Clinical features and outcomes of childhood hypertrophic cardiomyopathy: Results from a national population-based study

Alan W. Nugent, Piers E F Daubeney, Patty Chondros, John B. Carlin, Steven D. Colan, Michael Cheung, Andrew M. Davis, C. W. Chow, Robert G. Weintraub

Research output: Contribution to journalArticle

117 Scopus citations

Abstract

Background - Population-based studies have provided insight into the natural history of adult hypertrophic cardiomyopathy, but comparable information for affected children is lacking. Methods and Results - All Australian children who presented with primary cardiomyopathy at 0 to 10 years of age between January 1, 1987, and December 31, 1996, were enrolled in a longitudinal cohort study. A single cardiologist reviewed serial cardiac investigations on each subject. A total of 80 subjects with hypertrophic cardiomyopathy were identified. An underlying syndromal, genetic, or metabolic condition was identified in 46 subjects (57.5%). There were no cases of sudden death at presentation. Left ventricular outflow tract obstruction was present in 32 subjects (40%); right ventricular outflow obstruction was present in 10 (12.5%). Freedom from death or transplantation was 83% (95% CI, 73 to 90) 5 years after presentation and 76% (95% CI, 62 to 86) 10 years after presentation. By proportional-hazards regression analysis, risk factors for death or transplantation included concentric left ventricular hypertrophy, age at presentation <1 year, lower initial fractional shortening Z score, and increasing left ventricular posterior wall thickness relative to body surface area. At the latest follow-up, 54 of 65 surviving subjects had no symptoms, and 46 were receiving no regular medication. Conclusions - Syndromal, genetic, and metabolic causes predominate in children with hypertrophic cardiomyopathy. Ventricular outflow tract obstruction is common. The clinical status of long-term survivors is good. This population-based study identifies children with hypertrophic cardiomyopathy who are at risk of adverse events.

Original languageEnglish (US)
Pages (from-to)1332-1338
Number of pages7
JournalCirculation
Volume112
Issue number9
DOIs
StatePublished - Aug 30 2005

Keywords

  • Cardiomyopathy
  • Pediatrics
  • Sudden death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

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    Nugent, A. W., Daubeney, P. E. F., Chondros, P., Carlin, J. B., Colan, S. D., Cheung, M., Davis, A. M., Chow, C. W., & Weintraub, R. G. (2005). Clinical features and outcomes of childhood hypertrophic cardiomyopathy: Results from a national population-based study. Circulation, 112(9), 1332-1338. https://doi.org/10.1161/CIRCULATIONAHA.104.530303