Undiagnosed interrupted aortic arch presenting with acute aortic dissection in a teenager: A rare case and successful repair
JTCVS Structural and Endovascular, vol.8, no.1, pp.1, 2025 (Peer-Reviewed Journal)
- Publication Type: Article / Case Report
- Volume: 8 Issue: 1
- Publication Date: 2025
- Journal Name: JTCVS Structural and Endovascular
- Page Numbers: pp.1
- Istanbul University Affiliated: Yes
Abstract
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.1 Most 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.