Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry

Hossein Ardeschir Ghofrani*, Miguel Angel Gomez Sanchez, Marc Humbert, David Pittrow, Gérald Simonneau, Henning Gall, Ekkehard Grünig, Hans Klose, Michael Halank, David Langleben, Repke J. Snijder, Pilar Escribano Subias, Lisa M. Mielniczuk, Tobias J. Lange, Jean Luc Vachiéry, Hubert Wirtz, Douglas S. Helmersen, Iraklis Tsangaris, Joan A. Barberá, Joanna Pepke-ZabaAnco Boonstra, Stephan Rosenkranz, Silvia Ulrich, Regina Steringer-Mascherbauer, Marion Delcroix, Pavel Jansa, Iveta Šimková, George Giannakoulas, Jens Klotsche, Evgenia Williams, Christian Meier, Marius M. Hoeper, Jorge Caneva, Graciela Tuhay, Mirta Diez, Maria Lujan Talavera, Adriana Acosta, Norberto Vulcano, Martin Bosio, Lorena Maldonado, Sabino Deleo, Luciano Melatini, Anne Keogh, Eugene Kotlyar, John Feenstra, Nathan Dwyer, Heath Adams, Wendy Stevens, Peter Steele, Myriam Alexandre, NEW COLLABORATORS LIST

*Corresponding author for this work

Research output: Contribution to journalArticleResearchpeer-review

18 Citations (Scopus)


Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice. Methods: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat. Patients were followed for at least 1 year and up to 4 years from enrollment or until 30 days after stopping riociguat treatment. Primary safety outcomes were adverse events (AEs) and serious adverse events (SAEs) coded using Medical Dictionary for Regulatory Activities preferred terms and System Organ Classes version 21.0, collected during routine clinic visits and collated via case report forms. Results: In total, 956 patients with CTEPH were included in the analysis. The most common AEs in these patients were peripheral edema/edema (11.7%), dizziness (7.5%), right ventricular (RV)/cardiac failure (7.7%), and pneumonia (5.0%). The most common SAEs were RV/cardiac failure (7.4%), pneumonia (4.1%), dyspnea (3.6%), and syncope (2.5%). Exposure-adjusted rates of hemoptysis/pulmonary hemorrhage and hypotension were low and comparable to those in the long-term extension study of riociguat (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase–Stimulator Trial [CHEST-2]). Conclusion: Data from EXPERT show that in patients with CTEPH, the safety of riociguat in routine practice was consistent with the known safety profile of the drug, and no new safety concerns were identified.

Original languageEnglish
Article number106220
JournalRespiratory Medicine
Publication statusPublished - Mar 2021
Externally publishedYes


  • Chronic thromboembolic pulmonary hypertension
  • Clinical practice
  • Real-world
  • Registry
  • Riociguat
  • Safety


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