Treatments for double outlet right ventricle (DORV) in children

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Contact the Complex Biventricular Repair Program

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.

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We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

Boston Children's Hospital 300 Longwood Avenue, Boston, MA 02115 617-355-6000 | 800-355-7944

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