Double outlet right ventricle

Double outlet right ventricle (DORV) is a congenital cardiac anomaly where both the aorta and pulmonary trunk arise from the morphologically right ventricle. It is reported to account for ~2% of congenital cardiac defects 1. It is usually classed as a conotruncal anomaly. There is almost always a concurrent ventricular septal defect (VSD) 4.

The estimated incidence is at ~1:10,000 births.

Most cases are thought to have a sporadic occurrence.

According to the position of the great vessels 5:

  • side by side positioning of great vessels
  • right-sided malpositioning of great vessels
  • left-sided malpositioning of great vessels

According to where the VSD is located about the great vessels 6:

  • DORV with subaortic VSD
    • VSD is located just below the aorta
  • DORV with subpulmonary VSD (also called Taussig-Bing anomaly)
    • VSD is located below the pulmonary artery
  • DORV with doubly committed VSD
    • VSD under both of the great arteries
  • DORV with non-committed (or remote) VSD
    • VSD is not located near the aorta or the pulmonary artery

Appearances on chest radiographs are variable depending on the subclassification and presence of concurrent other anomalies. May show evidence of right ventricular enlargement.

On echocardiography, there is typically a lack of communication between the posterior aortic root and the anterior mitral valve leaflet.

Allows direct visualization of abnormal anatomy.

Congenital heart disease

There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorized, it is helpful to have a working understanding of normal and fetal circulation, as well as an understanding of the segmental approach to imaging in congenital heart disease.

Article information

rID: 7415
Synonyms or Alternate Spellings:
  • Double outlet right ventricle (DORV)

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Cases and figures

  •  Case 1
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  • Case 2: with dextrocardia
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  • Case 3: prenatal US: DORV with persistent LSVC
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  • Case 4: DORV after Rastelli procedure
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  • Case 5
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  • Case 6: with subpulmonary VSD (fetal echocardiogram)
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