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Pediatric Cardiology

, Volume 40, Issue 4, pp 694–704 | Cite as

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

  • Samuel G. WittekindEmail author
  • Thomas D. Ryan
  • Zhiqian Gao
  • Farhan Zafar
  • Richard J. Czosek
  • Clifford W. Chin
  • John L. Jefferies
Original Article
  • 145 Downloads

Abstract

Background

Pediatric restrictive cardiomyopathy (RCM) has high mortality in historical cohorts, and traditional management often involves early referral for heart transplantation (HTx). This study sought to determine outcomes of pediatric RCM at a center that has favored medical management over early listing for HTx.

Methods

All patients (N = 43) with pure RCM phenotype (RCM, N = 26) and hypertrophic cardiomyopathy with restrictive physiology (RCM/HCM, N = 17) managed at our center over a 15-year period were investigated. Outcomes of those listed for HTx (N = 18) were compared to a benchmark of contemporaneous pediatric RCM patients in the UNOS database (N = 377). Proportional hazards models were used to determine predictors of adverse outcomes.

Results

The mean age was 11 ± 9 years and 49% were male. 14 of 18 patients listed received HTx. Overall mortality (12%) was identical between the phenotypes; however, RCM patients were more likely to be listed (P = 0.001) and receive HTx (P = 0.02) compared to RCM/HCM. Prior to HTx, 60% had documented arrhythmia, 16% had cardiac arrest, and 7% required mechanical circulatory support. 4 of 17 patients with an ICD/PM received device therapies (four of five shocks appropriate for VT/VF, and two effective anti-tachycardia pacing interventions). Outcomes of those listed for HTx at our center were similar to the UNOS benchmark. In multivariate analysis, markers of congestive heart failure were associated with adverse outcomes.

Conclusion

Heart failure and arrhythmia treatments can delay or possibly prevent the need for HTx in some cases of pediatric RCM. Survival post-HTx is not compromised using this approach.

Keywords

Cardiomyopathy Heart failure Heart transplantation Implantable cardioverter defibrillator Pediatrics 

Notes

Acknowledgements

We acknowledge Tricia A. Hengehold, BS for her substantial efforts in data collection.

Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Research Involving Human and Animal Participants

This article does not contain any studies with animals performed by any of the authors.

Informed Consent

Informed consent was obtained from all individual participants included in the study.

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Cincinnati Children’s Hospital Medical Center, Heart InstituteCincinnatiUSA
  2. 2.Department of PediatricsUniversity of Cincinnati College of MedicineCincinnatiUSA
  3. 3.Cincinnati Children’s Hospital Medical Center, Heart Institute, Heart Failure/Transplant ProgramCincinnatiUSA

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