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Haemodynamic effects of riociguat in CTEPH and PAH: a 10-year observational study
Suqiao Yang1,2,3, Yuanhua Yang1,2,3, Yixiao Zhang1,2,3
1Dept of Pulmonary and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.
Insights
Riociguat treatment significantly improved pulmonary vascular resistance and cardiac index in patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) over 8 years. World Health Organization functional class showed predictive value for long-term outcomes.
Area of Science:
- Cardiology
- Pulmonology
- Pharmacology
Background:
- Long-term riociguat treatment enhances exercise capacity in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH).
- Previous studies indicated riociguat's benefit in exercise capacity, but long-term hemodynamic effects required further evaluation.
Purpose of the Study:
- To evaluate the long-term hemodynamic effects of riociguat in patients with PAH and inoperable CTEPH.
- To assess the impact of riociguat on pulmonary vascular resistance, cardiac index, and survival over an extended period.
Main Methods:
- A single-center, long-term observational study involving 37 patients (19 CTEPH, 18 PAH).
- Riociguat was administered at up to 2.5 mg three times daily.
- Primary outcome: pulmonary vascular resistance (PVR). Secondary outcomes included mean pulmonary arterial pressure (PAP), cardiac index, mortality, clinical worsening events, 6-minute walk distance (6MWD), and World Health Organization functional class (WHO FC).
Main Results:
- Median follow-up was 96 months. Survival estimates at 8 years were 0.61 for all patients.
- Riociguat significantly decreased PVR (p<0.001) and increased cardiac index (p<0.001).
- 6MWD increased by 43.1±59.6 m, and 40% of patients improved their WHO FC. PAP did not improve. 8-year clinical worsening-free survival was significantly better for patients in WHO FC I/II at baseline (0.51 vs 0.19, p=0.026).
Conclusions:
- Riociguat demonstrated sustained hemodynamic improvements in PVR and cardiac index for up to 8 years in PAH and inoperable CTEPH patients.
- Mean pulmonary arterial pressure did not show significant improvement.
- World Health Organization functional class at baseline may predict long-term prognosis in these patient groups.
Background:
Long-term treatment with riociguat has been shown to enhance exercise capacity in patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH). This study sought to evaluate the long-term haemodynamic effects of riociguat in patients with PAH and inoperable CTEPH.
Methods:
During this single-centre long-term observational study, riociguat was administered at a three-times-daily dose of up to 2.5 mg. The primary outcome was pulmonary vascular resistance (PVR). The secondary outcomes included mean pulmonary arterial pressure (PAP), cardiac index, mortality, clinical worsening events, 6-min walk distance (6MWD) and World Health Organization functional class (WHO FC).
Results:
37 patients (CTEPH n=19; PAH n=18) were included. The median follow-up period was 96 months. The survival estimates for all the patients at 1/3/5/8 years were 0.97/0.86/0.72/0.61, without significant differences between patients with CTEPH and PAH. At the final data cut-off, PVR decreased (1232±462 dyn·s·cm-5 versus 835±348 dyn·s·cm-5, p<0.001), cardiac index increased (1.7±0.4 L·min-1·m-2 versus 2.4±0.5 L·min-1·m-2, p<0.001), 6MWD increased by 43.1±59.6 m, and WHO FC improved/stabilised/worsened in 40%/35%/25% of patients versus baseline. Improvement in PAP was not shown. Compared with patients in WHO FC I/II and III/IV at baseline, the 8-year clinical worsening-free survival estimates were 0.51 versus 0.19 (p=0.026).
Conclusions:
Riociguat improved PVR and cardiac index for up to 8 years, but not PAP. WHO FC may have certain predictive value for the long-term prognosis.
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