title: Total cavopulmonary connections in children with a previous Norwood procedure.
abstract: BACKGROUND
  Outcomes of the Fontan operation in children initially palliated with the modified Norwood procedure are incompletely defined.
  
  
  METHODS
  From August 1993 to January 2000, 45 patients (mean age 2.6 +/- 1.1 years, weight 12.7 +/- 2.8 kg) who were palliated with staged Norwood procedures (hypoplastic left heart syndrome, n = 32; nonhypoplastic left heart syndrome, n = 13) underwent a modified Fontan operation. Preoperative features included moderate/severe atrioventricular valve regurgitation (n = 5, 11%), reduced ventricular function on echocardiography in 11 patients, McGoon index 1.56 +/- 0.38, and pulmonary artery distortion in 18 patients (40%).
  
  
  RESULTS
  A lateral tunnel (n = 16) or an extracardiac conduit (n = 29) connection with fenestration in 38 patients (84%) was used. Concomitant procedures included pulmonary artery reconstruction (n = 24, 53%), atrioventricular valve repair (n = 4, 9%) or replacement (n = 1). Before Fontan, 12 patients (27%) had an intervention to address neoaortic obstruction, and 7 patients required balloon dilation/stenting of the left (n = 5) or right pulmonary artery (n = 5). Intraoperatively, left (n = 5) or right pulmonary artery (n = 1) stenting was performed in 5 patients (11%). On follow-up, 8 patients required additional interventional procedures to address left pulmonary artery narrowing (n = 5), or venous (n = 5) or arteriopulmonary collaterals (n = 1). Perioperative mortality was 4.4% (n = 2). There were 2 late deaths at a mean follow-up of 39 +/- 20 months.
  
  
  CONCLUSIONS
  In relatively high-risk patients, midterm results of the Fontan operation for children initially palliated with the Norwood procedure were good. Combined interventional-surgical treatment algorithms can lead to improved outcomes.
authors.affiliation: Department of Surgery, The Hospital For Sick Children, University of Toronto School of Medicine, Ontario, Canada.
authors.forename: A
authors.initials: A
authors.lastname: Azakie
authors.forename: B W
authors.initials: BW
authors.lastname: McCrindle
authors.forename: L N
authors.initials: LN
authors.lastname: Benson
authors.forename: G S
authors.initials: GS
authors.lastname: Van Arsdell
authors.forename: J L
authors.initials: JL
authors.lastname: Russell
authors.forename: J G
authors.initials: JG
authors.lastname: Coles
authors.forename: D
authors.initials: D
authors.lastname: Nykanen
authors.forename: R M
authors.initials: RM
authors.lastname: Freedom
authors.forename: W G
authors.initials: WG
authors.lastname: Williams
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure; D018610:Heart Bypass, Right / Q000379:methods*; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D006439:Hemodynamics / Q000502:physiology; D017052:Hospital Mortality; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality / Q000601:surgery*; D007223:Infant; D008297:Male; D010166:Palliative Care; D011183:Postoperative Complications / Q000401:mortality / Q000601:surgery; D012086:Reoperation; D015996:Survival Rate