Pulmonary Atresia—Child
Definition
Pulmonary atresia (PA) is a rare heart defect. It is when the heart does not have a pulmonary valve. This prevents blood from flowing into the pulmonary artery. This is the artery that brings blood to the lungs. Other heart problems may also be present.
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Blood Flow Through the HeartCopyright © Nucleus Medical Media, Inc. |
Risk Factors TOP
Things that may raise the risk of this problem are:
- Having other family members with a congenital heart defect
- Having other heart defects
- Certain problems with chromosomes, such as Down Syndrome
Diagnosis TOP
The doctor will ask about your child's symptoms and health history. A physical exam will be done.
Pictures may be taken of your child's chest. This can be done with:
Treatment TOP
The goal of treatment is to repair the heart defect and improve blood flow to the lungs. Some defects may be so severe that they are hard to treat. Choices are:
Medications
Medicine will be given to keep a vessel that connects the pulmonary artery and the aorta open. This opening lets some blood flow to the lungs. This is a short-term treatment.
Surgery
Sometimes a shunt can be placed between the aorta and pulmonary artery. This is done as short-term treatment to help blood flow to the lungs.
The surgery chosen depends on the size of the pulmonary artery and right ventricle. It also depends on whether a child has other heart defects.
Open heart surgery choices may be to:
- Remove the short-term shunt
- Close any holes between the chambers of the heart
- Make the pulmonary artery larger
- Place an artificial valve
- 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.
Lifelong Monitoring TOP
Lifelong monitoring will be needed by a heart specialist.
RESOURCES:
American Heart Association
http://www.heart.org
Healthy Children—American Academy of Pediatrics
http://www.healthychildren.org
CANADIAN RESOURCES:
Canadian Cardiovascular Society
http://www.ccs.ca
Heart and Stroke Foundation of Canada
http://www.heartandstroke.com
REFERENCES:
Monaco M, Williams I. Tetralogy of Fallot: fetal diagnosis to surgical correction. Minerva Pediatr. 2012 Oct;64(5):461-470.
Single ventricle defects. American Heart Association website. Available at:
...(Click grey area to select URL)
Accessed November 5, 2020.
Tetralogy of Fallot in infants and children. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/tetralogy-of-fallot-in-infants-and-children. Accessed November 5, 2020.
Last reviewed September 2020 by EBSCO Medical Review Board
Kari Kassir, MD
Last Updated: 5/11/2021