title: Outcomes for children with cardiomyopathy awaiting transplantation.
abstract: OBJECTIVE
  To determine factors associated with outcomes after listing for transplantation in children with cardiomyopathies.
  
  
  BACKGROUND
  Childhood cardiomyopathies form a heterogeneous group of diseases, and in many, the prognosis is poor, irrespective of the etiology. When profound heart failure develops, cardiac transplantation can be the only viable option for survival.
  
  
  METHODS
  We included all children with cardiomyopathy listed for transplantation between 12/89 and 4/98 in this historical cohort study.
  
  
  RESULTS
  We listed 31 patients, 15 male and 16 female, 27 with dilated and 4 with restrictive cardiomyopathy, for transplantation. The median age at listing was 5.7 years, with a range from fetal life to 17.8 years. Transplantation was achieved in 23 (74%), with a median interval from listing of 54 days, and a range from zero to 11.4 years. Of the patients, 14 were transplanted within 30 days of listing. Five patients (16%) died before transplantation. Within the Canadian algorithm, one of these was in the third state, and four in the fourth state. One patient was removed from the list after 12 days, having recovered from myocarditis, and two remain waiting transplantation after intervals of 121 and 476 days, respectively. Patients who died were more likely to be female (5/5 vs. 11/26; p=0.04) and to have been in the third or fourth states at listing (5/5 vs. 15/26; p=0.04). The use of mechanical ventricular assistance, in 10 patients, was not a predictor of an adverse outcome. While not statistically significant, survival to transplantation was associated with treatment using inhibitors of angiotensin converting enzyme, less mitral regurgitation, a higher mean ejection fraction and cardiac index, and lower right ventricular systolic pressure.
  
  
  CONCLUSIONS
  Children with cardiomyopathy awaiting transplantation have a mortality of 16% related to their clinical state at the time of listing.
authors.affiliation: Department of Paediatrics, The Hospital for Sick Children, The University of Toronto School of Medicine, Ontario, Canada.
authors.forename: L E
authors.initials: LE
authors.lastname: Nield
authors.forename: B W
authors.initials: BW
authors.lastname: McCrindle
authors.forename: D J
authors.initials: DJ
authors.lastname: Bohn
authors.forename: L J
authors.initials: LJ
authors.lastname: West
authors.forename: J G
authors.initials: JG
authors.lastname: Coles
authors.forename: R M
authors.initials: RM
authors.lastname: Freedom
authors.forename: L N
authors.initials: LN
authors.lastname: Benson
mesh_terms: D000293:Adolescent; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D002311:Cardiomyopathy, Dilated / Q000150:complications* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D002313:Cardiomyopathy, Restrictive / Q000150:complications* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008944:Mitral Valve Insufficiency / Q000209:etiology; D009864:Ontario / Q000453:epidemiology; D011379:Prognosis; D013318:Stroke Volume; D016019:Survival Analysis; D013599:Systole; D017725:Ventricular Pressure; D014850:Waiting Lists
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors