Double outlet right ventricle versus aortic dextroposition: morphologically distinct defects

Abstract

This study concerns the morphological differentiation between double outlet right ventricle (DORV) and aortic dextroposition (AD) defects, namely tetralogy of Fallot and Eisenmenger anomaly. Indeed, despite the similar condition in terms of sequential ventriculo-arterial connections, DORV and AD are two distinct morphological entities. It is proposed that the borderline between these two groups of malformations is represented by the specific insertion of the infundibular septum into the left anterior cranial division of the septomarginal trabeculation (or septal band) occurring in ADs and lacking in DORV. Furthermore, the spiraliform versus straight parallel arrangement of the great arteries in the two groups of anomalies is emphasized as an additional and distinctive morphological feature. Emphasis is also given to the association of straight parallel great arteries conotruncal malformations, DORV and transposition of the great arteries, with the asplenia type of heterotaxy laterality defects. Within this context, the absence of subaortic ventricular septal defect and concomitantly of spiraliform great arteries in the asplenia group of heterotaxy anomalies, as detected by this study, further substantiates our belief of not mixing collectively the ADs with the DORV in clinico-pathological diagnosis.