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 Dario Vizza, Iréne Lang, Christian Meier, Ekkehard Grünig
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.