title: IVI epoprostenol as salvage therapy in pulmonary arterial hypertension: an Australian perspective.
abstract: BACKGROUND
  IVI epoprostenol is the only therapy for pulmonary arterial hypertension (PAH) with a randomized controlled trial demonstrating improved survival, when used as first-line monotherapy. In Australia it is used as salvage therapy for those failing treatment with other targeted therapies or presenting in World Health Organization functional class (FC) IV.
  
  
  OBJECTIVE
  Report experience with IVI epoprostenol, administered as salvage therapy for the treatment of adults with PAH in a single Australian PAH centre.
  
  
  METHODS
  Retrospective case series of all patients commenced on IVI epoprostenol for PAH, between 2002 and 2010. Review of case notes with collection of data at baseline and after treatment, including FC, 6-min walk test (6MWT), right ventricular systolic pressure (RVSP) on echocardiogram, patient survival and treatment complications. Change in indices was assessed using the Wilcoxon Sign Rank Test and is expressed as median (inter-quartile range).
  
  
  RESULTS
  A total of 23 patients was included. Treatment was generally well tolerated with few major complications. At the end of the study period, nine patients were successfully bridged to transplant, five had a sustained response to IVI epoprostenol, six had an incomplete response but were clinically stabilized, two died awaiting transplant and one died who was not a candidate for transplantation. Overall, when measured at best level post initiation of IVI epoprostenol, there were significant improvements in FC -1 [0 to -1] (P < 0.0001), 6MWT (m) +117 [70-264] (P= 0.002) and RVSP (mmHg) -7.0 [4.0 to -45] (P= 0.03).
  
  
  CONCLUSIONS
  Findings support efficacy of epoprostenol as salvage therapy for patients with PAH.
authors.affiliation: Department of Allergy, Immunology and Respiratory Medicine, The Alfred Hospital and Monash University Department of Epidemiology and Preventative Medicine, Monash University, Melbourne, Victoria, Australia. mattsalamonsen@gmail.com
authors.forename: M
authors.initials: M
authors.lastname: Salamonsen
authors.forename: D
authors.initials: D
authors.lastname: Keating
authors.forename: H
authors.initials: H
authors.lastname: Whitford
authors.forename: M
authors.initials: M
authors.lastname: Bailey
authors.forename: T
authors.initials: T
authors.lastname: Miller
authors.forename: C
authors.initials: C
authors.lastname: Manterfield
authors.forename: T
authors.initials: T
authors.lastname: Williams
mesh_terms: D000328:Adult; D000368:Aged; D001315:Australia / Q000453:epidemiology; D002648:Child; D011464:Epoprostenol / Q000008:administration & dosage*; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000453:epidemiology*; D007262:Infusions, Intravenous; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016879:Salvage Therapy / Q000379:methods; D055815:Young Adult
chemical_list: D011464:Epoprostenol