title: Mid-term efficacy of beraprost, an oral prostacyclin analog, in the treatment of distal CTEPH: a case control study.
abstract: BACKGROUND
  Prostanoids are a well-established therapy for pulmonary arterial hypertension (PAH), and observational studies suggest their efficacy even in chronic thromboembolic pulmonary hypertension (CTEPH) patients.
  
  
  OBJECTIVE
  To compare the effects of 6 months of treatment with beraprost, an orally-active prostacyclin analog, in patients with distal CTEPH and PAH.
  
  
  METHODS
  Case-control study.
  
  
  METHODS
  Sixteen patients with severe pulmonary hypertension (NYHA II-IV), eight with distal CTEPH matched with eight patients with idiopathic PAH for similar effort tolerance.
  
  
  METHODS
  All patients were in stable clinical and hemodynamic condition for 3 months with maximal standard therapy. During the titration phase (4 weeks) beraprost was increased to maximal tolerated dose (mean daily dosage: CTEPH 275 +/- 47 microg, PAH 277 +/- 47 microg) in adjunction of standard therapy, patients were followed-up for 6 months.
  
  
  METHODS
  World Heart Organization (WHO) functional class, exercise capacity measured by distance walked in 6 min, and systolic pulmonary pressure (echocardiography), were evaluated at baseline, and at 1-, 3- and 6-month interval.
  
  
  RESULTS
  At 6 months WHO class decreased significantly in both groups (CTEPH from 2.7 +/- 0.6 to 2.0 +/- 0.24, p < 0.05; PAH from 3.0 +/- 0.26 to 2.1 +/- 0.25, p < 0.05), similarly the 6-min walk distance increased significantly from baseline (CTEPH from 312 +/- 31 to 373 +/- 29 m, p < 0.003; PAH from 303 +/- 31 to 347 +/- 29, p < 0.0003). Systolic pulmonary artery pressure showed a trend toward lower value (CTEPH from 85 +/- 7 m to 81 +/- 6 mm Hg, p = NS; PAH from 89 +/- 7 to 82 +/- 5, p = NS). During the observation period we did not have any death. The drug was well-tolerated with minor side-effects.
  
  
  CONCLUSIONS
  In patients with CTEPH beraprost had similar mid-term clinical and hemodynamic improvements than in patients with PAH.
authors.affiliation: Department of Cardiovascular and Respiratory Sciences, University La Sapienza, Rome, Italy. dario.vizza@uniroma1.it
authors.forename: Carmine Dario
authors.initials: CD
authors.lastname: Vizza
authors.forename: Roberto
authors.initials: R
authors.lastname: Badagliacca
authors.forename: Susanna
authors.initials: S
authors.lastname: Sciomer
authors.forename: Roberto
authors.initials: R
authors.lastname: Poscia
authors.forename: Alessandro
authors.initials: A
authors.lastname: Battagliese
authors.forename: Mauro
authors.initials: M
authors.lastname: Schina
authors.forename: Luciano
authors.initials: L
authors.lastname: Agati
authors.forename: Francesco
authors.initials: F
authors.lastname: Fedele
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D002908:Chronic Disease; D011464:Epoprostenol / Q000031:analogs & derivatives* / Q000627:therapeutic use; D005080:Exercise Test; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D013923:Thromboembolism / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D016896:Treatment Outcome
chemical_list: C048081:beraprost; D011464:Epoprostenol