What is double outlet right ventricle?

Double outlet right ventricle (DORV) is a congenital heart defect. There is a malformation of the fetus heart in the womb leading to the right ventricle that has the two major arteries, namely the aorta and pulmonary artery arising from it.

How are hearts with DORV different?

Normal hearts have four chambers: two atria on top and two ventricles on the bottom. Two major blood vessels, or “great” arteries, normally connect to the ventricles.

  • The aorta leaves the left ventricle and carries blood to the rest of the body.
  • The pulmonary artery leaves the right ventricle and takes blood to the lungs. The blood picks up oxygen for the body.

With DORV, both great arteries connect to the right ventricle — either totally or in part. The left ventricle has just a part of the either of the great arteries, or no artery at all.

Depending on the connections to the right ventricle, the body could receive oxygen desaturated blood or the lung could receive excessive blood flow. The heart has to work harder to make up for the shortfall. DORV can also cause too much blood to get pumped into the lungs. This extra flow can damage both the heart and the lungs.

How common is DORV?

DORV is rare. It happens just once for every 6,000 to 10,000 newborns.

Are there different kinds of DORV?

Babies with DORV always have a ventricular septal defect (VSD), too. VSD is a hole in the septum, the wall between the two ventricles. Blood flows through the hole and between the ventricles. The other possible associated anomaly is pulmonary valve stenosis.

Healthcare providers classify DORV by the location of the hole:

  • DORV with subaortic VSD: The VSD is just under the aorta.
  • DORV with subpulmonary VSD (also called Taussig-Bing): The VSD is just under the pulmonary artery.
  • DORV with doubly committed VSD: There is a VSD under each of the great arteries.
  • DORV with non-committed (or remote) VSD: The VSD is not near the aorta or the pulmonary artery.

What causes DORV?

Scientists don’t completely understand what causes DORV. In some cases, it happens to babies who have problems with their chromosomes, the cell structures that hold their DNA.

What are the symptoms of DORV?

Symptoms of DORV usually appear during the first days or weeks after birth. They’re like symptoms of other congenital heart problems and include:

  • Cyanosis (blue-ish or purple-ish skin, lips or nails).
  • Difficulty breathing, such as tachypnea (really fast breathing) or shortness of breath.
  • Difficulty eating or gaining weight.
  • Heart murmur (extra sound in the heartbeat, which a healthcare provider can hear with a stethoscope).
  • Sweating a lot, such as while eating.
  • Tachycardia (heart rate faster than 100 beats a minute).
  • Unusual sleepiness or seeming “out of it.”

What other problems can a baby with DORV have?

Babies with DORV often have other congenital problems:

  • Ciliary dysfunction: Problems develop with tiny hair-like structures that line the airway.
  • Heterotaxy: Organs in the chest and belly are not in the usual places.
  • Intestinal malrotation: With intestinal malrotation, a baby’s intestines twist in a way that isn’t normal.
  • Pulmonary stenosis: With pulmonary stenosis, the pulmonary valve (between the right ventricle and pulmonary artery) is too small or stiff.
  • Ventricular hypoplasia: One or both ventricles are smaller than normal.

Last reviewed by a Cleveland Clinic medical professional on 12/21/2020.

References

  • Artrip JH, Sauer H, Campbell DN, et al. Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification. Eur J Cardiothorac Surg. 2006;29(4):545-50. Accessed 12/15/2020.
  • Centers for Disease Control and Prevention. What Are Congenital Heart Defects? Accessed 12/15/2020.
  • Orphanet. Double Outlet Right Ventricle. Accessed 12/15/2020.
  • Pang K-J, Meng H, Hu S-S, et al. Echocardiographic classification and surgical approaches to double-outlet right ventricle for great arteries arising almost exclusively from the right ventricle. Tex Heart Inst J. 2017;44(4):245-51. Accessed 12/15/2020.
  • Centers for Disease Control and Prevention. Data & Statistics on Birth Defects. Accessed 12/15/2020.

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