Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood: An outcomes study from the pediatric cardiomyopathy registry

Susan R. Foerster, Charles E. Canter, Amy Cinar, Lynn A. Sleeper, Steven A. Webber, Elfriede Pahl, Paul F. Kantor, Jorge A. Alvarez, Steven D. Colan, John Jefferies, Jacqueline M. Lamour, Renee Margossian, Jane E. Messere, Paolo G. Rusconi, Robert E. Shaddy, Jeffrey Towbin, James D. Wilkinson, Steven E. Lipshultz

Research output: Contribution to journalArticle

66 Citations (Scopus)

Abstract

Background - Myocarditis is a cause of a new-onset dilated cardiomyopathy phenotype in children, with small studies reporting high rates of recovery of left ventricular (LV) function. Methods and Results - The presenting characteristics and outcomes of children with myocarditis diagnosed clinically and with biopsy confirmation (n=119) or with probable myocarditis diagnosed clinically or by biopsy alone (n=253) were compared with children with idiopathic dilated cardiomyopathy (n=1123). Characteristics at presentation were assessed as possible predictors of outcomes. The distributions of time to death, transplantation, and echocardiographic normalization in the biopsy-confirmed myocarditis and probable myocarditis groups did not differ (P≥0.5), but both groups differed significantly from the idiopathic dilated cardiomyopathy group (all P≤0.003). In children with myocarditis, lower LV fractional shortening z-score at presentation predicted greater mortality (hazard ratio, 0.85; 95% confidence interval, 0.73 to 0.98; P=0.03) and greater LV posterior wall thickness predicted transplantation (hazard ratio, 1.17; 95% confidence interval, 1.02 to 1.35; P=0.03). In those with decreased LV fractional shortening at presentation, independent predictors of echocardiographic normalization were presentation with an LV end-diastolic dimension z-score >2 (hazard ratio, 0.36; 95% confidence interval, 0.22 to 0.58; P<0.001) and greater septal wall thickness (hazard ratio, 1.16; 95% confidence interval, 1.01 to 1.34; P=0.04). Conclusions - Children with biopsy-confirmed or probable myocarditis had similar proportions of death, transplantation, and echocardiographic normalization 3 years after presentation and better outcomes than those of children with idiopathic dilated cardiomyopathy. In children with myocarditis who had impaired LV ejection at presentation, rates of echocardiographic normalization were greater in those without LV dilation and in those with greater septal wall thickness at presentation. Clinical Trial Registration - URL:http://www.clinicaltrials.gov. Unique identifier: NCT00005391.(Circ Heart Fail. 2010;3:689-697.).

Original languageEnglish (US)
Pages (from-to)689-697
Number of pages9
JournalCirculation: Heart Failure
Volume3
Issue number6
DOIs
StatePublished - Nov 1 2010

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Ventricular Remodeling
Myocarditis
Dilated Cardiomyopathy
Cardiomyopathies
Registries
Outcome Assessment (Health Care)
Pediatrics
Confidence Intervals
Biopsy
Transplantation
Left Ventricular Function
Dilatation
Clinical Trials
Phenotype
Mortality

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

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Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood : An outcomes study from the pediatric cardiomyopathy registry. / Foerster, Susan R.; Canter, Charles E.; Cinar, Amy; Sleeper, Lynn A.; Webber, Steven A.; Pahl, Elfriede; Kantor, Paul F.; Alvarez, Jorge A.; Colan, Steven D.; Jefferies, John; Lamour, Jacqueline M.; Margossian, Renee; Messere, Jane E.; Rusconi, Paolo G.; Shaddy, Robert E.; Towbin, Jeffrey; Wilkinson, James D.; Lipshultz, Steven E.

In: Circulation: Heart Failure, Vol. 3, No. 6, 01.11.2010, p. 689-697.

Research output: Contribution to journalArticle

Foerster, SR, Canter, CE, Cinar, A, Sleeper, LA, Webber, SA, Pahl, E, Kantor, PF, Alvarez, JA, Colan, SD, Jefferies, J, Lamour, JM, Margossian, R, Messere, JE, Rusconi, PG, Shaddy, RE, Towbin, J, Wilkinson, JD & Lipshultz, SE 2010, 'Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood: An outcomes study from the pediatric cardiomyopathy registry', Circulation: Heart Failure, vol. 3, no. 6, pp. 689-697. https://doi.org/10.1161/CIRCHEARTFAILURE.109.902833
Foerster, Susan R. ; Canter, Charles E. ; Cinar, Amy ; Sleeper, Lynn A. ; Webber, Steven A. ; Pahl, Elfriede ; Kantor, Paul F. ; Alvarez, Jorge A. ; Colan, Steven D. ; Jefferies, John ; Lamour, Jacqueline M. ; Margossian, Renee ; Messere, Jane E. ; Rusconi, Paolo G. ; Shaddy, Robert E. ; Towbin, Jeffrey ; Wilkinson, James D. ; Lipshultz, Steven E. / Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood : An outcomes study from the pediatric cardiomyopathy registry. In: Circulation: Heart Failure. 2010 ; Vol. 3, No. 6. pp. 689-697.
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abstract = "Background - Myocarditis is a cause of a new-onset dilated cardiomyopathy phenotype in children, with small studies reporting high rates of recovery of left ventricular (LV) function. Methods and Results - The presenting characteristics and outcomes of children with myocarditis diagnosed clinically and with biopsy confirmation (n=119) or with probable myocarditis diagnosed clinically or by biopsy alone (n=253) were compared with children with idiopathic dilated cardiomyopathy (n=1123). Characteristics at presentation were assessed as possible predictors of outcomes. The distributions of time to death, transplantation, and echocardiographic normalization in the biopsy-confirmed myocarditis and probable myocarditis groups did not differ (P≥0.5), but both groups differed significantly from the idiopathic dilated cardiomyopathy group (all P≤0.003). In children with myocarditis, lower LV fractional shortening z-score at presentation predicted greater mortality (hazard ratio, 0.85; 95{\%} confidence interval, 0.73 to 0.98; P=0.03) and greater LV posterior wall thickness predicted transplantation (hazard ratio, 1.17; 95{\%} confidence interval, 1.02 to 1.35; P=0.03). In those with decreased LV fractional shortening at presentation, independent predictors of echocardiographic normalization were presentation with an LV end-diastolic dimension z-score >2 (hazard ratio, 0.36; 95{\%} confidence interval, 0.22 to 0.58; P<0.001) and greater septal wall thickness (hazard ratio, 1.16; 95{\%} confidence interval, 1.01 to 1.34; P=0.04). Conclusions - Children with biopsy-confirmed or probable myocarditis had similar proportions of death, transplantation, and echocardiographic normalization 3 years after presentation and better outcomes than those of children with idiopathic dilated cardiomyopathy. In children with myocarditis who had impaired LV ejection at presentation, rates of echocardiographic normalization were greater in those without LV dilation and in those with greater septal wall thickness at presentation. Clinical Trial Registration - URL:http://www.clinicaltrials.gov. Unique identifier: NCT00005391.(Circ Heart Fail. 2010;3:689-697.).",
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T1 - Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood

T2 - An outcomes study from the pediatric cardiomyopathy registry

AU - Foerster, Susan R.

AU - Canter, Charles E.

AU - Cinar, Amy

AU - Sleeper, Lynn A.

AU - Webber, Steven A.

AU - Pahl, Elfriede

AU - Kantor, Paul F.

AU - Alvarez, Jorge A.

AU - Colan, Steven D.

AU - Jefferies, John

AU - Lamour, Jacqueline M.

AU - Margossian, Renee

AU - Messere, Jane E.

AU - Rusconi, Paolo G.

AU - Shaddy, Robert E.

AU - Towbin, Jeffrey

AU - Wilkinson, James D.

AU - Lipshultz, Steven E.

