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July 18, 2026European Heart JournalOpen Access

Pediatric LQTS shows a 3.8% 6-year major arrhythmic event rate, predominantly with QTc ≥550 ms.

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Population

371 children diagnosed with congenital long QT syndrome across 30 tertiary centres, median diagnosis age 6.0…

Design

Cohort

Follow-up

median 6-year

Key result

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.

Authors

FPFrancesca PerínACAntonio J. CartónFBFrancisco Bermúdez-Jiménez

Discussion

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Overview

Supports genotype- and QTc-guided risk stratification in paediatric LQTS; leaves open prospective trials to refine therapies.

Key Points

  • To characterize clinical features, management strategies, and predictors of major arrhythmic events in a nationwide cohort of paediatric long QT syndrome.
  • Retrospective multicentre analysis of children diagnosed with long QT syndrome across 30 tertiary centres.
  • Used 2022 European Society of Cardiology criteria for disease classification.
  • Followed up over a median of 6 years with MAEs defined as sudden cardiac death, aborted cardiac arrest, or appropriate ICD therapy.
  • Among 371 children, 14 (3.8%) experienced major arrhythmic events, predominantly those with high-risk genotypes and QTc ≥550 ms.
  • Beta-blockers were prescribed to 92.4% of the cohort; left cardiac sympathetic denervation showed >80% arrhythmia-free survival without major complications.
  • ICDs were implanted in 33 children (8.9%), with 10 (30%) receiving appropriate therapies and 8 (24%) experiencing complications.

Study Design

Type

Cohort (n=371)

Multicenter

Yes

Structured PICO

P
Population
371 children (<18 years) diagnosed with congenital long QT syndrome across 30 Spanish tertiary centres, followed for a median of 6 years.
E
Exposure
Guideline-directed management including beta-blockers (92.4%), implantable cardioverter-defibrillators (8.9%), and left cardiac sympathetic denervation (8.9%)
O
Outcome
Major arrhythmic events (MAEs) defined as sudden cardiac death, aborted cardiac arrest, or appropriate implantable cardioverter-defibrillator (ICD) therapycomposite

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.

Cite This Study

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.

synapsesocial.com/papers/6a5b39288167787360d24f5dhttps://doi.org/10.1093/eurheartj/ehag513
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