Initial Riociguat Monotherapy and Transition from Sildenafil to Riociguat in Patients with Idiopathic Pulmonary Arterial Hypertension: Influence on Right Heart Remodeling and Right Ventricular-Pulmonary Arterial Coupling

Irina N Taran, Anna A Belevskaya, Marina A Saidova, Tamila V Martynyuk, Irina E Chazova, Irina N Taran, Anna A Belevskaya, Marina A Saidova, Tamila V Martynyuk, Irina E Chazova

Abstract

Purpose: To evaluate the influence of riociguat on World Health Organization functional class (WHO FC), 6-min walk distance (6MWD), right heart remodeling, and right ventricular-pulmonary arterial (RV-PA) coupling in patients with idiopathic pulmonary arterial hypertension (IPAH) who are treatment-naïve or who have failed to achieve treatment goals with sildenafil therapy.

Methods: Twenty patients with IPAH were enrolled: 12 had not previously received PAH-targeted therapy (treatment-naïve subgroup) and 8 had been receiving sildenafil therapy but failed to achieve treatment goals; on entering this pilot study these 8 patients were switched from sildenafil to riociguat therapy (treatment-switch subgroup). Patients received riociguat individually dose-adjusted up to a maximum of 2.5 mg three times daily. After 12 weeks, patients were assessed for WHO FC, 6MWD, right heart remodeling, and RV-PA coupling.

Results: Riociguat significantly improved WHO FC in treatment-naïve patients (from 0/4/8/0 patients in WHO I/II/III/IV at baseline to 1/6/5/0 at week 12) and in treatment-switch patients (from 0/4/4/0 patients in WHO I/II/III/IV at baseline to 1/4/3/0 at week 12). Additionally, treatment-naïve and treatment-switch patients showed significant improvements at week 12 versus baseline in 6MWD (increases of + 76.8 m and + 71.6 m, respectively), RV systolic function, and RV-PA coupling.

Conclusion: These results support the proven efficacy of riociguat in patients with IPAH, including treatment-naïve patients and those switching to riociguat following failure to achieve treatment goals with sildenafil, and suggest that it may be possible to delay disease progression in this patient group.

Keywords: Echocardiography; Heart remodeling; Idiopathic pulmonary arterial hypertension; Right ventricular–pulmonary arterial coupling; Riociguat; Sildenafil.

Conflict of interest statement

Conflict of interest

The authors declare that they have no conflict of interest. This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from all individual participants included in the study.

Figures

Fig. 1
Fig. 1
Riociguat dose-adjustment scheme. SBP systolic blood pressure, TID three times daily
Fig. 2
Fig. 2
WHO FC after 12 weeks of riociguat treatment. IPAH idiopathic pulmonary arterial hypertension, WHO FC World Health Organization functional class

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Source: PubMed

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