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

Author: Hoeper, Marius M., Gomez Sanchez, Miguel Angel, Humbert, Marc, Pittrow, David, Simonneau, Gerald, Gall, Henning, Grünig, Ekkehard, Klose, Hans, Halank, M., Langleben, David, Snijder, Repke R.J., Escribano Subias, Pilar, Mielniczuk, L., Lange, Tobias Johannes, Vachiery, Jean-Luc, Wirtz, Hubert, Helmersen, Doug D.S., Tsangaris, Iraklis, Barberà, Joan Albert, Pepke-Zaba, Joanna, Boonstra, Anco, Rosenkranz, Stephan, Ulrich, Silvia, Steringer-Mascherbauer, Regina, Delcroix, Marion, Jansa, Pavel, Šimková, Iveta, Giannakoulas, George, Klotsche, Jens, Williams, Evgenia, Meier, Christian, Ghofrani, Hossein Ardeschir, Hoeper, Marius M., Gomez Sanchez, Miguel Angel, Humbert, Marc, Pittrow, David, Simonneau, Gerald, Gall, Henning, Grünig, Ekkehard, Klose, Hans, Halank, M., Langleben, David, Snijder, Repke R.J., Escribano Subias, Pilar, Mielniczuk, L., Lange, Tobias Johannes, Vachiery, Jean-Luc, Wirtz, Hubert, Helmersen, Doug D.S., Tsangaris, Iraklis, Barberà, Joan Albert, Pepke-Zaba, Joanna, Boonstra, Anco, Rosenkranz, Stephan, Ulrich, Silvia, Steringer-Mascherbauer, Regina, Delcroix, Marion, Jansa, Pavel, Šimková, Iveta, Giannakoulas, George, Klotsche, Jens, Williams, Evgenia, Meier, Christian, and Ghofrani, Hossein Ardeschir
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., SCOPUS: ar.j, info:eu-repo/semantics/published
Published: 2021

152. Cardiac Rehabilitation in German Speaking Countries of Europe—Evidence-Based Guidelines from Germany, Austria and Switzerland LLKardReha-DACH—Part 1

Author: Rauch, Bernhard; https://orcid.org/0000-0003-2391-8466, Salzwedel, Annett; https://orcid.org/0000-0003-2530-2673, Bjarnason-Wehrens, Birna, Albus, Christian, Meng, Karin, Schmid, Jean-Paul; https://orcid.org/0000-0002-9008-8274, Benzer, Werner, Hackbusch, Matthes, Jensen, Katrin, Schwaab, Bernhard; https://orcid.org/0000-0003-4801-1107, Altenberger, Johann, Benjamin, Nicola, Bestehorn, Kurt, Bongarth, Christa, Dörr, Gesine, Eichler, Sarah, Einwang, Hans-Peter, Falk, Johannes, Glatz, Johannes, Gielen, Stephan, Grilli, Maurizio; https://orcid.org/0000-0001-7649-7242, Grünig, Ekkehard, Guha, Manju, Hermann, Matthias; https://orcid.org/0000-0002-0694-7719, Hoberg, Eike, Höfer, Stefan; https://orcid.org/0000-0002-6128-3687, Kaemmerer, Harald, Ladwig, Karl-Heinz, Mayer-Berger, Wolfgang, Metzendorf, Maria-Inti, et al, von Känel, Roland; https://orcid.org/0000-0002-8929-5129, The Cardiac Rehabilitation Guideline Group, Rauch, Bernhard; https://orcid.org/0000-0003-2391-8466, Salzwedel, Annett; https://orcid.org/0000-0003-2530-2673, Bjarnason-Wehrens, Birna, Albus, Christian, Meng, Karin, Schmid, Jean-Paul; https://orcid.org/0000-0002-9008-8274, Benzer, Werner, Hackbusch, Matthes, Jensen, Katrin, Schwaab, Bernhard; https://orcid.org/0000-0003-4801-1107, Altenberger, Johann, Benjamin, Nicola, Bestehorn, Kurt, Bongarth, Christa, Dörr, Gesine, Eichler, Sarah, Einwang, Hans-Peter, Falk, Johannes, Glatz, Johannes, Gielen, Stephan, Grilli, Maurizio; https://orcid.org/0000-0001-7649-7242, Grünig, Ekkehard, Guha, Manju, Hermann, Matthias; https://orcid.org/0000-0002-0694-7719, Hoberg, Eike, Höfer, Stefan; https://orcid.org/0000-0002-6128-3687, Kaemmerer, Harald, Ladwig, Karl-Heinz, Mayer-Berger, Wolfgang, Metzendorf, Maria-Inti, et al, von Känel, Roland; https://orcid.org/0000-0002-8929-5129, and The Cardiac Rehabilitation Guideline Group
Abstract: Background: Although cardiovascular rehabilitation (CR) is well accepted in general, CR-attendance and delivery still considerably vary between the European countries. Moreover, clinical and prognostic effects of CR are not well established for a variety of cardiovascular diseases. Methods: The guidelines address all aspects of CR including indications, contents and delivery. By processing the guidelines, every step was externally supervised and moderated by independent members of the “Association of the Scientific Medical Societies in Germany” (AWMF). Four meta-analyses were performed to evaluate the prognostic effect of CR after acute coronary syndrome (ACS), after coronary bypass grafting (CABG), in patients with severe chronic systolic heart failure (HFrEF), and to define the effect of psychological interventions during CR. All other indications for CR-delivery were based on a predefined semi-structured literature search and recommendations were established by a formal consenting process including all medical societies involved in guideline generation. Results: Multidisciplinary CR is associated with a significant reduction in all-cause mortality in patients after ACS and after CABG, whereas HFrEF-patients (left ventricular ejection fraction <40%) especially benefit in terms of exercise capacity and health-related quality of life. Patients with other cardiovascular diseases also benefit from CR-participation, but the scientific evidence is less clear. There is increasing evidence that the beneficial effect of CR strongly depends on “treatment intensity” including medical supervision, treatment of cardiovascular risk factors, information and education, and a minimum of individually adapted exercise volume. Additional psychologic interventions should be performed on the basis of individual needs. Conclusions: These guidelines reinforce the substantial benefit of CR in specific clinical indications, but also describe remaining deficits in CR-delivery in clinical pr
Published: 2021

153. Treatment of Pulmonary Arterial Hypertension in Connective Tissue Disease

Author: Grünig, Ekkehard
Published: 2012
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154. Author Correction:Therapeutic potential of KLF2-induced exosomal microRNAs in pulmonary hypertension (Nature Communications, (2020), 11, 1, (1185), 10.1038/s41467-020-14966-x)

Author: Sindi, Hebah A., Russomanno, Giusy, Satta, Sandro, Abdul-Salam, Vahitha B., Jo, Kyeong Beom, Qazi-Chaudhry, Basma, Ainscough, Alexander J., Szulcek, Robert, Bogaard, Harm Jan, Morgan, Claire C., Pullamsetti, Soni S., Alzaydi, Mai M., Rhodes, Christopher J., Piva, Roberto, Eichstaedt, Christina A., Grünig, Ekkehard, Wilkins, Martin R., and Wojciak-Stothard, Beata
Abstract: An amendment to this paper has been published and can be accessed via a link at the top of the paper.
Published: 2020

155. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study

Author: Coghlan, J Gerry, Denton, Christopher P, Grünig, Ekkehard, Bonderman, Diana, Distler, Oliver, Khanna, Dinesh, Müller-Ladner, Ulf, Pope, Janet E, Vonk, Madelon C, Doelberg, Martin, Chadha-Boreham, Harbajan, Heinzl, Harald, Rosenberg, Daniel M, McLaughlin, Vallerie V, and Seibold, James R
Published: 2014
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156. Echocardiography of the Pulmonary Circulation and Right Ventricular Function: Exploring the Physiologic Spectrum in 1,480 Normal Subjects

Author: D’Andrea, Antonello, Naeije, Robert, Grünig, Ekkehard, Caso, Pio, D’Alto, Michele, Di Palma, Enza, Nunziata, Luigi, Riegler, Lucia, Scarafile, Raffaella, Cocchia, Rosangela, Vriz, Olga, Citro, Rodolfo, Calabrò, Raffaele, Russo, Maria Giovanna, and Bossone, Eduardo
Published: 2014
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157. Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry

Author: Ghofrani, Hossein-Ardeschir, primary, Gomez Sanchez, Miguel-Angel, additional, Humbert, Marc, additional, Pittrow, David, additional, Simonneau, Gérald, additional, Gall, Henning, additional, Grünig, Ekkehard, additional, Klose, Hans, additional, Halank, Michael, additional, Langleben, David, additional, Snijder, Repke J., additional, Escribano Subias, Pilar, additional, Mielniczuk, Lisa M., additional, Lange, Tobias J., additional, Vachiéry, Jean-Luc, additional, Wirtz, Hubert, additional, Helmersen, Douglas S., additional, Tsangaris, Iraklis, additional, Barberá, Joan A., additional, Pepke-Zaba, Joanna, additional, Boonstra, Anco, additional, Rosenkranz, Stephan, additional, Ulrich, Silvia, additional, Steringer-Mascherbauer, Regina, additional, Delcroix, Marion, additional, Jansa, Pavel, additional, Šimková, Iveta, additional, Giannakoulas, George, additional, Klotsche, Jens, additional, Williams, Evgenia, additional, Meier, Christian, additional, Hoeper, Marius M., additional, Caneva, Jorge, additional, Tuhay, Graciela, additional, Diez, Mirta, additional, Talavera, Maria Lujan, additional, Acosta, Adriana, additional, Vulcano, Norberto, additional, Bosio, Martin, additional, Maldonado, Lorena, additional, Deleo, Sabino, additional, Melatini, Luciano, additional, Keogh, Anne, additional, Kotlyar, Eugene, additional, Feenstra, John, additional, Dwyer, Nathan, additional, Adams, Heath, additional, Stevens, Wendy, additional, Steele, Peter, additional, Proudman, Susanna, additional, Minson, Robert, additional, Reeves, Glenn, additional, Lavender, Melanie, additional, Ng, Benjamin, additional, Mackenzie, Michele, additional, Barry, Lisa, additional, Gruenberger, Margarethe, additional, Huber, Charlotte, additional, Lang, Irene, additional, Tilea, Ioana, additional, Sadushi-Kolici, Roela, additional, Löffler-Ragg, Judith, additional, Feistmantl, Lisa-Theresa, additional, Evrard, Patrick, additional, Louis, Renaud, additional, Guiot, Julien, additional, Naldi, Marco, additional, De Pauw, Michel, additional, Mehta, Sanjay, additional, Camacho, Rafael Conde, additional, Tovar, Patricia Parada, additional, Londoño, Alejandro, additional, Campo, Felipe, additional, Garcia, Paula, additional, Lema, Camila, additional, Orozco-Levi, Mauricio, additional, Martinez, William, additional, Gomez, Juan Esteban, additional, Nielsen-Kudsk, Jens Erik, additional, Mellemkjaer, Soren, additional, Anton, Ly, additional, Altraja, Alan, additional, Vihinen, Tapani, additional, Vasankari, Tuija, additional, Sitbon, Olivier, additional, Cottin, Vincent, additional, Têtu, Laurent, additional, Noël-Savina, Elise, additional, Shearman, Nicole, additional, Tayler, Susanne, additional, Olzik, Ilona, additional, Kulka, Christine, additional, Grimminger, Jan, additional, Simon, Marcel, additional, Nolde, Anna, additional, Oqueka, Tim, additional, Harbaum, Lars, additional, Egenlauf, Benjamin, additional, Ewert, Ralf, additional, Schulz, Christian, additional, Regotta, Sabine, additional, Kramer, Tilmann, additional, Knoop-Busch, Susanne, additional, Gerhardt, Felix, additional, Konstantinides, Stavros, additional, Pitsiou, Georgia, additional, Stanopoulos, Ioannis, additional, Sourla, Evdokia, additional, Mouratoglou, Sofia, additional, Karvounis, Haralambos, additional, Pappas, Athanasios, additional, Georgopoulos, Dimitrios, additional, Fanaridis, Michail, additional, Mitrouska, Ioanna, additional, Michalis, Lampros, additional, Pappas, Konstantinos, additional, Kotsia, Anna, additional, Gaine, Sean, additional, Vizza, Carmine Dario, additional, Manzi, Giovanna, additional, Poscia, Roberto, additional, Badagliacca, Roberto, additional, Agostoni, Piergiuseppe, additional, Bruno, Noemi, additional, Farina, Stefania, additional, D'Alto, Michele, additional, Argiento, Paola, additional, Correra, Anna, additional, Di Marco, Giovanni Maria, additional, Cresci, Chiara, additional, Vannucchi, Vieri, additional, Torricelli, Elena, additional, Garcea, Alessio, additional, Pesci, Alberto, additional, Sardella, Luca, additional, Paciocco, Giuseppe, additional, Pane, Federico, additional, D'Armini, Andrea Maria, additional, Pin, Maurizio, additional, Grazioli, Valentina, additional, Massola, Giulia, additional, Sciortino, Antonio, additional, Prediletto, Renato, additional, Bauleo, Carolina, additional, Airò, Edoardo, additional, Ndreu, Rudina, additional, Pavlickova, Ivana, additional, Lunardi, Claudio, additional, Mulè, Massimiliano, additional, Farruggio, Silvia, additional, Costa, Serena, additional, Galgano, Giuseppe, additional, Petruzzi, Mario, additional, De Luca, Anna, additional, Lombardi, Francesco, additional, Roncon, Loris, additional, Conte, Luca, additional, Picariello, Claudio, additional, Wirtz, Gil, additional, Alexandre, Myriam, additional, Vonk-Noordegraaf, A., additional, Boogaard, H., additional, Mager, J., additional, Reesink, H., additional, van den Toorn, Leon M., additional, Boomars, Karin, additional, Andreassen, Arne K., additional, Castro, Graça, additional, Tania, Gonçalves, additional, Baptista, Rui, additional, Marinho, António, additional, Shiang, Teresa, additional, Oliveira, Ana, additional, Coutinho, Daniel, additional, Sousa, Joana, additional, Loureiro, Maria José, additional, Repolho, Débora, additional, Martins Jesus, Susana Maria, additional, Capinha, Marta, additional, Agostinho, João, additional, Cardoso, Tania, additional, Rocha, Andreia, additional, Espinha, Mafalda, additional, Ivanov, Kyundyul Ivanovich, additional, Alexeeva, Dalyana Eduardovna, additional, Batalina, Marina Vadimovna, additional, Hegya, Daria Viktorovna, additional, Zvereva, Tatyana Nikolaevna, additional, Avdeev, Sergey Nikolaevich, additional, Tsareva, Natalia Anatolievna, additional, Galyavich, Albert Sarvatovich, additional, Nikolaevich, Bykov Aleksander, additional, Filippov, Evgeny Vladimirovich, additional, Yakovleva, Olga Eduardovna, additional, Pavlova, Olga Borisovna, additional, Skripkina, Elena Sergeevna, additional, Martynyuk, Tamila Vitalievna, additional, Bukatova, Irina Fedorovna, additional, Tregubova, Anna Viktorovna, additional, Platonov, Dmitry Yurievich, additional, Kolomeytseva, Tatyana Mikhaylovna, additional, Al Dalaan, Abdullah, additional, Abdelsayed, Abeer Abeer, additional, Weheba, Ihab, additional, Saleemi, Sarferaz, additional, Sakkijha, Hussam, additional, Bohacekova, Marcela, additional, Valkovicova, Tatiana, additional, Farkasova, Iveta, additional, Quezada, Carlos Andres, additional, Piccari, Lucilla, additional, Blanco, Isabel, additional, Sebastian, Laura, additional, Roman, Antonio, additional, Lopez, Manuel, additional, Otero, Remedios, additional, Elias, Teresa, additional, Jara, Luis, additional, Asencio, Isabel, additional, Arjona, Josefa Jiménez, additional, Almagro, Raúl Menor, additional, Cárdenas, Salvador López, additional, García, Salvador Alcaraz, additional, Rodríguez, Patricia Villanueva, additional, Lopez, Raquel, additional, Garcia, Alberto, additional, Avilés, Francisco Fernandez, additional, De La Pava, Sebastian, additional, Yotti, Raquel, additional, Peñate, Gregorio Pérez, additional, Marrero, Fernando León, additional, Cifrián Martínez, José Manuel, additional, Martinez-Meñaca, Amaya, additional, Alonso, Lecue Pilar, additional, Rozas, Sonia Fernandez, additional, Fernandez, David Iturbe, additional, Cuesta, Victor Mora, additional, Söderberg, Stefan, additional, Bartfay, Sven-Erik, additional, Rundqvist, Bengt, additional, Alfetlawi, Monthir, additional, Wodlin, Peter, additional, Schwarz, Esther Irene, additional, Speich, Rudolf, additional, Lador, Frédéric, additional, Rochat, Thierry, additional, Gasche-Soccal, Paola, additional, Hsu, Chih-Hsin, additional, Lin, Tsung-Hsien, additional, Su, Ho-Ming, additional, Lai, Wen-Ter, additional, Chu, Chun Yuan, additional, Hsu, Po-Chao, additional, Voon, Wen-Chol, additional, Yen, Hsueh-Wei, additional, Yih-Jer Wu, Jacob, additional, Wu, Shu-Hao, additional, Huang, Wen-Pin, additional, Fong, Man-Cai, additional, Huang, Chien-Lung, additional, Kuo, Ping-Hung, additional, Lin, Yen-Hung, additional, Lin, Jiunn-Lee, additional, Hung, Chi-Sheng, additional, Wu, Cho-Kai, additional, Sung, Shih-Hsien, additional, Huang, Wei-Chun, additional, Cheng, Chin-Chang, additional, Kuo, Shu-Hung, additional, Wang, Wen-Hwa, additional, Ho, Wan-Jing, additional, Hsu, Tsu-Shiu, additional, Mutlu, Bülent, additional, Atas, Halil, additional, Ongen, Gul, additional, Un, Zeynep, additional, Okumus, Gulfer, additional, Hanta, Ismail, additional, Corris, Paul, additional, Peacock, Andrew, additional, Church, Colin, additional, Toshner, Mark, additional, and Newnham, Michael, additional
Published: 2021
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158. Riociguat treatment in patients with pulmonary arterial hypertension: Final safety data from the EXPERT registry

