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1. Imaging the right atrium in pulmonary hypertension: A systematic review and meta-analysis.

Author

Richter, M.J., Fortuni, F., Alenezi, F., D'Alto, M., Badagliacca, R., Brunner, N.W., Dijk, A.P. van, Douschan, P., Gall, H., Ghio, S., Giudice, F.L., Grünig, E., Haddad, F., Howard, L., Rajagopal, Sudarshan, Stens, N.A., Stolfo, D., Thijssen, D.H.J., Vizza, C.D., Zamanian, R.T., Zhong, L., Seeger, W., Ghofrani, H.A., Tello, K., Richter, M.J., Fortuni, F., Alenezi, F., D'Alto, M., Badagliacca, R., Brunner, N.W., Dijk, A.P. van, Douschan, P., Gall, H., Ghio, S., Giudice, F.L., Grünig, E., Haddad, F., Howard, L., Rajagopal, Sudarshan, Stens, N.A., Stolfo, D., Thijssen, D.H.J., Vizza, C.D., Zamanian, R.T., Zhong, L., Seeger, W., Ghofrani, H.A., and Tello, K.

Abstract

01 april 2023, Item does not contain fulltext, BACKGROUND: Right atrial (RA) imaging has emerged as a promising tool for the evaluation of patients with pulmonary hypertension (PH), albeit without systematic validation. METHODS: PubMed, Web of Science and the Cochrane library were searched for studies investigating the prognostic value of RA imaging assessment in patients with PH from 2000 to June 2021 (PROSPERO Identifier: CRD42020212850). An inverse variance-weighted meta-analysis of univariable hazard ratios (HRs) was performed using a random effects model. RESULTS: Thirty-five studies were included (3,476 patients with PH; 74% female, 86% pulmonary arterial hypertension). Risk of bias was low/moderate (Quality of Prognosis Studies checklist). RA area (HR 1.06; 95% confidence interval [CI] 1.04-1.08), RA indexed area (HR 1.09; 95% CI 1.04-1.14), RA peak longitudinal strain (PLS; HR 0.94; 95% CI 0.91-0.97) and RA total emptying fraction (HR 0.96; 95% CI 0.94-0.98) were significantly associated with combined end-points including death, clinical worsening and/or lung transplantation; RA volume and volume index showed marginal significant associations. RA area (HR 1.06; 95% CI 1.04-1.07), RA indexed area (HR 1.12; 95% CI 1.07-1.17) and RA PLS (HR 0.98; 95% CI 0.97-0.99) showed significant associations with mortality; RA total emptying fraction showed a marginal association. CONCLUSIONS: Imaging-based RA assessment qualifies as a relevant prognostic marker in PH. RA area reliably predicts composite end-points and mortality, which underscores its clinical utility. RA PLS emerged as a promising imaging measure, but is currently limited by the number of studies and different acquisition methods.

Published
2023

3. Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis

Author

Hoeper, M.M. Dwivedi, K. Pausch, C. Lewis, R.A. Olsson, K.M. Huscher, D. Pittrow, D. Grünig, E. Staehler, G. Vizza, C.D. Gall, H. Distler, O. Opitz, C. Gibbs, J.S.R. Delcroix, M. Park, D.-H. Ghofrani, H.A. Ewert, R. Kaemmerer, H. Kabitz, H.-J. Skowasch, D. Behr, J. Milger, K. Lange, T.J. Wilkens, H. Seyfarth, H.-J. Held, M. Dumitrescu, D. Tsangaris, I. Vonk-Noordegraaf, A. Ulrich, S. Klose, H. Claussen, M. Eisenmann, S. Schmidt, K.-H. Swift, A.J. Thompson, A.A.R. Elliot, C.A. Rosenkranz, S. Condliffe, R. Kiely, D.G. Halank, M. and Hoeper, M.M. Dwivedi, K. Pausch, C. Lewis, R.A. Olsson, K.M. Huscher, D. Pittrow, D. Grünig, E. Staehler, G. Vizza, C.D. Gall, H. Distler, O. Opitz, C. Gibbs, J.S.R. Delcroix, M. Park, D.-H. Ghofrani, H.A. Ewert, R. Kaemmerer, H. Kabitz, H.-J. Skowasch, D. Behr, J. Milger, K. Lange, T.J. Wilkens, H. Seyfarth, H.-J. Held, M. Dumitrescu, D. Tsangaris, I. Vonk-Noordegraaf, A. Ulrich, S. Klose, H. Claussen, M. Eisenmann, S. Schmidt, K.-H. Swift, A.J. Thompson, A.A.R. Elliot, C.A. Rosenkranz, S. Condliffe, R. Kiely, D.G. Halank, M.

