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201 results on '"Escribano-Subias P."'

3. 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
Pharmacology and Pharmaceutical Sciences, Biomedical and Clinical Sciences, Cardiovascular Medicine and Haematology, Clinical Sciences, Lung, Cardiovascular, Clinical Research, Clinical Trials and Supportive Activities, 6.1 Pharmaceuticals, Evaluation of treatments and therapeutic interventions, 6.2 Cellular and gene therapies, arterial hypertension, hemodynamics, recombinant human angiotensin-converting enzyme 2, renin-angiotensin system, rhACE2, recombinant human angiotensin‐converting enzyme 2, renin‐angiotensin system, Cardiorespiratory Medicine and Haematology, Cardiovascular medicine and haematology
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.

Published
2022

5. Results of a Planned Analysis of the Effects of Genetic Background on Sotatercept for the Treatment of Pulmonary Arterial Hypertension (PAH)

Author

Montani, D., primary, Mclaughlin, V.V., additional, Gibbs, J.S.S.R., additional, Gomberg-Maitland, M., additional, Hoeper, M.M., additional, Preston, I.R., additional, Souza, R., additional, Waxman, A.B., additional, Escribano Subias, P., additional, Feldman, J.P., additional, Bohns Meyer, G.M., additional, Olsson, K.M., additional, Manimaran, S., additional, Zhao, Y., additional, Lau, A., additional, Pena, J., additional, Badesch, D.B., additional, and Humbert, M.J.C., additional

Published
2024
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7. PH CARE COVID survey: an international patient survey on the care for pulmonary hypertension patients during the early phase of the COVID-19 pandemic

Author

Godinas, Laurent, Iyer, Keerthana, Meszaros, Gergely, Quarck, Rozenn, Escribano-Subias, Pilar, Vonk Noordegraaf, Anton, Jansa, Pavel, D’Alto, Michele, Luknar, Milan, Milutinov Ilic, Senka, Belge, Catharina, Sitbon, Olivier, Reis, Abílio, Rosenkranz, Stephan, Pepke-Zaba, Joanna, Humbert, Marc, and Delcroix, Marion

Published
2021
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8. Performance of Prognostic Risk Scores in Chronic Heart Failure Patients Enrolled in the European Society of Cardiology Heart Failure Long-Term Registry

