Keleş Selek İ., Canbay Sarılar Ç., Akdoğan B., Bulut S., Ercan C. C., Çiçek M. S.
JTCVS Structural and Endovascular, cilt.8, sa.1, ss.1, 2025 (Hakemli Dergi)
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Yayın Türü:
Makale / Vaka Takdimi
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Cilt numarası:
8
Sayı:
1
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Basım Tarihi:
2025
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Dergi Adı:
JTCVS Structural and Endovascular
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Sayfa Sayıları:
ss.1
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İstanbul Üniversitesi Adresli:
Evet
Özet
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.