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

Research output: Contribution to journalArticle

154 Citations (Scopus)

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 2006

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Dilated Cardiomyopathy
Transplantation
Population
Mortality
Myocarditis
Sudden Death
Cardiomyopathies
Survivors
Histology
Cohort Studies
Regression Analysis

Keywords

  • Cardiomyopathy
  • Heart failure
  • Myocarditis
  • Pediatrics

ASJC Scopus subject areas

  • Physiology
  • Cardiology and Cardiovascular Medicine

Cite this

Daubeney, P. E. F., Nugent, A. W., Chondros, P., Carlin, J. B., Colan, S. D., Cheung, M., ... 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

Clinical features and outcomes of childhood dilated cardiomyopathy : Results from a national population-based study. / Daubeney, Piers E F; Nugent, Alan W.; Chondros, Patty; Carlin, John B.; Colan, Steven D.; Cheung, Michael; Davis, Andrew M.; Chow, C. W.; Weintraub, Robert G.

In: Circulation, Vol. 114, No. 24, 12.2006, p. 2671-2678.

Research output: Contribution to journalArticle

Daubeney, PEF, Nugent, AW, Chondros, P, Carlin, JB, Colan, SD, Cheung, M, Davis, AM, Chow, CW & Weintraub, RG 2006, 'Clinical features and outcomes of childhood dilated cardiomyopathy: Results from a national population-based study', Circulation, vol. 114, no. 24, pp. 2671-2678. https://doi.org/10.1161/CIRCULATIONAHA.106.635128
Daubeney, Piers E F ; Nugent, Alan W. ; Chondros, Patty ; Carlin, John B. ; Colan, Steven D. ; Cheung, Michael ; Davis, Andrew M. ; Chow, C. W. ; Weintraub, Robert G. / Clinical features and outcomes of childhood dilated cardiomyopathy : Results from a national population-based study. In: Circulation. 2006 ; Vol. 114, No. 24. pp. 2671-2678.
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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.",
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T1 - Clinical features and outcomes of childhood dilated cardiomyopathy

T2 - Results from a national population-based study

AU - Daubeney, Piers E F

AU - Nugent, Alan W.

AU - Chondros, Patty

AU - Carlin, John B.

AU - Colan, Steven D.

AU - Cheung, Michael

AU - Davis, Andrew M.

AU - Chow, C. W.

AU - Weintraub, Robert G.

PY - 2006/12

Y1 - 2006/12

N2 - 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.

AB - 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.

KW - Cardiomyopathy

KW - Heart failure

KW - Myocarditis

KW - Pediatrics

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