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Sudden death in childhood cardiomyopathy: results from a long-term national population-based study

Tara Bharucha1, Katherine J Lee2, Piers E F Daubeney3

  • 1University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom.

Journal of the American College of Cardiology
|May 30, 2015
PubMed

Insights

Sudden cardiac death (SCD) risk varies in children with cardiomyopathy (CM). Phenotype, family history, and LV dysfunction severity are key predictors, highlighting the need for ongoing monitoring.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Research
  • Genetics of Heart Disease

Background:

  • Children with cardiomyopathy (CM) face a significant risk of sudden cardiac death (SCD).
  • The precise incidence and contributing risk factors for SCD in pediatric CM remain incompletely understood.
  • Understanding these factors is crucial for risk stratification and management.

Purpose of the Study:

  • To determine the incidence of SCD in children with various cardiomyopathy phenotypes.
  • To identify specific risk factors associated with SCD across different CM types.
  • To leverage data from a long-term population-based study of childhood CM.

Main Methods:

  • Utilized data from the National Australian Childhood Cardiomyopathy Study (NACCS), a longitudinal cohort.
  • Included children diagnosed with primary CM between 1987 and 1996, under 10 years of age.
  • Employed survival analysis to explore cumulative incidence and risk factors for SCD.

Main Results:

  • Out of 289 patients, 16 (5.5%) experienced SCD over a median follow-up of 11.9 years.
  • SCD risk significantly varied by CM phenotype (p=0.007).
  • Cumulative 15-year SCD incidence: 5% (DCM), 6% (HCM), 12% (restrictive CM), 23% (LV noncompaction). Risk factors included age, family history, LV dysfunction (DCM), and posterior wall thickness Z-score (HCM).

Conclusions:

  • Cardiomyopathy phenotype, family history, LV systolic dysfunction severity, and LV hypertrophy extent are key predictors of SCD.
  • The identified risk factors provide targets for early intervention and risk stratification.
  • Continued follow-up into adulthood is essential, as SCD risk may persist.
Abstract

Keywords:
cardiomyopathyepidemiologypediatricssudden death

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