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

Hoeper, Marius M.; Sánchez, María Jesús García; Humbert, Marc; Pittrow, David; Simonneau, Gérald; Gall, Henning; Grünig, Ekkehard; Klose, Hans; Halank, Michael; Langleben, David; Snijder, Repke J.; Escribano, Pilar; Mielniczuk, Lisa; Lange, Tobias; Vachiéry, Jean‐Luc; Wirtz, Hubert; Helmersen, Douglas; Tsangaris, Iraklis; Barberà, Joan Albert; Pepke‐Zaba, Joanna; Boonstra, Anco; Rosenkranz, Stephan; Ulrich, Silvia; Steringer‐Mascherbauer, Regina; Delcroix, Marion; Jansa, Pavel; Šimková, Iveta; Giannakoulas, George; Klotsche, Jens; Williams, E. T.; Meier, Christian; Ghofrani, Hossein‐Ardeschir; Cáneva, Jorge; Tuhay, Graciela; Díez, Mirta; Talavera, María L.; Acosta, Adriana; Vulcano, Norberto; Bosio, Martín; Maldonado, Lorena; Deleo, Sabino; Melatini, Luciano; Keogh, Anne; Kotlyar, Eugene; Feenstra, John; Dwyer, Nathan; Adams, Heath; Stevens, Wendy; Steele, Peter; Proudman, Susanna; Minson, Robert; Reeves, Glenn; Lavender, Melanie; Ng, Benjamin; Mackenzie, Michele; Barry, Lisa C.; Gruenberger, Margarethe; Huber, Charlotte; Lang, Irene; Tilea, Ioana; Sadushi-Koliçi, Roela; Löffler‐Ragg, Judith; Feistmantl, Lisa-Theresa; Evrard, P; Guiot, Julien; Naldi, Marco; Pauw, Michel De; Louis, Renaud; Mehta, Sanjay R.; Camacho, Rafael Conde; Tovar, Patricia Parada; Londoño, Alejandro; Campo, Felipe; Garcia, P Awamleh; Lema, Camila; Orozco-Levi, Mauricio; Martínez, William; Gómez, Juan Esteban; Nielsen‐Kudsk, Jens Erik; Mellemkjær, Søren; Anton, Ly; Altraja, Alan; Vihinen, Tapani; Vasankari, Tuija; Sitbon, Olivier; Cottin, Vincent; Têtu, L.; Noël-Savina, É.; Shearman, Nicole; Tayler, Susanne; Olzik, Ilona; Kulka, Christine; Grimminger, Jan; Simon, Marcel; Nolde, A.; Oqueka, Tim; Harbaum, Lars; Egenlauf, Benjamin; Ewert, Ralf; Schulz, Christian; Regotta, Sabine; Kramer, Tilmann; Knoop-Busch, Susanne; Gerhardt, Felix; Konstantinides, Stavros; Pitsiou, Georgia; Stanopoulos, Ioannis; Sourla, Evdokia; Mouratoglou, Sofia; Karvounis, Haralambos; Pappas, Athanasios; Mitrouska, Ioanna; Georgopoulos, Dimitrios; Fanaridis, Michail; Michalis, Lampros K.; Παππάς, Κωνσταντίνος; Kotsia, Anna; Gaine, Seán; Vizza, Carmine Dario; Manzi, Giovanna; Poscia, Roberto; Badagliacca, Roberto; Agostoni, Piergiuseppe; Bruno, Noemi; Farina, Stefania; D′Alto, Michele; Argiento, Paola; Correra, Anna; Marco, Giovanni Maria Di; Cresci, Chiara; Vannucchi, Vieri; Torricelli, Elena; Garcea, Alessio; Pesci, Alberto; Sardella, Luca; Paciocco, Giuseppe; Pane, Federico; D’Armini, Andrea