title: Clinical characteristics and prognostic factors of primary pulmonary vein stenosis or atresia in children.
abstract: BACKGROUND
  Primary pulmonary vein stenosis or atresia (PVS/A) is a rare entity with a high mortality rate. The aim of this study was to elucidate the clinical characteristics, progression, and prognostic factors of primary PVS/A in children.
  
  
  METHODS
  We reviewed the medical records of patients who had primary PVS/A with normally connected pulmonary veins (PVs) at five pediatric cardiology centers in Korea between 1995 and 2010.
  
  
  RESULTS
  A total of 34 cases were identified. The median age at diagnosis was 12.0 months. During the follow-up period (median, 18 months; range, 2 to 185 months), PVS/A progressed to previously uninvolved veins in 9 patients. Surgical interventions were performed on 29 patients (venoplasty on 25 and pneumonectomy on 4). Nineteen of the patients who underwent venoplasty had restenosis after a median of 2 months. The sutureless technique did not reduce the rate of restenosis, progression of the disease to previously uninvolved PVs, or mortality rate. The mortality rate was 46.7%, the median age of death was 10.8 months, and the median interval between diagnosis and death was 3.0 months. In univariate analysis, predictors of death included involvement of at least three PVs, bilateral PV involvement, infancy-onset PVS/A, restenosis after surgery, and progression to previously uninvolved PVs. In multivariate analysis, significant risk factors for death were involvement of at least three PVs (hazard ratio, 8.8; p < 0.0001) and progression to uninvolved PVs (hazard ratio, 4.2; p = 0.014).
  
  
  CONCLUSIONS
  Primary PVS/A may carry a significant risk of recurrent and progressive PV obstruction or death even after surgical venoplasty.
authors.affiliation: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Republic of Korea.
authors.forename: Mi Kyoung
authors.initials: MK
authors.lastname: Song
authors.forename: Eun Jung
authors.initials: EJ
authors.lastname: Bae
authors.forename: Soo In
authors.initials: SI
authors.lastname: Jeong
authors.forename: I Seok
authors.initials: IS
authors.lastname: Kang
authors.forename: Nam Kyun
authors.initials: NK
authors.lastname: Kim
authors.forename: Jae Young
authors.initials: JY
authors.lastname: Choi
authors.forename: Soo Jin
authors.initials: SJ
authors.lastname: Kim
authors.forename: Young Hwue
authors.initials: YH
authors.lastname: Kim
authors.forename: Woong Han
authors.initials: WH
authors.lastname: Kim
mesh_terms: D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D011667:Pulmonary Veins / Q000002:abnormalities*; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis* / Q000401:mortality; D056910:Republic of Korea / Q000453:epidemiology; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate / Q000639:trends; D054079:Vascular Malformations / Q000175:diagnosis* / Q000401:mortality