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

Tara Bharucha, Katherine J. Lee, Piers E F Daubeney, Alan W. Nugent, Christian Turner, Gary F. Sholler, Terry Robertson, Robert Justo, Jim Ramsay, John B. Carlin, Steven D. Colan, Ingrid King, Robert G. Weintraub, Andrew M. Davis

Research output: Contribution to journalArticlepeer-review

96 Scopus citations


Background Children with cardiomyopathy (CM) are at risk of sudden cardiac death (SCD), but the incidence and risk factors for this outcome are not clear. Objectives This study sought to determine the incidence and risk factors for SCD in children with varying CM phenotypes from a long-term population-based study of childhood CM. Methods The NACCS (National Australian Childhood Cardiomyopathy Study) is an ongoing longitudinal cohort study including all children in Australia with primary CM who were diagnosed between January 1, 1987, and December 31, 1996, and were <10 years of age. The cumulative incidence and risk factors for SCD within individual CM phenotypes were explored using survival analysis. Results Of 289 eligible patients, 16 (5.5%) experienced SCD over a median follow-up of 11.9 years (interquartile range: 1.7 to 15.4). The risk of SCD varied according to CM phenotype (p = 0.007). The cumulative incidence of SCD at 15 years was 5% for dilated cardiomyopathy (DCM), 6% for hypertrophic cardiomyopathy (HCM), 12% for restrictive cardiomyopathy, and 23% for left ventricular (LV) noncompaction. Older age at diagnosis, positive family history of CM, and severity of LV dysfunction were related to increased risk of SCD in patients with DCM, and a higher posterior wall thickness Z-score was the sole risk factor identified for patients with HCM. Conclusions Predictors of SCD include CM phenotype, family history of CM (DCM), severity of systolic dysfunction (DCM), and extent of LV hypertrophy (HCM). Continuing follow-up of this cohort into adulthood is likely to reveal an ongoing risk of SCD.

Original languageEnglish (US)
Pages (from-to)2302-2310
Number of pages9
JournalJournal of the American College of Cardiology
Issue number21
StatePublished - Jun 2 2015


  • cardiomyopathy
  • epidemiology
  • pediatrics
  • sudden death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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