Author: Hoeper, Marius M., primary, Gomez Sanchez, Miguel-Angel, additional, Humbert, Marc, additional, Pittrow, David, additional, Simonneau, Gérald, additional, Gall, Henning, additional, Grünig, Ekkehard, additional, Klose, Hans, additional, Halank, Michael, additional, Langleben, David, additional, Snijder, Repke J., additional, Escribano Subias, Pilar, additional, Mielniczuk, Lisa M., additional, Lange, Tobias J., additional, Vachiéry, Jean-Luc, additional, Wirtz, Hubert, additional, Helmersen, Douglas S., additional, Tsangaris, Iraklis, additional, Barberà, Joan A., additional, Pepke-Zaba, Joanna, additional, Boonstra, Anco, additional, Rosenkranz, Stephan, additional, Ulrich, Silvia, additional, Steringer-Mascherbauer, Regina, additional, Delcroix, Marion, additional, Jansa, Pavel, additional, Šimková, Iveta, additional, Giannakoulas, George, additional, Klotsche, Jens, additional, Williams, Evgenia, additional, Meier, Christian, additional, Ghofrani, Hossein-Ardeschir, additional, Caneva, Jorge, additional, Tuhay, Graciela, additional, Diez, Mirta, additional, Talavera, Maria Lujan, additional, Acosta, Adriana, additional, Vulcano, Norberto, additional, Bosio, Martin, additional, Maldonado, Lorena, additional, Deleo, Sabino, additional, Melatini, Luciano, additional, Keogh, Anne, additional, Kotlyar, Eugene, additional, Feenstra, John, additional, Dwyer, Nathan, additional, Adams, Heath, additional, Stevens, Wendy, additional, Steele, Peter, additional, Proudman, Susanna, additional, Minson, Robert, additional, Reeves, Glenn, additional, Lavender, Melanie, additional, Ng, Benjamin, additional, Mackenzie, Michele, additional, Barry, Lisa, additional, Gruenberger, Margarethe, additional, Huber, Charlotte, additional, Lang, Irene, additional, Tilea, Ioana, additional, Sadushi-Kolici, Roela, additional, Löffler-Ragg, Judith, additional, Feistmantl, Lisa-Theresa, additional, Evrard, Patrick, additional, Guiot, Julien, additional, Naldi, Marco, additional, De Pauw, Michel, additional, Louis, Renaud, additional, Mehta, Sanjay, additional, Camacho, Rafael Conde, additional, Tovar, Patricia Parada, additional, Londoño, Alejandro, additional, Campo, Felipe, additional, Garcia, Paula, additional, Lema, Camila, additional, Orozco-Levi, Mauricio, additional, Martinez, William, additional, Gomez, Juan Esteban, additional, Nielsen-Kudsk, Jens Erik, additional, Mellemkjaer, Soren, additional, Anton, Ly, additional, Altraja, Alan, additional, Vihinen, Tapani, additional, Vasankari, Tuija, additional, Sitbon, Olivier, additional, Cottin, Vincent, additional, Têtu, Laurent, additional, Noël-Savina, Elise, additional, Shearman, Nicole, additional, Tayler, Susanne, additional, Olzik, Ilona, additional, Kulka, Christine, additional, Grimminger, Jan, additional, Simon, Marcel, additional, Nolde, Anna, additional, Oqueka, Tim, additional, Harbaum, Lars, additional, Egenlauf, Benjamin, additional, Ewert, Ralf, additional, Schulz, Christian, additional, Regotta, Sabine, additional, Kramer, Tilmann, additional, Knoop-Busch, Susanne, additional, Gerhardt, Felix, additional, Konstantinides, Stavros, additional, Pitsiou, Georgia, additional, Stanopoulos, Ioannis, additional, Sourla, Evdokia, additional, Mouratoglou, Sofia, additional, Karvounis, Haralambos, additional, Pappas, Athanasios, additional, Mitrouska, Ioanna, additional, Georgopoulos, Dimitrios, additional, Fanaridis, Michail, additional, Michalis, Lampros, additional, Pappas, Konstantinos, additional, Kotsia, Anna, additional, Gaine, Sean, additional, Vizza, Carmine Dario, additional, Manzi, Giovanna, additional, Poscia, Roberto, additional, Badagliacca, Roberto, additional, Agostoni, Piergiuseppe, additional, Bruno, Noemi, additional, Farina, Stefania, additional, D'Alto, Michele, additional, Argiento, Paola, additional, Correra, Anna, additional, Di Marco, Giovanni Maria, additional, Cresci, Chiara, additional, Vannucchi, Vieri, additional, Torricelli, Elena, additional, Garcea, Alessio, additional, Pesci, Alberto, additional, Sardella, Luca, additional, Paciocco, Giuseppe, additional, Pane, Federico, additional, D'Armini, Andrea Maria, additional, Pin, Maurizio, additional, Grazioli, Valentina, additional, Massola, Giulia, additional, Sciortino, Antonio, additional, Prediletto, Renato, additional, Bauleo, Carolina, additional, Airò, Edoardo, additional, Ndreu, Rudina, additional, Pavlickova, Ivana, additional, Lunardi, Claudio, additional, Farruggio, Silvia, additional, Costa, Serena, additional, Mulè, Massimiliano, additional, Galgano, Giuseppe, additional, Petruzzi, Mario, additional, De Luca, Anna, additional, Lombardi, Francesco, additional, Roncon, Loris, additional, Conte, Luca, additional, Picariello, Claudio, additional, Wirtz, Gil, additional, Alexandre, Myriam, additional, Vonk-Noordegraaf, A., additional, Boogaard, H., additional, Mager, J., additional, Reesink, H., additional, van den Toorn, Leon M., additional, Boomars, Karin, additional, Andreassen, Arne K., additional, Castro, Graça, additional, Tania, Gonçalves, additional, Baptista, Rui, additional, Marinho, António, additional, Shiang, Teresa, additional, Oliveira, Ana, additional, Coutinho, Daniel, additional, Sousa, Joana, additional, Loureiro, Maria José, additional, Repolho, Débora, additional, Martins Jesus, Susana Maria, additional, Capinha, Marta, additional, Agostinho, João, additional, Cardoso, Tania, additional, Rocha, Andreia, additional, Espinha, Mafalda, additional, Ivanov, Kyundyul Ivanovich, additional, Alexeeva, Dalyana Eduardovna, additional, Batalina, Marina Vadimovna, additional, Hegya, Daria Viktorovna, additional, Zvereva, Tatyana Nikolaevna, additional, Avdeev, Sergey Nikolaevich, additional, Tsareva, Natalia Anatolievna, additional, Galyavich, Albert Sarvatovich, additional, Nikolaevich, Bykov Aleksander, additional, Filippov, Evgeny Vladimirovich, additional, Yakovleva, Olga Eduardovna, additional, Pavlova, Olga Borisovna, additional, Skripkina, Elena Sergeevna, additional, Martynyuk, Tamila Vitalievna, additional, Bukatova, Irina Fedorovna, additional, Tregubova, Anna Viktorovna, additional, Platonov, Dmitry Yurievich, additional, Kolomeytseva, Tatyana Mikhaylovna, additional, Al Dalaan, Abdullah, additional, Abdelsayed, Abeer Abeer, additional, Weheba, Ihab, additional, Saleemi, Sarferaz, additional, Sakkijha, Hussam, additional, Bohacekova, Marcela, additional, Valkovicova, Tatiana, additional, Farkasova, Iveta, additional, Quezada, Carlos Andres, additional, Piccari, Lucilla, additional, Blanco, Isabel, additional, Sebastian, Laura, additional, Roman, Antonio, additional, Lopez, Manuel, additional, Otero, Remedios, additional, Elias, Teresa, additional, Jara, Luis, additional, Asencio, Isabel, additional, Arjona, Josefa Jiménez, additional, Almagro, Raúl Menor, additional, Cárdenas, Salvador López, additional, García, Salvador Alcaraz, additional, Rodríguez, Patricia Villanueva, additional, Lopez, Raquel, additional, Garcia, Alberto, additional, Avilés, Francisco Fernandez, additional, De La Pava, Sebastian, additional, Yotti, Raquel, additional, Peñate, Gregorio Pérez, additional, Marrero, Fernando León, additional, Cifrián Martínez, José Manuel, additional, Martinez-Meñaca, Amaya, additional, Alonso, Lecue Pilar, additional, Rozas, Sonia Fernandez, additional, Fernandez, David Iturbe, additional, Cuesta, Victor Mora, additional, Söderberg, Stefan, additional, Bartfay, Sven-Erik, additional, Rundqvist, Bengt, additional, Alfetlawi, Monthir, additional, Wodlin, Peter, additional, Schwarz, Esther Irene, additional, Speich, Rudolf, additional, Lador, Frédéric, additional, Rochat, Thierry, additional, Gasche-Soccal, Paola, additional, Hsu, Chih-Hsin, additional, Lin, Tsung-Hsien, additional, Su, Ho-Ming, additional, Lai, Wen-Ter, additional, Chu, Chun Yuan, additional, Hsu, Po-Chao, additional, Voon, Wen-Chol, additional, Yen, Hsueh-Wei, additional, Yih-Jer Wu, Jacob, additional, Wu, Shu-Hao, additional, Huang, Wen-Pin, additional, Fong, Man-Cai, additional, Huang, Chien-Lung, additional, Kuo, Ping-Hung, additional, Lin, Yen-Hung, additional, Lin, Jiunn-Lee, additional, Hung, Chi-Sheng, additional, Wu, Cho-Kai, additional, Sung, Shih-Hsien, additional, Huang, Wei-Chun, additional, Cheng, Chin-Chang, additional, Kuo, Shu-Hung, additional, Wang, Wen-Hwa, additional, Ho, Wan-Jing, additional, Hsu, Tsu-Shiu, additional, Mutlu, Bülent, additional, Atas, Halil, additional, Ongen, Gul, additional, Un, Zeynep, additional, Okumus, Gulfer, additional, Hanta, Ismail, additional, Corris, Paul, additional, Peacock, Andrew, additional, Church, Colin, additional, Toshner, Mark, additional, and Newnham, Michael, additional
Published: 2021
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159. Bayesian Inference Associates Rare KDR Variants With Specific Phenotypes in Pulmonary Arterial Hypertension

