Clinical Characteristics and Outcomes of Pediatric Patients With Long QT Syndrome: A Korean Multicenter Cohort Study
Abstract
This multicenter nationwide cohort study comprehensively analyzed the clinical features, genetic architecture, and outcomes of Korean pediatric patients with long QT syndrome (LQTS).Prolonged QT intervals, fetal arrhythmia, and LQT2/3 genotypes compared to LQT1 were independent risk factors for life-threatening arrhythmic events.Notably, S6-segment mutations in LQT1 patients were associated with a poor prognosis, consistent with Western cohorts, while the impact of other transmembrane variants varied.Beta-blocker therapyparticularly nadolol or propranolol-was found to be protective against arrhythmic events.These findings highlight the importance of genotype-based management and beta-blocker therapy to improve prognosis in pediatric LQTS.
// Source
Authors: Mi Kyoung Song, So Yon Jun, Junghye Kwon, Ji‐Eun Ban, Hye Won Kwon, Young Hye Ryu, Seung Min Baek, Ah Young Kim, Mi Jin Kim, Jae Suk Baek, Ja Kyoung Yoon, Hee Joung Choi, Min-Jung Cho, Chang Sin Kim, Insu Choi, Jihye You, Joung‐Hee Byun, Heirim Lee, Jae Yoon Na, Jue Seong Lee, Y.-H. Cho, Joowon Lee, Lucy Youngmin Eun, Young Tae Lim, June Huh, Eun Jung Bae
Institutions: Chonnam National University Hospital, Pusan National University Yangsan Hospital, Yonsei University, Ulsan College, Seoul National University Bundang Hospital, Samsung Medical Center, University of Ulsan, Asan Medical Center, Keimyung University, Kyungpook National University, Korea University, Gyeongsang National University, Gangnam Severance Hospital, Pusan National University Hospital, Hanyang University, Chosun University, Jeonbuk National University, Seoul National University Children's Hospital, Sejong General Hospital, Changwon National University, Gyeongsang National University Changwon Hospital