Definition

Double-outlet right ventricle (DORV) is a rare heart defect. The aorta, which is the largest artery in the body, 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) usually 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.

Along with these problems, the pulmonary valve may be narrowed. DORV is a serious health problem that needs care right away.

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

For many heart defects, 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. Your child may have:

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

Diagnosis

You will be asked about your child’s symptoms and health history. A physical exam will be done. During the exam, the doctor may also find aheart murmur.

Pictures 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 withelectrocardiogram (EKG).

Treatment

Talk with the doctor about the best plan for your child. Often, surgery is needed right away. Your child’s options are:

Surgery

Surgery can be done 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

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

Prevention

DORV can’t be prevented because the cause is not known.

Revision Information

  • Reviewer: Kari Kassir, MD
  • Review Date: 06/2018 -
  • Update Date: 06/28/2018 -