The persistent fifth aortic arch is rare vascular anomaly. To our knowledge, this is the 24th reported case of the persistent fifth aortic arch. This patient was a 31 days old male infant and had the persistent fifth aortic arch associated with atresia of the fourth aortic arch, patent ductus arteriosus, a double-outlet right ventricle, and a mesocardia. He underwent a fifth aortic arch division and an extended aortic arch anastomosis with a division of ductus arteriosus. There was no blood pressure gradient between upper and lower limbs after the repair. However, no weaning from a cardiopulmonary bypass after the subsequent radical operation for double-outlet right ventricle caused his death.
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