Abstract

Background: Among patients meeting diagnostic criteria for idiopathic pulmonary arterial hypertension (IPAH), there is an emerging lung phenotype characterised by a low diffusion capacity for carbon monoxide (DLCO) and a smoking history. The present study aimed at a detailed characterisation of these patients. Methods: We analysed data from two European pulmonary hypertension registries, COMPERA (launched in 2007) and ASPIRE (from 2001 onwards), to identify patients diagnosed with IPAH and a lung phenotype defined by a DLCO of less than 45% predicted and a smoking history. We compared patient characteristics, response to therapy, and survival of these patients to patients with classical IPAH (defined by the absence of cardiopulmonary comorbidities and a DLCO of 45% or more predicted) and patients with pulmonary hypertension due to lung disease (group 3 pulmonary hypertension). Findings: The analysis included 128 (COMPERA) and 185 (ASPIRE) patients with classical IPAH, 268 (COMPERA) and 139 (ASPIRE) patients with IPAH and a lung phenotype, and 910 (COMPERA) and 375 (ASPIRE) patients with pulmonary hypertension due to lung disease. Most patients with IPAH and a lung phenotype had normal or near normal spirometry, a severe reduction in DLCO, with the majority having no or a mild degree of parenchymal lung involvement on chest computed tomography. Patients with IPAH and a lung phenotype (median age, 72 years [IQR 65–78] in COMPERA and 71 years [65–76] in ASPIRE) and patients with group 3 pulmonary hypertension (median age 71 years [65–77] in COMPERA and 69 years [63–74] in ASPIRE) were older than those with classical IPAH (median age, 45 years [32–60] in COMPERA and 52 years [38–64] in ASPIRE; p<0·0001 for IPAH with a lung phenotype vs classical IPAH in both registries). While 99 (77%) patients in COMPERA and 133 (72%) patients in ASPIRE with classical IPAH were female, there was a lower proportion of female patients in the IPAH and a lung phenotype cohort (95 [35%]

Published
2022

4. Prognostic value of improvement endpoints in pulmonary arterial hypertension trials: A COMPERA analysis

Author

Hoeper, M.M. Pausch, C. Olsson, K.M. Huscher, D. Pittrow, D. Grünig, E. Staehler, G. Vizza, C.D. Gall, H. Distler, O. Opitz, C. Gibbs, J.S.R. Delcroix, M. Ghofrani, H.A. Ewert, R. Kaemmerer, H. Kabitz, H.-J. Skowasch, D. Behr, J. Milger, K. Halank, M. Wilkens, H. Seyfarth, H.-J. Held, M. Dumitrescu, D. Tsangaris, I. Vonk-Noordegraaf, A. Ulrich, S. Klose, H. Claussen, M. Eisenmann, S. Schmidt, K.-H. Rosenkranz, S. Lange, T.J. and Hoeper, M.M. Pausch, C. Olsson, K.M. Huscher, D. Pittrow, D. Grünig, E. Staehler, G. Vizza, C.D. Gall, H. Distler, O. Opitz, C. Gibbs, J.S.R. Delcroix, M. Ghofrani, H.A. Ewert, R. Kaemmerer, H. Kabitz, H.-J. Skowasch, D. Behr, J. Milger, K. Halank, M. Wilkens, H. Seyfarth, H.-J. Held, M. Dumitrescu, D. Tsangaris, I. Vonk-Noordegraaf, A. Ulrich, S. Klose, H. Claussen, M. Eisenmann, S. Schmidt, K.-H. Rosenkranz, S. Lange, T.J.

