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). Normally, the right ventricle pumps blood that is low in oxygen through the pulmonary valve to the lungs. There it picks up more oxygen. The heart valves help the blood flow in the correct way through the heart’s 4 chambers and out into the body. 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 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 an extra blood vessel called the ductus arteriosus. Instead of going to the lungs, blood that is low in oxygen goes through a hole in the wall between the right and left atrium. That causes the blood going out to the body to be very low in oxygen. This lack of oxygen can lead to many symptoms.
Some children with pulmonary atresia may also have a hole between the walls of the ventricles. It’s called a ventricular septal defect (VSD).
The type of surgery used to fix pulmonary atresia with VSD varies. It 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. He or she then places a tube (conduit) there and closes 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.
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, he or she will 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.
All procedures have risks. Some possible risks of this procedure include:
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 the child will need follow-up surgeries or catheterization procedures later on. It doesn’t matter what kind of repair the healthcare provider does at first.
Ask your child’s healthcare 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 with breathing. 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. 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 done to better look at the heart blood vessels or lung blood vessels. Or to measure the pressures in the heart and lungs.
CT or MRI scans. These imaging tests help to better see the lung blood vessels.
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 painlessly during the surgery. He or she won’t remember it later.
The repair will take a few hours.
Your child’s vital signs will be closely watched during the procedure.
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 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.
Once all the repairs have been done and the heart is beating again, the heart-lung machine will be removed.
The breastbone will be put back together with wires.
The muscle and the skin incisions will be closed. A bandage will be applied.
Ask your child’s healthcare provider about what to expect. In general, after your child’s surgery:
Your child may be groggy and confused when he or she wakes 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 he or she 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 he or she can handle them.
Your child will likely have to stay in the hospital for a week or more.
After your child leaves the hospital:
Keep all follow-up appointments.
Talk with your child’s healthcare provider about what sort of activity 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 you are 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.
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.
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