Author

Crespo-Leiro, M., Anker, S., Mebazaa, A., Coats, A., Filippatos, G., Ferrari, R., Maggioni, A.P., Piepoli, M.F., Amir, O., Chioncel, O., Dahlström, U., Delgado Jimenez, J.F., Drozdz, J., Erglis, A., Fazlibegovic, E., Fonseca, C., Fruhwald, F., Gatzov, P., Goncalvesova, E., Hassanein, M., Hradec, J., Kavoliuniene, A., Lainscak, M., Logeart, D., Merkely, B., Metra, M., Otljanska, M., Seferovic, P.M., Srbinovska Kostovska, E., Temizhan, A., Tousoulis, D., Ferreira, T., Andarala, M., Fiorucci, E., Folkesson Lefrancq, E., Glémot, M., Gracia, G., Konte, M., Laroche, C., McNeill, P.A., Missiamenou, V., Taylor, C., Auer, J., Ablasser, K., Dolze, T., Brandner, K., Gstrein, S., Poelzl, G., Moertl, D., Reiter, S., Podczeck-Schweighofer, A., Muslibegovic, A., Vasilj, M., Cesko, M., Zelenika, D., Palic, B., Pravdic, D., Cuk, D., Vitlianova, K., Katova, T., Velikov, T., Kurteva, T., Kamenova, D., Antova, M., Sirakova, V., Krejci, J., Mikolaskova, M., Spinar, J., Krupicka, J., Malek, F., Hegarova, M., Lazarova, M., Monhart, Z., Sobhy, M., El Messiry, F., El Shazly, A.H., Elrakshy, Y., Youssef, A., Moneim, A.A., Noamany, M., Reda, A., Abdel Dayem, T.K., Farag, N., Ibrahim Halawa, S., Abdel Hamid, M., Said, K., Saleh, A., Ebeid, H., Hanna, R., Aziz, R., Louis, O., Enen, M.A., Ibrahim, B.S., Nasr, G., Elbahry, A., Sobhy, H., Ashmawy, M., Gouda, M., Aboleineen, W., Bernard, Y., Luporsi, P., Meneveau, N., Pillot, M., Morel, M., Seronde, M.-F., Schiele, F., Briand, F., Delahaye, F., Damy, T., Eicher, J.-C., de Groote, P., Fertin, M., Lamblin, N., Isnard, R., Lefol, C., Thevenin, S., Hagege, A., Jondeau, G., Le Marcis, V., Ly, J.-F., Coisne, D., Lequeux, B., Le Moal, V., Mascle, S., Lotton, P., Behar, N., Donal, E., Thebault, C., Ridard, C., Reynaud, A., Basquin, A., Bauer, F., Codjia, R., Galinier, M., Tourikis, P., Stavroula, M., Stefanadis, C., Chrysohoou, C., Kotrogiannis, I., Matzaraki, V., Dimitroula, T., Karavidas, A., Tsitsinakis, G., Kapelios, C., Nanas, J., Kampouri, H., Nana, E., Kaldara, E., Eugenidou, A., Vardas, P., Saloustros, I., Patrianakos, A., Tsaknakis, T., Evangelou, S., Nikoloulis, N., Tziourganou, H., Tsaroucha, A., Papadopoulou, A., Douras, A., Polgar, L., Kosztin, A., Nyolczas, N., Csaba Nagy, A., Halmosi, R., Elber, J., Alony, I., Shotan, A., Vazan Fuhrmann, A., Romano, S., Marcon, S., Penco, M., Di Mauro, M., Lemme, E., Carubelli, V., Rovetta, R., Bulgari, M., Quinzani, F., Lombardi, C., Bosi, S., Schiavina, G., Squeri, A., Barbieri, A., Di Tano, G., Pirelli, S., Fucili, A., Passero, T., Musio, S., Di Biase, M., Correale, M., Salvemini, G., Brognoli, S., Zanelli, E., Giordano, A., Agostoni, P., Italiano, G., Salvioni, E., Copelli, S., Modena, M.G., Reggianini, L., Valenti, C., Olaru, A., Bandino, S., Deidda, M., Mercuro, G., Cadeddu Dessalvi, C., Marino, P.N., Di Ruocco, M.V., Sartori, C., Piccinino, C., Parrinello, G., Licata, G., Torres, D., Giambanco, S., Busalacchi, S., Arrotti, S., Novo, S., Inciardi, R.M., Pieri, P., Chirco, P.R., Ausilia Galifi, M., Teresi, G., Buccheri, D., Minacapelli, A., Veniani, M., Frisinghelli, A., Priori, S.G., Cattaneo, S., Opasich, C., Gualco, A., Pagliaro, M., Mancone, M., Fedele, F., Cinque, A., Vellini, M., Scarfo, I., Romeo, F., Ferraiuolo, F., Sergi, D., Anselmi, M., Melandri, F., Leci, E., Iori, E., Bovolo, V., Pidello, S., Frea, S., Bergerone, S., Botta, M., Canavosio, F.G., Gaita, F., Merlo, M., Cinquetti, M., Sinagra, G., Ramani, F., Fabris, E., Stolfo, D., Artico, J., Miani, D., Fresco, C., Daneluzzi, C., Proclemer, A., Cicoira, M., Zanolla, L., Marchese, G., Torelli, F., Vassanelli, C., Voronina, N., Tamakauskas, V., Smalinskas, V., Karaliute, R., Petraskiene, I., Kazakauskaite, E., Rumbinaite, E., Vysniauskas, V., Brazyte-Ramanauskiene, R., Petraskiene, D., Stankala, S., Switala, P., Juszczyk, Z., Sinkiewicz, W., Gilewski, W., Pietrzak, J., Orzel, T., Kasztelowicz, P., Kardaszewicz, P., Lazorko-Piega, M., Gabryel, J., Mosakowska, K., Bellwon, J., Rynkiewicz, A., Raczak, G., Lewicka, E., Dabrowska-Kugacka, A., Bartkowiak, R., Sosnowska-Pasiarska, B., Wozakowska-Kaplon, B., Krzeminski, A., Zabojszcz, M., Mirek-Bryniarska, E., Grzegorzko, A., Bury, K., Nessler, J., Zalewski, J., Furman, A., Broncel, M., Poliwczak, A., Bala, A., Zycinski, P., Rudzinska, M., Jankowski, L., Kasprzak, J.D., Michalak, L., Wojtczak Soska, K., Huziuk, I., Retwinski, A., Flis, P., Weglarz, J., Bodys, A., Grajek, S., Kaluzna-Oleksy, M., Straburzynska-Migaj, E., Dankowski, R., Szymanowska, K., Grabia, J., Szyszka, A., Nowicka, A., Samcik, M., Wolniewicz, L., Baczynska, K., Komorowska, K., Poprawa, I., Komorowska, E., Sajnaga, D., Zolbach, A., Dudzik-Plocica, A., Abdulkarim, A.