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

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.

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Conflict of interest: R.L. Benza reports receiving grants from Actelion, Bayer AG, Bellerophon Therapeutics and Eiger Biopharmaceuticals. Conflict of interest: D. Langleben reports receiving honoraria, consultation fees, research support and/or travel expenses from Acceleron Pharma, Actelion, Arena Pharmaceuticals, Bayer AG, Northern Therapeutics, PhaseBio and United Therapeutics outside the submitted work. Conflict of interest: A.R. Hemnes has received grants from the NIH and CMREF. She has served as a consultant to Acceleron Pharma, Bayer, Gossamer Bio, Janssen Pharmaceuticals and United Therapeutics. She holds stock in Tenax Therapeutics. Conflict of interest: A. Vonk Noordegraaf is supported by the Netherlands CardioVascular Research Initiative (CVON-2012-08 PHAEDRA, CVON-2017-10 DOLPHIN-GENESIS) and the Netherlands Organization for Scientific Research (NWO-VICI: 918.16.610), has received speaker fees from Ferrer and Johnson & Johnson in the past 3 years and has served as a member of the scientific advisory board of Morphogen-XI. Conflict of interest: S. Rosenkranz reports remunerations for lectures and/or consultancy from Abbott, Acceleron Pharma, Actelion, Arena Pharmaceuticals, Bayer AG, Bristol Myers Squibb, Ferrer, GlaxoSmithKline, Janssen Pharmaceuticals, Merck Sharp & Dohme Corp., Novartis, Pfizer and United Therapeutics; and research support to his institution from Actelion, Bayer, Novartis, Pfizer and United Therapeutics outside the submitted work. Conflict of interest: T. Thenappan reports consultation fees from Actelion and United Therapeutics outside the submitted work. Conflict of interest: P.M. Hassoun is serving on a scientific advisory board for Merck & Co. Conflict of interest: I.R. Preston reports grants and personal fees from Actelion, Arena Pharmaceuticals, Bayer AG, Gilead Sciences and United Therapeutics; grants from Liquidia; and personal fees from Pfizer and Reata Pharmaceuticals outside the submitted work. Conflict of interest: S. Ghio has nothing to disclose. Conflict of interest: R. Badagliacca reports grants from Bayer, Dompè, Merck Sharp & Dohme Corp., Ferrer, Galenica, GlaxoSmithKline, Janssen Pharmaceuticals and United Therapeutics. Conflict of interest: C.D. Vizza reports fees for lectures and/or consultations from Acceleron Pharma, Actelion, Bayer, GlaxoSmithKline, Merck Sharp & Dohme Corp. and United Therapeutics outside the submitted work. Conflict of interest: I.M. Lang has served as a steering committee member for Actelion and has received speaker fees and grants from Actelion, AOP Orphan Pharmaceuticals, Ferrer and United Therapeutics outside the submitted work. Conflict of interest: C. Meier is an employee of Bayer AG. Conflict of interest: E. Grünig reports fees for lectures and/or consultations from Actelion, Bayer AG, GlaxoSmithKline, Merck Sharp & Dohme Corp., Pfizer and United Therapeutics outside the submitted work.