title: The impact of heart failure severity at time of listing for cardiac transplantation on survival in pediatric cardiomyopathy.
abstract: BACKGROUND
  The survival benefit of heart transplantation in adult heart failure is greatest for the sickest patients and negligible for patients not requiring inotropic or mechanical support. We hypothesized a similar survival benefit of heart transplantation for childhood cardiomyopathies with heart failure.
  
  
  METHODS
  A merged data set of children registered in both the Pediatric Cardiomyopathy Registry and the Pediatric Heart Transplant Study was used to assess differences in mortality before and after transplant in patients with different levels of heart failure severity. Severity was scored 2 if mechanical ventilatory or circulatory support was required, 1 if intravenous inotropes were required, or 0 if no support was required.
  
  
  RESULTS
  For 332 eligible children, 12-month mortality after listing was 9% for those with a severity score of 0 (n = 105), 16% with a score of 1 (n = 118), and 26% with a score of 2 (n = 109; p = 0.002) with a 3%, 8%, and 20% mortality with severity scores at listing of 0, 1, and 2, respectively, occurring before transplant. Patients listed with a score of 0 frequently deteriorated: 50% received an allograft or died before transplant with severity scores of 1 or 2. The risk of deterioration increased with previous surgery (relative risk, 3.84; p = 0.03) in the short-term and with lower left ventricular mass z-score at time of presentation (relative risk, 1.74; p = 0.003) in the longer-term.
  
  
  CONCLUSIONS
  Pediatric cardiomyopathy patients who require high levels of support receive a survival benefit from heart transplantation that is not shared by patients not requiring intravenous inotropic or mechanical support.
authors.affiliation: Department of Pediatrics, Loma Linda University, Loma Linda, California, USA.
authors.forename: Ranae L
authors.initials: RL
authors.lastname: Larsen
authors.forename: Charles E
authors.initials: CE
authors.lastname: Canter
authors.forename: David C
authors.initials: DC
authors.lastname: Naftel
authors.forename: Margaret
authors.initials: M
authors.lastname: Tressler
authors.forename: David N
authors.initials: DN
authors.lastname: Rosenthal
authors.forename: Elizabeth D
authors.initials: ED
authors.lastname: Blume
authors.forename: William T
authors.initials: WT
authors.lastname: Mahle
authors.forename: Delphine
authors.initials: D
authors.lastname: Yung
authors.forename: William R
authors.initials: WR
authors.lastname: Morrow
authors.forename: E John
authors.initials: EJ
authors.lastname: Orav
authors.forename: James D
authors.initials: JD
authors.lastname: Wilkinson
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Towbin
authors.forename: Stephen E
authors.initials: SE
authors.lastname: Lipshultz
mesh_terms: D001243:Assisted Circulation; D009202:Cardiomyopathies / Q000401:mortality*; D002316:Cardiotonic Agents / Q000494:pharmacology / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006333:Heart Failure / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D016027:Heart Transplantation / Q000401:mortality; D006801:Humans; D008297:Male; D011446:Prospective Studies; D012121:Respiration, Artificial; D012306:Risk; D012720:Severity of Illness Index; D015996:Survival Rate; D016896:Treatment Outcome; D014850:Waiting Lists / Q000401:mortality*
chemical_list: D002316:Cardiotonic Agents