title: Survival and quality of life for children with end-stage heart failure who are not candidates for cardiac transplant.
abstract: BACKGROUND
  Some pediatric patients referred for heart transplant (HTx) are sub-optimal candidates. Their outcomes without HTx are presumed to be dismal, but have not been well described. Knowledge about their outcomes is critical when weighing the risks between a high-risk transplant and "terminal" palliation.
  
  
  METHODS
  We retrospectively reviewed all HTx referrals from January 2005 to July 2013. We excluded those who were listed for HTx, or who were denied HTx due to being "too well," seeking only those who were in need of but not suitable for HTx. End-points included mortality and length of survival.
  
  
  RESULTS
  Of 212 referrals, 39 (19%) (age 0 to 19 years, median 3.5 years) were denied HTx for reasons other than being too well. Twenty-eight (72%) had palliated congenital heart disease. Overall mortality during the follow-up period was 38% (n = 15) with a median follow-up time of 195 days (8 to 2,832 days). Ten patients received subsequent cardiac surgery with 1 death (10%) and median follow-up of 2.6 years. Mortality risk was not influenced by age, weight, growth failure, congenital heart disease or single-ventricle physiology. Mechanical ventilation (hazard ratio 6.31, p = 0.001) and inotrope dependence (hazard ratio 4.79, p = 0.006) were associated with the highest risk of mortality. Quality of life was measured with the PedsQL cardiac module and completed by 11 of 16 eligible patients with an overall average score of 70.2 ± 23.9.
  
  
  CONCLUSIONS
  An advanced heart failure program can achieve satisfactory results for pediatric patients who are not suitable candidates for HTx. For some children, high-risk palliative surgery can result in better outcome than high-risk HTx. Mortality was related to the degree of heart failure at presentation rather than underlying heart disease.
authors.affiliation: Division of Cardiology. Electronic address: asabati@chla.usc.edu.
authors.forename: Arash Alen
authors.initials: AA
authors.lastname: Sabati
authors.affiliation: Division of Cardiology.
authors.forename: Jacqueline R
authors.initials: JR
authors.lastname: Szmuszkovicz
authors.affiliation: Department of Cardiothoracic Surgery.
authors.forename: Cynthia
authors.initials: C
authors.lastname: Herrington
authors.affiliation: Division of Cardiology.
authors.forename: Mark
authors.initials: M
authors.lastname: Hermes
authors.affiliation: Division of Cardiology.
authors.forename: Hesham A
authors.initials: HA
authors.lastname: Mahmoud
authors.affiliation: Clinical Investigation Center at the Saban Research Institute; Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, California.
authors.forename: Choo
authors.initials: C
authors.lastname: Phei Wee
authors.affiliation: Department of Cardiothoracic Surgery.
authors.forename: Vaughn A
authors.initials: VA
authors.lastname: Starnes
authors.affiliation: Division of Cardiology.
authors.forename: JonDavid
authors.initials: J
authors.lastname: Menteer
mesh_terms: D000293:Adolescent; D002140:California / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000401:mortality* / Q000523:psychology / Q000628:therapy; D016027:Heart Transplantation; D006353:Heart-Assist Devices; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D011788:Quality of Life; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: Pediatric heart failure; alloimmunization; congenital heart disease; congenital heart surgery; panel reactive antibodies; pediatric heart transplant