Double-Outlet Right Ventricle

Disparate lesions are grouped together under the heading of double-outlet right ventricle (DORV) simply because both of the arterial trunks are supported in their greater part by the morphologically right ventricle. This chapter focuses on the forms of DORV with two functional ventricles. For a long period, controversy revolved around whether the definition of DORV should include the presence of aortic to mitral discontinuity. Almost nine-tenths of surgical patients with DORV have concordant atrioventricular connections. Doubly committed defects occur in approximately one-tenth of surgical series of DORV. DORV has most often been classified anatomically in the literature according to the pure relational anatomy of the defect to the arterial roots: subaortic, doubly committed, subpulmonary, and noncommitted. Without other major cardiac anomalies, the clinical presentation of patients with DORV depends on the relationship of the defect to the great arteries and the presence or absence of pulmonary stenosis.