FREE NEJM E-TOC    HOME   |   SUBSCRIBE   |   CURRENT ISSUE   |   PAST ISSUES   |   COLLECTIONS   |   Search Term  Advanced Search

Sign in | Get NEJM's E-Mail Table of Contents — Free | Subscribe

Previous Volume 346:896-903 March 21, 2002 Number 12 Next

	Full Text PDF PDA Full Text PowerPoint Slide Set Editorial by Newman, J. H. Letters Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited Related Article PubMed Citation

ABSTRACT

Background Endothelin-1 is a potent vasoconstrictor and smooth-muscle mitogen. In a preliminary study, the orally administered dual endothelin-receptor antagonist bosentan improved exercise capacity and cardiopulmonary hemodynamics in patients with pulmonary arterial hypertension. The present trial investigated the effect of bosentan on exercise capacity in a larger number of patients and compared two doses.

Methods In this double-blind, placebo-controlled study, we randomly assigned 213 patients with pulmonary arterial hypertension (primary or associated with connective-tissue disease) to receive placebo or to receive 62.5 mg of bosentan twice daily for 4 weeks followed by either of two doses of bosentan (125 or 250 mg twice daily) for a minimum of 12 weeks. The primary end point was the degree of change in exercise capacity. Secondary end points included the change in the Borg dyspnea index, the change in the World Health Organization (WHO) functional class, and the time to clinical worsening.

Results At week 16, patients treated with bosentan had an improved six-minute walking distance; the mean difference between the placebo group and the combined bosentan groups was 44 m (95 percent confidence interval, 21 to 67; P<0.001). Bosentan also improved the Borg dyspnea index and WHO functional class and increased the time to clinical worsening.

Conclusions The endothelin-receptor antagonist bosentan is beneficial in patients with pulmonary arterial hypertension and is well tolerated at a dose of 125 mg twice daily. Endothelin-receptor antagonism with oral bosentan is an effective approach to therapy for pulmonary arterial hypertension.

Source Information

From the Division of Pulmonary and Critical Care Medicine, University of California at San Diego, La Jolla (L.J.R.); the University of Colorado Health Sciences Center, Denver (D.B.B.); Babies and Children's Hospital, Columbia Presbyterian Medical Center, New York (R.J.B.); the Università di Bologna, Bologna, Italy (N.G.); the Royal Free Hospital School of Medicine, London (C.M.B.); St. Vincent's Hospital, Darlinghurst, Australia (A.K.); the Instituto Nacional de Cardiología, Mexico City, Mexico (T.P.); Baylor College of Medicine and Methodist Hospital, Houston (A.F.); Actelion, Allschwil, Switzerland (S.R., I.L.); Children's Hospital and Harvard Medical School, Boston (M.L.); and Hôpital Antoine Béclère, Clamart, France (G.S.).

Address reprint requests to Dr. Rubin at the Division of Pulmonary and Critical Care Medicine, University of California at San Diego, 9300 Campus Point Dr., M/C 7372, La Jolla, CA 92037-1330, or at ljrubin{at}ucsd.edu.

Full Text of this Article

Related Letters:

Bosentan for Pulmonary Hypertension
Pereira B. N., Salvi S., Dietrich C. G., Geier A., Lammert F., Rubin L. J., Galiè N., Simonneau G., the Bosentan Randomized Trial of Endothelin Antagonist Therapy Study Investigators
Extract | Full Text | PDF  
N Engl J Med 2002; 347:292-294, Jul 25, 2002. Correspondence

This article has been cited by other articles:

• Ghofrani, H. A., Wilkins, M. W., Rich, S. (2008). Uncertainties in the Diagnosis and Treatment of Pulmonary Arterial Hypertension. Circulation 118: 1195-1201 [Abstract] [Full Text]  
• Thonhofer, R., Siegel, C., Hafner, F., Gotschuli, G., Brodmann, M. (2008). Successful bosentan treatment of critical ischaemia induced by vasculitis in an SCLE patient. Rheumatology (Oxford) 0: ken354v1-ken354 [Full Text]  
• Kuwano, K., Hashino, A., Noda, K., Kosugi, K., Kuwabara, K. (2008). A Long-Acting and Highly Selective Prostacyclin Receptor Agonist Prodrug, 2-{4-[(5,6-Diphenylpyrazin-2-yl)(isopropyl)amino]butoxy}-N-(methylsulfonyl)acetamide (NS-304), Ameliorates Rat Pulmonary Hypertension with Unique Relaxant Responses of Its Active Form, {4-[(5,6-Diphenylpyrazin-2-yl)(isopropyl)amino]butoxy}acetic Acid (MRE-269), on Rat Pulmonary Artery. J. Pharmacol. Exp. Ther. 326: 691-699 [Abstract] [Full Text]  
• Wells, A U, Hirani, N, on behalf of the BTS Interstitial Lung Disease Gui, (2008). Interstitial lung disease guideline. Thorax 63: v1-v58 [Full Text]  
• Denton, C P, Pope, J E, Peter, H-H, Gabrielli, A, Boonstra, A, van den Hoogen, F H J, Riemekasten, G, De Vita, S, Morganti, A, Dolberg, M, Berkani, O, Guillevin, L, (on behalf of the TRacleer Use in PAH associated w, (2008). Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Ann Rheum Dis 67: 1222-1228 [Abstract] [Full Text]  
• Yuen, S. Y., Pope, J. E. (2008). Learning from past mistakes: assessing trial quality, power and eligibility in non-renal systemic lupus erythematosus randomized controlled trials. Rheumatology (Oxford) 47: 1367-1372 [Abstract] [Full Text]  
• Dhaun, N., Goddard, J., Kohan, D. E., Pollock, D. M., Schiffrin, E. L., Webb, D. J. (2008). Role of Endothelin-1 in Clinical Hypertension: 20 Years On. Hypertension 52: 452-459 [Full Text]  
• Opitz, C. F., Ewert, R., Kirch, W., Pittrow, D. (2008). Inhibition of endothelin receptors in the treatment of pulmonary arterial hypertension: does selectivity matter?. Eur Heart J 29: 1936-1948 [Abstract] [Full Text]  
• Boutet, K., Montani, D., Jais, X., Yaici, A., Sitbon, O., Simonneau, G., Humbert, M. (2008). Review: Therapeutic advances in pulmonary arterial hypertension. Therapeutic Advances in Respiratory Disease 2: 249-265 [Abstract]  
• Frey, R., Muck, W., Unger, S., Artmeier-Brandt, U., Weimann, G., Wensing, G. (2008). Single-Dose Pharmacokinetics, Pharmacodynamics, Tolerability, and Safety of the Soluble Guanylate Cyclase Stimulator BAY 63-2521: An Ascending-Dose Study in Healthy Male Volunteers. J Clin Pharmacol 48: 926-934 [Abstract] [Full Text]  
• Iannone, F, Riccardi, M T, Guiducci, S, Bizzoca, R, Cinelli, M, Matucci-Cerinic, M, Lapadula, G (2008). Bosentan regulates the expression of adhesion molecules on circulating T cells and serum soluble adhesion molecules in systemic sclerosis-associated pulmonary arterial hypertension. Ann Rheum Dis 67: 1121-1126 [Abstract] [Full Text]  
• Hill, N. S., Preston, I. R., Roberts, K. E. (2008). Patients with Pulmonary Arterial Hypertension in Clinical Trials: Who Are They?. Proc Am Thorac Soc 5: 603-609 [Abstract] [Full Text]  
• Ventetuolo, C. E., Benza, R. L., Peacock, A. J., Zamanian, R. T., Badesch, D. B., Kawut, S. M. (2008). Surrogate and Combined End Points in Pulmonary Arterial Hypertension. Proc Am Thorac Soc 5: 617-622 [Abstract] [Full Text]  
• Epstein, B. J (2008). Efficacy and Safety of Darusentan: A Novel Endothelin Receptor Antagonist. The Annals of Pharmacotherapy 42: 1060-1069 [Abstract] [Full Text]  
• Haworth, S G (2008). The management of pulmonary hypertension in children. Arch. Dis. Child. 93: 620-625 [Abstract] [Full Text]  
• Galie, N., Olschewski, H., Oudiz, R. J., Torres, F., Frost, A., Ghofrani, H. A., Badesch, D. B., McGoon, M. D., McLaughlin, V. V., Roecker, E. B., Gerber, M. J., Dufton, C., Wiens, B. L., Rubin, L. J., for the Ambrisentan in Pulmonary Arterial Hyperten, (2008). Ambrisentan for the Treatment of Pulmonary Arterial Hypertension: Results of the Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy (ARIES) Study 1 and 2. Circulation 117: 3010-3019 [Abstract] [Full Text]  
• Avouac, J, Wipff, J, Kahan, A, Allanore, Y (2008). Effects of oral treatments on exercise capacity in systemic sclerosis related pulmonary arterial hypertension: a meta-analysis of randomised controlled trials. Ann Rheum Dis 67: 808-814 [Abstract] [Full Text]  
• Wrishko, R. E., Dingemanse, J., Yu, A., Darstein, C., Phillips, D. L., Mitchell, M. I. (2008). Pharmacokinetic Interaction Between Tadalafil and Bosentan in Healthy Male Subjects. J Clin Pharmacol 48: 610-618 [Abstract] [Full Text]  
• Chin, K. M., Rubin, L. J. (2008). Pulmonary arterial hypertension.. J Am Coll Cardiol 51: 1527-1538 [Abstract] [Full Text]  
• Ohno, T., Muneuchi, J., Ihara, K., Yuge, T., Kanaya, Y., Yamaki, S., Hara, T. (2008). Pulmonary Hypertension in Patients With Congenital Portosystemic Venous Shunt: A Previously Unrecognized Association. Pediatrics 121: e892-e899 [Abstract] [Full Text]  
• Mathier, M. A., Zhang, J., Ramanathan, R. C. (2008). Dramatic Functional Improvement Following Bariatric Surgery in a Patient With Pulmonary Arterial Hypertension and Morbid Obesity. Chest 133: 789-792 [Abstract] [Full Text]  
• National Pulmonary Hypertension Centres of the UK, (2008). Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. Heart 94: i1-i41 [Full Text]  
• National Pulmonary Hypertension Centres of the UK, (2008). Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. Thorax 63: ii1-ii41 [Full Text]  
• Moore, S. C., DeSantis, E. R. H. (2008). Treatment of complications associated with systemic sclerosis. Am J Health Syst Pharm 65: 315-321 [Abstract] [Full Text]  
• Wong, C. M., Cheema, A. K., Zhang, L., Suzuki, Y. J. (2008). Protein Carbonylation as a Novel Mechanism in Redox Signaling. Circ. Res. 102: 310-318 [Abstract] [Full Text]  
• Dupuis, J., Hoeper, M. M. (2008). Endothelin receptor antagonists in pulmonary arterial hypertension. Eur Respir J 31: 407-415 [Abstract] [Full Text]  
• Petersen, B., Deja, M., Bartholdy, R., Donaubauer, B., Laudi, S., Francis, R. C. E., Boemke, W., Kaisers, U., Busch, T. (2008). Inhalation of the ETA receptor antagonist LU-135252 selectively attenuates hypoxic pulmonary vasoconstriction. Am. J. Physiol. Regul. Integr. Comp. Physiol. 294: R601-R605 [Abstract] [Full Text]  
• Ivy, D. D., Doran, A. K., Smith, K. J., Mallory, G. B. Jr, Beghetti, M., Barst, R. J., Brady, D., Law, Y., Parker, D., Claussen, L., Abman, S. H. (2008). Short- and Long-Term Effects of Inhaled Iloprost Therapy in Children With Pulmonary Arterial Hypertension. J Am Coll Cardiol 51: 161-169 [Abstract] [Full Text]  
• Kirsch, M., Kemp-Harper, B., Weissmann, N., Grimminger, F., Schmidt, H. H. H. W. (2008). Sildenafil in hypoxic pulmonary hypertension potentiates a compensatory up-regulation of NO-cGMP signaling. FASEB J. 22: 30-40 [Abstract] [Full Text]  
• King, T. E. Jr., Behr, J., Brown, K. K., du Bois, R. M., Lancaster, L., de Andrade, J. A., Stahler, G., Leconte, I., Roux, S., Raghu, G. (2008). BUILD-1: A Randomized Placebo-controlled Trial of Bosentan in Idiopathic Pulmonary Fibrosis. Am. J. Respir. Crit. Care Med. 177: 75-81 [Abstract] [Full Text]  
• Hemnes, A. R., Zaiman, A., Champion, H. C. (2008). PDE5A inhibition attenuates bleomycin-induced pulmonary fibrosis and pulmonary hypertension through inhibition of ROS generation and RhoA/Rho kinase activation. Am. J. Physiol. Lung Cell. Mol. Physiol. 294: L24-L33 [Abstract] [Full Text]  
• Hoeper, M. M., Seyfarth, H. J., Hoeffken, G., Wirtz, H., Spiekerkoetter, E., Pletz, M. W., Welte, T., Halank, M. (2007). Experience with inhaled iloprost and bosentan in portopulmonary hypertension. Eur Respir J 30: 1096-1102 [Abstract] [Full Text]  
• Thenappan, T., Shah, S. J., Rich, S., Gomberg-Maitland, M. (2007). A USA-based registry for pulmonary arterial hypertension: 1982 2006. Eur Respir J 30: 1103-1110 [Abstract] [Full Text]  
• Diller, G.-P., Dimopoulos, K., Kafka, H., Ho, S. Y., Gatzoulis, M. A. (2007). Model of chronic adaptation: right ventricular function in Eisenmenger syndrome. Eur Heart J Suppl 9: H54-H60 [Abstract] [Full Text]  
• Kamata, Y., Iwamoto, M., Minota, S. (2007). Consecutive use of sildenafil and bosentan for the treatment of pulmonary arterial hypertension associated with collagen vascular disease: sildenafil as reliever and bosentan as controller. Lupus 16: 901-903 [Abstract]  
• Girgis, R. E, Frost, A. E, Hill, N. S, Horn, E. M, Langleben, D., McLaughlin, V. V, Oudiz, R. J, Robbins, I. M, Seibold, J. R, Shapiro, S., Tapson, V. F, Barst, R. J (2007). Selective endothelinA receptor antagonism with sitaxsentan for pulmonary arterial hypertension associated with connective tissue disease. Ann Rheum Dis 66: 1467-1472 [Abstract] [Full Text]  
• Shapiro, S., Hill, N. S. (2007). Transition From IV to Subcutaneous Prostacyclin: Premature Withdrawal?. Chest 132: 741-743 [Full Text]  
• Patel, N. M., Lederer, D. J., Borczuk, A. C., Kawut, S. M. (2007). Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis. Chest 132: 998-1006 [Abstract] [Full Text]  
• Helman, D. L. Jr, Brown, A. W., Jackson, J. L., Shorr, A. F. (2007). Analyzing the Short-term Effect of Placebo Therapy in Pulmonary Arterial Hypertension: Potential Implications for the Design of Future Clinical Trials. Chest 132: 764-772 [Abstract] [Full Text]  
• Servettaz, A, Guilpain, P, Goulvestre, C, Chereau, C, Hercend, C, Nicco, C, Guillevin, L, Weill, B, Mouthon, L, Batteux, F (2007). Radical oxygen species production induced by advanced oxidation protein products predicts clinical evolution and response to treatment in systemic sclerosis. Ann Rheum Dis 66: 1202-1209 [Abstract] [Full Text]  
• Date, H., Kusano, K. F., Matsubara, H., Ogawa, A., Fujio, H., Miyaji, K., Okazaki, M., Yamane, M., Toyooka, S., Aoe, M., Sano, Y., Hanazaki, M., Goto, K., Kasahara, S., Sano, S., Ohe, T. (2007). Living-Donor Lobar Lung Transplantation for Pulmonary Arterial Hypertension After Failure of Epoprostenol Therapy. J Am Coll Cardiol 50: 523-527 [Abstract] [Full Text]  
• Gaine, S. P., Behr, J. (2007). Confidence from experience: dual endothelin receptor antagonism in PAH. ERR 16: 1-2 [Full Text]  
• Humbert, M. (2007). Dual endothelin receptor antagonism: setting standards in PAH. ERR 16: 13-18 [Abstract] [Full Text]  
• du Bois, R. M. (2007). Blocking endothelin: breaking new ground. ERR 16: 24-29 [Abstract] [Full Text]  
