Pulmonary atresia is a condition where the heart lacks 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 right ventricle pumps blood that is 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 4 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 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 travels 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 a number of symptoms.
Often the right ventricle might be small and poorly developed in this condition. The tricuspid valve between the right atrium and right ventricle may not form well in some cases. Sometimes there are abnormalities in the coronary arteries as well.
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 other problems that are present. Sometimes the surgeon can fix the pulmonary valve with 2 working ventricles (biventricular repair). In other cases, the surgeon fixes this health problem using only 1 pumping ventricle in a series of surgeries. Those procedures may include a Glenn and Fontan palliation (single-ventricle repair). Other times, the surgeon may do a surgery that provides 2 functioning ventricles. But the right ventricle only pumps some of the blood it normally would (one-and-a-half ventricle repair).
Experts don't know what causes pulmonary atresia without VSD. It is a severe condition. It is almost always fatal 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 is not an option for every child.
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 a biventricular repair. It needs fewer surgeries. It also often leads to fewer long-term complications. But it may not be possible if the right ventricle is underdeveloped. Or if there are problems with the coronary arteries. Ask your child’s healthcare provider about 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
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.
Ask your child’s healthcare 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 healthcare 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. 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.
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. Certain types of repairs may call for multiple surgeries. In general:
A healthcare provider will give your child anesthesia before the surgery starts. Your child will sleep deeply and painlessly during the procedure. He or she won’t remember it later. The repair will take several hours.
During the procedure, your child’s vital signs will be closely watched.
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 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.
In a single-ventricle repair, the surgeon might connect a tube from the aorta to the pulmonary artery. Or he or she may connect the superior vena cava to the pulmonary arteries.
The surgeon will do other surgical repairs as needed.
Once all repairs have been done and the heart is again beating, 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.
Ask your child’s healthcare provider about what to expect. In general, after your child’s surgery:
Your child may be groggy and disoriented when he or she wakes up.
Your child’s vital signs will be carefully watched. These include the 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 a day or 2 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:
Your child’s stitches or staples will be removed in 7 to 10 days. 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. That is especially true in the case of a single (or one-and-a-half) ventricle-style repair. Many children with pulmonary atresia without 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