-F., Lauko-Rachocka, A., Kaminski, L., Kostka, A., Cichy, A., Ruszkowski, P., Splawski, M., Fitas, G., Szymczyk, A., Serwicka, A., Fiega, A., Zysko, D., Krysiak, W., Szabowski, S., Skorek, E., Pruszczyk, P., Bienias, P., Ciurzynski, M., Welnicki, M., Mamcarz, A., Folga, A., Zielinski, T., Rywik, T., Leszek, P., Sobieszczanska-Malek, M., Piotrowska, M., Kozar-Kaminska, K., Komuda, K., Wisniewska, J., Tarnowska, A., Balsam, P., Marchel, M., Opolski, G., Kaplon-Cieslicka, A., Gil, R.J., Mozenska, O., Byczkowska, K., Gil, K., Pawlak, A., Michalek, A., Krzesinski, P., Piotrowicz, K., Uzieblo-Zyczkowska, B., Stanczyk, A., Skrobowski, A., Ponikowski, P., Jankowska, E., Rozentryt, P., Polonski, L., Gadula-Gacek, E., Nowalany-Kozielska, E., Kuczaj, A., Kalarus, Z., Szulik, M., Przybylska, K., Klys, J., Prokop-Lewicka, G., Kleinrok, A., Tavares Aguiar, C., Ventosa, A., Pereira, S., Faria, R., Chin, J., De Jesus, I., Santos, R., Silva, P., Moreno, N., Queirós, C., Lourenço, C., Pereira, A., Castro, A., Andrade, A., Oliveira Guimaraes, T., Martins, S., Placido, R., Lima, G., Brito, D., Francisco, A.R., Cardiga, R., Proenca, M., Araujo, I., Marques, F., Moura, B., Leite, S., Campelo, M., Silva-Cardoso, J., Rodrigues, J., Rangel, I., Martins, E., Sofia Correia, A., Peres, M., Marta, L., Ferreira da Silva, G., Severino, D., Durao, D., Leao, S., Magalhaes, P., Moreira, I., Filipa Cordeiro, A., Ferreira, C., Araujo, C., Ferreira, A., Baptista, A., Radoi, M., Bicescu, G., Vinereanu, D., Sinescu, C.-J., Macarie, C., Popescu, R., Daha, I., Dan, G.-A., Stanescu, C., Dan, A., Craiu, E., Nechita, E., Aursulesei, V., Christodorescu, R., Otasevic, P., Simeunovic, D., Ristic, A.D., Celic, V., Pavlovic-Kleut, M., Suzic Lazic, J., Stojcevski, B., Pencic, B., Stevanovic, A., Andric, A., Iric-Cupic, V., Jovic, M., Davidovic, G., Milanov, S., Mitic, V., Atanaskovic, V., Antic, S., Pavlovic, M., Stanojevic, D., Stoickov, V., Ilic, S., Deljanin Ilic, M., Petrovic, D., Stojsic, S., Kecojevic, S., Dodic, S., Cemerlic Adic, N., Cankovic, M., Stojiljkovic, J., Mihajlovic, B., Radin, A., Radovanovic, S., Krotin, M., Klabnik, A., Pernicky, M., Murin, J., Kovar, F., Kmec, J., Semjanova, H., Strasek, M., Savnik Iskra, M., Ravnikar, T., Cernic Suligoj, N., Komel, J., Fras, Z., Jug, B., Glavic, T., Losic, R., Bombek, M., Krajnc, I., Krunic, B., Horvat, S., Kovac, D., Rajtman, D., Cencic, V., Letonja, M., Winkler, R., Valentincic, M., Melihen-Bartolic, C., Bartolic, A., Pusnik Vrckovnik, M., Kladnik, M., Slemenik Pusnik, C., Marolt, A., Klen, J., Drnovsek, B., Leskovar, B., Fernandez Anguita, M.J., Gallego Page, J.C., Salmeron Martinez, F.M., Andres, J., Genis, A.B., Mirabet, S., Mendez, A., Garcia-Cosio, L., Roig, E., Leon, V., Gonzalez-Costello, J., Muntane, G., Garay, A., Alcade-Martinez, V., Lopez Fernandez, S., Rivera-Lopez, R., Puga-Martinez, M., Fernandez-Alvarez, M., Serrano-Martinez, J.L., Grille-Cancela, Z., Marzoa-Rivas, R., Blanco-Canosa, P., Paniagua-Martin, M.J., Barge-Caballero, E., Laynez Cerdena, I., Famara Hernandez Baldomero, I., Lara Padron, A., Ofelia Rosillo, S., Dalmau Gonzalez-Gallarza, R., Salvador Montanes, O., Iniesta Manjavacas, A.M., Castro Conde, A., Araujo, A., Soria, T., Garcia-Pavia, P., Gomez-Bueno, M., Cobo-Marcos, M., Alonso-Pulpon, L., Segovia Cubero, J., Sayago, I., Gonzalez-Segovia, A., Briceno, A., Escribano Subias, P., Vicente Hernandez, M., Ruiz Cano, M.J., Gomez Sanchez, M.A., Barrios Garrido-Lestache, E., Garcia Pinilla, J.M., Garcia de la Villa, B., Sahuquillo, A., Bravo Marques, R., Torres Calvo, F., Perez-Martinez, M.T., Gracia-Rodenas, M.R., Garrido-Bravo, I.P., Pastor-Perez, F., Pascual-Figal, D.A., Diaz Molina, B., Orus, J., Epelde Gonzalo, F., Bertomeu, V., Valero, R., Martinez-Abellan, R., Quiles, J., Rodrigez-Ortega, J.A., Mateo, I., ElAmrani, A., Fernandez-Vivancos, C., Bierge Valero, D., Almenar-Bonet, L., Sanchez-Lazaro, I.J., Marques-Sule, E., Facila-Rubio, L., Perez-Silvestre, J., Garcia-Gonzalez, P., Ridocci-Soriano, F., Garcia-Escriva, D., Pellicer-Cabo, A., de la Fuente Galan, L., Lopez Diaz, J., Recio Platero, A., Arias, J.C., Blasco-Peiro, T., Sanz Julve, M., Sanchez-Insa, E., Aured-Guallar, C., Portoles-Ocampo, A., Melin, M., Hägglund, E., Stenberg, A., Lindahl, I.-M., Asserlund, B., Olsson, L., Afzelius, M., Karlström, P., Tengvall, L., Wiklund, P.-A., Olsson, B., Kalayci, S., Cavusoglu, Y., Gencer, E., Yilmaz, M.B., Gunes, H., Canepa, Marco, Fonseca, Candida, Chioncel, Ovidiu, Laroche, Cécile, Crespo-Leiro, Maria G., Coats, Andrew J.S., Mebazaa, Alexandre, Piepoli, Massimo F., Tavazzi, Luigi, and Maggioni, Aldo P.

