Selexipag for the treatment of chronic thromboembolic pulmonary hypertension

Abstract

Treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) remain limited. Selexipag, an oral selective IP prostacyclin-receptor agonist approved for pulmonary arterial hypertension, is a potential treatment option for CTEPH.In this multicentre, randomised, double-blind, placebo-controlled study, 78 Japanese patients with inoperable CTEPH or persistent/recurrent pulmonary hypertension after pulmonary endarterectomy and/or balloon pulmonary angioplasty were randomly assigned to receive placebo or selexipag. The primary endpoint was the change in pulmonary vascular resistance (PVR) from baseline to week 20. The secondary endpoints were changes in other haemodynamic parameters, 6-min walk distance (6 WMD), Borg Dyspnoea Scale score, World Health Organisation (WHO) functional class, EuroQol 5 dimensions 5-level and N-terminal pro-brain natriuretic peptide.The change in PVR was −98.2±111.3 dyn·s·cm−5 and −4.6±163.6 dyn·s·cm−5 in the selexipag and placebo groups, respectively (mean difference, −93.5 dyn·s·cm−5; 95% confidence interval, −156.8, −30.3; p=0.006). The changes in cardiac index (p<0.001) and Borg Dyspnoea Scale score (p=0.036) were also significantly improved over placebo. 6WMD and WHO functional class were not significantly improved. The common adverse events in the selexipag group were corresponded to those generally observed following a prostacyclin analogue is administered.Selexipag significantly improved PVR and other haemodynamic variables in patients with CTEPH, although exercise capacity remained unchanged. Further large-scale investigation is necessary to prove the role of selexipag in CTEPH.

Emergent balloon pulmonary angioplasty in a patient with central‐type chronic thromboembolic pulmonary hypertension with cardiac shock

“… 1 The treatment strategy has been extended recently to anticoagulation, pulmonary vasodilators (riociguat and selexipag), BPA, and PEA. 1 , 4 , 5 These current advances provide a good prognosis, and the 5‐year survival rate is 92.0%, which is extremely good compared to those of other types of PH. 3 Generally, this disease has a chronic course; however, in some cases, the condition of the patient deteriorates, as in our case.…”

“…Chronic thromboembolic pulmonary hypertension is defined as Group 4 PH 1 . The treatment strategy has been extended recently to anticoagulation, pulmonary vasodilators (riociguat and selexipag), BPA, and PEA 1,4,5 . These current advances provide a good prognosis, and the 5‐year survival rate is 92.0%, which is extremely good compared to those of other types of PH 3 .…”

Pulmonary Hypertension Association's 2022 International Conference Scientific Sessions Overview

“… 94 The CTREPH trial 95 also demonstrated significant improvement in 6MWD with high‐dose subcutaneous treprostinil in inoperable CTEPH. Selexipag has also been shown to reduce PVR in patients with CTEPH in a small RCT, leading to its approval for this condition in Japan 96 ; however, a larger study—the SELECT study (NCT03689244) was stopped early due to futility so utility in this condition is unclear.…”

Corsair rotational bougie technique for facilitating balloon passage through a total occlusion lesion: a case report of balloon pulmonary angioplasty

“…Selexipag was reported to be effective for CTEPH. 1 In Japan, it has been approved for CTEPH since August 2021. The patient’s PAP was significantly high; therefore, we initiated combination therapy of riociguat and selexipag.…”