Survival Without Cardiac Transplantation Among Children With Dilated Cardiomyopathy

Rakesh K. Singh, Charles E. Canter, Ling Shi, Steven D. Colan, Debra A. Dodd, Melanie D. Everitt, Daphne T. Hsu, John L. Jefferies, Paul F. Kantor, Elfriede Pahl, Joseph W. Rossano, Jeffrey A. Towbin, James D. Wilkinson, Steven E Lipshultz

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

Background Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation. Objectives This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death. Methods Children <18 years of age with some type of DCM enrolled in the Pediatric Cardiomyopathy Registry were divided by year of diagnosis into an early cohort (1990 to 1999) and a late cohort (2000 to 2009). Competing risks and multivariable modeling were used to estimate the cumulative incidence of death, transplant, and echocardiographic normalization by cohort and to identify the factors associated with death. Results Of 1,953 children, 1,199 were in the early cohort and 754 were in the late cohort. Most children in both cohorts had idiopathic DCM (64% vs. 63%, respectively). Median age (1.6 vs. 1.7 years), left ventricular end-diastolic z-scores (+4.2 vs. +4.2), and left ventricular fractional shortening (16% vs. 17%) at diagnosis were similar between cohorts. Although the rates of echocardiographic normalization (30% and 27%) and heart transplantation (24% and 24%) were similar, the death rate was higher in the early cohort than in the late cohort (18% vs. 9%; p = 0.04). Being in the early cohort (hazard ratio: 1.4; 95% confidence interval: 1.04 to 1.9; p = 0.03) independently predicted death. Conclusions Children with DCM have improved survival in the more recent era. This appears to be associated with factors other than heart transplantation, which was equally prevalent in both eras. (Pediatric Cardiomyopathy Registry [PCMR]; NCT00005391)

Original languageEnglish (US)
Pages (from-to)2663-2673
Number of pages11
JournalJournal of the American College of Cardiology
Volume70
Issue number21
DOIs
StatePublished - Nov 28 2017
Externally publishedYes

Fingerprint

Dilated Cardiomyopathy
Heart Transplantation
Cardiomyopathies
Registries
Pediatrics
Transplants
Confidence Intervals
Mortality
Incidence

Keywords

  • cardiomyopathy
  • echocardiography
  • heart failure
  • pediatrics
  • transplantation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Singh, R. K., Canter, C. E., Shi, L., Colan, S. D., Dodd, D. A., Everitt, M. D., ... Lipshultz, S. E. (2017). Survival Without Cardiac Transplantation Among Children With Dilated Cardiomyopathy. Journal of the American College of Cardiology, 70(21), 2663-2673. https://doi.org/10.1016/j.jacc.2017.09.1089

Survival Without Cardiac Transplantation Among Children With Dilated Cardiomyopathy. / Singh, Rakesh K.; Canter, Charles E.; Shi, Ling; Colan, Steven D.; Dodd, Debra A.; Everitt, Melanie D.; Hsu, Daphne T.; Jefferies, John L.; Kantor, Paul F.; Pahl, Elfriede; Rossano, Joseph W.; Towbin, Jeffrey A.; Wilkinson, James D.; Lipshultz, Steven E.

In: Journal of the American College of Cardiology, Vol. 70, No. 21, 28.11.2017, p. 2663-2673.

