Arrhythmias & electrophysiologyCohort study
Paediatric long QT syndrome: clinical outcomes and therapy in the Spanish National Registry
- Retrospective cohort study
- multicentre
- nationwide registry
- 30 tertiary centres
Authors' conclusion
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.
Study context
- Population
- Children (<18 years) with long QT syndrome (LQTS) (2022 ESC criteria), Spain, 2004–2458% male
- Participants
- 371 children
- Outcome
- MAEs (SCD, aborted cardiac arrest or appropriate ICD therapy)
- Follow-up
- median 6-year follow-up
Main finding
As reported in the abstract
MAEs
Children with major arrhythmic events (MAEs)
14 (3.8%)
Observational data: associations, not effects.
Further findings
Genetics and therapy
- Pathogenic/likely pathogenic variants (mainly KCNQ1, KCNH2, SCN5A)
- 86.5%
- High-risk genotypes (Jervell-Lange-Nielsen, calmodulinopathies, CACNA1C/Timothy)
- 5.7%
- Beta-blockers prescribed
- 92.4%
Foetal/neonatal bradycardia
- Foetal/neonatal bradycardia, correlated with high-risk genotypes and adverse outcomes
- 5.1%
Implantable cardioverter-defibrillator (ICD)
- ICD implanted
- 33 (8.9%)
- Appropriate ICD therapies
- 10 (30%)
Left cardiac sympathetic denervation
- Performed, mainly high-risk patients
- 33 (8.9%)
- Arrhythmia-free survival, without major complications
- >80%
Safety
ICD complications
- ICD recipients with complications
- 8 (24%)
Original publication
Paediatric long QT syndrome: clinical outcomes and therapy in the Spanish National Registry. Eur Heart J. Published 17 July 2026. doi:10.1093/eurheartj/ehag513
Summarised from the published abstract; every number is checked against it