Abstract

BACKGROUND: The prognostic value of improvement endpoints that have been used in clinical trials of treatments for pulmonary arterial hypertension (PAH) needs to be further investigated. METHODS: Using the COMPERA database, we evaluated the prognostic value of improvements in functional class (FC) and absolute or relative improvements in 6-min walking distance (6MWD) and N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP). In addition, we investigated multicomponent endpoints based on prespecified improvements in FC, 6MWD and NT-proBNP that have been used in recent PAH trials. Finally, we assessed the predictive value of improvements determined by risk stratification tools. The effects of changes from baseline to first follow-up (3-12 months after initiation of PAH therapy) on consecutive survival were determined by Kaplan-Meier analysis with Log-Rank testing and Cox proportional hazard analyses. RESULTS: All analyses were based on 596 patients with newly diagnosed PAH for whom complete data were available at baseline and first follow-up. Improvements in FC were associated with improved survival, whereas absolute or relative improvements in 6MWD had no predictive value. For NT-proBNP, absolute declines conferred no prognostic information while relative declines by ≥35% were associated with better survival. Improvements in multicomponent endpoints were associated with improved survival and the same was found for risk stratification tools. CONCLUSION: While sole improvements in 6MWD and NT-proBNP had minor prognostic relevance, improvements in multicomponent endpoints and risk stratification tools based on FC, 6MWD, and NT-proBNP were associated with improved survival. These tools should be further explored as outcome measures in PAH trials. © 2022 The Authors

Published
2022

6. 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

7. 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. and 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.

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

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. (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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9. Determinants of pulmonary vascular resistance reduction with upfront oral therapy in idiopathic pulmonary arterial hypertension: relevance in risk assessment

Author

Badagliacca, R, primary, D'Alto, M, additional, Ghio, S, additional, Greco, A, additional, Papa, S, additional, Romeo, E, additional, Casu, G, additional, Corda, M, additional, Paciocco, P, additional, D'Agostino, C, additional, Galgano, G, additional, Stolfo, D, additional, Correale, M, additional, Naeije, R, additional, and Vizza, C.D, additional

Published
2020
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12. Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry

Author

Hoeper, M.M. Pausch, C. Grünig, E. Klose, H. Staehler, G. Huscher, D. Pittrow, D. Olsson, K.M. Vizza, C.D. Gall, H. Benjamin, N. Distler, O. Opitz, C. Gibbs, J.S.R. Delcroix, M. Ghofrani, H.A. Rosenkranz, S. Ewert, R. Kaemmerer, H. Lange, T.J. Kabitz, H.-J. Skowasch, D. Skride, A. Jureviciene, E. Paleviciute, E. Miliauskas, S. Claussen, M. Behr, J. Milger, K. Halank, M. Wilkens, H. Wirtz, H. Pfeuffer-Jovic, E. Harbaum, L. Scholtz, W. Dumitrescu, D. Bruch, L. Coghlan, G. Neurohr, C. Tsangaris, I. Gorenflo, M. Scelsi, L. Vonk-Noordegraaf, A. Ulrich, S. Held, M.

Abstract

The term idiopathic pulmonary arterial hypertension (IPAH) is used to categorize patients with pre-capillary pulmonary hypertension of unknown origin. There is considerable variability in the clinical presentation of these patients. Using data from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension, we performed a cluster analysis of 841 patients with IPAH based on age, sex, diffusion capacity of the lung for carbon monoxide (DLCO

Published
2020

13. Long term effects of bosentan treatment in adult patients with pulmonary arterial hypertension related to congenital heart disease (Eisenmenger physiology): safety, tolerability, clinical, and haemodynamic effect

Author

D'Alto, M., Vizza, C.D., Romeo, E., Badagliacca, R., Santoro, G., Poscia, R., Sarubbi, B., Mancone, M., Argiento, P., Ferrante, F., Russo, M.G., Fedele, F., and Calabro, R.

