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2. Seralutinib in adults with pulmonary arterial hypertension (TORREY): a randomised, double-blind, placebo-controlled phase 2 trial

Author

Adir, Yochai, Baillie, Timothy, Baratz, David, Burger, Charles, Chakinala, Murali M., Cifrián Martínez, Jose Manual, Delcroix, Marion, Dwyer, Nathan, Elwing, Jean M., Fisher, Micah, Franco, Veronica, Grünig, Ekkehard, Highland, Kristin, Hill, Nicholas, Hirani, Naushad, Hoeper, Marius, Jansa, Pavel, Keogh, Anne, Kingrey, John, Lopez-Meseguer, Manuel, McConnell, John W., Mehta, Sanjay, Melendres-Groves, Lana, Opitz, Christian, Pepke-Zaba, Joanna, Pillutla, Priya, Rahaghi, Franck, Raina, Amresh, Raviv, Yael, Robinson, Jeffrey, Ryan, John, Sager, Jeffrey, Shapiro, Shelley, Simon, Marc, Smith, Kerri, Sobol, Irina R., Sood, Namita, Spikes, Leslie A., Stadler, Stefan, Stevens, Wendy, Sulica, Roxana, White, R. James, Frantz, Robert P, McLaughlin, Vallerie V, Sahay, Sandeep, Escribano Subías, Pilar, Zolty, Ronald L, Benza, Raymond L, Channick, Richard N, Chin, Kelly M, Hemnes, Anna R, Howard, Luke S, Sitbon, Olivier, Vachiéry, Jean-Luc, Zamanian, Roham T, Cravets, Matt, Roscigno, Robert F, Mottola, David, Osterhout, Robin, Bruey, Jean-Marie, Elman, Erin, Tompkins, Cindy-ann, Parsley, Ed, Aranda, Richard, Zisman, Lawrence S, and Ghofrani, Hossein-Ardeschir

Published
2024
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6. Value of exercise right heart catheterization in the differential diagnosis of chronic thromboembolic pulmonary disease

Author

Huertas Nieto, Sergio, Velázquez Martín, Maite, Sarnago Cebada, Fernando, Jiménez López-Guarch, Carmen, Maneiro Melón, Nicolás, Flox Camacho, Ángela, Segura de la Cal, Teresa, Cruz Utrilla, Alejandro, Aguilar Colindres, Ricardo, López Gude, María Jesús, Quezada Loaiza, Carlos Andrés, Revilla Ostolaza, Yolanda, Alonso Charterina, Sergio, Gómez Cuervo, Covadonga, Arribas Ynsaurriaga, Fernando, and Escribano Subías, Pilar

Published
2024
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7. Impacto del cateterismo derecho de ejercicio en el diagnóstico diferencial de la enfermedad tromboembólica pulmonar crónica

Author

Huertas Nieto, Sergio, Velázquez Martín, Maite, Sarnago Cebada, Fernando, Jiménez López-Guarch, Carmen, Maneiro Melón, Nicolás, Flox Camacho, Ángela, Segura de la Cal, Teresa, Cruz Utrilla, Alejandro, Aguilar Colindres, Ricardo, López Gude, María Jesús, Quezada Loaiza, Carlos Andrés, Revilla Ostolaza, Yolanda, Alonso Charterina, Sergio, Gómez Cuervo, Covadonga, Arribas Ynsaurriaga, Fernando, and Escribano Subías, Pilar

Published
2024
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8. Evolución y pronóstico de la insuficiencia tricuspídea moderada-grave tras tromboendarterectomía pulmonar o angioplastia de arterias pulmonares

Author

Hinojosa, Williams, Cruz-Utrilla, Alejandro, Jiménez López-Guarch, Carmen, Velázquez-Martín, Maite, Segura de la Cal, Teresa, Gómez-Burgueño, Lorena, Otero, Macarena, López-Gude, María Jesús, Morales, Rafael, Cortina-Romero, José María, Solís, Jorge, Arribas Ynsurriaga, Fernando, and Escribano-Subías, Pilar

Published
2023
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14. 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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15. 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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17. Seralutinib in adults with pulmonary arterial hypertension (TORREY): a randomised, double-blind, placebo-controlled phase 2 trial.

