Source: Annual Congress 2006 - Pulmonary hypertension: thromo-embolic processes
Disease area: Pulmonary vascular diseases

Abstract

Background: Bosentan, an oral endothelin ETA/ETB receptor antagonist, is effective in the short-term treatment of inoperable chronic thromboembolic pulmonary arterial hypertension (CTEPH). We investigated hemodynamics, safety and efficacy of bosentan therapy at one year of therapy, in 21 patients (13f/8m, mean age 71±12years) who were treated off-label over 16±6 months.
Results: After one year of treatment, NYHA functional class had improved by one class in 14 patients. Mean six-minute walking distances increased from 299±131m at baseline to 387±121m (p=0.04). In parallel, proBNP decreased from 3365±2923pg/ml to 1579±2103pg/ml (p=0.02). Overall, mean pulmonary arterial pressure (mPAP) decreased from 48±10, to 43±12mmHg (p=0.17), pulmonary vascular resistance (PVR) changed from 653±247 to 468±205dynes.cm.s-5 (p=0.04). If hemodynamic non-responders to therapy were excluded (n=5), mPAP decreased from 50±10, to 42±11mmHg (p=0.17), and PVR changed from 757±232 to 420±137dynes.cm.s-5 (p=0.015). Neither AST (25±2 versus 25±2U/l, p=0.25) nor ALT (23±12 versus 24±9U/l, p=0.57) changed significantly. Two deaths occurred from causes unrelated to pulmonary hypertension.
Conclusions: Our study suggests a beneficial long-term effect of the oral dual endothelin receptor antagonist bosentan in patients with inoperable CTEPH. Non-responders to bosentan therapy must be further characterized.

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Citations should be made in the following way:
D. Bonderman, R. Hitsch, N. Skoro-Sajer, M. Kneussl, W. Klepetko, I. M. Lang (Vienna, Austria). Bosentan for inoperable chronic thromboembolic pulmonary hypertension. Eur Respir J 2006; 28: Suppl. 50, 2355

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