Double outlet right ventricle

Last revised by Daniel J Bell on 1 Mar 2021

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.

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