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Contemporary Outcomes of Pediatric Restrictive Cardiomyopathy: A Single-Center Experience

Samuel G Wittekind1,2,3, Thomas D Ryan4,5, Zhiqian Gao4

  • 1Cincinnati Children's Hospital Medical Center, Heart Institute, Cincinnati, OH, USA. Samuel.Wittekind@cchmc.org.

Pediatric Cardiology
|December 14, 2018
PubMed

Insights

Medical management of pediatric restrictive cardiomyopathy (RCM) can delay or prevent the need for heart transplantation (HTx). This approach does not compromise survival outcomes post-heart transplantation for pediatric RCM patients.

Area of Science:

  • Pediatric Cardiology
  • Cardiomyopathy Research
  • Heart Transplantation

Background:

  • Pediatric restrictive cardiomyopathy (RCM) historically has high mortality.
  • Traditional management often involves early heart transplantation (HTx) referral.
  • This study evaluates outcomes favoring medical management over early HTx listing.

Purpose of the Study:

  • To determine outcomes of pediatric RCM managed medically.
  • To compare outcomes of medically managed patients to a benchmark for HTx.
  • To identify predictors of adverse outcomes in pediatric RCM.

Main Methods:

  • Retrospective analysis of 43 pediatric patients with RCM or RCM/HCM phenotypes.
  • Comparison of outcomes for 18 listed patients against 377 UNOS database patients.
  • Proportional hazards models used to identify adverse outcome predictors.

Main Results:

  • Overall mortality was 12% and identical between RCM and RCM/HCM phenotypes.
  • Patients with RCM were more likely to be listed and receive HTx than RCM/HCM.
  • Congestive heart failure markers were associated with adverse outcomes in multivariate analysis.

Conclusions:

  • Medical management, including heart failure and arrhythmia treatments, can delay or prevent HTx in pediatric RCM.
  • Survival post-HTx is not compromised by this conservative management approach.
  • This strategy offers a viable alternative to early HTx listing for select pediatric RCM patients.
Abstract

Keywords:
CardiomyopathyHeart failureHeart transplantationImplantable cardioverter defibrillatorPediatrics

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