title: Impact of the evolution of the Fontan operation on early and late mortality: a single-center experience of 405 patients over 3 decades.
abstract: BACKGROUND
  Postoperative mortality has decreased in patients undergoing the Fontan operation, and the determinants of such mortality may also have changed significantly.
  
  
  METHODS
  We conducted a study intended to focus on clarifying the determinants of mortality in 405 consecutive patients who had undergone a Fontan operation (62 patients after an atriopulmonary connection, 105 after an intra-atrial rerouting, and 238 patients after an extracardiac rerouting) between 1979 and 2010.
  
  
  RESULTS
  The overall 1-year, 5-year, 10-year, and 15-year rates of survival were 87.1%, 84.6%, 83.4%, and 81.6%, respectively, and the type of procedure as well as heterotaxy syndrome, ventricular ejection fraction, and atrioventricular valve repair at the time of the Fontan operation were independent predictors of overall mortality (p < 0.05 for all). Heterotaxy syndrome and atrioventricular (AV) valve repair were independent predictors of early (less than 6 months postoperative) mortality and the type of procedure was an independent predictor of late (6 or more months postoperative) mortality (p < 0.05 to 0.01). In the era of intra-atrial rerouting, heterotaxy syndrome was the only independent predictor of total, early, and late mortality (p < 0.05 for all), whereas a low ejection fraction, AV valve repair, and repair of a total anomalous pulmonary vein connection, rather than heterotaxy syndrome, were independent predictors of total or early mortality or both (p < 0.05 for all) in the era of extracardiac rerouting.
  
  
  CONCLUSIONS
  Even in the modern era of extracardiac rerouting in the Fontan operation, a low ventricular ejection fraction, AV valve dysfunction, or a total anomalous pulmonary vein connection remain significant risk factors for mortality in patients with a single-ventricle physiology.
authors.affiliation: Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan. hohuchi@hsp.ncvc.go.jp
authors.forename: Hideo
authors.initials: H
authors.lastname: Ohuchi
authors.forename: Koji
authors.initials: K
authors.lastname: Kagisaki
authors.forename: Aya
authors.initials: A
authors.lastname: Miyazaki
authors.forename: Masataka
authors.initials: M
authors.lastname: Kitano
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yazaki
authors.forename: Heima
authors.initials: H
authors.lastname: Sakaguchi
authors.forename: Hajime
authors.initials: H
authors.lastname: Ichikawa
authors.forename: Osamu
authors.initials: O
authors.lastname: Yamada
authors.forename: Toshikatsu
authors.initials: T
authors.lastname: Yagihara
mesh_terms: D002423:Cause of Death / Q000639:trends; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000401:mortality / Q000706:statistics & numerical data*; D006330:Heart Defects, Congenital / Q000401:mortality* / Q000601:surgery; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D011184:Postoperative Period; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D013997:Time Factors