KidsMD Health Topics

Pulmonary Atresia in Children

  • One thing that sets Boston Children's apart is our experience performing catheter-based procedures. This avoids the need to do open heart surgery.

    --Catherine Allan, MD, assistant in Cardiology

    If your infant or child has been diagnosed with pulmonary atresia (PA), an understanding of the condition will help you to cope with this rare and complex congenital (present at birth) heart defect. While very serious, pulmonary atresia is treatable surgically at Boston Children's Hospital, and recent surgical advances have greatly improved your child’s outlook for success.

    pulmonary atresiaIn pulmonary atresia, the pulmonary valve—which is normally located between the right ventricle and the pulmonary artery—is abnormal and doesn’t open. This means that oxygen-poor (blue) blood can’t flow forward from the right ventricle to the lungs to get oxygenated. The failure of the pulmonary valve to develop can also result in a small (or missing) right ventricle that can’t adequately pump blood to the lungs.

    • Pulmonary atresia isn’t just a single defect, but, rather, a cluster of associated defects.
    • PA affects one out of every 10,000 babies.
    • Most babies with PA show symptoms on the day they’re born.
    • Surgery or interventional catheterization is necessary to improve permanent blood flow to the baby’s lungs so that enough oxygen is added to his bloodstream to meet his body’s needs.
    • A series of three staged operations may be performed to re-route blood flow, starting shortly after birth and concluding when a child is a few years old.

    Comparison with normal heart (click images to enlarge)

      

    In a normal heart, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs, where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, then is pumped through the aorta out to the body.

    But in PA, the abnormal development of the pulmonary valve prevents oxygen-poor (blue) blood from passing from the right atrium to the right ventricle and on to the lungs as it should. Medical and surgical interventions are required, since oxygen-poor (blue) blood cannot meet the body’s demands, and this situation cannot support life.

    How Boston Children's Hospital approaches pulmonary atresia

    Our team in Boston Children’s Cardiac Surgery Program understands how distressing a diagnosis of a pulmonary atresia can be for parents. You can have peace of mind knowing that our surgeons treat some of the most complex pediatric heart conditions in the world, with overall success rates approaching 98 percent—among the highest in the nation among large pediatric cardiac centers.

    At Boston Children’s, we provide families with a wealth of information, resources, programs and support—before, during and after your child’s treatment. With our compassionate, family-centered approach to expert treatment and care, you and your child are in the best possible hands.

    Pulmonary atresia: Reviewed by Catherine Allan, MD
    © Boston Children's Hospital, 2010

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