Pulmonary Atresia Child
Overview
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.
Heart Chambers and Valves |
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Blood Flow Through the Heart |
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SymptomsandDiagnosis
Treatments
Treatment
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
Lifelong monitoring will be needed by a heart specialist.
This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.
Edits to original content made by Denver Health.
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All rights reserved.
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: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects%5FUCM%5F307037%5FArticle.jsp. 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.