Inhaled Iloprost for Severe Pulmonary Hypertension
Horst Olschewski, M.D., Gerald Simonneau, M.D., Nazzareno Galiè, M.D., Timothy Higenbottam, M.D., Robert Naeije, M.D., Lewis J. Rubin, M.D., Sylvia Nikkho, M.D., Rudolf Speich, M.D., Marius M. Hoeper, M.D., Jürgen Behr, M.D., Jörg Winkler, M.D., Olivier Sitbon, M.D., Wladimir Popov, M.D., H. Ardeschir Ghofrani, M.D., Alessandra Manes, M.D., David G. Kiely, M.D., Ralph Ewert, M.D., Andreas Meyer, M.D., Paul A. Corris, F.R.C.P., Marion Delcroix, M.D., Miguel Gomez-Sanchez, M.D., Harald Siedentop, Dipl.Stat., Werner Seeger, M.D., for the Aerosolized Iloprost Randomized Study Group
Background Uncontrolled studies suggested that aerosolized iloprost,a stable analogue of prostacyclin, causes selective pulmonaryvasodilatation and improves hemodynamics and exercise capacityin patients with pulmonary hypertension.
Methods We compared repeated daily inhalations of 2.5 or 5.0µg of iloprost (six or nine times per day; median inhaleddose, 30 µg per day) with inhalation of placebo. A totalof 203 patients with selected forms of severe pulmonary arterialhypertension and chronic thromboembolic pulmonary hypertension(New York Heart Association [NYHA] functional class III or IV)were included. The primary end point was met if, after week12, the NYHA class and distance walked in six minutes were improvedby at least one class and at least 10 percent, respectively,in the absence of clinical deterioration according to predefinedcriteria and death.
Results The combined clinical end point was met by 16.8 percentof the patients receiving iloprost, as compared with 4.9 percentof the patients receiving placebo (P=0.007). There were increasesin the distance walked in six minutes of 36.4 m in the iloprostgroup as a whole (P=0.004) and of 58.8 m in the subgroup ofpatients with primary pulmonary hypertension. Overall, 4.0 percentof patients in the iloprost group (including one who died) and13.7 percent of those in the placebo group (including four whodied) did not complete the study (P=0.024); the most commonreason for withdrawal was clinical deterioration. As comparedwith base-line values, hemodynamic values were significantlyimproved at 12 weeks when measured after iloprost inhalation(P<0.001), were largely unchanged when measured before iloprostinhalation, and were significantly worse in the placebo group.Further significant beneficial effects of iloprost treatmentincluded an improvement in the NYHA class (P=0.03), dyspnea(P=0.015), and quality of life (P=0.026). Syncope occurred withsimilar frequency in the two groups but was more frequentlyrated as serious in the iloprost group, although this adverseeffect was not associated with clinical deterioration.
Conclusions Inhaled iloprost is an effective therapy for patientswith severe pulmonary hypertension.
Source Information
From the Department of Internal Medicine II, University Clinic, Giessen, Germany (H.O., H.A.G., W.S.); Service de Pneumologie, Hôpital Antoine Béclère, Clamart, France (G.S., O.S.); Istituto di Cardiologia, Università di Bologna, Bologna, Italy (N.G., A.M.); Royal Hallamshire Hospital, Sheffield, United Kingdom (T.H., D.G.K.); Department of Cardiology, Erasme University Hospital, Brussels, Belgium (R.N.); University of California at San Diego Medical Center, La Jolla (L.J.R.); Schering, Berlin, Germany (S.N., H.S.); Department of Internal Medicine, University Hospital, Zurich, Switzerland (R.S., W.P.); Department of Pneumology, Hannover Medical School, Hannover, Germany (M.M.H.); the Division of Pulmonary Diseases, University of MunichGroßhadern, Munich, Germany (J.B.); Department of Pneumology, University Clinic, Leipzig, Germany (J.W.); Department of Pneumology and Infectious Diseases, Ernst Moritz Arndt University, Greisswald, Germany (R.E.); Bereich Pneumologie, Medizinische Kernklinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany (A.M.); Freeman Hospital, High Heaton, Newcastle-upon-Tyne, United Kingdom (P.A.C.); Department of Pneumology, Gasthuisberg University Clinic, Leuven, Belgium (M.D.); and Pulmonary Hypertension Unit, Hospital 12 de Octubre, Madrid (M.G.-S.).
Address reprint requests to Dr. Seeger at the Department of Internal Medicine II, University Clinic, Klinikstr. 36, D-35392 Giessen, Germany, or at werner.seeger{at}innere.med.uni-giessen.de.
Warnes, C. A., Williams, R. G., Bashore, T. M., Child, J. S., Connolly, H. M., Dearani, J. A., del Nido, P., Fasules, J. W., Graham, T. P. Jr, Hijazi, Z. M., Hunt, S. A., King, M. E., Landzberg, M. J., Miner, P. D., Radford, M. J., Walsh, E. P., Webb, G. D.
(2008). ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol
52: e1-e121
[Full Text]
Warnes, C. A., Williams, R. G., Bashore, T. M., Child, J. S., Connolly, H. M., Dearani, J. A., del Nido, P., Fasules, J. W., Graham, T. P. Jr, Hijazi, Z. M., Hunt, S. A., King, M. E., Landzberg, M. J., Miner, P. D., Radford, M. J., Walsh, E. P., Webb, G. D.
(2008). ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol
52: 1890-1947
[Full Text]
Warnes, C. A., Williams, R. G., Bashore, T. M., Child, J. S., Connolly, H. M., Dearani, J. A., del Nido, P., Fasules, J. W., Graham, T. P. Jr, Hijazi, Z. M., Hunt, S. A., King, M. E., Landzberg, M. J., Miner, P. D., Radford, M. J., Walsh, E. P., Webb, G. D.
(2008). ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation
118: e714-e833
[Full Text]
Warnes, C. A., Williams, R. G., Bashore, T. M., Child, J. S., Connolly, H. M., Dearani, J. A., del Nido, P., Fasules, J. W., Graham, T. P. Jr, Hijazi, Z. M., Hunt, S. A., King, M. E., Landzberg, M. J., Miner, P. D., Radford, M. J., Walsh, E. P., Webb, G. D.
(2008). ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation
118: 2395-2451
[Full Text]
Leuchte, H. H., Baezner, C., Baumgartner, R. A., Bevec, D., Bacher, G., Neurohr, C., Behr, J.
(2008). Inhalation of vasoactive intestinal peptide in pulmonary hypertension. Eur Respir J
32: 1289-1294
[Abstract][Full Text]
Hirose, K., Marui, A., Arai, Y., Kushibiki, T., Kimura, Y., Sakaguchi, H., Yuang, H., Chandra, B.I.R. S., Ikeda, T., Amano, S., Tabata, Y., Komeda, M.
(2008). Novel approach with intratracheal administration of microgelatin hydrogel microspheres incorporating basic fibroblast growth factor for rescue of rats with monocrotaline-induced pulmonary hypertension.. J. Thorac. Cardiovasc. Surg.
136: 1250-1256
[Abstract][Full Text]
Simonneau, G., Rubin, L. J., Galie, N., Barst, R. J., Fleming, T. R., Frost, A. E., Engel, P. J., Kramer, M. R., Burgess, G., Collings, L., Cossons, N., Sitbon, O., Badesch, D. B., for the PACES Study Group,
(2008). Addition of Sildenafil to Long-Term Intravenous Epoprostenol Therapy in Patients with Pulmonary Arterial Hypertension: A Randomized Trial. ANN INTERN MED
149: 521-530
[Abstract][Full Text]
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]
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]
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]
Hill, N. S., Preston, I. R., Roberts, K. E.
(2008). Inoperable Chronic Thromboembolic Pulmonary Hypertension: Treatable With Medical Therapy. Chest
134: 221-223
[Full Text]
Suntharalingam, J., Treacy, C. M., Doughty, N. J., Goldsmith, K., Soon, E., Toshner, M. R., Sheares, K. K., Hughes, R., Morrell, N. W., Pepke-Zaba, J.
(2008). Long-term Use of Sildenafil in Inoperable Chronic Thromboembolic Pulmonary Hypertension. Chest
134: 229-236
[Abstract][Full Text]
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]
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]
Gomberg-Maitland, M.
(2008). Traditional and Alternative Designs for Pulmonary Arterial Hypertension Trials. Proc Am Thorac Soc
5: 610-616
[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]
Chen, H., Taichman, D. B., Doyle, R. L.
(2008). Health-related Quality of Life and Patient-reported Outcomes in Pulmonary Arterial Hypertension. Proc Am Thorac Soc
5: 623-630
[Abstract][Full Text]
Lai, Y.-J., Pullamsetti, S. S., Dony, E., Weissmann, N., Butrous, G., Banat, G.-A., Ghofrani, H. A., Seeger, W., Grimminger, F., Schermuly, R. T.
