Early predictors of survival to and after heart transplantation in children with dilated cardiomyopathy

Biagio A. Pietra, Paul F. Kantor, Heather L. Bartlett, Clifford Chin, Charles E. Canter, Ranae L. Larsen, R. Erik Edens, Steven D. Colan, Jeffrey A. Towbin, Steven E. Lipshultz, James K. Kirklin, David C. Naftel, Daphne T. Hsu

Research output: Contribution to journalArticle

42 Citations (Scopus)

Abstract

Background-The importance of clinical presentation and pretransplantation course on outcome in children with dilated cardiomyopathy listed for heart transplantation is not well defined. Methods and Results-The impact of age, duration of illness, sex, race, ventricular geometry, and diagnosis of myocarditis on outcome in 261 children with dilated cardiomyopathy enrolled in the Pediatric Cardiomyopathy Registry and Pediatric Heart Transplant Study was studied. End points included listing as United Network for Organ Sharing status 1, death while waiting, and death after transplantation. The median age at the time of diagnosis was 3.4 years, and the mean time from diagnosis to listing was 0.62±1.3 years. Risk factors associated with death while waiting were ventilator use and older age at listing in patients not mechanically ventilated (P=0.0006 and P=0.03, respectively). Shorter duration of illness (P=0.04) was associated with listing as United Network for Organ Sharing status 1. Death after transplantation was associated with myocarditis at presentation (P=0.009), nonwhite race (P<0.0001), and a lower left ventricular end-diastolic dimension z score at presentation (P=0.04). In the myocarditis group, 17% (4 of 23) died of acute rejection after transplantation. Conclusions-Mechanical ventilator use and older age at listing predicted death while waiting, whereas nonwhite race, smaller left ventricular dimension, and myocarditis were associated with death after transplantation. Although 97% of children with clinically or biopsy-diagnosed myocarditis at presentation survived to transplantation, they had significantly higher posttransplantation mortality compared with children without myocarditis, raising the possibility that preexisting viral infection or inflammation adversely affects graft survival.

Original languageEnglish (US)
Pages (from-to)1079-1086
Number of pages8
JournalCirculation
Volume126
Issue number9
DOIs
StatePublished - Aug 21 2012

Fingerprint

Myocarditis
Dilated Cardiomyopathy
Heart Transplantation
Survival
Transplantation
Mechanical Ventilators
Pediatrics
Graft Rejection
Graft Survival
Virus Diseases
Cardiomyopathies
Registries
Inflammation
Transplants
Biopsy
Mortality

Keywords

  • Cardiomyopathy
  • Dilated
  • Heart transplantation
  • Myocarditis
  • Pediatrics

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Cite this

Early predictors of survival to and after heart transplantation in children with dilated cardiomyopathy. / Pietra, Biagio A.; Kantor, Paul F.; Bartlett, Heather L.; Chin, Clifford; Canter, Charles E.; Larsen, Ranae L.; Edens, R. Erik; Colan, Steven D.; Towbin, Jeffrey A.; Lipshultz, Steven E.; Kirklin, James K.; Naftel, David C.; Hsu, Daphne T.

In: Circulation, Vol. 126, No. 9, 21.08.2012, p. 1079-1086.

Research output: Contribution to journalArticle

Pietra, BA, Kantor, PF, Bartlett, HL, Chin, C, Canter, CE, Larsen, RL, Edens, RE, Colan, SD, Towbin, JA, Lipshultz, SE, Kirklin, JK, Naftel, DC & Hsu, DT 2012, 'Early predictors of survival to and after heart transplantation in children with dilated cardiomyopathy', Circulation, vol. 126, no. 9, pp. 1079-1086. https://doi.org/10.1161/CIRCULATIONAHA.110.011999
Pietra, Biagio A. ; Kantor, Paul F. ; Bartlett, Heather L. ; Chin, Clifford ; Canter, Charles E. ; Larsen, Ranae L. ; Edens, R. Erik ; Colan, Steven D. ; Towbin, Jeffrey A. ; Lipshultz, Steven E. ; Kirklin, James K. ; Naftel, David C. ; Hsu, Daphne T. / Early predictors of survival to and after heart transplantation in children with dilated cardiomyopathy. In: Circulation. 2012 ; Vol. 126, No. 9. pp. 1079-1086.
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abstract = "Background-The importance of clinical presentation and pretransplantation course on outcome in children with dilated cardiomyopathy listed for heart transplantation is not well defined. Methods and Results-The impact of age, duration of illness, sex, race, ventricular geometry, and diagnosis of myocarditis on outcome in 261 children with dilated cardiomyopathy enrolled in the Pediatric Cardiomyopathy Registry and Pediatric Heart Transplant Study was studied. End points included listing as United Network for Organ Sharing status 1, death while waiting, and death after transplantation. The median age at the time of diagnosis was 3.4 years, and the mean time from diagnosis to listing was 0.62±1.3 years. Risk factors associated with death while waiting were ventilator use and older age at listing in patients not mechanically ventilated (P=0.0006 and P=0.03, respectively). Shorter duration of illness (P=0.04) was associated with listing as United Network for Organ Sharing status 1. Death after transplantation was associated with myocarditis at presentation (P=0.009), nonwhite race (P<0.0001), and a lower left ventricular end-diastolic dimension z score at presentation (P=0.04). In the myocarditis group, 17{\%} (4 of 23) died of acute rejection after transplantation. Conclusions-Mechanical ventilator use and older age at listing predicted death while waiting, whereas nonwhite race, smaller left ventricular dimension, and myocarditis were associated with death after transplantation. Although 97{\%} of children with clinically or biopsy-diagnosed myocarditis at presentation survived to transplantation, they had significantly higher posttransplantation mortality compared with children without myocarditis, raising the possibility that preexisting viral infection or inflammation adversely affects graft survival.",
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AU - Pietra, Biagio A.

