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Women's & Children's Hospital

Double-Outlet Right Ventricle -- Child


Double-outlet right ventricle (DORV) is a rare heart defect. In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle. Next, the blood travels to the lungs through the pulmonary valve. Here, it picks up fresh oxygen. The blood returns to the left atrium and goes into the left ventricle. The blood moves out to the rest of the body.

Normally, the aorta, which is the largest artery in the body, comes from the heart’s left ventricle. But when DORV occurs, the aorta and the pulmonary artery are attached to the right ventricle.

Another heart defect called ventricular septal defect (VSD) usually occurs with DORV. VSD is a hole in the wall between the right and left ventricle. DORV can be categorized into several types depending on the position of the VSD.

Along with these conditions, the pulmonary valve may be narrowed. DORV is a serious condition that requires immediate care.

Heart Chambers and Valves
heart anatomy
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Blood Flow Through the Heart
Copyright © Nucleus Medical Media, Inc.


DORV is present at birth. It is not known exactly why the heart does not develop normally.

Risk Factors

For many heart defects, the risk factors are unclear. Some risk factors for DORV may include:

  • Family history of congenital heart defects
  • Certain chromosomal disorders


Symptoms may vary depending on where the VSD is located. Symptoms may include:

  • Bluish skin color
  • Poor feeding/slow weight gain
  • Fatigue
  • Sweating
  • Rapid breathing
  • Shortness of breath


Your doctor will ask about your child’s symptoms and medical history. A physical exam will be done. During the exam, the doctor may also detect a heart murmur.

Images may be taken of your child's bodily structures. This can be done with:

Your child's heart activity may be measured. This can be done with electrocardiogram (EKG).


Talk with the doctor about the best treatment plan for your child. Often, surgery is needed right away. Treatment options include:


Surgery can be done to correct DORV. The goal of surgery is to connect the aorta to the left ventricle. Surgery can range in complexity. The doctor may insert a shunt or create a new tunnel to connect the left ventricle to the aorta through the VSD. Pulmonary artery banding may be used to limit blood flow to the lungs. Depending on other abnormalities, a more complex surgery may be needed to change the position of the large arteries and reconnect other vessels.

Lifelong Monitoring

Your child will have regular exams from a heart specialist. In some cases, your child may need antibiotics prior to some medical or dental procedures. This is to prevent an infection in the heart.


There are no current guidelines to prevent DORV because the cause is unknown. Getting appropriate prenatal care is always important.

Revision Information

  • American Heart Association

  • Healthy Children—American Academy of Pediatrics

  • Canadian Cardiovascular Society

  • Heart and Stroke Foundation of Canada

  • Congenital ventricular septal defect (VSD) in children and adults. EBSCO DynaMed website. Available at: Updated May 15, 2013. Accessed July 19, 2013.

  • Double-outlet right ventricle in children. Boston Children's Hospital website. Available at: Accessed July 19, 2013.

  • Double-outlet right ventricle. Johns Hopkins Children's Center website. Available at: Updated May 16, 2011. Accessed July 19, 2013.

  • Transposition of the great arteries. EBSCO DynaMed website. Available at: Updated June 23, 2013. Accessed July 19, 2013.