Double outlet right ventricle (DORV) symptoms & causes in children

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Contact the Complex Biventricular Repair Program

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 of Double Outlet Right Ventricle (DORV)

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 of Double Outlet Right Ventricle (DORV)

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.

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- Sandra L. Fenwick, President and CEO

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