Double Outlet Right Ventricle (DORV) in Children

  • If your infant or child has been diagnosed with double outlet right ventricle (DORV), an understanding of the condition will help you to cope with this very rare congenital (present at birth) heart defect. In a double outlet right ventricle defect, the pulmonary artery and the aorta—the heart’s two great arteries—both arise from the right ventricle. While serious, double outlet right ventricle is treatable surgically.

    Comparison with normal heart

    In a normal heart, the pulmonary artery that carries blood to the lungs to receive oxygen arises from the right ventricle. And the aorta, which carries oxygenated blood from the heart to the body, arises from the left ventricle.

    Normal vs Double Outlet Right Ventricle Heart

    Additional defects

    In addition to both of the great arteries arising from the same right ventricle, most DORV defects are accompanied by other defects. Additional defects that may be present include:

    • a ventricular septal defect (VSD), which is essentially a hole in the tissue wall (septum) that normally separates the right and left ventricles. The VSD is actually necessary in this condition, since it allows blood to pass from the left ventricle to the aorta and pulmonary artery. However, the VSD lets oxygen-rich blood mix with oxygen-poor blood, so the oxygen level in the blood is usually reduced. Exactly where the hole is in the septum will affect the surgery needed.
       
    • pulmonary (valve) stenosis (PVS, PS), a narrowing or blockage of the passageway to the lungs
       
    • transposition of the great arteries (TGA), in which the aorta and pulmonary artery can be reversed, so that the aorta is more closely related to the right ventricle and the pulmonary artery to the left ventricle 

    If your child has a DORV, the type of treatment and the degree of urgency will vary, depending on which additional defects she has.

    How Boston Children's Hospital approaches DORV

    The experienced surgeons in the Boston Children’s Cardiac Surgery Department understand how distressing a diagnosis of DORV 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. The success rates of the operations used to repair most forms of DORV are in the same range.

    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.

    Boston Children’s Cardiac Neurodevelopment Program 

    Children who’ve had surgery for heart disease as infants are at greater risk of neurodevelopmental problems. By school age, they tend to have more academic, behavioral and coordination difficulties than other children. Boston Children’s Cardiac Neurodevelopment Program—one of the few in the United States—provides expert screening, evaluation and care for infants, children and teenagers with congenital heart disease who are at risk for neurodevelopmental problems.

    Care begins soon after your child’s first cardiac surgery and continues as your child grows to make sure she’s hitting her developmental milestones.

    Double outlet right ventricle: Reviewed by Michael Freed, MD
    © Boston Children's Hospital, 2010

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