Pulmonary atresia (PA) is a rare heart defect. With PA, there is no pulmonary valve in the heart. Blood cannot flow into the pulmonary artery. This is the artery that brings blood to the lungs. Other heart problems, like a small right ventricle, may also be present.
These factors raise the chance of PA in your child:
- Other family members with a congenital heart defect
- Other heart defects
- Certain problems with chromosomes, such as Down Syndrome
You will be asked about your child's symptoms and health history. A physical exam will be done. Your child's doctor may also detect a heart murmur during the exam.
Pictures may be taken of your child's chest. This can be done with:
Talk with the doctor about the best plan for your child. Some defects may be so severe that they are hard to treat. Your child may have:
Medicine will be given to keep a vessel that connects the pulmonary artery and the aorta open. This opening lets some blood continue to reach the lungs, especially when the ventricular septum is healthy. This is a short term treatment.
Sometimes a shunt can be placed between the aorta and pulmonary artery. This is done to help blood flow to the lungs.
Many surgeries may be considered depending on:
- The size of the pulmonary artery and right ventricle
- Other heart abnormalities that your child may have
Open heart surgery aims to:
- Remove the short term shunt
- Close any holes between the chambers of the heart, if they are there
- Make the pulmonary artery larger, if needed
- Place an artificial valve, if needed
- Reconnect veins and arteries for proper blood flow
When the right ventricle is too small to pump blood, other surgeries may be done. These can reroute blood to the lungs.
Your child will need to see a heart specialist regularly. Your child may need to take antibiotics before certain medical or dental procedures. This is to prevent heart infections.
- Reviewer: Kari Kassir, MD
- Review Date: 05/2018 -
- Update Date: 06/29/2018 -