Double Outlet Right Ventricle

Double outlet right ventricle (DORV) is a congenital heart defect that causes the aorta to be connected to the heart in the wrong place.

Usually, the aorta is attached to the left ventricle (pumping chamber) and the pulmonary artery is attached to the right ventricle. In babies with DORV, both vessels are attached to the right ventricle.

Along with the misplaced aorta, babies with DORV also have a ventricular septal defect (VSD), a hole in the wall that separates the left and right ventricles. Babies born with DORV almost always show signs of the problem within a few days of birth. Surgery is needed to correct the problem.

As the leading providers of pediatric heart care in Louisville and Southern Indiana, cardiothoracic surgeons at Norton Children’s Heart Institute are experienced with successfully repairing double outlet right ventricle.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute, affiliated with the University of Louisville, have the skill and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child.

Infants who have a repaired DORV will need to see a pediatric cardiologist throughout childhood, adolescence and adulthood.

In a baby with DORV, oxygen-rich blood from the lungs returns to the left ventricle, but can’t leave through the aorta as it does in a healthy heart. The blood is forced through the hole — the ventricular septal defect — where it mixes with the oxygen-poor blood returning from the body in the right ventricle.

With each heartbeat, the right ventricle squeezes the mixed blood out to the lungs through the pulmonary artery. It also pumps blood into the misplaced aorta. The mixing of oxygen-rich and oxygen-poor blood makes the heart with DORV work harder than it should.

The position and size of the VSD and the positions of the pulmonary artery and aorta are different for each baby. Some babies with DORV have more severe symptoms than others.

Double Outlet Right Ventricle Symptoms

Within a few days of birth, a newborn with DORV usually will show these signs:

  • Breathing fast or hard
  • Pale or bluish skin
  • Sweating
  • Poor feeding with little weight gain
  • Sleepiness

Double Outlet Right Ventricle Causes

DORV is due to an error in the way the heart forms very early in pregnancy. The cause is unknown and doctors and scientists have not yet found a way to prevent DORV. Babies with certain genetic conditions — such as trisomy 13, trisomy 18, or DiGeorge syndrome (22q11.2 deletion) — are more at risk for DORV.

Double Outlet Right Ventricle Diagnosis

DORV sometimes is seen on ultrasound scans before birth. A fetal echocardiogram, a more detailed ultrasound of a fetus before birth, may provide more information and guide the delivery team’s preparations.

If the diagnosis is made after birth, the baby’s skin color and difficulty breathing will indicate a problem. Doctors listening to the baby’s heartbeat may hear an abnormal sound called a murmur. Possible tests include:

  • Pulse oximetry: Measurement of the oxygen level in blood using a light at a fingertip or toe
  • Chest X-ray: An image of the heart and surrounding organs
  • Electrocardiogram (ECG or EKG): A recording of the heart’s electrical activity
  • Echocardiogram: Ultrasound images and videos of the heart
  • Cardiac magnetic resonance imaging (MRI)or computed tomography (CT) scan

In some cases, a cardiac catheterization is needed. Babies born with DORV often have more than one heart problem.

Double Outlet Right Ventricle Treatment

Surgery is needed to correct DORV. Medicines might help the heart work better, but a baby with DORV cannot get better for long without surgery.

DORV can be corrected in a single-ventricle palliation approach, likely with multiple surgeries, or a complete two-ventricle repair. The decision on what repair to choose will depend on the size of the ventricles and other heart defects that are present.

Why Norton Children’s Heart Institute?

Norton Children’s Heart Institute, affiliated with the University of Louisville, is a comprehensive pediatric heart surgery, heart failure and heart transplant program serving Kentucky, Southern Indiana and beyond.

The goal of the full-service Norton Children’s Heart Institute is to provide care for the child and the whole family. Our specialists are prepared to repair even the most complex congenital and acquired heart conditions.

Our heart team includes:

  • Pediatric cardiothoracic surgeons
  • Pediatric transplant surgeons
  • Pediatric cardiologists
    • Fetal cardiologists
    • Adult congenital heart cardiologists
    • Heart failure/heart transplant cardiologist
    • Pediatric electrophysiologists
    • Pediatric cardiac catheterization cardiologists
  • Pediatric cardiovascular anesthesiologists
  • Pediatric intensive care physicians
  • Specially trained nurses
  • Specially trained pharmacists
  • Family support team
  • Child life specialists
  • Rehabilitation specialists
  • Social workers
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