Author: Swietlik, Emilia M., primary, Greene, Daniel, additional, Zhu, Na, additional, Megy, Karyn, additional, Cogliano, Marcella, additional, Rajaram, Smitha, additional, Pandya, Divya, additional, Tilly, Tobias, additional, Lutz, Katie A., additional, Welch, Carrie C.L., additional, Pauciulo, Michael W., additional, Southgate, Laura, additional, Martin, Jennifer M., additional, Treacy, Carmen M., additional, Penkett, Christopher J., additional, Stephens, Jonathan C., additional, Bogaard, Harm J., additional, Church, Colin, additional, Coghlan, Gerry, additional, Coleman, Anna W., additional, Condliffe, Robin, additional, Eichstaedt, Christina A., additional, Eyries, Mélanie, additional, Gall, Henning, additional, Ghio, Stefano, additional, Girerd, Barbara, additional, Grünig, Ekkehard, additional, Holden, Simon, additional, Howard, Luke, additional, Humbert, Marc, additional, Kiely, David G., additional, Kovacs, Gabor, additional, Lordan, Jim, additional, Machado, Rajiv D., additional, MacKenzie Ross, Robert V., additional, McCabe, Colm, additional, Moledina, Shahin, additional, Montani, David, additional, Olschewski, Horst, additional, Pepke-Zaba, Joanna, additional, Price, Laura, additional, Rhodes, Christopher J., additional, Seeger, Werner, additional, Soubrier, Florent, additional, Suntharalingam, Jay, additional, Toshner, Mark R., additional, Vonk Noordegraaf, Anton, additional, Wharton, John, additional, Wild, James M., additional, Wort, Stephen John, additional, Lawrie, Allan, additional, Wilkins, Martin R., additional, Trembath, Richard C., additional, Shen, Yufeng, additional, Chung, Wendy K., additional, Swift, Andrew J., additional, Nichols, William C., additional, Morrell, Nicholas W., additional, Gräf, Stefan, additional, Abbs, Stephen, additional, Abulhoul, Lara, additional, Adlard, Julian, additional, Ahmed, Munaza, additional, Aitman, Timothy J., additional, Alachkar, Hana, additional, Allsup, David J., additional, Ancliff, Philip, additional, Antrobus, Richard, additional, Armstrong, Ruth, additional, Arno, Gavin, additional, Ashford, Sofie, additional, Astle, William J., additional, Attwood, Anthony, additional, Aurora, Paul, additional, Babbs, Christian, additional, Bacchelli, Chiara, additional, Bakchoul, Tamam, additional, Banka, Siddharth, additional, Bariana, Tadbir, additional, Barwell, Julian, additional, Batista, Joana, additional, Baxendale, Helen E., additional, Beales, Phil L., additional, Bennett, David L., additional, Bierzynska, Agnieszka, additional, Biss, Tina, additional, Bitner-Glindzicz, Maria A.K., additional, Black, Graeme C., additional, Bleda, Marta, additional, Blesneac, Iulia, additional, Bockenhauer, Detlef, additional, Boyce, Sara, additional, Bradley, John R., additional, Breen, Gerome, additional, Brennan, Paul, additional, Brewer, Carole, additional, Brown, Matthew, additional, Browning, Andrew C., additional, Browning, Michael J., additional, Buchan, Rachel J., additional, Buckland, Matthew S., additional, Bueser, Teofila, additional, Diz, Carmen Bugarin, additional, Burn, John, additional, Burns, Siobhan O., additional, Burren, Oliver S., additional, Burrows, Nigel, additional, Campbell, Carolyn, additional, Carr-White, Gerald, additional, Carss, Keren, additional, Casey, Ruth, additional, Caulfield, Mark J., additional, Chambers, Jenny, additional, Chambers, John, additional, Chan, Melanie M.Y., additional, Cheng, Floria, additional, Chinnery, Patrick F., additional, Chitre, Manali, additional, Christian, Martin T., additional, Clayton-Smith, Jill, additional, Cleary, Maureen, additional, Brod, Naomi Clements, additional, Colby, Elizabeth, additional, Cole, Trevor R.P., additional, Collins, Janine, additional, Collins, Peter W., additional, Compton, Cecilia J., additional, Cook, H. Terence, additional, Cook, Stuart, additional, Cooper, Nichola, additional, Corris, Paul A., additional, Curry, Nicola S., additional, Daniels, Matthew J., additional, Dattani, Mehul, additional, Daugherty, Louise C., additional, Davis, John, additional, De Soyza, Anthony, additional, Deevi, Sri V.V., additional, Dent, Timothy, additional, Deshpande, Charu, additional, Dewhurst, Eleanor F., additional, Dixon, Peter H., additional, Douzgou, Sofia, additional, Downes, Kate, additional, Drazyk, Anna M., additional, Drewe, Elizabeth, additional, Duarte, Daniel, additional, Dutt, Tina, additional, Edgar, J. David M., additional, Edwards, Karen, additional, Egner, William, additional, Ekani, Melanie N., additional, Elliott, Perry, additional, Erber, Wendy N., additional, Erwood, Marie, additional, Estiu, Maria C., additional, Evans, Dafydd Gareth, additional, Evans, Gillian, additional, Everington, Tamara, additional, Fassihi, Hiva, additional, Favier, Remi, additional, Fletcher, Debra, additional, Flinter, Frances A., additional, Floto, R. Andres, additional, Fowler, Tom, additional, Fox, James, additional, Frary, Amy J., additional, French, Courtney E., additional, Freson, Kathleen, additional, Frontini, Mattia, additional, Furnell, Abigail, additional, Gale, Daniel P., additional, Ganesan, Vijeya, additional, Gattens, Michael, additional, Ghofrani, Hossein-Ardeschir, additional, Gibbs, J. Simon R., additional, Gibson, Kate, additional, Gilmour, Kimberly C., additional, Gleadall, Nicholas S., additional, Goddard, Sarah, additional, Gomez, Keith, additional, Gordins, Pavels, additional, Gosal, David, additional, Graham, Jodie, additional, Grassi, Luigi, additional, Greenhalgh, Lynn, additional, Greinacher, Andreas, additional, Gresele, Paolo, additional, Griffiths, Philip, additional, Grigoriadou, Sofia, additional, Grozeva, Detelina, additional, Gurnell, Mark, additional, Hackett, Scott, additional, Hadinnapola, Charaka, additional, Hague, Rosie, additional, Hague, William M., additional, Haimel, Matthias, additional, Hall, Matthew, additional, Hanson, Helen L., additional, Haque, Eshika, additional, Harkness, Kirsty, additional, Harper, Andrew R., additional, Harris, Claire L., additional, Hart, Daniel, additional, Hassan, Ahamad, additional, Hayman, Grant, additional, Henderson, Alex, additional, Herwadkar, Archana, additional, Hoffman, Jonathan, additional, Horvath, Rita, additional, Houlden, Henry, additional, Houweling, Arjan C., additional, Hu, Fengyuan, additional, Hudson, Gavin, additional, Huissoon, Aarnoud P., additional, Hurles, Matthew, additional, Irving, Melita, additional, Izatt, Louise, additional, James, Roger, additional, Johnson, Sally A., additional, Jolles, Stephen, additional, Jolley, Jennifer, additional, Josifova, Dragana, additional, Jurkute, Neringa, additional, Kasanicki, Mary A., additional, Kazkaz, Hanadi, additional, Kazmi, Rashid, additional, Kelleher, Peter, additional, Kelly, Anne M, additional, Kelsall, Wilf, additional, Kempster, Carly, additional, Kingston, Nathalie, additional, Koelling, Nils, additional, Kostadima, Myrto, additional, Koziell, Ania, additional, Kreuzhuber, Roman, additional, Kuijpers, Taco W., additional, Kumar, Ajith, additional, Kumararatne, Dinakantha, additional, Kurian, Manju A., additional, Laffan, Michael A., additional, Lalloo, Fiona, additional, Lambert, Michele, additional, Allen, Hana Lango, additional, Layton, D. Mark, additional, Lentaigne, Claire, additional, Lester, Tracy, additional, Levine, Adam P., additional, Linger, Rachel, additional, Longhurst, Hilary, additional, Lorenzo, Lorena E., additional, Louka, Eleni, additional, Lyons, Paul A., additional, Madan, Bella, additional, Maher, Eamonn R., additional, Maimaris, Jesmeen, additional, Malka, Samantha, additional, Mangles, Sarah, additional, Mapeta, Rutendo, additional, Marchbank, Kevin J., additional, Marks, Stephen, additional, Markus, Hugh S., additional, Marschall, Hanns-Ulrich, additional, Marshall, Andrew, additional, Mathias, Mary, additional, Matthews, Emma, additional, Maxwell, Heather, additional, McAlinden, Paul, additional, McCarthy, Mark I., additional, McKinney, Harriet, additional, Meacham, Stuart, additional, Mead, Adam J., additional, Mehta, Sarju G., additional, Michaelides, Michel, additional, Millar, Carolyn, additional, Mohammed, Shehla N., additional, Moore, Anthony T., additional, Mozere, Monika, additional, Muir, Keith W., additional, Mumford, Andrew D., additional, Nemeth, Andrea H., additional, Newman, William G., additional, Newnham, Michael, additional, Noorani, Sadia, additional, Nurden, Paquita, additional, O’Sullivan, Jennifer, additional, Obaji, Samya, additional, Odhams, Chris, additional, Okoli, Steven, additional, Olschewski, Andrea, additional, Ong, Kai Ren, additional, Oram, S. Helen, additional, Ormondroyd, Elizabeth, additional, Ouwehand, Willem H., additional, Palles, Claire, additional, Papadia, Sofia, additional, Park, Soo-Mi, additional, Parry, David, additional, Patel, Smita, additional, Paterson, Joan, additional, Peacock, Andrew, additional, Pearce, Simon H., additional, Peerlinck, Kathelijne, additional, Petersen, Romina, additional, Pilkington, Clarissa, additional, Poole, Kenneth E.S., additional, Psaila, Bethan, additional, Pyle, Angela, additional, Quinton, Richard, additional, Rahman, Shamima, additional, Rao, Anupama, additional, Raymond, F. Lucy, additional, Rayner-Matthews, Paula J., additional, Rendon, Augusto, additional, Renton, Tara, additional, Rice, Andrew S.C., additional, Richter, Alex, additional, Robert, Leema, additional, Roberts, Irene, additional, Rose, Sarah J., additional, Ross-Russell, Robert, additional, Roughley, Catherine, additional, Roy, Noemi B.A., additional, Ruddy, Deborah M., additional, Sadeghi-Alavijeh, Omid, additional, Saleem, Moin A., additional, Samani, Nilesh, additional, Samarghitean, Crina, additional, Sanchis-Juan, Alba, additional, Sargur, Ravishankar B., additional, Sarkany, Robert N., additional, Satchell, Simon, additional, Savic, Sinisa, additional, Sayer, Genevieve, additional, Sayer, John A., additional, Scelsi, Laura, additional, Schaefer, Andrew M., additional, Schulman, Sol, additional, Scott, Richard, additional, Scully, Marie, additional, Searle, Claire, additional, Sen, Arjune, additional, Sewell, W.A. Carrock, additional, Seyres, Denis, additional, Shah, Neil, additional, Shamardina, Olga, additional, Shapiro, Susan E., additional, Shaw, Adam C., additional, Sibson, Keith, additional, Side, Lucy, additional, Simeoni, Ilenia, additional, Simpson, Michael A., additional, Sims, Matthew C., additional, Sivapalaratnam, Suthesh, additional, Smedley, Damian, additional, Smith, Katherine R., additional, Smith, Kenneth G.C., additional, Snape, Katie, additional, Soranzo, Nicole, additional, Spasic-Boskovic, Olivera, additional, Staines, Simon, additional, Staples, Emily, additional, Stark, Hannah, additional, Stirrups, Kathleen E., additional, Stuckey, Alex, additional, Syrris, Petros, additional, Tait, R. Campbell, additional, Talks, Kate, additional, Tan, Rhea Y.Y., additional, Taylor, Jenny C., additional, Taylor, John M., additional, Thaventhiran, James E., additional, Themistocleous, Andreas C., additional, Thomas, David, additional, Thomas, Ellen, additional, Thomas, Moira J., additional, Thomas, Patrick, additional, Thomson, Kate, additional, Thrasher, Adrian J., additional, Thys, Chantal, additional, Tischkowitz, Marc, additional, Titterton, Catherine, additional, Toh, Cheng-Hock, additional, Tomlinson, Ian P., additional, Traylor, Matthew, additional, Treadaway, Paul, additional, Tuna, Salih, additional, Turro, Ernest, additional, Twiss, Philip, additional, Vale, Tom, additional, Van Geet, Chris, additional, van Zuydam, Natalie, additional, Vandersteen, Anthony M, additional, Vazquez-Lopez, Marta, additional, von Ziegenweidt, Julie, additional, Wagner, Annette, additional, Waisfisz, Quinten, additional, Walker, Neil, additional, Walker, Suellen M., additional, Ware, James S., additional, Watkins, Hugh, additional, Watt, Christopher, additional, Webster, Andrew R., additional, Wedderburn, Lucy, additional, Wei, Wei, additional, Welch, Steven B., additional, Wessels, Julie, additional, Westbury, Sarah K., additional, Westwood, John-Paul, additional, Whitehorn, Deborah, additional, Whitworth, James, additional, Wilkie, Andrew O.M., additional, Williamson, Catherine, additional, Wilson, Brian T., additional, Wong, Edwin K.S., additional, Wood, Nicholas, additional, Wood, Yvette, additional, Woods, Christopher Geoffrey, additional, Woodward, Emma R., additional, Worth, Austen, additional, Wright, Michael, additional, Yates, Katherine, additional, Yong, Patrick F.K., additional, Young, Timothy, additional, Yu, Ping, additional, Yu-Wai-Man, Patrick, additional, Zlamalova, Eliska, additional, Hirsch, Russel, additional, White, R. James, additional, Simon, Marc, additional, Badesch, David, additional, Rosenzweig, Erika, additional, Burger, Charles, additional, Chakinala, Murali, additional, Thenappan, Thenappan, additional, Elliott, Greg, additional, Simms, Robert, additional, Farber, Harrison, additional, Frantz, Robert, additional, Elwing, Jean, additional, Hill, Nicholas, additional, Ivy, Dunbar, additional, Klinger, James, additional, Nathan, Steven, additional, Oudiz, Ronald, additional, Robbins, Ivan, additional, Schilz, Robert, additional, Fortin, Terry, additional, Wilt, Jeffrey, additional, Yung, Delphine, additional, Austin, Eric, additional, Ahmad, Ferhaan, additional, Bhatt, Nitin, additional, Lahm, Tim, additional, Frost, Adaani, additional, Safdar, Zeenat, additional, Rehman, Zia, additional, Walter, Robert, additional, Torres, Fernando, additional, Bakshi, Sahil, additional, Archer, Stephen, additional, Argula, Rahul, additional, Barnett, Christopher, additional, Benza, Raymond, additional, Desai, Ankit, additional, and Maddipati, Veeranna, additional
Published: 2021
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160. sj-pdf-1-pul-10.1177_2045894020973124 - Supplemental material for Assessment of the REPLACE study composite endpoint in riociguat-treated patients in the PATENT study

