Twenty-Year Experience With Heart Transplantation for Infants and Children With Restrictive Cardiomyopathy: 1986–2006 |
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Authors: | 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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Institution: | Division of Cardiothoracic Surgery, Department of Surgery, College of Physicians and Surgeons, Columbia University, New York, NY;Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada;Department of Anesthesiology, College of Physicians and Surgeons, Columbia University, New York, NY;Division of Cardiology, Department of Pediatrics, College of Physicians and Surgeons, Columbia University, New York, NY;Department of Cardiothoracic Surgery, Weill Medical College of Cornell University, New York, NY |
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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. × 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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Keywords: | Cardiomyopathy cardiothoracic surgery outcomes pediatric cardiomyopathy/heart transplant pediatric heart pediatric heart transplantation |
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