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Results of the Prospective, Multicenter, Single-Arm, Open-Label, Phase 4 MOTION Study of Riociguat in Pulmonary Arterial Hypertension

Namita Sood

Alvaro Aranda

David Platt

Frank Kleinjung

Gerald O'Brien


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Conference: 2018 PHA International PH Conference & Scientific Sessions

Release Date: 06.28.2018

Presentation Type: Abstracts

File Download: Conference 2018_1059

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Pulmonary arterial hypertension (PAH) is a progressive disease with debilitating impact on exercise capacity and health-related quality of life (HRQoL). Treatment studies typically aim to improve functional capacity—as measured by treatment-related change in 6-minute walk distance (6MWD)—and to prevent progression of disease. Few trials have evaluated treatment-related change in patient-reported outcomes (PROs) such as HRQoL. The MOTION study (NCT02191137) is designed to explore PROs in patients with PAH who are treated with riociguat.


MOTION is a prospective, multicenter, single-arm, open-label, phase 4, 24-week trial designed to assess whether riociguat monotherapy will improve PROs among newly diagnosed PAH patients not on therapy. Patients received riociguat over a 10-week titration phase, from a starting dosage of 0.5 mg 3 times daily (TID) increased every 2 weeks in 0.5-mg increments, until individual optimal dosage (up to a maximum of 2.5 mg TID) was reached. Patients remained on therapy during a 14-week maintenance phase. The primary endpoint is the reported change from baseline (BL) in the Living with Pulmonary Hypertension (LPH) questionnaire after 24 weeks of treatment. LPH scores range from 0–105; higher scores indicate worse HRQoL. Secondary variables include change from BL to Week 16 in the LPH, as well as change from BL to Weeks 16 and 24 in the Work Limitation Questionnaire-8, the Short Form-12, World Health Organization Functional Class, Modified Borg Dyspnea Scale, and 6MWD.1


All data are reported as mean ± standard deviation (SD). There were 97 patients screened; 75 patients were enrolled, and 10 patients did not complete the trial due to death (n=4), adverse event (n=2), patient withdrawal (n=3), and other reasons (n=1). Of the 65 patients who completed the trial, Week 24 LPH data were available for 58 patients. The study population was predominantly female (88%, n=66) and white non-Hispanic/Latino (79%, n=59), with mean age of 62 (±11) years. PAH was categorized as idiopathic (65%, n=49), connective tissue disease–associated (28%, n=21), portal hypertension (3%, n=2), anorexigen/amphetamine-associated PAH (1%, n=1), and familial (1%, n=1). LPH score improved from baseline to last visit by −5.4 (±27.8) in the intent-to-treat analysis (n=75, P=0.048), and from baseline to Week 24 by −10.8 (±21.9) in the completers analysis (n=58, P=0.0002).


These data suggest that riociguat monotherapy may significantly improve HRQoL, as measured by improvements in the LPH score among newly diagnosed patients with PAH.