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4. Доклад ERS по хронической тромбоэмболической легочной гипертензии

Author

Delcroix, Marion, Torbicki, Adam, Gopalan, Deepa, Sitbon, Olivier, Klok, Frederikus A., Lang, Irene, Jenkins, David, Kim, Nick H., Humbert, Marc, Jais, Xavier, Vonk Noordegraaf, Anton, Pepke-Zaba, Joanna, Brénot, Philippe, Dorfmuller, Peter, Fadel, Elie, Ghofrani, Hossein-Ardeschir, Hoeper, Marius M., Jansa, Pavel, Madani, Michael, Matsubara, Hiromi, Ogo, Takeshi, Grunig, Ekkehard, D'Armini, Andrea, Galie, Nazzareno, Meyer, Bernhard, Corkery, Patrick, Meszaros, Gergely, Mayer, Eckhard, Simonneau, G. rald, Pulmonary medicine, and ACS - Pulmonary hypertension & thrombosis

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, either symptomatic or not. The occlusion of proximal pulmonary arteries by fibrotic intravascular material, in combination with a secondary microvasculopathy of vessels < 500 μm, leads to increased pulmonary vascular resistance and progressive right heart failure. The mechanism responsible for the transformation of red clots into fibrotic material remnants has not yet been elucidated. In patients with pulmonary hypertension, the diagnosis is suspected when a ventilation/perfusion lung scan shows mismatched perfusion defects, and confirmed by right heart catheterisation and vascular imaging. Today, in addition to lifelong anticoagulation, treatment modalities include surgery, angioplasty and medical treatment according to the localisation and characteristics of the lesions. This statement outlines a review of the literature and current practice concerning diagnosis and management of CTEPH. It covers the definitions, diagnosis, epidemiology, follow-up after acute pulmonary embolism, pathophysiology, treatment by pulmonary endarterectomy, balloon pulmonary angioplasty, drugs and their combination, rehabilitation and new lines of research in CTEPH. It represents the first collaboration of the European Respiratory Society, the International CTEPH Association and the European Reference Network-Lung in the pulmonary hypertension domain. The statement summarises current knowledge, but does not make formal recommendations for clinical practice.

Published
2022

5. Changes in cMRI parameters following a switch to riociguat from phosphodiesterase type 5 inhibitors (PDE5i) in patients with pulmonary arterial hypertension: a REPLACE substudy

Author

Rosenkranz, S, primary, Benza, R L, additional, Ghofrani, H A, additional, Gruenig, E, additional, Hoeper, M M, additional, Peacock, A, additional, Simonneau, G, additional, Vizza, D, additional, Meier, C, additional, Vogtlaender, K, additional, and Vonk-Noordegraaf, A, additional

Published
2021
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6. Treatment effect of selexipag on time to disease progression when initiated early in pulmonary arterial hypertension (PAH) patients: GRIPHON and TRITON pooled analysis

Author

Coghlan, G J, primary, Gaine, S, additional, Channick, R N, additional, Chin, K M, additional, Du Roure, C, additional, Gibbs, J S R, additional, Hoeper, M M, additional, Lang, I M, additional, Mathai, S C, additional, McLaughlin, V V, additional, Mitchell, L, additional, Simonneau, G, additional, Sitbon, O, additional, Tapson, V, additional, and Galie, N, additional

Published
2021
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7. Riociguat treatment in patients with pulmonary arterial hypertension: Final safety data from the EXPERT registry

Author

Hoeper, M.M. Gomez Sanchez, M.-A. Humbert, M. Pittrow, D. Simonneau, G. Gall, H. Grünig, E. Klose, H. Halank, M. Langleben, D. Snijder, R.J. Escribano Subias, P. Mielniczuk, L.M. Lange, T.J. Vachiéry, J.-L. Wirtz, H. Helmersen, D.S. Tsangaris, I. Barberà, J.A. Pepke-Zaba, J. Boonstra, A. Rosenkranz, S. Ulrich, S. Steringer-Mascherbauer, R. Delcroix, M. Jansa, P. Šimková, I. Giannakoulas, G. Klotsche, J. Williams, E. Meier, C. Ghofrani, H.-A.

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 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 (usually every 3–6 months) and collated via case report forms. Results: In total, 326 patients with PAH were included in the analysis. The most common AEs in these patients were dizziness (11.7%), right ventricular (RV)/cardiac failure (10.7%), edema/peripheral edema (10.7%), diarrhea (8.6%), dyspnea (8.0%), and cough (7.7%). The most common SAEs were RV/cardiac failure (10.1%), pneumonia (6.1%), dyspnea (4.0%), and syncope (3.4%). The exposure-adjusted rate of hemoptysis/pulmonary hemorrhage was 2.5 events per 100 patient-years. Conclusion: Final data from EXPERT show that in patients with PAH, the safety of riociguat in clinical practice was consistent with clinical trials, with no new safety concerns identified and a lower exposure-adjusted rate of hemoptysis/pulmonary hemorrhage than in the long-term extension of the Phase 3 trial in PAH. © 2020 The Author(s)

Published
2021

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

Author

Ghofrani, H.-A. Gomez Sanchez, M.-A. Humbert, M. Pittrow, D. Simonneau, G. Gall, H. Grünig, E. Klose, H. Halank, M. Langleben, D. Snijder, R.J. Escribano Subias, P. Mielniczuk, L.M. Lange, T.J. Vachiéry, J.-L. Wirtz, H. Helmersen, D.S. Tsangaris, I. Barberá, J.A. Pepke-Zaba, J. Boonstra, A. Rosenkranz, S. Ulrich, S. Steringer-Mascherbauer, R. Delcroix, M. Jansa, P. Šimková, I. Giannakoulas, G. Klotsche, J. Williams, E. Meier, C. Hoeper, M.M. Caneva, J. Tuhay, G. Diez, M. Talavera, M.L. Acosta, A. Vulcano, N. Bosio, M. Maldonado, L. Deleo, S. Melatini, L. Keogh, A. Kotlyar, E. Feenstra, J. Dwyer, N. Adams, H. Stevens, W. Steele, P. Proudman, S. Minson, R. Reeves, G. Lavender, M. Ng, B. Mackenzie, M. Barry, L. Gruenberger, M. Huber, C. Lang, I. Tilea, I. Sadushi-Kolici, R. Löffler-Ragg, J. Feistmantl, L.-T. Evrard, P. Louis, R. Guiot, J. Naldi, M. De Pauw, M. Mehta, S. Camacho, R.C. Tovar, P.P. Londoño, A. Campo, F. Garcia, P. Lema, C. Orozco-Levi, M. Martinez, W. Gomez, J.E. Nielsen-Kudsk, J.E. Mellemkjaer, S. Anton, L. Altraja, A. Vihinen, T. Vasankari, T. Sitbon, O. Cottin, V. Têtu, L. Noël-Savina, E. Shearman, N. Tayler, S. Olzik, I. Kulka, C. Grimminger, J. Simon, M. Nolde, A. Oqueka, T. Harbaum, L. Egenlauf, B. Ewert, R. Schulz, C. Regotta, S. Kramer, T. Knoop-Busch, S. Gerhardt, F. Konstantinides, S. Pitsiou, G. Stanopoulos, I. Sourla, E. Mouratoglou, S. Karvounis, H. Pappas, A. Georgopoulos, D. Fanaridis, M. Mitrouska, I. Michalis, L. Pappas, K. Kotsia, A. Gaine, S. Vizza, C.D. Manzi, G. Poscia, R. Badagliacca, R. Agostoni, P. Bruno, N. Farina, S. D'Alto, M. Argiento, P. Correra, A. Di Marco, G.M. Cresci, C. Vannucchi, V. Torricelli, E. Garcea, A. Pesci, A. Sardella, L. Paciocco, G. Pane, F. D'Armini, A.M. Pin, M. Grazioli, V. Massola, G. Sciortino, A. Prediletto, R. Bauleo, C. Airò, E. Ndreu, R. Pavlickova, I. Lunardi, C. Mulè, M. Farruggio, S. Costa, S. Galgano, G. Petruzzi, M. De Luca, A. Lombardi, F. Roncon, L. Conte, L. Picariello, C. Wirtz, G. Alexandre, M. Vonk-Noordegraaf, A. Boogaard, H. Mager, J. Reesink, H. van den Toorn, L.M. Boomars, K. Andreassen, A.K. Castro, G. Tania, G. Baptista, R. Marinho, A. Shiang, T. Oliveira, A. Coutinho, D. Sousa, J. Loureiro, M.J. Repolho, D. Martins Jesus, S.M. Capinha, M. Agostinho, J. Cardoso, T. Rocha, A. Espinha, M. Ivanov, K.I. Alexeeva, D.E. Batalina, M.V. Hegya, D.V. Zvereva, T.N. Avdeev, S.N. Tsareva, N.A. Galyavich, A.S. Nikolaevich, B.A. Filippov, E.V. Yakovleva, O.E. Pavlova, O.B. Skripkina, E.S. Martynyuk, T.V. Bukatova, I.F. Tregubova, A.V. Platonov, D.Y. Kolomeytseva, T.M. Al Dalaan, A. Abdelsayed, A.A. Weheba, I. Saleemi, S. Sakkijha, H. Bohacekova, M. Valkovicova, T. Farkasova, I. Quezada, C.A. Piccari, L. Blanco, I. Sebastian, L. Roman, A. Lopez, M. Otero, R. Elias, T. Jara, L. Asencio, I. Arjona, J.J. Almagro, R.M. Cárdenas, S.L. García, S.A. Rodríguez, P.V. Lopez, R. Garcia, A. Avilés, F.F. De La Pava, S. Yotti, R. Peñate, G.P. Marrero, F.L. Cifrián Martínez, J.M. Martinez-Meñaca, A. Alonso, L.P. Rozas, S.F. Fernandez, D.I. Cuesta, V.M. Söderberg, S. Bartfay, S.-E. Rundqvist, B. Alfetlawi, M. Wodlin, P. Schwarz, E.I. Speich, R. Lador, F. Rochat, T. Gasche-Soccal, P. Hsu, C.-H. Lin, T.-H. Su, H.-M. Lai, W.-T. Chu, C.Y. Hsu, P.-C. Voon, W.-C. Yen, H.-W. Yih-Jer Wu, J. Wu, S.-H. Huang, W.-P. Fong, M.-C. Huang, C.-L. Kuo, P.-H. Lin, Y.-H. Lin, J.-L. Hung, C.-S. Wu, C.-K. Sung, S.-H. Huang, W.-C. Cheng, C.-C. Kuo, S.-H. Wang, W.-H. Ho, W.-J. Hsu, T.-S. Mutlu, B. Atas, H. Ongen, G. Un, Z. Okumus, G. Hanta, I. Corris, P. Peacock, A. Church, C. Toshner, M. Newnham, M. NEW COLLABORATORS LIST

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

Published
2021

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

Author

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

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

Published
2021
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10. Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry.

Author

Ghofrani, H-A, Gomez Sanchez, M-A, Humbert, M, Pittrow, D, Simonneau, G, Gall, H, Grünig, E, Klose, H, Halank, M, Langleben, D, Snijder, RJ, Escribano Subias, P, Mielniczuk, LM, Lange, TJ, Vachiéry, J-L, Wirtz, H, Helmersen, DS, Tsangaris, I, Barberá, JA, Pepke-Zaba, J, Boonstra, A, Rosenkranz, S, Ulrich, S, Steringer-Mascherbauer, R, Delcroix, M, Jansa, P, Šimková, I, Giannakoulas, G, Klotsche, J, Williams, E, Meier, C, Hoeper, MM, NEW COLLABORATORS LIST, Ghofrani, H-A, Gomez Sanchez, M-A, Humbert, M, Pittrow, D, Simonneau, G, Gall, H, Grünig, E, Klose, H, Halank, M, Langleben, D, Snijder, RJ, Escribano Subias, P, Mielniczuk, LM, Lange, TJ, Vachiéry, J-L, Wirtz, H, Helmersen, DS, Tsangaris, I, Barberá, JA, Pepke-Zaba, J, Boonstra, A, Rosenkranz, S, Ulrich, S, Steringer-Mascherbauer, R, Delcroix, M, Jansa, P, Šimková, I, Giannakoulas, G, Klotsche, J, Williams, E, Meier, C, Hoeper, MM, and NEW COLLABORATORS LIST

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.

Published
2021

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

Author

Ghofrani, HA, Gomez Sanchez, MA, Humbert, M, Pittrow, D, Simonneau, G, Gall, H, Grünig, E, Klose, H, Halank, M, Langleben, D, Snijder, RJ, Escribano Subias, P, Mielniczuk, LM, Lange, TJ, Vachiéry, JL, Wirtz, H, Helmersen, DS, Tsangaris, I, Barberá, JA, Pepke-Zaba, J, Boonstra, Andre, Rosenkranz, S, Ulrich, S, Steringer-Mascherbauer, R, Delcroix, M, Jansa, P, Šimková, I, Giannakoulas, G, Klotsche, J, Williams, E, Meier, C, Hoeper, MM, Caneva, J, Tuhay, G, Diez, M, Talavera, ML, Acosta, A, Vulcano, N, Bosio, M, Maldonado, L, Deleo, S, Melatini, L, Keogh, A, Kotlyar, E, Feenstra, J, Dwyer, N, Adams, H, Stevens, W, Steele, P, Proudman, S, Minson, R, Reeves, G, Lavender, M, Ng, B, MacKenzie, M, Barry, L, Gruenberger, M, Huber, C, Lang, I, Tilea, I, Sadushi-Kolici, R, Löffler-Ragg, J, Feistmantl, LT, Evrard, P, Louis, R, Guiot, J, Naldi, M, Pauw, M, Mehta, S, Camacho, RC, Tovar, PP, Londoño, A, Campo, F, Garcia, P, Lema, C, Orozco-Levi, M, Martinez, W, Gomez, JE, Nielsen-Kudsk, JE, Mellemkjaer, S, Anton, L, Altraja, A, Vihinen, T, Vasankari, T, Sitbon, O, Cottin, V, Têtu, L, Noël-Savina, E, Shearman, N, Tayler, S, Olzik, I, Kulka, C, Grimminger, J, Simon, M, Nolde, A, Oqueka, T, Harbaum, L, Egenlauf, B, Ewert, R, Schulz, C, Regotta, S, Kramer, T, Knoop-Busch, S, Gerhardt, F, Konstantinides, S, Pitsiou, G, Stanopoulos, I, Sourla, E, Mouratoglou, S, Karvounis, H, Pappas, A, Georgopoulos, D, Fanaridis, M, Mitrouska, I, Michalis, L, Pappas, K, Kotsia, A, Gaine, S, Vizza, CD, Manzi, G, Poscia, R, Badagliacca, R, Agostoni, P, Bruno, N, Farina, S, D'Alto, M, Argiento, P, Correra, A, Di Marco, GM, Cresci, C, Vannucchi, V, Torricelli, E, Garcea, A, Pesci, A, Sardella, L, Paciocco, G, Pane, F, D'Armini, AM, Pin, M, Grazioli, V, Massola, G, Sciortino, A, Prediletto, R, Bauleo, C, Airò, E, Ndreu, R, Pavlickova, I, Lunardi, C, Mulè, M, Farruggio, S, Costa, S, Galgano, G, Petruzzi, M, Luca, A, Lombardi, F, Roncon, L, Conte, L, Picariello, C, Wirtz, G, Alexandre, M, Vonk-Noordegraaf, A, van den Boogaard, H, Mager, J, Reesink, H, van den Toorn, Leon, Boomars, Karin, Andreassen, AK, Castro, G, Tania, G, Baptista, R, Marinho, A, Shiang, T, Oliveira, A, Coutinho, D, Sousa, J, Loureiro, MJ, Repolho, D, Martins Jesus, SM, Capinha, M, Agostinho, J, Cardoso, T, Rocha, A, Espinha, M, Ivanov, KI, Alexeeva, DE, Batalina, MV, Hegya, DV, Zvereva, TN, Avdeev, SN, Tsareva, NA, Galyavich, AS, Nikolaevich, BA, Filippov, EV, Yakovleva, OE, Pavlova, OB, Skripkina, ES, Martynyuk, TV, Bukatova, IF, Tregubova, AV, Platonov, DY, Kolomeytseva, TM, Al Dalaan, A, Abdelsayed, AA, Weheba, I, Saleemi, S, Sakkijha, H, Bohacekova, M, Valkovicova, T, Farkasova, I, Quezada, CA, Piccari, L, Blanco, I, Sebastian, L, Roman, A, Lopez, M, Otero, R, Elias, T, Jara, L, Asencio, I, Arjona, JJ, Almagro, RM, Cárdenas, SL, García, SA, Rodríguez, PV, Lopez, R, Garcia, A, Avilés, FF, De La Pava, S, Yotti, R, Peñate, GP, Marrero, FL, Cifrián Martínez, JM, Martinez-Meñaca, A, Alonso, LP, Rozas, SF, Fernandez, DI, Cuesta, VM, Söderberg, S, Bartfay, SE, Rundqvist, B, Alfetlawi, M, Wodlin, P, Schwarz, EI, Speich, R, Lador, F, Rochat, T, Gasche-Soccal, P, Hsu, CH, Lin, TH, Su, HM, Lai, WT, Chu, CY, Hsu, PC, Voon, WC, Yen, HW, Yih-Jer Wu, J, Wu, SH, Huang, WP, Fong, MC, Huang, CL, Kuo, PH, Lin, YH, Lin, JL, Hung, CS, Wu, CK, Sung, SH, Huang, WC, Cheng, CC, Kuo, SH, Wang, WH, Ho, WJ, Hsu, TS, Mutlu, B, Atas, H, Ongen, G, Un, Z, Okumus, G, Hanta, I, Corris, P, Peacock, A, Church, C, Toshner, M, Ghofrani, HA, Gomez Sanchez, MA, Humbert, M, Pittrow, D, Simonneau, G, Gall, H, Grünig, E, Klose, H, Halank, M, Langleben, D, Snijder, RJ, Escribano Subias, P, Mielniczuk, LM, Lange, TJ, Vachiéry, JL, Wirtz, H, Helmersen, DS, Tsangaris, I, Barberá, JA, Pepke-Zaba, J, Boonstra, Andre, Rosenkranz, S, Ulrich, S, Steringer-Mascherbauer, R, Delcroix, M, Jansa, P, Šimková, I, Giannakoulas, G, Klotsche, J, Williams, E, Meier, C, Hoeper, MM, Caneva, J, Tuhay, G, Diez, M, Talavera, ML, Acosta, A, Vulcano, N, Bosio, M, Maldonado, L, Deleo, S, Melatini, L, Keogh, A, Kotlyar, E, Feenstra, J, Dwyer, N, Adams, H, Stevens, W, Steele, P, Proudman, S, Minson, R, Reeves, G, Lavender, M, Ng, B, MacKenzie, M, Barry, L, Gruenberger, M, Huber, C, Lang, I, Tilea, I, Sadushi-Kolici, R, Löffler-Ragg, J, Feistmantl, LT, Evrard, P, Louis, R, Guiot, J, Naldi, M, Pauw, M, Mehta, S, Camacho, RC, Tovar, PP, Londoño, A, Campo, F, Garcia, P, Lema, C, Orozco-Levi, M, Martinez, W, Gomez, JE, Nielsen-Kudsk, JE, Mellemkjaer, S, Anton, L, Altraja, A, Vihinen, T, Vasankari, T, Sitbon, O, Cottin, V, Têtu, L, Noël-Savina, E, Shearman, N, Tayler, S, Olzik, I, Kulka, C, Grimminger, J, Simon, M, Nolde, A, Oqueka, T, Harbaum, L, Egenlauf, B, Ewert, R, Schulz, C, Regotta, S, Kramer, T, Knoop-Busch, S, Gerhardt, F, Konstantinides, S, Pitsiou, G, Stanopoulos, I, Sourla, E, Mouratoglou, S, Karvounis, H, Pappas, A, Georgopoulos, D, Fanaridis, M, Mitrouska, I, Michalis, L, Pappas, K, Kotsia, A, Gaine, S, Vizza, CD, Manzi, G, Poscia, R, Badagliacca, R, Agostoni, P, Bruno, N, Farina, S, D'Alto, M, Argiento, P, Correra, A, Di Marco, GM, Cresci, C, Vannucchi, V, Torricelli, E, Garcea, A, Pesci, A, Sardella, L, Paciocco, G, Pane, F, D'Armini, AM, Pin, M, Grazioli, V, Massola, G, Sciortino, A, Prediletto, R, Bauleo, C, Airò, E, Ndreu, R, Pavlickova, I, Lunardi, C, Mulè, M, Farruggio, S, Costa, S, Galgano, G, Petruzzi, M, Luca, A, Lombardi, F, Roncon, L, Conte, L, Picariello, C, Wirtz, G, Alexandre, M, Vonk-Noordegraaf, A, van den Boogaard, H, Mager, J, Reesink, H, van den Toorn, Leon, Boomars, Karin, Andreassen, AK, Castro, G, Tania, G, Baptista, R, Marinho, A, Shiang, T, Oliveira, A, Coutinho, D, Sousa, J, Loureiro, MJ, Repolho, D, Martins Jesus, SM, Capinha, M, Agostinho, J, Cardoso, T, Rocha, A, Espinha, M, Ivanov, KI, Alexeeva, DE, Batalina, MV, Hegya, DV, Zvereva, TN, Avdeev, SN, Tsareva, NA, Galyavich, AS, Nikolaevich, BA, Filippov, EV, Yakovleva, OE, Pavlova, OB, Skripkina, ES, Martynyuk, TV, Bukatova, IF, Tregubova, AV, Platonov, DY, Kolomeytseva, TM, Al Dalaan, A, Abdelsayed, AA, Weheba, I, Saleemi, S, Sakkijha, H, Bohacekova, M, Valkovicova, T, Farkasova, I, Quezada, CA, Piccari, L, Blanco, I, Sebastian, L, Roman, A, Lopez, M, Otero, R, Elias, T, Jara, L, Asencio, I, Arjona, JJ, Almagro, RM, Cárdenas, SL, García, SA, Rodríguez, PV, Lopez, R, Garcia, A, Avilés, FF, De La Pava, S, Yotti, R, Peñate, GP, Marrero, FL, Cifrián Martínez, JM, Martinez-Meñaca, A, Alonso, LP, Rozas, SF, Fernandez, DI, Cuesta, VM, Söderberg, S, Bartfay, SE, Rundqvist, B, Alfetlawi, M, Wodlin, P, Schwarz, EI, Speich, R, Lador, F, Rochat, T, Gasche-Soccal, P, Hsu, CH, Lin, TH, Su, HM, Lai, WT, Chu, CY, Hsu, PC, Voon, WC, Yen, HW, Yih-Jer Wu, J, Wu, SH, Huang, WP, Fong, MC, Huang, CL, Kuo, PH, Lin, YH, Lin, JL, Hung, CS, Wu, CK, Sung, SH, Huang, WC, Cheng, CC, Kuo, SH, Wang, WH, Ho, WJ, Hsu, TS, Mutlu, B, Atas, H, Ongen, G, Un, Z, Okumus, G, Hanta, I, Corris, P, Peacock, A, Church, C, and Toshner, M

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.

Published
2021

13. Efficacy and safety of selexipag in pulmonary arterial hypertension (PAH) patients with and without significant cardiovascular (CV) comorbidities

Author

Rosenkranz, S., Channick, R., Chin, K., Jenner, B., Gaine, S., Galie, N., Ghofrani, H. A., Hoeper, M. M., McLaughlin, V, Preiss, R., Rubin, L., Simonneau, G., Sitbon, O., Tapson, V, Lang, I, Rosenkranz, S., Channick, R., Chin, K., Jenner, B., Gaine, S., Galie, N., Ghofrani, H. A., Hoeper, M. M., McLaughlin, V, Preiss, R., Rubin, L., Simonneau, G., Sitbon, O., Tapson, V, and Lang, I

Published
2020

19. Long-term outcomes in newly diagnosed pulmonary arterial hypertension (PAH) patients receiving initial triple oral combination therapy: insights from the randomised controlled TRITON study

Author

Galie, N, primary, Sitbon, O, additional, Doelberg, M, additional, Gibbs, J.S.R, additional, Hoeper, M.M, additional, Martin, N, additional, Mathai, S.C, additional, McLaughlin, V, additional, Perchenet, L, additional, Simonneau, G, additional, and Chin, K, additional

Published
2020
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20. Plasma proteome analysis in patients with pulmonary arterial hypertension: an observational cohort study

Author

Rhodes, C.J., Wharton, J., Ghataorhe, P., Watson, G., Girerd, B., Howard, L.S., Gibbs, J.S.R., Condliffe, R., Elliot, C.A., Kiely, D.G., Simonneau, G., Montani, D., Sitbon, O., Gall, H., Schermuly, R.T., Ghofrani, H.A., Lawrie, A., Humbert, M., Wilkins, M.R., Imperial College Healthcare NHS Trust, National Institute for Health Research, British Heart Foundation, Wellcome Trust, and Medical Research Council (MRC)

Subjects
EXPRESSION, Adult, Male, Proteomics, Proteome, Hypertension, Pulmonary, Respiratory System, BIOMARKERS, DIAGNOSIS, Risk Assessment, 1117 Public Health and Health Services, Cohort Studies, PREDICTING SURVIVAL, Critical Care Medicine, Risk Factors, General & Internal Medicine, Humans, Arterial Pressure, Familial Primary Pulmonary Hypertension, Aged, Science & Technology, IDENTIFICATION, RECEPTOR, Gene Expression Profiling, 1103 Clinical Sciences, Blood Proteins, Articles, Middle Aged, ST2, Hypertension, Female, Life Sciences & Biomedicine, 1199 Other Medical and Health Sciences
Abstract

Summary Background Idiopathic and heritable pulmonary arterial hypertension form a rare but molecularly heterogeneous disease group. We aimed to measure and validate differences in plasma concentrations of proteins that are associated with survival in patients with idiopathic or heritable pulmonary arterial hypertension to improve risk stratification. Methods In this observational cohort study, we enrolled patients with idiopathic or heritable pulmonary arterial hypertension from London (UK; cohorts 1 and 2), Giessen (Germany; cohort 3), and Paris (France; cohort 4). Blood samples were collected at routine clinical appointment visits, clinical data were collected within 30 days of blood sampling, and biochemical data were collected within 7 days of blood sampling. We used an aptamer-based assay of 1129 plasma proteins, and patient clinical details were concealed to the technicians. We identified a panel of prognostic proteins, confirmed with alternative targeted assays, which we evaluated against the established prognostic risk equation for pulmonary arterial hypertension derived from the REVEAL registry. All-cause mortality was the primary endpoint. Findings 20 proteins differentiated survivors and non-survivors in 143 consecutive patients with idiopathic or heritable pulmonary arterial hypertension with 2 years' follow-up (cohort 1) and in a further 75 patients with 2·5 years' follow-up (cohort 2). Nine proteins were both prognostic independent of plasma NT-proBNP concentrations and confirmed by targeted assays. The functions of these proteins relate to myocardial stress, inflammation, pulmonary vascular cellular dysfunction and structural dysregulation, iron status, and coagulation. A cutoff-based score using the panel of nine proteins provided prognostic information independent of the REVEAL equation, improving the C statistic from area under the curve 0·83 (for REVEAL risk score, 95% CI 0·77–0·89; p

Published
2017

27. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Author

Galie, N, Humbert, M, Vachiery, J, Gibbs, S, Lang, I, Torbicki, A, Simonneau, G, Peacock, A, Vonk Noordegraaf, A, Beghetti, M, Ghofrani, A, Gomez Sanchez, M, Hansmann, G, Klepetko, W, Lancellotti, P, Matucci, M, Mcdonagh, T, Pierard, L, Trindade, P, Zompatori, M, Hoeper, M, Aboyans, V, Vaz Carneiro, A, Achenbach, S, Agewall, S, Allanore, Y, Asteggiano, R, Badano, L, Albert Barbera, J, Bouvaist, H, Bueno, H, Byrne, R, Carerj, S, Castro, G, Erol, C, Falk, V, Funck-Brentano, C, Gorenflo, M, Granton, J, Iung, B, Kiely, D, Kirchhof, P, Kjellstrom, B, Landmesser, U, Lekakis, J, Lionis, C, Lip, G, Orfanos, S, Park, M, Piepoli, M, Ponikowski, P, Revel, M, Rigau, D, Rosenkranz, S, Voller, H, Luis Zamorano, J, Myftiu, S, Bonderman, D, Firdovsi, I, Lazareva, I, De Pauw, M, Sokolovic, S, Velchev, V, Cikes, M, Moutiris, J, Jansa, P, Nielsen-Kudsk, J, Anton, L, Jaaskelainen, P, Bauer, F, Chukhrukidze, A, Opitz, C, Giannakoulas, G, Karlocai, K, Oddsson, O, Gaine, S, Menachemi, D, Emdin, M, Sooronbaev, T, Rudzitis, A, Gumbiene, L, Lebrun, F, Micallef, J, Botnaru, V, Oukerraj, L, Andreassen, A, Kurzyna, M, Leite Baptista, M, Coman, I, Moiseeva, O, Stefanovic, B, Simkova, I, Wikstrom, G, Schwerzmann, M, Srbinovska-Kostovska, E, van Dijk, A, Mahdhaoui, A, Kaymaz, C, Coghlan, G, Sirenko, Y, Galie N., Humbert M., Vachiery J. -L., Gibbs S., Lang I., Torbicki A., Simonneau G., Peacock A., Vonk Noordegraaf A., Beghetti M., Ghofrani A., Gomez Sanchez M. A., Hansmann G., Klepetko W., Lancellotti P., Matucci M., McDonagh T., Pierard L. A., Trindade P. T., Zompatori M., Hoeper M., Aboyans V., Vaz Carneiro A., Achenbach S., Agewall S., Allanore Y., Asteggiano R., Badano L., Albert Barbera J., Bouvaist H., Bueno H., Byrne R. A., Carerj S., Castro G., Erol C., Falk V., Funck-Brentano C., Gorenflo M., Granton J., Iung B., Kiely D. G., Kirchhof P., Kjellstrom B., Landmesser U., Lekakis J., Lionis C., Lip G. Y. H., Orfanos S. E., Park M. H., Piepoli M. F., Ponikowski P., Revel M. -P., Rigau D., Rosenkranz S., Voller H., Luis Zamorano J., Myftiu S., Bonderman D., Firdovsi I., Lazareva I., De Pauw M., Sokolovic S., Velchev V., Cikes M., Moutiris J. A., Jansa P., Nielsen-Kudsk J. E., Anton L., Jaaskelainen P., Bauer F., Chukhrukidze A., Opitz C., Giannakoulas G., Karlocai K., Oddsson O., Gaine S., Menachemi D., Emdin M., Sooronbaev T., Rudzitis A., Gumbiene L., Lebrun F., Micallef J., Botnaru V., Oukerraj L., Andreassen A. K., Kurzyna M., Leite Baptista M. J. R., Coman I. M., Moiseeva O., Stefanovic B. S., Simkova I., Wikstrom G., Schwerzmann M., Srbinovska-Kostovska E., van Dijk A. P. J., Mahdhaoui A., Kaymaz C., Coghlan G., Sirenko Y., Galie, N, Humbert, M, Vachiery, J, Gibbs, S, Lang, I, Torbicki, A, Simonneau, G, Peacock, A, Vonk Noordegraaf, A, Beghetti, M, Ghofrani, A, Gomez Sanchez, M, Hansmann, G, Klepetko, W, Lancellotti, P, Matucci, M, Mcdonagh, T, Pierard, L, Trindade, P, Zompatori, M, Hoeper, M, Aboyans, V, Vaz Carneiro, A, Achenbach, S, Agewall, S, Allanore, Y, Asteggiano, R, Badano, L, Albert Barbera, J, Bouvaist, H, Bueno, H, Byrne, R, Carerj, S, Castro, G, Erol, C, Falk, V, Funck-Brentano, C, Gorenflo, M, Granton, J, Iung, B, Kiely, D, Kirchhof, P, Kjellstrom, B, Landmesser, U, Lekakis, J, Lionis, C, Lip, G, Orfanos, S, Park, M, Piepoli, M, Ponikowski, P, Revel, M, Rigau, D, Rosenkranz, S, Voller, H, Luis Zamorano, J, Myftiu, S, Bonderman, D, Firdovsi, I, Lazareva, I, De Pauw, M, Sokolovic, S, Velchev, V, Cikes, M, Moutiris, J, Jansa, P, Nielsen-Kudsk, J, Anton, L, Jaaskelainen, P, Bauer, F, Chukhrukidze, A, Opitz, C, Giannakoulas, G, Karlocai, K, Oddsson, O, Gaine, S, Menachemi, D, Emdin, M, Sooronbaev, T, Rudzitis, A, Gumbiene, L, Lebrun, F, Micallef, J, Botnaru, V, Oukerraj, L, Andreassen, A, Kurzyna, M, Leite Baptista, M, Coman, I, Moiseeva, O, Stefanovic, B, Simkova, I, Wikstrom, G, Schwerzmann, M, Srbinovska-Kostovska, E, van Dijk, A, Mahdhaoui, A, Kaymaz, C, Coghlan, G, Sirenko, Y, Galie N., Humbert M., Vachiery J. -L., Gibbs S., Lang I., Torbicki A., Simonneau G., Peacock A., Vonk Noordegraaf A., Beghetti M., Ghofrani A., Gomez Sanchez M. A., Hansmann G., Klepetko W., Lancellotti P., Matucci M., McDonagh T., Pierard L. A., Trindade P. T., Zompatori M., Hoeper M., Aboyans V., Vaz Carneiro A., Achenbach S., Agewall S., Allanore Y., Asteggiano R., Badano L., Albert Barbera J., Bouvaist H., Bueno H., Byrne R. A., Carerj S., Castro G., Erol C., Falk V., Funck-Brentano C., Gorenflo M., Granton J., Iung B., Kiely D. G., Kirchhof P., Kjellstrom B., Landmesser U., Lekakis J., Lionis C., Lip G. Y. H., Orfanos S. E., Park M. H., Piepoli M. F., Ponikowski P., Revel M. -P., Rigau D., Rosenkranz S., Voller H., Luis Zamorano J., Myftiu S., Bonderman D., Firdovsi I., Lazareva I., De Pauw M., Sokolovic S., Velchev V., Cikes M., Moutiris J. A., Jansa P., Nielsen-Kudsk J. E., Anton L., Jaaskelainen P., Bauer F., Chukhrukidze A., Opitz C., Giannakoulas G., Karlocai K., Oddsson O., Gaine S., Menachemi D., Emdin M., Sooronbaev T., Rudzitis A., Gumbiene L., Lebrun F., Micallef J., Botnaru V., Oukerraj L., Andreassen A. K., Kurzyna M., Leite Baptista M. J. R., Coman I. M., Moiseeva O., Stefanovic B. S., Simkova I., Wikstrom G., Schwerzmann M., Srbinovska-Kostovska E., van Dijk A. P. J., Mahdhaoui A., Kaymaz C., Coghlan G., and Sirenko Y.

Abstract

Document Reviewers: Victor Aboyans (CPG Review Coordinator) (France), Antonio Vaz Carneiro (CPG Review Coordinator) (Portugal), Stephan Achenbach (Germany), Stefan Agewall (Norway), Yannick Allanore (France), Riccardo Asteggiano (Italy), Luigi Paolo Badano (Italy), Joan Albert Barbera (Spain), Helene Bouvaist (France), Hector Bueno (Spain), Robert A. Byrne (Germany), Scipione Carerj (Italy), Graca Castro (Portugal), Cetin Erol (Turkey), Volkmar Falk (Germany), Christian Funck-Brentano (France), Matthias Gorenflo (Germany), John Granton (Canada), Bernard Iung (France), David G. Kiely (UK), Paulus Kirchhof (Germany/UK), Barbro Kjellstrom (Sweden), Ulf Landmesser (Switzerland), John Lekakis (Greece), Christos Lionis (Greece), Gregory Y. H. Lip (UK), Stylianos E. Orfanos (Greece), Myung H. Park (USA), Massimo F. Piepoli (Italy), Piotr Ponikowski (Poland), Marie-Pierre Revel (France), David Rigau (ERS methodologist) (Switzerland), Stephan Rosenkranz (Germany), Heinz Voller (Germany), and Jose Luis Zamorano (Spain)

Published
2016

28. ESC/ERS 2015 guidelines for the diagnosis and treatment of pulmonary hypertension

Author

Galie, N, Humbert, M, Vachiery, J, Gibbs, S, Lang, I, Torbicki, A, Simonneau, G, Peacock, A, Noordegraaf, A, Beghetti, M, Ghofrani, A, Sanchez, M, Hansmann, G, Klepetko, W, Lancellotti, P, Matucci, M, Mcdonagh, T, Pierard, L, Trindade, P, Zompatori, M, Hoeper, M, Aboyans, V, Carneiro, A, Achenbach, S, Agewall, S, Allanore, Y, Asteggiano, R, Badano, L, Barbera, J, Bouvaist, H, Bueno, H, Byrne, R, Carerj, S, Castro, G, Erol, C, Falk, V, Funck-Brentano, C, Gorenflo, M, Grantonc, J, Iung, B, Kiely, D, Kirchhof, P, Kjellstrom, B, Landmesser, U, Lekakis, J, Lionis, C, Lip, G, Orfanos, S, Park, M, Piepoli, M, Ponikowski, P, Revel, M, Rigau, D, Rosenkranz, S, Voller, H, Zamorano, J, Galie N., Humbert M., Vachiery J. -L., Gibbs S., Lang I., Torbicki A., Simonneau G., Peacock A., Noordegraaf A. V., Beghetti M., Ghofrani A., Sanchez M. A. G., Hansmann G., Klepetko W., Lancellotti P., Matucci M., McDonagh T., Pierard L. A., Trindade P. T., Zompatori M., Hoeper M., Aboyans V., Carneiro A. V., Achenbach S., Agewall S., Allanore Y., Asteggiano R., Badano L., Barbera J. A., Bouvaist H., Bueno H., Byrne R. A., Carerj S., Castro G., Erol C., Falk V., Funck-Brentano C., Gorenflo M., Grantonc J., Iung B., Kiely D. G., Kirchhof P., Kjellstrom B., Landmesser U., Lekakis J., Lionis C., Lip G. Y. H., Orfanos S. E., Park M. H., Piepoli M. F., Ponikowski P., Revel M. -P., Rigau D., Rosenkranz S., Voller H., Zamorano J. L., Galie, N, Humbert, M, Vachiery, J, Gibbs, S, Lang, I, Torbicki, A, Simonneau, G, Peacock, A, Noordegraaf, A, Beghetti, M, Ghofrani, A, Sanchez, M, Hansmann, G, Klepetko, W, Lancellotti, P, Matucci, M, Mcdonagh, T, Pierard, L, Trindade, P, Zompatori, M, Hoeper, M, Aboyans, V, Carneiro, A, Achenbach, S, Agewall, S, Allanore, Y, Asteggiano, R, Badano, L, Barbera, J, Bouvaist, H, Bueno, H, Byrne, R, Carerj, S, Castro, G, Erol, C, Falk, V, Funck-Brentano, C, Gorenflo, M, Grantonc, J, Iung, B, Kiely, D, Kirchhof, P, Kjellstrom, B, Landmesser, U, Lekakis, J, Lionis, C, Lip, G, Orfanos, S, Park, M, Piepoli, M, Ponikowski, P, Revel, M, Rigau, D, Rosenkranz, S, Voller, H, Zamorano, J, Galie N., Humbert M., Vachiery J. -L., Gibbs S., Lang I., Torbicki A., Simonneau G., Peacock A., Noordegraaf A. V., Beghetti M., Ghofrani A., Sanchez M. A. G., Hansmann G., Klepetko W., Lancellotti P., Matucci M., McDonagh T., Pierard L. A., Trindade P. T., Zompatori M., Hoeper M., Aboyans V., Carneiro A. V., Achenbach S., Agewall S., Allanore Y., Asteggiano R., Badano L., Barbera J. A., Bouvaist H., Bueno H., Byrne R. A., Carerj S., Castro G., Erol C., Falk V., Funck-Brentano C., Gorenflo M., Grantonc J., Iung B., Kiely D. G., Kirchhof P., Kjellstrom B., Landmesser U., Lekakis J., Lionis C., Lip G. Y. H., Orfanos S. E., Park M. H., Piepoli M. F., Ponikowski P., Revel M. -P., Rigau D., Rosenkranz S., Voller H., and Zamorano J. L.