Research output: Contribution to journalArticle

Singh, RK, Canter, CE, Shi, L, Colan, SD, Dodd, DA, Everitt, MD, Hsu, DT, Jefferies, JL, Kantor, PF, Pahl, E, Rossano, JW, Towbin, JA, Wilkinson, JD & Lipshultz, SE 2017, 'Survival Without Cardiac Transplantation Among Children With Dilated Cardiomyopathy', Journal of the American College of Cardiology, vol. 70, no. 21, pp. 2663-2673. https://doi.org/10.1016/j.jacc.2017.09.1089
Singh, Rakesh K. ; Canter, Charles E. ; Shi, Ling ; Colan, Steven D. ; Dodd, Debra A. ; Everitt, Melanie D. ; Hsu, Daphne T. ; Jefferies, John L. ; Kantor, Paul F. ; Pahl, Elfriede ; Rossano, Joseph W. ; Towbin, Jeffrey A. ; Wilkinson, James D. ; Lipshultz, Steven E. / Survival Without Cardiac Transplantation Among Children With Dilated Cardiomyopathy. In: Journal of the American College of Cardiology. 2017 ; Vol. 70, No. 21. pp. 2663-2673.
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abstract = "Background Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation. Objectives This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death. Methods Children <18 years of age with some type of DCM enrolled in the Pediatric Cardiomyopathy Registry were divided by year of diagnosis into an early cohort (1990 to 1999) and a late cohort (2000 to 2009). Competing risks and multivariable modeling were used to estimate the cumulative incidence of death, transplant, and echocardiographic normalization by cohort and to identify the factors associated with death. Results Of 1,953 children, 1,199 were in the early cohort and 754 were in the late cohort. Most children in both cohorts had idiopathic DCM (64{\%} vs. 63{\%}, respectively). Median age (1.6 vs. 1.7 years), left ventricular end-diastolic z-scores (+4.2 vs. +4.2), and left ventricular fractional shortening (16{\%} vs. 17{\%}) at diagnosis were similar between cohorts. Although the rates of echocardiographic normalization (30{\%} and 27{\%}) and heart transplantation (24{\%} and 24{\%}) were similar, the death rate was higher in the early cohort than in the late cohort (18{\%} vs. 9{\%}; p = 0.04). Being in the early cohort (hazard ratio: 1.4; 95{\%} confidence interval: 1.04 to 1.9; p = 0.03) independently predicted death. Conclusions Children with DCM have improved survival in the more recent era. This appears to be associated with factors other than heart transplantation, which was equally prevalent in both eras. (Pediatric Cardiomyopathy Registry [PCMR]; NCT00005391)",
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AU - Singh, Rakesh K.

AU - Canter, Charles E.

AU - Shi, Ling

AU - Colan, Steven D.

AU - Dodd, Debra A.

AU - Everitt, Melanie D.

AU - Hsu, Daphne T.

AU - Jefferies, John L.

AU - Kantor, Paul F.

AU - Pahl, Elfriede

AU - Rossano, Joseph W.

AU - Towbin, Jeffrey A.

AU - Wilkinson, James D.

AU - Lipshultz, Steven E

PY - 2017/11/28

Y1 - 2017/11/28

N2 - Background Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation. Objectives This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death. Methods Children <18 years of age with some type of DCM enrolled in the Pediatric Cardiomyopathy Registry were divided by year of diagnosis into an early cohort (1990 to 1999) and a late cohort (2000 to 2009). Competing risks and multivariable modeling were used to estimate the cumulative incidence of death, transplant, and echocardiographic normalization by cohort and to identify the factors associated with death. Results Of 1,953 children, 1,199 were in the early cohort and 754 were in the late cohort. Most children in both cohorts had idiopathic DCM (64% vs. 63%, respectively). Median age (1.6 vs. 1.7 years), left ventricular end-diastolic z-scores (+4.2 vs. +4.2), and left ventricular fractional shortening (16% vs. 17%) at diagnosis were similar between cohorts. Although the rates of echocardiographic normalization (30% and 27%) and heart transplantation (24% and 24%) were similar, the death rate was higher in the early cohort than in the late cohort (18% vs. 9%; p = 0.04). Being in the early cohort (hazard ratio: 1.4; 95% confidence interval: 1.04 to 1.9; p = 0.03) independently predicted death. Conclusions Children with DCM have improved survival in the more recent era. This appears to be associated with factors other than heart transplantation, which was equally prevalent in both eras. (Pediatric Cardiomyopathy Registry [PCMR]; NCT00005391)

AB - Background Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation. Objectives This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death. Methods Children <18 years of age with some type of DCM enrolled in the Pediatric Cardiomyopathy Registry were divided by year of diagnosis into an early cohort (1990 to 1999) and a late cohort (2000 to 2009). Competing risks and multivariable modeling were used to estimate the cumulative incidence of death, transplant, and echocardiographic normalization by cohort and to identify the factors associated with death. Results Of 1,953 children, 1,199 were in the early cohort and 754 were in the late cohort. Most children in both cohorts had idiopathic DCM (64% vs. 63%, respectively). Median age (1.6 vs. 1.7 years), left ventricular end-diastolic z-scores (+4.2 vs. +4.2), and left ventricular fractional shortening (16% vs. 17%) at diagnosis were similar between cohorts. Although the rates of echocardiographic normalization (30% and 27%) and heart transplantation (24% and 24%) were similar, the death rate was higher in the early cohort than in the late cohort (18% vs. 9%; p = 0.04). Being in the early cohort (hazard ratio: 1.4; 95% confidence interval: 1.04 to 1.9; p = 0.03) independently predicted death. Conclusions Children with DCM have improved survival in the more recent era. This appears to be associated with factors other than heart transplantation, which was equally prevalent in both eras. (Pediatric Cardiomyopathy Registry [PCMR]; NCT00005391)

KW - cardiomyopathy

KW - echocardiography

KW - heart failure

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