Subjects
Pulmonary hypertension -- Care and treatment, Bosentan -- Dosage and administration, Bosentan -- Research, Health
Published
2007

21. FRI0445 Efficacy and Safety of Riociguat in Patients with Pulmonary Arterial Hypertension (PAH) Associated with Connective Tissue Disease (CTD): Results from Patent-1 and Patent-2

Author

Denton, C., primary, Coghlan, J.G., additional, Ghofrani, H.-A., additional, Grimminger, F., additional, He, J., additional, Riemekasten, G., additional, Vizza, C.D., additional, Boeckenhoff, A., additional, Meier, C., additional, Nikkho, S., additional, Pena, J., additional, and Humbert, M., additional

Published
2015
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25. Elevated serum levels of macrophage migration inhibitory factor and stem cell growth factor β in patients with idiopathic and systemic sclerosis associated pulmonary arterial hypertension

Author

Stefanantoni, K., primary, Sciarra, I., additional, Vasile, M., additional, Badagliacca, R., additional, Poscia, R., additional, Pendolino, M., additional, Alessandri, C., additional, Vizza, C.D., additional, Valesini, G., additional, and Riccieri, V., additional

Published
2015
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27. Right Ventricular Dyssynchrony in Idiopathic Pulmonary Arterial Hypertension: Insights From Echocardiographic and Cardiac Magnetic Resonance Imaging

Author

Badagliacca, R., primary, Poscia, R., additional, Pezzuto, B., additional, Papa, S., additional, Gambardella, C., additional, Francone, M., additional, Mezzapesa, M., additional, Nocioni, M., additional, Nona, A., additional, Rosati, R., additional, Sciomer, S., additional, Fedele, F., additional, and Vizza, C.D., additional

Published
2014
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31. Mode of Death in Patients with Pulmonary Arterial Hypertension

Author

Vizza, C.D., primary, Pezzuto, B., additional, Badagliacca, R., additional, Poscia, R., additional, Mezzapesa, M., additional, Nocioni, M., additional, D’Alto, M., additional, Ghio, S., additional, Vitulo, P., additional, Mulè, M., additional, Albera, C., additional, and Fedele, F., additional

Published
2013
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35. Pulmonary hemodynamics contribute to indicate priority for lung transplantation in patients with cystic fibrosis

Author

Venuta, F., Rendina, E.A., Rocca, G.D., De Giacomo, T., Pugliese, F., Ciccone, A.M., Vizza, C.D., Coloni, G.F., and Patterson, G.

Abstract

Objective: Lung transplantation is a viable option for patients with cystic fibrosis. The current strategy of selection, based on spirometry and deterioration of quality of life, results in a high mortality on the waiting list. We reviewed the case histories of patients with cystic fibrosis accepted for lung transplantation to ascertain whether pulmonary hemodynamics could contribute to predict life expectancy. Methods: Forty-five patients with cystic fibrosis were accepted: 11 died on the waiting list (group I), 24 underwent transplantation (group II), and 10 are still waiting (group III). During evaluation we recorded spirometry, oxygen requirement, ratio of arterial oxygen tension to inspired oxygen fraction (PaO"2/FIO"2), arterial carbon dioxide tension (PaCO"2), 6-minute walk test results, right ventricular ejection fraction, echocardiography, and pulmonary hemodynamics. We compared data from group I, II, and III patients. A comparison was also made within group II between the data collected at the time of evaluation and at the time of transplantation to quantify the deterioration during the waiting time. Results: The waiting time, spirometry, 6-minute walk test results, and right ventricular ejection fraction did not differ among the three groups. A statistically significant difference was found for PaO"2/FIO"2, PaCO"2, mean pulmonary artery pressure, cardiac index, pulmonary arterial wedge pressure, and intrapulmonary shunt between groups I and II. Groups I and III showed statistically significant differences for mean pulmonary artery pressure, PaO"2/FIO"2, and systemic vascular resistance indexed. No differences were observed between groups II and III. The comparison within group II showed a significant deterioration of pulmonary hemodynamics during the waiting time. Conclusions: Pulmonary hemodynamics are worst in patients dying on the waiting list and deteriorate significantly during the waiting time. They may thus contribute to establish priority for lung transplantation in patients with cystic fibrosis. (J Thorac Cardiovasc Surg 2000;119:682-9)

Published
2000
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36. Interleukin-32 in systemic sclerosis, a potential new biomarker for pulmonary arterial hypertension

