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Dual-Dye Optical Mapping of Hearts from RyR2R2474S Knock-In Mice of Catecholaminergic Polymorphic Ventricular Tachycardia
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Catecholaminergic polymorphic ventricular tachycardia in children-incidence and trends in detection, presentation and

Nicholas Fitzgerald1, Claire Lawley2,3, Ansley Morrish4

  • 1The Heart Centre for Children, The Sydney Children's Hospitals Network, Sydney, New South Wales, Australia.

Archives of Disease in Childhood
|June 25, 2025
PubMed

Insights

The birth rate for catecholaminergic polymorphic ventricular tachycardia (CPVT) diagnosed in childhood is approximately 1 in 65,000 live births. Recurrent cardiac events in CPVT patients were linked to poor adherence and beta-blocker monotherapy.

Area of Science:

  • Pediatric Cardiology
  • Genetics
  • Epidemiology

Background:

  • Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare, inherited arrhythmia.
  • Early diagnosis and management are crucial for preventing life-threatening cardiac events in children.

Purpose of the Study:

  • To determine the birth rate of childhood-diagnosed CPVT.
  • To analyze trends in CPVT presentation and management in pediatric patients.
  • To identify factors associated with adverse outcomes in CPVT.

Main Methods:

  • Retrospective cohort study of children diagnosed with CPVT (0-16 years) in New South Wales, Australia (2002-2021).
  • Clinical data extraction and analysis for trends in diagnosis and management.
  • Calculation of CPVT birth rate using birth year data.

Main Results:

  • 32 children diagnosed with CPVT; 88% presented with symptoms, including cardiac arrest (62.5%).
  • Genetic variants identified in 78% of cases.
  • The birth rate of CPVT in childhood was 1 in 65,000 live births in NSW.
  • 10% of patients experienced symptomatic cardiac events during follow-up, often linked to suboptimal adherence or beta-blocker monotherapy.

Conclusions:

  • The incidence of CPVT diagnosed in childhood is approximately 1 in 65,000 live births.
  • Suboptimal adherence and beta-blocker monotherapy (without flecainide) were associated with recurrent cardiac events in pediatric CPVT patients.
Abstract

Keywords:
CardiologyGeneticsPaediatrics

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