title: Lung transplantation for pulmonary vascular disease.
abstract: BACKGROUND
  Pulmonary hypertension (PHT) is a lethal condition resulting in markedly diminished life expectancy. Continuous prostaglandin I2 infusion has made an important contribution to symptom management, but it is not a panacea. Lung or heart-lung transplantation remains an important treatment option for end-stage PHT patients unresponsive to prostaglandin I2. This study reviews the outcomes after transplantation for PHT in our program.
  
  
  METHODS
  A retrospective chart review was performed for 100 consecutive patients with either primary PHT (48%) or secondary PHT (52%) transplants since 1989. Living recipients were contacted to confirm health and functional status.
  
  
  RESULTS
  Fifty-five adult and 45 pediatric patients underwent 51 bilateral lung transplants, 39 single lung transplants, and 10 heart-lung transplants. Mean age was 23.7 years (range, 1.2 months to 54.8 years) and mean pre-transplant New York Heart Association class was 3.2. Pre-transplant hemodynamics revealed a mean right atrial pressure of 9.6+/-5.4 mm Hg and mean pulmonary artery pressure of 64+/-14.4 mm Hg. Hospital mortality was 17% with early death predominantly because of graft failure and infection. With an average follow-up of 5.0 years, 1- and 5-year actuarial survival was 75% and 57%, respectively. Mean pulmonary artery pressure on follow-up catheterization was 22+/-6.0 mm Hg, and mean follow-up New York Heart Association class was 1.3 (p < 0.001 for both compared with pre-transplant). Diagnosis and type of transplant did not confer a significant difference in survival between groups.
  
  
  CONCLUSIONS
  Whereas lung or heart-lung transplant for PHT is associated with higher early mortality than other pulmonary disease entities, it provides similar long-term outcomes with dramatic improvement in both quality of life and physiologic aspects.
authors.affiliation: Department of Surgery, Washington University School of Medicine, St. Louis, Missouri, USA. mendeloffe@msnotes.wustl.edu
authors.forename: Eric N
authors.initials: EN
authors.lastname: Mendeloff
authors.forename: Bryan F
authors.initials: BF
authors.lastname: Meyers
authors.forename: Thoralf M
authors.initials: TM
authors.lastname: Sundt
authors.forename: Tracey J
authors.initials: TJ
authors.lastname: Guthrie
authors.forename: Stuart C
authors.initials: SC
authors.lastname: Sweet
authors.forename: Maite
authors.initials: M
authors.lastname: de la Morena
authors.forename: Steve
authors.initials: S
authors.lastname: Shapiro
authors.forename: David T
authors.initials: DT
authors.lastname: Balzer
authors.forename: Elbert P
authors.initials: EP
authors.lastname: Trulock
authors.forename: John P
authors.initials: JP
authors.lastname: Lynch
authors.forename: Michael K
authors.initials: MK
authors.lastname: Pasque
authors.forename: Joel D
authors.initials: JD
authors.lastname: Cooper
authors.forename: Charles B
authors.initials: CB
authors.lastname: Huddleston
authors.forename: G Alexander
authors.initials: GA
authors.lastname: Patterson
mesh_terms: D000293:Adolescent; D000328:Adult; D001989:Bronchiolitis Obliterans / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000008:administration & dosage; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000601:surgery*; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007223:Infant; D016040:Lung Transplantation / Q000379:methods / Q000401:mortality; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016019:Survival Analysis
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine