TY - JOUR
T1 - Sudden death in childhood cardiomyopathy
T2 - Results from a long-term national population-based study
AU - Bharucha, Tara
AU - Lee, Katherine J.
AU - Daubeney, Piers E.F.
AU - Nugent, Alan W.
AU - Turner, Christian
AU - Sholler, Gary F.
AU - Robertson, Terry
AU - Justo, Robert
AU - Ramsay, Jim
AU - Carlin, John B.
AU - Colan, Steven D.
AU - King, Ingrid
AU - Weintraub, Robert G.
AU - Davis, Andrew M.
N1 - Funding Information:
This study was supported by the Royal Children’s Hospital Research Foundation (grant 98001 ), the National Heart Foundation of Australia (grants G 98M 0159, G 04M 1586, G 05M 2151, and G 07M 3180 ), the Australia and New Zealand Children’s Heart Research Centre (NACCS grant), and Heartkids Australia (NACCS grant). Dr. Daubeney is supported by the Biomedical Research Unit at the Royal Brompton Hospital. The Department of Cardiology, Royal Children's Hospital, receives fellowship support from Medtronic and St. Jude Medical. Dr. Weintraub has served on an advisory board for Actelion Pharmaceuticals. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Publisher Copyright:
© 2015 American College of Cardiology Foundation Published By Elsevier Inc.
PY - 2015/6/2
Y1 - 2015/6/2
N2 - 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.
AB - 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.
KW - cardiomyopathy
KW - epidemiology
KW - pediatrics
KW - sudden death
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U2 - 10.1016/j.jacc.2015.03.552
DO - 10.1016/j.jacc.2015.03.552
M3 - Article
C2 - 26022819
AN - SCOPUS:84930021311
VL - 65
SP - 2302
EP - 2310
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
SN - 0735-1097
IS - 21
ER -