title: Evaluating failing Fontans for heart transplantation: predictors of death.
abstract: BACKGROUND
  Late complications of the Fontan operation represent a significant management challenge. Failing Fontan patients have two modes of presentation: impaired ventricular function (IVF) and those with preserved ventricular function (PVF) but with failing Fontan physiology (protein-losing enteropathy [PLE] and plastic bronchitis [PB]). This study evaluated whether failing Fontan patients referred for heart transplantation had a different outcome based on the mode of presentation.
  
  
  METHODS
  The medical records of all Fontan patients evaluated for heart transplantation at a single institution from 1994 to 2008 were retrospectively reviewed. Demographic, hemodynamic, and laboratory data were collected. Patients were stratified into an IVF or PVF group by echocardiographic criteria. Descriptive statistics and Kaplan-Meier analysis were used for hypothesis testing.
  
  
  RESULTS
  Thirty-four Fontan patients were evaluated for heart transplantation. According to echo description of systolic function, 18 were categorized as IVF and 16 as PVF. The IVF group had a significantly lower cardiac index and venous oxygen saturation, and significantly higher systemic vascular resistance vs the PVF group (p < 0.05). PLE or PB was present in 13 PVF patients and none in the IVF group. Twenty patients underwent transplantation, with similar rates amongst the IVF and PVF groups. Within 1 year from evaluation, 2 IVG patients and 7 PVF patients had died (p = 0.052).
  
  
  CONCLUSIONS
  Failing Fontan patients with PVF have decreased overall survival independent of whether they underwent transplantation. This trend indicates a need to improve the management and timing for transplantation amongst this population.
authors.affiliation: Department of Cardiac Surgery Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA.
authors.forename: Eric R
authors.initials: ER
authors.lastname: Griffiths
authors.forename: Aditya K
authors.initials: AK
authors.lastname: Kaza
authors.forename: Moritz C
authors.initials: MC
authors.lastname: Wyler von Ballmoos
authors.forename: Hugo
authors.initials: H
authors.lastname: Loyola
authors.forename: Anne Marie
authors.initials: AM
authors.lastname: Valente
authors.forename: Elizabeth D
authors.initials: ED
authors.lastname: Blume
authors.forename: Pedro
authors.initials: P
authors.lastname: del Nido
mesh_terms: D000293:Adolescent; D001991:Bronchitis / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000601:surgery; D016027:Heart Transplantation; D006439:Hemodynamics; D006801:Humans; D008297:Male; D018579:Patient Selection; D011504:Protein-Losing Enteropathies / Q000453:epidemiology; D051437:Renal Insufficiency / Q000453:epidemiology; D012189:Retrospective Studies; D017211:Treatment Failure; D018754:Ventricular Dysfunction / Q000453:epidemiology; D016276:Ventricular Function; D055815:Young Adult