Treatment of pulmonary hypertension with riociguat: a review of current evidence and future perspectives

ABSTRACT

Introduction

Pulmonary arterial hypertension (PAH) is still a chronic disorder characterized by high morbidity and mortality. Chronic thromboembolic pulmonary hypertension (CTEPH) is another form of pulmonary hypertension (PH) for which pulmonary endarterectomy (PEA) is the treatment of choice. However, not all patients are operable, while PH is often recurrent or persistent. Thus, for both disorders novel treatment options are urgently needed.

Areas covered

This review describes the mechanism of action of riociguat, a soluble guanylate cyclase (sGC) stimulator, with a dual mode of action. The most relevant publications are presented regarding the efficacy of riociguat in PAH and CTEPH, and also data regarding its potential effect on other forms of PH.

Expert opinion

Riociguat is a first-in-class drug approved for the treatment of PAH as a monotherapy or added to endothelin-receptor antagonists as a sequential combination therapy, and for the treatment of inoperable CTEPH or persistent/recurrent PH after PEA. As it can stimulate sGC independently of NO, it could be beneficial in PAH patients with inadequate response to phosphodiesterase 5 inhibitors (PDE5i). Future studies are needed to evaluate whether drug switching is beneficial in PAH and which baseline markers could guide the optimal initial treatment selection.

KEYWORDS:

• Riociguat
• pulmonary arterial hypertension
• chronic thromboembolic pulmonary hypertension
• monotherapy
• combination treatment

Declaration of interest

AK Boutou declares having received honoraria for lectures from Chiesi Farmaceutici and Elpen Hellas. He was also the recipient of a European Respiratory Society Short-Term Research Fellowship in 2013. Meanwhile, G Pitsiou has received honoraria for lectures from Merck Sharp and Dohme, GlaxoSmithKline, Actelion and Bayer. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

One referee declares that they received honoraria for advisory board participation and/or speaker fees from Actelion, Bayer, Pfizer, GlaxoSmithKline, Boehringer Ingelheim and Roche. Peer reviewers on this manuscript have no other relevant financial relationships or otherwise to disclose.

Additional information

Funding

This manuscript was not funded.