title: Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy.
abstract: OBJECTIVE
  Hypertrophic cardiomyopathy (HCM) is the commonest inherited cause of sudden cardiac death in children; current guidelines suggest HCM screening after 12-15 years of age. The study aims to establish the age range at highest risk.
  
  
  RESULTS
  Cohort study from six regional centres of paediatric cardiology, including children presenting with sudden death; n = 150 (59% = male; 39% familial HCM). Age- and gender-specific mortality was calculated, and compared with rates calculated from the Swedish National Cause of Death Registry. There were 56 deaths within the cohort, 39 were sudden arrhythmia deaths, with 31 at <19 years of age. Between 9-13.9 years of age annual sudden death mortality averages 7.2%, vs. 1.7% after 16 years of age; P = 0.025, odds ratio for proportions 3.75 [95% confidence intervals (CI) 1.18-11.91], similar in both familial and idiopathic HCM. The risk for sudden death peaks earlier in girls (10-11 years), with male preponderance after the age of 15. National cause of death statistics confirm that the mortality rate from HCM is significantly higher in the 8-16 year olds (0.112 per 100,000 age-specific population) than in the 17-30 year olds (0.055 per 100,000; 95% CI 0.011-0.099).
  
  
  CONCLUSIONS
  In families with HCM, children should be screened at an early age.
authors.affiliation: Division of Paediatrics, Department of Clinical Sciences, Sahlgren Academy, Queen Silvia Childreńs Hospital SE-416 85, Gothenburg, Sweden. ingegerd.ostman-smith@pediat.gu.se
authors.forename: Ingegerd
authors.initials: I
authors.lastname: Ostman-Smith
authors.forename: Göran
authors.initials: G
authors.lastname: Wettrell
authors.forename: Barry
authors.initials: B
authors.lastname: Keeton
authors.forename: Daniel
authors.initials: D
authors.lastname: Holmgren
authors.forename: Ulf
authors.initials: U
authors.lastname: Ergander
authors.forename: Steven
authors.initials: S
authors.lastname: Gould
authors.forename: Colene
authors.initials: C
authors.lastname: Bowker
authors.forename: Mario
authors.initials: M
authors.lastname: Verdicchio
mesh_terms: D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D017677:Age Distribution; D000367:Age Factors; D000728:Androgens / Q000378:metabolism; D002312:Cardiomyopathy, Hypertrophic / Q000188:drug therapy / Q000401:mortality*; D002648:Child; D015331:Cohort Studies; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000209:etiology*; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D017410:Practice Guidelines as Topic; D018570:Risk Assessment; D012737:Sex Factors; D014694:Ventricular Outflow Obstruction / Q000188:drug therapy / Q000401:mortality*
chemical_list: D000319:Adrenergic beta-Antagonists; D000728:Androgens