Transverse aortic arch hypoplasia involving some or all segments of the arch (tubular hypoplasia) may exist in association with intra-cardiac anomalies of varying severity. Surgical repair of the distal transverse aortic arch and isthmus are adequately managed by an extended end-to-end coarctation repair in most infants via a left thoracotomy. The surgical management and timing of proximal aortic arch obstruction is controversial but almost always requires an approach via sternotomy using cardiopulmonary bypass.