Outcome of Pediatric Patients With Dilated Cardiomyopathy Listed for Transplant

A Multi-institutional Study

Richard Kirk, David Naftel, Timothy M. Hoffman, Christopher Almond, Gerard Boyle, Randall L. Caldwell, James K. Kirklin, Kirstie White, Anne I. Dipchand

Research output: Contribution to journalArticle

46 Citations (Scopus)

Abstract

Background: The course of dilated cardiomyopathy (DCM) leading to heart failure in children varies; survival with conventional treatment is 64% at 5 years. Heart transplantation (HTx) enables improved survival; however, outcomes from listing for transplant are not well described. This study reports survival of patients with DCM from listing with the availability of mechanical bridge to transplant. Methods: Patients with a primary diagnosis of DCM (n = 1,098) were identified from a multi-institutional, prospective, registry of patients aged < 18 years listed for HTx from January 1, 1993, to December 31, 2006. Results: Characteristics of DCM patients at listing included a mean age of 7.3 years; 51% male, 64% white ethnicity, 77% United Network for Organ Sharing status I, 66% on inotropic support, 28% mechanically ventilated, and 15% on mechanical support. Waitlist mortality was 11%, and 75% underwent HTx at 2 years after listing. Overall 10-year survival after listing was 72%, with higher risk of death associated with arrhythmias, mechanical ventilation, and extracorporeal membrane oxygenation (ECMO) support, but not ventricular assist device (VAD) support. Survival at 10 years post-HTx was 72%, with a higher risk of death associated with black race, older age, mechanical ventilation, longer ischemic time, and earlier era of transplant. Conclusions: Transplantation for DCM in the pediatric population offers enhanced survival compared with the natural history. Overall waitlist mortality for DCM is low, with the exception of patients on ECMO, mechanically ventilated, or with arrhythmias. DCM patients fared well after transplant, making HTx a key therapeutic intervention.

Original languageEnglish (US)
Pages (from-to)1322-1328
Number of pages7
JournalJournal of Heart and Lung Transplantation
Volume28
Issue number12
DOIs
StatePublished - Dec 1 2009

Fingerprint

Dilated Cardiomyopathy
Pediatrics
Transplants
Survival
Extracorporeal Membrane Oxygenation
Artificial Respiration
Cardiac Arrhythmias
Heart-Assist Devices
Mortality
Heart Transplantation
Natural History
Registries
Heart Failure
Transplantation
Therapeutics
Population

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine
  • Transplantation

Cite this

Outcome of Pediatric Patients With Dilated Cardiomyopathy Listed for Transplant : A Multi-institutional Study. / Kirk, Richard; Naftel, David; Hoffman, Timothy M.; Almond, Christopher; Boyle, Gerard; Caldwell, Randall L.; Kirklin, James K.; White, Kirstie; Dipchand, Anne I.

In: Journal of Heart and Lung Transplantation, Vol. 28, No. 12, 01.12.2009, p. 1322-1328.

Research output: Contribution to journalArticle

Kirk, R, Naftel, D, Hoffman, TM, Almond, C, Boyle, G, Caldwell, RL, Kirklin, JK, White, K & Dipchand, AI 2009, 'Outcome of Pediatric Patients With Dilated Cardiomyopathy Listed for Transplant: A Multi-institutional Study', Journal of Heart and Lung Transplantation, vol. 28, no. 12, pp. 1322-1328. https://doi.org/10.1016/j.healun.2009.05.027
Kirk, Richard ; Naftel, David ; Hoffman, Timothy M. ; Almond, Christopher ; Boyle, Gerard ; Caldwell, Randall L. ; Kirklin, James K. ; White, Kirstie ; Dipchand, Anne I. / Outcome of Pediatric Patients With Dilated Cardiomyopathy Listed for Transplant : A Multi-institutional Study. In: Journal of Heart and Lung Transplantation. 2009 ; Vol. 28, No. 12. pp. 1322-1328.
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abstract = "Background: The course of dilated cardiomyopathy (DCM) leading to heart failure in children varies; survival with conventional treatment is 64{\%} at 5 years. Heart transplantation (HTx) enables improved survival; however, outcomes from listing for transplant are not well described. This study reports survival of patients with DCM from listing with the availability of mechanical bridge to transplant. Methods: Patients with a primary diagnosis of DCM (n = 1,098) were identified from a multi-institutional, prospective, registry of patients aged < 18 years listed for HTx from January 1, 1993, to December 31, 2006. Results: Characteristics of DCM patients at listing included a mean age of 7.3 years; 51{\%} male, 64{\%} white ethnicity, 77{\%} United Network for Organ Sharing status I, 66{\%} on inotropic support, 28{\%} mechanically ventilated, and 15{\%} on mechanical support. Waitlist mortality was 11{\%}, and 75{\%} underwent HTx at 2 years after listing. Overall 10-year survival after listing was 72{\%}, with higher risk of death associated with arrhythmias, mechanical ventilation, and extracorporeal membrane oxygenation (ECMO) support, but not ventricular assist device (VAD) support. Survival at 10 years post-HTx was 72{\%}, with a higher risk of death associated with black race, older age, mechanical ventilation, longer ischemic time, and earlier era of transplant. Conclusions: Transplantation for DCM in the pediatric population offers enhanced survival compared with the natural history. Overall waitlist mortality for DCM is low, with the exception of patients on ECMO, mechanically ventilated, or with arrhythmias. DCM patients fared well after transplant, making HTx a key therapeutic intervention.",
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AU - Kirk, Richard

