title: Outcomes of surgery for young children with multivessel pulmonary vein stenosis.
abstract: OBJECTIVE
  We pursued a multimodality approach to the treatment of patients with pulmonary vein stenosis, incorporating sutureless surgical repair, catheter interventions, and adjunctive chemotherapy. We report our outcomes after surgery.
  
  
  METHODS
  Between January 2007 and August 2013, 49 patients with multivessel pulmonary vein stenosis underwent operations at our institution. We retrospectively reviewed data from a pulmonary vein stenosis registry and the medical records.
  
  
  RESULTS
  At the time of the index operation, the median patient age was 6 months (range, 32 days-48 months) and weight was 4.9 kg (range, 2.1-13.4 kg). Fourteen patients (28%) died during the follow-up period (median follow-up was 0.5 years [range, 0.04-4.9 years]). There were 2 deaths (4%) within 30 days. Age at repair <6 months, weight at repair <3 kg, and a preoperative right ventricular systolic pressure < ¾ systemic were found to be associated with mortality. One patient required repeat operation for recurrent stenosis. Thirty-nine patients (80%) underwent postoperative catheterizations. The median number of catheterizations per patient was 2 (range, 0-14). Twenty-nine patients (59%) underwent catheterizations with pulmonary vein intervention. The median number of catheterizations with intervention per patient was 1 (range, 0-14). There were no identifiable associations with need for or number of catheterizations with intervention. Ten patients were listed for lung transplantation: 4 patients were de-listed, 3 patients died waiting, and 3 patients underwent transplant.
  
  
  CONCLUSIONS
  Using a multimodality approach, we observed acceptable early survival after operation in patients with pulmonary vein stenosis, despite the need for catheter reinterventions. Lung transplantation remains a viable option.
authors.affiliation: Department of Cardiac Surgery, Boston Children's Hospital, Boston, Mass. Electronic address: luis.quinonez@cardio.chboston.org.
authors.forename: Luis G
authors.initials: LG
authors.lastname: Quinonez
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, Mass.
authors.forename: Kimberlee
authors.initials: K
authors.lastname: Gauvreau
authors.affiliation: Department of Cardiac Surgery, Boston Children's Hospital, Boston, Mass.
authors.forename: Michele
authors.initials: M
authors.lastname: Borisuk
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, Mass.
authors.forename: Christina
authors.initials: C
authors.lastname: Ireland
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, Mass.
authors.forename: Audrey M
authors.initials: AM
authors.lastname: Marshall
authors.affiliation: Department of Cardiac Surgery, Boston Children's Hospital, Boston, Mass.
authors.forename: John E
authors.initials: JE
authors.lastname: Mayer
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, Mass.
authors.forename: Kathy J
authors.initials: KJ
authors.lastname: Jenkins
authors.affiliation: Department of Cardiac Surgery, Boston Children's Hospital, Boston, Mass.
authors.forename: Francis E
authors.initials: FE
authors.lastname: Fynn-Thompson
authors.affiliation: Department of Cardiac Surgery, Boston Children's Hospital, Boston, Mass.
authors.forename: Christopher W
authors.initials: CW
authors.lastname: Baird
mesh_terms: D002675:Child, Preschool; D003251:Constriction, Pathologic; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011667:Pulmonary Veins; D012189:Retrospective Studies; D016896:Treatment Outcome; D014652:Vascular Diseases / Q000601:surgery*; D014656:Vascular Surgical Procedures / Q000379:methods
keywords: congenital heart disease; lung transplanation; pulmonary hypertension; pulmonary vein; pulmonary vein dilation; pulmonary vein operation; pulmonary vein stenosis