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Published on: June 14, 2016
Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy
Ingegerd Ostman-Smith1, Göran Wettrell, Barry Keeton
1Division of Paediatrics, Department of Clinical Sciences, Sahlgren Academy, Queen Silvia Childreńs Hospital SE-416 85, Gothenburg, Sweden. ingegerd.ostman-smith@pediat.gu.se
Insights
Hypertrophic cardiomyopathy (HCM) poses the highest risk for sudden cardiac death in children aged 9-13.9 years, necessitating earlier screening than current guidelines recommend.
Area of Science:
- Cardiology
- Genetics
- Pediatrics
Background:
- Hypertrophic cardiomyopathy (HCM) is a leading inherited cause of sudden cardiac death in children.
- Current screening guidelines recommend HCM assessment starting at 12-15 years of age.
Purpose of the Study:
- To determine the specific age range at highest risk for sudden cardiac death in pediatric patients with HCM.
- To evaluate age- and gender-specific mortality rates in children with HCM.
Main Methods:
- A cohort study involving 150 children from six pediatric cardiology centers, including those who experienced sudden death.
- Analysis of age- and gender-specific mortality, compared with national death registry data.
- Calculation of sudden death mortality rates within specific age brackets.
Main Results:
- Sudden arrhythmia deaths occurred in 31 children under 19 years old.
- The annual sudden death mortality rate averaged 7.2% between 9-13.9 years, significantly higher than the 1.7% observed after 16 years (P=0.025).
- Sudden death risk peaked earlier in girls (10-11 years) and showed a male predominance after age 15. National data confirmed higher HCM mortality in 8-16 year olds compared to 17-30 year olds.
Conclusions:
- The findings indicate that children with a family history of HCM are at the highest risk for sudden cardiac death at a younger age than currently screened.
- Early screening for hypertrophic cardiomyopathy in children is crucial, particularly within the 9-13.9 age group.
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.
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