Reference Work Entry

Encyclopedia of Molecular Mechanisms of Disease

pp 543-545

Double Outlet Right Ventricle

  • Mary Redmond HutsonAffiliated withDepartment of Pediatrics, NPRI, Duke University
  • , Margaret L. KirbyAffiliated withDepartment of Pediatrics, NPRI, Duke University


DORV; Dextroposed aorta; Overriding aorta; Taussig-Bing anomaly; Tetralogy of Fallot

Definition and Characteristics

DORV encompasses a range of heart defects, in which the great vessels are misaligned with respect to the ventricles. Normally the pulmonary trunk arises from the right ventricle (RV) and aorta from the left ventricle (LV). In DORV, both the pulmonary artery and the aorta arise completely or mostly from the RV. If the aorta obtains 50% of its blood from the RV, the defect is classified as DORV [1]. Because both outflow vessels originate predominately from the RV, there is a concomitant ventricular septal defect (VSD).

The DORV is categorized based on the position of the VSD in relation to the outflow vessels [1] (Fig. 1). A VSD below the aorta is called DORV with sub-aortic VSD. The great arteries are in a “side-by-side” position with the aortic valve shifted to the right of the pulmonary valve. A VSD below the pulmonary artery is ...

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