Published
2016

29. P2540Sex-specific differences in the clinical presentation, surgical complications, and course of chronic thromboembolic pulmonary hypertension

Author

Barco, S, primary, Klok, F A, additional, Konstantinides, S V, additional, Dartevelle, P, additional, Fadel, E, additional, Jenkins, D, additional, Kim, N H, additional, Madani, M, additional, Matsubara, M, additional, Mayer, E, additional, Pepke-Zaba, J, additional, Simonneau, G, additional, Delcroix, M, additional, and Lang, I M, additional

Published
2019
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30. 4973Efficacy and safety of selexipag in pulmonary arterial hypertension (PAH) patients with and without significant cardiovascular (CV) comorbidities

Author

Rosenkranz, S, primary, Channick, R, additional, Chin, K, additional, Jenner, B, additional, Gaine, S, additional, Galie, N, additional, Ghofrani, H A, additional, Hoeper, M M, additional, McLaughlin, V V, additional, Preiss, R, additional, Rubin, L J, additional, Simonneau, G, additional, Sitbon, O, additional, Tapson, V, additional, and Lang, I M, additional

Published
2019
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31. Riociguat for the Treatment of Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: Safety Update from the EXPERT Registry

Author

Klose, H., Gall, H., Ghofrani, H., Gruenig, E., Humbert, M., Simonneau, G., Halank, M., Langleben, D., Snijder, R. J., Escribano, P., Mielniczuk, L. M., Lange, T., Vachiery, J., Wirtz, H., Helmersen, D. S., Tsangaris, I., Barbera, J. A., Pepke-Zaba, J., Boonstra, A., Rosenkranz, S., Ulrich, S., Mascherbauer, R., Delcroix, M., Jansa, P., Gomez Sanchez, M. A., Klotsche, J., Meier, C., Pittrow, D., Hoeper, M. M., Klose, H., Gall, H., Ghofrani, H., Gruenig, E., Humbert, M., Simonneau, G., Halank, M., Langleben, D., Snijder, R. J., Escribano, P., Mielniczuk, L. M., Lange, T., Vachiery, J., Wirtz, H., Helmersen, D. S., Tsangaris, I., Barbera, J. A., Pepke-Zaba, J., Boonstra, A., Rosenkranz, S., Ulrich, S., Mascherbauer, R., Delcroix, M., Jansa, P., Gomez Sanchez, M. A., Klotsche, J., Meier, C., Pittrow, D., and Hoeper, M. M.

Published
2018

33. S.4.1 N-terminal pro-brain natriuretic peptide levels predict incident pulmonary arterial hypertension in SSc

Author

Thakkar, V., Stevens, W., Prior, D., Byron, J., Patterson, K., Hissaria, P., Moore, O., Roddy, J., Zochling, J., Sahhar, J., Nash, P., Tymms, K., Youssef, P., Proudman, S., Nikpour, M., Launay, D., Sitbon, O., Cordier, J. F., Hachulla, E., Mouthon, L., Gressin, V., Rottat, L., Clerson, P., Simonneau, G., Humbert, M., Carreira, P., Carmona, L., Joven, B. E., Denton, C. P., Allanore, Y., Walker, U. A., Matucci-Cerinic, M., Muller-Ladner, U., Hsu, V., Cheng, Q., and Steen, V.

Abstract

Introduction. Pulmonary arterial hypertension (PAH) is a major cause of mortality in SSc. NT-proBNP may be a useful biomarker of prevalent PAH but its role in screening for incident PAH has not been evaluated. Methods. Patients recruited into the Australian Scleroderma Cohort Study undergo annual echocardiography, pulmonary function tests (PFTs), 6-min walk test (6MWT) and have serum NT-proBNP measured (ElecsysproBNP II). The diagnosis of PAH is based on Dana point criteria at right heart catheterization (RHC). Patients with LV dysfunction or eGFR 36 mmHg, (ii) FVC/DLCO% >1.6 and no significant ILD, (iii) DLCO 189.2 pg/ml had a likelihood ratio of 26.4 for presence of PAH (c-statistic = 0.9; sensitivity 85%; specificity 97%). An NT-proBNP level 189.2 pg/ml and

Published
2017

34. P2614Factors associated with survival in patients with not-operated chronic thromboembolic pulmonary hypertension (CTEPH) in the modern management era

Author

Taniguchi, Y, primary, Jais, X, additional, Boucly, A, additional, Weatherald, J, additional, Jevnikar, M, additional, Brenot, P, additional, Planche, O, additional, Parent, F, additional, Savale, L, additional, Montani, D, additional, Fadel, E, additional, Humbert, M, additional, Simonneau, G, additional, and Sitbon, O, additional

Published
2018
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35. Rationale And Design Of The Replace Trial: Riociguat Replacing Phosphodiesterase 5 Inhibitor (pde5i) Therapy Evaluated Against Continued Pde5i Therapy In Patients With Pulmonary Arterial Hypertension (pah)

Author

Hoeper, M. M., Ghofrani, H. -A., Benza, R. L., Corris, P. A., Gibbs, S., Klinger, J. R., Langleben, D., McLaughlin, V. V., Peacock, A., Rosenkranz, S., Vonk-Noordegraaf, A., White, J., Kleinjung, F., Meier, C., Simonneau, G., Hoeper, M. M., Ghofrani, H. -A., Benza, R. L., Corris, P. A., Gibbs, S., Klinger, J. R., Langleben, D., McLaughlin, V. V., Peacock, A., Rosenkranz, S., Vonk-Noordegraaf, A., White, J., Kleinjung, F., Meier, C., and Simonneau, G.

Published
2017

36. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT)

Author

Authors/Task Force Members, Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez Sanchez MA, Jondeau G, Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger M, Simonneau G, ESC Committee for Practice Guidelines, Vahanian A, Auricchio A, Bax J, Ceconi C, Dean V, Filippatos G, Funck Brentano C, Hobbs R, Kearney P, McDonagh T, McGregor K, Popescu BA, Reiner Z, Sechtem U, Sirnes PA, Tendera M, Vardas P, Widimsky P, Document Reviewers, Al Attar N, Andreotti F, Aschermann M, Asteggiano R, Benza R, Berger R, Bonnet D, Delcroix M, Howard L, Kitsiou AN, Lang I, Maggioni A, Nielsen Kudsk JE, Park M, Price S, Domenech MT, Vonk Noordegraaf A, Zamorano J.L., PERRONE FILARDI, PASQUALE, Authors/Task Force, Member, Galiè, N, Hoeper, Mm, Humbert, M, Torbicki, A, Vachiery, Jl, Barbera, Ja, Beghetti, M, Corris, P, Gaine, S, Gibbs, J, Gomez Sanchez, Ma, Jondeau, G, Klepetko, W, Opitz, C, Peacock, A, Rubin, L, Zellweger, M, Simonneau, G, ESC Committee for Practice, Guideline, Vahanian, A, Auricchio, A, Bax, J, Ceconi, C, Dean, V, Filippatos, G, Funck Brentano, C, Hobbs, R, Kearney, P, Mcdonagh, T, Mcgregor, K, Popescu, Ba, Reiner, Z, Sechtem, U, Sirnes, Pa, Tendera, M, Vardas, P, Widimsky, P, Document, Reviewer, Al Attar, N, Andreotti, F, Aschermann, M, Asteggiano, R, Benza, R, Berger, R, Bonnet, D, Delcroix, M, Howard, L, Kitsiou, An, Lang, I, Maggioni, A, Nielsen Kudsk, Je, Park, M, PERRONE FILARDI, Pasquale, Price, S, Domenech, Mt, Vonk Noordegraaf, A, Zamorano, J. L., Cardiovascular Centre (CVC), Galie N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez MA, Jondeau G, Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger M, and Simonneau G.

Subjects
medicine.medical_treatment, Diagnostic Techniques, Cardiovascular, Cardiovascular Agents/therapeutic use, Cardiovascular, Anoxia, Risk Factors, Biological Markers/blood, Hypoxia, Referral and Consultation, Heart Diseases/complications/diagnosis/therapy, media_common, Terminal Care, ddc:618, Exercise Tolerance, Pulmonary, Prognosis, Terminal Care/methods, Thromboembolism/complications/diagnosis/therapy, Hypertension, Cardiology, Cardiology and Cardiovascular Medicine, Algorithms, Lung Transplantation, medicine.medical_specialty, Hypertension, Pulmonary/*diagnosis/etiology/*therapy, Heart Diseases, media_common.quotation_subject, Hypertension, Pulmonary, MEDLINE, Pulmonary Veno-Occlusive Disease/complications/diagnosis/therapy, Internal medicine, Thromboembolism, Exercise Tolerance/physiology, medicine, Lung transplantation, Humans, Quality (business), Task force, business.industry, Conflict of interest, Cardiovascular Agents, Evidence-based medicine, Diagnostic Techniques, Clinical trial, Cardiovascular agent, Anoxia/complications/diagnosis/therapy, Pulmonary Veno-Occlusive Disease, business, Biomarkers
Abstract

Guidelines and Expert Consensus Documents summarize and evaluate all currently available evidence on a particular issue with the aim to assist physicians in selecting the best management strategies for a typical patient, suffering from a given condition, taking into account the impact on outcome, as well as the risk/benefit ratio of particular diagnostic or therapeutic means. Guidelines are no substitutes for textbooks. The legal implications of medical guidelines have been discussed previously. A great number of Guidelines and Expert Consensus Documents have been issued in recent years by the European Society of Cardiology (ESC) as well as by other societies and organizations. Because of the impact on clinical practice, quality criteria for development of guidelines have been established in order to make all decisions transparent to the user. The recommendations for formulating and issuing ESC Guidelines and Expert Consensus Documents can be found on the ESC website (http://www.escardio.org/knowledge/guidelines). In brief, experts in the field are selected and undertake a comprehensive review of the published evidence for management and/or prevention of a given condition. Unpublished clinical trial results are not taken into account. A critical evaluation of diagnostic and therapeutic procedures is performed including assessment of the risk/benefit ratio. Estimates of expected health outcomes for larger societies are included, where data exist. The level of evidence and the strength of recommendation of particular treatment options are weighed and graded according to predefined scales, as outlined in Tables 1 and 2 . View this table: Table 1 Classes of recommendations View this table: Table 2 Levels of evidence The experts of the writing panels have provided disclosure statements of all relationships they may have which might be perceived as real or potential sources of conflicts of interest. These disclosure forms are kept on file at the European Heart House, headquarters of the ESC. Any changes in conflict of interest that arise …

Published
2009

38. 5022Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease

Author

Girerd, B., primary, Montani, D., additional, Jais, X., additional, Levy, M., additional, Savale, L., additional, Dorfmuller, P., additional, Lau, E., additional, Le Pavec, J., additional, Parent, F., additional, Bonnet, D., additional, Soubrier, F., additional, Fadel, E., additional, Sitbon, O., additional, Simonneau, G., additional, and Humbert, M., additional

Published
2017
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39. Platelet-derived growth factor expression in primary pulmonary hypertension: comparison of HIV seropositive and HIV seronegative patients

Author

Marc Humbert, Monti G, Fartoukh M, Magnan A, Brenot F, Rain B, Capron F, Galanaud P, Duroux P, Simonneau G, and Emilie D

Subjects
Adult, Male, Platelet-Derived Growth Factor, Pulmonary and Respiratory Medicine, Biopsy, Hypertension, Pulmonary, Gene Expression, HIV Infections, Middle Aged, Polymerase Chain Reaction, Immunoenzyme Techniques, Case-Control Studies, HIV Seronegativity, HIV Seropositivity, HIV-1, Humans, Female, RNA, Messenger, Lung, In Situ Hybridization
Abstract

Primary pulmonary hypertension (PPH) is characterized by intimal fibrosis and cell proliferation (including fibroblasts, smooth muscle and endothelial cells) in the distal pulmonary arterial tree. Considerable interest has been generated by recent reports of PPH in human immunodeficiency virus (HIV)-1-infected individuals. Although the lack of evidence for a pulmonary artery infection has suggested that in such cases HIV may act through mediator release rather than by direct endothelial infection, the mechanisms underlying HIV-associated PPH remain poorly defined. Platelet-derived growth factor (PDGF) has the ability to induce smooth muscle cell and fibroblast proliferation and migration. Given these considerations, we have attempted to document a possible role for PDGF in PPH occurring in HIV seropositive and seronegative patients. Using semiquantitative polymerase chain reaction (PCR), PDGF A-chain messenger ribonucleic acid (mRNA) expression was analysed in surgical lung biopsies from 13 HIV seronegative patients and one HIV seropositive patient, all displaying severe PPH. In parallel, lung samples from two patients with HIV-1-associated PPH were studied by immunohistochemistry and in situ hybridization. Results were compared to those obtained in three HIV-1-infected individuals with no pulmonary complication (as demonstrated by clinical, radiological, bacteriological, and necropsy findings) and five control lung biopsies. As compared to controls, PDGF A-chain mRNA expression is elevated in lung biopsies from patients displaying PPH (p=0.029). In HIV-1-associated PPH, interstitial perivascular cells expressing PDGF A-chain mRNA and protein could be detected by in situ hybridization and immunohistochemistry, respectively. Platelet-derived growth factor expression is elevated in lung biopsies of patients displaying primary pulmonary hypertension. Growth factors such as platelet-derived growth factor may play a part in the initiation and/or progression of primary pulmonary hypertension.

Published
1998

40. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology

Author

GALIE', NAZZARENO, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, Olschewski H, Peacock A, Pietra G, Rubin LJ, Simonneau G, Priori SG, Garcia MA, Blanc JJ, Budaj A, Cowie M, Dean V, Deckers J, Burgos EF, Lekakis J, Lindahl B, Mazzotta G, McGregor K, Morais J, Oto A, Smiseth OA, Barbera JA, Gibbs S, Hoeper M, Humbert M, Naeije R, Pepke Zaba J., Galie N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, Olschewski H, Peacock A, Pietra G, Rubin LJ, Simonneau G, Priori SG, Garcia MA, Blanc JJ, Budaj A, Cowie M, Dean V, Deckers J, Burgos EF, Lekakis J, Lindahl B, Mazzotta G, McGregor K, Morais J, Oto A, Smiseth OA, Barbera JA, Gibbs S, Hoeper M, Humbert M, Naeije R, and Pepke-Zaba J

Subjects
Arterial Occlusive Disease, Hypertension, Pulmonary, Arterial Occlusive Diseases, Cardiovascular Agents, HIV Infections, Dyspnea, Cardiovascular Agent, Risk Factors, Humans, HIV Infection, Connective Tissue Diseases, Hemangioma, Connective Tissue Disease, Human
Abstract

