The epidemiology of childhood cardiomyopathy in Australia

Alan W. Nugent, Piers E F Daubeney, Patty Chondros, John B. Carlin, Michael Cheung, Lynette C. Wilkinson, Andrew M. Davis, Stephen G. Kahler, C. W. Chow, James L. Wilkinson, Robert G. Weintraub

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Abstract

BACKGROUND: The incidence and age distribution of primary cardiomyopathy in children are not well defined. We undertook a population-based, retrospective cohort study in Australia to document the epidemiology of childhood cardiomyopathy. METHODS: We analyzed all cases of primary cardiomyopathy in children who presented between 1987 and 1996 and who were younger than 10 years ofage. Children were recruited from multiple sources, and cases of cardiomyopathy were classified according to World Health Organization guidelines. RESULTS: Over the 10-year period, 314 new cases of primary cardiomyopathy were identified, for an annual incidence of 1.24 per 100,000 children younger than 10 years ofage (95 percent confidence interval, 1.11 to 1.38). Dilated cardiomyopathy made up 58.6 percent of cases, hypertrophic cardiomyopathy 25.5 percent, restrictive cardiomyopathy 2.5 percent, and left ventricular noncompaction 9.2 percent of cases. The incidence of all types of cardiomyopathy except restrictive declined rapidly after infancy. In 11 cases (3.5 percent), sudden death was the first symptom. There was a male predominance among children with hypertrophic and unclassified cardiomyopathy. Indigenous children had a higher incidence of dilated cardiomyopathy than nonindigenous children (relative risk, 2.67; 95 percent confidence interval, 1.42 to 4.63) and a higher rate of death as the presenting symptom (16.7 percent vs. 2.6 percent, P=0.02). Lymphocytic myocarditis was present in 25 of 62 children with dilated cardiomyopathy (40.3 percent) who underwent cardiac histologic examination within two months after presentation. CONCLUSIONS: Lymphocytic myocarditis and left ventricular noncompaction are important causes of childhood cardiomyopathy in Australia. The timing and severity of presentation in children with cardiomyopathy are related to the type of cardiomyopathy, as well as to genetic and ethnic factors.

Original languageEnglish (US)
Pages (from-to)1639-1646
Number of pages8
JournalNew England Journal of Medicine
Volume348
Issue number17
DOIs
StatePublished - Apr 24 2003

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Cardiomyopathies
Epidemiology
Dilated Cardiomyopathy
Restrictive Cardiomyopathy
Hypertrophic Cardiomyopathy
Incidence
Myocarditis
Confidence Intervals
Age Distribution
Sudden Death
Cohort Studies
Retrospective Studies
Guidelines
Mortality
Population

ASJC Scopus subject areas

  • Medicine(all)

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Nugent, A. W., Daubeney, P. E. F., Chondros, P., Carlin, J. B., Cheung, M., Wilkinson, L. C., ... Weintraub, R. G. (2003). The epidemiology of childhood cardiomyopathy in Australia. New England Journal of Medicine, 348(17), 1639-1646. https://doi.org/10.1056/NEJMoa021737

The epidemiology of childhood cardiomyopathy in Australia. / Nugent, Alan W.; Daubeney, Piers E F; Chondros, Patty; Carlin, John B.; Cheung, Michael; Wilkinson, Lynette C.; Davis, Andrew M.; Kahler, Stephen G.; Chow, C. W.; Wilkinson, James L.; Weintraub, Robert G.

In: New England Journal of Medicine, Vol. 348, No. 17, 24.04.2003, p. 1639-1646.

Research output: Contribution to journalArticle

Nugent, AW, Daubeney, PEF, Chondros, P, Carlin, JB, Cheung, M, Wilkinson, LC, Davis, AM, Kahler, SG, Chow, CW, Wilkinson, JL & Weintraub, RG 2003, 'The epidemiology of childhood cardiomyopathy in Australia', New England Journal of Medicine, vol. 348, no. 17, pp. 1639-1646. https://doi.org/10.1056/NEJMoa021737
Nugent AW, Daubeney PEF, Chondros P, Carlin JB, Cheung M, Wilkinson LC et al. The epidemiology of childhood cardiomyopathy in Australia. New England Journal of Medicine. 2003 Apr 24;348(17):1639-1646. https://doi.org/10.1056/NEJMoa021737
Nugent, Alan W. ; Daubeney, Piers E F ; Chondros, Patty ; Carlin, John B. ; Cheung, Michael ; Wilkinson, Lynette C. ; Davis, Andrew M. ; Kahler, Stephen G. ; Chow, C. W. ; Wilkinson, James L. ; Weintraub, Robert G. / The epidemiology of childhood cardiomyopathy in Australia. In: New England Journal of Medicine. 2003 ; Vol. 348, No. 17. pp. 1639-1646.
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abstract = "BACKGROUND: The incidence and age distribution of primary cardiomyopathy in children are not well defined. We undertook a population-based, retrospective cohort study in Australia to document the epidemiology of childhood cardiomyopathy. METHODS: We analyzed all cases of primary cardiomyopathy in children who presented between 1987 and 1996 and who were younger than 10 years ofage. Children were recruited from multiple sources, and cases of cardiomyopathy were classified according to World Health Organization guidelines. RESULTS: Over the 10-year period, 314 new cases of primary cardiomyopathy were identified, for an annual incidence of 1.24 per 100,000 children younger than 10 years ofage (95 percent confidence interval, 1.11 to 1.38). Dilated cardiomyopathy made up 58.6 percent of cases, hypertrophic cardiomyopathy 25.5 percent, restrictive cardiomyopathy 2.5 percent, and left ventricular noncompaction 9.2 percent of cases. The incidence of all types of cardiomyopathy except restrictive declined rapidly after infancy. In 11 cases (3.5 percent), sudden death was the first symptom. There was a male predominance among children with hypertrophic and unclassified cardiomyopathy. Indigenous children had a higher incidence of dilated cardiomyopathy than nonindigenous children (relative risk, 2.67; 95 percent confidence interval, 1.42 to 4.63) and a higher rate of death as the presenting symptom (16.7 percent vs. 2.6 percent, P=0.02). Lymphocytic myocarditis was present in 25 of 62 children with dilated cardiomyopathy (40.3 percent) who underwent cardiac histologic examination within two months after presentation. CONCLUSIONS: Lymphocytic myocarditis and left ventricular noncompaction are important causes of childhood cardiomyopathy in Australia. The timing and severity of presentation in children with cardiomyopathy are related to the type of cardiomyopathy, as well as to genetic and ethnic factors.",
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T1 - The epidemiology of childhood cardiomyopathy in Australia

