Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension.
OBJECTIVE
We performed an open-label multicenter study to evaluate the safety and efficacy of the dual endothelin receptor antagonist bosentan in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH).


METHODS
Nineteen patients with inoperable CTEPH were enrolled.


METHODS
The primary end point was a change in pulmonary vascular resistance (PVR). Secondary end points included 6-min walk test, peak oxygen uptake (V(O2)), New York Heart Association functional class, serum levels of N-terminal-pro brain natriuretic peptide (NT-pro-BNP), and various other hemodynamic parameters.


RESULTS
After 3 months of treatment with bosentan, PVR decreased from 914 +/- 329 to 611 +/- 220 dyne.s.cm(-5) (p < 0.001). Functional class and peak V(O2) remained unchanged, but 6-min walk distance increased from 340 +/- 102 to 413 +/- 130 m (p = 0.009), and serum NT-pro BNP levels improved from 2,895 +/- 2,620 to 2,179 +/- 2,301 (p = 0.027). One patient died, presumably from influenza A infection, and another patient experienced progressive fluid retention despite reduction of PVR. Other than that, treatment was well tolerated by all patients.


CONCLUSIONS
This open-label pilot trial suggests that bosentan may offer a therapeutic option for patients with inoperable CTEPH. Randomized controlled trials are warranted to confirm these findings.
Hoeper
Marius M
MM
Department of Respiratory Medicine, Hannover Medical School, 30623 Hannover, Germany. hoeper.marius@mh-hannover.de
Kramm
Thorsten
T
Wilkens
Heinrike
H
Schulze
Christine
C
Schäfers
Hans Joachim
HJ
Welte
Tobias
T
Mayer
Eckhard
E
D000368:Aged; D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D004232:Diuretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010101:Oxygen Consumption; D010865:Pilot Projects; D013449:Sulfonamides / Q000627:therapeutic use*; D013923:Thromboembolism / Q000503:physiopathology*; D014655:Vascular Resistance; D016138:Walking / Q000502:physiology
D000925:Anticoagulants; D000959:Antihypertensive Agents; D004232:Diuretics; D013449:Sulfonamides; D020097:Natriuretic Peptide, Brain; D000077300:Bosentan