title: Bosentan for patients with chronic thromboembolic pulmonary hypertension.
abstract: BACKGROUND
  Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease leading to worsening functional status and reduced survival for those patients who cannot undergo pulmonary endarterectomy (PEA). Pharmacotherapy with novel drugs for pulmonary hypertension may be useful in treating patients who are poor candidates for surgery, but there are still few clinical data on medical therapy for CTEPH. The aim of this prospective open-label, multicenter, study is to compare the efficacy of 1-year bosentan treatment to standard drugs in nonoperated patients with CTEPH.
  
  
  METHODS
  Thirty-four nonoperated patients with CTEPH were enrolled. Functional assessment included 6 minute walk test (6MWT), Borg index, WHO classification, arterial blood gases and echocardiography systolic pulmonary artery pressure (sPAP). Seventeen patients received bosentan (62.5 mg b.i.d. for 4 weeks and then 125 mg b.i.d.); 17 patients were treated with standard therapy alone.
  
  
  RESULTS
  At admission sPAP was 76.18+/-5.96 mmHg in bosentan group and 71.48+/-3.71 mmHg in controls, p(a)O(2) 64.68+/-2.25 mmHg in bosentan group, and 59.52+/-2.05 mmHg in controls, 6MWT 297.53+/-34.25 mt in bosentan group, and 268.47+/-36.54 mt in controls. After 12 months there were significant differences between the groups in the 6MWT (+57.24+/-22.21 m vs -73.13+/-21.23 m, p<0.001), dyspnoea index (Borg score 4.29+/-0.49 vs 7.06+/-0.32, p<0.001) and oxygenation (p(a)O(2) 65.93+/-3.76 mmHg vs 48.48+/-1.31 mmHg, p<0.001). The sPAP was stable after 12 months of bosentan (76.18+/-5.96 mmHg vs 71.00+/-5.41 mmHg, p=0.221) in contrast to controls (71.48+/-3.71 mmHg vs 80.44+/-4.70 mmHg, p=0.029).
  
  
  CONCLUSIONS
  The data of this open-label study in nonoperated CTEPH patients suggest an improvement in functional outcomes adding Bosentan to diuretics and oral anticoagulants. No improvement was observed using only standard drugs after 1-year.
authors.affiliation: Department of Pneumology, University Hospital of Trieste, Trieste, Italy.
authors.forename: Fabio Giuseppe
authors.initials: FG
authors.lastname: Vassallo
authors.forename: Metka
authors.initials: M
authors.lastname: Kodric
authors.forename: Cleante
authors.initials: C
authors.lastname: Scarduelli
authors.forename: Sergio
authors.initials: S
authors.lastname: Harari
authors.forename: Alfredo
authors.initials: A
authors.lastname: Potena
authors.forename: Angelo
authors.initials: A
authors.lastname: Scarda
authors.forename: Marco
authors.initials: M
authors.lastname: Piattella
authors.forename: Roberto
authors.initials: R
authors.lastname: Cassandro
authors.forename: Marco
authors.initials: M
authors.lastname: Confalonieri
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000925:Anticoagulants / Q000008:administration & dosage; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D000077300:Bosentan; D002908:Chronic Disease; D004232:Diuretics / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D004417:Dyspnea / Q000188:drug therapy; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011655:Pulmonary Embolism / Q000188:drug therapy*; D013449:Sulfonamides / Q000008:administration & dosage*; D016896:Treatment Outcome; D016138:Walking; D055815:Young Adult
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D004232:Diuretics; D013449:Sulfonamides; D000077300:Bosentan