Definition
Definition
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.
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Blood Flow Through the HeartCopyright © Nucleus Medical Media, Inc.
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Causes
Causes
Pulmonary atresia is present at birth. It is not known exactly why the heart does not develop normally.
Risk Factors
Risk Factors
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
Symptoms
Symptoms may include:
- Blue skin color
- Rapid or difficult breathing
- Fatigue
- Irritability
Diagnosis
Diagnosis
Your doctor will ask 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:
Treatment
Treatment
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
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.
Surgery
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.
Lifelong Monitoring
Your child will need to see a heart specialist regularly. Your child may need to take antibiotics prior to certain medical or dental procedures. This is to prevent heart infections.
Prevention
Prevention
There are no current guidelines to prevent pulmonary atresia because the cause is unknown.. Getting appropriate prenatal care is always important.
RESOURCES:
Healthy Children—American Academy of Pediatrics http://www.healthychildren.org
CANADIAN RESOURCES:
Heart and Stroke Foundation of Canada http://www.heartandstroke.ca
References:
http://www.ebscohost.com/dynamed
Updated July 15, 2013. Accessed July 16, 2013.
Pulmonary atresia. Johns Hopkins Children’s Center website. Available at:
http://www.hopkinschildrens.org/Pulmonary-Atresia.aspx
Updated May 16, 2011. Accessed July 16, 2013.
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 January 8, 2013. Accessed July 16, 2013.
Last Updated: 5/11/2013