@article{3102729,
    title = "Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry",
    author = "Ghofrani, H.-A. and Gomez Sanchez, M.-A. and Humbert, M. and Pittrow, D. and Simonneau, G. and Gall, H. and Grünig, E. and Klose, H. and Halank, M. and Langleben, D. and Snijder, R.J. and Escribano Subias, P. and Mielniczuk, L.M. and Lange, T.J. and Vachiéry, J.-L. and Wirtz, H. and Helmersen, D.S. and Tsangaris, I. and Barberá, J.A. and Pepke-Zaba, J. and Boonstra, A. and Rosenkranz, S. and Ulrich, S. and Steringer-Mascherbauer, R. and Delcroix, M. and Jansa, P. and Šimková, I. and Giannakoulas, G. and Klotsche, J. and Williams, E. and Meier, C. and Hoeper, M.M. and Caneva, J. and Tuhay, G. and Diez, M. and Talavera, M.L. and Acosta, A. and Vulcano, N. and Bosio, M. and Maldonado, L. and Deleo, S. and Melatini, L. and Keogh, A. and Kotlyar, E. and Feenstra, J. and Dwyer, N. and Adams, H. and Stevens, W. and Steele, P. and Proudman, S. and Minson, R. and Reeves, G. and Lavender, M. and Ng, B. and Mackenzie, M. and Barry, L. and Gruenberger, M. and Huber, C. and Lang, I. and Tilea, I. and Sadushi-Kolici, R. and Löffler-Ragg, J. and Feistmantl, L.-T. and Evrard, P. and Louis, R. and Guiot, J. and Naldi, M. and De Pauw, M. and Mehta, S. and Camacho, R.C. and Tovar, P.P. and Londoño, A. and Campo, F. and Garcia, P. and Lema, C. and Orozco-Levi, M. and Martinez, W. and Gomez, J.E. and Nielsen-Kudsk, J.E. and Mellemkjaer, S. and Anton, L. and Altraja, A. and Vihinen, T. and Vasankari, T. and Sitbon, O. and Cottin, V. and Têtu, L. and Noël-Savina, E. and Shearman, N. and Tayler, S. and Olzik, I. and Kulka, C. and Grimminger, J. and Simon, M. and Nolde, A. and Oqueka, T. and Harbaum, L. and Egenlauf, B. and Ewert, R. and Schulz, C. and Regotta, S. and Kramer, T. and Knoop-Busch, S. and Gerhardt, F. and Konstantinides, S. and Pitsiou, G. and Stanopoulos, I. and Sourla, E. and Mouratoglou, S. and Karvounis, H. and Pappas, A. and Georgopoulos, D. and Fanaridis, M. and Mitrouska, I. and Michalis, L. and Pappas, K. and Kotsia, A. and Gaine, S. and Vizza, C.D. and Manzi, G. and Poscia, R. and Badagliacca, R. and Agostoni, P. and Bruno, N. and Farina, S. and D'Alto, M. and Argiento, P. and Correra, A. and Di Marco, G.M. and Cresci, C. and Vannucchi, V. and Torricelli, E. and Garcea, A. and Pesci, A. and Sardella, L. and Paciocco, G. and Pane, F. and D'Armini, A.M. and Pin, M. and Grazioli, V. and Massola, G. and Sciortino, A. and Prediletto, R. and Bauleo, C. and Airò, E. and Ndreu, R. and Pavlickova, I. and Lunardi, C. and Mulè, M. and Farruggio, S. and Costa, S. and Galgano, G. and Petruzzi, M. and De Luca, A. and Lombardi, F. and Roncon, L. and Conte, L. and Picariello, C. and Wirtz, G. and Alexandre, M. and Vonk-Noordegraaf, A. and Boogaard, H. and Mager, J. and Reesink, H. and van den Toorn, L.M. and Boomars, K. and Andreassen, A.K. and Castro, G. and Tania, G. and Baptista, R. and Marinho, A. and Shiang, T. and Oliveira, A. and Coutinho, D. and Sousa, J. and Loureiro, M.J. and Repolho, D. and Martins Jesus, S.M. and Capinha, M. and Agostinho, J. and Cardoso, T. and Rocha, A. and Espinha, M. and Ivanov, K.I. and Alexeeva, D.E. and Batalina, M.V. and Hegya, D.V. and Zvereva, T.N. and