Author: Simonneau, Gérald, Hossein-Ardeschir Ghofrani, Corris, Paul A., Rosenkranz, Stephan, Grünig, Ekkehard, White, Jim, Vallerie V. McLaughlin, Langleben, David, Meier, Christian, Busse, Dennis, Kleinjung, Frank, and Benza, Raymond L.
Subjects: 110203 Respiratory Diseases, FOS: Clinical medicine, Cardiology
Abstract: Supplemental material, sj-pdf-1-pul-10.1177_2045894020973124 for Assessment of the REPLACE study composite endpoint in riociguat-treated patients in the PATENT study by Gérald Simonneau, Hossein-Ardeschir Ghofrani, Paul A. Corris, Stephan Rosenkranz, Ekkehard Grünig, Jim White, Vallerie V. McLaughlin, David Langleben, Christian Meier, Dennis Busse, Frank Kleinjung and Raymond L. Benza in Pulmonary Circulation
Published: 2020
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161. sj-pdf-1-pul-10.1177_2045894020942121 - Supplemental material for Efficacy and safety of riociguat in combination therapy for patients with pulmonary arterial hypertension (PATENT studies)

Author: Hossein-Ardeschir Ghofrani, Grünig, Ekkehard, Jansa, Pavel, Langleben, David, Rosenkranz, Stephan, Preston, Ioana R., Rahaghi, Franck, Sood, Namita, Busse, Dennis, Meier, Christian, and Humbert, Marc
Subjects: 110203 Respiratory Diseases, FOS: Clinical medicine, Cardiology, humanities
Abstract: Supplemental material, sj-pdf-1-pul-10.1177_2045894020942121 for Efficacy and safety of riociguat in combination therapy for patients with pulmonary arterial hypertension (PATENT studies) by Hossein-Ardeschir Ghofrani, Ekkehard Grünig, Pavel Jansa, David Langleben, Stephan Rosenkranz, Ioana R. Preston, Franck Rahaghi, Namita Sood, Dennis Busse, Christian Meier and Marc Humbert in Pulmonary Circulation
Published: 2020
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162. Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry

Author: Hoeper, Marius M, Pausch, Christine, Grünig, Ekkehard, Klose, Hans, Staehler, Gerd, Huscher, Doerte, Pittrow, David, Olsson, Karen M, Vizza, Carmine Dario, Gall, Henning, Benjamin, Nicola, Distler, Oliver, Opitz, Christian, Gibbs, J Simon R, Delcroix, Marion, Ghofrani, H Ardeschir, Rosenkranz, Stephan, Ewert, Ralf, Kaemmerer, Harald, Lange, Tobias J, Kabitz, Hans-Joachim, Skowasch, Dirk, Skride, Andris, Jurevičienė, Elena, Paleviciute, Egle, Miliauskas, Skaidrius, Claussen, Martin, Behr, Juergen, Milger, Katrin, Halank, Michael, et al, University of Zurich, and Hoeper, Marius M
Subjects: 2740 Pulmonary and Respiratory Medicine, 2747 Transplantation, 10051 Rheumatology Clinic and Institute of Physical Medicine, 610 Medicine & health, 10178 Clinic for Pneumology, 2705 Cardiology and Cardiovascular Medicine, 2746 Surgery
Published: 2020
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163. Cardiac Rehabilitation in German Speaking Countries of Europe—Evidence-Based Guidelines from Germany, Austria and Switzerland LLKardReha-DACH—Part 1

Author: Rauch, Bernhard;Salzwedel, Annett;Bjarnason-Wehrens, Birna;Albus, Christian;Meng, Karin;Schmid, Jean-Paul;Benzer, Werner;Hackbusch, Matthes;Jensen, Katrin;Schwaab, Bernhard;Altenberger, Johann;Benjamin, Nicola;Bestehorn, Kurt;Bongarth, Christa;Dörr, Gesine;Eichler, Sarah;Einwang, Hans-Peter;Falk, Johannes;Glatz, Johannes;Gielen, Stephan;Grilli, Maurizio;Grünig, Ekkehard;Guha, Manju;Hermann, Matthias;Hoberg, Eike;Höfer, Stefan;Kaemmerer, Harald;Ladwig, Karl-Heinz;Mayer-Berger, Wolfgang;Metzendorf, Maria-Inti;Nebel, Roland;Neidenbach, Rhoia;Niebauer, Josef;Nixdorff, Uwe;Oberhoffer, Renate;Reibis, Rona;Reiss, Nils;Saure, Daniel;Schlitt, Axel;Völler, Heinz;von Känel, Roland;Weinbrenner, Susanne;Westphal, Ronja and Rauch, Bernhard;Salzwedel, Annett;Bjarnason-Wehrens, Birna;Albus, Christian;Meng, Karin;Schmid, Jean-Paul;Benzer, Werner;Hackbusch, Matthes;Jensen, Katrin;Schwaab, Bernhard;Altenberger, Johann;Benjamin, Nicola;Bestehorn, Kurt;Bongarth, Christa;Dörr, Gesine;Eichler, Sarah;Einwang, Hans-Peter;Falk, Johannes;Glatz, Johannes;Gielen, Stephan;Grilli, Maurizio;Grünig, Ekkehard;Guha, Manju;Hermann, Matthias;Hoberg, Eike;Höfer, Stefan;Kaemmerer, Harald;Ladwig, Karl-Heinz;Mayer-Berger, Wolfgang;Metzendorf, Maria-Inti;Nebel, Roland;Neidenbach, Rhoia;Niebauer, Josef;Nixdorff, Uwe;Oberhoffer, Renate;Reibis, Rona;Reiss, Nils;Saure, Daniel;Schlitt, Axel;Völler, Heinz;von Känel, Roland;Weinbrenner, Susanne;Westphal, Ronja
Published: 2020

164. Combination Therapy with Oral Treprostinil for Pulmonary Arterial Hypertension:A Double-Blind Placebo-controlled Clinical Trial

Author: White, R James, Jerjes-Sanchez, Carlos, Bohns Meyer, Gisela Martina, Pulido, Tomas, Sepulveda, Pablo, Wang, Kuo Yang, Grünig, Ekkehard, Hiremath, Shirish, Yu, Zaixin, Gangcheng, Zhang, Yip, Wei Luen James, Zhang, Shuyang, Khan, Akram, Deng, C Q, Grover, Rob, Tapson, Victor F, Svetliza, Graciela Noemi, Lescano, Adrian Jose, Bortman, Guillermo Roberto, Carlsen, Jørn, McDonough, Clark, White, James R, Rischard, Franz, White, R James, Jerjes-Sanchez, Carlos, Bohns Meyer, Gisela Martina, Pulido, Tomas, Sepulveda, Pablo, Wang, Kuo Yang, Grünig, Ekkehard, Hiremath, Shirish, Yu, Zaixin, Gangcheng, Zhang, Yip, Wei Luen James, Zhang, Shuyang, Khan, Akram, Deng, C Q, Grover, Rob, Tapson, Victor F, Svetliza, Graciela Noemi, Lescano, Adrian Jose, Bortman, Guillermo Roberto, Carlsen, Jørn, McDonough, Clark, White, James R, and Rischard, Franz
Abstract: Rationale: Oral treprostinil improves exercise capacity in patients with pulmonary arterial hypertension (PAH), but the effect on clinical outcomes was unknown.Objectives: To evaluate the effect of oral treprostinil compared with placebo on time to first adjudicated clinical worsening event in participants with PAH who recently began approved oral monotherapy.Methods: In this event-driven, double-blind study, we randomly allocated 690 participants (1:1 ratio) with PAH to receive placebo or oral treprostinil extended-release tablets three times daily. Eligible participants were using approved oral monotherapy for over 30 days before randomization and had a 6-minute-walk distance 150 m or greater. The primary endpoint was the time to first adjudicated clinical worsening event: death; hospitalization due to worsening PAH; initiation of inhaled or parenteral prostacyclin therapy; disease progression; or unsatisfactory long-term clinical response.Measurements and Main Results: Clinical worsening occurred in 26% of the oral treprostinil group compared with 36% of placebo participants (hazard ratio, 0.74; 95% confidence interval, 0.56-0.97; P = 0.028). Key measures of disease status, including functional class, Borg dyspnea score, and N-terminal pro-brain natriuretic peptide, all favored oral treprostinil treatment at Week 24 and beyond. A noninvasive risk stratification analysis demonstrated that oral treprostinil-assigned participants had a substantially higher mortality risk at baseline but achieved a lower risk profile from Study Weeks 12-60. The most common adverse events in the oral treprostinil group were headache, diarrhea, flushing, nausea, and vomiting.Conclusions: In participants with PAH, addition of oral treprostinil to approved oral monotherapy reduced the risk of clinical worsening.Clinical trial registered with www.clinicaltrials.gov (NCT01560624).
Published: 2020

165. Right Atrial Pressure During Exercise Predicts Survival in Patients With Pulmonary Hypertension

Author: Lichtblau, Mona, Bader, Patrick R, Saxer, Stéphanie, Berlier, Charlotte, Schwarz, Esther I, Hasler, Elisabeth D, Furian, Michael, Grünig, Ekkehard, Bloch, Konrad E, Ulrich, Silvia, Lichtblau, Mona, Bader, Patrick R, Saxer, Stéphanie, Berlier, Charlotte, Schwarz, Esther I, Hasler, Elisabeth D, Furian, Michael, Grünig, Ekkehard, Bloch, Konrad E, and Ulrich, Silvia
Abstract: Background We investigated changes in right atrial pressure (RAP) during exercise and their prognostic significance in patients assessed for pulmonary hypertension (PH). Methods and Results Consecutive right heart catheterization data, including RAP recorded during supine, stepwise cycle exercise in 270 patients evaluated for PH, were analyzed retrospectively and compared among groups of patients with PH (mean pulmonary artery pressure [mPAP] ≥25 mm Hg), exercise-induced PH (exPH; resting mPAP <25 mm Hg, exercise mPAP >30 mm Hg, and mPAP/cardiac output >3 Wood Units (WU)), and without PH (noPH). We investigated RAP changes during exercise and survival over a median (quartiles) observation period of 3.7 (2.8-5.6) years. In 152 patients with PH, 58 with exPH, and 60 with noPH, median (quartiles) resting RAP was 8 (6-11), 6 (4-8), and 6 (4-8) mm Hg (P<0.005 for noPH and exPH versus PH). Corresponding peak changes (95% CI) in RAP during exercise were 5 (4-6), 3 (2-4), and -1 (-2 to 0) mm Hg (noPH versus PH P<0.001, noPH versus exPH P=0.027). RAP increase during exercise correlated with mPAP/cardiac output increase (r=0.528, P<0.001). The risk of death or lung transplantation was higher in patients with exercise-induced RAP increase (hazard ratio, 4.24; 95% CI, 1.69-10.64; P=0.002) compared with patients with unaltered or decreasing RAP during exercise. Conclusions In patients evaluated for PH, RAP during exercise should not be assumed as constant. RAP increase during exercise, as observed in exPH and PH, reflects hemodynamic impairment and poor prognosis. Therefore, our data suggest that changes in RAP during exercise right heart catheterization are clinically important indexes of the cardiovascular function.
Published: 2020

166. Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry

Author: Hoeper, Marius M; https://orcid.org/0000-0001-9086-2293, Pausch, Christine, Grünig, Ekkehard, Klose, Hans, Staehler, Gerd, Huscher, Doerte, Pittrow, David, Olsson, Karen M, Vizza, Carmine Dario; https://orcid.org/0000-0002-3540-4983, Gall, Henning, Benjamin, Nicola; https://orcid.org/0000-0003-4730-969X, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Opitz, Christian, Gibbs, J Simon R, Delcroix, Marion; https://orcid.org/0000-0001-8394-9809, Ghofrani, H Ardeschir, Rosenkranz, Stephan; https://orcid.org/0000-0001-6237-1470, Ewert, Ralf, Kaemmerer, Harald, Lange, Tobias J, Kabitz, Hans-Joachim, Skowasch, Dirk, Skride, Andris, Jurevičienė, Elena; https://orcid.org/0000-0003-4331-5179, Paleviciute, Egle, Miliauskas, Skaidrius, Claussen, Martin, Behr, Juergen, Milger, Katrin, Halank, Michael, et al, Hoeper, Marius M; https://orcid.org/0000-0001-9086-2293, Pausch, Christine, Grünig, Ekkehard, Klose, Hans, Staehler, Gerd, Huscher, Doerte, Pittrow, David, Olsson, Karen M, Vizza, Carmine Dario; https://orcid.org/0000-0002-3540-4983, Gall, Henning, Benjamin, Nicola; https://orcid.org/0000-0003-4730-969X, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Opitz, Christian, Gibbs, J Simon R, Delcroix, Marion; https://orcid.org/0000-0001-8394-9809, Ghofrani, H Ardeschir, Rosenkranz, Stephan; https://orcid.org/0000-0001-6237-1470, Ewert, Ralf, Kaemmerer, Harald, Lange, Tobias J, Kabitz, Hans-Joachim, Skowasch, Dirk, Skride, Andris, Jurevičienė, Elena; https://orcid.org/0000-0003-4331-5179, Paleviciute, Egle, Miliauskas, Skaidrius, Claussen, Martin, Behr, Juergen, Milger, Katrin, Halank, Michael, and et al
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; <45% vs ≥45% predicted), smoking status, and presence of comorbidities (obesity, hypertension, coronary heart disease, and diabetes mellitus). A hierarchical agglomerative clustering algorithm was performed using Ward's minimum variance method. The clusters were analyzed in terms of baseline characteristics; survival; and response to pulmonary arterial hypertension (PAH) therapy, expressed as changes from baseline to follow-up in functional class, 6-minute walking distance, cardiac biomarkers, and risk. Three clusters were identified: Cluster 1 (n = 106; 12.6%): median age 45 years, 76% females, no comorbidities, mostly never smokers, DLCO ≥45%; Cluster 2 (n = 301; 35.8%): median age 75 years, 98% females, frequent comorbidities, no smoking history, DLCO mostly ≥45%; and Cluster 3 (n = 434; 51.6%): median age 72 years, 72% males, frequent comorbidities, history of smoking, and low DLCO. Patients in Cluster 1 had a better response to PAH treatment than patients in the 2 other clusters. Survival over 5 years was 84.6% in Cluster 1, 59.2% in Cluster 2, and 42.2% in Cluster 3 (unadjusted p < 0.001 for comparison between all groups). The population of patients diagnosed with IPAH is heterogenous. This cluster analysis identified distinct phenotypes, which differed in clinical presentation, response to therapy, and survival.
Published: 2020

167. Quantitative 3D pulmonary MR-perfusion in patients with pulmonary arterial hypertension: Correlation with invasive pressure measurements

Author: Ley, Sebastian, Mereles, Derliz, Risse, Frank, Grünig, Ekkehard, Ley-Zaporozhan, Julia, Tecer, Zueleyha, Puderbach, Michael, Fink, Christian, and Kauczor, Hans-Ulrich
Published: 2007
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168. Value of high spatial and high temporal resolution magnetic resonance angiography for differentiation between idiopathic and thromboembolic pulmonary hypertension: initial results

Author: Ley, Sebastian, Fink, Christian, Zaporozhan, Julia, Borst, Mathias M., Meyer, F. Joachim, Puderbach, Michael, Eichinger, Monika, Plathow, Christian, Grünig, Ekkehard, Kreitner, Karl-Friedrich, and Kauczor, Hans-Ulrich
Published: 2005
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169. Effect of Supervised Training Therapy on Pulmonary Arterial Compliance and Stroke Volume in Severe Pulmonary Arterial Hypertension and Inoperable or Persistent Chronic Thromboembolic Pulmonary Hypertension

Author: Nagel, Christian, primary, Benjamin, Nicola, additional, Egenlauf, Benjamin, additional, Eichstaedt, Christina A., additional, Fischer, Christine, additional, Palevičiūtė, Eglė, additional, Čelutkienė, Jelena, additional, Harutyunova, Satenik, additional, Mayer, Eckhard, additional, Nasereddin, Mohammed, additional, Marra, Alberto M., additional, Grünig, Ekkehard, additional, and Guth, Stefan, additional
Published: 2021
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170. The Experience, Prerequisites, and the Barriers in Organizing a Specialized Rehabilitation Program for Patients with Pulmonary Hypertension

Author: Palevičiūtė, Eglė, primary, Gumbienė, Lina, additional, Jurevičienė, Elena, additional, Šimbelytė, Toma, additional, Laucevičienė, Ieva, additional, Laucevičius, Aleksandras, additional, Barysienė, Jūratė, additional, Eichstaedt, Christina A., additional, Benjamin, Nicola, additional, Grünig, Ekkehard, additional, and Čelutkienė, Jelena, additional
Published: 2021
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171. Right Heart Size and Right Ventricular Reserve in Pulmonary Hypertension: Impact on Management and Prognosis

Author: Grünig, Ekkehard, primary, Eichstaedt, Christina A., additional, Seeger, Rebekka, additional, and Benjamin, Nicola, additional
Published: 2020
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172. ERS statement on chronic thromboembolic pulmonary hypertension

Author: Delcroix, Marion, primary, Torbicki, Adam, additional, Gopalan, Deepa, additional, Sitbon, Olivier, additional, Klok, Frederikus A., additional, Lang, Irene, additional, Jenkins, David, additional, Kim, Nick H., additional, Humbert, Marc, additional, Jais, Xavier, additional, Vonk Noordegraaf, Anton, additional, Pepke-Zaba, Joanna, additional, Brénot, Philippe, additional, Dorfmuller, Peter, additional, Fadel, Elie, additional, Ghofrani, Hossein-Ardeschir, additional, Hoeper, Marius M., additional, Jansa, Pavel, additional, Madani, Michael, additional, Matsubara, Hiromi, additional, Ogo, Takeshi, additional, Grünig, Ekkehard, additional, D'Armini, Andrea, additional, Galie, Nazzareno, additional, Meyer, Bernhard, additional, Corkery, Patrick, additional, Meszaros, Gergely, additional, Mayer, Eckhard, additional, and Simonneau, Gérald, additional
Published: 2020
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173. Response to: ‘ Correspondence on ‘Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension’’ by Iudici et al

Author: Grünig, Ekkehard, primary, Benjamin, Nicola, additional, Xanthouli, Panagiota, additional, Jordan, Suzana, additional, Milde, Nicklas, additional, Marra, Alberto, additional, Blank, Norbert, additional, Egenlauf, Benjamin, additional, Gorenflo, Matthias, additional, Harutyunova, Satenik, additional, Lorenz, Hanns-Martin, additional, Nagel, Christian, additional, Theobald, Vivienne, additional, Lichtblau, Mona, additional, Berlier, Charlotte, additional, Ulrich, Silvia, additional, and Distler, Oliver, additional
Published: 2020
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174. Standardized exercise training is feasible, safe, and effective in pulmonary arterial and chronic thromboembolic pulmonary hypertension: results from a large European multicentre randomized controlled trial

Author: Grünig, Ekkehard, primary, MacKenzie, Alison, additional, Peacock, Andrew J, additional, Eichstaedt, Christina A, additional, Benjamin, Nicola, additional, Nechwatal, Robert, additional, Ulrich, Silvia, additional, Saxer, Stéphanie, additional, Bussotti, Maurizio, additional, Sommaruga, Marinella, additional, Ghio, Stefano, additional, Gumbiene, Lina, additional, Palevičiūtė, Eglė, additional, Jurevičienė, Elena, additional, Cittadini, Antonio, additional, Stanziola, Anna A, additional, Marra, Alberto M, additional, Kovacs, Gabor, additional, Olschewski, Horst, additional, Barberà, Joan-Albert, additional, Blanco, Isabel, additional, Spruit, Martijn A, additional, Franssen, Frits M E, additional, Vonk Noordegraaf, Anton, additional, Reis, Abílio, additional, Santos, Mário, additional, Viamonte, Sofia Gonçalves, additional, Demeyer, Heleen, additional, Delcroix, Marion, additional, Bossone, Eduardo, additional, and Johnson, Martin, additional
Published: 2020
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175. Right Atrial Pressure During Exercise Predicts Survival in Patients With Pulmonary Hypertension

Author: Lichtblau, Mona, primary, Bader, Patrick R., additional, Saxer, Stéphanie, additional, Berlier, Charlotte, additional, Schwarz, Esther I., additional, Hasler, Elisabeth D., additional, Furian, Michael, additional, Grünig, Ekkehard, additional, Bloch, Konrad E., additional, and Ulrich, Silvia, additional
Published: 2020
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176. Riociguat for the Treatment of Pulmonary Arterial Hypertension

Author: Ghofrani, Hossein-Ardeschir, Galiè, Nazzareno, Grimminger, Friedrich, Grünig, Ekkehard, Humbert, Marc, Jing, Zhi-Cheng, Keogh, Anne M., Langleben, David, Kilama, Michael Ochan, Fritsch, Arno, Neuser, Dieter, and Rubin, Lewis J.
Published: 2013
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177. Effect of Exercise and Respiratory Training on Clinical Progression and Survival in Patients with Severe Chronic Pulmonary Hypertension

Author: Grünig, Ekkehard, Ehlken, Nicola, Ghofrani, Ardeschir, Staehler, Gerd, Meyer, Joachim F., Juenger, Jana, Opitz, Christian F., Klose, Hans, Wilkens, Heinrike, Rosenkranz, Stephan, Olschewski, Horst, and Halank, Michael
Published: 2011
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178. BMPR2 Promoter Variants Effect Gene Expression in Pulmonary Arterial Hypertension Patients

Author: Song, Jie, primary, Hinderhofer, Katrin, additional, Kaufmann, Lilian T., additional, Benjamin, Nicola, additional, Fischer, Christine, additional, Grünig, Ekkehard, additional, and Eichstaedt, Christina A., additional
Published: 2020
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179. Assessment of the REPLACE study composite endpoint in riociguat‐treated patients in the PATENT study

Author: Simonneau, Gérald, primary, Ghofrani, Hossein‐Ardeschir, additional, Corris, Paul A., additional, Rosenkranz, Stephan, additional, Grünig, Ekkehard, additional, White, Jim, additional, McLaughlin, Vallerie V., additional, Langleben, David, additional, Meier, Christian, additional, Busse, Dennis, additional, Kleinjung, Frank, additional, and Benza, Raymond L., additional
Published: 2020
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180. SWITCHING TO RIOCIGUAT IN PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION NOT AT TREATMENT GOAL WITH PHOSPHODIESTERASE TYPE-5 INHIBITORS: SUBGROUP ANALYSIS RESULTS OF THE REPLACE STUDY

Author: Hoeper, Marius, primary, Ghofrani, Hossein, additional, Al-Hiti, Hikmet, additional, Benza, Raymond, additional, Chang, Sung A., additional, Corris, Paul, additional, Gibbs, J. Simon, additional, Grünig, Ekkehard, additional, Jansa, Pavel, additional, Klinger, James, additional, Langleben, David, additional, McLaughlin, Vallerie, additional, Meyer, Gisela, additional, Ota-Arakaki, Jaquelina, additional, Peacock, Andrew, additional, Pulido, Tomas, additional, Rosenkranz, Stephan, additional, Vizza, Carmine Dario, additional, Noordegraaf, Anton Vonk, additional, White, R., additional, Chang, Mikyung, additional, Kleinjung, Frank, additional, Meier, Christian, additional, Paraschin, Karen, additional, and Simonneau, Gerald, additional
Published: 2020
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181. Late Breaking Abstract - Switching from PDE5i to riociguat in patients with PAH: The REPLACE study

Author: Hoeper, Marius M., primary, Ghofrani, Hossein-Ardeschir, additional, Al-Hiti, Hikmet, additional, Benza, Raymond L., additional, Chang, Sung-A, additional, Corris, Paul A., additional, Gibbs, Simon, additional, Grünig, Ekkehard, additional, Jansa, Pavel, additional, Klinger, James R., additional, Langleben, David, additional, Mclaughlin, Vallerie V., additional, Meyer, Gisela, additional, Ota-Arakaki, Jaquelina S., additional, Peacock, Andrew, additional, Pulido, Tomás, additional, Rosenkranz, Stephan, additional, Vizza, Dario, additional, Vonk-Noordegraaf, Anton, additional, White, James, additional, Chang, Mikyung, additional, Kleinjung, Frank, additional, Meier, Christian, additional, Paraschin, Karen, additional, and Simonneau, Gérald, additional
Published: 2020
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182. Genetic findings in patients with different forms of pulmonary hypertension

Author: Eichstaedt, Christina, primary, Saßmannshausen, Zoe, additional, Gall, Henning, additional, Ghofrani, Ardeschir, additional, Seyfarth, Hans-Jürgen, additional, Lerche, Marianne, additional, Halank, Michael, additional, Xanthouli, Panagiota, additional, Harutyunova, Satenik, additional, Egenlauf, Benjamin, additional, Milger-Kneidinger, Katrin, additional, Rosenkrankz, Stefan, additional, Ewert, Ralf, additional, Lankeit, Mareike, additional, Lange, Tobias, additional, Hinderhofer, Katrin, additional, and Grünig, Ekkehard, additional
Published: 2020
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183. Risk stratification and prognostic factors in patients with pulmonary arterial hypertension and comorbidities

Author: Xanthouli, Panagiota, primary, Kögler, Maria, additional, Marra, Alberto, additional, Benjamin, Nicola, additional, Fischer, Lukas, additional, Eichstaedt, Christina, additional, Harutyunova, Satenik, additional, Nagel, Christian, additional, Grünig, Ekkehard, additional, and Egenlauf, Benjamin, additional
Published: 2020
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184. Supervised exercise training in patients with chronic thromboembolic pulmonary hypertension (CTEPH) as early follow-up treatment after pulmonary endarterectomy (PEA) – a prospective cohort study

Author: Nagel, Christian, primary, Nasereddin, Mohammed, additional, Benjamin, Nicola, additional, Egenlauf, Benjamin, additional, Harutyunova, Satenik, additional, Eichstaedt, Christina, additional, Xanthouli, Panagiota, additional, Mayer, Eckhard, additional, Grünig, Ekkehard, additional, and Guth, Stefan, additional
Published: 2020
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185. Impact of SARS‐CoV‐2 pandemic on pulmonary hypertension out‐patient clinics in Germany: a multi‐centre study

Author: Yogeswaran, Athiththan, primary, Gall, Henning, additional, Tello, Khodr, additional, Grünig, Ekkehard, additional, Xanthouli, Panagiota, additional, Ewert, Ralf, additional, Kamp, Jan C., additional, Olsson, Karen M., additional, Wißmüller, Max, additional, Rosenkranz, Stephan, additional, Klose, Hans, additional, Harbaum, Lars, additional, Lange, Tobias J., additional, Opitz, Christian F., additional, Waelde, Andrea, additional, Milger, Katrin, additional, Sommer, Natascha, additional, Seeger, Werner, additional, Ghofrani, Hossein Ardeschir, additional, and Richter, Manuel J., additional
Published: 2020
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186. Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

Author: Kaemmerer, Harald, primary, Gorenflo, Matthias, additional, Huscher, Dörte, additional, Pittrow, David, additional, Apitz, Christian, additional, Baumgartner, Helmut, additional, Berger, Felix, additional, Bruch, Leonhard, additional, Brunnemer, Eva, additional, Budts, Werner, additional, Claussen, Martin, additional, Coghlan, Gerry, additional, Dähnert, Ingo, additional, D’Alto, Michele, additional, Delcroix, Marion, additional, Distler, Oliver, additional, Dittrich, Sven, additional, Dumitrescu, Daniel, additional, Ewert, Ralf, additional, Faehling, Martin, additional, Germund, Ingo, additional, Ghofrani, Hossein Ardeschir, additional, Grohé, Christian, additional, Grossekreymborg, Karsten, additional, Halank, Michael, additional, Hansmann, Georg, additional, Harzheim, Dominik, additional, Nemes, Attila, additional, Havasi, Kalman, additional, Held, Matthias, additional, M. Hoeper, Marius, additional, Hofbeck, Michael, additional, Hohenfrost-Schmidt, Wolfgang, additional, Jurevičienė, Elena, additional, Gumbienè, Lina, additional, Kabitz, Hans-Joachim, additional, Klose, Hans, additional, Köhler, Thomas, additional, Konstantinides, Stavros, additional, Köestenberger, Martin, additional, Kozlik-Feldmann, Rainer, additional, Kramer, Hans-Heiner, additional, Kropf-Sanchen, Cornelia, additional, Lammers, Astrid, additional, Lange, Tobias, additional, Meyn, Philipp, additional, Miera, Oliver, additional, Milger-Kneidinger, Katrin, additional, Neidenbach, Rhoia, additional, Neurohr, Claus, additional, Opitz, Christian, additional, Perings, Christian, additional, Remppis, Bjoern Andrew, additional, Riemekasten, Gabriele, additional, Scelsi, Laura, additional, Scholtz, Werner, additional, Simkova, Iveta, additional, Skowasch, Dirk, additional, Skride, Andris, additional, Stähler, Gerd, additional, Stiller, Brigitte, additional, Tsangaris, Iraklis, additional, Vizza, Carmine Dario, additional, Vonk Noordegraaf, Anton, additional, Wilkens, Heinrike, additional, Wirtz, Hubert, additional, Diller, Gerhard-Paul, additional, Grünig, Ekkehard, additional, and Rosenkranz, Stephan, additional
Published: 2020
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187. Myeloproliferative Diseases as Possible Risk Factor for Development of Chronic Thromboembolic Pulmonary Hypertension—A Genetic Study

Author: Eichstaedt, Christina A., primary, Verweyen, Jeremias, additional, Halank, Michael, additional, Benjamin, Nicola, additional, Fischer, Christine, additional, Mayer, Eckhard, additional, Guth, Stefan, additional, Wiedenroth, Christoph B., additional, Egenlauf, Benjamin, additional, Harutyunova, Satenik, additional, Xanthouli, Panagiota, additional, Marra, Alberto M., additional, Wilkens, Heinrike, additional, Ewert, Ralf, additional, Hinderhofer, Katrin, additional, and Grünig, Ekkehard, additional
Published: 2020
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188. Genetics of pulmonary hypertension and high-altitude pulmonary edema

Author: Eichstaedt, Christina A., primary, Benjamin, Nicola, additional, and Grünig, Ekkehard, additional
Published: 2020
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189. Comparison of MRI and VQ-SPECT as a Screening Test for Patients With Suspected CTEPH: CHANGE-MRI Study Design and Rationale

Author: Lasch, Florian, primary, Karch, Annika, additional, Koch, Armin, additional, Derlin, Thorsten, additional, Voskrebenzev, Andreas, additional, Alsady, Tawfik Moher, additional, Hoeper, Marius M., additional, Gall, Henning, additional, Roller, Fritz, additional, Harth, Sebastian, additional, Steiner, Dagmar, additional, Krombach, Gabriele, additional, Ghofrani, Hossein Ardeschir, additional, Rengier, Fabian, additional, Heußel, Claus Peter, additional, Grünig, Ekkehard, additional, Beitzke, Dietrich, additional, Hacker, Marcus, additional, Lang, Irene M., additional, Behr, Jürgen, additional, Bartenstein, Peter, additional, Dinkel, Julien, additional, Schmidt, Kai-Helge, additional, Kreitner, Karl-Friedrich, additional, Frauenfelder, Thomas, additional, Ulrich, Silvia, additional, Hamer, Okka W., additional, Pfeifer, Michael, additional, Johns, Christopher S., additional, Kiely, David G., additional, Swift, Andrew James, additional, Wild, Jim, additional, and Vogel-Claussen, Jens, additional
Published: 2020
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190. Genetic Predisposition to High-Altitude Pulmonary Edema

