What is surgery for pulmonary atresia without VSD for children?

Pulmonary atresia is a condition where the pulmonary valve didn't form in the heart. The goal of surgery is to improve blood flow through the heart and to the lungs.

The heart has 4 chambers: 2 upper (atria) and 2 lower (ventricles). The right ventricle pumps blood that's low in oxygen through the pulmonary artery to the lungs. There it picks up more oxygen. Between the right ventricle and the pulmonary artery lies the pulmonary valve, one of the heart’s four valves. These valves help the blood flow the correct way through the heart’s chambers and out into the body. Normally the pulmonary valve opens when the heart contracts. Blood can then flow forward into the lungs.

In pulmonary atresia, the pulmonary valve doesn’t form correctly. So there is no connection between the right ventricle and the pulmonary artery. As a result, only a little blood can get to the lungs through a blood vessel (ductus arteriosus) that's only present during fetal life and shortly after birth. Instead of going to the lungs, blood that's low in oxygen travels through a hole in the wall between the right and left atria. That causes the blood going out to the body to be lower in oxygen. This lack of oxygen can lead to a number of symptoms and/or long-term problems.

Often the right ventricle will be small and poorly developed in a child with this condition. The tricuspid valve between the right atrium and right ventricle may not form well in some cases. And sometimes there are also problems with the coronary arteries.

In some cases, an abnormal hole is present between the walls of the ventricles. It’s called a ventricular septal defect (VSD). Children with VSD need special treatment, including surgery. For children who have pulmonary atresia without VSD, treatments vary based on if the right ventricle is working as it should.

There are 3 ways a surgeon may treat this condition:

• Biventricular repair. This is done to keep both the left and right ventricles working.

• Single-ventricle repair. A series of procedures, often 3, is done to keep only the left ventricle working. These procedures may include a Glenn and Fontan palliation.

• One-and-a-half ventricle repair. This hybrid approach keeps the left ventricle working fully. The right ventricle only works enough to pump a small amount of blood.

Why might my child need surgery for pulmonary atresia without VSD?

Experts don't know what causes pulmonary atresia without VSD. It's a severe condition that almost always causes death without surgery. Infants may look blue because of the decreased amount of oxygen reaching the body. They may also breathe quickly and have problems breathing.

In some cases, healthcare providers are able to treat milder forms (pulmonary atresia) without surgery. They may be able to use a long, thin tube (catheter) threaded through a blood vessel to open the valve. Sometimes a cardiologist can expand a tiny coil (stent) in an extra blood vessel. That can keep a steady supply of blood flow to the lungs for weeks or months. But it’s only a short-term (temporary) measure. It isn't an option for every child.

Your child’s provider will carefully study your child’s heart before deciding on the best type of surgery. If possible, they will likely choose to do a biventricular repair. It needs fewer surgeries and often leads to fewer long-term complications. But it may not be possible if the right ventricle is underdeveloped or there are problems with the coronary arteries. Ask your child’s provider about which procedure or surgery makes the most sense for your child.

What are the risks of surgery for pulmonary atresia without 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 may need a pacemaker

• Complications from anesthesia

• Death

Your child’s risks may be higher in the case of a single-ventricle repair. Often children who get either type of treatment need follow-up care. They may need more surgeries or catheterization.

How can I help my child get ready for surgery for pulmonary atresia without VSD?

Ask your child’s provider about how your child should 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 certain medicines beforehand. Have your child follow any directions given for not eating or drinking before the surgery.

Your child’s provider may want some extra tests before the surgery. These might include:

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

• Electrocardiogram (ECG). This is done to check the heart rhythm.

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

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

• Heart catheterization. This is to better look at the coronary blood vessels or measure the pressures in the heart and lungs.

What happens during surgery for pulmonary atresia without VSD for a child?

Talk with your child’s provider about what to expect during the surgery. The repair will take several hours. The details of your child’s surgery will vary based on the kind of repair. Certain types of repairs may call for multiple surgeries. In general:

• A provider will give your child medicine (anesthesia) before the surgery starts. Your child will sleep deeply and painlessly during the procedure. They won’t remember it later.

• During the procedure, a provider will watch your child’s vital signs closely. These include the heart rate, breathing, blood pressure, and oxygen levels.

• 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 connected to a heart-lung machine. This machine will act as your child’s heart and lungs during the procedure.

• If your child is having a 2-ventricle repair, 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 use a cadaver donor valve to replace the valve.

• If your child is having a single-ventricle repair, the surgeon might connect a tube from the aorta to the pulmonary artery. Or they may connect the superior vena cava to the pulmonary arteries.

• The surgeon will do other surgical repairs as needed.

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

• The surgeon will put the breastbone back together with wires.

• The surgeon will close the muscle and the skin. A provider will apply a bandage.

What happens after surgery for pulmonary atresia without 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 disoriented when they wake up.

• Your child’s vital signs will be carefully watched.

• 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 a day or 2 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 1 week.

After your child leaves the hospital:

• A provider will remove your child’s stitches or staples in 7 to 10 days. 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 drainage from the wound, or any severe symptoms.

• Follow all the instructions you're given.

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.

After the surgery, your child will need follow-up care from a heart doctor (cardiologist). This is especially true if your child had a single or one-and-a-half ventricle repair. Many children with pulmonary atresia without 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