• Humbert, M., Segal, E. S., Kiely, D. G., Carlsen, J., Schwierin, B., Hoeper, M. M. (2007). Results of European post-marketing surveillance of bosentan in pulmonary hypertension. Eur Respir J 30: 338-344 [Abstract] [Full Text]  
• Treiber, A., Schneiter, R., Hausler, S., Stieger, B. (2007). Bosentan Is a Substrate of Human OATP1B1 and OATP1B3: Inhibition of Hepatic Uptake as the Common Mechanism of Its Interactions with Cyclosporin A, Rifampicin, and Sildenafil. Drug Metab. Dispos. 35: 1400-1407 [Abstract] [Full Text]  
• Tidswell, M., Higgins, T. L. (2007). The Anesthesiologist and Pulmonary Arterial Hypertension. SEMIN CARDIOTHORAC VASC ANESTH 11: 93-95  
• Takaoka, S., Faul, J. L., Doyle, R. (2007). Current Therapies for Pulmonary Arterial Hypertension. SEMIN CARDIOTHORAC VASC ANESTH 11: 137-148 [Abstract]  
• Badesch, D. B., Abman, S. H., Simonneau, G., Rubin, L. J., McLaughlin, V. V. (2007). Medical Therapy for Pulmonary Arterial Hypertension: Updated ACCP Evidence-Based Clinical Practice Guidelines. Chest 131: 1917-1928 [Abstract] [Full Text]  
• Kevin, L. G., Barnard, M. (2007). Right ventricular failure. Contin Educ Anaesth Crit Care Pain 7: 89-94 [Full Text]  
• D'Alto, M, Vizza, C D, Romeo, E, Badagliacca, R, Santoro, G, Poscia, R, Sarubbi, B, Mancone, M, Argiento, P, Ferrante, F, Russo, M G, Fedele, F, Calabro, R (2007). Long term effects of bosentan treatment in adult patients with pulmonary arterial hypertension related to congenital heart disease (Eisenmenger physiology): safety, tolerability, clinical, and haemodynamic effect. Heart 93: 621-625 [Abstract] [Full Text]  
• Gonzalez-Fernandez, M. A., Garcia-Consuegra, J. (2007). Polyarteritis Nodosa Resistant to Conventional Treatment in a Pediatric Patient. The Annals of Pharmacotherapy 41: 885-890 [Abstract] [Full Text]  
• Wang, X.-X., Zhang, F.-R., Shang, Y.-P., Zhu, J.-H., Xie, X.-D., Tao, Q.-M., Zhu, J.-H., Chen, J.-Z. (2007). Transplantation of Autologous Endothelial Progenitor Cells May Be Beneficial in Patients With Idiopathic Pulmonary Arterial Hypertension: A Pilot Randomized Controlled Trial. J Am Coll Cardiol 49: 1566-1571 [Abstract] [Full Text]  
• Gunther, A., Enke, B., Markart, P., Hammerl, P., Morr, H., Behr, J., Stahler, G., Seeger, W., Grimminger, F., Leconte, I., Roux, S., Ghofrani, H-A. (2007). Safety and tolerability of bosentan in idiopathic pulmonary fibrosis: an open label study. Eur Respir J 29: 713-719 [Abstract] [Full Text]  
• Hoeper, M. M. (2007). Observational trials in pulmonary arterial hypertension: low scientific evidence but high clinical value. Eur Respir J 29: 432-434 [Full Text]  
• Mathai, S. C., Girgis, R. E., Fisher, M. R., Champion, H. C., Housten-Harris, T., Zaiman, A., Hassoun, P. M. (2007). Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J 29: 469-475 [Abstract] [Full Text]  
• Apostolopoulou, S C, Manginas, A, Cokkinos, D V, Rammos, S (2007). Long-term oral bosentan treatment in patients with pulmonary arterial hypertension related to congenital heart disease: a 2-year study. Heart 93: 350-354 [Abstract] [Full Text]  
• Diller, G.-P., Gatzoulis, M. A. (2007). Pulmonary Vascular Disease in Adults With Congenital Heart Disease. Circulation 115: 1039-1050 [Abstract] [Full Text]  
• Benedict, N. J. (2007). Sitaxsentan in the management of pulmonary arterial hypertension. Am J Health Syst Pharm 64: 363-368 [Abstract] [Full Text]  
• Reesink, H. J., van der Plas, M. N., Verhey, N. E., van Steenwijk, R. P., Kloek, J. J., Bresser, P. (2007). Six-minute walk distance as parameter of functional outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. J. Thorac. Cardiovasc. Surg. 133: 510-516 [Abstract] [Full Text]  
