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Hypoplastic Heart Disease

Hypoplastic left heart syndrome is a congenital heart disease in newborn babies where the left side of the heart does not form property and is unable to pump blood. Some newborns may present other abnormalities of the heart in addition to this syndrome. The advancement of open heart surgery in newborn children has made it possible to treat the defect with a surgical procedure.

  1. Identification

    • Hypoplastic left heart syndrome is a congenital heart disease that is present at birth. The left side of the heart does not develop fully and is unable to pump blood effectively. This causes the right side of the heart to overcompensate as it must pump all the blood. According to WebMD, this type of heart disease is one of the most rare and complex of congenital heart conditions.

    Symptoms

    • An infant that is born with the hypoplastic heart condition is gravely ill at birth. Symptoms include a gray-blue skin tone, difficulty breathing, and drowsy or not active. The infant will not feed well and will have cold feet and hands. If connectors between the left and right heart are closed, the infant will go into shock. Symptoms that the baby is in shock are the skin turns cold and clammy, the pulse is weak and rapid, and pupils dilate with dull eyes that stare.

    Cause

    • The cause of the hypoplastic left heart syndrome is unknown; however it occurs during fetal growth of the baby. The risk of having another child with the disease increases with each pregnancy. This suggests the cause may be related to genetics. The development of the heart may be hindered due to a lack of fetal blood flow during the heart development stages.

    Diagnosis

    • An ultrasound during pregnancy will show signs of the hypoplastic left heart syndrome as it displays a heart that is not formed fully. Once the baby is born, an echocardiogram will diagnose the heart problem as the image shows a smaller left side heart. A chest x-ray may be completed and will show the shape and size of the heart chambers; however the image is not clear enough to diagnose the type of heart problem.

    Treatment

    • The only treatment for the hypoplastic left heart syndrome is heart surgery. A transplant will replace the heart with a healthy donor heart from an infant. A heart transplant involves getting on a waiting list as there is a shortage of organs for newborns. A heart procedure called the Norwood operation is another option available instead of a transplant. This surgery is completed in three stages with the final stage done between 2-4 years of age. The surgery involves reworking the blood flow and changing the structure of the heart to improve function. According to the Mayo Clinic, the survival rate is 95 percent if the child makes it through the second surgery.

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