title: Fontan palliation in the modern era: factors impacting mortality and morbidity.
abstract: BACKGROUND
  Advances in management of the Fontan patient include interval superior cavopulmonary shunt, total cavopulmonary connection, either lateral tunnel or extracardiac conduit, and the use of a fenestration. Coincident with these improvements, Fontan palliation has been applied to a wider ranger of anatomic subgroups.
  
  
  METHODS
  A cross-sectional analysis of 256 consecutive patients undergoing a total cavopulmonary connection Fontan after superior cavopulmonary shunt between January 1, 1994, and June 30, 2007 were studied. Fenestration was used selectively. Fontan failure was defined as death, transplant, or takedown. Event-free survival was defined as freedom from death, transplant, Fontan takedown, functional class III to IV, pacemaker, antiarrhythmic medication, protein-losing enteropathy, stroke, or thrombus.
  
  
  RESULTS
  Survival was 97% +/- 1%, 96% +/- 1%, and 94% +/- 2%, respectively, at 1, 5, and 10 years. Event-free survival was 96% +/- 1%, 87% +/- 3%, and 64% +/- 6%, respectively, at 1, 5, and 10 years. Factors predicting worse event-free survival included longer cross-clamp time (p = 0.003), fenestration (p = 0.014), and longer hospital length of stay (p = 0.016). Ventricular morphology did not predict outcome. Left ventricle (n = 113, 44%) versus right ventricle (n = 142, 56%) failure-free survival (death, transplant, or Fontan takedown) at 10 years was 92% +/- 4% versus 91% +/- 3%, respectively (p = 0.19). Left ventricle versus right ventricle event-free survival at 10 years was 75% +/- 7% versus 67% +/- 9%, respectively (p > 0.1).
  
  
  CONCLUSIONS
  Survival for patients undergoing a completion Fontan in the current era is excellent, but patients remain at risk for morbid events. In the intermediate follow-up period, we could not identify a difference in outcome between dominant left and right ventricle morphology.
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, Sections of Pediatric Cardiology and Critical Care, Medical College of Wisconsin and Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, Wisconsin 53226, USA. jtweddell@chw.org
authors.forename: James S
authors.initials: JS
authors.lastname: Tweddell
authors.forename: Matthew
authors.initials: M
authors.lastname: Nersesian
authors.forename: Kathleen A
authors.initials: KA
authors.lastname: Mussatto
authors.forename: Melodee
authors.initials: M
authors.lastname: Nugent
authors.forename: Pippa
authors.initials: P
authors.lastname: Simpson
authors.forename: Michael E
authors.initials: ME
authors.lastname: Mitchell
authors.forename: Nancy S
authors.initials: NS
authors.lastname: Ghanayem
authors.forename: Andrew N
authors.initials: AN
authors.lastname: Pelech
authors.forename: Rammohan
authors.initials: R
authors.lastname: Marla
authors.forename: George M
authors.initials: GM
authors.lastname: Hoffman
mesh_terms: D002675:Child, Preschool; D003430:Cross-Sectional Studies; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods*; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D008297:Male; D009017:Morbidity / Q000639:trends; D011183:Postoperative Complications / Q000453:epidemiology*; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D016896:Treatment Outcome; D014922:Wisconsin / Q000453:epidemiology