Transposition of the Great Arteries (TGA) in Children

  • Parents have peace of mind knowing that Boston Children's uses the most innovative technologies and therapies for their child.

    --David Harrild, MD, assistant in Cardiology

    This discussion refers to a heart defect known as “d-transposition of the great arteries” (“d-TGA,” “transposition,” “TGA”). Another type—“l-transposition” (“l-TGA,” “corrected transposition”)—is similar in some ways to d-TGA, but very different in others, and requires a different treatment program from what’s described here.

    If your infant or child has been diagnosed with transposition of the great arteries (TGA), an understanding of the condition will help you to cope with this rare and complex congenital (present at birth) heart defect. While very serious, TGA is treatable surgically at Children’s Hospital Boston, and the outlook for the vast majority of babies with this cardiac defect is excellent.

    In TGA, the “great” arteries are reversed in their origins from the heart, with the aorta connected to the right ventricle, and the pulmonary artery connected to the left ventricle—exactly the opposite of the normal heart’s anatomy.

    Transposition of the great arteries

    With these arteries reversed, oxygen-poor (blue) blood returns to the right atrium from the body, passes into the right ventricle, then goes into the aorta and back to the body. And oxygen-rich (red) blood returns to the left atrium from the lungs and passes into the left ventricle, which pumps it back to the lungs—the opposite of the way blood normally circulates. The oxygen-poor (blue) blood cannot meet the body’s demands, and this situation cannot support life.

    • TGA is almost always diagnosed in the first hours after birth.
    • Most babies with TGA are born with a small hole between their atria, which allows just enough red blood to get to the body to maintain life for a few hours.
    • TGA is life-threatening and in order to survive, babies with TGA need special therapy urgently soon after birth.
    • The most commonly used initial therapy is balloon atrial septostomy, where a balloon at the end of a catheter (small, flexible tube) is used to enlarge the opening between the atria.
    • Complete open heart repair generally takes place a few days later.
    • If there are no unusual risk factors, more than 98 percent of surgically treated infants survive their infancy.
    • Most children who’ve had TGA surgery recover and grow normally, although they can be at some risk in the future for arrhythmias, leaky valves and other heart issues.
    • While relatively rare (one in every 4,000 babies), TGA is among the most common cyanotic (“blue baby”) heart defects, and it affects boys more than girls.

    How Boston Children's Hospital approaches TGA

    Our team in Boston Children’s Cardiac Surgery Department understands how distressing a diagnosis of TGA 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. We use the following elements to provide the best possible outcomes:

    • accurate diagnosis and assessment: Subtle variations in heart anatomy—such as the arrangement of the arteries that feed the heart—can negatively impact surgical outcomes if not identified ahead of time. We utilize the most advanced techniques available for precisely determining the patient’s cardiac anatomy, with interpretation by highly experienced cardiologists.
       
    • availability of the most sophisticated and effective types of therapy, 24/7: Many babies with TGA require emergency balloon septostomy, and a few need even more aggressive emergency therapy. Our cardiac intensive care unit (CICU), cardiac cath labs, and operating rooms are well-prepared to deal with the urgent needs of transposition patients.
       
    • close and expert medical follow-up before and after surgical repair: If you live in the Boston area, one of Boston Children’s cardiologists will follow you or your child; if you live in another part of the country or the world, a designated Boston Children’s cardiologist will work closely with your local cardiologist. Adult patients with TGA are followed by Boston Children’s cardiologists who have special training for adults with congenital heart problems.
       
    • highly experienced and skilled team of professionals to perform surgery and other procedures: Boston Children’s cardiac surgeons have vast experience in the surgical procedures used to repair this defect, and they work with nurses and doctors who are focused on providing expert care after surgery. Boston Children’s CICU is one of the very first such units to be developed anywhere, and professionals from many countries visit the CICU to learn advanced techniques of post-operative care.

    Transposition of the great arteries: Reviewed by David Harrild, MD
    © Boston Children's Hospital, 2010

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