Avdeev, S.N. and Tsareva, N.A. and Galyavich, A.S. and Nikolaevich, B.A. and Filippov, E.V. and Yakovleva, O.E. and Pavlova, O.B. and Skripkina, E.S. and Martynyuk, T.V. and Bukatova, I.F. and Tregubova, A.V. and Platonov, D.Y. and Kolomeytseva, T.M. and Al Dalaan, A. and Abdelsayed, A.A. and Weheba, I. and Saleemi, S. and Sakkijha, H. and Bohacekova, M. and Valkovicova, T. and Farkasova, I. and Quezada, C.A. and Piccari, L. and Blanco, I. and Sebastian, L. and Roman, A. and Lopez, M. and Otero, R. and Elias, T. and Jara, L. and Asencio, I. and Arjona, J.J. and Almagro, R.M. and Cárdenas, S.L. and García, S.A. and Rodríguez, P.V. and Lopez, R. and Garcia, A. and Avilés, F.F. and De La Pava, S. and Yotti, R. and Peñate, G.P. and Marrero, F.L. and Cifrián Martínez, J.M. and Martinez-Meñaca, A. and Alonso, L.P. and Rozas, S.F. and Fernandez, D.I. and Cuesta, V.M. and Söderberg, S. and Bartfay, S.-E. and Rundqvist, B. and Alfetlawi, M. and Wodlin, P. and Schwarz, E.I. and Speich, R. and Lador, F. and Rochat, T. and Gasche-Soccal, P. and Hsu, C.-H. and Lin, T.-H. and Su, H.-M. and Lai, W.-T. and Chu, C.Y. and Hsu, P.-C. and Voon, W.-C. and Yen, H.-W. and Yih-Jer Wu, J. and Wu, S.-H. and Huang, W.-P. and Fong, M.-C. and Huang, C.-L. and Kuo, P.-H. and Lin, Y.-H. and Lin, J.-L. and Hung, C.-S. and Wu, C.-K. and Sung, S.-H. and Huang, W.-C. and Cheng, C.-C. and Kuo, S.-H. and Wang, W.-H. and Ho, W.-J. and Hsu, T.-S. and Mutlu, B. and Atas, H. and Ongen, G. and Un, Z. and Okumus, G. and Hanta, I. and Corris, P. and Peacock, A. and Church, C. and Toshner, M. and Newnham, M. and NEW COLLABORATORS LIST",
    journal = "Advances in Respiratory Medicine",
    year = "2021",
    volume = "178",
    publisher = "W.B. Saunders Ltd",
    doi = "10.1016/j.rmed.2020.106220",
    keywords = "ambrisentan;  bosentan;  guanylate cyclase;  iloprost;  macitentan;  prostacyclin;  prostanoid;  riociguat;  treprostinil;  pyrazole derivative;  pyrimidine derivative;  riociguat, adverse event;  aged;  Article;  chronic thromboembolic pulmonary hypertension;  clinical practice;  clinical trial;  cohort analysis;  combination drug therapy;  disease registry;  dizziness;  drug safety;  dyspnea;  faintness;  female;  heart right ventricle failure;  hemoptysis;  human;  hypotension;  lung hemorrhage;  major clinical study;  male;  Medical Dictionary for Regulatory Activities;  monotherapy;  multicenter study;  peripheral edema;  phase 3 clinical trial;  pneumonia;  priority journal;  prospective study;  pulmonary hypertension;  randomized controlled trial;  chronic disease;  complication;  data analysis;  lung embolism;  middle aged;  multicenter study (topic);  pulmonary hypertension;  randomized controlled trial (topic);  recurrent disease;  register;  safety;  time factor;  treatment outcome, Aged;  Chronic Disease;  Data Analysis;  Female;  Humans;  Hypertension, Pulmonary;  Male;  Middle Aged;  Multicenter Studies as Topic;  Prospective Studies;  Pulmonary Embolism;  Pyrazoles;  Pyrimidines;  Randomized Controlled Trials as Topic;  Recurrence;  Registries;  Safety;  Time Factors;  Treatment Outcome",
    abstract = "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. © 2020 The Authors"
}