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1. 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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2. 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. 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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14. 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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15. 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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17. Evaluation and management of patients with chronic thromboembolic pulmonary hypertension - consensus statement from the ISHLT

Author

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

Published
2021
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18. Bosentan for Treatment of Inoperable Chronic Thromboembolic Pulmonary Hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a Randomized, Placebo-Controlled Trial

Author

Jaïs, Xavier, D'Armini, Andrea M., Jansa, Pavel, Torbicki, Adam, Delcroix, Marion, Ghofrani, Hossein A., Hoeper, Marius M., Lang, Irene M., Mayer, Eckhard, Pepke-Zaba, Joanna, Perchenet, Loïc, Morganti, Adele, Simonneau, Gérald, and Rubin, Lewis J.

Published
2008
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25. Long-term Outcome after Pulmonary Endarterectomy

Author

Corsico, Angelo G., DʼArmini, Andrea M., Cerveri, Isa, Klersy, Catherine, Ansaldo, Elena, Niniano, Rosanna, Gatto, Elena, Monterosso, Cristian, Morsolini, Marco, Nicolardi, Salvatore, Tramontin, Corrado, Pozzi, Ernesto, and Viganò, Mario

Published
2008
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31. RETRACTED: 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

Lang, Irene, Kähler, Christian, Delcroix, Marion, Bshouty, Zoheir, Varela, Pablo Sepulveda, Jing, Zhi-Cheng, Yang, Yuanhua, Liu, Jinming, Zhang, Gangcheng, Zhang, Nuofu, Mi, Yuhong, Zhu, Xianyang, Jansa, Pavel, Jaïs, Xavier, Prévot, Grégoire, Bouvaist, Hélène, Sanchez, Olivier, Grimminger, Friedrich, Held, Matthias, Wilkens, Heinrike, Rosenkranz, Stephan, Grünig, Ekkehard, Karlócai, Kristóf, Temesvári, András, Edes, Istvan, Aidietienė, Sigita, Miliauskas, Skaidrius, Zamudio, Tomas Rene Pulido, Sanchez, Carlos Jerjes, Noordegraaf, Anton Vonk, Lewczuk, Jerzy, Podolec, Piotr, Kasprzak, Jarosław, Mularek-Kubzdela, Tatiana, Grzywna, Ryszard, Dheda, Keertan, Moiseeva, Olga, Chernyavskiy, Alexander, Shipulin, Vladimir, Barbarash, Olga, Martynyuk, Tamila, Kim, Hyung-Kwan, Park, Jun-Bean, Lee, Jae Seung, Speich, Rudolf, Ulrich, Silvia, Aubert, John-David, Phrommintikul, Arintaya, Jaimchariyatam, Nattapong, Sompradeekul, Suree, Onen, Zeynep Pinar, Okumus, Gulfer, Solovey, Lyubomyr, Gavrysyuk, Volodymyr, Howard, Luke, Pepke-Zaba, Joanna, Condliffe, Robin, McConnell, John, Kerr, Kim, Nguyen, Lan Hieu, Pham, Nguyen Vinh, Ghofrani, Hossein-Ardeschir, Simonneau, Gérald, D'Armini, Andrea M, Fedullo, Peter, Howard, Luke S, Jenkins, David P, Madani, Michael M, Martin, Nicolas, Mayer, Eckhard, Papadakis, Kelly, Richard, Dominik, and Kim, Nick H

Published
2017
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34. Riociguat in patients with chronic thromboembolic pulmonary hypertension: results from an early access study

Author

McLaughlin, Vallerie V., primary, Jansa, Pavel, additional, Nielsen-Kudsk, Jens E., additional, Halank, Michael, additional, Simonneau, Gérald, additional, Grünig, Ekkehard, additional, Ulrich, Silvia, additional, Rosenkranz, Stephan, additional, Gómez Sánchez, Miguel A., additional, Pulido, Tomás, additional, Pepke-Zaba, Joanna, additional, Barberá, Joan Albert, additional, Hoeper, Marius M., additional, Vachiéry, Jean-Luc, additional, Lang, Irene, additional, Carvalho, Francine, additional, Meier, Christian, additional, Mueller, Katharina, additional, Nikkho, Sylvia, additional, and D’Armini, Andrea M., additional