No abstract available

Published
2004

41. Apixaban for extended treatment of venous thromboembolism

Author

Agnelli, G, Buller, H, Cohen, A, Gallus, A, Raskob, G, Weitz, J, Prins, M, Brandjes, D, Kolbach, D, Limburg, M, Mac Gillavry, M, Otten, Jm, Peters, R, Roos, Y, Segers, A, Slagboom, T, Bounameaux, H, Hirsh, J, Samama, Mm, Wedel, H, Curto, M, Johnson, M, Masiukiewicz, U, Pak, R, Porcari, A, Sanders, P, Sisson, M, Sullivan, B, Thompson, J, Auerbach, J, Cesario, L, Gamero, M, Gordon, M, Griffiths, A, Noble, M, Ott, J, Pennington, A, Peffer, A, Reinhold, P, Simmons, M, Urwin, K, Ceresetto, J, Mcrae, S, Pabinger, I, Pereira, Ah, Spencer, F, Gorican, K, Husted, Se, Mottier, D, Harenberg, J, Pinjala, R, Zeltser, D, Imberti, D, Sandset, M, Torbicki, A, Fijalkowska, A, Albino, Jp, Kirienko, A, Shvarts, Y, Monreal, M, Jacobson, B, Dolan, G, Gudz, I, Ortel, T, Spyropoulos, A, Skupyy, O, Beryer Westendorf, J, De Pellegrin, A, Prasol, V, Schellong, S, Falvo, N, Abramov, I, Cizek, V, Husted, S, Desai, S, Barillari, G, Sergeev, O, Chetter, I, Inbal, A, Mccollum, C, Shvalb, P, Torp Pedersen, C, Vasylyuk, S, Kraemmer Nielsen, H, Pernod, G, Schmidt, J, Bova, C, Gerasymov, V, Pabinger Fasching, I, Skalicka, L, Zaichuk, A, Achkar, A, Bremmelgaard, A, Chochola, J, Gould, T, Khalafallah, A, Jakobsen, T, Rose, P, Zhukov, B, Dedek, V, Mirete Ferrer, J, Pesant, Y, Repin, A, Salem, H, Solis Morales, L, Spacek, R, Cannon, K, Grzelakowski, P, Jindal, R, Pereira, A, Zidkova, E, Ambrosio, G, Cardozo, M, Dunaj, M, Gavish, D, Ghanima, W, Leduc, Jj, Mismetti, P, Panico, M, Porreca, E, Riera, A, Bareford, D, Chong, B, Dvoryashina, I, Gómez Cerezo, J, Kobza, I, Nielsen, T, Pendleton, R, Pullman, J, Schiffman, G, Stanbro, M, Zwettler, U, Aquilanti, S, Bratsch, H, Cohen, K, Elias, D, Gan, E, Holaj, R, Klinke, W, Liu, Hs, Sandset, Pm, van Nieuwenhuizen, E, Álvarez Sala LA, Basson, M, Braester, A, Bura Riviere, A, Calvo Vargas, C, Correa, J, Elias, M, Frost, L, Landolfi, R, Marschang, P, Moreira, R, Natarajan, S, Pottier, P, Tosetto, A, Tuxen, C, Vöhringer, Hf, Alexander, A, Barbarash, O, Fajardo Campos, P, Graham, M, Gubka, O, Hudcovic, M, Hussein, O, Jackson, D, Katelnitskiy, I, Lawall, H, Palareti, G, Poggio, R, Roos, J, Simonneau, G, Smith, Sw, Szopinski, P, Zimlichman, R, Bridgers, D, Colan, D, Czekalski, P, De Jong, D, Fortinez, Jt, Garcia Bragado, F, Harrington, D, Izbicki, G, Kadr, H, Koslow, A, Loftus, I, Marais, H, Neumeister, A, Oliven, A, Palla, A, Pop, C, Prandoni, Paolo, Puskas, A, Sanchez Llamas, F, Shotan, A, Singh, P, Tveit, A, Baker, R, Borja, V, Brenner, B, Brown, H, Cha, Tj, Cohen, Y, D'Angelo, A, Dhar, A, Friis, E, Hueur, H, Jiménez Rodríguez Madridejos, R, Karl, J, Karrasch, J, Lishner, M, Manenti, E, Meneveau, N, Nguyen, D, Sanchez Escalante, L, Santoscoy Ibarra, J, Sokurenko, G, Staroverov, I, Stein, R, Abdullah, I, Alcocer Gamba, M, Balanda, J, Bruckner, I, Calabuig Alborch, J, Caraco, Y, Comerota, A, Cromer, M, de Araujo Filho, J, De los Rios Ibarra, M, Diaz Castañon, J, Doshi, A, Ebrahim, I, Fessel, Wj, Fletcher, E, Fourie, N, Fu, C, Gutowski, P, Haddad, G, Hoffman, U, Jardula, M, Kvasnicka, T, Lewczuk, J, Leyden, M, Livneh, A, Lodigiani, C, Lovell, C, Miekus, P, Paloma, Mj, Parakh, R, Raval, M, Schmidt Lucke, J, Shtutin, O, Soroka, V, Stevens, D, Sulik, P, Tay, Jc, Vejby Christensen, H, Vinereanu, D, Baghestanian, M, Bono, J, Cerana, S, Freire, A, Gibson, K, Giumelli, C, Iastrebner, C, Karpenko, A, Kelly, A, Lacroix, P, Lafata, J, Lobo, S, Macik, Bg, Marchena Yglesias, P, Nishinari, K, Podczeck Schweighofer, A, Raby, K, Sirpal, S, Solymoss, S, van Zyl, L, Vargas Núñez JA, von Bilderling, P, Warr, T, Wronski, J, Wurster, M, Albino, Ja, Albuquerque, L, Averill, F, Baek, Sh, Bello, F, Bergoeing, M, Blanc, Fx, Bloomberg, R, Bolster, D, Brockmyre, A, Calimano, C, Checketts, D, Cieplinski, W, Chervu, A, Collado, F, Denaro, C, Gaciong, Z, Game, M, Iskander, A, Kaatz, S, Kim, Di, Koura, F, Laguna, F, Lanas Zanetti, F, Lindhoff Last, E, Melaniuk, M, Meade, A, Murphy, T, Ng, Hj, Páramo Fernández JA, Patil, C, Piovella, F, Prisco, D, Pruszczyk, P, Reimers, G, Rivera, E, Rodriguez Cintron, W, Rosenthal, S, Salbach, P, Salvador, D, Schuller, D, Siragusa, S, Staniszewski, R, Torp, R, Vora, K, Yip, G, Alfieri, A, Belaji, V, Bhagavan, N, Carnovali, M, Cobos Segarra, J, Di Todaro, F, Dowell, A, Corder, C, Crispin, P, Cuadrado, J, Flippo, G, Fraiz, J, Guillaumon, A, Gvora, T, Hakki, S, Harris, L, Ison, R, Htun, Pt, Jasani, R, Kates, M, Kaminski, L, Kamerkar, D, Kroger, K, Laperna, L, Leiva, J, Luber, J, Mccann, A, Mckenzie, W, Menna Barreto, S, Moran, J, Nikulnikov, P, Paliwal, Y, Patel, M, Pilger, E, Renwick, W, Shevela, A, Starosiliz, D, Stringam, S, To, R, Updegrove, J, Van Bellen, B, Waintrub, M, White, J, Yeo, E, Zangroniz, P, Zeltser, D., ACS - Amsterdam Cardiovascular Sciences, Vascular Medicine, APH - Amsterdam Public Health, Cardiology, ANS - Amsterdam Neuroscience, Neurology, Department of Vascular Medicine (DVM - AMC), Academic Medical Center - Academisch Medisch Centrum [Amsterdam] (AMC), University of Amsterdam [Amsterdam] (UvA)-University of Amsterdam [Amsterdam] (UvA), Thrombosis and Atherosclerosis Research Institute (TARI), McMaster University [Hamilton, Ontario], Centre d'Investigation Clinique (CIC - Brest), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM), Groupe d'Etude de la Thrombose de Bretagne Occidentale (GETBO), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), and Université de Brest (UBO)-Université de Brest (UBO)

Subjects
Male, Kaplan-Meier Estimate, 030204 cardiovascular system & hematology, Placebo group, DISEASE, law.invention, 0302 clinical medicine, Randomized controlled trial, law, Recurrence, Fibrinolytic agents, 030212 general & internal medicine, IDRAPARINUX, Administration of drugs, Follow up studies, food and beverages, General Medicine, Venous Thromboembolism, Middle Aged, 3. Good health, Intention to Treat Analysis, Treatment Outcome, Treatment dose, Anesthesia, Creatinine, Factor Xa, Fibrinolítics, Apixaban, Female, Administració de medicaments, Major bleeding, medicine.drug, ARTERIAL CARDIOVASCULAR EVENTS, INTENSITY WARFARIN THERAPY, PULMONARY-EMBOLISM, LONG-TERM, PREVENTION, Adult, Pyridones, Hemorrhage, 03 medical and health sciences, Double-Blind Method, Fibrinolytic Agents, Thromboembolism, medicine, Humans, Tromboembolisme, Aged, Intention-to-treat analysis, business.industry, fungi, Pyrazoles, business, Venous thromboembolism, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, Factor Xa Inhibitors, Follow-Up Studies
Abstract

International audience; Background Apixaban, an oral factor Xa inhibitor that can be administered in a simple, fixed-dose regimen, may be an option for the extended treatment of venous thromboembolism. Methods In this randomized, double-blind study, we compared two doses of apixaban (2.5 mg and 5 mg, twice daily) with placebo in patients with venous thromboembolism who had completed 6 to 12 months of anticoagulation therapy and for whom there was clinical equipoise regarding the continuation or cessation of anticoagulation therapy. The study drugs were administered for 12 months. Results A total of 2486 patients underwent randomization, of whom 2482 were included in the intention-to-treat analyses. Symptomatic recurrent venous thromboembolism or death from venous thromboembolism occurred in 73 of the 829 patients (8.8%) who were receiving placebo, as compared with 14 of the 840 patients (1.7%) who were receiving 2.5 mg of apixaban (a difference of 7.2 percentage points; 95% confidence interval [CI], 5.0 to 9.3) and 14 of the 813 patients (1.7%) who were receiving 5 mg of apixaban (a difference of 7.0 percentage points; 95% CI, 4.9 to 9.1) (P

Published
2012

42. [HIV-related pulmonary arterial hypertension]

Author

Savale, L, Lador, Frédéric, Jais, X, Montani, D, Simonneau, G, Humbert, M, and Sitbon, O

Subjects
ddc:616, Hypertension, Pulmonary, HIV-1/physiology, Diagnostic Techniques, Cardiovascular, HIV-1, virus diseases, Diagnostic Techniques, Respiratory System, Humans, HIV Infections, HIV Infections/complications/diagnosis/epidemiology/therapy, Hypertension, Pulmonary/diagnosis/epidemiology/etiology/therapy, Models, Biological
Abstract

Pulmonary arterial hypertension (PAH) is a rare but potentially fatal complication of human immunodeficiency virus (HIV). It may occur in HIV-1 or 2 infection, irrespective of the route of transmission or the degree of immunosuppression. The improved survival of patients infected with HIV in the era of highly active antiretroviral therapy (HAART) justifies systematic screening for PAH according to an algorithm in patients with unexplained dyspnea. In all cases, right heart catheterization must be performed to establish the definitive diagnosis of pulmonary hypertension. The prevalence of PAH is about 0.5% in patients with HIV infection. A beneficial effect of HAART on the course of HIV-related PAH has not been clearly established. In contrast, PAH-specific therapies such as epoprostenol and bosentan have been demonstrated to be efficacious for short- and long-term outcomes in this context. Notably, some patients pulmonary hemodynamics and functional class normalized or near normalized with these treatments. Other PAH-specific therapies remain to be evaluated. The advent of HAART associated with the development of PAH-specific therapies has improved the prognosis of patients HIV-related PAH, with a survival rate of about 70% at 3 years.

Published
2011

43. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome

Author

Cottin, V., Le Pavec, J., Prévot, G., Mal, H., Humbert, M., Simonneau, G., Cordier, J.-F., Berezné, A., Coëtmeur, D., Danner-Boucher, I., Funke, D., Israel-Biet, D., Marchand, Eric, Mouthon, L., Rétrovirus et Pathologie Comparée (RPC), Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Ecole Nationale Vétérinaire de Lyon (ENVL), Hôpital Louis Pradel, Service de Pneumologie, Centre de Référence National des Maladies Pulmonaires Rares, Hospices Civils de Lyon (HCL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Service de Pneumologie et Réanimation, Centre de reference de l'hypertension artérielle pulmonaire, Hôpital Antoine Béclère, Assistance Publique - Hôpitaux de Paris (AP-HP), Université Paris Sud (Paris 11), Hôpital Larrey, Service de Pneumologie, Centre Hospitalier Universitaire de Toulouse, Hôpital Bichat - Claude Bernard, Assistance Publique - Hôpitaux de Paris (AP-HP), Groupe de recherches sur les Maladies Orphelines Pulmonaires (GERMOP), European Respiratory Society [ERS TF 2008-08], UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, UCL - (MGD) Service de pneumologie, Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE), and Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL)

Subjects
Male, emphysème pulmonaire, Pulmonary Fibrosis, Cardiac index, 030204 cardiovascular system & hematology, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Pulmonary function testing, 0302 clinical medicine, Pulmonary fibrosis, pulmonary hypertension, disproportionate, Aged, 80 and over, Chronic obstructive pulmonary disease, Respiratory disease, Syndrome, Middle Aged, Combined pulmonary fibrosis and emphysema, Tobacco smoking, 3. Good health, Survival Rate, medicine.anatomical_structure, emphysema, Pulmonary Emphysema, Cardiology, Female, Pulmonary and Respiratory Medicine, medicine.medical_specialty, fibrose pulmonaire, Hypertension, Pulmonary, Pulmonary hypertension, chronic obstructive pulmonary disease, 03 medical and health sciences, Internal medicine, medicine.artery, medicine, hypertension pulmonaire, Humans, tabagisme, tobacco smoking, Aged, Retrospective Studies, Emphysema, Disproportionate, business.industry, Hemodynamics, medicine.disease, Surgery, 030228 respiratory system, Pulmonary artery, Vascular resistance, business, taux de survie
Abstract

This study aims to describe the haemodynamic and survival characteristics of patients with pulmonary hypertension in the recently individualised syndrome of combined pulmonary fibrosis and emphysema. A retrospective multicentre study was conducted in 40 patients (38 males; age 68±9 yrs; 39 smokers) with combined pulmonary fibrosis and emphysema, and pulmonary hypertension at right heart catheterisation. Dyspnoea was functional class II in 15%, III in 55% and IV in 30%. 6-min walk distance was 244±126 m. Forced vital capacity was 86±18%, forced expiratory volume in 1 s 78±19%, and carbon monoxide diffusion transfer coefficient 28±16% of predicted. Room air arterial oxygen tension was 7.5±1.6 kPa (56±12 mmHg). Mean pulmonary artery pressure was 40±9 mmHg, cardiac index 2.5±0.7 L·min -1·m -2 and pulmonary vascular resistance 521±205 dyn·s·cm -5. 1-yr survival was 60%. Higher pulmonary vascular resistance, higher heart rate, lower cardiac index and lower carbon monoxide diffusion transfer were associated with shorter survival. Patients with combined pulmonary fibrosis and emphysema syndrome and pulmonary hypertension confirmed by right heart catheterisation have a dismal prognosis despite moderately altered lung volumes and flows and moderately severe haemodynamic parameters. Copyright©ERS Journals Ltd 2010.

Published
2009

44. Tadalafil Therapy for Pulmonary Arterial Hypertension

Author

Galie, N., Brundage, B. H., Ghofrani, H. A., Oudiz, R. J., Simonneau, G., Safdar, Z., Shapiro, S., White, R. J., Chan, M., Beardsworth, A., Frumkin, L., Barst, R. J., Arterial Hypertension Pulmonary, Tadalafil Study Group: Response, T. O., Fedele, Francesco, and Inconnu

Subjects
Relative risk reduction, Adult, Endothelin Receptor Antagonists, Male, medicine.medical_specialty, hypertension, medicine.drug_mechanism_of_action, pulmonary, phosphodiesterase inhibitors, tadalafil, Phosphodiesterase Inhibitors, Hypertension, Pulmonary, [SDV]Life Sciences [q-bio], Placebo, Tadalafil, Double-Blind Method, Physiology (medical), Internal medicine, medicine, Humans, Adverse effect, Aged, Sulfonamides, Exercise Tolerance, business.industry, Endothelin receptor antagonist, Bosentan, Middle Aged, medicine.disease, Pulmonary hypertension, Surgery, Treatment Outcome, Cardiology, Quality of Life, Female, Cardiology and Cardiovascular Medicine, business, Phosphodiesterase 5 inhibitor, medicine.drug, Carbolines
Abstract

Background— Treatment options for pulmonary arterial hypertension target the prostacyclin, endothelin, or nitric oxide pathways. Tadalafil, a phosphodiesterase type-5 inhibitor, increases cGMP, the final mediator in the nitric oxide pathway. Methods and Results— In this 16-week, double-blind, placebo-controlled study, 405 patients with pulmonary arterial hypertension (idiopathic or associated), either treatment-naive or on background therapy with the endothelin receptor antagonist bosentan, were randomized to placebo or tadalafil 2.5, 10, 20, or 40 mg orally once daily. The primary end point was the change from baseline to week 16 in the distance walked in 6 minutes. Changes in World Health Organization functional class, clinical worsening, and health-related quality of life were also assessed. Patients completing the 16-week study could enter a long-term extension study. Tadalafil increased the distance walked in 6 minutes in a dose-dependent manner; only the 40-mg dose met the prespecified level of statistical significance ( P P =0.041), incidence of clinical worsening (68% relative risk reduction; P =0.038), and health-related quality of life. The changes in World Health Organization functional class were not statistically significant. The most common treatment-related adverse events reported with tadalafil were headache, myalgia, and flushing. Conclusions— In patients with pulmonary arterial hypertension, tadalafil 40 mg was well tolerated and improved exercise capacity and quality of life measures and reduced clinical worsening.

Published
2009

45. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The task force on diagnosis and treatment of pulmonary arterial hypertension of the European society of cardiology

Author

Galiè, N, Torbicki, A, Barst, R, Dartevelle, P, Haworth, S, Higenbottam, T, Olschewski, H, Peacock, A, Pietra, G, Rubin, LJ, Simonneau, G, Priori, SG, Garcia, MAA, le Blanc, J-J, Budaj, A, Cowie, M, Dean, V, Deckers, Jaap, Burgos, EF, Lekakis, J, Lindahl, B, Mazzotta, G, McGregor, K, Morais, J, Oto, A, Smiseth, O, and Cardiology

Published
2004

48. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry

Author

Pepke-Zaba, J, Delcroix, M, Lang, I, Mayer, E, Jansa, P, Ambroz, D, Treacy, C, D'Arminio Monforte, A, Morsolini, M, Snijder, R, Bresser, P, Torbicki, A, Kristensen, B, Lewczuk, J, Simkova, I, Barberà, J A, de Perrot, M, Hoeper, M M, Gaine, S, Speich, R, Gomez-Sanchez, M A, Kovacs, G, Hamid, A M, Jaïs, X, Simonneau, G, Pepke-Zaba, J, Delcroix, M, Lang, I, Mayer, E, Jansa, P, Ambroz, D, Treacy, C, D'Arminio Monforte, A, Morsolini, M, Snijder, R, Bresser, P, Torbicki, A, Kristensen, B, Lewczuk, J, Simkova, I, Barberà, J A, de Perrot, M, Hoeper, M M, Gaine, S, Speich, R, Gomez-Sanchez, M A, Kovacs, G, Hamid, A M, Jaïs, X, and Simonneau, G

Abstract

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with fatal natural history; however, many cases can be cured by pulmonary endarterectomy. The clinical characteristics and current management of patients enrolled in an international CTEPH registry was investigated. METHODS AND RESULTS: The international registry included 679 newly diagnosed (≤6 months) consecutive patients with CTEPH, from February 2007 until January 2009. Diagnosis was confirmed by right heart catheterization, ventilation-perfusion lung scintigraphy, computerized tomography, and/or pulmonary angiography. At diagnosis, a median of 14.1 months had passed since first symptoms; 427 patients (62.9%) were considered operable, 247 (36.4%) nonoperable, and 5 (0.7%) had no operability data; 386 patients (56.8%, ranging from 12.0%- 60.9% across countries) underwent surgery. Operable patients did not differ from nonoperable patients relative to symptoms, New York Heart Association class, and hemodynamics. A history of acute pulmonary embolism was reported for 74.8% of patients (77.5% operable, 70.0% nonoperable). Associated conditions included thrombophilic disorder in 31.9% (37.1% operable, 23.5% nonoperable) and splenectomy in 3.4% of patients (1.9% operable, 5.7% nonoperable). At the time of CTEPH diagnosis, 37.7% of patients initiated at least 1 pulmonary arterial hypertension-targeted therapy (28.3% operable, 53.8% nonoperable). Pulmonary endarterectomy was performed with a 4.7% documented mortality rate. CONCLUSIONS: Despite similarities in clinical presentation, operable and nonoperable CTEPH patients may have distinct associated medical conditions. Operability rates vary considerably across countries, and a substantial number of patients (operable and nonoperable) receive off-label pulmonary arterial hypertension-targeted treatments.

Published
2011

49. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome

Author

UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, UCL - (MGD) Service de pneumologie, Cottin, V., Le Pavec, J., Prévot, G., Mal, H., Humbert, M., Simonneau, G., Cordier, J.-F., Berezné, A., Coëtmeur, D., Danner-Boucher, I., Funke, D., Israel-Biet, D., Marchand, Eric, Mouthon, L., UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, UCL - (MGD) Service de pneumologie, Cottin, V., Le Pavec, J., Prévot, G., Mal, H., Humbert, M., Simonneau, G., Cordier, J.-F., Berezné, A., Coëtmeur, D., Danner-Boucher, I., Funke, D., Israel-Biet, D., Marchand, Eric, and Mouthon, L.

Abstract

This study aims to describe the haemodynamic and survival characteristics of patients with pulmonary hypertension in the recently individualised syndrome of combined pulmonary fibrosis and emphysema. A retrospective multicentre study was conducted in 40 patients (38 males; age 68±9 yrs; 39 smokers) with combined pulmonary fibrosis and emphysema, and pulmonary hypertension at right heart catheterisation. Dyspnoea was functional class II in 15%, III in 55% and IV in 30%. 6-min walk distance was 244±126 m. Forced vital capacity was 86±18%, forced expiratory volume in 1 s 78±19%, and carbon monoxide diffusion transfer coefficient 28±16% of predicted. Room air arterial oxygen tension was 7.5±1.6 kPa (56±12 mmHg). Mean pulmonary artery pressure was 40±9 mmHg, cardiac index 2.5±0.7 L·min -1·m -2 and pulmonary vascular resistance 521±205 dyn·s·cm -5. 1-yr survival was 60%. Higher pulmonary vascular resistance, higher heart rate, lower cardiac index and lower carbon monoxide diffusion transfer were associated with shorter survival. Patients with combined pulmonary fibrosis and emphysema syndrome and pulmonary hypertension confirmed by right heart catheterisation have a dismal prognosis despite moderately altered lung volumes and flows and moderately severe haemodynamic parameters. Copyright©ERS Journals Ltd 2010.

Published
2010

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