Author: Eichstaedt, Christina A., primary, Mairbäurl, Heimo, additional, Song, Jie, additional, Benjamin, Nicola, additional, Fischer, Christine, additional, Dehnert, Christoph, additional, Schommer, Kai, additional, Berger, Marc M., additional, Bärtsch, Peter, additional, Grünig, Ekkehard, additional, and Hinderhofer, Katrin, additional
Published: 2020
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191. Flow rate variance of a fully implantable pump for the delivery of intravenous treprostinil in pulmonary arterial hypertension

Author: Richter, Manuel J., primary, Harutyunova, Satenik, additional, Bollmann, Tom, additional, Classen, Simon, additional, Fuge, Jan, additional, Gall, Henning, additional, Gerhardt, Felix, additional, Ghofrani, Hossein A., additional, Gunkel, Hartmut, additional, Grünig, Ekkehard, additional, Halank, Michael, additional, Heine, Alexander, additional, Klose, Hans, additional, Lange, Tobias J., additional, Neurohr, Claus, additional, Nickolaus, Kai, additional, Opitz, Christian F., additional, Rosenkranz, Stephan, additional, Seyfarth, Hans-Jürgen, additional, Tello, Khodr, additional, Ewert, Ralf, additional, and Olsson, Karen M., additional
Published: 2020
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192. When Pulmonary Hypertension Complicates Heart Failure

Author: Marra, Alberto-Maria, primary, Benjamin, Nicola, additional, Cittadini, Antonio, additional, Bossone, Eduardo, additional, and Grünig, Ekkehard, additional
Published: 2020
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193. Supervised Exercise Training in Patients with Chronic Thromboembolic Pulmonary Hypertension as Early Follow-Up Treatment after Pulmonary Endarterectomy: A Prospective Cohort Study

Author: Nagel, Christian, primary, Nasereddin, Mohammed, additional, Benjamin, Nicola, additional, Egenlauf, Benjamin, additional, Harutyunova, Satenik, additional, Eichstaedt, Christina A., additional, Xanthouli, Panagiota, additional, Mayer, Eckhard, additional, Grünig, Ekkehard, additional, and Guth, Stefan, additional
Published: 2020
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194. Computed tomography and magnetic resonance imaging of pulmonary hypertension: Pulmonary vessels and right ventricle

Author: Ley, Sebastian, Grünig, Ekkehard, Kiely, David G., van Beek, Edwin, and Wild, Jim
Published: 2010
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195. Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension

Author: Xanthouli, Panagiota, primary, Jordan, Suzana, additional, Milde, Nicklas, additional, Marra, Alberto, additional, Blank, Norbert, additional, Egenlauf, Benjamin, additional, Gorenflo, Matthias, additional, Harutyunova, Satenik, additional, Lorenz, Hanns-Martin, additional, Nagel, Christian, additional, Theobald, Vivienne, additional, Lichtblau, Mona, additional, Berlier, Charlotte, additional, Ulrich, Silvia, additional, Grünig, Ekkehard, additional, Benjamin, Nicola, additional, and Distler, Oliver, additional
Published: 2019
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196. Multicentre trials on specialised exercise training and rehabilitation are useful in patients with pulmonary hypertension

Author: Grünig, Ekkehard, primary, Benjamin, Nicola, additional, Eichstaedt, Christina A., additional, and Peacock, Andrew J., additional
Published: 2019
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197. Sequence Variants in BMPR2 and Genes Involved in the Serotonin and Nitric Oxide Pathways in Idiopathic Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: Relation to Clinical Parameters and Comparison with Left Heart Disease

Author: Ulrich, Silvia, Szamalek-Hoegel, Justyna, Hersberger, Martin, Fischler, Manuel, Garcia, Jesus Solera, Huber, Lars C., Grünig, Ekkehard, Janssen, Bart, and Speich, Rudolf
Published: 2010
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198. An open‐label, dose‐escalation study to evaluate the safety, tolerability, pharmacokinetics, and pharmacodynamics of single doses of GSK2586881 in participants with pulmonary arterial hypertension.

Author: Simon, Marc A., Hanrott, Kate, Budd, David C., Torres, Fernando, Grünig, Ekkehard, Escribano‐Subias, Pilar, Meseguer, Manuel L., Halank, Michael, Opitz, Christian, Hall, David A., Hewens, Deborah, Powley, William M., Siederer, Sarah, Bayliffe, Andrew, Lazaar, Aili L., Cahn, Anthony, and Rosenkranz, Stephan
Subjects: PULMONARY arterial hypertension, ANGIOTENSIN converting enzyme, RENIN-angiotensin system, ANGIOTENSIN II, PHARMACOKINETICS
Abstract: Preclinical and early clinical studies suggest that angiotensin‐converting enzyme type 2 activity may be impaired in patients with pulmonary arterial hypertension (PAH); therefore, administration of exogenous angiotensin‐converting enzyme type 2 (ACE2) may be beneficial. This Phase IIa, multi‐center, open‐label, exploratory, single‐dose, dose‐escalation study (NCT03177603) assessed the potential vasodilatory effects of single doses of GSK2586881 (a recombinant human ACE2) on acute cardiopulmonary hemodynamics in hemodynamically stable adults with documented PAH who were receiving background PAH therapy. Successive cohorts of participants were administered a single intravenous dose of GSK2586881 of 0.1, 0.2, 0.4, or 0.8 mg/kg. Dose escalation occurred after four or more participants per cohort were dosed and a review of safety, tolerability, pharmacokinetics, and hemodynamic data up to 24 h postdose was undertaken. The primary endpoint was a change in cardiopulmonary hemodynamics (pulmonary vascular resistance, cardiac index, and mean pulmonary artery pressure) from baseline. Secondary/exploratory objectives included safety and tolerability, effect on renin‐angiotensin system peptides, and pharmacokinetics. GSK2586881 demonstrated no consistent or sustained effect on acute cardiopulmonary hemodynamics in participants with PAH receiving background PAH therapy (N = 23). All doses of GSK2586881 were well tolerated. GSK2586881 was quantifiable in plasma for up to 4 h poststart of infusion in all participants and caused a consistent and sustained reduction in angiotensin II and a corresponding increase in angiotensin (1–7) and angiotensin (1–5). While there does not appear to be a consistent acute vasodilatory response to single doses of GSK2586881 in participants with PAH, the potential benefits in terms of chronic vascular remodeling remain to be determined. [ABSTRACT FROM AUTHOR]
Published: 2022
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199. Age-changes in right ventricular function–pulmonary circulation coupling: from pediatric to adult stage in 1899 healthy subjects. The RIGHT Heart International NETwork (RIGHT-NET).

Author: Vriz, Olga, Veldman, Gruschen, Gargani, Luna, Ferrara, Francesco, Frumento, Paolo, D'Alto, Michele, D'Andrea, Antonello, Radaan, Sarah Aldosari, Cocchia, Rosangela, Marra, Alberto Maria, Ranieri, Brigida, Salzano, Andrea, Stanziola, Anna Agnese, Voilliot, Damien, Agoston, Gergely, Cademartiri, Filippo, Cittadini, Antonio, Kasprzak, Jaroslaw D., Grünig, Ekkehard, and Bandera, Francesco
Abstract: The present study analyzes age-specific changes in RV function and RV–PA coupling in a large cohort of apparently healthy subjects with a wide age-range, to identify reference values and to study the influence of clinical and echocardiographic cofactors. 1899 Consecutive healthy subjects underwent a standardized transthoracic echocardiographic examination. Tricuspid annular plane systolic excursion (TAPSE) and systolic pulmonary artery pressure (SPAP) were measured. Ventriculo-arterial coupling was then inferred from the TAPSE/SPAP ratio. A quantile regression analysis was used to estimate quantiles 0.05, 0.10, 0.50 (median), 0.90, and 0.95 of TAPSE, SPAP and TAPSE/SPAP. The association between age and each of these values was determined. The mean age of the group was 45.2 ± 18.5 years (range 1 to 102 years), 971 were males. SPAP increased with age, whereas TAPSE and TAPSE/SPAP ratio decreased. Upon multivariate modeling, the most significant positive associations for TAPSE were body surface area (BSA) driven by the pediatric group, stroke volume (SV), E/A and negatively heart rate and E/e′ ratio. SPAP was positively associated with increasing age, SV, E/A, E/e′ and negatively with BSA. TAPSE/SPAP ratio was negatively associated with age, female sex, and E/e′ and positively with BSA. A preserved relationship between TAPSE and SPAP was found across the different age groups. TAPSE, SPAP and TAPSE/SPAP demonstrate important trends and associations with advancing age, impaired diastolic function, affected by female sex and BSA However the relationship between TAPSE and SPAP is relatively well preserved across the age spectrum. [ABSTRACT FROM AUTHOR]
Published: 2021
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200. Prognostic impact of hypochromic erythrocytes in patients with pulmonary arterial hypertension.

Author: Xanthouli, Panagiota, Theobald, Vivienne, Benjamin, Nicola, Marra, Alberto M., D'Agostino, Anna, Egenlauf, Benjamin, Shaukat, Memoona, Ding, Cao, Cittadini, Antonio, Bossone, Eduardo, Kögler, Maria, Grünig, Ekkehard, Muckenthaler, Martina U., and Eichstaedt, Christina A.
Subjects: PULMONARY arterial hypertension, IRON deficiency anemia, PULMONARY hypertension, ERYTHROCYTES, IRON deficiency, HOMEOSTASIS
Abstract: Background: Iron deficiency affects up to 50% of patients with pulmonary arterial hypertension (PAH) but iron markers such as ferritin and serum iron are confounded by several non-disease related factors like acute inflammation and diet. The aim of this study was to identify a new marker for iron deficiency and clinical outcome in PAH patients.Methods: In this single-center, retrospective study we assessed indicators of iron status and clinical parameters specifying the time to clinical worsening (TTCW) and survival in PAH patients at time of initial diagnosis and at 1-year follow-up using univariable and multivariable analysis.Results: In total, 150 patients were included with an invasively confirmed PAH and complete data on iron metabolism. The proportion of hypochromic erythrocytes > 2% at initial diagnosis was identified as an independent predictor for a shorter TTCW (p = 0.0001) and worse survival (p = 0.002) at initial diagnosis as well as worse survival (p = 0.016) at 1-year follow-up. Only a subset of these patients (64%) suffered from iron deficiency. Low ferritin or low serum iron neither correlated with TTCW nor survival. Severe hemoglobin deficiency at baseline was significantly associated with a shorter TTCW (p = 0.001).Conclusions: The presence of hypochromic erythrocytes > 2% was a strong and independent predictor of mortality and shorter TTCW in this cohort of PAH patients. Thus, it can serve as a valuable indicator of iron homeostasis and prognosis even in patients without iron deficiency or anemia. Further studies are needed to confirm the results and to investigate therapeutic implications. [ABSTRACT FROM AUTHOR]
Published: 2021
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