Pulmonary atresia (PA) is a heart defect. It happens when the fetal heart doesn’t form as it should. This can happen during the first 8 weeks of pregnancy.
PA is a problem with the opening of the pulmonary valve. This valve connects the right ventricle and the main pulmonary artery, which carries blood to the lungs. With pulmonary atresia, blood can’t flow to the lungs.
Since blood can’t flow from the right ventricle to the pulmonary artery, it takes other routes. The normal opening (foramen ovale) between the right and left upper chambers (atria) of the fetal heart normally closes soon after birth. In a newborn with PA, it stays open to let oxygen-poor (blue) blood flow from the right atrium to the left atrium to mix with the oxygen-rich (red) blood returning from the lungs. This mixture of oxygen-poor and oxygen-rich blood is then pumped by the left ventricle out to the body.In addition, a newborn with PA must rely on a temporary connection (called ductus arteriosus) between the aorta and the pulmonary artery. The ductus arteriosus is also part of normal fetal blood flow. But it also closes soon after birth. While it is open, some of the mixed blood pumped by the left ventricle flows to the lungs via the patent ductus (PDA) and picks up oxygen. When PA is present, and the ductus arteriosus closes, no blood reaches the lungs to get oxygen.
Because the blood doesn't get enough oxygen, every cell in the newborn's body gets less oxygen. A newborn with pulmonary atresia can’t live long without treatment.In some cases, the tricuspid valve or right ventricle may be underdeveloped with significant enlargement of the muscle.
Symptoms may happen shortly after birth or later as the ductus arteriosus closes. The most obvious symptom is a bluish color of the skin (cyanosis) in a newborn.
These are other common symptoms:
The symptoms of PA may look like other health conditions or heart problems. Make sure your child sees his or her healthcare provider for a diagnosis.
PA may be found during a routine ultrasound during pregnancy.
At birth, the healthcare providers and nurses will examine your baby. They will listen to your baby's heart and lungs with a stethoscope and note any signs of a heart defect.
Testing for heart defects varies by the child's age, condition, and other things. Some tests that may be done include:
Your baby will be treated by a pediatric cardiologist. This is a doctor with special training to treat heart problems in children. Your baby will most likely be in the intensive care unit (ICU). At first, your baby may be put on oxygen, and possibly on a ventilator, to help with breathing. Your child may get IV medicines to help his or her heart and lungs work better.
The following treatments allow time for the oxygen levels in your baby's heart to even out while other repairs are planned:
Your child will need surgery to improve blood flow to the lungs. What type of surgery your child has will depend on whether the tricuspid valve or right ventricle can deliver enough blood to the lungs. Usually, the surgeon will do a series of operations to reroute the blood flow to allow oxygen-poor blood to reach the lungs and pick up oxygen. The first surgery may be done shortly after birth. The final surgery is done when the child is a few years old.
Without surgery, the heart can’t pump oxygenated blood to the body and can’t support life. Surgery can be very successful in those with normal sized tricuspid valves and right ventricles.
As your child grows, he or she may need to limit physical activity. Your child may also have developmental delays. He or she may need therapy and special help.
After the surgical repair and time for recovery in the hospital, your baby will be able to go home. Your doctor may recommend pain medicines such as acetaminophen or ibuprofen to keep your baby comfortable. Your child's heart care team will talk about pain control before your child goes home.
The nursing staff will show you how to give any special treatments at home, if needed. Or you may need a home health agency to help. Your child will likely need special formula and supplemental feedings to get enough nutrition.
You may get other instructions from your child's pediatric cardiologist and the hospital staff.
The outlook varies from child to child. Be sure to get regular follow-up care at a center offering pediatric congenital heart care. Your child likely will need more surgery.
After each surgery, your pediatric cardiologist will follow your baby’s recovery. He or she will make changes to medicines, help you with feeding problems, measure oxygen levels, and determine when and if it is time for the next surgery.
Your child may need to be on long-term antibiotics, or take antibiotics before dental or other procedures. Your healthcare provider can tell you if this is needed.
It is important that your child get all recommended vaccines. Talk with your healthcare provider about this.
Throughout your baby's life, pregnancy and non-heart surgeries may be very risky. They require careful evaluation and discussion with a cardiologist.
Your child will need regular follow-up care at a center offering pediatric or adult congenital heart care for the rest of his or her life.
Tips to help you get the most from a visit to your child’s healthcare provider: