title: 18 years of the Fontan operation at a single institution: results from 771 consecutive patients.
abstract: OBJECTIVE
  The aim of this study was to evaluate Fontan peri-operative outcomes for 771 consecutive patients.
  
  
  BACKGROUND
  Since the initial description by Fontan, mortality associated with the Fontan operation has declined substantially. However, postoperative effusions remain a significant challenge. Effusions are a key determinant of postoperative length-of-stay and have been shown to be associated with the development of protein-losing enteropathy and with decreased survival.
  
  
  METHODS
  This study was a single-center, retrospective review of 771 patients who underwent Fontan palliation from 1992 to 2009.
  
  
  RESULTS
  Patients were divided into 3 eras dictated by shift in clinical practice. Overall mortality was 3.5%, 1% since 1996. Importantly, age at Stage II palliation decreased from Era 1 to Era 3 (7.1 vs. 5.9 months; p = 0.0001), whereas age at Fontan increased (1.7 vs. 2.8 years; p = 0.0001). The proportion of patients with prolonged hospital stay (46.7% vs. 8.2% vs. 19.5%, p < 0.001) decreased substantially after Era 1. A diagnosis of hypoplastic left heart syndrome and longer operative support times were associated with prolonged pleural drainage (odds ratio [OR]: 2.17, p < 0.001; OR: 1.2, p = 0.001) and hospital stay (OR: 1.48, p = 0.05; OR: 1.34, p < 0.001). In patients who underwent invasive assessment, higher pulmonary artery pressure was associated with death (OR: 1.37, p < 0.001) and prolonged hospital stay (OR: 1.09, p = 0.019). Pulmonary arterial pressure ≥15 mm Hg was 90% specific for discriminating unfavorable outcomes.
  
  
  CONCLUSIONS
  Mortality in the modern era is rare, whereas postoperative pleural drainage remains the dominant morbidity. Elevated pulmonary artery pressure seems to be a marker of unfavorable outcome. Continued investigation is warranted to determine whether medical interventions or alterations to operative strategy can alter peri-operative results and improve long-term outcomes.
authors: The Cardiac Center, The Children's Hospital of Philadelphia, Pennsylvania, USA.
authors: Lindsay S
authors: LS
authors: Rogers
authors: Andrew C
authors: AC
authors: Glatz
authors: Chitra
authors: C
authors: Ravishankar
authors: Thomas L
authors: TL
authors: Spray
authors: Susan C
authors: SC
authors: Nicolson
authors: Jack
authors: J
authors: Rychik
authors: Christina Hayden
authors: CH
authors: Rush
authors: J William
authors: JW
authors: Gaynor
authors: David J
authors: DJ
authors: Goldberg
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects / Q000401:mortality / Q000706:statistics & numerical data*; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology*; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult