Retrospective single-centre study of 39 patients with PH (21 PAH, 18 CTEPH) in PATENT-1, PATENT-2, PATENT PLUS, CHEST-1, CHEST-2 or CTEPH Early Access Study (EAS) | 1.0–2.5 mg three times daily for 3–12 months | Function
Increased TAPSE at 6 months (p=0.025) and 12 months (p=0.002) Increased tricuspid annular velocity at 12 months (p=0.006) Decreased systolic PA pressure and mPAP at 3 months (both p=0.03) Increased CO and CI at 3 months (both p<0.001) Decreased PVR at 3 months (p<0.001) Size
Decreased RV area at 3 months (p=0.002), 6 months and 12 months (both p<0.001) Decreased RA area at 6 months (p=0.06) and 12 months (p<0.001) Decreased RV free wall thickness at 3 months (p<0.05), 6 months and 12 months (both p<0.01) Other remodelling
| [35] |
Retrospective multicentre study of 71 patients with PH (32 PAH, 39 CTEPH) in phase II, PATENT-1, PATENT-2, PATENT PLUS, CHEST-1, CHEST-2 or CTEPH EAS (RIVER study) | 1.0–2.5 mg three times daily for 3–12 months | Function
Improved RV systolic function at 12 months (p=0.016) Increased TAPSE at 12 months (p<0.001) Decreased tricuspid regurgitation velocity at 12 months (p=0.005) RV fractional area change at 12 months (p<0.001) Size
Decreased RV area at 3 months, 6 months and 12 months (all p<0.001) Decreased RA area at 6 months and 12 months (both p<0.001) Decreased RV thickness at 12 months (p=0.023) Other remodelling
| [36] |
Retrospective study of 27 patients with PH (7 PAH, 20 CTEPH) | Mean dose of 7.3±0.7 mg administered for a mean 220 days | Function
Size
Decreased basal, mid and longitudinal RV diameters (all p=0.001) Decreased RV end-diastolic area index (p=0.0005)
| [37] |
Retrospective analysis of 45 patients with PH (14 PAH, 31 CTEPH) | Mean final daily dose of 7.2±0.9 mg administered for a mean 234 days | Function
Decreased mPAP (p=0.008) Increased CI (p=0.04) Decreased PVR (p=0.016) Increased RV systolic excursion velocity (p=0.001) Decreased RV global longitudinal strain (p<0.001) Increased fractional area change (p<0.001) Decreased RV dyssynchrony index (p=0.012) Decreased estimated PA systolic pressure (p<0.001) Decreased tricuspid regurgitation pressure gradient (p<0.001) Size
Decreased RV basal (p<0.001), mid (p<0.001) and longitudinal (p=0.002) diameters Decreased RV end-diastolic and end-systolic area indices (p<0.001) Decreased RA area index (p=0.0014)
| [38] |
Post hoc analysis of 341 patients with PAH in PATENT-1 and 238 patients with CTEPH in CHEST-1 | Up to 2.5 mg three times daily for 12 weeks for PATENT-1 and 16 weeks for CHEST-1 | Function compared with placebo treated
Increased stroke volume, SVI and cardiac efficiency in PATENT-1 and CHEST-1 (all p<0.0001) Increased RV work in PATENT-1 (p=0.0002) and CHEST-1 (p=0.0317) Increased RV work index in PATENT-1 (p<0.0001) and CHEST-1 (p=0.0338) Increased RV power in PATENT-1 (p=0.0002) and CHEST-1 (p=0.0317) Decreased PA elastance in PATENT-1 and CHEST-1 (both p<0.0001)
| [39] |
Single-centre prospective randomised open-label trial of 21 patients with CTEPH who had mPAP <30 mmHg after undergoing BPA | Up to 2.5 mg three times daily for 6 months (8 of the 10 patients in the riociguat arm received less than the maximum dose) | Function at peak workload compared with standard of care treated
| [40] |
Post hoc analysis of the RESPITE study in 61 patients with PAH | 1.0–2.5 mg three times daily for 24 weeks following a 1–3-day PDE5i treatment-free period | Function following switch
Increased cardiac efficiency (p=0.0004) Increased stroke volume (p=0.0044) Increased SVI (p=0.0052) Maintained RV work (p=0.1770) Maintained RV work index (p=0.0793) Maintained RV power (p=0.1770) Maintained PA elastance (p=0.0888)
| [41] |
Retrospective analysis of 28 patients with CTEPH who were switched from sildenafil to riociguat | Up to 2.5 mg three times daily for a median follow-up time of 5.8 (3.6–7.9) months following switching | Function following switch
| [42] |
Single-centre retrospective analysis of 26 patients with PAH receiving triple therapy with riociguat, macitentan and selexipag | Of 26 patients, 24 received riociguat up-titrated to maximum tolerated dose for a median observation period of 441 days | Function
Decreased mean RA pressure (p=0.005) Decreased mPAP (p<0.001) Increased CO (p<0.001) Increased CI (p<0.001) Decreased PVR (p<0.001) Increased TAPSE (p=0.001) Increased RV systolic excursion velocity (p=0.001) Increased fractional area change (p<0.001) Size
| [43] |
Prospective study of 6 patients with CTEPH | 0.5–2.5 mg three times daily for 6 months | Function
Other remodelling
| [44] |
Single-site pilot study of 20 patients with PAH (12 treatment-naïve patients and 8 who were switched from sildenafil to riociguat) | 1.0–2.5 mg three times daily for 12 weeks | Function
Decreased systolic PA pressure (p=0.003) Decreased mPAP (p=0.007) Increased RV fractional area change (p=0.04) Decreased RV end-diastolic volume (p=0.003) Decreased RV end-systolic volume (p=0.002) Size
Other remodelling
| [45] |