title: Valved homograft replacement of aneurysmal pulmonary arteries for severely symptomatic absent pulmonary valve syndrome.
abstract: BACKGROUND
  Patients with absent pulmonary valve syndrome (APVS) with respiratory distress (RD) have previously had a high mortality. In 1990 we adopted a strategy of primary repair including total replacement of the aneurysmal central pulmonary arteries (PAs) for patients with RD.
  
  
  METHODS
  Retrospective review was made of 54 consecutive patients with APVS between 1960 and 1998. Median age and weight were 4 months and 4.8 kg. RD was present in 23 patients (10 neonates, 16 required ventilation). Fifteen patients had repair with homograft replacement of the PAs and VSD closure (group 1). Twenty-seven patients had transannular patch with VSD closure with PA-plasty (group 2, n = 21) or without PA plasty (group 3, n = 6). Twelve had miscellaneous procedures (group 4); in 6 the VSD was left open.
  
  
  RESULTS
  Operative, 1-, 5-, and 10-year survivals were 83%, 80%, 78%, and 78%, respectively. Risk factors for operative mortality in multivariate analysis were RD (p = 0.04), neonates (p = 0.02), weight less than 3 kg (p = 0.02), open VSD (p = 0.02) and surgery before 1990 (p = 0.04). Since 1990 operative mortality has decreased to 11% (p = 0.04). RD was the only time-related predictor of survival in multivariate analysis (p = 0.004). In patients with RD, survival with homograft was 73% versus 41% with other techniques (p = 0.2). Mean follow-up was 72 +/- 50 months. There were no significant differences in freedom from reintervention rates among the surgical groups (p = 0.08).
  
  
  CONCLUSIONS
  Aggressive homograft replacement of the central pulmonary arteries has been associated with improved survival in patients with APVS especially in neonates with severe RD.
authors.affiliation: Department of Cardiac Surgery, Children's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA.
authors.forename: Chee Chin
authors.initials: CC
authors.lastname: Hew
authors.forename: Sabine H
authors.initials: SH
authors.lastname: Daebritz
authors.forename: David
authors.initials: D
authors.lastname: Zurakowski
authors.forename: Pedro I
authors.initials: PI
authors.lastname: del Nido
authors.forename: John E
authors.initials: JE
authors.lastname: Mayer
authors.forename: Richard A
authors.initials: RA
authors.lastname: Jonas
mesh_terms: D000015:Abnormalities, Multiple / Q000401:mortality / Q000601:surgery*; D000783:Aneurysm / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000637:transplantation*; D011664:Pulmonary Valve / Q000002:abnormalities*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate; D013577:Syndrome; D013771:Tetralogy of Fallot / Q000401:mortality / Q000601:surgery*; D014656:Vascular Surgical Procedures / Q000379:methods