Abstract
Background: Riociguat was well tolerated and improved exercise and functional capacity in patients with pulmonary arterial hypertension (PAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH) in a 12 -week Phase II trial. We present final data from the long-term extension phase of this study. Methods: During this multicenter, open-label, uncontrolled long-term extension study, riociguat dose could be changed at the physician's discretion (range 0.5-2.5 mg three times daily). The primary outcome was long-term safety and tolerability of riociguat;secondary outcomes included 6-minute walking distance, World Health Organization functional class, survival, and clinical worsening-free survival. Results: Sixty-eight patients (inoperable CTEPH, n = 41;PAH, n = 27) entered the long-term extension. Median treatment duration at the final data cut-off was 77 months. The most common adverse events were nasopharyngitis (57%) and peripheral edema (37%). Three patients (4%) experienced serious adverse events of hemoptysis: two moderate, one severe, none fatal or considered drug-related. At Month 48, 6-minute walking distace increased from baseline by 69 +/- 105 m, and World Health Organization functional class improved/stabilized/worsened versus baseline in 50/45/5% of patients. Three-year survival and clinical worsening-free survival were 91% and 49%, respectively (with patients censored if they withdrew without experiencing an event). Starting a new PAH treatment was the most frequent clinical worsening event. Conclusions: Improvements in exercise and functional capacity were maintained at 4 years in patients remaining on treatment, with no new safety signals identified. These data support riociguat as a long-term treatment option for PAH and inoperable CTEPH. Trial registered at: ClinicalTrials.gov. Registration number: NCT00454558.
Dokumententyp: | Zeitschriftenartikel |
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Fakultät: | Medizin |
Themengebiete: | 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin und Gesundheit |
URN: | urn:nbn:de:bvb:19-epub-50886-4 |
ISSN: | 0954-6111 |
Sprache: | Englisch |
Dokumenten ID: | 50886 |
Datum der Veröffentlichung auf Open Access LMU: | 14. Jun. 2018, 09:44 |
Letzte Änderungen: | 04. Nov. 2020, 13:28 |