Definition

Double-outlet right ventricle (DORV) is a rare heart defect. The aorta comes from the heart’s left ventricle. With DORV, the aorta and the pulmonary artery are attached to the right ventricle.

A heart defect called ventricular septal defect (VSD) often happens with DORV. VSD is a hole in the wall between the right and left ventricle. DORV can be categorized based on the position of the VSD. The pulmonary valve may also be narrowed.

Heart Chambers and Valves
heart anatomy

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Blood Flow Through the Heart

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Causes

DORV is present at birth. It is not known exactly why the heart does not form the right way.

Risk Factors

The risk factors are not clear. Some factors for DORV may be:

  • Family history of congenital heart defects
  • Certain chromosomal problems

Symptoms

Symptoms may vary based on where the VSD is. A child may have:

  • Bluish skin color
  • Problems feeding
  • Slow weight gain
  • Lack of energy
  • Sweating
  • Rapid breathing
  • Shortness of breath

Diagnosis

The doctor will ask about your child’s symptoms and health history. A physical exam will be done.

Pictures may be taken of your child's body. This can be done with:

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

Treatment

Surgery is often needed right away to fix DORV. The goal is to connect the aorta to the left ventricle. Surgery can range in complexity. The doctor may insert a shunt or make 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. If there are other defects, a more complex surgery may be needed to change the position of the large arteries and reconnect other vessels.

Lifelong monitoring by a heart specialist will be needed.

Prevention

There are no known guidelines to prevent this health problem.

RESOURCES:

American Heart Association
http://www.heart.org

Healthy Children—American Academy of Pediatrics
http://www.healthychildren.org

CANADIAN RESOURCES:

Canadian Cardiovascular Society
http://www.ccs.ca

Heart and Stroke Foundation of Canada
http://www.heartandstroke.ca

REFERENCES:

Ventricular septal defect (VSD). EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/ventricular-septal-defect. Accessed November 3, 2020.

Double-outlet right ventricle in children. Boston Children's Hospital website. Available at: http://www.childrenshospital.org/conditions-and-treatments/conditions/double-outlet-right-ventricle-dorv. Accessed November 3, 2020.

Skinner J, Hornung T, et al. Transposition of the great arteries: from fetus to adult. Heart. 2008 Sep;94(9):1227-1235.

Transposition of the great vessels. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/transposition-of-the-great-vessels. Accessed November 3, 2020.

Last reviewed September 2020 by EBSCO Medical Review Board Kari Kassir, MD  Last Updated: 11/3/2020