PY - 2010/11/1

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N2 - Background - Myocarditis is a cause of a new-onset dilated cardiomyopathy phenotype in children, with small studies reporting high rates of recovery of left ventricular (LV) function. Methods and Results - The presenting characteristics and outcomes of children with myocarditis diagnosed clinically and with biopsy confirmation (n=119) or with probable myocarditis diagnosed clinically or by biopsy alone (n=253) were compared with children with idiopathic dilated cardiomyopathy (n=1123). Characteristics at presentation were assessed as possible predictors of outcomes. The distributions of time to death, transplantation, and echocardiographic normalization in the biopsy-confirmed myocarditis and probable myocarditis groups did not differ (P≥0.5), but both groups differed significantly from the idiopathic dilated cardiomyopathy group (all P≤0.003). In children with myocarditis, lower LV fractional shortening z-score at presentation predicted greater mortality (hazard ratio, 0.85; 95% confidence interval, 0.73 to 0.98; P=0.03) and greater LV posterior wall thickness predicted transplantation (hazard ratio, 1.17; 95% confidence interval, 1.02 to 1.35; P=0.03). In those with decreased LV fractional shortening at presentation, independent predictors of echocardiographic normalization were presentation with an LV end-diastolic dimension z-score >2 (hazard ratio, 0.36; 95% confidence interval, 0.22 to 0.58; P<0.001) and greater septal wall thickness (hazard ratio, 1.16; 95% confidence interval, 1.01 to 1.34; P=0.04). Conclusions - Children with biopsy-confirmed or probable myocarditis had similar proportions of death, transplantation, and echocardiographic normalization 3 years after presentation and better outcomes than those of children with idiopathic dilated cardiomyopathy. In children with myocarditis who had impaired LV ejection at presentation, rates of echocardiographic normalization were greater in those without LV dilation and in those with greater septal wall thickness at presentation. Clinical Trial Registration - URL:http://www.clinicaltrials.gov. Unique identifier: NCT00005391.(Circ Heart Fail. 2010;3:689-697.).

AB - Background - Myocarditis is a cause of a new-onset dilated cardiomyopathy phenotype in children, with small studies reporting high rates of recovery of left ventricular (LV) function. Methods and Results - The presenting characteristics and outcomes of children with myocarditis diagnosed clinically and with biopsy confirmation (n=119) or with probable myocarditis diagnosed clinically or by biopsy alone (n=253) were compared with children with idiopathic dilated cardiomyopathy (n=1123). Characteristics at presentation were assessed as possible predictors of outcomes. The distributions of time to death, transplantation, and echocardiographic normalization in the biopsy-confirmed myocarditis and probable myocarditis groups did not differ (P≥0.5), but both groups differed significantly from the idiopathic dilated cardiomyopathy group (all P≤0.003). In children with myocarditis, lower LV fractional shortening z-score at presentation predicted greater mortality (hazard ratio, 0.85; 95% confidence interval, 0.73 to 0.98; P=0.03) and greater LV posterior wall thickness predicted transplantation (hazard ratio, 1.17; 95% confidence interval, 1.02 to 1.35; P=0.03). In those with decreased LV fractional shortening at presentation, independent predictors of echocardiographic normalization were presentation with an LV end-diastolic dimension z-score >2 (hazard ratio, 0.36; 95% confidence interval, 0.22 to 0.58; P<0.001) and greater septal wall thickness (hazard ratio, 1.16; 95% confidence interval, 1.01 to 1.34; P=0.04). Conclusions - Children with biopsy-confirmed or probable myocarditis had similar proportions of death, transplantation, and echocardiographic normalization 3 years after presentation and better outcomes than those of children with idiopathic dilated cardiomyopathy. In children with myocarditis who had impaired LV ejection at presentation, rates of echocardiographic normalization were greater in those without LV dilation and in those with greater septal wall thickness at presentation. Clinical Trial Registration - URL:http://www.clinicaltrials.gov. Unique identifier: NCT00005391.(Circ Heart Fail. 2010;3:689-697.).

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