Author

Frantz, Robert P, McLaughlin, Vallerie V, Sahay, Sandeep, Escribano Subías, Pilar, Zolty, Ronald L, Benza, Raymond L, Channick, Richard N, Chin, Kelly M, Hemnes, Anna R, Howard, Luke S, Sitbon, Olivier, Vachiéry, Jean-Luc, Zamanian, Roham T, Cravets, Matt, Roscigno, Robert F, Mottola, David, Osterhout, Robin, Bruey, Jean-Marie, Elman, Erin, and Tompkins, Cindy-ann

Subjects
PULMONARY arterial hypertension, C-kit protein, MACROPHAGE colony-stimulating factor, PLATELET-derived growth factor receptors, VASCULAR remodeling, COUGH, PULMONARY hypertension
Abstract

Morbidity and mortality in pulmonary arterial hypertension (PAH) remain high. Activation of platelet-derived growth factor receptor, colony stimulating factor 1 receptor, and mast or stem cell growth factor receptor kinases stimulates inflammatory, proliferative, and fibrotic pathways driving pulmonary vascular remodelling in PAH. Seralutinib, an inhaled kinase inhibitor, targets these pathways. We aimed to evaluate the efficacy and safety of seralutinib in patients with PAH receiving standard background therapy. The TORREY trial was a phase 2, randomised, multicentre, multinational, double-blind, placebo-controlled study. Patients with PAH from 40 hospital and community sites were randomly assigned 1:1 via interactive response technologies to receive seralutinib (60 mg twice daily for 2 weeks, then increased to 90 mg twice daily as tolerated) or placebo by dry powder inhaler twice daily for 24 weeks. Randomisation was stratified by baseline pulmonary vascular resistance (PVR; <800 dyne·s/cm5 and ≥800 dyne·s/cm5). Patients were eligible if classified as WHO Group 1 PH (PAH), WHO Functional Class II or III, with a PVR of 400 dyne·s/cm5 or more, and a 6 min walk distance of between 150 m and 550 m. The primary endpoint was change in PVR from baseline to 24 weeks. Analyses for efficacy endpoints were conducted in randomly assigned patients (intention-to-treat population). Safety analyses included all patients who received the study drug. TORREY was registered with ClinicalTrials.gov (NCT04456998) and EudraCT (2019-002669-37) and is completed. From Nov 12, 2020, to April 20, 2022, 151 patients were screened for eligibility, and following exclusions, 86 adults receiving PAH background therapy were randomly assigned to seralutinib (n=44; four male, 40 female) or placebo (n=42; four male, 38 female), and comprised the intention-to-treat population. At baseline, treatment groups were balanced except for a higher representation of WHO Functional Class II patients in the seralutinib group. The least squares mean change from baseline to week 24 in PVR was 21·2 dyne·s/cm5 (95% CI −37·4 to 79·8) for the placebo group and −74·9 dyne·s/cm5 (−139·7 to −10·2) for the seralutinib group. The least squares mean difference between the seralutinib and placebo groups for change in PVR was −96·1 dyne·s/cm5 (95% CI −183·5 to −8·8; p=0·03). The most common treatment-emergent adverse event in both treatment groups was cough: 16 (38%) of 42 patients in the placebo group; 19 (43%) of 44 patients in the seralutinib group. Treatment with inhaled seralutinib significantly decreased PVR, meeting the primary endpoint of the study among patients receiving background therapy for PAH. Gossamer Bio. [ABSTRACT FROM AUTHOR]

Published
2024
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18. Tromboendarterectomía pulmonar en pacientes con hipertensión pulmonar tromboembólica crónica y afectación distal

Author

Gotor-Pérez, Consuelo A., López-Gude, María J., Benito-Arnaiz, Victoria, Pérez de la Sota, Enrique, Centeno-Rodríguez, Jorge E., Eixerés-Esteve, Andrea, Aguilar-Blanco, Eva M., Barajas-Díaz, Carolina, Velázquez-Martín, María T., Pérez-Núñez, Marta, Pérez-Vela, José L., Escribano-Subías, Pilar, and Cortina-Romero, José M.

Published
2020
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23. Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease.

