Dayton Children’s Hospital

Double Outlet Right Ventricle (DORV)

What is double outlet right ventricle?

Babies diagnosed with double outlet right ventricle (DORV) are born with a heart defect in which both arteries arise from the right ventricle instead of one from each ventricle. This is a problem because it often allows too much blood to flow to the lungs and not enough to flow to the body or vice versa. The right ventricle normally pumps blood through the pulmonary artery to the lungs where it picks up oxygen; the oxygen-rich blood then goes to the left ventricle and is pumped out through the aorta to the body.

Ventricular septal defect (VSD), another congenital heart condition, always occurs in conjunction with DORV. Other conditions that may also occur in babies who have DORV include pulmonary valve stenosis and transposition of the great arteries.

How is DORV diagnosed?

While examining your baby, your doctor will first listen for a heart murmur with a stethoscope. If your doctor hears a heart murmur or if your baby is showing symptoms of DORV, tests may be done to confirm the diagnosis.

Babies who have DORV may show these symptoms:

  • Tiring easily, especially when feeding
  • Bluish color of the skin and lips
  • Clubbing (thickening of the nail beds) of the fingers and toes
  • Poor growth or poor weight gain
  • Pale skin
  • Sweating
  • Difficulty breathing
  • Rapid breathing
  • Rapid heartbeat

Tests to diagnose DORV may include:

  • Chest X-ray: a painless test that creates pictures of the heart and lungs
  • Cardiac catheterization: a procedure where a thin, flexible tube inserted through a large artery and into the heart allows your doctor to see the flow of blood through your baby’s heart and blood vessels on an X-ray image
  • Echocardiogram: a painless test that uses sound waves to create a moving picture of the heart
  • Magnetic resonance imaging (MRI)

How is DORV treated?

If your baby has DORV, doctors will repair the VSD and reroute blood from the left ventricle into the aorta during surgery. Surgery may also be needed to repair or replace the pulmonary valve if it is abnormal.

The type and number of operations your baby will need depend on the type and severity of your baby’s DORV, what other heart defects your baby has and your baby’s overall condition.

How will DORV affect my baby during and after surgery?

During surgery, your baby will be given general anesthesia, which means the baby will be comfortable and sleeping. After surgery, your baby will need to stay in a cardiac intensive care unit (CICU) for the first few days and may be connected to several tubes and wires to allow your baby’s doctor to best monitor the baby’s condition. Pain control will be used to make sure your baby is comfortable.

Most of the tubes and wires will be disconnected before your baby leaves the CICU, after which he or she will spend several more days in the hospital in a step down unit.

How well your baby does after surgery will depend on:

  • The size and location of the VSD
  • The function of your baby’s ventricles
  • The status of the pulmonary valve
  • Whether or not your baby has other heart conditions
  • Your baby’s overall health at the time of diagnosis
  • Whether or not lung damage has occurred from too much blood flowing to the lungs