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European Heart Journal Advance Access published online on May 11, 2005

European Heart Journal, doi:10.1093/eurheartj/ehi283
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European Heart Journal © The European Society of Cardiology 2005; all rights reserved
Received May 5, 2004
Revised February 4, 2005
Accepted March 24, 2005

Clinical research

Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension

Christian F. Opitz 1*, Roland Wensel 2, Jörg Winkler 3, Michael Halank 4, Leonhard Bruch 5, Franz-Xaver Kleber 5, Gert Höffken 4, Stefan D. Anker 6, Abdissa Negassa 7, Stephan B. Felix 8, Roland Hetzer 9, and Ralf Ewert 8

1 Department of Cardiology, DRK-Kliniken Berlin Westend, Spandauer Damm 130, 14050 Berlin, Germany
2 Department of Internal Medicine II, Universität Regensburg, Regensburg, Germany
3 Department of Internal Medicine I, Universität Leipzig, Leipzig, Germany
4 Department of Internal Medicine I, Carl Gustav Carus Universität Dresden, Dresden, Germany
5 Department of Cardiology, Unfallkrankenhaus Berlin, Berlin, Germany
6 Applied Cachexia Research, Department of Cardiology, Universitätsklinikum Charité Berlin, Campus Virchow-Klinikum, Berlin, Germany
7 Department of Epidemiology and Population Health, Albert Einstein College of Medicine of Yeshiva University, Bronx, NY, USA
8 Department of Internal Medicine B, Ernst-Moritz-Arndt Universität Greifswald, Greifswald, Germany
9 Department for Heart, Thoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany

* To whom correspondence should be addressed.
Christian F. Opitz, E-mail: c.opitz{at}drk-kliniken-westend.de


   Abstract

Aims To describe the long-term clinical efficacy of inhaled iloprost as first-line vasodilator mono-therapy in patients with idiopathic pulmonary arterial hypertension (IPAH).

Methods and results Seventy-six IPAH patients were prospectively identified and treated with inhaled iloprost. Clinical, haemodynamic, and exercise parameters were obtained at baseline, after 3 and 12 months of therapy and yearly thereafter. Four endpoints were prospectively defined as follows: (i) death, (ii) transplantation, (iii) switch to intravenous (i.v.) therapy, or (iv) addition of or switch to other active oral therapy. During follow-up (535 ± 61 days), 11 patients died, six were transplanted, 25 were switched to i.v. prostanoids, 16 received additional or other oral therapy, and 12 patients discontinued iloprost inhalation for other reasons. Event-free survival at 3, 12, 24, 36, 48, and 60 months was 81, 53, 29, 20, 17 and 13%, respectively. Among haemodynamic and exercise parameters, mixed venous oxygen saturation (P < 0.001), right atrial pressure (P < 0.001), and peak oxygen uptake (P = 0.002) were associated with event-free survival.

Conclusion In this study, only a minority of patients could be stabilized with inhaled iloprost mono-therapy during a follow-up period of up to 5 years. In the presence of multiple treatment options, chronic iloprost inhalation as mono-therapy appears to have a limited role.

Keywords: Pulmonary arterial hypertension; Inhaled iloprost; Chronic therapy.
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