(2008). Role of the Prostanoid EP4 Receptor in Iloprost-mediated Vasodilatation in Pulmonary Hypertension. Am. J. Respir. Crit. Care Med.
178: 188-196
[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]
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]
Gomberg-Maitland, M., Olschewski, H.
(2008). Prostacyclin therapies for the treatment of pulmonary arterial hypertension. Eur Respir J
31: 891-901
[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]
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]
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]
Obata, H., Sakai, Y., Ohnishi, S., Takeshita, S., Mori, H., Kodama, M., Kangawa, K., Aizawa, Y., Nagaya, N.
(2008). Single Injection of a Sustained-release Prostacyclin Analog Improves Pulmonary Hypertension in Rats. Am. J. Respir. Crit. Care Med.
177: 195-201
[Abstract][Full Text]
Pepke-Zaba, J., Gilbert, C., Collings, L., Brown, M. C. J.
(2008). Sildenafil Improves Health-Related Quality of Life in Patients With Pulmonary Arterial Hypertension. Chest
133: 183-189
[Abstract][Full Text]
Wryobeck, J. M., Lippo, G., McLaughlin, V., Riba, M., Rubenfire, M.
(2007). Psychosocial Aspects of Pulmonary Hypertension: A Review. Psychosomatics
48: 467-475
[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]
Reichenberger, F., Voswinckel, R., Enke, B., Rutsch, M., El Fechtali, E., Schmehl, T., Olschewski, H., Schermuly, R., Weissmann, N., Ghofrani, H. A., Grimminger, F., Mayer, E., Seeger, W.
(2007). Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension. Eur Respir J
30: 922-927
[Abstract][Full Text]
Rubenfire, M., McLaughlin, V. V., Allen, R. P., Elliott, G., Park, M. H., Wade, M., Schilz, R.
(2007). Transition From IV Epoprostenol to Subcutaneous Treprostinil in Pulmonary Arterial Hypertension: A Controlled Trial. Chest
132: 757-763
[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]
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]
Hsu, H.-H., Chen, J.-S., Chen, Y.-S., Ko, W.-J., Kuo, S.-W., Lee, Y.-C.
(2007). Short-term intravenous iloprost for treatment of reperfusion lung oedema after pulmonary thromboendarterectomy. Thorax
62: 459-461
[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]
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]
Hoeper, M. M., Lee, S. H., Voswinckel, R., Palazzini, M., Jais, X., Marinelli, A., Barst, R. J., Ghofrani, H. A., Jing, Z.-C., Opitz, C., Seyfarth, H.-J., Halank, M., McLaughlin, V., Oudiz, R. J., Ewert, R., Wilkens, H., Kluge, S., Bremer, H.-C., Baroke, E., Rubin, L. J.
(2006). Complications of Right Heart Catheterization Procedures in Patients With Pulmonary Hypertension in Experienced Centers. J Am Coll Cardiol
48: 2546-2552
[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]
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]
Fantozzi, I., Platoshyn, O., Wong, A. H., Zhang, S., Remillard, C. V., Furtado, M. R., Petrauskene, O. V., Yuan, J. X.-J.
(2006). Bone morphogenetic protein-2 upregulates expression and function of voltage-gated K+ channels in human pulmonary artery smooth muscle cells. Am. J. Physiol. Lung Cell. Mol. Physiol.
291: L993-L1004
[Abstract][Full Text]
Fox, D J, Khattar, R S
(2006). Pulmonary arterial hypertension: classification, diagnosis and contemporary management. Postgrad. Med. J.
82: 717-722
[Full Text]
Voswinckel, R., Enke, B., Reichenberger, F., Kohstall, M., Kreckel, A., Krick, S., Gall, H., Gessler, T., Schmehl, T., Ghofrani, H. A., Schermuly, R. T., Grimminger, F., Rubin, L. J., Seeger, W., Olschewski, H.
(2006). Favorable Effects of Inhaled Treprostinil in Severe Pulmonary Hypertension: Results From Randomized Controlled Pilot Studies. J Am Coll Cardiol
48: 1672-1681
[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]
Mereles, D., Ehlken, N., Kreuscher, S., Ghofrani, S., Hoeper, M. M., Halank, M., Meyer, F. J., Karger, G., Buss, J., Juenger, J., Holzapfel, N., Opitz, C., Winkler, J., Herth, F. F.J., Wilkens, H., Katus, H. A., Olschewski, H., Grunig, E.