Author

Onorina Berardicurti, Paola Di Benedetto, Ganna D. Radchenko, Roberto Badagliacca, Roberto Giacomelli, Noemi Panzera, Valeria Riccieri, Piero Ruscitti, Giovanna Manzi, Carmine Dario Vizza, Giuliana Guggino, Paola Cipriani, Nicolò Grazia, Vasiliki Liakouli, Francesco Ciccia, Di Benedetto P., Guggino G., Manzi G., Ruscitti P., Berardicurti O., Panzera N., Grazia N., Badagliacca R., Riccieri V., Vizza C.D., Radchenko G., Liakouli V., Ciccia F., Cipriani P., Giacomelli R., Di Benedetto, P., Guggino, G., Manzi, G., Ruscitti, P., Berardicurti, O., Panzera, N., Grazia, N., Badagliacca, R., Riccieri, V., Vizza, C. D., Radchenko, G., Liakouli, V., Ciccia, F., Cipriani, P., and Giacomelli, R.

Subjects
medicine.medical_specialty, lcsh:Diseases of the musculoskeletal system, Hypertension, Pulmonary, medicine.medical_treatment, 030204 cardiovascular system & hematology, Pulmonary arterial hypertension, Gastroenterology, Proinflammatory cytokine, Systemic sclerosi, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine.artery, systemic sclerosis, pulmonary arterial hypertension, IL-32, medicine, Humans, skin and connective tissue diseases, 030203 arthritis & rheumatology, Scleroderma, Systemic, integumentary system, business.industry, Interleukins, Rheumatology, Interleukin 32, Cytokine, medicine.anatomical_structure, Systemic sclerosis, Pulmonary artery, Vascular resistance, Biomarker (medicine), Immunohistochemistry, lcsh:RC925-935, business, Biomarkers, Research Article
Abstract

Background Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc), associated with a progressive elevation in pulmonary vascular resistance and subsequent right heart failure and death. Due to unspecific symptoms, the diagnosis of PAH is often delayed. On this basis, it is of great value to improve current diagnostic methods and develop new strategies for evaluating patients with suspected PAH. Interleukin-32 (IL-32) is a proinflammatory cytokine expressed in damaged vascular cells, and the present study aimed to assess if this cytokine could be a new biomarker of PAH during SSc. Methods The IL-32 expression was evaluated in the sera and skin samples of 18 SSc-PAH patients, 21 SSc patients without PAH, 15 patients with idiopathic PAH (iPAH) and 14 healthy controls (HCs), by enzyme-linked immunosorbent assay (ELISA) and immunohistochemistry (IHC). Receiver-operating characteristic (ROC) curves were performed to evaluate the cut-off of IL-32 in identifying patients with PAH. Furthermore, in SSc patients, correlation analyses were performed between IL-32 sera levels and mean pulmonary artery pressure (mPAP) evaluated by right heart catheterization (RHC) and systolic pulmonary artery pressure (sPAP), obtained by echocardiography. Additionally, the number of skin IL-32+ cells was correlated with modified Rodnan skin score (mRSS). Results In SSc-PAH patients, IL-32 sera levels were significantly higher when compared with SSc patients without PAH and patients affected by iPAH. The analysis of ROC curve showed that IL-32 sera levels above 11.12 pg/ml were able to predict patients with PAH (sensitivity = 90%, specificity = 100%). Furthermore, the IL-32 sera levels of patients with SSc correlated with both mPAP and sPAP. In the skin derived from SSc-PAH patients, the number of IL-32+ cells was significantly increased when compared with the skin derived from SSc patients without PAH, correlating with the mRSS. Conclusion Our study suggested that sera determination of IL-32 may be a promising approach to evaluate the presence of PAH in SSc patients and together with longitudinal future studies could help to increase the understanding how these biomarkers mirror the vascular changes and the inflammatory process during SSc.

