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What is the long-term outlook for a child born with pulmonary atresia (PA)?

Although new innovations have improved survival rates for children born with pulmonary atresia, continuing problems and even death still can be high with all types of pulmonary atresia. Because of the wide variety of types of pulmonary atresia, the long-term outlook can vary widely as well.

Even though a baby with pulmonary atresia lacks an outlet to conduct blood to the lungs, development is usually normal before birth. This is because fetal circulation conducts oxygen-rich blood through a hole in the heart wall between the heart’s upper chambers and also through the fetal blood vessel called the ductus arteriosus. Immediately after birth, this status changes and the baby will not do well. Within a few hours of birth, the baby’s condition may deteriorate because of decreased blood flow to the lungs. He or she may develop an intense bluish discoloration of the skin called cyanosis, which indicates that the blood is not being adequately oxygenated.

If untreated, children with this form of heart disease will die from its complications. This is why children born with pulmonary atresia must see a cardiologist regularly throughout their lives to have their hearts checked. They also must consult their cardiologist before undertaking physical activities and exercise regimens.

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Important: This content reflects information from various individuals and organizations and may offer alternative or opposing points of view. It should not be used for medical advice, diagnosis or treatment. As always, you should consult with your healthcare provider about your specific health needs.