A right-sided circumflex aortic arch (as defined by its bow-like curvature) is an extremely rare defect with only a few cases reported in the literatureReference Song, Kim and Kim¹ to date. We report a case of a newborn who presented with respiratory distress and poor lower extremity pulses described by a preliminary echocardiogram to have a right-sided aortic arch with a hypoplastic transverse segment and a ventricular septal defect. A subsequent computed tomogram demonstrated a right ascending aorta (Fig 1) connected to a very hypoplastic transverse aorta (Arch), which was retro-oesophageal in location (Figs 2 and 3). An aberrant left subclavian artery arose from the distal end of the transverse arch (Fig 3: small arrow) and finally descended on the left side of the vertebral column (Fig 3: asterisk). A three-dimensional volume-rendered reconstruction (Fig 4) was also of great benefit to our cardiothoracic surgery team which formulated a novel surgical approach to this particular defect using an extended end-to-side repair with arch augmentation without the need of a conduit.

Figure 1

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Figure 4