• Buch, M H, Denton, C P, Furst, D E, Guillevin, L, Rubin, L J, Wells, A U, Matucci-Cerinic, M, Riemekasten, G, Emery, P, Chadha-Boreham, H, Charef, P, Roux, S, Black, C M, Seibold, J R (2007). Submaximal exercise testing in the assessment of interstitial lung disease secondary to systemic sclerosis: reproducibility and correlations of the 6-min walk test. Ann Rheum Dis 66: 169-173 [Abstract] [Full Text]  
• Villalba, W. O., Sampaio-Barros, P. D., Pereira, M. C., Cerqueira, E. M. F. P., Leme, C. A. Jr, Marques-Neto, J. F., Paschoal, I. A. (2007). Six-Minute Walk Test for the Evaluation of Pulmonary Disease Severity in Scleroderma Patients. Chest 131: 217-222 [Abstract] [Full Text]  
• Wittbrodt, E. T, Abubakar, A. (2007). Sitaxsentan for Treatment of Pulmonary Hypertension. The Annals of Pharmacotherapy 41: 100-105 [Abstract] [Full Text]  
• Barst, R. J., Galie, N., Naeije, R., Simonneau, G., Jeffs, R., Arneson, C., Rubin, L. J. (2006). Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil. Eur Respir J 28: 1195-1203 [Abstract] [Full Text]  
• Collins, N., Bastian, B., Quiqueree, L., Jones, C., Morgan, R., Reeves, G. (2006). Abnormal pulmonary vascular responses in patients registered with a systemic autoimmunity database: Pulmonary Hypertension Assessment and Screening Evaluation using stress echocardiography (PHASE-I). Eur J Echocardiogr 7: 439-446 [Abstract] [Full Text]  
• McLaughlin, V. V., Oudiz, R. J., Frost, A., Tapson, V. F., Murali, S., Channick, R. N., Badesch, D. B., Barst, R. J., Hsu, H. H., Rubin, L. J. (2006). Randomized Study of Adding Inhaled Iloprost to Existing Bosentan in Pulmonary Arterial Hypertension. Am. J. Respir. Crit. Care Med. 174: 1257-1263 [Abstract] [Full Text]  
• Distler, O., Behrens, F., Huscher, D., Foeldvari, I., Zink, A., Nash, P., Denton, C. P., Humbert, M., Matucci-Cerinic, M., Seibold, J., Rubin, L., Furst, D. E. (2006). Need for improved outcome measures in pulmonary arterial hypertension related to systemic sclerosis. Rheumatology (Oxford) 45: 1455-1457 [Full Text]  
• Steiner, M. K., Preston, I. R., Klinger, J. R., Criner, G. J., Waxman, A. B., Farber, H. W., Hill, N. S. (2006). Conversion to bosentan from prostacyclin infusion therapy in pulmonary arterial hypertension: a pilot study.. Chest 130: 1471-1480 [Abstract] [Full Text]  
• Channick, R. N., Olschewski, H., Seeger, W., Staub, T., Voswinckel, R., Rubin, L. J. (2006). Safety and Efficacy of Inhaled Treprostinil as Add-On Therapy to Bosentan in Pulmonary Arterial Hypertension. J Am Coll Cardiol 48: 1433-1437 [Abstract] [Full Text]  
• Newman, J. H., Robbins, I. M. (2006). Exercise Training in Pulmonary Hypertension: Implications for the Evaluation of Drug Trials. Circulation 114: 1448-1449 [Full Text]  
• Hoeper, M. M., Leuchte, H., Halank, M., Wilkens, H., Meyer, F. J., Seyfarth, H. J., Wensel, R., Ripken, F., Bremer, H., Kluge, S., Hoeffken, G., Behr, J. (2006). Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 28: 691-694 [Abstract] [Full Text]  
• Rich, S. (2006). The current treatment of pulmonary arterial hypertension: time to redefine success.. Chest 130: 1198-1202 [Abstract] [Full Text]  
• Dempsey, O.J., Kerr, K.M., Gomersall, L., Remmen, H., Currie, G.P. (2006). Idiopathic pulmonary fibrosis: an update. QJM 99: 643-654 [Abstract] [Full Text]  
• Said, S. I. (2006). Mediators and modulators of pulmonary arterial hypertension. Am. J. Physiol. Lung Cell. Mol. Physiol. 291: L547-L558 [Abstract] [Full Text]  