AU - Kantor, Paul F.

AU - Bartlett, Heather L.

AU - Chin, Clifford

AU - Canter, Charles E.

AU - Larsen, Ranae L.

AU - Edens, R. Erik

AU - Colan, Steven D.

AU - Towbin, Jeffrey A.

AU - Lipshultz, Steven E.

AU - Kirklin, James K.

AU - Naftel, David C.

AU - Hsu, Daphne T.

PY - 2012/8/21

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N2 - Background-The importance of clinical presentation and pretransplantation course on outcome in children with dilated cardiomyopathy listed for heart transplantation is not well defined. Methods and Results-The impact of age, duration of illness, sex, race, ventricular geometry, and diagnosis of myocarditis on outcome in 261 children with dilated cardiomyopathy enrolled in the Pediatric Cardiomyopathy Registry and Pediatric Heart Transplant Study was studied. End points included listing as United Network for Organ Sharing status 1, death while waiting, and death after transplantation. The median age at the time of diagnosis was 3.4 years, and the mean time from diagnosis to listing was 0.62±1.3 years. Risk factors associated with death while waiting were ventilator use and older age at listing in patients not mechanically ventilated (P=0.0006 and P=0.03, respectively). Shorter duration of illness (P=0.04) was associated with listing as United Network for Organ Sharing status 1. Death after transplantation was associated with myocarditis at presentation (P=0.009), nonwhite race (P<0.0001), and a lower left ventricular end-diastolic dimension z score at presentation (P=0.04). In the myocarditis group, 17% (4 of 23) died of acute rejection after transplantation. Conclusions-Mechanical ventilator use and older age at listing predicted death while waiting, whereas nonwhite race, smaller left ventricular dimension, and myocarditis were associated with death after transplantation. Although 97% of children with clinically or biopsy-diagnosed myocarditis at presentation survived to transplantation, they had significantly higher posttransplantation mortality compared with children without myocarditis, raising the possibility that preexisting viral infection or inflammation adversely affects graft survival.

AB - Background-The importance of clinical presentation and pretransplantation course on outcome in children with dilated cardiomyopathy listed for heart transplantation is not well defined. Methods and Results-The impact of age, duration of illness, sex, race, ventricular geometry, and diagnosis of myocarditis on outcome in 261 children with dilated cardiomyopathy enrolled in the Pediatric Cardiomyopathy Registry and Pediatric Heart Transplant Study was studied. End points included listing as United Network for Organ Sharing status 1, death while waiting, and death after transplantation. The median age at the time of diagnosis was 3.4 years, and the mean time from diagnosis to listing was 0.62±1.3 years. Risk factors associated with death while waiting were ventilator use and older age at listing in patients not mechanically ventilated (P=0.0006 and P=0.03, respectively). Shorter duration of illness (P=0.04) was associated with listing as United Network for Organ Sharing status 1. Death after transplantation was associated with myocarditis at presentation (P=0.009), nonwhite race (P<0.0001), and a lower left ventricular end-diastolic dimension z score at presentation (P=0.04). In the myocarditis group, 17% (4 of 23) died of acute rejection after transplantation. Conclusions-Mechanical ventilator use and older age at listing predicted death while waiting, whereas nonwhite race, smaller left ventricular dimension, and myocarditis were associated with death after transplantation. Although 97% of children with clinically or biopsy-diagnosed myocarditis at presentation survived to transplantation, they had significantly higher posttransplantation mortality compared with children without myocarditis, raising the possibility that preexisting viral infection or inflammation adversely affects graft survival.

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KW - Dilated

KW - Heart transplantation

KW - Myocarditis

KW - Pediatrics

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