Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation

Anna E. Tsirka, Kathryn Trinkaus, Su Chiung Chen, Steven E Lipshultz, Jeffrey A. Towbin, Steven D. Colan, Vernat Exil, Arnold W. Strauss, Charles E. Canter

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Abstract

Objectives We studied the outcomes of pediatric patients diagnosed with dilated cardiomyopathy (DCM) and their relation to epidemiologic and echocardiographic variables at the time of presentation. Background The outcome of pediatric DCM patients ranges from recovery to a 50% to 60% chance of death within five years of diagnosis. The impact of heart transplantation and other emerging therapies on the outcomes of pediatric DCM patients is uncertain. Methods We performed a retrospective study of the outcomes in 91 pediatric patients diagnosed with DCM from 1990 to 1999. Routine therapy included use of digoxin, diuretics, angiotensin-converting enzyme inhibitors, and heart transplantation. Results At the time of last follow-up, 11 patients (12%) had died without transplantation; 20 (22%) underwent transplantation; 27 (30%) had persistent cardiomyopathy; and 33 (36%) had recovery of left ventricular systolic function. Overall actuarial one-year survival was 90%, and five-year survival was 83%. However, actuarial freedom from "heart death" (death or transplantation) was only 70% at one year and 58% at five years. Multivariate analysis found age <1 year (hazard ratio 7.1), age >12 years (hazard ratio 4.5), and female gender (hazard ratio 3.0) to be significantly associated with a greater risk of death or transplantation and a higher left ventricular shortening fraction at presentation (hazard ratio 0.92), with a slightly decreased risk of death or transplantation. Conclusions Pediatric DCM patients continue to have multiple outcomes, with recovery of left ventricular systolic function occurring most frequently. Utilization of heart transplantation has led to improved survival after the diagnosis of pediatric DCM.

Original languageEnglish
Pages (from-to)391-397
Number of pages7
JournalJournal of the American College of Cardiology
Volume44
Issue number2
DOIs
StatePublished - Jul 21 2004

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Dilated Cardiomyopathy
Heart Transplantation
Pediatrics
Transplantation
Left Ventricular Function
Survival
Digoxin
Cardiomyopathies
Angiotensin-Converting Enzyme Inhibitors
Diuretics
Multivariate Analysis
Retrospective Studies
Therapeutics

Keywords

  • ACE
  • angiotensin-converting enzyme
  • CI
  • confidence interval
  • DCM
  • dilated cardiomyopathy
  • ICU
  • intensive care unit
  • intravenous gamma globulin
  • IVGG
  • left ventricular
  • left ventricular ejection fraction
  • left ventricular end-diastolic dimension
  • LV
  • LVEDD
  • LVEF

ASJC Scopus subject areas

  • Nursing(all)

Cite this

Tsirka, A. E., Trinkaus, K., Chen, S. C., Lipshultz, S. E., Towbin, J. A., Colan, S. D., ... Canter, C. E. (2004). Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation. Journal of the American College of Cardiology, 44(2), 391-397. https://doi.org/10.1016/j.jacc.2004.04.035

Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation. / Tsirka, Anna E.; Trinkaus, Kathryn; Chen, Su Chiung; Lipshultz, Steven E; Towbin, Jeffrey A.; Colan, Steven D.; Exil, Vernat; Strauss, Arnold W.; Canter, Charles E.

In: Journal of the American College of Cardiology, Vol. 44, No. 2, 21.07.2004, p. 391-397.

