Total Anomalous Pulmonary Venous Connection—Child

(TAPVC—Child; Total Anomalous Pulmonary Venous Drainage—Child; TAPVD—Child; Total Anomalous Pulmonary Venous Return—Child; TAPVR—Child)
  • Definition

    Total anomalous pulmonary venous connection (TAPVC) 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 through the tricuspid valve. The blood travels to the lungs through the pulmonary valve to pick up fresh oxygen. Next, the blood returns to the left atrium, goes into the left ventricle, and goes out to the rest of the body.
    Heart Chambers and Valves
    heart anatomy
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    Blood Flow Through the Heart
    IMAGE
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    With TAPVC, the pulmonary veins that return oxygenated blood from the lungs connect to the right side of the heart, instead of the left atrium. This leads to the mixing of oxygenated and de-oxygenated blood. The body tissue does not receive as much oxygen as it is supposed to. TAPVC can be mild to severe. There can be a range of connection problems. Other heart problems may be present, as well.
  • Causes

    TAPVC is a congenital defect. This means that the heart forms incorrectly when the baby is in the womb. It is not known exactly why the heart develops this way in some babies.
  • Risk Factors

    More research is needed to confirm causes and risk factors for TAPVC. Risk factors thought to be related to this condition include:
    • Family history of heart defects
    • Having other heart defects
    • Environmental exposures
  • Symptoms

    Symptoms may include:
    • Blue or pale grayish skin color
    • Trouble breathing (shortness of breath)
    • Frequent respiratory tract infections
    • Slow growth
    The doctor may also detect an irregular heart rate during the exam.
    These symptoms may be due to other conditions. If your child has any of these, talk to the doctor right away.
  • Diagnosis

    The doctor will ask about your child’s symptoms and medical history. A physical exam will be done. Tests may include:
    • Blood tests and oxygen saturation levels
    • Echocardiogram —an imaging test that uses sound waves to look at the size, shape, and motion of the heart
    • Chest x-ray —an imaging test that uses low amounts of radiation to create an image of the chest
    • Electrocardiogram —a test that measures the electrical activity of the heart
    • Cardiac catheterization —a test that uses a catheter (tube) and x-ray machine to assess the heart and its blood supply
  • Treatment

    Talk with the doctor about the best treatment plan for your child. Treatment options include:
    Surgery
    Surgery is needed to correct the defect. It can range from a simple repair to a complex repair. If blood flow is obstructed, the doctor will need to do emergency surgery. The goal of surgery is to reconnect the pulmonary veins to the left atrium.
    Lifelong Monitoring
    Your child will have regular exams from a heart specialist. Your child may also need antibiotics prior to medical or dental procedures. This is to prevent an infection in the heart.
  • Prevention

    Preventing fetal heart defects may not always be possible, but you can reduce your risk by:
    • Practicing good prenatal care:
      • Visit the doctor regularly to monitor your health and the health of the baby. (Prenatal tests may detect a heart defect in a growing fetus.)
      • Make sure you:
        • Have a healthy lifestyle
        • Eat nutritious food and take prenatal vitamins
        • Do not drink alcohol, smoke , or use drugs during pregnancy
    • Practicing good hygiene and staying away from people who are sick
    • Avoiding exposure to environmental toxins
  • RESOURCES

    American Family Physician http://www.aafp.org/

    American Heart Association http://www.americanheart.org/

    CANADIAN RESOURCES

    Canadian Cardiovascular Society http://www.ccs.ca/

    Heart and Stroke Foundation of Canada http://ww2.heartandstroke.ca/splash/

    References

    American Heart Association. Total anomalous pulmonary venous connection (TAPVC). American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Total-Anomalous-Pulmonary-Venous-Connection-TAPVC%5FUCM%5F307039%5FArticle.jsp . Accessed July 8, 2010.

    DynaMed Editorial Team. Total anomalous pulmonary venous connection. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated February 11, 2010. Accessed July 22, 2010.

    Johns Hopkins University, Cove Point Foundation. Total anomalous pulmonary venous return. Johns Hopkins University, Cove Point Foundation website. Available at: http://www.pted.org/?id=tapvr1 . Accessed July 8, 2010.

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