What is surgery for pulmonary atresia with VSD for children?

Pulmonary atresia is a condition where the heart is missing a pulmonary valve. This surgery improves blood flow through the heart and out to the lungs.

The heart has 4 chambers: 2 upper (atria) and 2 lower (ventricles). The heart valves help the blood flow in the correct way through the heart’s 4 chambers and out into the body. Normally, the pulmonary valve opens when the heart contracts. This allows the right ventricle to pump blood that's low in oxygen forward into the lungs. There it picks up more oxygen.

In pulmonary atresia, the pulmonary valve doesn’t form right. So there is no link between the right ventricle and the pulmonary artery. As a result, only a little blood can get to the lungs through a blood vessel normally present during fetal life called the ductus arteriosus. Blood not flowing through the ductus arteriosus goes through a hole in the wall between the right and left atrium. That causes blood that's low in oxygen to go into the body, instead of the lungs. The resulting lack of oxygen can lead to many symptoms when left untreated.

Some children with pulmonary atresia may also have a hole between the walls of the ventricles (ventricular septal defect or VSD).

The type of surgery used to fix pulmonary atresia with VSD depends on other problems that are present. Sometimes surgeons can fix the problem with only 1 surgery. In these cases, the surgeon makes an opening between the right ventricle and pulmonary artery. They then place a tube (conduit) there and close the hole. In other cases, the surgeon might first fix the opening between the right ventricle and pulmonary arteries. The hole between the ventricles may be closed in a later surgery.

Why might my child need surgery for pulmonary atresia with VSD?

No one knows what causes pulmonary atresia with VSD. The severity can vary based on the part that is affected. But the condition always needs surgery. Babies may look blue because of less oxygen reaching the body. They may also breathe quickly and have problems breathing.

Your child’s healthcare provider will carefully study your child’s heart before deciding on the best type of surgery. If possible, they'll likely choose to do the repair in a single surgery. Ask your child’s provider which procedure or surgery makes the most sense for your child.

What are the risks of surgery for pulmonary atresia with VSD 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 rhythm

• Heart block, which can make a pacemaker necessary

• Complications from anesthesia

Often a child will need follow-up surgeries or catheterization procedures later on. Typically, this is the case no matter what kind of repair the healthcare provider had done in the past.

How can I help my child get ready for surgery for pulmonary atresia with VSD?

Ask your child’s provider about how to help your child get ready for the surgery. Your child may need supportive care with extra medicine and oxygen. Some children may need a machine (ventilator) to help them breathe before the surgery. Your child may need to stop taking certain medicines beforehand. Follow any directions your child is given for not eating or drinking before the surgery.

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

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

• Electrocardiogram (ECG). This checks the heart rhythm.

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

• Echocardiogram. This shows the heart anatomy and checks blood flow through the heart.

• Heart catheterization. This shows the blood vessels of the heart or lungs. It can also measure the pressures in the heart and lungs.

• CT scan or MRI. These imaging tests show the lung blood vessels.

What happens during surgery for pulmonary atresia with VSD for a child?

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

• A healthcare provider will give your child anesthesia before the surgery starts. Your child will sleep deeply and not feel pain during surgery. They won’t remember it later.

• The repair will take a few hours.

• A provider will closely watch your child’s vital signs during the procedure.

• The surgeon will make 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 will act as your child’s heart and lungs during the procedure.

• The surgeon may use a patch to enlarge the outflow part of the right ventricle and the valve area. In some cases, the surgeon may replace the valve with a cadaver donor valve.

• In some cases, the surgeon will also fix the hole between the left and right ventricles. Or the surgeon might do this in a later procedure.

• The surgeon will do other surgical repairs as needed.

• The heart-lung machine will be removed after all the repairs have been done and the heart is beating again.

• The breastbone will be put back together with wires.

• The surgeon will close the muscle and the skin incisions. Then a bandage will be put on the skin.

What happens after surgery for pulmonary atresia with VSD for a child?

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

• Your child may be groggy and confused when they wake up.

• Your child’s vital signs will be closely watched. This includes heart rate, breathing, blood pressure, and oxygen levels.

• Your child will feel some soreness. But they shouldn’t feel severe pain. Pain medicines are available if needed.

• Your child may be able to drink the day after surgery. Your child can have regular foods as soon as they can handle them.

• Your child will likely have to stay in the hospital for at least a week.

After your child leaves the hospital:

• Keep all follow-up appointments.

• Talk with your child’s provider about what activities and diet are right 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 your child's provider gives to you.

Your child might need to take antibiotics before certain medical or dental procedures. That will help prevent an infection of the heart valves. Some children may also need to take medicine to prevent blood clots.

Your child will need follow-up care from a cardiologist after the surgery. Many children with pulmonary atresia with VSD do quite well. But follow-up surgery or other 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 didn't have the test or procedure

• Any alternative tests or procedures to think about

• When and how you'll get the results

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

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