title: Endothelin receptor antagonist and airway dysfunction in pulmonary arterial hypertension creator: Droste, Annette S. creator: Rohde, David creator: Völkers, Mirko creator: Filusch, Arthur creator: Bruckner, Thomas creator: Borst, Mathias Maximilian creator: Katus, Hugo A. creator: Meyer, F. Joachim subject: ddc-610 subject: 610 Medical sciences Medicine description: Background: In idiopathic pulmonary arterial hypertension (IPAH), peripheral airway obstruction is frequent. This is partially attributed to the mediator dysbalance, particularly an excess of endothelin-1 (ET-1), to increased pulmonary vascular and airway tonus and to local inflammation. Bosentan (ET-1 receptor antagonist) improves pulmonary hemodynamics, exercise limitation, and disease severity in IPAH. We hypothesized that bosentan might affect airway obstruction. Methods: In 32 IPAH-patients (19 female, WHO functional class II (n = 10), III (n = 22); (data presented as mean ± standard deviation) pulmonary vascular resistance (11 ± 5 Wood units), lung function, 6 minute walk test (6-MWT; 364 ± 363.7 (range 179.0-627.0) m), systolic pulmonary artery pressure, sPAP, 79 ± 19 mmHg), and NT-proBNP serum levels (1427 ± 2162.7 (range 59.3-10342.0) ng/L) were measured at baseline, after 3 and 12 months of oral bosentan (125 mg twice per day). Results and Discussion: At baseline, maximal expiratory flow at 50 and 25% vital capacity were reduced to 65 ± 25 and 45 ± 24% predicted. Total lung capacity was 95.6 ± 12.5% predicted and residual volume was 109 ± 21.4% predicted. During 3 and 12 months of treatment, 6-MWT increased by 32 ± 19 and 53 ± 69 m, respectively; p < 0.01; whereas sPAP decreased by 7 ± 14 and 10 ± 19 mmHg, respectively; p < 0.05. NT-proBNP serum levels tended to be reduced by 123 ± 327 and by 529 ± 1942 ng/L; p = 0.11). There was no difference in expiratory flows or lung volumes during 3 and 12 months. Conclusion: This study gives first evidence in IPAH, that during long-term bosentan, improvement of hemodynamics, functional parameters or serum biomarker occur independently from persisting peripheral airway obstruction. publisher: BioMed Central date: 2009-12-30 type: Article type: info:eu-repo/semantics/article type: NonPeerReviewed format: application/pdf identifier: https://archiv.ub.uni-heidelberg.de/volltextserver/20193/1/12931_2009_Article_843.pdf identifier: DOI: identifier: urn:nbn:de:bsz:16-heidok-201938 identifier: Droste, Annette S. ; Rohde, David ; Völkers, Mirko ; Filusch, Arthur ; Bruckner, Thomas ; Borst, Mathias Maximilian ; Katus, Hugo A. ; Meyer, F. Joachim (2009) Endothelin receptor antagonist and airway dysfunction in pulmonary arterial hypertension. Respiratory Research, 10 (129). pp. 1-7. ISSN 1465-993X relation: https://archiv.ub.uni-heidelberg.de/volltextserver/20193/ rights: info:eu-repo/semantics/openAccess rights: Please see front page of the work (Sorry, Dublin Core plugin does not recognise license id) language: eng