title: Intermediate-term outcome following the fontan operation: a survival, functional and risk-factor analysis.
abstract: OBJECTIVE
  To investigate survival and risk factors influencing intermediate outcome after the Fontan procedure.
  
  
  METHODS
  Retrospective analysis of 122 patients operated between April 1991 and September 2002. Poor outcome was defined as late death or poor functional status (intractable supraventricular arrhythmias/NYHA 3-4) necessitating revision surgery.
  
  
  RESULTS
  64(52%) patients had an intermediate bi-directional cavo-pulmonary shunt (BCPS). 91 (76%) patients had a lateral tunnel total cavo-pulmonary connection, 21(17%) patients had an atrio-pulmonary connection and 10 (8%)patients had a Kawashima connection. There were 6 (5%)early deaths. Over a median follow up of 54 months (1-133), 12 (10%)patients have had surgical revision for poor functional status. There were 7 (6%) late deaths, 5 of which occurred after revision surgery. Univariate analysis identified older age at operation (>4 years) (P=0.04), higher postoperative pulmonary artery pressure at 24h (P=0.012), arrhythmia postoperatively (P=0.03) or during follow-up (P=0.01) and the requirement for anticoagulation during follow-up (P=0.03) as significant predictors of poorer outcome. Patients who had an intermediate BCPS (P=0.002) or Norwood Stage 1 (P=0.05) had a better outcome. Multivariate analysis identified an intermediate Glenn shunt and lower postoperative pulmonary artery pressure as significant predictors of better outcome. Actuarial freedom from death or revision is 93% (+/-2), 88% (+/-3), 86% (+/-4) and 69% (+/-7) at 1, 5, 7 and 9 years respectively. Actuarial freedom from death or revision for the lateral tunnel group is 92% (+/-2), 89% (+/-3), 85% (+/-5) and 66% (+/-10) respectively.
  
  
  CONCLUSIONS
  Atrio-pulmonary connection results in a higher incidence of arrhythmias and failure than the lateral tunnel Fontan. Even in patients with a lateral tunnel Fontan there is a continuing hazard phase in the intermediate term. Mid-term outcome appears to be favorably influenced by an intermediate BCPS.
authors.affiliation: Department of Congenital Heart Disease, Guy's Hospital, Guy's and St Thomas Hospitals NHS Trust, St Thomas Street, London SE1 9RT, UK. nelson.a@bigfoot.com
authors.forename: Nelson
authors.initials: N
authors.lastname: Alphonso
authors.forename: Max
authors.initials: M
authors.lastname: Baghai
authors.forename: Prem
authors.initials: P
authors.lastname: Sundar
authors.forename: Robert
authors.initials: R
authors.lastname: Tulloh
authors.forename: Conal
authors.initials: C
authors.lastname: Austin
authors.forename: David
authors.initials: D
authors.lastname: Anderson
mesh_terms: D000704:Analysis of Variance; D001145:Arrhythmias, Cardiac / Q000209:etiology; D001778:Blood Coagulation Disorders / Q000188:drug therapy / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000379:methods* / Q000401:mortality; D006330:Heart Defects, Congenital / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006351:Heart Valves / Q000002:abnormalities / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000209:etiology; D011300:Preoperative Care / Q000379:methods; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome