PMID- 16236930 OWN - NLM STAT- MEDLINE DCOM- 20051121 LR - 20181201 IS - 0012-3692 (Print) IS - 0012-3692 (Linking) VI - 128 IP - 4 DP - 2005 Oct TI - Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. PG - 2599-603 AB - BACKGROUND: Bosentan, an oral endothelin (ET)-A/ET-B receptor antagonist, is effective in the treatment of pulmonary arterial hypertension. OBJECTIVE: To investigate the safety and efficacy of bosentan therapy in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). DESIGN: Case series. SETTING: Pulmonary Hypertension Unit of the Medical University of Vienna, Austria. PATIENTS: Sixteen patients (9 women and 7 men; mean age +/- SD, 70 +/- 13 years). INTERVENTION: Off-label bosentan treatment over 6 months. MEASUREMENTS: Changes from baseline in liver enzymes, New York Heart Association (NYHA) functional class, 6-min walking distance (6-MWD), and serum amino-terminal pro-brain natriuretic peptide (proBNP). RESULTS: After 6 months, NYHA functional class improved by one class in 11 patients. Mean 6-MWDs increased from 299 +/- 131 m at baseline to 391 +/- 110 m at 6 months (p = 0.01). In parallel, proBNP decreased from 3,365 +/- 2,923 to 1,755 +/- 1,812 pg/mL (p = 0.01). Neither aspartate aminotransferase (25 +/- 2 U/L vs 25 +/- 2 U/L, p = 0.25) nor alanine aminotransferase (23 +/- 12 U/L vs 24 +/- 9 U/L, p = 0.57) changed significantly. Limitations of the study were uncontrolled design and small sample size. CONCLUSIONS: Our study suggests a beneficial effect of the oral dual ET receptor antagonist bosentan in patients with inoperable CTEPH, urging the need for a randomized, placebo-controlled trial. FAU - Bonderman, Diana AU - Bonderman D AD - Department of Cardiology, Medical University of Vienna, Wahringer Gurtel 18-20, 1090 Vienna, Austria. FAU - Nowotny, Regina AU - Nowotny R FAU - Skoro-Sajer, Nika AU - Skoro-Sajer N FAU - Jakowitsch, Johannes AU - Jakowitsch J FAU - Adlbrecht, Christopher AU - Adlbrecht C FAU - Klepetko, Walter AU - Klepetko W FAU - Lang, Irene M AU - Lang IM LA - eng PT - Clinical Trial PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Chest JT - Chest JID - 0231335 RN - 0 (Antihypertensive Agents) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) RN - EC 2.6.1.1 (Aspartate Aminotransferases) RN - EC 2.6.1.2 (Alanine Transaminase) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Alanine Transaminase/blood MH - Antihypertensive Agents/*therapeutic use MH - Aspartate Aminotransferases/blood MH - Bosentan MH - Endothelin Receptor Antagonists MH - Exercise Test MH - Female MH - Follow-Up Studies MH - Humans MH - Hypertension, Pulmonary/*drug therapy MH - Liver Function Tests MH - Male MH - Middle Aged MH - Sulfonamides/*therapeutic use MH - Thromboembolism/*drug therapy MH - Walking EDAT- 2005/10/21 09:00 MHDA- 2005/12/13 09:00 CRDT- 2005/10/21 09:00 PHST- 2005/10/21 09:00 [pubmed] PHST- 2005/12/13 09:00 [medline] PHST- 2005/10/21 09:00 [entrez] AID - S0012-3692(15)52678-0 [pii] AID - 10.1378/chest.128.4.2599 [doi] PST - ppublish SO - Chest. 2005 Oct;128(4):2599-603. doi: 10.1378/chest.128.4.2599.