A right-sided circumflex retroesophageal aortic arch combined with a ventricular septal defect and severe coarctation is an extremely rare condition. Moreover, surgical correction is significant when performing an anatomical reconstruction of the left aortic arch by end-to-side and native tissue-to-tissue anastomosis instead of using a bypass graft. The authors describe an unusual case of a 1 month-old neonate with a ventricular septal defect and coarctation of a right-sided circumflex retroesophageal aortic arch who underwent successful surgical correction.
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