Double Outlet Right Ventricle (DORV)

What is double outlet right ventricle?

Double outlet right ventricle (DORV) is a rare congenital heart defect, meaning it’s a condition a baby is born with. In DORV, the pulmonary artery and the aorta — the heart’s two major arteries — both connect to the right ventricle. In a normal heart, the pulmonary artery connects to the right ventricle, and the aorta connects to the left ventricle. DORV creates a problem because the right ventricle carries oxygen-poor blood, which then gets circulated in the body.

Another heart condition, called a ventricular septal defect (VSD), always occurs with DORV. This is a hole in the tissue wall (septum) that normally separates the right and left ventricles. The VSD allows oxygen-rich blood to pass from the left ventricle to the aorta and pulmonary artery. But even with this added oxygen, the body may still not get enough, causing the heart to work harder.

Normal heart and DORV heart illustration

A child with DORV may also have other heart problems, including:

If your child has DORV, the severity of the condition and type of treatment, including the type of surgical repair, will vary depending on which types of defects he or she has. Although DORV is a serious condition, it is treatable with surgery.

What are the symptoms of double outlet right ventricle?

Common symptoms of double outlet right ventricle (DORV) in babies include:

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

In older children, symptoms may include:

  • fatigue
  • shortness of breath

How we care for double outlet right ventricle

The experienced surgeons in the Boston Children’s Hospital Cardiac Surgery Department 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.