Author

Luna-Lopez, Raquel, Segura de la Cal, Teresa, Sarnago Cebada, Fernando, Martin de Miguel, Irene, Hinojosa, Williams, Cruz-Utrilla, Alejandro, Velazquez, Maria Teresa, Delgado, Juan F., Mendoza, Alberto, Arribas Ynsaurriaga, Fernando, and Escribano-Subías, Pilar

Subjects
PULMONARY arterial hypertension, CONGENITAL heart disease, INFORMED consent (Medical law), ATRIAL septal defects, PATIENT selection, TRANSPOSITION of great vessels, PULMONARY hypertension
Published
2024
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24. Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease

Author

Luna-Lopez, Raquel, primary, Segura de la Cal, Teresa, additional, Sarnago Cebada, Fernando, additional, Martin de Miguel, Irene, additional, Hinojosa, Williams, additional, Cruz-Utrilla, Alejandro, additional, Velazquez, Maria Teresa, additional, Delgado, Juan F, additional, Mendoza, Alberto, additional, Arribas Ynsaurriaga, Fernando, additional, and Escribano-Subías, Pilar, additional

Published
2023
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25. Seven Additional Patients with SOX17 Related Pulmonary Arterial Hypertension and Review of the Literature

Author

Gallego-Zazo, Natalia, primary, Miranda-Alcaraz, Lucía, additional, Cruz-Utrilla, Alejandro, additional, del Cerro Marín, María Jesús, additional, Álvarez-Fuente, María, additional, del Mar Rodríguez Vázquez del Rey, María, additional, Guillén Rodríguez, Inmaculada, additional, Becerra-Munoz, Victor Manuel, additional, Moya-Bonora, Amparo, additional, Ochoa Parra, Nuria, additional, Parra, Alejandro, additional, Pascual, Patricia, additional, Cazalla, Mario, additional, Silván, Cristina, additional, Arias, Pedro, additional, Valverde, Diana, additional, de Jesús-Pérez, Vinicio, additional, Lapunzina, Pablo, additional, Escribano-Subías, Pilar, additional, and Tenorio-Castano, Jair, additional

Published
2023
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26. Use of Long-term Oxygen Therapy in Patients With Pulmonary Hypertension in Spain

Author

Torres Castro, Rodrigo, primary, Pérez Peñate, Gregorio Miguel, additional, Pérez Sagredo, Jesús, additional, Bravo Marqués, Rafael, additional, Soto Abánades, Clara, additional, Ramírez Martín, Purificación, additional, Becerra Muñoz, Víctor M., additional, Escribano Subías, Pilar, additional, Barberà, Joan Albert, additional, Blanco, Isabel, additional, and On Behalf Of The Rehap Investigators, ., additional

Published
2023
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33. Assessing the clinical benefit, safety, and patient-reported outcomes with the use of the PAHcare™ digital platform in pulmonary arterial hypertension: a pilot study.

Author

Pérez Peñate, Gregorio, Ochoa Parra, Nuria, Domingo Morera, Juan Antonio, Martínez Meñaca, Amaya, López Ramón, Marta, Cadenas Menéndez, Sergio, León Marrero, Fernando, de la Cal, Sara Gómara, Ghadban-Garrido, Cristina, Royo Tolosana, Patricia, Martín Puentes, Javier, Aldonza Aguayo, Rebeca, Mahdavi, Hadis, Bacchini Jeanneret, Gabriela, and Escribano Subías, Pilar

Published
2024
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35. First Genotype-Phenotype Study in TBX4 Syndrome: Gain-of-Function Mutations Causative for Lung Disease

