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Key Points

  • Double outlet right ventricle (DORV) is a complex cardiac malformation in which the majority of both pulmonary and aortic trunks arise from the right ventricle, typically coexisting with a VSD.

  • While DORV represents a heterogeneous group of abnormalities with various classification systems, a combined anatomic-surgical classification system is now used as it predicts optimal surgical intervention.

  • Prenatal diagnosis depends on accurate delineation of the outflow tract paths, including localizing the VSD.

  • The majority of cases have associated cardiac malformations, abnormalities of organ situs, or chromosomal abnormalities.

  • Because of the variable clinical presentation at birth, delivery should occur in controlled circumstances at a center with pediatric cardiology backup available, and the ability to begin a prostaglandin infusion.

  • Definitive surgical correction should occur during the first year of life, with most cases achieving a biventricular repair using an intraventricular tunnel.


Double outlet right ventricle (DORV) refers to a congenital cardiac malformation in which most of the pulmonary artery and the aorta arise from the right ventricle, one subtype of which is known as the Taussig–Bing heart. Such an abnormality is generally compatible with life only when there is an additional malformation that allows blood flow from one side of the heart to the other, most commonly a ventricular septal defect (VSD).

Attempts have been made to define the malformation based on the relative area of each outflow tract arising from the right ventricle, with an anatomic threshold of 50% being used. The surgical definition of DORV requires that at least 90% of the area of the outflow tracts arise from the right ventricle. The most commonly used definition requires one great artery to arise fully over the right ventricle and at least 50% of the other great artery to also originate from the right ventricle (Aoki et al., 1994). Therefore, cases of tetralogy of Fallot (see Chapter 52) in which the majority of the aortic outflow tract arises from the right ventricle could also be defined as DORV. Such anomalies may arise because of arrest of normal rotation of the outflow tracts (Bostrom and Hutchins, 1988). DORV has also been classified based on the relationship of the outflow tracts. The most common is where the aortic outflow tract is situated posterior to the pulmonary outflow tract, each spiraling around the other as they exit the heart (Romero et al., 1988). The Taussig–Bing heart is a subtype of DORV in which the aortic outflow tract ascends posterior to the pulmonary outflow tract in a parallel fashion. In the third type of DORV, the aortic outflow tract ascends anterior to the pulmonary outflow tract in a parallel fashion. DORV may also be classified based on the location of the VSD, either subaortic or subpulmonary; in the Taussig–Bing heart the VSD is subpulmonary (Bashore, 2007).

Because of this confusion ...

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