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Double Outlet Right Ventricle (DORV)

  • 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

  • The DORV defect is challenging to treat. But the dedicated, compassionate team at Boston Children's Hospital is well-qualified to care for your child.

    We’re known for our science-driven approach. In fact, we’re home to the world's most extensive pediatric hospital research enterprise; and we partner with elite health care and biotech organizations around the globe. But as specialists in innovative, family-centered care, our physicians never forget that your child is precious, and not just a patient.

    What are the different types of DORV defect?

    There are four major types of DORV defect, which can be classified by the location of the hole (ventricular septal defect or VSD) with respect to the aorta and the pulmonary artery. The most common of these types is a DORV where the VSD is located:

    • just below the aorta (subaortic)

    The location of the VSD also defines the other three types of DORV. These other types locate the VSD:

    • below the pulmonary artery (subpulmonary)
    • below both the aorta and the pulmonary artery (doubly committed)
    • in a position away from the great arteries (non-committed)

    These four types of DORV—as well as the presence or absence of associated pulmonary stenosis and other defects—will determine how severe your baby’s condition and symptoms are.

    Additional but somewhat less common defects that may be present with DORV include:

    Causes

    It’s important to understand that as parents, you’ve done nothing to cause DORV and its accompanying defects. Nothing you've ingested or have been exposed to environmentally has been linked to having a child with DORV. Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development.

    Symptoms

    In infants and babies:

    • rapid breathing
    • rapid heartbeat
    • sweating
    • disinterest in feeding or tiring while feeding
    • poor weight gain
    • blue color of the skin, lips and nailbeds (cyanosis)
    • heart murmur (detected by doctor)

    In older children:

    • fatigue
    • shortness of breath

    When to seek medical advice
     

    Call your health care provider immediately if your baby or child is having difficulty breathing or is breathing rapidly, has a bluish color, tires easily or is uninterested in eating.

    Who’s at risk

    Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development. So, it’s difficult to predict who’s at risk. Familial cases have been reported. Occasionally, chromosomal abnormalities have been found in some children with DORV.

    Complications

    Children who've undergone DORV repair could be susceptible to congestive heart failure, or lung damage resulting from high blood pressure in the lungs. However, with improved treatment techniques and follow-up care, these complications are becoming increasingly less common.

    There’s a risk of arrhythmias occurring, especially for patients who've had their repairs later in life. And some people who've undergone DORV repair will require lifelong medication to prevent heart infections.

    Long-term outlook

    The large majority of DORV defects can be corrected, so the outlook for your child is good. Arrhythmias and other long-term complications can occur—particularly if patients undergo repair at a later age—but these are usually manageable.

    Your cardiologist will help you create a long-term care program as your baby matures into childhood, the teen years and even adulthood. Most people who have had congenital heart disease repair have an ongoing relationship with their cardiologist; we will consult and treat your child if complications arise, and will advise on daily-life issues such as exercise, activity levels, nutrition and pregnancy precautions.

    Will my child be OK?

    Open heart surgery at Boston Children’s has among the highest success rates in the United States among large pediatric cardiac centers. With our advanced surgical techniques and timely, family-centered care, the overwhelming odds are that your child will have a successful surgical DORV repair.   

    Note: Infants who’ve had initial surgery for a single ventricle defect are typically enrolled in Boston Children’s Home Monitoring Program between the baby’s Stage I and Stage II operations in the Fontan sequence.

    What about later in life?

    We've found that the large majority of DORV defects can be corrected. Abnormal heart rhythms and other long-term complications can occur—particularly if patients undergo repair at a later age—but these are usually manageable.

    Your cardiologist will help you create a long-term care program as your baby grows into childhood, the teen years and even adulthood. Most people who've had congenital heart disease repair will have an ongoing relationship with their cardiologist; we will consult and treat your child if complications arise, and will advise on daily-life issues such as exercise, activity levels, nutrition and pregnancy precautions.

    Where can my child find care and support when she grows up?

    BACH is an international center for excellence, with physicians and services from Children’s, Brigham and Women’s Hospital and Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.

    • The Adult Congenital Heart Association (ACHA) is a national not-for-profit organization dedicated to improving and extending the life of adults with congenital heart defects. The organization serves and supports the many adults with congenital heart defects (now thought to number at least 1 million), their families and the medical community, as well as conducting research and providing advocacy, outreach and education.
  • At Boston Children's Hospital, we know that the first step in treating your child is forming an accurate and timely diagnosis.

