Pulmonary atresia is a condition in which 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, where 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 is able to fix the pulmonary valve with two working ventricles (biventricular repair). In other cases, the surgeon fixes this health problem using only one pumping ventricle in a series of operations. Those procedures may include a Glenn and Fontan palliation (single-ventricle repair). Other times, the surgeon may do an operation that provides two functioning ventricles. But the right ventricle only pumps some of the blood it would (one-and-a-half ventricle repair).
No one knows 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 pulmonary atresia (milder forms) 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 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.
Many children do well with this surgery. But complications can develop. Risk factors may vary based on your child’s overall health and the condition of his or her heart. Ask your child’s healthcare provider about the specific risks for your child. Possible risks include:
- Excess bleeding
- 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. He or she should not eat or drink anything after midnight before the day of the surgery.
Your child’s healthcare provider may want some extra tests before the surgery. These might include:
- Chest X-ray
- Electrocardiogram, to look at heart rhythm
- Blood tests, to check general health
- Echocardiogram, to look at heart anatomy and blood flow through the heart
- Heart catheterization, 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 operations. In general:
- A healthcare provider will give your child anesthesia before the surgery starts. Your child will sleep deeply and painlessly during the operation. He or she won’t remember it afterward. 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 two-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, such as heart rate, breathing, blood pressure, and oxygen levels, will be carefully watched.
- 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 two after surgery. Your child can have regular foods as soon as he or she can handle them.
- Your child will probably 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. Be sure to 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 healthcare 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.
Tips to help you get the most from a visit to your child’s healthcare provider:
- Know the reason for the visit and what you want to happen.
- Before your visit, write down questions you want answered.
- At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
- Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
- Ask if your child’s condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if your child does not take the medicine or have the test or procedure.
- If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
- Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.
October 26, 2017
Pulmonary atresia with intact ventricular septum, Up To Date
Ayden, Scott, MD,Bass, Pat F., III, MD, MPH