Published
2018
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13. Resting RV-PA uncoupling as predictor of exercise induced pulmonary hypertension in patients with chronic thromboembolic pulmonary disease

Author

Jimenez Lopez Guarch, M C, primary, Huertas-Nieto, S, additional, Sarnago Cebada, F, additional, Velazquez Martin, M, additional, Aguilar, R, additional, Segura De La Cal, T, additional, Garcia Robles, J A, additional, Maneiro Melon, N, additional, Martin De Miguel, I, additional, Cruz Utrilla, A, additional, Solis Martin, J, additional, Arribas-Ynsaurriaga, F, additional, and Escribano Subias, P, additional

Published
2023
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14. Value of exercise right heart catheterization for differential diagnosis of dyspnoea in chronic thromboembolic pulmonary disease

Author

Huertas Nieto, S, primary, Velazquez Martin, M V, additional, Sarnago Cebada, F, additional, Manerio Melon, N, additional, Albarran Gonzalez-Trevilla, A, additional, Rivadulla Varela, C, additional, Jimenez Lopez-Guarch, C, additional, Aguilar Colindres, R, additional, Segura De La Cal, T, additional, Flox Camacho, A, additional, Cruz Utrilla, A, additional, Martin De Miguel, I, additional, Revilla Ostozala, Y, additional, Arribas Ynsaurriaga, F, additional, and Escribano Subias, P, additional

Published
2023
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15. TAPSE/sPAP at peak exercise as a surrogate of RV-PA uncoupling can predict exercise-induced pulmonary hypertension in symptomatic patients with chronic thromboembolic pulmonary disease

Author

Jimenez Lopez Guarch, M C, primary, Huertas-Nieto, S, additional, Sarnago Cebada, F, additional, Aguilar, R, additional, Segura De La Cal, T, additional, Maneiro Melon, N, additional, Garcia Robles, J A, additional, Cruz Utrilla, A, additional, Velazquez Martin, M, additional, Martin De Miguel, I, additional, Solis Martin, J, additional, Arribas-Ynsaurriaga, F, additional, and Escribano Subias, P, additional

Published
2023
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16. Usefulness of cardiopulmonary exercise test to detect exercise pulmonary hypertension in the post pulmonary embolim follow-up

Author

Huertas Nieto, S, primary, Segura De La Cal, T, additional, Cruz Utrilla, A, additional, Aguilar Colindres, R, additional, Hinojosa Camargo, W, additional, Flox Camacho, A, additional, Velazquez Martin, M, additional, Sarnago Cebada, F, additional, Manerio Melon, N, additional, Albarran Gonzalez-Trevilla, A, additional, Rivadulla Varela, C M, additional, Jimenez Lopez- Guarch, C, additional, Revilla Ostozala, Y, additional, Arribas Ynsaurriaga, F, additional, and Escribano Subias, P, additional

Published
2023
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27. Hemodynamic pulmonary response to exercise according to different resting pulmonary hypertension thresholds in chronic thromboembolic pulmonary disease

Author

Huertas Nieto, S, primary, Velazquez Martin, M T, additional, Sarnago Cebada, F, additional, Manerio Melon, N, additional, Jimenez Lopez Guarch, C, additional, Segura De La Cal, T, additional, Cruz Utrilla, A, additional, Olazabal Valiente, A, additional, Ropero Cristo, M J, additional, Flox Camacho, A, additional, Nuche Berenguer, J, additional, Arribas Ynsaurriaga, F, additional, and Escribano Subias, P, additional

Published
2022
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28. Ventilatory inefficiency predicts abnormal hemodynamic response to exercise in chronic thromboembolic disease

Author

Huertas Nieto, S, primary, Segura De La Cal, T, additional, Ropero Cristo, M J, additional, Flox Camacho, A, additional, Perez Olivares, C, additional, Velazquez Martin, M T, additional, Sarnago Cebada, F, additional, Jimenez Lopez Guarch, C, additional, Cruz Utrilla, A, additional, Manerio Melon, N, additional, Berenguer, J, additional, Arribas Ynsaurriaga, F, additional, and Escribano Subias, P, additional

