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

Bharucha, Tara, Lee, Katherine J., Daubeney, Piers E. F., Nugent, Alan W., Turner, Christian, Sholler, Gary F., Robertson, Terry, Justo, Robert, Ramsay, Jim, Carlin, John B., Colan, Steven D., King, Ingrid, Weintraub, Robert G. and Davis, Andrew M. (2015) Sudden death in childhood cardiomyopathy: Results from a long-term national population-based study. Journal of the American College of Cardiology, 65 21: 2302-2310. doi:10.1016/j.jacc.2015.03.552

Author Bharucha, Tara
Lee, Katherine J.
Daubeney, Piers E. F.
Nugent, Alan W.
Turner, Christian
Sholler, Gary F.
Robertson, Terry
Justo, Robert
Ramsay, Jim
Carlin, John B.
Colan, Steven D.
King, Ingrid
Weintraub, Robert G.
Davis, Andrew M.
Title Sudden death in childhood cardiomyopathy: Results from a long-term national population-based study
Journal name Journal of the American College of Cardiology   Check publisher's open access policy
ISSN 1558-3597
Publication date 2015-06-02
Sub-type Article (original research)
DOI 10.1016/j.jacc.2015.03.552
Open Access Status Not Open Access
Volume 65
Issue 21
Start page 2302
End page 2310
Total pages 9
Place of publication San Diego, CA United States
Publisher Elsevier
Collection year 2016
Language eng
Formatted abstract

Children with cardiomyopathy (CM) are at risk of sudden cardiac death (SCD), but the incidence and risk factors for this outcome are not clear.


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.


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.


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.


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.
Keyword Cardiomyopathy
Sudden death
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Non HERDC
School of Medicine Publications
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Citation counts: TR Web of Science Citation Count  Cited 7 times in Thomson Reuters Web of Science Article | Citations
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