Abstract
Bosentan, an oral endothelin (ET)-A/ET-B receptor antagonist, is effective in the treatment of pulmonary arterial hypertension. To investigate the safety and efficacy of bosentan therapy in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Case series. Pulmonary Hypertension Unit of the Medical University of Vienna, Austria. Sixteen patients (9 women and 7 men; mean age +/- SD, 70 +/- 13 years). Off-label bosentan treatment over 6 months. 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). 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. 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.
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