title: The modern Fontan operation shows no increase in mortality out to 20 years: a new paradigm.
abstract: OBJECTIVE
  Dating back to the first published report of the Fontan circulation in 1971, multiple studies have examined the long-term results of this standard procedure for palliation of single-ventricle heart disease in children. Although the technique has evolved over the last 4 decades to include a polytetrafluorethylene (PTFE) conduit for a large percentage of patients, the long-term outcome has not yet been established. The aim of the current study was to investigate the possibility of a late increasing risk for death after 15 years among patients with a modern Fontan operation and to evaluate late morbidity.
  
  
  METHODS
  Between January 1, 1988, and December 31, 2011, 207 patients underwent the Fontan procedure using an internal or external PTFE conduit plus a bidirectional cavopulmonary connection. Survival and late adverse events were analyzed. Risk factors for early and late mortality were examined using hazard function methodology.
  
  
  RESULTS
  At 1, 10, and 20 years, survival for the entire cohort was 95%, 88%, and 76%, respectively, with no deaths in the last 6 years of the study. Hazard modeling showed a 1.3% risk of death per year 24 years after the Fontan procedure, with no late increasing hazard phase. Freedom from reoperations was greater than 90% at 20 years and freedom from thrombotic complications was 98% at 20 years (with greater than 80% of patients on aspirin alone). Survival curves were superimposable for 16- to 20-mm conduits, and the freedom from any reoperation including transplantation was greater than 90% after 20 years. Multivariable risk factor analysis identified only earlier date of operation as a predictor of early and late mortality. By era of surgery, the 10-year predicated survival is 89% for patients undergoing surgery in 2000 and 94% for patients in 2010.
  
  
  CONCLUSIONS
  Early and late survival after a Fontan operation with a PTFE conduit is excellent, with no late phase of increasing death risk after 20 years. Late functional status is good, the need for late reoperation is rare, and thrombotic complications are uncommon on a standard medical regimen including aspirin as the only anticoagulation medication.
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, University of Alabama at Birmingham, Birmingham, Ala. Electronic address: rdabal@uab.edu.
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Dabal
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, University of Alabama at Birmingham, Birmingham, Ala.
authors.forename: James K
authors.initials: JK
authors.lastname: Kirklin
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, University of Alabama at Birmingham, Birmingham, Ala.
authors.forename: Manisha
authors.initials: M
authors.lastname: Kukreja
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, University of Alabama at Birmingham, Birmingham, Ala.
authors.forename: Robert N
authors.initials: RN
authors.lastname: Brown
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, University of Alabama at Birmingham, Birmingham, Ala.
authors.forename: David C
authors.initials: DC
authors.lastname: Cleveland
authors.affiliation: School of Medicine, University of Alabama at Birmingham, Birmingham, Ala.
authors.forename: Michael C
authors.initials: MC
authors.lastname: Eddins
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, Ala.
authors.forename: Yung
authors.initials: Y
authors.lastname: Lau
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D018729:Fontan Procedure / Q000009:adverse effects / Q000295:instrumentation / Q000401:mortality*; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D006801:Humans; D053208:Kaplan-Meier Estimate; D015999:Multivariate Analysis; D010166:Palliative Care; D011183:Postoperative Complications / Q000401:mortality / Q000601:surgery; D016016:Proportional Hazards Models; D012086:Reoperation; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D017741:Survivors; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants