Riociguat and the right ventricle in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Raymond L Benza; David Langleben; Anna R Hemnes; Anton Vonk Noordegraaf; Stephan Rosenkranz; Thenappan Thenappan; Paul M Hassoun; Ioana R Preston; Stefano Ghio; Roberto Badagliacca; Carmine D Vizza; Irene M Lang; Christian Meier; Ekkehard Grünig

doi:10.1183/16000617.0061-2022

Riociguat and the right ventricle in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Eur Respir Rev. 2022 Oct 5;31(166):220061. doi: 10.1183/16000617.0061-2022. Print 2022 Dec 31.

Authors

Affiliations

¹ Dept of Medicine, Ohio State University Wexner Medical Center, Columbus, OH, USA Raymond.Benza@osumc.edu.
² Center for Pulmonary Vascular Disease, Division of Cardiology, Jewish General Hospital, McGill University, Montreal, Canada.
³ Division of Allergy, Pulmonary and Critical Care Medicine, Dept of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
⁴ VU University Medical Center, Pulmonary Diseases, Amsterdam, The Netherlands.
⁵ Dept of Cardiology and Cologne Cardiovascular Research Center, Cologne University Heart Center, Cologne, Germany.
⁶ Cardiovascular Division, Dept of Medicine, University of Minnesota, Minneapolis, MN, USA.
⁷ Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD, USA.
⁸ Pulmonary, Critical Care and Sleep Medicine Division, Tufts Medical Center, Boston, MA, USA.
⁹ Division of Cardiology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
¹⁰ Dept of Cardiology, La Sapienza University, Rome, Italy.
¹¹ Division of Cardiology, Dept of Internal Medicine II, Medical University of Vienna, Allgemeines Krankenhaus, Vienna, Austria.
¹² Global Medical Affairs, Bayer AG, Berlin, Germany.
¹³ Centre for Pulmonary Hypertension, Thoraxklinik-Heidelberg gGmbH, Heidelberg University Hospital, Heidelberg, Germany.
¹⁴ Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg, Germany.

Abstract

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are progressive diseases that can lead to right heart failure and death. Right ventricular dysfunction, hypertrophy and maladaptive remodelling are consequences of increased right ventricular (RV) afterload in PAH and CTEPH and are indicative of long-term outcomes. Because RV failure is the main cause of morbidity and mortality in PAH and CTEPH, successful treatments should lead to improvements in RV parameters. Riociguat is a soluble guanylate cyclase stimulator approved for the treatment of PAH and inoperable or persistent/recurrent CTEPH after pulmonary endarterectomy. This review examines the current evidence showing the effect of riociguat on the right ventricle, with particular focus on remodelling, function and structural parameters in preclinical models and patients with PAH or CTEPH.

Publication types

Review

MeSH terms

Chronic Disease
Familial Primary Pulmonary Hypertension
Heart Ventricles
Humans
Hypertension, Pulmonary* / diagnosis
Hypertension, Pulmonary* / drug therapy
Hypertension, Pulmonary* / etiology
Pulmonary Arterial Hypertension*
Pulmonary Embolism* / complications
Pulmonary Embolism* / drug therapy
Pyrazoles
Pyrimidines
Soluble Guanylyl Cyclase / therapeutic use

Substances

Pyrazoles
Pyrimidines
Soluble Guanylyl Cyclase
riociguat