title: Is the "perfect Fontan" operation routinely achievable in the modern era?
abstract: OBJECTIVE
  In 1990, Fontan, Kirklin, and colleagues published equations for survival after the so-called "Perfect Fontan" operation. After 1988, we evolved a protocol using an internal or external polytetraflouroethylene tube of 16 to 19 millimetres diameter placed from the inferior caval vein to either the right or left pulmonary artery along with a bidirectional cava-pulmonary connection. The objective of this study was to test the hypothesis that a "perfect" outcome is routinely achievable in the current era when using a standardized surgical procedure.
  
  
  METHODS
  Between 1 January, 1988, and 12 December, 2005, 112 patients underwent the Fontan procedure using an internal or external polytetraflouroethylene tube plus a bidirectional cava-pulmonary connection, the latter usually having been constructed as a previous procedure. This constituted 45% of our overall experience in constructing the Fontan circulation between 1988 and 1996, and 96% of the experience between 1996 and 2005. Among all surviving patients, the median follow-up was 7.3 years. We calculated the expected survival for an optimal candidate, given from the initial equations, and compared this to our entire experience in constructing the Fontan circulation.
  
  
  RESULTS
  An internal tube was utilized in 61 patients, 97% of whom were operated prior to 1998, and an external tube in 51 patients, the latter accounting for 95% of all operations since 1999. At 1, 5, 10 and 15 years, survival of the entire cohort receiving polytetraflouroethylene tubes is superimposable on the curve calculated for a "perfect" outcome. Freedom from replacement or revision of the tube was 97% at 10 years.
  
  
  CONCLUSIONS
  Using a standardized operative procedure, combining a bidirectional cavopulmonary connection with a polytetraflouroethylene tube placed from the inferior caval vein to the pulmonary arteries for nearly all patients with functionally univentricular hearts, early and late survival within the "perfect" outcome as predicted by the initial equations of Fontan and Kirklin is routinely achievable in the current era. The need for late revision or replacement of the tube is rare.
authors.affiliation: Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, AL 35294, USA. jkirklin@uab.edu
authors.forename: James K
authors.initials: JK
authors.lastname: Kirklin
authors.forename: Robert N
authors.initials: RN
authors.lastname: Brown
authors.forename: Ayesha S
authors.initials: AS
authors.lastname: Bryant
authors.forename: David C
authors.initials: DC
authors.lastname: Naftel
authors.forename: Edward V
authors.initials: EV
authors.lastname: Colvin
authors.forename: F Bennett
authors.initials: FB
authors.lastname: Pearce
authors.forename: Robb L
authors.initials: RL
authors.lastname: Romp
authors.forename: Walter H
authors.initials: WH
authors.lastname: Johnson
authors.forename: Yung R
authors.initials: YR
authors.lastname: Lau
authors.forename: William S
authors.initials: WS
authors.lastname: McMahon
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Knott-Craig
authors.forename: Albert D
authors.initials: AD
authors.lastname: Pacifico
mesh_terms: D018729:Fontan Procedure / Q000295:instrumentation / Q000379:methods / Q000401:mortality; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery; D006801:Humans; D015999:Multivariate Analysis; D011138:Polytetrafluoroethylene; D012086:Reoperation; D016896:Treatment Outcome
chemical_list: D011138:Polytetrafluoroethylene