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Background  Vascular rings are rare congenital abnormalities of the aortic arch. There are many embryological variants including a double aortic arch. In symptomatic children, division of ring and release of airway structures may be sufficient. Persistence of symptoms can be related to an anterior angle formed between the two arches. The aim of this study is to evaluate the clinical efficacy in improving symptoms and on changing this angle at the primary surgery.    Methods  All children who had surgery for double aortic arch between 2005 and 2020, were studied. Relevant factors were analyzed for persistent symptoms including anatomical substrates and surgical details.    Results  A total of 87 out of 224 children had surgery for a double aortic arch. At presentation, airway symptoms ( n  = 74/87) were more common than esophageal symptoms ( n  = 27/87). Early onset symptoms within 1 year were seen in 49 children. In addition to division of one arch, surgical steps also included realigning the anterior left arch, thereby eliminating the acute angle in 36 children (after 2014). After surgery, symptom relief within 12 months following surgery was seen in 64% of children (56 out of 87) but in 27 out of 36 children (75%) with additional surgical modification, as against 29 out of 51 (57%) in those with division of the arch. Symptoms persisted beyond 1 year needing reintervention in eight children.    Conclusion  Anterior arch angulation plays an important role in double aortic arch by causing a “nutcracker” phenomenon. Repair in double aortic arch should consider this aspect and include modification of surgical steps by realigning the corresponding aortic arch branches and an anterior pexy in selected cases.

Importance of Acute Anterior Angulation in Double Aortic Arch Needing Attention at Primary Surgery