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Twenty‐Year Experience With Heart Transplantation for Infants and Children With Restrictive Cardiomyopathy: 1986–2006
Author(s) -
Bograd A. J.,
Mital S.,
Schwarzenberger J. C.,
Mosca R. S.,
Quaegebeur J. M.,
Addonizio L. J.,
Hsu D. T.,
Lamour J. M.,
Chen J. M.
Publication year - 2008
Publication title -
american journal of transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.89
H-Index - 188
eISSN - 1600-6143
pISSN - 1600-6135
DOI - 10.1111/j.1600-6143.2007.02027.x
Subject(s) - medicine , vascular resistance , heart transplantation , cardiology , cardiomyopathy , pulmonary artery , restrictive cardiomyopathy , transplantation , diastole , blood pressure , pulmonary hypertension , surgery , heart failure
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. × m 2 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. × m 2 , respectively. At time of most recent follow‐up (mean = 5.7 ± 4.6 years), the mean PVRI was 2.0 ± 1.0 W.U. × m 2 . Increasing preoperative mean pulmonary artery pressure (PA) pressure (p = 0.04) and PVRI > 6 W.U. × m 2 (χ 2 = 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. × m 2 survived the first postoperative year. We report excellent survival for patients undergoing HTX for RCM despite the high proportion of high‐risk patients.