title: Outcome after repair of tetralogy of Fallot with absent pulmonary valve.
abstract: BACKGROUND
  Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is associated with pulmonary artery dilatation and airway compression.
  
  
  METHODS
  Since January 1, 1984, 28 patients with TOF/ APV have undergone complete repair (median age 11 days, range 1 day to 16 years).
  
  
  RESULTS
  Thirteen patients were ventilated for respiratory failure preoperatively and extracorporeal membrane oxygenation was used in 3. Twenty-six patients underwent pulmonary artery plication (11 anterior, 15 anterior/ posterior). The right ventricular outflow tract (RVOT) was reconstructed with a patch (19), valved conduit (5), or monocusp valve (4). Early mortality was 21.4% (6/28), with 1 late death. All early deaths occurred in infants intubated preoperatively. Survival was 77% (95% confidence limit [CL] 56%, 89%) at 1 year and 72% (95% CL 50%, 86%) at 10 years. After surgery, 3 patients underwent reoperation for persistent respiratory symptoms, which resolved after repeat plication and placement of a valved conduit. Freedom from death or reoperation was 68% (95% CL 46%, 83%) at 1 year and 52% (95% CL 29%, 71%) at 10 years. In a multivariable analysis, only preoperative intubation was associated with a worse outcome (p = 0.04).
  
  
  CONCLUSIONS
  Long-term outcome for patients with TOF/APV who survive the initial repair is good. Repeat plication and pulmonary valve implantation may improve outcome in patients with persistent airway compression.
authors.affiliation: Department of Anesthesia and Critical Care, the Cardiac Center, The Children's Hospital of Philadelphia, Pennsylvania 19104-4399, USA.
authors.forename: B E
authors.initials: BE
authors.lastname: McDonnell
authors.forename: G W
authors.initials: GW
authors.lastname: Raff
authors.forename: J W
authors.initials: JW
authors.lastname: Gaynor
authors.forename: J
authors.initials: J
authors.lastname: Rychik
authors.forename: R I
authors.initials: RI
authors.lastname: Godinez
authors.forename: W M
authors.initials: WM
authors.lastname: DeCampli
authors.forename: T L
authors.initials: TL
authors.lastname: Spray
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007442:Intubation, Intratracheal; D008297:Male; D016016:Proportional Hazards Models; D011664:Pulmonary Valve / Q000002:abnormalities*; D012086:Reoperation; D016019:Survival Analysis; D013771:Tetralogy of Fallot / Q000401:mortality / Q000601:surgery*; D016896:Treatment Outcome