title: Long-term outcomes of patients with absent pulmonary valve syndrome: 38 years of experience.
abstract: BACKGROUND
  Absent pulmonary valve syndrome is associated with aneurysmal dilatation of the pulmonary arteries and compression of the tracheobronchial tree and may lead to significant respiratory compromise. We describe the outcomes of surgical correction of absent pulmonary valve syndrome and risk factors for mortality and reoperation.
  
  
  METHODS
  A review of 52 patients with absent pulmonary valve syndrome who underwent surgical correction between 1975 and 2013 was conducted. The median age and weight at repair were 9 months (range, 4 days to 24.2 years) and 6.9 kg (range, 1.8 to 56 kg). Preoperative intubation was required in 15 patients (29%), and 21 patients (40%) underwent urgent repair. The pulmonary valve was replaced with a valved conduit in 16 patients (31%) or monocusp valve in 16 patients (31%). Valveless repair was performed in 20 patients (38%). Pulmonary artery reduction was performed in 39 patients (75%), and 2 patients (4%) underwent a Lecompte maneuver.
  
  
  RESULTS
  The median follow-up time was 13 years (range, 1 month to 35 years). Early mortality was 18.8% (3 of 16) during 1975 through 1989, 19% (4 of 21) during 1990 through 2000, and 0% (0 of 15) during 2001 through 2013. Late mortality was 6.7% (3 of 45). Overall survival at 5, 10, and 20 years was 81.4%±5.6%. On multivariate analysis, preoperative ventilation (p=0.009) was the only risk factor for overall mortality. Freedom from late reoperation at 5, 10, and 20 years was 79.7%±6.9%, 69.4%±8.2%, and 52.1%±9.8%, respectively. No difference in reoperation rates was found between valved conduit, monocusp, or valveless techniques. Risk factors for late reoperation on multivariate analysis were prematurity (p=0.001) and neonatal primary repair (p=0.007). Longer postoperative ventilation periods were predicted by preoperative ventilation (p<0.001) and surgery during infancy (p=0.01).
  
  
  CONCLUSIONS
  Long-term survival for absent pulmonary valve syndrome has improved during the last decade. Preoperative ventilation predicted longer postoperative ventilation and mortality.
authors.affiliation: Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia.
authors.forename: Matthew S
authors.initials: MS
authors.lastname: Yong
authors.affiliation: Royal Children's Hospital, Melbourne, Australia.
authors.forename: Deane
authors.initials: D
authors.lastname: Yim
authors.affiliation: Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Christian P
authors.initials: CP
authors.lastname: Brizard
authors.affiliation: Women's and Children's Hospital, Adelaide, Australia.
authors.forename: Terry
authors.initials: T
authors.lastname: Robertson
authors.affiliation: Princess Margaret Hospital for Children, Perth, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Bullock
authors.affiliation: Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
authors.affiliation: Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia. Electronic address: igor.konstantinov@rch.org.au.
authors.forename: Igor E
authors.initials: IE
authors.lastname: Konstantinov
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D000293:Adolescent; D001315:Australia; D006348:Cardiac Surgical Procedures / Q000379:methods; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation / Q000379:methods*; D006776:Hospitals, Pediatric; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000401:mortality / Q000503:physiopathology / Q000601:surgery; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000601:surgery*; D035583:Rare Diseases; D012086:Reoperation / Q000379:methods; D012189:Retrospective Studies; D018570:Risk Assessment; D015996:Survival Rate; D013577:Syndrome; D013771:Tetralogy of Fallot / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult