Double-outlet right atrium (DORA) associated with dextro-transposition of the great arteries with intact ventricular septum (d-TGA-IVS): an unusual atrioventricular junction variant.

Dextro-transposition of the great arteries with intact ventricular septum (d-TGA-IVS) typically requires neonatal repair, as regression of left ventricular (LV) mass and compliance may limit the feasibility of delayed anatomical correction. Survival beyond the neonatal period without intervention is uncommon and usually reflects additional shunt lesions or atypical intracardiac physiology. Double-outlet right atrium (DORA) is a rare atrioventricular (AV) junction malalignment in which the right atrium (RA) drains into both ventricles through separate AV valve orifices, resulting in abnormal atrial-level streaming. We report a 40-day-old infant with d-TGA-IVS who presented with heart failure and intraoperative findings revealed a complex AV junction anatomy with three distinct AV valve orifices consistent with DORA physiology. A tailored biventricular repair consisting of arterial switch operation (ASO) and atrial-level reconstruction was performed to preserve unobstructed LV inflow in the presence of a borderline mitral valve (MV). At mid-term follow-up, ventricular function was preserved, LV inflow remained unobstructed, and interval growth of the native MV was observed. This case highlights the importance of recognizing atypical AV junction anatomy and flow-dependent physiology in d-TGA-IVS, as these factors may influence clinical presentation and surgical strategy.