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Bosentan Therapy for Inoperable Chronic Thromboembolic Pulmonary Hypertension^*

^* From the Departments of Cardiology (Drs. Bonderman, Skoro-Sajer, Jakowitsch, Adlbrecht, and Langand and Ms. Nowotny) and Cardiothoracic Surgery (Dr. Klepetko), Medical University of Vienna, Vienna, Austria.

Correspondence to: Irene M Lang, MD, Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria; e-mail: irene.lang{at}meduniwien.ac.at

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.

Key Words: bosentan • pulmonary hypertension • pulmonary thromboembolism

This article has been cited by other articles:

				P. Bresser, J. Pepke-Zaba, X. Jais, M. Humbert, and M. M. Hoeper Medical Therapies for Chronic Thromboembolic Pulmonary Hypertension: An Evolving Treatment Paradigm Proceedings of the ATS, September 1, 2006; 3(7): 594 - 600. [Abstract] [Full Text] [PDF]

				L. J. Rubin, M. M. Hoeper, W. Klepetko, N. Galie, I. M. Lang, and G. Simonneau Current and Future Management of Chronic Thromboembolic Pulmonary Hypertension: From Diagnosis to Treatment Responses Proceedings of the ATS, September 1, 2006; 3(7): 601 - 607. [Abstract] [Full Text] [PDF]

				A. Peacock, G. Simonneau, and L. Rubin Controversies, Uncertainties and Future Research on the Treatment of Chronic Thromboembolic Pulmonary Hypertension Proceedings of the ATS, September 1, 2006; 3(7): 608 - 614. [Abstract] [Full Text] [PDF]

				R. J. Hughes, X. Jais, D. Bonderman, J. Suntharalingam, M. Humbert, I. Lang, G. Simonneau, and J. Pepke-Zaba The efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension: a 1-year follow-up study Eur. Respir. J., July 1, 2006; 28(1): 138 - 143. [Abstract] [Full Text] [PDF]