title: Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome.
abstract: OBJECTIVE
  Primary repair of tetralogy of Fallot with absent pulmonary valve syndrome has been associated with significant mortality, particularly for neonates in respiratory distress. Controversy persists regarding the method of establishing right ventricle-pulmonary artery continuity.
  
  
  METHODS
  Anatomic and demographic parameters were evaluated for patients undergoing repair of tetralogy of Fallot with absent pulmonary valve syndrome from 1990 to 2005, as were perioperative and late postoperative parameters (airway complications, reoperation or catheter-based intervention, and mortality).
  
  
  RESULTS
  Twenty-three patients underwent repair. Median age was 15 days (range 2-1154 days). Patients were followed up for 5.3 +/- 3.9 years. Seventeen (85%) required preoperative ventilatory assistance. One patient died within 24 hours; 1 patient died 8 months postoperatively. Four patients received valved homografts, and the remainder had valveless connections. All patients underwent reduction pulmonary arterioplasty and mobilization, unifocalization (in 3), and ventricular septal defect closure. Valveless connection recipients had a transannular hood. No patient underwent a Lecompte maneuver. Four patients underwent reoperation for conversion to valveless connection (n = 1), reduction arterioplasty (n = 1), and repair of pulmonary stenosis (n = 2). Three patients required catheter-based intervention, with balloon angioplasty (n = 3) and stent placement (n = 1); 2 now demonstrate equal quantitative lung perfusion. No patient has had significant debility from airway compromise. All patients demonstrate free pulmonary insufficiency and good biventricular function.
  
  
  CONCLUSIONS
  We report excellent overall survival (89%) and low postoperative morbidity for neonates and infants undergoing primary repair of tetralogy of Fallot with absent pulmonary valve syndrome. Our recent experience supports the use of a valveless right ventricle-pulmonary artery connection, which, combined with catheter-based intervention, reduces the likelihood of reoperation necessitated by homograft placement.
authors.affiliation: Department of Cardiothoracic Surgery, Weill Medical School at Cornell University, New York, NY 10021, USA. jmc23@columbia.edu
authors.forename: Jonathan M
authors.initials: JM
authors.lastname: Chen
authors.forename: Julie S
authors.initials: JS
authors.lastname: Glickstein
authors.forename: Renee
authors.initials: R
authors.lastname: Margossian
authors.forename: Michelle L
authors.initials: ML
authors.lastname: Mercando
authors.forename: William E
authors.initials: WE
authors.lastname: Hellenbrand
authors.forename: Ralph S
authors.initials: RS
authors.lastname: Mosca
authors.forename: Jan M
authors.initials: JM
authors.lastname: Quaegebeur
mesh_terms: D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000601:surgery*; D020127:Recovery of Function; D012086:Reoperation; D016019:Survival Analysis; D013771:Tetralogy of Fallot / Q000601:surgery*; D016896:Treatment Outcome