Twenty-year experience with heart transplantation for infants and children with restrictive cardiomyopathy: 1986-2006

A. J. Bograd, S. Mital, J. C. Schwarzenberger, R. S. Mosca, J. M. Quaegebeur, L. J. Addonizio, D. T. Hsu, J. M. Lamour, J. M. Chen

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35 Scopus citations


Idiopathic restrictive cardiomyopathy (RCM) is a rare cardiomyopathy in children notable for severe diastolic dysfunction and progressive elevation of pulmonary vascular resistance (PVR). Traditionally, those with pulmonary vascular resistance indices (PVRI) >6 W.U. × m2 have been precluded from heart transplantation (HTX). The clinical course of all patients transplanted for RCM between 1986 and 2006 were reviewed. Preoperative, intraoperative and postoperative variables were evaluated. A total of 23 patients underwent HTX for RCM, with a mean age of 8.8 ± 5.6 years and a mean time from listing to HTX of 43 ± 60 days. Preoperative and postoperative (114 ± 40 days) PVRI were 5.9 ± 4.4 and 2.9 ± 1.5 W.U. × m2, respectively. At time of most recent follow-up (mean = 5.7 ± 4.6 years), the mean PVRI was 2.0 ± 1.0 W.U. × m2. Increasing preoperative mean pulmonary artery pressure (PA) pressure (p = 0.04) and PVRI > 6 W.U. × m22 = 7.4, p < 0.01) were associated with the requirement of ECMO postoperatively. Neither PVRI nor mean PA pressure was associated with posttransplant mortality; 30-day and 1-year actuarial survivals were 96% and 86%, respectively. Five of the seven patients with preoperative PVRI > 6 W.U. × m2 survived the first postoperative year. We report excellent survival for patients undergoing HTX for RCM despite the high proportion of high-risk patients.

Original languageEnglish (US)
Pages (from-to)201-207
Number of pages7
JournalAmerican Journal of Transplantation
Issue number1
StatePublished - Jan 2008
Externally publishedYes


  • Cardiomyopathy
  • Cardiothoracic surgery
  • Outcomes
  • Pediatric cardiomyopathy/heart transplant
  • Pediatric heart
  • Pediatric heart transplantation

ASJC Scopus subject areas

  • Immunology and Allergy
  • Transplantation
  • Pharmacology (medical)


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