Published
2022
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29. Is bilateral lung transplantation in pulmonary artery hypertension related to congenital heart disease a real therapeutic option? Experience of a national referral centre

Author

Luna, R, primary, Segura De La Cal, T, additional, Alonso, R, additional, Quezada, A, additional, Real, I, additional, Cortes, M, additional, Sarnago Cebada, F, additional, Velazquez, M, additional, Lopez Gude, M, additional, Escribano Subias, P, additional, and Gamez, P, additional

Published
2022
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30. Different diagnostic criteria agreement for exercise pulmonary hypertension in chronic thromboembolic pulmonary disease

Author

Huertas Nieto, S, primary, Sarnago Cebada, F, additional, Velazquez Martin, M T, additional, Jimenez Lopez Guarch, C, additional, Segura De La Cal, T, additional, Manerio Melon, N, additional, Cruz Utrilla, A, additional, Olazabal Valiente, A, additional, Ropero Cristo, M J, additional, Flox Camacho, A, additional, Nuche Berenguer, J, additional, Arribas Ynsaurriaga, F, additional, and Escribano Subias, P, additional

Published
2022
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31. The patient with Down syndrome and pulmonary arterial hypertension associated with congenital heart disease: from childhood to adulthood

Author

Playan Escribano, J, primary, Garrido-Lestache, E, additional, Luna, R, additional, Alvarez Fuente, M, additional, Lopez-Meseguer, M, additional, Guillen Rodriguez, I, additional, Perez Penate, G M, additional, Sabate Rotes, A, additional, Elias Hernandez, T, additional, Lopez Ramon, M, additional, Rueda Soriano, J, additional, Perin, F, additional, Blanco, I, additional, Del Cerro Marin, M J, additional, and Escribano Subias, P, additional

Published
2022
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33. PULSAR Open-Label Extension: Long-Term Efficacy and Safety of Sotatercept for the Treatment of Pulmonary Arterial Hypertension (PAH)

Author

Humbert, M., primary, Mclaughlin, V.V., additional, Gibbs, S.R., additional, Gomberg-Maitland, M., additional, Hoeper, M., additional, Preston, I.R., additional, Souza, R., additional, Waxman, A.B., additional, Ghofrani, H.A., additional, Escribano Subias, P., additional, Feldman, J.P., additional, Bohns Meyer, G.M., additional, Montani, D., additional, Olsson, K.M., additional, Manimaran, S., additional, de Oliveira Pena, J., additional, and Badesch, D.B., additional

Published
2022
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34. Usefulness of genetics for clinical reclassification and refinement of prognostic stratification in pulmonary arterial hypertension.

Author

Cruz-Utrilla, Alejandro, Gallego-Zazo, Natalia, Pérez-Olivares, Carmen, Hernández-González, Ignacio, Bedate, Pedro, Martínez Meñaca, Amaya, López Meseguer, Manuel, Lapunzina, Pablo, Pérez Núñez, Marta, Ochoa Parra, Nuria, Valverde, Diana, Tenorio-Castaño, Jair Antonio, and Escribano-Subias, 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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37. Genetic background in pediatric pulmonary arterial hypertension. Should we change the current recommendations for genetic testing?

Author

Cruz Utrilla, A, primary, Gallego, N, additional, Torrent, A, additional, Garrido-Lestache, E, additional, Guillen, I, additional, Arias, S, additional, Moya, A, additional, Mendoza, A, additional, Espin, J, additional, Rodriguez Vazquez, M M, additional, Playan-Escribano, J, additional, Labrandero, C, additional, Tenorio Castano, J A, additional, Escribano Subias, P, additional, and Del Cerro, M J, additional

Published
2021
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39. Eisenmenger syndrome: is it the same entity in adulthood and pediatric age?

Author

Playan Escribano, J, primary, Garrido-Lestache, E, additional, Cristo Ropero, M J, additional, Alvarez Fuente, M, additional, Barbera, J A, additional, Moreno Galdo, A, additional, Rueda Soriano, J, additional, Rodriguez Ogando, A, additional, Elias Hernandez, T, additional, Guillen Rodriguez, I, additional, Lazaro Salvador, M, additional, Lozano Balseiro, M, additional, Lara Padron, A, additional, Escribano Subias, P, additional, and Del Cerro Marin, M J, additional

Published
2021
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40. The results of the balloon pulmonary angioplasty program in chronic thromboembolic pulmonary hypertension patients in a national and European reference center resemble those of Japanese series

Author

Velazquez Martin, M T, primary, Albarran, A, additional, Sarnago, F, additional, Maneiro, N M, additional, Huertas, S, additional, Olazabal, A, additional, Cristo Ropero, M J, additional, Cruz Utrilla, A, additional, Perez Olivares, C, additional, Segura De La Cal, T, additional, Alonso Charterina, S, additional, Revilla Ostolaza, Y, additional, Ruiz Curiel, A, additional, Otero Escudero, M, additional, and Escribano Subias, P, additional

