Surgical anatomy of double-outlet right ventricle--a reappraisal

In light of the recent developments in surgical treatment of double-outlet right ventricle, the anatomic observations on this lesion were reevaluated. For this review, double-outlet right ventricle was diagnosed when more than half of both arterial valves were connected to the same ventricle, although appreciating the reasons for using, in a clinical context, a "90% rule" rather than the "50% rule" used in this review. Although this ventriculo-arterial connection can exist with any segmental combination, most often it is found in the setting of usually arranged atrial chambers (solitus) and atrioventricular concordance. Categorization of this subset is then done on the basis of the relationships of the arterial trunks. Three main groups stand out: intertwining arterial trunks and "normally related" arterial valves, and parallel trunks, but with the aortic valve to the right side or left side. These groupings give information concerning the site of the ventricular septal defect, which in any group may be perimembranous, muscular or be doubly committed and subarterial. Infundibular morphology is also variable, and the proximity of the arterial valves to the roof of the defect is largely determined by the extent of the ventriculo-infundibular fold.