Author

Prapa, Matina, Lago-Docampo, Mauro, Swietlik, Emilia M, Montani, David, Eyries, Mélanie, Humbert, Marc, Welch, Carrie L, Chung, Wendy K, Berger, Rolf MF, Bogaard, Harm Jan, Danhaive, Olivier, Escribano-Subías, Pilar, Gall, Henning, Girerd, Barbara, Hernandez-Gonzalez, Ignacio, Holden, Simon, Hunt, David, Jansen, Samara MA, Kerstjens-Frederikse, Wilhelmina, Kiely, David G, Lapunzina, Pablo, McDermott, John, Moledina, Shahin, Pepke-Zaba, Joanna, Polwarth, Gary J, Schotte, Gwen, Tenorio-Castaño, Jair, Thompson, AA Roger, Wharton, John, Wort, Stephen J, Megy, Karyn, Mapeta, Rutendo, Treacy, Carmen M, Martin, Jennifer M, Li, Wei, Swift, Andrew J, Upton, Paul D, Morrell, Nicholas W, Gräf, Stefan, Valverde, Diana, NIHR BioResource For Translational Research–Rare Diseases, National Cohort Study Of Idiopathic And Heritable PAH, PAH Biobank Enrolling Centers’ Investigators, Prapa, Matina [0000-0002-2717-5463], Lago-Docampo, Mauro [0000-0002-8799-6079], Montani, David [0000-0002-9358-6922], Humbert, Marc [0000-0003-0703-2892], Welch, Carrie L [0000-0003-3521-4458], Chung, Wendy K [0000-0003-3438-5685], Berger, Rolf MF [0000-0002-4385-5784], Bogaard, Harm Jan [0000-0001-5371-0346], Danhaive, Olivier [0000-0002-8208-3086], Escribano-Subías, Pilar [0000-0002-6640-4839], Gall, Henning [0000-0001-7016-7373], Jansen, Samara MA [0000-0003-3493-3573], Kerstjens-Frederikse, Wilhelmina [0000-0003-2528-2203], Lapunzina, Pablo [0000-0002-6324-4825], McDermott, John [0000-0002-5220-8837], Moledina, Shahin [0000-0003-0262-2340], Pepke-Zaba, Joanna [0000-0003-3764-3563], Polwarth, Gary J [0000-0003-0344-3088], Tenorio-Castaño, Jair [0000-0002-5308-2316], Thompson, AA Roger [0000-0002-0717-4551], Wharton, John [0000-0001-8110-2575], Megy, Karyn [0000-0002-2826-3879], Li, Wei [0000-0002-1924-3120], Morrell, Nicholas W [0000-0001-5700-9792], Gräf, Stefan [0000-0002-1315-8873], Valverde, Diana [0000-0002-7024-1657], and Apollo - University of Cambridge Repository

Subjects
interstitial lung disease, Lung Diseases, gain-of-function, Phenotype, Genotype, pulmonary arterial hypertension, Gain of Function Mutation, Mutation, Humans, TBX4, T-Box Domain Proteins, Bone Morphogenetic Protein Receptors, Type II, lung developmental disease
Abstract

Rationale: Despite the increased recognition of TBX4 (T-BOX transcription factor 4)-associated pulmonary arterial hypertension (PAH), genotype-phenotype associations are lacking and may provide important insights. Objectives: To compile and functionally characterize all TBX4 variants reported to date and undertake a comprehensive genotype-phenotype analysis. Methods: We assembled a multicenter cohort of 137 patients harboring monoallelic TBX4 variants and assessed the pathogenicity of missense variation (n = 42) using a novel luciferase reporter assay containing T-BOX binding motifs. We sought genotype-phenotype correlations and undertook a comparative analysis with patients with PAH with BMPR2 (Bone Morphogenetic Protein Receptor type 2) causal variants (n = 162) or no identified variants in PAH-associated genes (n = 741) genotyped via the National Institute for Health Research BioResource-Rare Diseases. Measurements and Main Results: Functional assessment of TBX4 missense variants led to the novel finding of gain-of-function effects associated with older age at diagnosis of lung disease compared with loss-of-function effects (P = 0.038). Variants located in the T-BOX and nuclear localization domains were associated with earlier presentation (P = 0.005) and increased incidence of interstitial lung disease (P = 0.003). Event-free survival (death or transplantation) was shorter in the T-BOX group (P = 0.022), although age had a significant effect in the hazard model (P = 0.0461). Carriers of TBX4 variants were diagnosed at a younger age (P < 0.001) and had worse baseline lung function (FEV1, FVC) (P = 0.009) than the BMPR2 and no identified causal variant groups. Conclusions: We demonstrated that TBX4 syndrome is not strictly the result of haploinsufficiency but can also be caused by gain of function. The pleiotropic effects of TBX4 in lung disease may be in part explained by the differential effect of pathogenic mutations located in critical protein domains.

Published
2022
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36. Outcome of moderate-severe tricuspid regurgitation after pulmonary endarterectomy or balloon pulmonary angioplasty.

