title: Transplantation for idiopathic pulmonary arterial hypertension: improvement in the lung allocation score era.
abstract: BACKGROUND
  Lung transplantation and heart-lung transplantation represent surgical options for treatment of medically refractory idiopathic pulmonary arterial hypertension. The effect of the lung allocation score on wait-list and transplantation outcomes in patients with idiopathic pulmonary arterial hypertension is poorly described.
  
  
  RESULTS
  Adults diagnosed with idiopathic pulmonary arterial hypertension and listed for transplantation in the 80 months before and after the lung allocation score algorithm was implemented (n=1430) were identified in the United Network for Organ Sharing thoracic registry. Patients were stratified by organ listed and pre- and post-lung allocation score era. The cumulative incidences of transplantation and mortality for wait-listed patients in both eras were appraised with competing outcomes analysis. Posttransplantation survival was assessed with the Kaplan-Meier method. These analyses were repeated in propensity-matched subgroups. Cox proportional hazards analysis evaluated the effect of prelisting and pretransplantation characteristics on mortality. We found that patients in the post-lung allocation score era had significantly worse comorbidities; nevertheless, both lung transplantation and heart-lung transplantation candidates in this era enjoyed lower wait-list mortality and a higher incidence of transplantation in unmatched and propensity-matched analyses. On multivariable analysis, heart-lung transplantation and double-lung transplantation were associated with improved survival from the time of wait-listing, as was being listed at a medium- to high-volume institution. Donor/recipient sex matching predicted posttransplantation survival.
  
  
  CONCLUSIONS
  The incidence of transplantation has increased while wait-list mortality has decreased in patients with idiopathic pulmonary arterial hypertension wait-listed for transplantation in the post-lung allocation score era. Both heart-lung transplantation and double-lung transplantation are predictive of survival in transplantation candidates with idiopathic pulmonary arterial hypertension, as is being listed at a medium- to high-volume institution. Donor/recipient sex matching is associated with better posttransplantation survival.
authors.affiliation: Departments of Cardiothoracic Surgery, Stanford Hospital and Clinics, Stanford, CA, USA.
authors.forename: Justin M
authors.initials: JM
authors.lastname: Schaffer
authors.forename: Steve K
authors.initials: SK
authors.lastname: Singh
authors.forename: David L
authors.initials: DL
authors.lastname: Joyce
authors.forename: Bruce A
authors.initials: BA
authors.lastname: Reitz
authors.forename: Robert C
authors.initials: RC
authors.lastname: Robbins
authors.forename: Roham T
authors.initials: RT
authors.lastname: Zamanian
authors.forename: Hari R
authors.initials: HR
authors.lastname: Mallidi
mesh_terms: D000328:Adult; D000465:Algorithms; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D015276:Health Care Rationing / Q000639:trends*; D016027:Heart Transplantation / Q000706:statistics & numerical data; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality* / Q000601:surgery*; D015994:Incidence; D053208:Kaplan-Meier Estimate; D016040:Lung Transplantation / Q000706:statistics & numerical data; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D009927:Tissue and Organ Procurement / Q000379:methods*; D016896:Treatment Outcome; D014850:Waiting Lists / Q000401:mortality*
keywords: hypertension; hypertension, pulmonary; statistics; survival; transplantation