Published
2020
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37. 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism developed in collaboration with the European Respiratory Society (ERS)

Author

Konstantinides, Stavros V., Meyer, Guy, Becattini, Cecilia, Bueno, Hector, Geersing, Geert-Jan, Harjola, Veli-Pekka, Huisman, Menno V., Humbert, Marc, Jennings, Catriona Sian, Jimenez, David, Kucher, Nils, Lang, Irene Marthe, Lankeit, Mareike, Lorusso, Roberto, Mazzolai, Lucia, Meneveau, Nicolas, Ni Ainle, Fionnuala, Prandoni, Paolo, Pruszczyk, Piotr, Righini, Marc, Torbicki, Adam, Van Belle, Eric, Luis Zamorano, Jose, Nazzareno, Galié, J Simon, R Gibbs, Victor, Aboyans, Walter, Ageno, Stefan, Agewall, Ana, G Almeida, Felicita, Andreotti, Emanuele, Barbato, Johann, Bauersachs, Andreas, Baumbach, Farzin, Beygui, Jørn, Carlsen, Marco De Carlo, Marion, Delcroix, Victoria, Delgado, Pilar Escribano Subias, Donna, Fitzsimons, Sean, Gaine, Samuel, Z Goldhaber, Deepa, Gopalan, Gilbert, Habib, Sigrun, Halvorsen, David, Jenkins, Hugo, A Katus, Barbro, Kjellström, Mitja, Lainscak, Patrizio, Lancellotti, Geraldine, Lee, Grégoire Le Gal, Emmanuel, Messas, Joao, Morais, Steffen, E Petersen, Anna Sonia Petronio, Massimo Francesco Piepoli, Susanna, Price, Marco, Roffi, Aldo, Salvi, Olivier, Sanchez, Evgeny, Shlyakhto, Iain, A Simpson, Stefan, Stortecky, Matthias, Thielmann, Anton Vonk Noordegraaf, Cecilia, Becattini, Héctor, Bueno, Geert-Jan, Geersing, Veli-Pekka, Harjola, Menno, V Huisman, Marc, Humbert, Catriona Sian Jennings, David, Jiménez, Nils, Kucher, Irene Marthe Lang, Mareike, Lankeit, Roberto, Lorusso, Lucia, Mazzolai, Nicolas, Meneveau, Fionnuala Ní Áinle, Paolo, Prandoni, Piotr, Pruszczyk, Marc, Righini, Adam, Torbicki, Eric, Vanbelle, José, Luiszamorano, Stephan, Windecker, Colin, Baigent, Jean-Philippe, Collet, Veronica, Dean, Chris, P Gale, Diederick, Grobbee, Gerhard, Hindricks, Bernard, Iung, Peter, Jüni, Ulf, Landmesser, Christophe, Leclercq, Maddalena, Lettino, Basil, S Lewis, Bela, Merkely, Christian, Mueller, Dimitrios, J Richter, Miguel, Sousa-Uva, Rhian, M Touyz, Naima, Hammoudi, Hamlet, Hayrapetyan, Julia, Mascherbauer, Firdovsi, Ibrahimov, Oleg, Polonetsky, Mariya, Tokmakova, Bosko, Skoric, Ioannis, Michaloliakos, Martin, Hutyra, Søren, Mellemkjaer, Mansour, Mostafa, Julia, Reinmets, Pertti, Jääskeläinen, Denis, Angoulvant, George, Giannakoulas, Endre, Zima, Vizza, Carmine Dario, Akhmetzhan, Sugraliyev, Ibadete, Bytyçi, Aija, Maca, Egle, Ereminiene, Steve, Huijnen, Robert, Xuereb, Nadejda, Diaconu, Nebojsa, Bulatovic, Ilyasse, Asfalou, Marijan, Bosevski, Bożena, Sobkowicz, Daniel, Ferreira, Antoniu Octavian Petris, Olga, Moiseeva, Marco, Zavatta, Slobodan, Obradovic, Iveta, Šimkova, Peter, Radsel, Borja, Ibanez, Gerhard, Wikström, Drahomir, Aujesky, Cihangir, Kaymaz, Alexander, Parkhomenko, Joanna, Pepke-Zaba, University of Zurich, Konstantinides, Stavros V, Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Institute of cardiometabolism and nutrition (ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Konstantinides S.V., Meyer G., Bueno H., Galie N., Gibbs J.S.R., Ageno W., Agewall S., Almeida A.G., Andreotti F., Barbato E., Baumbach A., Beygui F., Carlsen J., De Carlo M., Delcroix M., Subias P.E., Gaine S., Goldhaber S.Z., Gopalan D., Habib G., Jenkins D., Kjellstrom B., Lainscak M., Lee G., Le Gal G., Messas E., Morais J., Piepoli M.F., Price S., Salvi A., Sanchez O., Stortecky S., Thielmann M., Noordegraaf A.V., Becattini C., Geersing G.-J., Harjola V.-P., Huisman M.V., Humbert M., Jennings C.S., Jimenez D., Kucher N., Lang I., Lankeit M., Lorusso R., Mazzolai L., Meneveau N., Ainle F.N., Prandoni P., Pruszczyk P., Righini M., Torbicki A., Van Belle E., Zamorano J.L., Windecker S., Aboyans V., Baigent C., Collet J.-P., Dean V., Delgado V., Fitzsimons D., Gale C.P., Grobbee D.E., Hindricks G., Iung B., Juni P., Katus H.A., Landmesser U., Leclercq C., Lettino M., Lewis B.S., Merkely B., Mueller C., Petersen S.E., Petronio A.S., Richter D.J., Roffi M., Shlyakhto E., Simpson I.A., Sousa-Uva M., Touyz R.M., Hammoudi N., Hayrapetyan H., Mascherbauer J., Ibrahimov F., Polonetsky O., Lancellotti P., Tokmakova M., Skoric B., Michaloliakos I., Hutyra M., Mellemkjaer S., Mansour M., Reinmets J., Jaaskelainen P., Angoulvant D., Bauersachs J., Giannakoulas G., Zima E., Vizza C.D., Sugraliyev A., Bytyci I., Maca A., Ereminiene E., Huijnen S., Xuereb R., Diaconu N., Bulatovic N., Asfalou I., Bosevski M., Halvorsen S., Sobkowicz B., Ferreira D., Petris A.O., Moiseeva O., Zavatta M., Obradovic S., Simkova I., Radsel P., Ibanez B., Wikstrom G., Aujesky D., Kaymaz C., Parkhomenko A., Pepke-Zaba J., CTC, MUMC+: MA Med Staf Spec CTC (9), and RS: Carim - V04 Surgical intervention

