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

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

*Corresponding author for this work

Research output: Contribution to journalArticle

163 Scopus citations

Abstract

BACKGROUND - Despite considerable mortality, population-based prognostic factors for childhood dilated cardiomyopathy are lacking. METHODS AND RESULTS - A population-based cohort study was undertaken of all children in Australia who presented with cardiomyopathy at age 0 to 10 years between January 1, 1987, and December 31, 1996. A single cardiologist analyzed all cardiac investigations, and a single pathologist analyzed histopathological material. There were 184 subjects with dilated cardiomyopathy. Positive viral identification or lymphocytic myocarditis was found in 30 (68.2%) of 44 cases with available early histology and 8 of 9 cases presenting with sudden death. Freedom from death or transplantation was 72% (95% CI, 65% to 78%) 1 year after presentation and 63% (95% CI, 55% to 70%) at 5 years. By proportional hazards regression analysis, risk factors for death or transplantation comprised age >5 years at presentation (hazard ratio 5.6, 95% CI, 2.6 to 12.0), familial dilated cardiomyopathy (hazard ratio, 2.9; 95% CI, 1.5 to 5.6), lower initial fractional shortening z score (hazard ratio per z-score unit, 0.75; 95% CI, 0.65 to 0.87), and failure to increase fractional shortening z score during follow-up (hazard ratio per unit increase, 0.68; 95% CI, 0.58 to 0.79). At follow-up, 78 (44.6%) of 175 cases diagnosed during life have no symptoms and are not taking any cardiac medication. CONCLUSIONS - Early mortality is high in childhood dilated cardiomyopathy, but the clinical status of long-term survivors is good. This population-based study identifies children at risk of adverse events.

Original languageEnglish (US)
Pages (from-to)2671-2678
Number of pages8
JournalCirculation
Volume114
Issue number24
DOIs
StatePublished - Dec 1 2006

Keywords

  • Cardiomyopathy
  • Heart failure
  • Myocarditis
  • Pediatrics

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Fingerprint Dive into the research topics of 'Clinical features and outcomes of childhood dilated cardiomyopathy: Results from a national population-based study'. Together they form a unique fingerprint.

  • Cite this

    Daubeney, P. E. F., Nugent, A. W., Chondros, P., Carlin, J. B., Colan, S. D., Cheung, M., Davis, A. M., Chow, C. W., & Weintraub, R. G. (2006). Clinical features and outcomes of childhood dilated cardiomyopathy: Results from a national population-based study. Circulation, 114(24), 2671-2678. https://doi.org/10.1161/CIRCULATIONAHA.106.635128