Switch the risk: from PDE5i to Riociguat in real world pulmonary hypertension

Abstract Introduction Switching from phosphodiesterase-5 inhibitors (PDE5i) to Riociguat is associated with increased efficacy in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Objective To show clinical efficacy of replacing PDE5i to Riociguat in a portuguese PH-dedicated centre. Methods Retrospective single-centre study of PAH and inoperable/persistent/recurrent CTEPH patients (pts) who switched vasodilator therapy. Parameters were stratified according to the 2022 PH guidelines and stratification as low/intermediate/high-risk PH followed the COMPERA registry, before switching, at 3–6 months of follow-up (FUP) and until the last clinical evaluation, balloon pulmonary angioplasty (BPA), heart transplant or death. Results Of 13 pts, 75.0% had CTEPH (4 pts had persistent disease after surgical endarterectomy), 16.7% had PAH and 1pt had mixed PAH/CTEPH. Mean age was 56.2 ± 16.3years and 83.3% were females. Mean time until switch was 33.5 ± 25.5 months. Previous therapy was: 33.3% Sildenafil, 41.7% Sildenafil/Bosentan and 25% Sildenafil/Bosentan/prostanoid. Before switching, 66.7% had intermediate and 33.3% low-risk PH; at a FUP of 4.4 ± 1.7 months, 8.3% had intermediate and 91.7% low-risk PH. There was a significant benefit in the COMPERA risk (1.50[1.33;1.62] vs. 1.22[1.09;1.40], P = 0.011). Long-term FUP was performed in 8 pts at 48.2 ± 35.6 months (death 1pt, transplant 1pt, BPA 3 pts). Medical treatment was Riociguat in 33.3%, Riociguat and Bosentan in 58.3%, and 1pt with Riociguat, Bosentan and Treprostinil. Clinical benefit was significant at long-term FUP when compared to the initial evaluation (1.22[1.09;1.40], P = 0.011), but not to the first FUP. Conclusion This study corroborates previous evidence regarding the vasodilator switch and also suggests that the treatment goal is maintained throughout long-term FUP.