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, Daphne T. Hsu, Jacqueline M. Lamour, J. M. Chen

Research output: Contribution to journalArticle

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Abstract

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
Volume8
Issue number1
DOIs
StatePublished - Jan 2008
Externally publishedYes

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Restrictive Cardiomyopathy
Heart Transplantation
Vascular Resistance
Pressure
Cardiomyopathies
Pulmonary Artery
Survival

Keywords

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

ASJC Scopus subject areas

  • Immunology

Cite this

Bograd, A. J., Mital, S., Schwarzenberger, J. C., Mosca, R. S., Quaegebeur, J. M., Addonizio, L. J., ... Chen, J. M. (2008). Twenty-year experience with heart transplantation for infants and children with restrictive cardiomyopathy: 1986-2006. American Journal of Transplantation, 8(1), 201-207. https://doi.org/10.1111/j.1600-6143.2007.02027.x

Twenty-year experience with heart transplantation for infants and children with restrictive cardiomyopathy : 1986-2006. / Bograd, A. J.; Mital, S.; Schwarzenberger, J. C.; Mosca, R. S.; Quaegebeur, J. M.; Addonizio, L. J.; Hsu, Daphne T.; Lamour, Jacqueline M.; Chen, J. M.

In: American Journal of Transplantation, Vol. 8, No. 1, 01.2008, p. 201-207.

Research output: Contribution to journalArticle

Bograd, A. J. ; Mital, S. ; Schwarzenberger, J. C. ; Mosca, R. S. ; Quaegebeur, J. M. ; Addonizio, L. J. ; Hsu, Daphne T. ; Lamour, Jacqueline M. ; Chen, J. M. / Twenty-year experience with heart transplantation for infants and children with restrictive cardiomyopathy : 1986-2006. In: American Journal of Transplantation. 2008 ; Vol. 8, No. 1. pp. 201-207.
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N2 - 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. × m2 (χ2 = 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.

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