Published
2017
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35. Riociguat in patients with chronic thromboembolic pulmonary hypertension: results from an early access study

Author

McLaughlin, Vallerie V., Jansa, Pavel, Nielsen-Kudsk, Jens E., Halank, Michael, Simonneau, Gerald, Gruenig, Ekkehard, Ulrich, Silvia, Rosenkranz, Stephan, Gomez Sanchez, Miguel A., Pulido, Tomas, Pepke-Zaba, Joanna, Albert Barbera, Joan, Hoeper, Marius M., Vachiery, Jean-Luc, Lang, Irene, Carvalho, Francine, Meier, Christian, Mueller, Katharina, Nikkho, Sylvia, D'Armini, Andrea M., McLaughlin, Vallerie V., Jansa, Pavel, Nielsen-Kudsk, Jens E., Halank, Michael, Simonneau, Gerald, Gruenig, Ekkehard, Ulrich, Silvia, Rosenkranz, Stephan, Gomez Sanchez, Miguel A., Pulido, Tomas, Pepke-Zaba, Joanna, Albert Barbera, Joan, Hoeper, Marius M., Vachiery, Jean-Luc, Lang, Irene, Carvalho, Francine, Meier, Christian, Mueller, Katharina, Nikkho, Sylvia, and D'Armini, Andrea M.

Abstract

Background: Following positive results from the Phase III CHEST-1 study in patients with inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH), the Phase IIIb CTEPH early access study (EAS) was designed to assess the safety and tolerability of riociguat in real-world clinical practice, as well as to provide patients with early access to riociguat before launch. Riociguat is approved for the treatment of inoperable and persistent/recurrent CTEPH. Methods: We performed an open-label, uncontrolled, single-arm, early access study in which 300 adult patients with inoperable or persistent/recurrent CTEPH received riociguat adjusted from 1 mg three times daily (tid) to a maximum of 2.5 mg tid. Patients switching from unsatisfactory prior pulmonary arterial hypertension (PAH)-targeted therapy (n = 84) underwent a washout period of at least 3 days before initiating riociguat. The primary aim was to assess the safety and tolerability of riociguat, with World Health Organization functional class and 6-min walking distance (6MWD) as exploratory efficacy endpoints. Results: In total, 262 patients (87%) completed study treatment and entered the safety follow-up (median treatment duration 47 weeks). Adverse events were reported in 273 patients (91%). The most frequently reported serious adverse events were syncope (6%), right ventricular failure (3%), and pneumonia (2%). There were five deaths, none of which was considered related to study medication. The safety and tolerability of riociguat was similar in patients switched from other PAH-targeted therapies and those who were treatment naive. In patients with data available, mean +/- standard deviation 6MWD had increased by 33 +/- 42 m at Week 12 with no clinically relevant differences between the switched and treatment-naive subgroups. Conclusions: Riociguat was well tolerated in patients with CTEPH who were treatment naive, and in those who were switched from other PAH-targeted therapies. No ne

Published
2017

36. Riociguat in patients with chronic thromboembolic pulmonary hypertension: results from an early access study

Author

McLaughlin, Vallerie V, Jansa, Pavel, Nielsen-Kudsk, Jens E, Halank, Michael, Simonneau, Gérald, Grünig, Ekkehard, Ulrich, Silvia, Rosenkranz, Stephan, Gómez Sánchez, Miguel A, Pulido, Tomás, Pepke-Zaba, Joanna, Barberá, Joan Albert, Hoeper, Marius M, Vachiéry, Jean-Luc, Lang, Irene, Carvalho, Francine, Meier, Christian, Mueller, Katharina, Nikkho, Sylvia, D'Armini, Andrea M, McLaughlin, Vallerie V, Jansa, Pavel, Nielsen-Kudsk, Jens E, Halank, Michael, Simonneau, Gérald, Grünig, Ekkehard, Ulrich, Silvia, Rosenkranz, Stephan, Gómez Sánchez, Miguel A, Pulido, Tomás, Pepke-Zaba, Joanna, Barberá, Joan Albert, Hoeper, Marius M, Vachiéry, Jean-Luc, Lang, Irene, Carvalho, Francine, Meier, Christian, Mueller, Katharina, Nikkho, Sylvia, and D'Armini, Andrea M