Author

Hinojosa, Williams, Cruz-Utrilla, Alejandro, Jiménez López-Guarch, Carmen, Velázquez-Martín, Maite, Segura de la Cal, Teresa, Gómez-Burgueño, Lorena, Otero, Macarena, López-Gude, María Jesús, Morales, Rafael, Cortina-Romero, José María, Solís, Jorge, Arribas Ynsurriaga, Fernando, and Escribano-Subías, Pilar

Abstract

Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Published
2023
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42. Evaluation of a digital health system (PAHcare™) for routine care of patients with pulmonary arterial hypertension: The CBS-PAH study protocol

Author

Pérez Peñate, Gregorio, primary, Ochoa Parra, Nuria, additional, Domingo Morera, Juan Antonio, additional, Martínez Meñaca, Amaya, additional, López Ramón, Marta, additional, Cadenas Menéndez, Sergio, additional, León Marrero, Fernando, additional, Gómara de la Cal, Sara, additional, Ghadban Garrido, Cristina, additional, Royo Tolosana, Patricia, additional, Martin Puentes, Javier, additional, Aldonza Aguayo, Rebeca, additional, Mahdavi, Hadis, additional, Bacchini Jeanneret, Gabriela, additional, and Escribano Subías, Pilar, additional

Published
2022
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44. Novel Loss-of-Function KCNA5 Variants in Pulmonary Arterial Hypertension.

Author

Vera-Zambrano, Alba, Lago-Docampo, Mauro, Gallego, Natalia, Felipe Franco-Gonzalez, Juan, Morales-Cano, Daniel, Cruz-Utrilla, Alejandro, Villegas-Esguevillas, Marta, Fernández-Malavé, Edgar, Escribano-Subías, Pilar, Antonio Tenorio-Castaño, Jair, Perez-Vizcaino, Francisco, Valverde, Diana, González, Teresa, and Cogolludo, Angel

Subjects
PULMONARY arterial hypertension, POTASSIUM channels, ENDOTHELIN receptors, MUSCLE cells, PULMONARY artery, GENETIC variation, SMOOTH muscle
Abstract

Reduced expression and/or activity of Kv1.5 channels (encoded by KCNA5) is a common hallmark in human or experimental pulmonary arterial hypertension (PAH). Likewise, genetic variants in KCNA5 have been found in patients with PAH, but their functional consequences and potential impact on the disease are largely unknown. Herein, this study aimed to characterize the functional consequences of seven KCNA5 variants found in a cohort of patients with PAH. Potassium currents were recorded by patch-clamp technique in HEK293 cells transfected with wild-type or mutant Kv1.5 cDNA. Flow cytometry, Western blot, and confocal microscopy techniques were used for measuring protein expression and cell apoptosis in HEK293 and human pulmonary artery smooth muscle cells. KCNA5 variants (namely, Arg184Pro and Gly384Arg) found in patients with PAH resulted in a clear loss of potassium channel function as assessed by electrophysiological and molecular modeling analyses. The Arg184Pro variant also resulted in a pronounced reduction of Kv1.5 expression. Transfection with Arg184Pro or Gly384Arg variants decreased apoptosis of human pulmonary artery smooth muscle cells compared with the wild-type cells, demonstrating that KCNA5 dysfunction in both variants affects cell viability. Thus, in addition to affecting channel activity, both variants were associated with impaired apoptosis, a crucial process linked to the disease. The estimated prevalence of dysfunctional KCNA5 variants in the PAH population analyzed was around 1%. The data indicate that some KCNA5 variants found in patients with PAH have critical consequences for channel function, supporting the idea that KCNA5 pathogenic variants may be a causative or contributing factor for PAH. [ABSTRACT FROM AUTHOR]

Published
2023
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46. Rationale and Design of a Randomized Controlled Trial Evaluating Whole Muscle Exercise Training Effects in Outpatients with Pulmonary Arterial Hypertension (WHOLEi+12)

Author

Sanchis-Gomar, Fabian, González-Saiz, Laura, Sanz-Ayan, Paz, Fiuza-Luces, Carmen, Quezada-Loaiza, Carlos A., Flox-Camacho, Angela, Santalla, Alfredo, Munguía-Izquierdo, Diego, Santos-Lozano, Alejandro, Pareja-Galeano, Helios, Ara, Ignacio, Escribano-Subías, Pilar, and Lucia, Alejandro

Published
2015
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