Research output: Contribution to journalArticle

Tsirka, AE, Trinkaus, K, Chen, SC, Lipshultz, SE, Towbin, JA, Colan, SD, Exil, V, Strauss, AW & Canter, CE 2004, 'Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation', Journal of the American College of Cardiology, vol. 44, no. 2, pp. 391-397. https://doi.org/10.1016/j.jacc.2004.04.035
Tsirka, Anna E. ; Trinkaus, Kathryn ; Chen, Su Chiung ; Lipshultz, Steven E ; Towbin, Jeffrey A. ; Colan, Steven D. ; Exil, Vernat ; Strauss, Arnold W. ; Canter, Charles E. / Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation. In: Journal of the American College of Cardiology. 2004 ; Vol. 44, No. 2. pp. 391-397.
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abstract = "Objectives We studied the outcomes of pediatric patients diagnosed with dilated cardiomyopathy (DCM) and their relation to epidemiologic and echocardiographic variables at the time of presentation. Background The outcome of pediatric DCM patients ranges from recovery to a 50{\%} to 60{\%} chance of death within five years of diagnosis. The impact of heart transplantation and other emerging therapies on the outcomes of pediatric DCM patients is uncertain. Methods We performed a retrospective study of the outcomes in 91 pediatric patients diagnosed with DCM from 1990 to 1999. Routine therapy included use of digoxin, diuretics, angiotensin-converting enzyme inhibitors, and heart transplantation. Results At the time of last follow-up, 11 patients (12{\%}) had died without transplantation; 20 (22{\%}) underwent transplantation; 27 (30{\%}) had persistent cardiomyopathy; and 33 (36{\%}) had recovery of left ventricular systolic function. Overall actuarial one-year survival was 90{\%}, and five-year survival was 83{\%}. However, actuarial freedom from {"}heart death{"} (death or transplantation) was only 70{\%} at one year and 58{\%} at five years. Multivariate analysis found age <1 year (hazard ratio 7.1), age >12 years (hazard ratio 4.5), and female gender (hazard ratio 3.0) to be significantly associated with a greater risk of death or transplantation and a higher left ventricular shortening fraction at presentation (hazard ratio 0.92), with a slightly decreased risk of death or transplantation. Conclusions Pediatric DCM patients continue to have multiple outcomes, with recovery of left ventricular systolic function occurring most frequently. Utilization of heart transplantation has led to improved survival after the diagnosis of pediatric DCM.",
keywords = "ACE, angiotensin-converting enzyme, CI, confidence interval, DCM, dilated cardiomyopathy, ICU, intensive care unit, intravenous gamma globulin, IVGG, left ventricular, left ventricular ejection fraction, left ventricular end-diastolic dimension, LV, LVEDD, LVEF",
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AU - Towbin, Jeffrey A.

AU - Colan, Steven D.

AU - Exil, Vernat

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N2 - Objectives We studied the outcomes of pediatric patients diagnosed with dilated cardiomyopathy (DCM) and their relation to epidemiologic and echocardiographic variables at the time of presentation. Background The outcome of pediatric DCM patients ranges from recovery to a 50% to 60% chance of death within five years of diagnosis. The impact of heart transplantation and other emerging therapies on the outcomes of pediatric DCM patients is uncertain. Methods We performed a retrospective study of the outcomes in 91 pediatric patients diagnosed with DCM from 1990 to 1999. Routine therapy included use of digoxin, diuretics, angiotensin-converting enzyme inhibitors, and heart transplantation. Results At the time of last follow-up, 11 patients (12%) had died without transplantation; 20 (22%) underwent transplantation; 27 (30%) had persistent cardiomyopathy; and 33 (36%) had recovery of left ventricular systolic function. Overall actuarial one-year survival was 90%, and five-year survival was 83%. However, actuarial freedom from "heart death" (death or transplantation) was only 70% at one year and 58% at five years. Multivariate analysis found age <1 year (hazard ratio 7.1), age >12 years (hazard ratio 4.5), and female gender (hazard ratio 3.0) to be significantly associated with a greater risk of death or transplantation and a higher left ventricular shortening fraction at presentation (hazard ratio 0.92), with a slightly decreased risk of death or transplantation. Conclusions Pediatric DCM patients continue to have multiple outcomes, with recovery of left ventricular systolic function occurring most frequently. Utilization of heart transplantation has led to improved survival after the diagnosis of pediatric DCM.

AB - Objectives We studied the outcomes of pediatric patients diagnosed with dilated cardiomyopathy (DCM) and their relation to epidemiologic and echocardiographic variables at the time of presentation. Background The outcome of pediatric DCM patients ranges from recovery to a 50% to 60% chance of death within five years of diagnosis. The impact of heart transplantation and other emerging therapies on the outcomes of pediatric DCM patients is uncertain. Methods We performed a retrospective study of the outcomes in 91 pediatric patients diagnosed with DCM from 1990 to 1999. Routine therapy included use of digoxin, diuretics, angiotensin-converting enzyme inhibitors, and heart transplantation. Results At the time of last follow-up, 11 patients (12%) had died without transplantation; 20 (22%) underwent transplantation; 27 (30%) had persistent cardiomyopathy; and 33 (36%) had recovery of left ventricular systolic function. Overall actuarial one-year survival was 90%, and five-year survival was 83%. However, actuarial freedom from "heart death" (death or transplantation) was only 70% at one year and 58% at five years. Multivariate analysis found age <1 year (hazard ratio 7.1), age >12 years (hazard ratio 4.5), and female gender (hazard ratio 3.0) to be significantly associated with a greater risk of death or transplantation and a higher left ventricular shortening fraction at presentation (hazard ratio 0.92), with a slightly decreased risk of death or transplantation. Conclusions Pediatric DCM patients continue to have multiple outcomes, with recovery of left ventricular systolic function occurring most frequently. Utilization of heart transplantation has led to improved survival after the diagnosis of pediatric DCM.

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