    The exams

    If your newborn baby was born with a bluish tint to her skin, or if your young child is experiencing symptoms, your pediatrician will refer you to a pediatric cardiologist, who will perform a physical examination. The cardiologist will listen to your baby’s heart and lungs, measure the oxygen level in her blood (non-invasively) and make other observations that help to determine the diagnosis.

    Your Boston Children's Hospial cardiologist will also investigate whether your child has a heart murmur—a noise heard through the stethoscope that’s caused by the turbulence of blood flow. The location in the chest where the murmur is best heard, as well as the sound and character of the murmur itself, will give the cardiologist an initial idea of the kind of heart problem your baby may have.

    The tests

    Some combination (not necessarily all) of the following medical tests will also used to diagnose DORV and its related defects:

    • Electrocardiogram (EKG): An EKG evaluates the electrical activity of your child’s heart. An EKG is usually the initial test for evaluating the causes of symptoms and detecting heart abnormalities, including DORV. It's performed by placing electrodes on the arms, legs and chest to record the electrical activity. The test takes five minutes or less and involves no pain or discomfort.
       
    • Echocardiogram (cardiac ultrasound): An echocardiogram evaluates the structure and function of your child’s heart using electronically recorded sound waves that produce a moving picture of the heart and heart valves. No discomfort is involved. It takes 30-60 minutes. Some younger children may need to be sedated.

      If, during your pregnancy, a routine prenatal ultrasound or other signs raise your obstetrician’s suspicion of a congenital heart defect in the fetus, a cardiac ultrasound (described in the paragraph above) of the baby in utero will usually be the next step. The cardiac ultrasound—focusing exclusively on the baby’s heart—can usually detect whether a congenital heart defect is present.
       
    • Cardiac magnetic resonance imaging (MRI): An MRI is a non-invasive test using 3-D imaging technology produced by magnets to accurately determine the blood flow and functioning of your child’s heart. No pain is involved, although an IV may be needed. It takes about an hour. Children under 10 years of age usually need anesthesia.
       
    • Chest x-ray: A conventional chest x-ray will evaluate the size and spatial relationships of the heart within the child’s chest, as well as the presence of defects associated with DORV. It takes a few moments. There's no pain or discomfort.
       
    • Cardiac catheterization: This invasive procedure performed under sedation or anesthesia provides detailed visual information and measurements about the structures inside the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta.

    Watch some of Boston Children’s frequently used cardiac procedures in action

    Pediatric cardiologists and pediatric cardiovascular surgeons at Boston Children's Hospital have pioneered the interventional catheterization repair techniques now used widely for many congenital heart defects, including double outlet right ventricle.

  • Treatment options

    Having identified your child's condition, we're able to begin the process of treating her, so that we may ultimately return your child to good health.

    How we treat your child depends on her combination of heart defects. The associated defects—and their relationships to each other—largely determine which procedures we perform. At Boston Children's Hospital, we rarely perform palliative or temporary operations; for any age or size, we prefer to do a corrective procedure.

    Your child may have a:

    Despite these several variations, the common surgical goals for DORV are to

    • connect the aorta to the left ventricle, and the pulmonary artery to the right ventricle
    • correct whatever other defects are present

    These goals can be addressed through a variety of surgical approaches, including, but not limited to:

    • intraventricular tunnel repair: This is the most typical surgical procedure for DORV with a ventricular septal defect (VSD). Surgeons create a kind of tunnel (“baffle”) connecting the left ventricle to the aorta. A patch placed within the right ventricle directs left ventricle flow to the aorta.
       
    • arterial switch operation: This procedure is necessary only if, in addition to the arteries arising from the right ventricle, they are also reversed in relation to each other.

    A tunnel from the VSD to the pulmonary artery is created, connecting the left ventricle to the pulmonary artery. Then, the vessels are disconnected and reconnected so that the pulmonary artery becomes the aorta, and the aortic valve is connected to the pulmonary artery, and associated holes between the chambers of the heart are closed.

    • to repair pulmonary stenosis or pulmonary atresia
      • patch: If pulmonary stenosis is present, a patch may be necessary to open the passage to the lungs.
      • conduit: If pulmonary atresia is present, a tube (conduit) may need to be placed between the right ventricle and the pulmonary artery.
         

