title: Twenty Years of Anesthetic and Perioperative Management of Patients With Tetralogy of Fallot With Absent Pulmonary Valve.
abstract: OBJECTIVE
  Review the authors' institutional experience of the induction and perioperative airway management of children with tetralogy of Fallot with an absent pulmonary valve.
  
  
  METHODS
  Retrospective chart review.
  
  
  METHODS
  Large academic children's hospital.
  
  
  METHODS
  Patients with the diagnosis of tetralogy of Fallot with absent pulmonary valve undergoing primary cardiac repair over a 20-year period.
  
  
  METHODS
  None.
  
  
  RESULTS
  Forty-four patients were identified with tetralogy of Fallot with an absent pulmonary valve from January 1995 through August 2014. Forty-two patients (95%) required surgery in their first year of life. Sixteen patients (36%) required mechanical ventilation preoperatively, including 11 neonates. Of the 28 patients not intubated preoperatively, only 1 (3.8%) exhibited minor airway obstruction following induction that was managed uneventfully. All intubations in the operating room were performed in the supine position. Five patients who were mechanically ventilated preoperatively in a lateral or prone position were supinated in the operating room without significant cardiopulmonary compromise. There were no patients who suffered cardiopulmonary arrest or required mechanical circulatory support. The median time to extubation was 2 days (range 1-13 days) in those patients who were not mechanically ventilated prior to their surgery. Ultimately, 5 patients required tracheostomy and 1 patient underwent lobectomy. Although, there was no mortality at 30 days, 4 children died within 1 year of their surgery. All the children who died had a genetic syndrome and required mechanical ventilation preoperatively.
  
  
  CONCLUSIONS
  While there are theoretical concerns specific to the induction and airway management of tetralogy of Fallot with an absent pulmonary valve, there were no episodes of cardiorespiratory arrest or extracorporeal membrane oxygenation in the authors' series. Neonatal age at the time of surgery, preoperative need for mechanical ventilation, and concomitant genetic syndromes are risk factors for respiratory morbidity. Mortality in this study was low compared to historic reports, likely reflecting improvement in surgical technique and intensive care management.
authors.affiliation: Department of Anesthesiology, University of Wisconsin, Madison, WI.
authors.forename: John D
authors.initials: JD
authors.lastname: Jochman
authors.affiliation: Department of Anesthesiology, Perioperative and Pain Medicine, Division of Cardiac Anesthesia, Boston Children's Hospital, Boston, MA.
authors.forename: Douglas B
authors.initials: DB
authors.lastname: Atkinson
authors.affiliation: Division of Cardiovascular Surgery, Boston Children's Hospital, Boston, MA.
authors.forename: Luis G
authors.initials: LG
authors.lastname: Quinonez
authors.affiliation: Department of Anesthesiology, Perioperative and Pain Medicine, Division of Cardiac Anesthesia, Boston Children's Hospital, Boston, MA. Electronic address: morgan.brown@childrens.harvard.edu.
authors.forename: Morgan L
authors.initials: ML
authors.lastname: Brown
mesh_terms: D058109:Airway Management / Q000379:methods / Q000639:trends*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D019990:Perioperative Care / Q000379:methods / Q000639:trends*; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000601:surgery*; D012121:Respiration, Artificial / Q000379:methods / Q000639:trends; D012189:Retrospective Studies; D013771:Tetralogy of Fallot / Q000175:diagnosis* / Q000601:surgery*; D013997:Time Factors
keywords: absent pulmonary valve; airway; congenital heart disease; tetralogy of Fallot