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2. Worldwide CTEPH Registry: Long-Term Outcomes With Pulmonary Endarterectomy, Balloon Pulmonary Angioplasty, and Medical Therapy

Author

Delcroix, Marion, Pepke-Zaba, Joanna, D’Armini, Andrea M., Fadel, Elie, Guth, Stefan, Hoole, Stephen P., Jenkins, David P., Kiely, David G., Kim, Nick H., Madani, Michael M., Matsubara, Hiromi, Nakayama, Kazuhiko, Ogawa, Aiko, Ota-Arakaki, Jaquelina S., Quarck, Rozenn, Sadushi-Kolici, Roela, Simonneau, Gérald, Wiedenroth, Christoph B., Yildizeli, Bedrettin, Mayer, Eckhard, and Lang, Irene M.

Published
2024
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4. Current strategies for managing chronic thromboembolic pulmonary hypertension: results of the worldwide prospective CTEPH Registry

Author

Guth, Stefan, D'Armini, Andrea M, Delcroix, Marion, Nakayama, Kazuhiko, Fadel, Elie, Hoole, Stephen P, Jenkins, David P, Kiely, David G, Kim, Nick H, Lang, Irene M, Madani, Michael M, Matsubara, Hiromi, Ogawa, Aiko, Ota-Arakaki, Jaquelina S, Quarck, Rozenn, Sadushi-Kolici, Roela, Simonneau, Gérald, Wiedenroth, Christoph B, Yildizeli, Bedrettin, Mayer, Eckhard, and Pepke-Zaba, Joanna

Subjects
Rare Diseases, Lung, Cardiovascular
Abstract

Pulmonary endarterectomy (PEA), pulmonary arterial hypertension (PAH) therapy and balloon pulmonary angioplasty (BPA) are currently accepted therapies for chronic thromboembolic pulmonary hypertension (CTEPH). This international CTEPH Registry identifies clinical characteristics of patients, diagnostic algorithms and treatment decisions in a global context. 1010 newly diagnosed consecutive patients were included in the registry between February 2015 and September 2016. Diagnosis was confirmed by right heart catheterisation, ventilation-perfusion lung scan, computerised pulmonary angiography and/or invasive pulmonary angiography after at least 3 months on anticoagulation. Overall, 649 patients (64.3%) were considered for PEA, 193 (19.1%) for BPA, 20 (2.0%) for both PEA and BPA, and 148 (14.7%) for PAH therapy only. Reasons for PEA inoperability were technical inaccessibility (n=235), comorbidities (n=63) and patient refusal (n=44). In Europe and America and other countries (AAO), 72% of patients were deemed suitable for PEA, whereas in Japan, 70% of patients were offered BPA as first choice. Sex was evenly balanced, except in Japan where 75% of patients were female. A history of acute pulmonary embolism was reported for 65.6% of patients. At least one PAH therapy was initiated in 35.8% of patients (26.2% of PEA candidates, 54.5% of BPA candidates and 54.1% of those not eligible for either PEA or BPA). At the time of analysis, 39 patients (3.9%) had died of pulmonary hypertension-related causes (3.5% after PEA and 1.8% after BPA). The registry revealed noticeable differences in patient characteristics (rates of pulmonary embolism and sex) and therapeutic approaches in Japan compared with Europe and AAO.

Published
2021

7. Evaluation and management of patients with chronic thromboembolic pulmonary hypertension - consensus statement from the ISHLT

Author

de Perrot, Marc, Gopalan, Deepa, Jenkins, David, Lang, Irene M., Fadel, Elie, Delcroix, Marion, Benza, Raymond, Heresi, Gustavo A., Kanwar, Manreet, Granton, John T., McInnis, Micheal, Klok, Frederikus A., Kerr, Kim M., Pepke-Zaba, Joanna, Toshner, Mark, Bykova, Anastasia, D’Armini, Andrea M., Robbins, Ivan M., Madani, Michael, McGiffin, David, Wiedenroth, Christoph B., Mafeld, Sebastian, Opitz, Isabelle, Mercier, Olaf, Uber, Patricia A., Frantz, Robert P., and Auger, William R.

Published
2021
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9. Pulmonary endarterectomy in the management of chronic thromboembolic pulmonary hypertension

Author

Jenkins, David, Madani, Michael, Fadel, Elie, D'Armini, Andrea Maria, and Mayer, Eckhard

Subjects
Biomedical and Clinical Sciences, Cardiovascular Medicine and Haematology, Clinical Sciences, Lung, Cardiovascular, Chronic Disease, Endarterectomy, Hemodynamics, Hospital Mortality, Humans, Hypertension, Pulmonary, Patient Selection, Postoperative Complications, Pulmonary Artery, Pulmonary Embolism, Risk Assessment, Risk Factors, Time Factors, Treatment Outcome, Medical Physiology, Respiratory System, Cardiovascular medicine and haematology
Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension, resulting from fibrotic transformation of pulmonary artery clots causing chronic obstruction in macroscopic pulmonary arteries and associated vascular remodelling in the microvasculature.Pulmonary endarterectomy (PEA) offers the best chance of symptomatic and prognostic improvement in eligible patients; in expert centres, it has excellent results. Current in-hospital mortality rates are 90% at 1 year and >70% at 10 years. However, PEA, is a complex procedure and relies on a multidisciplinary CTEPH team led by an experienced surgeon to decide on an individual's operability, which is determined primarily by lesion location and the haemodynamic parameters. Therefore, treatment of patients with CTEPH depends largely on subjective judgements of eligibility for surgery by the CTEPH team.Other controversies discussed in this article include eligibility for PEA versus balloon pulmonary angioplasty, the new treatment algorithm in the European Society of Cardiology/European Respiratory Society guidelines and the definition of an "expert centre" for the management of this condition.

Published
2017

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

Author

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

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

Author

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

Published
2021
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15. Circulating Endothelin 1 but Not Transforming Growth Factor-β Levels Are Reduced after Pulmonary Endarterectomy in Subjects Affected by Chronic Thromboembolic Pulmonary Hypertension: A Prospective Cohort Study.

Author

Totaro, Pasquale, Tirelli, Claudio, De Amici, Mara, Grosjean, Fabrizio, Testa, Giorgia, Sacchi, Lucia, De Silvestri, Annalisa, Alloni, Alessia, Kushta, Eraldo, Albertini, Riccardo, Rampino, Teresa, and D'Armini, Andrea Maria

Subjects
PREPROENDOTHELIN, PULMONARY hypertension, VASCULAR remodeling, VASCULAR resistance, CARDIAC output, ENDARTERECTOMY
Abstract

Background and objectives: Endothelin-1 (ET-1) and transforming growth factor-β (TGF-β) play a pivotal role in the pathophysiology and vascular remodeling of chronic thromboembolic pulmonary hypertension (CTEPH) which is an under-diagnosed complication of acute pulmonary embolism (PE). Currently, pulmonary endarterectomy (PEA) is still the treatment of choice for selected patients suffering from CTEPH. The aim of this study was to evaluate the preoperative and postoperative circulating levels of ET-1 and TGF-β in subjects affected by CTEPH undergoing successful surgical treatment by PEA. Methods: The data from patients diagnosed with CTEPH who underwent PEA at the Foundation IRCCS Policlinico San Matteo Hospital (Pavia, Italy) were prospectively recorded in the Institutional database. Circulating ET-1 and TGF-β levels were assessed by an ELISA commercial kit before PEA, at 3 months and 1 year after PEA. The demographic data, preoperatory mean pulmonary arterial pressure (mPAP), cardiac output (CO), and pulmonary vascular resistance (PVR) were also recorded. Univariate and multivariate analyses were performed. Results: The analysis included 340 patients with complete ET-1 measurements and 206 patients with complete TGF-β measurements. ET-1 significantly decreased both at 3 months (p < 0.001) and at 1 year (p = 0.009) after PEA. On the other hand, preoperatory TGF-β levels did not significantly change after PEA. Furthermore, ET-1, but not TGF-β, was a good predictor for increased mPAP in multivariate analyses (p < 0.05). Conclusions: ET-1 but not TGF β was significantly modulated by PEA in subjects affected by CTEPH up to 1 year after surgery. The mechanisms leading to prolonged elevated circulating TGF-β levels and their clinical significance have to be further elucidated. [ABSTRACT FROM AUTHOR]

Published
2024
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21. Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study.

Author

Ghofrani, Hossein-Ardeschir, Simonneau, Gérald, D'Armini, Andrea M, Fedullo, Peter, Howard, Luke S, Jaïs, Xavier, Jenkins, David P, Jing, Zhi-Cheng, Madani, Michael M, Martin, Nicolas, Mayer, Eckhard, Papadakis, Kelly, Richard, Dominik, and Kim, Nick H

Subjects
PULMONARY hypertension, PHOSPHODIESTERASE inhibitors, PULMONARY arterial hypertension, THROMBOEMBOLISM, HOSPITAL patients
Abstract

Macitentan is beneficial for long-term treatment of pulmonary arterial hypertension. The microvasculopathy of chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension are similar. The phase 2, double-blind, randomised, placebo-controlled MERIT-1 trial assessed macitentan in 80 patients with CTEPH adjudicated as inoperable. Patients identified as WHO functional class II–IV with a pulmonary vascular resistance (PVR) of at least 400 dyn·s/cm5 and a walk distance of 150–450 m in 6 min were randomly assigned (1:1), via an interactive voice/web response system, to receive oral macitentan (10 mg once a day) or placebo. Treatment with phosphodiesterase type-5 inhibitors and oral or inhaled prostanoids was permitted for WHO functional class III/IV patients. The primary endpoint was resting PVR at week 16, expressed as percentage of PVR measured at baseline. Analyses were done in all patients who were randomly assigned to treatment; safety analyses were done in all patients who received at least one dose of the study drug. This study is registered with ClinicalTrials.gov , number NCT02021292. Between April 3, 2014, and March 17, 2016, we screened 186 patients for eligibility at 48 hospitals across 20 countries. Of these, 80 patients in 36 hospitals were randomly assigned to treatment (40 patients to macitentan, 40 patients to placebo). At week 16, geometric mean PVR decreased to 71·5% of baseline in the macitentan group and to 87·6% in the placebo group (geometric means ratio 0·81, 95% CI 0·70–0·95, p=0·0098). The most common adverse events in the macitentan group were peripheral oedema (9 [23%] of 40 patients) and decreased haemoglobin (6 [15%]). In MERIT-1, macitentan significantly improved PVR in patients with inoperable CTEPH and was well tolerated. Actelion Pharmaceuticals Ltd. [ABSTRACT FROM AUTHOR]

Published
2024
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28. Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome)

Author

Groh, Matthieu, Masciocco, Gabriella, Kirchner, Elizabeth, Kristen, Arnt, Pellegrini, Carlo, Varnous, Shaïda, Bortman, Guillermo, Rosenberg, Mark, Brucato, Antonio, Waterworth, Paul, Bonacina, Edgardo, Facchetti, Fabio, Calabrese, Leonard, Gregorini, Gina, Scali, Juan Jose, Starling, Randall, Frigerio, Maria, D’Armini, Andrea Maria, and Guillevin, Loïc

Published
2014
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30. The Pathologic Basis of Recovery

Author

Narula, Nupoor, Agozzino, Manuela, Gazzoli, Fabrizio, Concardi, Monica, Pagani, Francesco, Favalli, Valentina, Kodama, Takahide, Mazzola, Alessandro, D’Armini, Andrea M., and Arbustini, Eloisa

Published
2014
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33. Chronic thromboembolic pulmonary hypertension risk score evaluation and validation (CTEPH SOLUTION): proposal of a study protocol aimed to realize a validated risk score for early diagnosis

Author

MIOTTI, Cristiano, primary, D’ARMINI, Andrea M., additional, SCARDOVI, Beatrice, additional, GHIO, Stefano, additional, SINAGRA, Gianfranco, additional, SERRA, Walter, additional, ROMANIELLO, Antonella, additional, GALGANO, Giuseppe, additional, RONCON, Loris, additional, D’ALTO, Michele, additional, GIANNAZZO, Daniela, additional, VITULO, Patrizio, additional, BONGARZONI, Amedeo, additional, RUZZOLINI, Matteo, additional, ALBERA, Carlo, additional, CASU, Gavino, additional, PERAZZOLO MARRA, Martina, additional, PIERDOMENICO, Sante D., additional, LUONGO, Federico, additional, MANZI, Giovanna, additional, PAPA, Silvia, additional, SCOCCIA, Gianmarco, additional, CEDRONE, Nadia, additional, BADAGLIACCA, Roberto, additional, and VIZZA, Carmine D., additional

Published
2022
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42. Доклад ERS по хронической тромбоэмболической легочной гипертензии

Author

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

Abstract

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

Published
2022
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47. Pulmonary Endarterectomy in Chronic Thromboembolic Pulmonary Hypertension: Relationship between Treated Branches and Outcome

Author

D&apos;Armini, Andrea, primary, Pin, Maurizio, additional, Celentano, Anna, additional, Masiglat, Leslie Joyce Te, additional, Borrelli, Ermelinda, additional, Vanini, Benedetta, additional, Klersy, Catherine, additional, Silvaggio, Giuseppe, additional, Monterosso, Cristian, additional, Alloni, Alessia, additional, Pellegrini, Carlo, additional, and Ghio, Stefano, additional

Published
2022
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50. 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
Full Text
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