title: Sudden death in childhood cardiomyopathy: results from a long-term national population-based study.
abstract: 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.
  
  
  OBJECTIVE
  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.
authors.affiliation: University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom.
authors.forename: Tara
authors.initials: T
authors.lastname: Bharucha
authors.affiliation: Murdoch Children's Research Institute, Parkville, Victoria, Australia.
authors.forename: Katherine J
authors.initials: KJ
authors.lastname: Lee
authors.affiliation: Imperial College and Royal Brompton Hospital, London, United Kingdom.
authors.forename: Piers E F
authors.initials: PE
authors.lastname: Daubeney
authors.affiliation: University of Texas Southwestern Medical Center, Dallas, Texas.
authors.forename: Alan W
authors.initials: AW
authors.lastname: Nugent
authors.affiliation: Children's Hospital at Westmead, Sydney, Australia.
authors.forename: Christian
authors.initials: C
authors.lastname: Turner
authors.affiliation: Children's Hospital at Westmead, Sydney, Australia.
authors.forename: Gary F
authors.initials: GF
authors.lastname: Sholler
authors.affiliation: Women's and Children's Hospital, Adelaide, Australia.
authors.forename: Terry
authors.initials: T
authors.lastname: Robertson
authors.affiliation: Mater Children's Hospital, Brisbane, Australia.
authors.forename: Robert
authors.initials: R
authors.lastname: Justo
authors.affiliation: Princess Margaret Hospital for Children, Perth, Australia.
authors.forename: Jim
authors.initials: J
authors.lastname: Ramsay
authors.affiliation: Murdoch Children's Research Institute, Parkville, Victoria, Australia.
authors.forename: John B
authors.initials: JB
authors.lastname: Carlin
authors.affiliation: Boston Children's Hospital and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
authors.forename: Steven D
authors.initials: SD
authors.lastname: Colan
authors.affiliation: Murdoch Children's Research Institute, Parkville, Victoria, Australia.
authors.forename: Ingrid
authors.initials: I
authors.lastname: King
authors.affiliation: Murdoch Children's Research Institute, Parkville, Victoria, Australia; Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia.
authors.forename: Robert G
authors.initials: RG
authors.lastname: Weintraub
authors.affiliation: Murdoch Children's Research Institute, Parkville, Victoria, Australia; Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia. Electronic address: andrew.davis@rch.org.au.
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Davis
mesh_terms: D000293:Adolescent; D001315:Australia / Q000453:epidemiology; D009202:Cardiomyopathies / Q000401:mortality* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000453:epidemiology* / Q000209:etiology; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D012307:Risk Factors
keywords: cardiomyopathy; epidemiology; pediatrics; sudden death