Pulmonary atresia is a rare heart defect. In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle. Next, the blood travels to the lungs through the pulmonary valve. There, it picks up fresh oxygen. The blood then returns to the left atrium and goes into the left ventricle. The blood moves out to the rest of the body.
With this defect, 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.
Heart Chambers and Valves
Blood Flow Through the Heart
Pulmonary atresia is present at birth. It is not known exactly why the heart does not develop normally.
These factors increase the chance of pulmonary atresia in your child:
- Family history of congenital heart defect
- Other heart defects
- Certain chromosomal disorders, such as Down Syndrome
Symptoms may include:
- Blue skin color
- Rapid or difficult breathing
You will be asked about your child's symptoms and medical history. A physical exam will be done. Your child's doctor may also detect a heart murmur during the exam.
Images may be taken of your child's chest. This can be done with:
Talk with the doctor about the best treatment plan for your child. Some defects may be so severe that they are difficult to treat. Treatment options include:
Medications will be given to keep a vessel that connects the pulmonary artery and the aorta open. This opening allows some blood to continue to reach the lungs, especially when the ventricular septum is intact. This is a temporary treatment.
Sometimes a shunt can be placed between the aorta and pulmonary artery. This is done to improve blood flow to the lungs.
Several 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 temporary shunt
- Close any holes between the chambers of the heart, if they are present
- Enlarge the pulmonary artery, if needed
- Insert an artificial valve, if needed
- Reconnect veins and arteries for proper circulation
When the right ventricle is too small to pump blood effectively, other surgeries may be done. These can reroute blood to the lungs.
There are no current guidelines to prevent pulmonary atresia because the cause is unknown.. Getting appropriate prenatal care is always important.
American Heart Association
Healthy Children—American Academy of Pediatrics
Canadian Cardiovascular Society
Heart and Stroke Foundation of Canada
Single ventricle defects. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects_UCM_307037_Article.jsp. Updated October 21, 2015. Accessed June 6, 2016.
Tetralogy of Fallot in infants and children. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T115349/Tetralogy-of-Fallot-in-infants-and-children. Updated January 25, 2016. Accessed June 3, 2016.
Last reviewed June 2016 by Kari Kassir, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.