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Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy

Östman-Smith, Ingegerd; Wettrell, Göran GWE LU ; Keeton, Barry; Holmgren, Daniel; Ergander, Ulf; Gould, Steven; Bowker, Colene and Verdicchio, Mario (2008) In European Heart Journal 29(9). p.1160-1167
Abstract
Aims 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. Methods and 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... (More)
Aims 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. Methods and 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). Conclusion In families with HCM, children should be screened at an early age. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
hypertrophic cardiomyopathy, mortality, gender, screening, familial hypertrophic cardiomyopathy, sudden death
in
European Heart Journal
volume
29
issue
9
pages
1160 - 1167
publisher
Oxford University Press
external identifiers
  • wos:000255523500015
  • scopus:45349098461
ISSN
1522-9645
DOI
10.1093/eurheartj/ehn122
language
English
LU publication?
yes
id
92af2c19-1b4f-41cf-b08b-84c796262b59 (old id 1204773)
date added to LUP
2008-09-18 09:59:00
date last changed
2017-11-05 04:02:36
@article{92af2c19-1b4f-41cf-b08b-84c796262b59,
  abstract     = {Aims 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. Methods and 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 &lt; 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). Conclusion In families with HCM, children should be screened at an early age.},
  author       = {Östman-Smith, Ingegerd and Wettrell, Göran GWE and Keeton, Barry and Holmgren, Daniel and Ergander, Ulf and Gould, Steven and Bowker, Colene and Verdicchio, Mario},
  issn         = {1522-9645},
  keyword      = {hypertrophic cardiomyopathy,mortality,gender,screening,familial hypertrophic cardiomyopathy,sudden death},
  language     = {eng},
  number       = {9},
  pages        = {1160--1167},
  publisher    = {Oxford University Press},
  series       = {European Heart Journal},
  title        = {Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy},
  url          = {http://dx.doi.org/10.1093/eurheartj/ehn122},
  volume       = {29},
  year         = {2008},
}