Subjects
ORAL ANTICOAGULANT-THERAPY, diagnosis, [SDV]Life Sciences [q-bio], Medizin, 030204 cardiovascular system & hematology, Embolectomy, Guideline, RECURRENT VENOUS THROMBOEMBOLISM, 0302 clinical medicine, Pregnancy, Daily practice, Diagnosis, Pulmonary medicine, Venous thrombosis, Pulmonary Medicine, Thrombolytic Therapy, DEEP-VEIN THROMBOSIS, Disease management (health), Societies, Medical, ComputingMilieux_MISCELLANEOUS, health care economics and organizations, Risk assessment, ddc:616, RIGHT-VENTRICULAR DYSFUNCTION, Disease Management, Shock, MOLECULAR-WEIGHT HEPARIN, Thrombolysis, humanities, 3. Good health, Pulmonary embolism, Europe, Anticoagulation, Biomarkers, Dyspnoea, Echocardiography, Guidelines, Heart failure, Right ventricle, Treatment, Venous thromboembolism, medicine.vein, Acute Disease, Medical emergency, Cardiology and Cardiovascular Medicine, guidelines, pulmonary embolism, venous thrombosis, shock dyspnoea, heart failure: right ventricle: diagnosis, risk assessment: echocardiography, biomarkers, treatment, anticoagulation, thrombolysis, pregnancy, venous thromboembolism, embolectomy, Diagnosi, education, Cardiology, MEDLINE, Thrombolysi, 610 Medicine & health, Inferior vena cava, 2705 Cardiology and Cardiovascular Medicine, EXTRACORPOREAL MEMBRANE-OXYGENATION, 03 medical and health sciences, Medical, medicine, Humans, RIGHT HEART THROMBI, VENTILATION-PERFUSION SCINTIGRAPHY, Health professionals, business.industry, INFERIOR VENA-CAVA, 10031 Clinic for Angiology, Anticoagulants, 030229 sport sciences, Biomarker, medicine.disease, INHALED NITRIC-OXIDE, Societies, business
Abstract

Guidelines summarize and evaluate available evidence with the aim of assisting health professionals in proposing the best management strategies for an individual patient with a given condition. Guidelines and their recommendations should facilitate decision making of health professionals in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.

Published
2019
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