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

Author

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

Abstract

Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice. Methods: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat. Patients were followed for at least 1 year and up to 4 years from enrollment or until 30 days after stopping riociguat treatment. Primary safety outcomes were adverse events (AEs) and serious adverse events (SAEs) coded using Medical Dictionary for Regulatory Activities preferred terms and System Organ Classes version 21.0, collected during routine clinic visits (usually every 3–6 months) and collated via case report forms. Results: In total, 326 patients with PAH were included in the analysis. The most common AEs in these patients were dizziness (11.7%), right ventricular (RV)/cardiac failure (10.7%), edema/peripheral edema (10.7%), diarrhea (8.6%), dyspnea (8.0%), and cough (7.7%). The most common SAEs were RV/cardiac failure (10.1%), pneumonia (6.1%), dyspnea (4.0%), and syncope (3.4%). The exposure-adjusted rate of hemoptysis/pulmonary hemorrhage was 2.5 events per 100 patient-years. Conclusion: Final data from EXPERT show that in patients with PAH, the safety of riociguat in clinical practice was consistent with clinical trials, with no new safety concerns identified and a lower exposure-adjusted rate of hemoptysis/pulmonary hemorrhage than in the long-term extension of the Phase 3 trial in PAH. © 2020 The Author(s)

Published
2021

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

Author

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

Abstract

Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice. Methods: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat. Patients were followed for at least 1 year and up to 4 years from enrollment or until 30 days after stopping riociguat treatment. Primary safety outcomes were adverse events (AEs) and serious adverse events (SAEs) coded using Medical Dictionary for Regulatory Activities preferred terms and System Organ Classes version 21.0, collected during routine clinic visits and collated via case report forms. Results: In total, 956 patients with CTEPH were included in the analysis. The most common AEs in these patients were peripheral edema/edema (11.7%), dizziness (7.5%), right ventricular (RV)/cardiac failure (7.7%), and pneumonia (5.0%). The most common SAEs were RV/cardiac failure (7.4%), pneumonia (4.1%), dyspnea (3.6%), and syncope (2.5%). Exposure-adjusted rates of hemoptysis/pulmonary hemorrhage and hypotension were low and comparable to those in the long-term extension study of riociguat (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase–Stimulator Trial [CHEST-2]). Conclusion: Data from EXPERT show that in patients with CTEPH, the safety of riociguat in routine practice was consistent with the known safety profile of the drug, and no new safety concerns were identified. © 2020 The Authors

Published
2021

45. Expanding the Evidence of a Semi-Dominant Inheritance in GDF2 Associated with Pulmonary Arterial Hypertension

Author

Gallego N, Cruz-Utrilla A, Guillén I, Bonora AM, Ochoa N, Arias P, Lapunzina P, Escribano-Subias P, Nevado J, and Tenorio-Castaño J

Subjects
genomic medicine, personalized medicine, GDF2, pulmonary arterial hypertension, hereditary hemorrhagic telangiectasia, massive parallel sequencing, NGS
Abstract

Pulmonary arterial hypertension (PAH) sometimes co-exists with hereditary hemorrhagic telangiectasia (HHT). Despite being clinically diagnosable according to Curaçao criteria, HHT can be difficult to diagnose due to its clinically heterogenicity and highly overlapping with PAH. Genetic analysis of the associated genes ACVRL1, ENG, SMAD4 and GDF2 can help to confirm or discard the presumptive diagnosis. As part of the clinical routine and to establish a genetic diagnosis, we have analyzed a cohort of patients with PAH and overlapping HHT features through a customized Next Generation Sequencing (NGS) panel of 21 genes, designed and validated in-house. We detected a homozygous missense variant in GDF2 in a pediatric patient diagnosed with PAH associated with HHT and a missense variant along with a heterozygous deletion in another idiopathic PAH patient (compound heterozygous inheritance). In order to establish variant segregation, we analyzed all available family members. In both cases, parents were carriers for the variants, but neither was affected. Our results expand the clinical spectrum and the inheritance pattern associated with GDF2 pathogenic variants suggesting incomplete penetrance and/or variability of expressivity with a semi-dominant pattern of inheritance.

