title: Pediatric heart transplantation for congenital and restrictive cardiomyopathy.
abstract: BACKGROUND
  Recent reports suggest worse outcomes in pediatric orthotopic heart transplantation (OHT) for congenital heart disease (CHD) and restrictive cardiomyopathy (RCM). We examined early outcomes in these diverse groups of patients in comparison with patients with dilatated cardiomyopathy (DCM).
  
  
  METHODS
  From 2000 to 2011, 209 patients were included: 50 with CHD, 23 with RCM, and 136 with DCM. Early survival was studied, as was the occurrence of acute rejection, donor-specific antibodies (DSAs) and nondonor-specific antibodies (NSDAs), incidence of pulmonary hypertension (PHT), right ventricular failure (RVF), and the need for mechanical circulatory support (MCS).
  
  
  RESULTS
  The incidence of preoperative PHT was greatest in the RCM group (χ(2)p = 0.0006); the requirement for mechanical support before OHT was greatest in patients with DCM. Thirty-day survival was 92.0%, 97.1%, and 100% for patients with CHD, DCM, and RCM respectively. The incidence of RVF was highest for patients with RCM (43.5%; versus CHD, 26.0%; versus DCM, 14.7%). One-year survival estimates for patients with CHD, DCM, and RCM were 92.0%, 97.8%, and 82.6%, respectively (log-rank p = 0.165). Multivariable analysis revealed 4 significant risk factors for mortality: age, incidence of acute rejection, preoperative PHT, and the presence of NDSAs. The occurrence of DSAs was similar, although there was a significantly higher incidence of NDSAs in the CHD and RCM groups (36.0% and 30.4%, respectively, versus 14.0% in the DCM group; χ(2)p = 0.0024).
  
  
  CONCLUSIONS
  Equivalent outcomes are achievable in pediatric OHT despite marked heterogeneity in anatomic and physiologic complexity in recipients. Physiologic factors such as PHT are likely to be more important than anatomic complexities in determining survival. The potential relevance of NDSAs warrants further investigation.
authors.affiliation: Department of Cardiac Surgery, Great Ormond Street Hospital for Children, London, United Kingdom.
authors.forename: Bari
authors.initials: B
authors.lastname: Murtuza
authors.forename: Matthew
authors.initials: M
authors.lastname: Fenton
authors.forename: Michael
authors.initials: M
authors.lastname: Burch
authors.forename: Arun
authors.initials: A
authors.lastname: Gupta
authors.forename: Nagarajan
authors.initials: N
authors.lastname: Muthialu
authors.forename: Martin J
authors.initials: MJ
authors.lastname: Elliott
authors.forename: Tain-Yen
authors.initials: TY
authors.lastname: Hsia
authors.forename: Victor T
authors.initials: VT
authors.lastname: Tsang
authors.forename: Martin
authors.initials: M
authors.lastname: Kostolny
mesh_terms: D000017:ABO Blood-Group System; D000293:Adolescent; D002311:Cardiomyopathy, Dilated / Q000401:mortality / Q000601:surgery; D002313:Cardiomyopathy, Restrictive / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006084:Graft Rejection; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects; D006650:Histocompatibility Testing; D006801:Humans; D007223:Infant; D008297:Male; D009017:Morbidity; D012189:Retrospective Studies
chemical_list: D000017:ABO Blood-Group System