Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension.
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).


METHODS
Case series.


METHODS
Pulmonary Hypertension Unit of the Medical University of Vienna, Austria.


METHODS
Sixteen patients (9 women and 7 men; mean age +/- SD, 70 +/- 13 years).


METHODS
Off-label bosentan treatment over 6 months.


METHODS
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.
Bonderman
Diana
D
Department of Cardiology, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria.
Nowotny
Regina
R
Skoro-Sajer
Nika
N
Jakowitsch
Johannes
J
Adlbrecht
Christopher
C
Klepetko
Walter
W
Lang
Irene M
IM
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000410:Alanine Transaminase / Q000097:blood; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D001219:Aspartate Aminotransferases / Q000097:blood; D000077300:Bosentan; D065128:Endothelin Receptor Antagonists; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D013449:Sulfonamides / Q000627:therapeutic use*; D013923:Thromboembolism / Q000188:drug therapy*; D016138:Walking
D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D000077300:Bosentan