Published
2021

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

Author

Ghofrani, H.-A. Gomez Sanchez, M.-A. Humbert, M. Pittrow, D. Simonneau, G. Gall, H. Grünig, E. Klose, H. Halank, M. Langleben, D. Snijder, R.J. Escribano Subias, P. Mielniczuk, L.M. Lange, T.J. Vachiéry, J.-L. Wirtz, H. Helmersen, D.S. Tsangaris, I. Barberá, J.A. Pepke-Zaba, J. Boonstra, A. Rosenkranz, S. Ulrich, S. Steringer-Mascherbauer, R. Delcroix, M. Jansa, P. Šimková, I. Giannakoulas, G. Klotsche, J. Williams, E. Meier, C. Hoeper, M.M. Caneva, J. Tuhay, G. Diez, M. Talavera, M.L. Acosta, A. Vulcano, N. Bosio, M. Maldonado, L. Deleo, S. Melatini, L. Keogh, A. Kotlyar, E. Feenstra, J. Dwyer, N. Adams, H. Stevens, W. Steele, P. Proudman, S. Minson, R. Reeves, G. Lavender, M. Ng, B. Mackenzie, M. Barry, L. Gruenberger, M. Huber, C. Lang, I. Tilea, I. Sadushi-Kolici, R. Löffler-Ragg, J. Feistmantl, L.-T. Evrard, P. Louis, R. Guiot, J. Naldi, M. De Pauw, M. Mehta, S. Camacho, R.C. Tovar, P.P. Londoño, A. Campo, F. Garcia, P. Lema, C. Orozco-Levi, M. Martinez, W. Gomez, J.E. Nielsen-Kudsk, J.E. Mellemkjaer, S. Anton, L. Altraja, A. Vihinen, T. Vasankari, T. Sitbon, O. Cottin, V. Têtu, L. Noël-Savina, E. Shearman, N. Tayler, S. Olzik, I. Kulka, C. Grimminger, J. Simon, M. Nolde, A. Oqueka, T. Harbaum, L. Egenlauf, B. Ewert, R. Schulz, C. Regotta, S. Kramer, T. Knoop-Busch, S. Gerhardt, F. Konstantinides, S. Pitsiou, G. Stanopoulos, I. Sourla, E. Mouratoglou, S. Karvounis, H. Pappas, A. Georgopoulos, D. Fanaridis, M. Mitrouska, I. Michalis, L. Pappas, K. Kotsia, A. Gaine, S. Vizza, C.D. Manzi, G. Poscia, R. Badagliacca, R. Agostoni, P. Bruno, N. Farina, S. D'Alto, M. Argiento, P. Correra, A. Di Marco, G.M. Cresci, C. Vannucchi, V. Torricelli, E. Garcea, A. Pesci, A. Sardella, L. Paciocco, G. Pane, F. D'Armini, A.M. Pin, M. Grazioli, V. Massola, G. Sciortino, A. Prediletto, R. Bauleo, C. Airò, E. Ndreu, R. Pavlickova, I. Lunardi, C. Mulè, M. Farruggio, S. Costa, S. Galgano, G. Petruzzi, M. De Luca, A. Lombardi, F. Roncon, L. Conte, L. Picariello, C. Wirtz, G. and Ghofrani, H.-A. Gomez Sanchez, M.-A. Humbert, M. Pittrow, D. Simonneau, G. Gall, H. Grünig, E. Klose, H. Halank, M. Langleben, D. Snijder, R.J. Escribano Subias, P. Mielniczuk, L.M. Lange, T.J. Vachiéry, J.-L. Wirtz, H. Helmersen, D.S. Tsangaris, I. Barberá, J.A. Pepke-Zaba, J. Boonstra, A. Rosenkranz, S. Ulrich, S. Steringer-Mascherbauer, R. Delcroix, M. Jansa, P. Šimková, I. Giannakoulas, G. Klotsche, J. Williams, E. Meier, C. Hoeper, M.M. Caneva, J. Tuhay, G. Diez, M. Talavera, M.L. Acosta, A. Vulcano, N. Bosio, M. Maldonado, L. Deleo, S. Melatini, L. Keogh, A. Kotlyar, E. Feenstra, J. Dwyer, N. Adams, H. Stevens, W. Steele, P. Proudman, S. Minson, R. Reeves, G. Lavender, M. Ng, B. Mackenzie, M. Barry, L. Gruenberger, M. Huber, C. Lang, I. Tilea, I. Sadushi-Kolici, R. Löffler-Ragg, J. Feistmantl, L.-T. Evrard, P. Louis, R. Guiot, J. Naldi, M. De Pauw, M. Mehta, S. Camacho, R.C. Tovar, P.P. Londoño, A. Campo, F. Garcia, P. Lema, C. Orozco-Levi, M. Martinez, W. Gomez, J.E. Nielsen-Kudsk, J.E. Mellemkjaer, S. Anton, L. Altraja, A. Vihinen, T. Vasankari, T. Sitbon, O. Cottin, V. Têtu, L. Noël-Savina, E. Shearman, N. Tayler, S. Olzik, I. Kulka, C. Grimminger, J. Simon, M. Nolde, A. Oqueka, T. Harbaum, L. Egenlauf, B. Ewert, R. Schulz, C. Regotta, S. Kramer, T. Knoop-Busch, S. Gerhardt, F. Konstantinides, S. Pitsiou, G. Stanopoulos, I. Sourla, E. Mouratoglou, S. Karvounis, H. Pappas, A. Georgopoulos, D. Fanaridis, M. Mitrouska, I. Michalis, L. Pappas, K. Kotsia, A. Gaine, S. Vizza, C.D. Manzi, G. Poscia, R. Badagliacca, R. Agostoni, P. Bruno, N. Farina, S. D'Alto, M. Argiento, P. Correra, A. Di Marco, G.M. Cresci, C. Vannucchi, V. Torricelli, E. Garcea, A. Pesci, A. Sardella, L. Paciocco, G. Pane, F. D'Armini, A.M. Pin, M. Grazioli, V. Massola, G. Sciortino, A. Prediletto, R. Bauleo, C. Airò, E. Ndreu, R. Pavlickova, I. Lunardi, C. Mulè, M. Farruggio, S. Costa, S. Galgano, G. Petruzzi, M. De Luca, A. Lombardi, F. Roncon, L. Conte, L. Picariello, C. Wirtz, G.