Maria; Pin, Maurizio; Grazioli, Valentina; Massola, Giulia; Sciortino, Antonio; Prediletto, Renato; Bauleo, Carolina; Airò, Edoardo; Ndreu, Rudina; Pavlickova, Ivana; Lunardi, Claudio; Farruggio, Silvia; Costa, Serena; Mulè, Massimiliano; Galgano, Giuseppe; M, Petruzzi; Luca, Anna De; Lombardi, Francesco; Roncon, Loris; Conte, Luca; Picariello, Claudio; Wirtz, Gil; Alexandre, Myriam; Vonk‐Noordegraaf, Anton; Boogaard, H.; Mager, Johannes J.; Reesink, Herre J.; Toorn, Leon M. van den; Boomars, Karin A.; Andreassen, Arne K.; Castro, Graça; Tania, Gonçalves; Baptista, Rui; Marinho, António; Shiang, Teresa; Oliveira, Ana de; Coutinho, Daniel; Sousa, Joana; Loureiro, María José; Repolho, Débora; Jesus, Susana; Capinha, Marta; Agostinho, João R.; Cardoso, Tânia Aparecida Marchiori Oliveira; Rocha, Andréia Silva da; Espinha, Mafalda; Ivanov, K. I.; Alexeeva, Dalyana Eduardovna; Batalina, Marina Vadimovna; Hegya, D.; Зверева, Т. Н.; Авдеев, С. Н.; Tsareva, Natalia A.; Galyavich, А. S.; Nikolaevich, Bykov Aleksander; Филиппов, Е В; Yakovleva, O. E.; Pavlova, Olga Borisovna; Skripkina, Elena; Martynyuk, T V; Bukatova, Irina Fedorovna; Tregubova, Anna V.; Platonov, Dmitry Yurievich; Kolomeytseva, Tatyana Mikhaylovna; Dalaan, Abdullah Al; Abd-Elsayed, A A; Weheba, Ihab; Saleemi, Sarferaz; Sakkijha, Hussam; Bohacekova, Marcela; Valkovicova, Tatiana; Farkasova, Iveta; Quezada, Carlos; Piccari, Lucilla; Blanco, Isabel; Sebastián, Laura; Román, Antonio; Lopez, M; Otero, Remedios; Elías, Teresa; Jara, Luis; Asencio, Isabel; Arjona, Josefa Jiménez; Almagro, Raúl Menor; López-Cárdenas, Salvador; García, Salvador Alcaraz; Rodríguez, Patricia Villanueva; López, Raquel; García, Alberto Eloy Martos; Fernández-Avilés, Francisco; Pava, Sebastian De La; Yotti, Raquel; Peñate, Gregorio Pérez; Marrero, Fernando León; Cifrián, José Manuel; Martínez-Meñaca, Amaya; Alonso, Lecue Pilar; Rozas, S. Fernández; Fernández, David Iturbe; Cuesta, V.M. Mora; Söderberg, Stefan; Bartfay, Sven‐Erik; Rundqvist, Bengt; Alfetlawi, Monthir; Wodlin, Peter; Schwarz, Esther I.; Speich, Rudolf; Lador, Frédéric; Rochat, Thierry; Gasche-Soccal, Paola Marina Alessandra; Hsu, Chih‐Hsin; Lin, Tsung‐Hsien; Su, Ho‐Ming; Lai, Wen‐Ter; Chu, Chun-Yuan; Hsu, Po-Chao; Voon, Wen‐Chol; Yen, Hsueh‐Wei; Wu, Jacob Yih-Jer; Wu, Shu-Hao; Huang, Wen-Pin; Fong, Man-Cai; Huang, Chien-Lung; Kuo, Ping-Hung; Lin, Yen‐Hung; Lin, Jiunn‐Lee; Hung, Chi‐Sheng; Wu, Cho-Kai; Sung, Shih‐Hsien; Huang, Wei-Chun; Cheng, Chin-Chang; Kuo, Shu-Hung; Wang, Wen-Hwa; Ho, Wan-Jing; Hsu, Tsu‐Shiu; Mutlu, Bülent; Atas, Halil; Öngen, Gül; Un, Zeynep; Okumuş, Gülfer; Hanta, İsmail; Corris, Paul A.; Peacock, Andrew J.; Church, Colin; Toshner, Mark; Newnham, Michael

doi:10.1016/j.rmed.2020.106241

Cited by 15 publications

(9 citation statements)

References 27 publications

(29 reference statements)

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“… Survival of incident patients in historical and contemporary pulmonary arterial hypertension (PAH) registries: 1-, 3- and 5- year survival rates are displayed with registries placed in chronological order. NIH: National Institutes of Health Pulmonary Hypertension Registry [ 3 ]; USA: M c L aughlin et al [ 4 ]; FPHN: French Pulmonary Hypertension Registry [ 26 ]; US-PHC: Pulmonary Hypertension Connection database [ 27 ]; REHAP-Spain: Spanish Registry of PAH [ 28 ]; UK: UK PAH Registry [ 29 ]; REVEAL: Registry to Evaluate Early And Long-term PAH Disease Management [ 30 ]; Swiss: Swiss PAH Registry [ 31 ]; COMPERA: European PAH Registry (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) [ 32 ]; Swedish: Swedish PAH Registry [ 33 ]; EXPERT: Exposure Registry Riociguat in Patients with Pulmonary Hypertension [ 34 ]. # : data from our Canadian PAH cohort of treatment-naïve Group 1 PAH patients.…”

Section: Resultsmentioning

confidence: 99%

Mortality trends in pulmonary arterial hypertension in Canada: a temporal analysis of survival per ESC/ERS guideline era

et al. 2021

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IntroductionThe evolution in pulmonary arterial hypertension (PAH) management has been summarized in three iterations of the European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. No study has assessed whether changes in management, as reflected in the changing guidelines, has translated to improved long-term survival in PAH.MethodsMixed retrospective/prospective analysis of treatment naïve, incident PAH patients (n=392) diagnosed at three major centers in Canada from 2009–2021. Patients were divided into two groups based on their diagnosis date and in accordance with three ESC/ERS guideline iterations: 2009 and 2015. Overall survival was assessed based on date of diagnosis and initial treatment strategy (i.e. mono versus combination).ResultsIn Canada, there was a shift towards more aggressive upfront management with combination therapy after the publication of the 2015 guidelines (10.4% and 30.8% in 2009–2015 patients, and 36.0% and 57.4% in 2016–2021 patients, for baseline and 2-year follow-up respectively). A key factor associated with combination therapy after 2015 was higher pulmonary vascular resistance (p=0.009). The 1, 3 and 5 year survival rates in Canada were 89.2%, 75.6%. and 56.0%, respectively. Despite changes in management, there was no improvement in long-term survival before and after publication of the 2015 ESC/ERS guideline (p=0.53).ConclusionsThere was an increase in the use of initial and sequential combination therapy in Canada after publication of the 2015 ESC/ERS guidelines, which was not associated with improved long-term survival. These data highlight the continued difficulties of managing this aggressive pulmonary disease in an era without a cure.

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Section: Resultsmentioning

confidence: 99%

Mortality trends in pulmonary arterial hypertension in Canada: a temporal analysis of survival per ESC/ERS guideline era

et al. 2021

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“…It can increase the level of cGMP in plasma alone or in combination with NO, causing vasodilation and anti-remodeling [ 71 ]. Riociguat has been shown to improve 6MWD, WHO FC, NT-proBNP, and PVR and to delay TTCW compared to placebo in patients with WHO FC II to III symptoms [ 72 ]. Adverse effects of riocguat include headache, dizziness, hypotension, syncope and indigestion.…”

Section: Specific Medications Of Pahmentioning

confidence: 99%

New progress in diagnosis and treatment of pulmonary arterial hypertension

Zhang

Zhu

Wang

et al. 2022

J Cardiothorac Surg

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Pulmonary arterial hypertension (PAH) is a progressive disease. Although great progress has been made in its diagnosis and treatment in recent years, its mortality rate is still very significant. The pathophysiology and pathogenesis of PAH are complex and involve endothelial dysfunction, chronic inflammation, smooth muscle cell proliferation, pulmonary arteriole occlusion, antiapoptosis and pulmonary vascular remodeling. These factors will accelerate the progression of the disease, leading to poor prognosis. Therefore, accurate etiological diagnosis, treatment and prognosis judgment are particularly important. Here, we systematically review the pathophysiology, diagnosis, genetics, prognosis and treatment of PAH.

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“…Racionale špecifickej liečby tkvie v konkomitantnom postihnutí malých ciev, podobnom ako pri idiopatickej pľúcnej artériovej hypertenzii (PAH). Priaznivé výsledky liečby v zmysle zlepšenia hemodynamiky u neoperabilných pacientov a pacientov s pooperačnou perzistentnou PH sa potvrdili multicentricky, vrátane pacientov z nášho centra v NÚSCH, a. s. (16).…”

Section: Ad 5 Dlhodobé Sledovanie a Vyhľadávanie Neskorých Následkovunclassified

Komentár k Odporúčaniam ESC pre diagnostiku a manažment akútnej pľúcnej embólie v roku 2019 / Comment on 2019 ESC Guidelines on the diagnosis and management of acute pulmonary embolism

Šimková¹,

Gašpar²

2021

Cardiol

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

Cited by 15 publications

References 27 publications

Mortality trends in pulmonary arterial hypertension in Canada: a temporal analysis of survival per ESC/ERS guideline era

Mortality trends in pulmonary arterial hypertension in Canada: a temporal analysis of survival per ESC/ERS guideline era

New progress in diagnosis and treatment of pulmonary arterial hypertension

Komentár k Odporúčaniam ESC pre diagnostiku a manažment akútnej pľúcnej embólie v roku 2019 / Comment on 2019 ESC Guidelines on the diagnosis and management of acute pulmonary embolism

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