title: Absent pulmonary valve syndrome. Surgical and clinical outcome with long-term follow-up.
abstract: OBJECTIVE
  To identify a large group of patients operated for absent pulmonary valve syndrome (APVS), and describe the outcomes of various subgroups and surgical strategies.
  
  
  METHODS
  Thirty-six consecutive patients operated during 1979-2004 were included. Thirty-one percent furthermore had other vascular abnormalities. Eighty-three percent had airway obstruction symptoms before surgery. Twenty-eight percent were ventilator dependent, while 72% underwent repair electively. The median age and weight at repair were 0.8 years (4 days-24 years) and 6.7 kg (1.8-56 kg). The surgical approach was modified several times, including the following: homograft conduit (14%), monocusp valve (39%), and no pulmonary valve (47%). All patients underwent ventricular septal defect (VSD) patching. Seventy-four percent underwent right ventricular outflow tract resection and 91% underwent transannular patching. Pulmonary artery reduction plasty was performed in 86%.
  
  
  RESULTS
  The follow-up was 94% complete. Nineteen percent had died. The median follow-up time was 9.2 years (1 day-20.5 years). Survival after repair was 82% at 1 year and 79% at 5, 10, and 15 years. Postoperative survival was strongly associated to preoperative ventilator dependency (p=0.002). The current New York Heart Association (NYHA) classification was established in 93% of survivors. Eighty-nine percent were in NYHA 1 or 2, 11% were in NYHA 3. No patient was in NYHA 4. Freedom from reoperation 1, 5, and 10 years after repair was 78, 65, and 55%, respectively.
  
  
  CONCLUSIONS
  The surgical outcome of APVS was closely related to preoperative ventilator dependency. Efforts to improve the surgical outcome should be focused on identifying and correcting this.
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.
authors.forename: Martin A
authors.initials: MA
authors.lastname: Nørgaard
authors.forename: Nelson
authors.initials: N
authors.lastname: Alphonso
authors.forename: Andrew E
authors.initials: AE
authors.lastname: Newcomb
authors.forename: Christian P
authors.initials: CP
authors.lastname: Brizard
authors.forename: Andrew D
authors.initials: AD
authors.lastname: Cochrane
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004812:Epidemiologic Methods; D005260:Female; D006324:Heart Arrest, Induced; D006345:Heart Septal Defects, Ventricular / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D011651:Pulmonary Artery / Q000601:surgery; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000601:surgery*; D012086:Reoperation; D012121:Respiration, Artificial; D016896:Treatment Outcome; D014694:Ventricular Outflow Obstruction / Q000601:surgery