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52. Pulmonary vascular remodeling patterns and expression of general control nonderepressible 2 (GCN2) in pulmonary veno-occlusive disease

Author

Nossent, Esther J., Antigny, Fabrice, Montani, David, Bogaard, Harm Jan, Ghigna, Maria Rosa, Lambert, Mélanie, Thomas de Montpréville, Vincent, Girerd, Barbara, Jaïs, Xavier, Savale, Laurent, Mercier, Olaf, Fadel, Elie, Soubrier, Florent, Sitbon, Olivier, Simonneau, Gérald, Vonk Noordegraaf, Anton, Humbert, Marc, Perros, Frédéric, and Dorfmüller, Peter

Published
2018
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53. Highlights from the International Chronic Thromboembolic Pulmonary Hypertension Congress 2021

Author

Simonneau, Gérald, Fadel, Elie, Vonk Noordegraaf, Anton, Toshner, Mark, Lang, Irene M, Klok, Frederikus A, McInnis, Micheal C, Screaton, Nicholas, Madani, Michael M, Martinez, Guillermo, Salaunkey, Kiran, Jenkins, David P, Matsubara, Hiromi, Brénot, Philippe, Hoeper, Marius M, Ghofrani, Hossein A, Jaïs, Xavier, Wiedenroth, Christoph B, Guth, Stefan, Kim, Nick H, Pepke-Zaba, Joanna, Delcroix, Marion, Mayer, Eckhard, Vonk Noordegraaf, Anton [0000-0002-4057-758X], Klok, Frederikus A [0000-0001-9961-0754], McInnis, Micheal C [0000-0002-5725-3258], Matsubara, Hiromi [0000-0002-3417-7651], Hoeper, Marius M [0000-0001-9086-2293], Ghofrani, Hossein A [0000-0002-2029-4419], Guth, Stefan [0000-0003-2238-9574], Delcroix, Marion [0000-0001-8394-9809], and Apollo - University of Cambridge Repository

Subjects
Hypertension, Pulmonary, Angioplasty, Medical Physiology, Respiratory System, Anticoagulants, Pulmonary, Endarterectomy, Pulmonary Artery, Cardiovascular, Hypertension, Chronic Disease, Humans, 2.1 Biological and endogenous factors, Aetiology, Pulmonary Embolism, Lung, Angioplasty, Balloon, Balloon
Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism. It is caused by persistent obstruction of pulmonary arteries by chronic organised fibrotic clots, despite adequate anticoagulation. The pulmonary hypertension is also caused by concomitant microvasculopathy which may progress without timely treatment. Timely and accurate diagnosis requires the combination of imaging and haemodynamic assessment. Optimal therapy should be individualised to each case and determined by an experienced multidisciplinary CTEPH team with the ability to offer all current treatment modalities. This report summarises current knowledge and presents key messages from the International CTEPH Conference, Bad Nauheim, Germany, 2021. Sessions were dedicated to 1) disease definition; 2) pathophysiology, including the impact of the hypertrophied bronchial circulation, right ventricle (dys)function, genetics and inflammation; 3) diagnosis, early after acute pulmonary embolism, using computed tomography and perfusion techniques, and supporting the selection of appropriate therapies; 4) surgical treatment, pulmonary endarterectomy for proximal and distal disease, and peri-operative management; 5) percutaneous approach or balloon pulmonary angioplasty, techniques and complications; and 6) medical treatment, including anticoagulation and pulmonary hypertension drugs, and in combination with interventional treatments. Chronic thromboembolic pulmonary disease without pulmonary hypertension is also discussed in terms of its diagnostic and therapeutic aspects.

Published
2023

54. Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: A COMPERA analysis

Author

Distler, Oliver, primary, Ofner, Christian, additional, Huscher, Dörte, additional, Jordan, Suzana, additional, Ulrich, Silvia, additional, Stähler, Gerd, additional, Grünig, Ekkehard, additional, Held, Matthias, additional, Ghofrani, H Ardeschir, additional, Claussen, Martin, additional, Lange, Tobias J, additional, Klose, Hans, additional, Rosenkranz, Stephan, additional, Vonk-Noordegraaf, Anton, additional, Vizza, C Dario, additional, Delcroix, Marion, additional, Opitz, Christian, additional, Pausch, Christine, additional, Scelsi, Laura, additional, Neurohr, Claus, additional, Olsson, Karen M, additional, Coghlan, J Gerry, additional, Halank, Michael, additional, Skowasch, Dirk, additional, Behr, Jürgen, additional, Milger, Katrin, additional, Remppis, Bjoern Andrew, additional, Skride, Andris, additional, Jureviciene, Elena, additional, Gumbiene, Lina, additional, Miliauskas, Skaidrius, additional, Löffler-Ragg, Judith, additional, Wilkens, Heinrike, additional, Pittrow, David, additional, Hoeper, Marius M, additional, and Ewert, Ralf, additional

Published
2023
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55. Discrimination Between Pre‐ and Postcapillary Pulmonary Hypertension Using Platelet RNA

Author

Arkani, Mohammad, primary, Kianzad, Azar, additional, Jansen, Samara, additional, Smit, Josien, additional, Post, Edward, additional, Ramaker, Jip, additional, Lagerweij, Tonny, additional, In’t Veld, Sjors G. J. G., additional, Noske, David P., additional, Vonk Noordegraaf, Anton, additional, Wurdinger, Thomas, additional, Best, