JCK-AP session 8 (III-JCKAP8)
ACHD

[III-JCKAP8-5] Long-term Prognosis of Isolated Congenitally Corrected Transposition of the Great Arteries: Insights from a Korean Multicenter Registry

^○Hyun Chung¹, Eung Re Kim², Ji Eun Ban¹, Joowon Lee³, Shinhyeung Kwak⁴, I-Seok Kang⁴, Jinyoung Song⁴, So Ick Jang¹ (1.The Department of Pediatrics, Bucheon Sejong Hospital, Bucheon 2.The Department of Cardiovascular and Thoracic Surgery, Bucheon Sejong Hospital, Bucheon 3.The Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam 4.The Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul)

キーワード：isolated ccTGA, ccTGA with intact ventricular septum, long-term outcomes

Objectives:Congenitally corrected transposition of the great arteries (ccTGA) is a conotruncal anomaly with unique characteristics. Particularly, isolated ccTGA is usually asymptomatic in childhood, leading to delayed diagnosis into adulthood and limited understanding of the disease. This study aimed to assess the long-term outcomes of patients with isolated ccTGA using data from the newly established Korean registry of conotruncal anomalies.Methods:Patients with ccTGA followed between January 1, 2000, and December 31, 2015, across 11 institutes in Korea were enrolled in the registry. Clinical data of patients with isolated ccTGA were extracted for analysis, including demographic characteristics and cumulative incidence of relevant outcomes.Results:Among 126 patients, 67 (53%) were male, with a median age of diagnosis of 18.7 years and a median follow-up duration of 13 years. Sixty five surgeries were performed in 42 patients (33%), with tricuspid valve replacement being the most common procedure. Anatomic repair was conducted in 5 patients (4%). Cumulative incidences of specific outcomes revealed that significant tricuspid regurgitation developed in 53.5% of patients by age 50, complete atrioventricular block in 30.4% by age 70, and systemic ventricular dysfunction in 27% by age 50.Conclusions:This study, based on a large population of patients with ccTGA, provides the first report on the long-term outcomes of isolated ccTGA in Korea. The late outcomes were relatively favorable compared to previous studies, offering valuable insights for physicians managing these patients.