    At home: caring for your child after DORV repair

    Your child's cardiologist will offer recommendations for post-operative follow-up care, including:

    • a nutritional program to encourage weight gain
    • an oral hygiene program to prevent infection
    • an appropriate exercise routine to build body mass and achieve fitness

    We'll also help you to create a longer-term care program as your baby grows into childhood, the teen years and even adulthood. Most persons who've had congenital heart disease repair will have an ongoing relationship with their cardiologist. We will consult and treat if complications arise, and will advise on daily-life issues such as exercise, activity levels, nutrition and pregnancy precautions.

    As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups. And to the greatest extent possible, encourage your child to live normally. Even if some physical activities are limited, your child and your family can enjoy a full life together.
     

    Boston Children's Home Monitoring Program for your child

    An infant with single ventricle anatomy needs support with shunt-dependent blood flow between his Stage I and Stage II surgical repairs (see surgery descriptions above on this page).

    The results of the Stage I surgery have improved—with nearly 90% of infants who are cared for in experienced centers discharged home after the first stage. So we can now focus new attention on reducing the known mortality of 10 to15 percent for these infants between their Stage I and Stage II surgical repairs.

    Research shows the vital importance of a Home Monitoring Program, including daily at-home assessments of oxygen saturations and weight between the Stage I and Stage II surgeries.

    Checking your baby's daily weight:

    • alerts you and your child's providers to dehydration
    • enables appropriate immediate intervention
    • decreases the risk of sudden death at this very fragile stage in your infant's multiple-surgery sequence

    During this period between the Stage I and Stage II surgeries, your child's pediatric cardiologist and pediatrician will be in close contact with you as your child's primary home caregiver. (After your child's Stage II repair, this intensive level of home monitoring will no longer be necessary.)

    In Boston Children's Home Monitoring Program:

    • You'll be given detailed guidelines and goals for your child's:
    • growth
    • weight gain
    • oxygen saturations
       
    • You'll be instructed to call your child's cardiologist if his goals for growth, weight gain and oxygen saturation aren't met, or if:
      • your child is breathing harder, faster or is fussy beyond his baseline
      • you have any questions or concerns
         
    • You'll be supplied with a pulse oximeter and a baby scale and shown how to use both instruments. You'll measure your child's saturations twice a day and weigh the baby daily—checking against given guidelines and goals.
       
    • You'll record your baby's weights, oxygen saturations and general observations in a daily log, and will share this log with the pediatrician/cardiologist during check ups.
       
    • You'll consult your pediatrician if your child:
    • is experiencing nausea/vomiting/diarrhea
    • is not having sufficient wet diapers
    • is feverish
       
    • You'll have weekly calls with your designated pediatric nurse practitioner, who will consult with you, and who will notify your child's cardiologist of any concerns that arise.
       

    As she grows: your child's long-term outlook

    Because of the complexity of double outlet right ventricle, your baby's recovery and prognosis can depend on many variables involving the VSD, the pumping chambers (ventricles) and additional defects.

    Nevertheless, Children's cardiologists and surgeons are confident that the great majority of DORV defects can be repaired. Arrhythmias and some other long-term complications can occur—particularly if patients undergo repair at a later age—but these are usually manageable.

    Thanks to ever-improving diagnoses and treatments for the DORV defect, damage to the baby's blood vessels or lungs is now rare. And the medical science on congenital heart defects continues to evolve and improve.
     

    Coping and support

    At Boston Children's, we understand that a hospital visit can be difficult, and sometimes overwhelming. So, we offer many amenities to make your child's—and your own—hospital experience as pleasant as possible. Visit The Center for Families for all you need to know about:

    • getting to Boston Children's
    • accommodations
    • navigating the hospital experience
    • resources that are available for your family

    In particular, we understand that you may have a lot of questions if your child is diagnosed with DORV. How will it affect my child long term? What do we do next? We can connect you with a number of resources to help you and your family through this difficult time, including:

    • patient education: From the office visit to pre-op to the recovery room, our nurses will be on hand to walk you through your child's treatment and help answer any questions you may have — How long will I be separated from my child during surgery? What will the operating room be like? They will also reach out to you by phone, continuing the care and support you received while at Children's.
       
