title: Twenty-year experience with heart transplantation for infants and children with restrictive cardiomyopathy: 1986-2006.
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. x 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. x 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. x m(2). Increasing preoperative mean pulmonary artery pressure (PA) pressure (p = 0.04) and PVRI > 6 W.U. x m(2) (chi(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. x 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.
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, College of Physicians and Surgeons, Columbia University, New York, NY, USA.
authors.forename: A J
authors.initials: AJ
authors.lastname: Bograd
authors.forename: S
authors.initials: S
authors.lastname: Mital
authors.forename: J C
authors.initials: JC
authors.lastname: Schwarzenberger
authors.forename: R S
authors.initials: RS
authors.lastname: Mosca
authors.forename: J M
authors.initials: JM
authors.lastname: Quaegebeur
authors.forename: L J
authors.initials: LJ
authors.lastname: Addonizio
authors.forename: D T
authors.initials: DT
authors.lastname: Hsu
authors.forename: J M
authors.initials: JM
authors.lastname: Lamour
authors.forename: J M
authors.initials: JM
authors.lastname: Chen
mesh_terms: D000293:Adolescent; D002313:Cardiomyopathy, Restrictive / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome