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Outcomes of children with cardiomyopathy listed for transplant: a multi-institutional study

Anne I Dipchand1, David C Naftel, Brian Feingold

  • 1Hospital for Sick Children, Ontario, Canada. anne.dipchand@sickkids.ca

The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation
|September 29, 2009
PubMed

Insights

Children with cardiomyopathies (CM) awaiting heart transplantation (HTx) have lower waitlist mortality and better survival rates post-transplant compared to non-CM patients. Dilated CM offers the best outcomes, while hypertrophic or restrictive CM in infants has the worst prognosis.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Surgery
  • Transplantation Medicine

Background:

  • Cardiomyopathies (CM) in children, including dilated (DCM), restrictive (RCM), and hypertrophic (HCM) types, present diverse clinical trajectories.
  • Heart transplantation (HTx) is a critical intervention for pediatric CM, but outcomes post-listing require detailed evaluation.

Purpose of the Study:

  • To compare outcomes for children listed for heart transplantation (HTx) based on cardiomyopathy (CM) diagnosis versus non-CM etiologies.
  • To identify risk factors influencing waitlist mortality and post-HTx survival in pediatric patients.

Main Methods:

  • Analysis of a multi-institutional registry data from 3,147 pediatric patients listed for HTx between January 1993 and December 2006.
  • Comparison of outcomes between 1,320 children with CM and 1,827 with non-CM etiologies, with sub-group analysis of DCM, RCM, and HCM.

Main Results:

  • Cardiomyopathy patients exhibited significantly lower waitlist mortality (17% vs. 32%) and improved 10-year survival post-listing (66% vs. 53%) compared to non-CM patients.
  • Younger age, Black race, mechanical ventilation, and extracorporeal membrane oxygenation were risk factors for waitlist mortality.
  • Post-HTx survival was better for CM patients (68% vs. 61%), with early risks including mechanical ventilation and ischemic time, and late risks including Black race, HCM/RCM, and older age.

Conclusions:

  • Children with CM demonstrate superior outcomes in terms of waitlist mortality and post-HTx survival compared to those with non-CM conditions.
  • Dilated cardiomyopathy (DCM) is associated with the most favorable overall outcomes, whereas hypertrophic cardiomyopathy (HCM) or restrictive cardiomyopathy (RCM) in infants (<1 year) carries the highest risk.
Abstract

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