(2006). Exercise and Respiratory Training Improve Exercise Capacity and Quality of Life in Patients With Severe Chronic Pulmonary Hypertension. Circulation
114: 1482-1489
[Abstract][Full Text]
Gomberg-Maitland, M.
(2006). Learning to pair therapies and the expanding matrix for pulmonary arterial hypertension: is more better?. Eur Respir J
28: 683-686
[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]
Cenedese, E., Speich, R., Dorschner, L., Ulrich, S., Maggiorini, M., Jenni, R., Fischler, M.
(2006). Measurement of quality of life in pulmonary hypertension and its significance. Eur Respir J
28: 808-815
[Abstract][Full Text]
Rich, S.
(2006). The current treatment of pulmonary arterial hypertension: time to redefine success.. Chest
130: 1198-1202
[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]
Riemekasten, G., Sunderkotter, C.
(2006). Vasoactive therapies in systemic sclerosis. Rheumatology (Oxford)
45: iii49-iii51
[Abstract][Full Text]
McLaughlin, V. V., McGoon, M. D.
(2006). Pulmonary Arterial Hypertension. Circulation
114: 1417-1431
[Full Text]
Ulrich, S., Fischler, M., Speich, R., Popov, V., Maggiorini, M.
(2006). Chronic thromboembolic and pulmonary arterial hypertension share acute vasoreactivity properties.. Chest
130: 841-846
[Abstract][Full Text]
Hughes, R. J., Jais, X., Bonderman, D., Suntharalingam, J., Humbert, M., Lang, I., Simonneau, G., Pepke-Zaba, J.
(2006). The efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension: a 1-year follow-up study. Eur Respir J
28: 138-143
[Abstract][Full Text]
Williams, M H, Das, C, Handler, C E, Akram, M R, Davar, J, Denton, C P, Smith, C J, Black, C M, Coghlan, J G
(2006). Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart
92: 926-932
[Abstract][Full Text]
Lang, I., Gomez-Sanchez, M., Kneussl, M., Naeije, R., Escribano, P., Skoro-Sajer, N., Vachiery, J.-L.
(2006). Efficacy of Long-term Subcutaneous Treprostinil Sodium Therapy in Pulmonary Hypertension. Chest
129: 1636-1643
[Abstract][Full Text]
Hoeper, M. M., Mayer, E., Simonneau, G., Rubin, L. J.
(2006). Chronic Thromboembolic Pulmonary Hypertension. Circulation
113: 2011-2020
[Full Text]
Robbins, I. M., Kawut, S. M., Yung, D., Reilly, M. P., Lloyd, W., Cunningham, G., Loscalzo, J., Kimmel, S. E., Christman, B. W., Barst, R. J.
(2006). A study of aspirin and clopidogrel in idiopathic pulmonary arterial hypertension.. Eur Respir J
27: 578-584
[Abstract][Full Text]
McLaughlin, V. V.
(2006). 'Raising the bar' for the treatment of pulmonary arterial hypertension. Eur Heart J
27: 510-511
[Full Text]
Elliot, C A, Kiely, D G
(2006). Pulmonary hypertension. Contin Educ Anaesth Crit Care Pain
6: 17-22
[Full Text]
Voswinckel, R., Ghofrani, H. A., Grimminger, F., Seeger, W., Olschewski, H.
(2006). Inhaled Treprostinil for Treatment of Chronic Pulmonary Arterial Hypertension. ANN INTERN MED
144: 149-150
[Full Text]
Rubin, L. J.
(2006). Pulmonary arterial hypertension.. Proc Am Thorac Soc
3: 111-115
[Abstract][Full Text]
Griffiths, M. J.D., Evans, T. W.
(2005). Inhaled Nitric Oxide Therapy in Adults. NEJM
353: 2683-2695
[Full Text]
Kataoka, M., Nagaya, N., Satoh, T., Itoh, T., Murakami, S., Iwase, T., Miyahara, Y., Kyotani, S., Sakai, Y., Kangawa, K., Ogawa, S.
(2005). A Long-Acting Prostacyclin Agonist with Thromboxane Inhibitory Activity for Pulmonary Hypertension. Am. J. Respir. Crit. Care Med.
172: 1575-1580
[Abstract][Full Text]
Kramm, T., Eberle, B., Guth, S., Mayer, E.
(2005). Inhaled iloprost to control residual pulmonary hypertension following pulmonary endarterectomy. Eur. J. Cardiothorac. Surg.
