Undiagnosed interrupted aortic arch presenting with acute aortic dissection in a teenager: A rare case and successful repair

Acute aortic dissection in the pediatric population is an extremely rare but life-threatening condition.The estimated incidence is less than 0.5 per million children annually. 1ost reported cases are associated with genetic aortopathies, including Marfan syndrome, Loeys-Dietz syndrome, and Ehlers-Danlos syndrome, although even in these cohorts, dissection during childhood or adolescence is uncommon.Interrupted aortic arch (IAA) is a rare congenital cardiac anomaly that represents approximately 1% of all congenital heart defects, with an incidence of about 3 per 1,000,000 live births. 2 If left untreated, IAA is associated with 90% mortality by the first week of life, attributable to impaired systemic perfusion.To our knowledge, no previously published report describes a patient who survived into adolescence without repair of a type A IAA and subsequently presented with an acute type A dissection.The current case provides an opportunity to highlight not only the exceptional nature of this clinical presentation but also the surgical strategy used for successful management-including extra-anatomic aortic reconstruction and preservation of the native aortic valve.