Circumflex aorta in children: Diagnostic challenges, contemporary surgical management, and early outcomes from a single-center experience

Background: Circumflex aorta is a rare congenital vascular anomaly in which the aortic arch passes posterior to the trachea and esophagus, crosses the midline, and continues as the descending thoracic aorta on the opposite side. This configuration frequently results in significant airway and esophageal compression and is often underrecognized. Methods: We describe a single-center experience of eight pediatric patients diagnosed with circumflex aorta who presented predominantly with respiratory symptoms. All patients underwent detailed preoperative imaging, including computed tomography angiography. Surgical management consisted of aortic uncrossing, with concomitant intracardiac repair performed when indicated. Results: All patients experienced effective relief of airway and esophageal compression following the aortic uncrossing procedure. Postoperative morbidity included transient recurrent laryngeal nerve palsy, tracheobronchomalacia, pulmonary hypertensive crises in patients with underlying cardiac anomalies, prolonged mechanical ventilation, and feeding difficulties. There was no operative mortality. At mid-term follow-up, all patients demonstrated significant clinical improvement, with resolution of respiratory symptoms and satisfactory growth. Conclusion: Circumflex aorta should be considered in infants and children with persistent or disproportionate respiratory symptoms, particularly when intracardiac findings do not adequately explain the clinical presentation. Early diagnosis and surgical correction using the aortic uncrossing technique yields excellent anatomical and clinical outcomes, although prolonged airway support may be required in selected cases.