Coarctation of the aorta (CoA) complicates with right aortic arch (RAA) is very rare, and its surgical treatment in the neonatal period is extremely uncommon. We performed surgical repair for a 27-day-old boy given a diagnosis of double outlet right ventricle (DORV) and CoA with RAA. The procedures consisted of an arterial switch, intra-ventricular re-routing, aortic arch reconstruction using an equine-pericardial roll and right ventricular outflow reconstruction (RVOTR) with autologous pericardium. We performed re-RVOTR 41 days after the operation because the autologous pericardium used for RVOTR showed aneurysmal dilatation. After the second operation, this patient has done well.
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