearby structures or because of the abnormal course they take. This can limit blood flow. Symptoms can become worse and can start very early in life, even shortly after birth.

There are typically 4 types of TAPVR:

• Supracardiac. The pulmonary veins from both lungs typically drain to a chamber just behind the left atrium

• Cardiac. The pulmonary veins from both lungs typically drain to a different part of the heart, rather than the left atrium.

• Infracardiac. The pulmonary veins from both lungs typically drain into a common vein. Over time this connects to one of the major veins of the body. This vein is below the muscle that separates the chest and belly (diaphragm).

• Mixed. The pulmonary veins from both lungs typically drain in a combination of the above types.

The type of repair advised 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. They will also close off the blood vessel into which it was draining before. 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 occur right away. If the TAPVR is less severe, symptoms might not occur until a little later. Healthcare providers advise surgery for all cases of TAPVR, even if your child’s symptoms are not severe right away. Untreated TAPVR often leads to death.

Over time, surgery is 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

• A machine (ventilator) to help with breathing

• Prostaglandin therapy to prevent closure of a patent ductus arteriosus

• Medicines to help the heart pump (inotropics)

• 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. In severe forms of TAPVR, some babies may need surgery shortly after birth.

What are the risks of TAPVR surgery for a child?

All procedures have risks. Some possible risks of this procedure 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 or oxygen. In some cases, a child may need a machine to help with breathing (ventilator). Your child may need to stop taking medicine beforehand. Follow any directions your child is given for not eating or drinking before the surgery.

Your child may need some extra tests before the surgery. These might include:

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

• Electrocardiogram. This checks the heart rhythm.

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

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

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

• CT or MRI scans. These imaging tests provide more details 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. They 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. They 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 they wake 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 they 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 provider about what sort of activity and diet will be OK for your child.

• Call your child’s 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 you will get the results

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

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