Discussion

This case illustrates a severe and rare presentation of double aortic arch and laryngotracheomalacia culminating in sudden cardiac arrest and profound neurological injury. The presence of two aortic arches encircling the trachea and oesophagus can result in variable degrees of compression.

A double aortic arch refers to the presence of two aortic arches (left and right) that encircle the trachea and oesophagus, forming a complete vascular ring. ^{Reference Backer and Mavroudis1} This can lead to various amounts of compression of the trachea, oesophagus, or both structures. ^{Reference Worhunsky, Levy and Stephens2} Double aortic arch can present as right-dominant, left-dominant, balanced, or codominant arches. ^{Reference Backer and Mavroudis1} Although vascular rings can result from several other congenital aortic arch anomalies (including right aortic arch with left ligamentum, innominate artery compression, and pulmonary artery sling), a ring with double aortic arch anatomy usually presents with more severe symptoms. ^{Reference Hernanz-Schulman3,Reference Backer, Mavroudis, Rigsby and Holinger4} Symptoms range from stridor and dysphagia to recurrent infections and respiratory distress. ^{Reference Hernanz-Schulman3,Reference Backer, Mavroudis, Rigsby and Holinger4} Advances in prenatal imaging have increased early detection rates, allowing for timely intervention. ^{Reference Evans, Acherman and Ciccolo5–Reference Stephens, Eltayeb and Kennedy7}

Despite this, there remains controversy regarding the timing of surgical correction, especially in asymptomatic or mildly symptomatic infants. Some literature supports early surgical intervention regardless of symptoms, citing low perioperative morbidity even in asymptomatic patients. ^{Reference Ajdaa, Carbonez and Hubrechts8} Worhunsky et al. propose a management algorithm based on symptomatology and imaging findings, recommending CT angiography by 2–4 months of age, with surgery indicated for significant tracheal compression. ^{Reference Worhunsky, Levy and Stephens2} In contrast, others advocate for a more conservative approach, particularly when symptoms are mild or absent, allowing time for potential spontaneous resolution. ^{Reference Hamad, Sawahreh and E’mar9}

However, this case challenges conservative strategies by highlighting a devastating outcome from delayed intervention. The patient, though clinically stable, had imaging-proven tracheal and oesophageal compression and comorbid laryngotracheomalacia—factors which significantly increased her risk. Sudden aspiration led to cardiopulmonary collapse, demonstrating the unpredictable and potentially fatal course in such cases. This case raises concern that waiting for clinical symptoms, ^{Reference Worhunsky, Levy and Stephens2} as suggested in more conservative algorithms or allowing the double aortic arch and laryngomalacia to self-resolve, ^{Reference Hamad, Sawahreh and E’mar9} may delay necessary intervention and increase the risk of life-threatening complications. Given the potential tracheal and oesophageal compression even in the absence of symptoms, we advocate for early imaging with CT angiography in all patients with suspected double aortic arch.

In conclusion, early diagnosis and prompt intervention are critical in the management of double aortic arch. While some patients may appear asymptomatic, structural compression from a vascular ring—particularly when coupled with airway anomalies—poses significant risk. Advanced imaging with CT angiography is essential for evaluating the extent of compression. If any tracheal, oesophageal, or laryngeal compromise is evident, surgical repair should be pursued urgently to prevent catastrophic complications such as cardiac arrest and hypoxic-ischaemic injury.