Double Outlet Right Ventricle (DORV)

Nemours Cardiac Center at Nemours/Alfred I. duPont Hospital for Children

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Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most. At the Cardiac Center, we specialize in early detection and repair of congenital heart defects (also often called, “congenital heart disease”).

If your child’s been diagnosed with double outlet right ventricle (or “DORV,” for short), we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.

What Is It?

Double outlet right ventricle (DORV) is a range of anatomic problems in which both of the main arteries that leave the heart (the pulmonary artery and the aorta) leave from the right ventricle rather than from the one leaving from each ventricle. There’s also a hole between the two ventricles (a ventricular septal defect, or “VSD”).

The result is that, as with tetralogy of Fallot, oxygen-poor blood flows from the right ventricle and out to the body. The blood oxygen level varies between children based on the exact anatomy of the connection between the aorta and right ventricle, but children may be bluish (called “cyanosis”) if a lot of oxygen-poor blood is able to flow out the aorta to the body.

Double outlet right ventricle is on a spectrum of congenital heart disease that starts at tetralogy of Fallot and ends at transposition of the great arteries.  Some children will have defects that are very similar to tetralogy of Fallot, whereas others will have defects very similar to transposition of the great arteries — and some will have types that are somewhere in between the two.

Among children with the type similar to tetralogy of Fallot, the pathway for oxygen-poor blood leaving the right ventricle to go to the lungs (called the “right ventricular outflow tract”) may be too small, which results in more “blue” blood traveling out the aorta to the body. Children with double outlet right ventricle (DORV) may also be at risk for having hypercyanotic spells.

How Does the Heart Normally Work?

When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »

Nemours’ experts at also offer these helpful resources to help both you and your child understand how the heart works:

How & When Is It Diagnosed?

Double outlet right ventricle (DORV) may be diagnosed fetally (in the womb during pregnancy) with a fetal echocardiogram (or “echo”), but may not be identified until after birth. Because there’s a wide range of anatomic defects in double outlet right ventricle (DORV), there’s a wide-range of symptoms as well. Some children may have heart failure symptoms very similar to a large ventricular septal defect (“VSD”).

Heart failure symptoms include:
  • rapid breathing
  • rapid heart rate
  • poor ability to feed and lack of weight gain (also known as “failure to thrive”)

Other children may have low oxygen levels in the body (which causes cyanosis) as with tetralogy of Fallot or transposition of the great arteries. 

How & When Is It Repaired?

When double outlet right ventricle (DORV) is diagnosed, children will usually require surgery within the first six months after birth. The surgical technique will depend on the type of DORV. “Simple” DORV may be repaired like a ventricular septal defect (VSD) closure, as compared to the tetralogy of Fallot type, which also requires repair of the pulmonary artery. Or surgery may be more complicated, requiring VSD closure in addition to an arterial switch operation.

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How Will It Affect My Child Long Term?

Because of the range of defects included within the category of double outlet right ventricle (DORV), there’s a range of long-term consequences. Some children will have a single surgery early in life and never need another. Others may require multiple operations over their lifetime, often to ensure that the blood flow out of the heart, through either the pulmonary artery or aorta, remains open.

If your child has double outlet right ventricle (DORV), know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart defect journey — and to help your child live the healthiest, most fulfilling life possible.

Learn More About Congenital Heart Defects »

Why Choose Us

From our outcomes to our family-centered care, find out all of the reasons why your child’s heart will be in good hands at the Nemours Cardiac Center. Learn More »

Make an Appointment

(800) 416-4441

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Information for Patients

Outpatient Services and Inpatient Units: (302) 651-6660

After 5 p.m. and Weekends:

Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690


+1 (302) 651-4993

Stories From the Heart

Meet children treated in the Nemours Cardiac Center who know what it’s like to live with a heart problem.

Read Their Inspiring Stories »