28: 882-888
[Abstract][Full Text]
Sandifer, B. L., Brigham, K. L., Lawrence, E. C., Mottola, D., Cuppels, C., Parker, R. E.
(2005). Potent effects of aerosol compared with intravenous treprostinil on the pulmonary circulation. J. Appl. Physiol.
99: 2363-2368
[Abstract][Full Text]
Provencher, S., Jais, X., Yaici, A., Sitbon, O., Humbert, M., Simonneau, G.
(2005). Clinical Challenges in Pulmonary Hypertension: Roger S. Mitchell Lecture. Chest
128: 622S-628S
[Abstract][Full Text]
Provencher, S., Jais, X., Yaici, A., Sitbon, O., Humbert, M., Simonneau, G.
(2005). Clinical Challenges in Pulmonary Hypertension: Roger S. Mitchell Lecture. Chest
128: 622S-628S
[Abstract][Full Text]
Galie, N., Ghofrani, H. A., Torbicki, A., Barst, R. J., Rubin, L. J., Badesch, D., Fleming, T., Parpia, T., Burgess, G., Branzi, A., Grimminger, F., Kurzyna, M., Simonneau, G., the Sildenafil Use in Pulmonary Arterial Hypertens,
(2005). Sildenafil Citrate Therapy for Pulmonary Arterial Hypertension. NEJM
353: 2148-2157
[Abstract][Full Text]
Hoeper, M. M., Markevych, I., Spiekerkoetter, E., Welte, T., Niedermeyer, J.
(2005). Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J
26: 858-863
[Abstract][Full Text]
Newman, J. H.
(2005). Pulmonary Hypertension. Am. J. Respir. Crit. Care Med.
172: 1072-1077
[Abstract][Full Text]
Kawut, S. M., Horn, E. M., Berekashvili, K. K., Widlitz, A. C., Rosenzweig, E. B., Barst, R. J.
(2005). von Willebrand Factor Independently Predicts Long-term Survival in Patients With Pulmonary Arterial Hypertension. Chest
128: 2355-2362
[Abstract][Full Text]
Leuchte, H. H., Holzapfel, M., Baumgartner, R. A., Neurohr, C., Vogeser, M., Behr, J.
(2005). Characterization of Brain Natriuretic Peptide in Long-term Follow-up of Pulmonary Arterial Hypertension. Chest
128: 2368-2374
[Abstract][Full Text]
Bonderman, D., Nowotny, R., Skoro-Sajer, N., Jakowitsch, J., Adlbrecht, C., Klepetko, W., Lang, I. M.
(2005). Bosentan Therapy for Inoperable Chronic Thromboembolic Pulmonary Hypertension. Chest
128: 2599-2603
[Abstract][Full Text]
Ghofrani, H. A., Seeger, W., Grimminger, F.
(2005). Imatinib for the treatment of pulmonary arterial hypertension.. NEJM
353: 1412-1413
[Full Text]
Opitz, C. F., Wensel, R., Winkler, J., Halank, M., Bruch, L., Kleber, F.-X., Hoffken, G., Anker, S. D., Negassa, A., Felix, S. B., Hetzer, R., Ewert, R.
(2005). Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. Eur Heart J
26: 1895-1902
[Abstract][Full Text]
Rubin, L. J., Badesch, D. B.
(2005). Evaluation and Management of the Patient with Pulmonary Arterial Hypertension. ANN INTERN MED
143: 282-292
[Abstract][Full Text]
Seyfarth, H.-J., Pankau, H., Hammerschmidt, S., Schauer, J., Wirtz, H., Winkler, J.
(2005). Bosentan Improves Exercise Tolerance and Tei Index in Patients With Pulmonary Hypertension and Prostanoid Therapy. Chest
128: 709-713
[Abstract][Full Text]
Schermuly, R. T., Yilmaz, H., Ghofrani, H. A., Woyda, K., Pullamsetti, S., Schulz, A., Gessler, T., Dumitrascu, R., Weissmann, N., Grimminger, F., Seeger, W.
(2005). Inhaled Iloprost Reverses Vascular Remodeling in Chronic Experimental Pulmonary Hypertension. Am. J. Respir. Crit. Care Med.
172: 358-363
[Abstract][Full Text]
Merklinger, S. L., Jones, P. L., Martinez, E. C., Rabinovitch, M.
(2005). Epidermal Growth Factor Receptor Blockade Mediates Smooth Muscle Cell Apoptosis and Improves Survival in Rats With Pulmonary Hypertension. Circulation
112: 423-431
[Abstract][Full Text]