Sudden Death in Childhood Cardiomyopathy Results From a Long-Term National Population-Based Study

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

卷:65

Article

Bharucha, Tara¹  Lee, Katherine J.²  Daubeney, Piers E. F.^3,4  Nugent, Alan W.⁵  Turner, Christian⁶  Sholler, Gary F.⁶  Robertson, Terry⁷  Justo, Robert⁸  Ramsay, Jim⁹  Carlin, John B.²  Colan, Steven D.¹⁰  King, Ingrid²  Weintraub, Robert G.^2,11,12,13  Davis, Andrew M.^2,11,12,13 

[1] Univ Hosp Southampton NHS Fdn Trust, Southampton, Hants, England

[2] Murdoch Childrens Res Inst, Parkville, Vic 3052, Australia

[3] Univ London Imperial Coll Sci Technol & Med, London, England

[4] Royal Brompton Hosp, London SW3 6LY, England

[5] Univ Texas SW Med Ctr Dallas, Dallas, TX 75390 USA

[6] Childrens Hosp Westmead, Sydney, NSW, Australia

[7] Womens & Childrens Hosp, Adelaide, SA, Australia

[8] Univ Queensland, Mater Childrens Hosp, Brisbane, Qld 4101, Australia

[9] Princess Margaret Hosp Children, Perth, WA, Australia

[10] Harvard Univ, Sch Med, Boston Childrens Hosp, Boston, MA USA

[11] Harvard Univ, Sch Med, Dept Pediat, Boston, MA 02115 USA

[12] Royal Childrens Hosp, Melbourne, Vic, Australia

[13] Univ Melbourne, Dept Paediat, Melbourne, Vic, Australia

关键词: cardiomyopathy;    epidemiology;    pediatrics;    sudden death;   

DOI  :  10.1016/j.jacc.2015.03.552

来源: Elsevier

【 摘 要 】

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. (C) 2015 by the American College of Cardiology Foundation.

【 授权许可】

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