Modified total arch replacement with the frozen elephant trunk technique for a right-sided aortic arch and aortic diverticulum in a teenager.

BACKGROUND: A right sided aortic arch (RAA) and an aortic diverticulum (AD) are a rare congenital anomaly associated with an aortic rupture and dissection. Recently, various methods for the surgical management have been described to treat RAAs and ADs.

CASE REPORT: We describe a case of an RAA with a mirror image branching (RAMI) and AD in a teenager who complained of chest pain. We planned a total arch replacement with the frozen elephant trunk technique. Further, we needed to devise a way to reconnect the cerebral vessels because of his small arch anatomy and limited space in the mediastinum. We performed a right subclavian artery debranching, creating an anastomosis using one graft and diamond anastomosis, and performed the left innominate and right carotid anastomosis in an island fashion. This surgical approach could be a treatment option for young or small patients with RAMIs and ADs, whose arch anatomy is very small.