Congenital long QT syndrome in a nationwide paediatric cohort was associated with a 3.8% rate of major arrhythmic events over 6 years, predominantly in those with high-risk genotypes or QTc ≥550 ms.
Cohort (n=371)
Yes
In a nationwide paediatric LQTS cohort, major arrhythmic events were rare (3.8% over 6 years) and primarily occurred in children with malignant genotypes, markedly prolonged QTc, or very early presentation.
BACKGROUND AND AIMS: Congenital long QT syndrome (LQTS) is a heterogeneous disorder in which genotype and QTc duration modulate the risk of major arrhythmic events (MAEs), but contemporary paediatric outcome data remain limited. This study aimed to characterize clinical features, management strategies, and predictors of MAEs in a nationwide paediatric LQTS cohort. METHODS: This retrospective multicentre study analysed children (80% arrhythmia-free survival without major complications. CONCLUSIONS: In this nationwide paediatric LQTS cohort, MAEs were uncommon and clustered in children with malignant genotypes, markedly prolonged QTc and very early presentation, particularly foetal or neonatal bradycardia. These data support the current genotype and QTc-guided management strategy, with beta-blockers as the cornerstone therapy and selective use of left cardiac sympathetic denervation and ICDs in high-risk profiles.
Perín et al. (2026) conducted a cohort in Congenital long QT syndrome (LQTS) (n=371). Congenital long QT syndrome was evaluated on Major arrhythmic events (sudden cardiac death, aborted cardiac arrest, or appropriate ICD therapy). Congenital long QT syndrome in a nationwide paediatric cohort was associated with a 3.8% rate of major arrhythmic events over 6 years, predominantly in those with high-risk genotypes or QTc ≥550 ms.