Single Ventricle Defects in Children

  • Single ventricle defects encompass a large spectrum of disorders, most of which require a series of staged surgeries. At Children's we treat a large volume of these and many other congenital heart problems, so we have a deep expertise.

    --David W. Brown, MD, Boston Children's Hospital Department of Cardiology

    If your infant or child has been diagnosed with a single ventricle defect, an understanding of the condition will help you to cope with this very rare congenital (present at birth) heart defect.

    “Single ventricle defect” is a general, non-specific term used to describe several congenital heart defects that differ from each other, but that share the same problem: The heart has only one adequate-sized functional pumping chamber (ventricle).

    normal heart
     Comparison with normal heart

    Normally, the heart has two pumping chambers (ventricles) that serve as the heart's pumping chambers. The right ventricle normally pumps blue blood (without oxygen) out of the heart through the pulmonary artery to the lungs for oxygen, and the left ventricle normally pumps red blood (with oxygen) through the aorta out of the heart to the body.

    In one of the single ventricle defects, only one of those pumping chambers is adequately-sized and functional. Additional defects and uniquely complex heart anatomy distinguish one single ventricle defect from another.

    HLHS normal heart

    Two examples of single ventricle heart defects. 

    The Boston Children's Hospital approach to single ventricle defects

    The experienced surgeons in the Boston Children’s Cardiac Surgery Department understand how distressing the diagnosis of a single ventricle defect 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 your child’s heart anatomy, with interpretation by highly experienced cardiologists.
    • sophisticated therapies: Babies with single ventricle defects usually need emergency therapy. Our cardiac intensive care unit (CICU), cardiac cath labs and operating rooms deal with the urgent needs of our smallest patients 24/7. Boston Children’s CICU was one of the first such units developed anywhere; professionals from many countries visit our CICU to learn advanced techniques of post-operative care.

      Boston Children’s Congenital Heart Valve Program cares for children with congenital heart defects that involve absent or malfunctioning heart valves. Our surgeons have a strong record of excellence in heart valve repair and replacement, including minimally invasive techniques.
    • experienced team of skilled 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.
    • close, expert medical follow-up: If you live in the Boston area, a Boston Children’s cardiologist will follow your child after surgical repair; if you live in another part of the country or the world, a Boston Children’s cardiologist will work closely with your local cardiologist. Adult patients with single ventricle defects are followed by Boston Children’s cardiologists with special training for adults with congenital heart problems.

    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.

    For visual and audio information on some congenital heart defects, visit Boston Children’s cardiovascular Multimedia Library.

    Single ventricle defect: Reviewed by David W. Brown, MD
    © Boston Children's Hospital, 2011

     

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