Abstract

Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice. Methods: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat. Patients were followed for at least 1 year and up to 4 years from enrollment or until 30 days after stopping riociguat treatment. Primary safety outcomes were adverse events (AEs) and serious adverse events (SAEs) coded using Medical Dictionary for Regulatory Activities preferred terms and System Organ Classes version 21.0, collected during routine clinic visits and collated via case report forms. Results: In total, 956 patients with CTEPH were included in the analysis. The most common AEs in these patients were peripheral edema/edema (11.7%), dizziness (7.5%), right ventricular (RV)/cardiac failure (7.7%), and pneumonia (5.0%). The most common SAEs were RV/cardiac failure (7.4%), pneumonia (4.1%), dyspnea (3.6%), and syncope (2.5%). Exposure-adjusted rates of hemoptysis/pulmonary hemorrhage and hypotension were low and comparable to those in the long-term extension study of riociguat (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase–Stimulator Trial [CHEST-2]). Conclusion: Data from EXPERT show that in patients with CTEPH, the safety of riociguat in routine practice was consistent with the known safety profile of the drug, and no new safety concerns were identified. © 2020 The Authors

Published
2021

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

Author

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

Abstract

OBJECTIVE: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice. METHODS: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat. Patients were followed for at least 1 year and up to 4 years from enrollment or until 30 days after stopping riociguat treatment. Primary safety outcomes were adverse events (AEs) and serious adverse events (SAEs) coded using Medical Dictionary for Regulatory Activities preferred terms and System Organ Classes version 21.0, collected during routine clinic visits and collated via case report forms. RESULTS: In total, 956 patients with CTEPH were included in the analysis. The most common AEs in these patients were peripheral edema/edema (11.7%), dizziness (7.5%), right ventricular (RV)/cardiac failure (7.7%), and pneumonia (5.0%). The most common SAEs were RV/cardiac failure (7.4%), pneumonia (4.1%), dyspnea (3.6%), and syncope (2.5%). Exposure-adjusted rates of hemoptysis/pulmonary hemorrhage and hypotension were low and comparable to those in the long-term extension study of riociguat (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase-Stimulator Trial [CHEST-2]). CONCLUSION: Data from EXPERT show that in patients with CTEPH, the safety of riociguat in routine practice was consistent with the known safety profile of the drug, and no new safety concerns were identified.

Published
2021

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

Author

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

Abstract

Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice. Methods: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat. Patients were followed for at least 1 year and up to 4 years from enrollment or until 30 days after stopping riociguat treatment. Primary safety outcomes were adverse events (AEs) and serious adverse events (SAEs) coded using Medical Dictionary for Regulatory Activities preferred terms and System Organ Classes version 21.0, collected during routine clinic visits and collated via case report forms. Results: In total, 956 patients with CTEPH were included in the analysis. The most common AEs in these patients were peripheral edema/edema (11.7%), dizziness (7.5%), right ventricular (RV)/cardiac failure (7.7%), and pneumonia (5.0%). The most common SAEs were RV/cardiac failure (7.4%), pneumonia (4.1%), dyspnea (3.6%), and syncope (2.5%). Exposure-adjusted rates of hemoptysis/pulmonary hemorrhage and hypotension were low and comparable to those in the long-term extension study of riociguat (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase–Stimulator Trial [CHEST-2]). Conclusion: Data from EXPERT show that in patients with CTEPH, the safety of riociguat in routine practice was consistent with the known safety profile of the drug, and no new safety concerns were identified.

Published
2021

49. Idiopathic pulmonary hypertension in Spanish pediatric registry: clinical characterization, management, and risk factors for survival

Author

Playan Escribano, J, primary, Segura De La Cal, T, additional, Escribano Subias, P, additional, Mendoza Soto, A, additional, Labrandero, C, additional, Guillen Rodriguez, I, additional, Caicedo Cuenca, L.M, additional, Sabate Rotes, A, additional, Villagra Albert, S, additional, Lopez Ramon, M, additional, Conejo Munoz, L, additional, Izquierdo Riezu, M.A, additional, Inigo Martin, G, additional, Escriba Bori, S, additional, and Del Cerro Marin, M.J, additional

Published
2020
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50. Pulmonary hypertension and congenital heart disease: medical treatment and risk factors for survival

Author

Playan Escribano, J, primary, Segura De La Cal, T, additional, Segovia Cubero, J, additional, Rueda Soriano, J, additional, Garcia Hernandez, F.J, additional, Lopez Meseguer, M, additional, Perez Penate, G.M, additional, Lara Padron, A, additional, Campo Ezquibela, A, additional, Sala Llinas, E, additional, Mombiela, T, additional, Guerra Ramos, F.J, additional, Samper, G.J, additional, Blanco, I, additional, and Escribano Subias, P, additional

Published
2020
Full Text
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