European Heart Journal Advance Access originally published online on April 2, 2008
European Heart Journal 2008 29(9):1160-1167; doi:10.1093/eurheartj/ehn122
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Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy
1 Division of Paediatrics, Department of Clinical Sciences, Sahlgren Academy, Queen Silvia Childre
s Hospital SE-416 85, Gothenburg, Sweden
2 Division of Paediatric Cardiology, University Hospital, Lund, Sweden
3 Wessex Cardiothoracic Centre, Southampton General Hospital, Southampton, UK
4 Division of Paediatric Cardiology, Astrid Lindgren Childrens Hospital, Stockholm, Sweden
5 Department of Paediatric Pathology, John Radcliffe Hospital, Oxford, UK
6 Institute of Forensic Medicine, Gothenburg, Sweden
Received 28 June 2007; revised 5 February 2008; accepted 29 February 2008; online publish-ahead-of-print 2 April 2008.
* Corresponding author. Tel: +46 31 3434512, Fax: +46 31 3435947, Email: ingegerd.ostman-smith{at}pediat.gu.se
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.
Key Words: Hypertrophic cardiomyopathy • Sudden death • Mortality • Screening • Familial hypertrophic cardiomyopathy • Gender