    • parent-to-parent: Want to talk with someone whose child has been treated for DORV? We often can put you in touch with other families who've been through the same procedure that you and your child are facing, and who will share their experiences.
       
    • faith-based support: If you're in need of spiritual support, we'll connect you with the Children's chaplaincy. Our program includes nearly a dozen clergy— representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions—who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.
       
    • social work: Our social workers and mental health clinicians have helped many families in your situation. We can offer counseling and assistance with issues such as coping with your child's diagnosis, stresses relating to coping with illness and dealing with financial difficulties.
       
    • As your child reaches adulthood, you'll want her to know about the Boston Adult Congenital Heart (BACH) and Pulmonary Hypertension Service. Children's is a founding institution of BACH, an international center for excellence providing long-term inpatient and outpatient care and advanced therapeutic options as needed for congenital heart disease patients as they reach and progress through adulthood.
  • A significant amount of the groundbreaking cardiac research being conducted at Children’s Hospital Boston aims to refine and advance the open heart surgery and catheterization procedures that correct congenital heart defects in newborns and young children—including double outlet right ventricle.

    Cardiac surgery research

    Members of the Children’s Cardiac Surgery Research Laboratory—a multidisciplinary team of basic and applied research investigators who hold faculty appointments at Harvard Medical School—are studying the mechanisms of heart disease and new treatments for children with congenital heart defects.

    Some principal areas of active research are:

    • surgical robotics and ultrasound-guided intracardiac surgery: The department is pioneering the experimental use of 3-D ultrasound and laparoscopic techniques in animals, to operate on the beating heart.
       
    • myocardial metabolism and myocardial hypertrophy and heart failure: Researchers are exploring new methods of myocardial preservation during heart surgery and the role of angiogenic growth factors in heart failure.
       
    • tissue engineering to stimulate the growth of new tissue to repair congenital defects, including valve abnormalities, right ventricular defects and arrhythmias.

    Learn more about Boston Children’s cardiac research.

    Cardiology research

    Boston Children’s is a world leader in opening new avenues of “translational research,” bringing laboratory advances to the bedside and doctor’s office as soon as possible. Senior medical staff members of the Department of Cardiology participate in clinical research activities, and many do laboratory research, as well.

    Learn more about Boston Children’s cardiology research.

    Innovations: Creating new ways to perform surgery

    Problem: When surgeons perform heart surgery on a baby, they need to open the infant’s chest and stop her heart—an inva­sive, lengthy procedure that can cause life-threatening complications. Pedro del Nido, MD, chief of Cardiac Surgery at Boston Children’s, had to perform surgery on his tiny patients using this method, or come up with a way to improve it.

    Innovative solution: Del Nido decided to develop a way to perform surgery on a still-beating heart. But he needed two things that didn’t exist: superior imaging tools that could show the structures inside the heart while it’s beating, and tiny instruments to perform the intricate surgery.

    So, he bor­rowed technology from the videogame industry and developed stereo-rendered 3-D ultrasound imaging that allows surgeons to see inside the beating heart as a hologram.

    Del Nido also designed new instruments. One is a millimeter-sized tool that extends into the heart through needle-sized incisions. Using a joystick controller and real-time imaging, a surgeon can now navigate through the beating heart’s chambers to remove blockages, repair faulty valves and close leaks.

    The other new instrument is a cardioport device that allows instruments to be safely introduced into the cardiac chambers with­out the usual risks of blood loss or an air embolism.

    Results: Del Nido’s 3-D tool appears not only to provide superior imaging, but also to yield faster surgery times. Researchers using it to operate on pigs with congenital heart disease performed the procedure 44 per­cent faster than before. Dr. Del Nido’s cardioport will soon be tested in clinical trials and will facilitate further development of similarly novel instruments for heart repair. Del Nido’s newly-developed cardioport
    will someday make possible faster, less invasive heart surgery.

    History of innovation

    In 1938, Boston Children’s cardiac surgeon Robert Gross, MD, performed the world’s first successful surgery to correct a child’s heart defect. Since that time, we have gained recognition around the globe for our leadership in pediatric cardiology and continue to make critical advances in the field. In 2010, U.S. News & World Report named Boston Children’s cardiology and cardiac surgery programs the best of any pediatric hospital in the country.

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