• Ghofrani, H. A., Voswinckel, R., Reichenberger, F., Weissmann, N., Schermuly, R. T., Seeger, W., Grimminger, F. (2006). Hypoxia- and non-hypoxia-related pulmonary hypertension - Established and new therapies. Cardiovasc Res 72: 30-40 [Abstract] [Full Text]  
• Denton, C P, Humbert, M, Rubin, L, Black, C M (2006). Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis 65: 1336-1340 [Abstract] [Full Text]  
• Sticherling, M. (2006). The role of endothelin in connective tissue diseases. Rheumatology (Oxford) 45: iii8-iii10 [Abstract] [Full Text]  
• Kahler, C. M., Colleselli, D. (2006). Pulmonary arterial hypertension (PAH) in connective tissue diseases. Rheumatology (Oxford) 45: iii11-iii13 [Abstract] [Full Text]  
• Sunderkotter, C., Riemekasten, G. (2006). Pathophysiology and clinical consequences of Raynaud's phenomenon related to systemic sclerosis. Rheumatology (Oxford) 45: iii33-iii35 [Abstract] [Full Text]  
• Selenko-Gebauer, N., Duschek, N., Minimair, G., Stingl, G., Karlhofer, F. (2006). Successful treatment of patients with severe secondary Raynaud's phenomenon with the endothelin receptor antagonist bosentan. Rheumatology (Oxford) 45: iii45-iii48 [Abstract] [Full Text]  
• Riemekasten, G., Sunderkotter, C. (2006). Vasoactive therapies in systemic sclerosis. Rheumatology (Oxford) 45: iii49-iii51 [Abstract] [Full Text]  
• Rubin, L. J. (2006). Endothelin-1 and the Pulmonary Vascular Response to Altitude: A New Therapeutic Target?. Circulation 114: 1350-1351 [Full Text]  
• McLaughlin, V. V., McGoon, M. D. (2006). Pulmonary Arterial Hypertension. Circulation 114: 1417-1431 [Full Text]  
• Modesti, P. A., Vanni, S., Morabito, M., Modesti, A., Marchetta, M., Gamberi, T., Sofi, F., Savia, G., Mancia, G., Gensini, G. F., Parati, G. (2006). Role of Endothelin-1 in Exposure to High Altitude: Acute Mountain Sickness and Endothelin-1 (ACME-1) Study. Circulation 114: 1410-1416 [Abstract] [Full Text]  
• Tripathi, D, Therapondos, G, Ferguson, J W, Newby, D E, Webb, D J, Hayes, P C (2006). Endothelin-1 contributes to maintenance of systemic but not portal haemodynamics in patients with early cirrhosis: a randomised controlled trial. Gut 55: 1290-1295 [Abstract] [Full Text]  
• Zhao, X.-S., Pan, W., Bekeredjian, R., Shohet, R. V. (2006). Endogenous Endothelin-1 Is Required for Cardiomyocyte Survival In Vivo. Circulation 114: 830-837 [Abstract] [Full Text]  
• Barbaro, G, Lucchini, A, Pellicelli, A M, Grisorio, B, Giancaspro, G, Barbarini, G (2006). Highly active antiretroviral therapy compared with HAART and bosentan in combination in patients with HIV-associated pulmonary hypertension.. Heart 92: 1164-1166 [Full Text]  
• Houweling, B., Merkus, D., Sorop, O., Boomsma, F., Duncker, D. J. (2006). Role of endothelin receptor activation in secondary pulmonary hypertension in awake swine after myocardial infarction. J. Physiol. 574: 615-626 [Abstract] [Full Text]  
• Galie, N., Beghetti, M., Gatzoulis, M. A., Granton, J., Berger, R. M.F., Lauer, A., Chiossi, E., Landzberg, M., for the Bosentan Randomized Trial of Endothelin An, (2006). Bosentan Therapy in Patients With Eisenmenger Syndrome: A Multicenter, Double-Blind, Randomized, Placebo-Controlled Study. Circulation 114: 48-54 [Abstract] [Full Text]  
• Diller, G.-P., Dimopoulos, K., Broberg, C. S., Kaya, M. G., Naghotra, U. S., Uebing, A., Harries, C., Goktekin, O., Gibbs, J. S. R., Gatzoulis, M. A. (2006). Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study. Eur Heart J 27: 1737-1742 [Abstract] [Full Text]