A full-term neonate was transferred to our facility after a postnatal diagnosis of dextro (D)-transposition and a ventricular septal defect (VSD). Initial transthoracic echocardiogram revealed atrial situs solitus (S), D-looped ventricles, ventriculo-arterial discordance, and levo-malposition of the great arteries. A single coronary artery was found to be originating from the rightward and anterior sinus of the aortic root. Therefore, we discussed our operative plan as a multidisciplinary team given the atypical anatomy and ultimately decided upon a full repair with an arterial switch operation and VSD closure. We describe our operative technique employed to tackle this uncommon anatomy.
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