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AU - Hoffman, Timothy M.

AU - Almond, Christopher

AU - Boyle, Gerard

AU - Caldwell, Randall L.

AU - Kirklin, James K.

AU - White, Kirstie

AU - Dipchand, Anne I.

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N2 - Background: The course of dilated cardiomyopathy (DCM) leading to heart failure in children varies; survival with conventional treatment is 64% at 5 years. Heart transplantation (HTx) enables improved survival; however, outcomes from listing for transplant are not well described. This study reports survival of patients with DCM from listing with the availability of mechanical bridge to transplant. Methods: Patients with a primary diagnosis of DCM (n = 1,098) were identified from a multi-institutional, prospective, registry of patients aged < 18 years listed for HTx from January 1, 1993, to December 31, 2006. Results: Characteristics of DCM patients at listing included a mean age of 7.3 years; 51% male, 64% white ethnicity, 77% United Network for Organ Sharing status I, 66% on inotropic support, 28% mechanically ventilated, and 15% on mechanical support. Waitlist mortality was 11%, and 75% underwent HTx at 2 years after listing. Overall 10-year survival after listing was 72%, with higher risk of death associated with arrhythmias, mechanical ventilation, and extracorporeal membrane oxygenation (ECMO) support, but not ventricular assist device (VAD) support. Survival at 10 years post-HTx was 72%, with a higher risk of death associated with black race, older age, mechanical ventilation, longer ischemic time, and earlier era of transplant. Conclusions: Transplantation for DCM in the pediatric population offers enhanced survival compared with the natural history. Overall waitlist mortality for DCM is low, with the exception of patients on ECMO, mechanically ventilated, or with arrhythmias. DCM patients fared well after transplant, making HTx a key therapeutic intervention.

AB - Background: The course of dilated cardiomyopathy (DCM) leading to heart failure in children varies; survival with conventional treatment is 64% at 5 years. Heart transplantation (HTx) enables improved survival; however, outcomes from listing for transplant are not well described. This study reports survival of patients with DCM from listing with the availability of mechanical bridge to transplant. Methods: Patients with a primary diagnosis of DCM (n = 1,098) were identified from a multi-institutional, prospective, registry of patients aged < 18 years listed for HTx from January 1, 1993, to December 31, 2006. Results: Characteristics of DCM patients at listing included a mean age of 7.3 years; 51% male, 64% white ethnicity, 77% United Network for Organ Sharing status I, 66% on inotropic support, 28% mechanically ventilated, and 15% on mechanical support. Waitlist mortality was 11%, and 75% underwent HTx at 2 years after listing. Overall 10-year survival after listing was 72%, with higher risk of death associated with arrhythmias, mechanical ventilation, and extracorporeal membrane oxygenation (ECMO) support, but not ventricular assist device (VAD) support. Survival at 10 years post-HTx was 72%, with a higher risk of death associated with black race, older age, mechanical ventilation, longer ischemic time, and earlier era of transplant. Conclusions: Transplantation for DCM in the pediatric population offers enhanced survival compared with the natural history. Overall waitlist mortality for DCM is low, with the exception of patients on ECMO, mechanically ventilated, or with arrhythmias. DCM patients fared well after transplant, making HTx a key therapeutic intervention.

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