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
heart anatomy

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Blood Flow Through the Heart

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Causes

PA is present at birth. It is not known exactly why the heart does not form as it should.

Risk Factors

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

Symptoms

Problems may be:

  • Blue skin color
  • Problems breathing
  • Lack of energy
  • Irritability

Diagnosis

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

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.

Prevention

There are no guidelines to prevent this health problem.

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_UCM_307037_Article.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.

Last reviewed September 2020 by EBSCO Medical Review Board Kari Kassir, MD  Last Updated: 11/5/2020