title: Lung transplant is a viable treatment option for patients with congenital and acquired pulmonary vein stenosis.
abstract: BACKGROUND
  Congenital pulmonary vein stenosis (PVS) is associated with high mortality because surgical repair is usually not feasible or is ineffective. In addition, acquired PVS after repair of congenital heart disease is a potential complication that occurs in 5% to 10% of patients and carries a poor prognosis. Lung transplantation has been proposed as a viable option. However, long-term outcomes after lung transplant in these patients remain unknown.
  
  
  METHODS
  This was a retrospective review of prospectively maintained database.
  
  
  RESULTS
  Between 1990 and 2010, 20 patients (12 girls, 8 boys) with PVS underwent transplantation. Of these, 8 had acquired stenosis from prior repair for total anomalous pulmonary venous return and 1 from atrioventricular canal repair. The median waiting time was 26 days. The mean age at transplant was 1.1 ± 0.89 years, and 16 of the 20 patients were white. All patients received bilateral lung transplants on cardiopulmonary bypass. Four patients (20%) were receiving extracorporeal membrane oxygenation (ECMO) support before transplant, and 3 (15%) required ECMO after transplant due to graft dysfunction. The mean intensive care unit stay was 33.5 ± 29.1 days, and the mean hospital stay was 58.7 ± 43.5 days. The 30-day mortality was 10%. ECMO support in the peri-operative period was the main predictor of 30-day and 1-year mortality (hazard ratio, 3.6; p = 0.01). The overall 5-year survival of the entire cohort was 59.8% (67.3% congenital vs 50.7% acquired). The predominant cause of long-term mortality was bronchiolitis obliterans. The 5-year bronchiolitis obliterans-free survival was 48% (57.2% congenital vs 41% acquired).
  
  
  CONCLUSIONS
  Lung transplant is a viable treatment option for PVS, particularly for patients with diffuse disease or failed surgical correction.
authors.affiliation: Division of Cardiothoracic Surgery, Washington University, St. Louis, MO, USA.
authors.forename: Ankit
authors.initials: A
authors.lastname: Bharat
authors.forename: Deirdre J
authors.initials: DJ
authors.lastname: Epstein
authors.forename: Mark
authors.initials: M
authors.lastname: Grady
authors.forename: Albert
authors.initials: A
authors.lastname: Faro
authors.forename: Peter
authors.initials: P
authors.lastname: Michelson
authors.forename: Stuart C
authors.initials: SC
authors.lastname: Sweet
authors.forename: Charles B
authors.initials: CB
authors.lastname: Huddleston
mesh_terms: D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D006801:Humans; D007223:Infant; D007902:Length of Stay; D016040:Lung Transplantation / Q000009:adverse effects; D008297:Male; D011446:Prospective Studies; D011668:Pulmonary Veno-Occlusive Disease / Q000151:congenital* / Q000401:mortality / Q000601:surgery*; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome