title: Persistent fenestration may be a marker for physiologic intolerance after Fontan completion.
abstract: BACKGROUND
  We sought to evaluate the medium-term implications of fenestration status.
  
  
  METHODS
  Between 1994 and 2012, 326 patients received an extracardiac Fontan (hospital mortality n = 6, 1.8%). A fenestration was routinely created (n = 306, 94%) unless there was technical difficulty. Three hundred patients discharged with an open fenestration were included. The primary end points were death and Fontan failure. Secondary outcomes were Fontan complications such as venovenous collaterals, protein-losing enteropathy, pacemaker requirement, and arrhythmias.
  
  
  RESULTS
  The fenestration was closed in 260 patients: 185 as a catheter intervention (62%) and 75 (25%) spontaneously. Forty patients (13%) had the fenestration open at a median follow-up period of 5.05 years. Of these patients, catheter-based closure failed in 10 (3%). There was no statistically significant difference in pre-Fontan hemodynamic parameters, such as pulmonary artery pressure and pulmonary vascular resistance between the patients with open fenestration and the ones with closed fenestration. Patients with an open fenestration had significantly more late deaths (P < .001), Fontan failure (P = .021), and Fontan complications (P = .011) compared with those with a closed fenestration. Multivariable Cox regression revealed open fenestration (P < .001) and indeterminate ventricular morphology (P = .002) as risk factors for death/Fontan failure, and ventricular dysfunction (P = .014) and open fenestration (P = .009) as risk factors for Fontan complications.
  
  
  CONCLUSIONS
  Persistent fenestration was a marker for physiologic intolerance as noted by increased rates of mortality and a higher incidence of Fontan failure/complications. The specificity of pre-Fontan physiologic data for fenestration status may not have the fidelity needed for long-term care and thus, the consequences of decision making regarding fenestration status may not be determined until well after the operation.
authors.affiliation: Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Kotani
authors.affiliation: Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Devin
authors.initials: D
authors.lastname: Chetan
authors.affiliation: Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Arezou
authors.initials: A
authors.lastname: Saedi
authors.affiliation: Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Jiaquan
authors.initials: J
authors.lastname: Zhu
authors.affiliation: Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children Toronto, Ontario, Canada; Department of Paediatrics, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Lars
authors.initials: L
authors.lastname: Grosse-Wortmann
authors.affiliation: Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
authors.forename: John G
authors.initials: JG
authors.lastname: Coles
authors.affiliation: Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Christopher A
authors.initials: CA
authors.lastname: Caldarone
authors.affiliation: Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Glen S
authors.initials: GS
authors.lastname: Van Arsdell
authors.affiliation: Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada. Electronic address: osami.honjo@sickkids.ca.
authors.forename: Osami
authors.initials: O
authors.lastname: Honjo
mesh_terms: D006328:Cardiac Catheterization; D016009:Chi-Square Distribution; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects* / Q000401:mortality; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006439:Hemodynamics; D017052:Hospital Mortality; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D015999:Multivariate Analysis; D011183:Postoperative Complications / Q000401:mortality / Q000628:therapy; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D016276:Ventricular Function