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European Heart Journal Advance Access originally published online on May 11, 2005
European Heart Journal 2005 26(18):1895-1902; doi:10.1093/eurheartj/ehi283
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© The European Society of Cardiology 2005. All rights reserved. For Permissions, please e-mail: journals.permissions@oupjournals.org

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

Christian F. Opitz1,*,{dagger}, Roland Wensel2,{dagger}, Jörg Winkler3, Michael Halank4, Leonhard Bruch5, Franz-Xaver Kleber5, Gert Höffken4, Stefan D. Anker6, Abdissa Negassa7, Stephan B. Felix8, Roland Hetzer9 and Ralf Ewert8

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

Received 5 May 2004; revised 4 February 2005; accepted 24 March 2005; online publish-ahead-of-print 11 May 2005.

* Corresponding author. Tel: +49 30 3035 4301; fax: +49 30 3035 4309. E-mail address: c.opitz{at}drk-kliniken-westend.de

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.

Key Words: Pulmonary arterial hypertension • Inhaled iloprost • Chronic therapy


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