title: Creation of a transcatheter fenestration in children with failure of Fontan circulation: Focus on extracardiac conduit connection.
abstract: OBJECTIVE
  We report our experience with a transcatheter technique to bypass the lung and to thus improve single-ventricle preload and reduce venous congestion in Fontan patients.
  
  
  BACKGROUND
  In the absence of a dedicated power source to serve the pulmonary circulation and a significantly elevated transpulmonary pressure gradient, fenestration of the Fontan circulation is an option to improve hemodynamics in patients by relieving excessive systemic venous pressure.
  
  
  RESULTS
  From 2005 to 2011, 22 transcatheter fenestrations were performed without any major complications in 19 patients (median age 3.2 years, interquartile range (IQR) 2.7-3.7 years)) with failing Fontan circulation and exceeding systemic venous pressure. In 16 patients, the procedure was performed for acute postoperative failure 1-24 days after surgery. After perforation of the conduit and atrial wall by a Brockenbrough needle and gradual balloon dilation, premounted stents were expanded to create a diabolo configuration with flaring stent edges, leaving a slight but definitive central waist. The procedure resulted in regression of pleural effusions and a significant decrease in systemic venous pressure. Clinical improvement was observed in 16 of the 19 treated patients. Follow-up demonstrated sustained fenestration in 85% of treated patients for at least 24 months.
  
  
  CONCLUSIONS
  Transcatheter creation of a Fontan fenestration is a safe approach despite the anatomic gap between the extracardiac conduit cavity and the atrial wall. Stent implantation allows defining the diameter of the fenestration, reduces spontaneous occlusion, and ensures sustained clinical improvement.
authors.affiliation: Pediatric Heart Center, University of Giessen and Marburg, Giessen, Germany.
authors.forename: Stefan
authors.initials: S
authors.lastname: Rupp
authors.affiliation: Pediatric Heart Center, University of Giessen and Marburg, Giessen, Germany.
authors.forename: Christin
authors.initials: C
authors.lastname: Schieke
authors.affiliation: Pediatric Heart Center, University of Giessen and Marburg, Giessen, Germany.
authors.forename: Gunter
authors.initials: G
authors.lastname: Kerst
authors.affiliation: Pediatric Heart Center, University of Giessen and Marburg, Giessen, Germany.
authors.forename: Nona
authors.initials: N
authors.lastname: Mazhari
authors.affiliation: Pediatric Heart Center, University of Giessen and Marburg, Giessen, Germany.
authors.forename: Axel
authors.initials: A
authors.lastname: Moysich
authors.affiliation: Pediatric Heart Center, University of Giessen and Marburg, Giessen, Germany.
authors.forename: Heiner
authors.initials: H
authors.lastname: Latus
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Vienna, Austria.
authors.forename: Ina
authors.initials: I
authors.lastname: Michel-Behnke
authors.affiliation: Pediatric Heart Center, University of Giessen and Marburg, Giessen, Germany.
authors.forename: Hakan
authors.initials: H
authors.lastname: Akintuerk
authors.affiliation: Pediatric Heart Center, University of Giessen and Marburg, Giessen, Germany.
authors.forename: Dietmar
authors.initials: D
authors.lastname: Schranz
mesh_terms: D015906:Angioplasty, Balloon, Coronary / Q000009:adverse effects / Q000295:instrumentation / Q000379:methods*; D006328:Cardiac Catheterization / Q000009:adverse effects / Q000295:instrumentation / Q000379:methods*; D002675:Child, Preschool; D003326:Coronary Circulation; D018729:Fontan Procedure; D005858:Germany; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D006439:Hemodynamics; D006801:Humans; D010690:Phlebography; D011474:Prosthesis Design; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D011652:Pulmonary Circulation; D011677:Punctures; D020127:Recovery of Function; D019233:Retreatment; D012307:Risk Factors; D015607:Stents; D013997:Time Factors; D017211:Treatment Failure; D014690:Venous Pressure
keywords: Fontan failure; extracardiac conduit; transcatheter fenestration