title: Management of early Fontan failure: a single-institution experience.
abstract: OBJECTIVE
  To analyse the incidence and outcomes of early Fontan failure (EFF) in a large contemporary cohort of palliated patients.
  
  
  METHODS
  A retrospective, single-centre study of all patients undergoing primary Fontan from 1 July 1995 to 31 December 2009 was performed. EFF was defined as death, need for extracorporeal membrane oxygenation (ECMO), Fontan takedown to superior cavopulmonary connection (SCPC) or transplantation within 30 days of the Fontan procedure. The incidence and outcomes were summarized with descriptive statistics, and risk factors for EFF were identified.
  
  
  RESULTS
  A total of 592 patients underwent primary Fontan procedure during the study period; 67% had a dominant right ventricle. An extracardiac conduit (ECC) was used for Fontan completion in 60.5%, with the remainder having a lateral tunnel. EFF occurred in 11 patients (1.9%), all of whom had ECC. ECMO was used in 5 patients, 5 had Fontan takedown and 2 had heart transplantation. Five of eleven, or 46%, study subjects died as opposed to an overall mortality for primary Fontan of 0.8%. Among patients who had Fontan takedown to SCPC, long-term survival was 80%. By univariate analysis, elevated ventricular end-diastolic pressure (9.5 ± 3.3 vs 7.4 ± 2.7 mmHg, P = 0.019) and total circulatory support time (99 ± 33 vs 71 ± 23 min, P = 0.001) were risk factors for EFF. The mean follow-up for the 6 hospital survivors was 5.9 years. There was one late transplant-related death. Of the 4 surviving patients who had Fontan takedown to a SCPC, 3 underwent subsequent Fontan completion and 1 underwent biventricular repair.
  
  
  CONCLUSIONS
  EFF is rare in the current era, but is associated with significant mortality. High filling pressures and a prolonged intraoperative course are risk factors for EFF. Of the management strategies available, Fontan takedown to an intermediate pathway appears to be associated with the best outcomes.
authors.affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, PA, USA moamurphy@mac.com.
authors.forename: Michael O
authors.initials: MO
authors.lastname: Murphy
authors.affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, PA, USA.
authors.forename: Andrew C
authors.initials: AC
authors.lastname: Glatz
authors.affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, PA, USA.
authors.forename: David J
authors.initials: DJ
authors.lastname: Goldberg
authors.affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, PA, USA.
authors.forename: Lindsay S
authors.initials: LS
authors.lastname: Rogers
authors.affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, PA, USA.
authors.forename: Chitra
authors.initials: C
authors.lastname: Ravishankar
authors.affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, PA, USA.
authors.forename: Susan C
authors.initials: SC
authors.lastname: Nicolson
authors.affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, PA, USA.
authors.forename: James M
authors.initials: JM
authors.lastname: Steven
authors.affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, PA, USA.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Fuller
authors.affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, PA, USA.
authors.forename: Thomas L
authors.initials: TL
authors.lastname: Spray
authors.affiliation: The Cardiac Center, The Children's Hospital of Philadelphia, PA, USA.
authors.forename: J William
authors.initials: JW
authors.lastname: Gaynor
mesh_terms: D000704:Analysis of Variance; D002675:Child, Preschool; D015199:Extracorporeal Membrane Oxygenation; D018729:Fontan Procedure / Q000379:methods*; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D007223:Infant; D012189:Retrospective Studies; D017211:Treatment Failure
keywords: ECMO; Early Fontan failure; Fontan takedown