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AU - Daubeney, Piers E F

AU - Chondros, Patty

AU - Carlin, John B.

AU - Cheung, Michael

AU - Wilkinson, Lynette C.

AU - Davis, Andrew M.

AU - Kahler, Stephen G.

AU - Chow, C. W.

AU - Wilkinson, James L.

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N2 - BACKGROUND: The incidence and age distribution of primary cardiomyopathy in children are not well defined. We undertook a population-based, retrospective cohort study in Australia to document the epidemiology of childhood cardiomyopathy. METHODS: We analyzed all cases of primary cardiomyopathy in children who presented between 1987 and 1996 and who were younger than 10 years ofage. Children were recruited from multiple sources, and cases of cardiomyopathy were classified according to World Health Organization guidelines. RESULTS: Over the 10-year period, 314 new cases of primary cardiomyopathy were identified, for an annual incidence of 1.24 per 100,000 children younger than 10 years ofage (95 percent confidence interval, 1.11 to 1.38). Dilated cardiomyopathy made up 58.6 percent of cases, hypertrophic cardiomyopathy 25.5 percent, restrictive cardiomyopathy 2.5 percent, and left ventricular noncompaction 9.2 percent of cases. The incidence of all types of cardiomyopathy except restrictive declined rapidly after infancy. In 11 cases (3.5 percent), sudden death was the first symptom. There was a male predominance among children with hypertrophic and unclassified cardiomyopathy. Indigenous children had a higher incidence of dilated cardiomyopathy than nonindigenous children (relative risk, 2.67; 95 percent confidence interval, 1.42 to 4.63) and a higher rate of death as the presenting symptom (16.7 percent vs. 2.6 percent, P=0.02). Lymphocytic myocarditis was present in 25 of 62 children with dilated cardiomyopathy (40.3 percent) who underwent cardiac histologic examination within two months after presentation. CONCLUSIONS: Lymphocytic myocarditis and left ventricular noncompaction are important causes of childhood cardiomyopathy in Australia. The timing and severity of presentation in children with cardiomyopathy are related to the type of cardiomyopathy, as well as to genetic and ethnic factors.

AB - BACKGROUND: The incidence and age distribution of primary cardiomyopathy in children are not well defined. We undertook a population-based, retrospective cohort study in Australia to document the epidemiology of childhood cardiomyopathy. METHODS: We analyzed all cases of primary cardiomyopathy in children who presented between 1987 and 1996 and who were younger than 10 years ofage. Children were recruited from multiple sources, and cases of cardiomyopathy were classified according to World Health Organization guidelines. RESULTS: Over the 10-year period, 314 new cases of primary cardiomyopathy were identified, for an annual incidence of 1.24 per 100,000 children younger than 10 years ofage (95 percent confidence interval, 1.11 to 1.38). Dilated cardiomyopathy made up 58.6 percent of cases, hypertrophic cardiomyopathy 25.5 percent, restrictive cardiomyopathy 2.5 percent, and left ventricular noncompaction 9.2 percent of cases. The incidence of all types of cardiomyopathy except restrictive declined rapidly after infancy. In 11 cases (3.5 percent), sudden death was the first symptom. There was a male predominance among children with hypertrophic and unclassified cardiomyopathy. Indigenous children had a higher incidence of dilated cardiomyopathy than nonindigenous children (relative risk, 2.67; 95 percent confidence interval, 1.42 to 4.63) and a higher rate of death as the presenting symptom (16.7 percent vs. 2.6 percent, P=0.02). Lymphocytic myocarditis was present in 25 of 62 children with dilated cardiomyopathy (40.3 percent) who underwent cardiac histologic examination within two months after presentation. CONCLUSIONS: Lymphocytic myocarditis and left ventricular noncompaction are important causes of childhood cardiomyopathy in Australia. The timing and severity of presentation in children with cardiomyopathy are related to the type of cardiomyopathy, as well as to genetic and ethnic factors.

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