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HomePediatrics: Cardiac Disease and Critical Care MedicineDouble Outlet Right Ventricle With Normally Related Great Arteries

Double Outlet Right Ventricle With Normally Related Great Arteries

Background

Double outlet right ventricle (DORV) was first pathologically described in the late 19th century as partial transposition. In 1957, Witham first used the term double outlet right ventricle to describe a partial transposition of the great arteries.
He described 4 hearts with 2 varieties of “complete aortic transposition with the pulmonary artery in normal position.”

Double outlet right ventricle is defined as a form of ventriculoarterial connection in which both great arteries arise completely or predominantly from the morphologic right ventricle. This definition is still controversial. For example, some researchers require that the aorta and the pulmonary artery arise entirely from the right ventricle. Others require that 90% of the great vessels arise from the morphologic right ventricle. Alternatively, the 50% rule states that more than one half of both arterial trunks must arise from the morphologic right ventricle. Some require only the presence of fibrous discontinuity between the mitral and semilunar valves. This is present in most specimens and is referred to as subpulmonic and subaortic conus.

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