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 issue within a few days of birth. Surgery is needed to correct it.

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

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

Double Outlet Right Ventricle Symptoms

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.

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

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:

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 which repair is best will depend on the size of the ventricles and other heart defects that are present.

Infants who have a repaired DORV will need to see a pediatric cardiologist throughout childhood, adolescence and adulthood. When the child becomes an adult, an adult congenital heart defect specialist can continue to provide care.

Why Choose Norton Children’s Heart Institute

The Adult Congenital Heart Association has recognized Norton Children's Heart Institute for its expertise treating adult congenital heart disease.

  • Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
  • The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
  • The Adult Congenital Heart Association has accredited Norton Children’s Heart Institute’s Adult Congenital Heart Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
  • More than 5,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
  • Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
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