Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy.
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.
Ostman-Smith
Ingegerd
I
Division of Paediatrics, Department of Clinical Sciences, Sahlgren Academy, Queen Silvia Childreńs Hospital SE-416 85, Gothenburg, Sweden. ingegerd.ostman-smith@pediat.gu.se
Wettrell
Göran
G
Keeton
Barry
B
Holmgren
Daniel
D
Ergander
Ulf
U
Gould
Steven
S
Bowker
Colene
C
Verdicchio
Mario
M
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*
D000319:Adrenergic beta-Antagonists; D000728:Androgens