Abstract

BACKGROUND Following positive results from the Phase III CHEST-1 study in patients with inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH), the Phase IIIb CTEPH early access study (EAS) was designed to assess the safety and tolerability of riociguat in real-world clinical practice, as well as to provide patients with early access to riociguat before launch. Riociguat is approved for the treatment of inoperable and persistent/recurrent CTEPH. METHODS We performed an open-label, uncontrolled, single-arm, early access study in which 300 adult patients with inoperable or persistent/recurrent CTEPH received riociguat adjusted from 1 mg three times daily (tid) to a maximum of 2.5 mg tid. Patients switching from unsatisfactory prior pulmonary arterial hypertension (PAH)-targeted therapy (n = 84) underwent a washout period of at least 3 days before initiating riociguat. The primary aim was to assess the safety and tolerability of riociguat, with World Health Organization functional class and 6-min walking distance (6MWD) as exploratory efficacy endpoints. RESULTS In total, 262 patients (87%) completed study treatment and entered the safety follow-up (median treatment duration 47 weeks). Adverse events were reported in 273 patients (91%). The most frequently reported serious adverse events were syncope (6%), right ventricular failure (3%), and pneumonia (2%). There were five deaths, none of which was considered related to study medication. The safety and tolerability of riociguat was similar in patients switched from other PAH-targeted therapies and those who were treatment naïve. In patients with data available, mean ± standard deviation 6MWD had increased by 33 ± 42 m at Week 12 with no clinically relevant differences between the switched and treatment-naïve subgroups. CONCLUSIONS Riociguat was well tolerated in patients with CTEPH who were treatment naïve, and in those who were switched from other PAH-targeted therapies. No new safety

Published
2017

37. 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, primary, Simonneau, Gérald, additional, D'Armini, Andrea M, additional, Fedullo, Peter, additional, Howard, Luke S, additional, Jaïs, Xavier, additional, Jenkins, David P, additional, Jing, Zhi-Cheng, additional, Madani, Michael M, additional, Martin, Nicolas, additional, Mayer, Eckhard, additional, Papadakis, Kelly, additional, Richard, Dominik, additional, Kim, Nick H, additional, Lang, Irene, additional, Kähler, Christian, additional, Delcroix, Marion, additional, Bshouty, Zoheir, additional, Varela, Pablo Sepulveda, additional, Yang, Yuanhua, additional, Liu, Jinming, additional, Zhang, Gangcheng, additional, Zhang, Nuofu, additional, Mi, Yuhong, additional, Zhu, Xianyang, additional, Jansa, Pavel, additional, Prévot, Grégoire, additional, Bouvaist, Hélène, additional, Sanchez, Olivier, additional, Grimminger, Friedrich, additional, Held, Matthias, additional, Wilkens, Heinrike, additional, Rosenkranz, Stephan, additional, Grünig, Ekkehard, additional, Karlócai, Kristóf, additional, Temesvári, András, additional, Edes, Istvan, additional, Aidietienė, Sigita, additional, Miliauskas, Skaidrius, additional, Zamudio, Tomas Rene Pulido, additional, Sanchez, Carlos Jerjes, additional, Noordegraaf, Anton Vonk, additional, Lewczuk, Jerzy, additional, Podolec, Piotr, additional, Kasprzak, Jarosław, additional, Mularek-Kubzdela, Tatiana, additional, Grzywna, Ryszard, additional, Dheda, Keertan, additional, Moiseeva, Olga, additional, Chernyavskiy, Alexander, additional, Shipulin, Vladimir, additional, Barbarash, Olga, additional, Martynyuk, Tamila, additional, Kim, Hyung-Kwan, additional, Park, Jun-Bean, additional, Lee, Jae Seung, additional, Speich, Rudolf, additional, Ulrich, Silvia, additional, Aubert, John-David, additional, Phrommintikul, Arintaya, additional, Jaimchariyatam, Nattapong, additional, Sompradeekul, Suree, additional, Onen, Zeynep Pinar, additional, Okumus, Gulfer, additional, Solovey, Lyubomyr, additional, Gavrysyuk, Volodymyr, additional, Howard, Luke, additional, Pepke-Zaba, Joanna, additional, Condliffe, Robin, additional, McConnell, John, additional, Kerr, Kim, additional, Nguyen, Lan Hieu, additional, and Pham, Nguyen Vinh, additional

Published
2017
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38. Macitentan for inoperable chronic thromboembolic pulmonary hypertension (CTEPH): results from the randomised controlled MERIT study

Author

Simonneau, Gérald, primary, Jing, Zhi C., additional, D'Armini, Andrea M., additional, Fedullo, Peter, additional, Howard, Luke, additional, Jaïs, Xavier, additional, Jenkins, David P., additional, Kim, Nick H., additional, Madani, Michael, additional, Martin, Nicholas, additional, Mayer, Eckhard, additional, Papadakis, Kelly, additional, Richard, Dominik, additional, and Ghofrani, Hossein A., additional

Published
2017
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39. Haemodynamic effects of riociguat in inoperable/recurrent chronic thromboembolic pulmonary hypertension

Author

Kim, Nick H, primary, D'Armini, Andrea M, additional, Grimminger, Friedrich, additional, Grünig, Ekkehard, additional, Hoeper, Marius M, additional, Jansa, Pavel, additional, Mayer, Eckhard, additional, Neurohr, Claus, additional, Simonneau, Gérald, additional, Torbicki, Adam, additional, Wang, Chen, additional, Fritsch, Arno, additional, Davie, Neil, additional, and Ghofrani, Hossein-Ardeschir, additional

Published
2016
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42. Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension

Author

Delcroix, Marion, primary, Lang, Irene, additional, Pepke-Zaba, Joanna, additional, Jansa, Pavel, additional, D’Armini, Andrea M., additional, Snijder, Repke, additional, Bresser, Paul, additional, Torbicki, Adam, additional, Mellemkjaer, Sören, additional, Lewczuk, Jerzy, additional, Simkova, Iveta, additional, Barberà, Joan A., additional, de Perrot, Marc, additional, Hoeper, Marius M., additional, Gaine, Sean, additional, Speich, Rudolf, additional, Gomez-Sanchez, Miguel A., additional, Kovacs, Gabor, additional, Jaïs, Xavier, additional, Ambroz, David, additional, Treacy, Carmen, additional, Morsolini, Marco, additional, Jenkins, David, additional, Lindner, Jaroslav, additional, Dartevelle, Philippe, additional, Mayer, Eckhard, additional, and Simonneau, Gérald, additional

Published
2016
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44. Haemodynamic effects of riociguat in inoperable/recurrent chronic thromboembolic pulmonary hypertension.

Author

Kim, Nick H., D'Armini, Andrea M., Grimminger, Friedrich, Grünig, Ekkehard, Hoeper, Marius M., Jansa, Pavel, Mayer, Eckhard, Neurohr, Claus, Simonneau, Gérald, Torbicki, Adam, Chen Wang, Fritsch, Arno, Davie, Neil, Ghofrani, Hossein-Ardeschir, and Wang, Chen

Subjects
HYPERTENSION, THERAPEUTICS, THROMBOEMBOLISM, PULMONARY artery, PULMONARY artery diseases, ENDARTERECTOMY
Abstract

Objective: We compared the haemodynamic effects of riociguat in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent/recurrent CTEPH after pulmonary endarterectomy in the Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase-Stimulator Trial 1 study.Methods: Patients with inoperable or persistent/recurrent CTEPH (n=261; mean± SD age 59±14 years; 66% women) were randomised to riociguat (up to 2.5 mg three times daily) or placebo. Haemodynamic parameters were assessed at baseline and week 16.Results: Riociguat decreased pulmonary vascular resistance (PVR) in inoperable (n=189; least-squares mean difference: -285 dyn s/cm5 (95% CI -357 to -213); p<0.0001) and persistent/recurrent (n=72; -131 dyn s/cm5 (95% CI -214 to -48); p=0.0025) patients. Cardiac index improved in inoperable patients by a least-squares mean difference of +0.6 L/min/m2 (95% CI 0.4 to 0.7; p<0.0001), while in persistent/recurrent patients the change was +0.2 L/min/m2 (95% CI -0.1 to 0.5; p=0.17). Mean pulmonary artery pressure decreased in inoperable and persistent/recurrent patients(-4.7 mm Hg (95% CI -6.9 to -2.6; p<0.0001 and -4.8 mm Hg (-8.2 to -1.5; p=0.0055), respectively). For all patients, changes in 6 min walk distance correlated with changes in PVR (r=-0.29 (95% CI -0.41 to -0.17); p<0.0001) and cardiac index (r=0.23 (95% CI 0.10 to 0.35); p=0.0004).Conclusions: Riociguat improved haemodynamics in patients with inoperable CTEPH or persistent/recurrent CTEPH.Trial Registration Number: NCT00855465. [ABSTRACT FROM AUTHOR]

Published
2017
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45. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2)

Author

Simonneau, Gérald, primary, D’Armini, Andrea M., additional, Ghofrani, Hossein-Ardeschir, additional, Grimminger, Friedrich, additional, Hoeper, Marius M., additional, Jansa, Pavel, additional, Kim, Nick H., additional, Wang, Chen, additional, Wilkins, Martin R., additional, Fritsch, Arno, additional, Davie, Neil, additional, Colorado, Pablo, additional, and Mayer, Eckhard, additional

Published
2014
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47. Long-term Riociguat Treatment in Inoperable and Persistent/Recurrent CTEPH Patients in WHO Functional Class (FC) I/II Versus FC III/IV at Baseline: Results From the 16-Week Phase III CHEST-1 Study and CHEST-2 Open-Label Extension

Author

Wang, Chen, primary, D'Armini, Andrea M, additional, Ghofrani, Hossein-Ardeschir, additional, Grimminger, Friedrich, additional, Hoeper, Marius M, additional, Jansa, Pavel, additional, Kim, Nick H, additional, Simonneau, Gerald, additional, Torbicki, Adam, additional, Wilkins, Martin, additional, Fritsch, Arno, additional, Davie, Neil, additional, and Mayer, Eckhard, additional

Published
2014
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48. Fibrinogen

Author

Arbustini, Eloisa, primary, Narula, Nupoor, additional, and D’Armini, Andrea M., additional

Published
2013
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50. Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Author

Pepke-Zaba, Joanna, primary, Delcroix, Marion, additional, Lang, Irene, additional, Mayer, Eckhard, additional, Jansa, Pavel, additional, Ambroz, David, additional, Treacy, Carmen, additional, D'Armini, Andrea M., additional, Morsolini, Marco, additional, Snijder, Repke, additional, Bresser, Paul, additional, Torbicki, Adam, additional, Kristensen, Bent, additional, Lewczuk, Jerzy, additional, Simkova, Iveta, additional, Barberà, Joan A., additional, de Perrot, Marc, additional, Hoeper, Marius M., additional, Gaine, Sean, additional, Speich, Rudolf, additional, Gomez-Sanchez, Miguel A., additional, Kovacs, Gabor, additional, Hamid, Abdul Monem, additional, Jaïs, Xavier, additional, and Simonneau, Gérald, additional

Published
2011
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