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Pediatric heart transplantation for congenital and restrictive cardiomyopathy

Bari Murtuza1, Matthew Fenton, Michael Burch

  • 1Department of Cardiac Surgery, Great Ormond Street Hospital for Children, London, United Kingdom.

The Annals of Thoracic Surgery
|April 9, 2013
PubMed

Insights

Pediatric heart transplant outcomes are comparable across congenital heart disease (CHD), restrictive cardiomyopathy (RCM), and dilated cardiomyopathy (DCM) groups. Physiologic factors like pulmonary hypertension (PHT) significantly impact survival more than anatomical complexities.

Area of Science:

  • Pediatric Cardiology
  • Transplantation Medicine
  • Immunology

Background:

  • Recent reports indicate potentially worse outcomes in pediatric heart transplantation for congenital heart disease (CHD) and restrictive cardiomyopathy (RCM).
  • This study compares early outcomes in these patient groups against those with dilated cardiomyopathy (DCM).

Purpose of the Study:

  • To evaluate and compare early outcomes of pediatric orthotopic heart transplantation (OHT) across diverse etiologies: CHD, RCM, and DCM.
  • To identify risk factors influencing mortality and morbidity in these pediatric OHT populations.

Main Methods:

  • A retrospective analysis of 209 pediatric patients undergoing OHT from 2000 to 2011.
  • Data collected included patient demographics, pre-transplant conditions (CHD, RCM, DCM), pulmonary hypertension (PHT), need for mechanical circulatory support (MCS), acute rejection, donor-specific antibodies (DSAs), nondonor-specific antibodies (NDSAs), right ventricular failure (RVF), and survival rates (30-day and 1-year).

Main Results:

  • Thirty-day survival was high across groups (92.0% CHD, 97.1% DCM, 100% RCM), with 1-year survival estimates of 92.0% (CHD), 97.8% (DCM), and 82.6% (RCM).
  • Pulmonary hypertension (PHT) was most prevalent pre-transplant in RCM, while DCM patients most frequently required mechanical support. Right ventricular failure (RVF) was highest in RCM.
  • Multivariable analysis identified age, acute rejection, preoperative PHT, and NDSAs as significant mortality risk factors. NDSAs were significantly more common in CHD and RCM groups.

Conclusions:

  • Pediatric OHT can achieve comparable outcomes despite significant heterogeneity in recipient anatomy and physiology.
  • Physiologic factors, particularly PHT, appear more critical for survival than anatomical complexities.
  • The role of NDSAs in pediatric OHT outcomes warrants further investigation.
Abstract

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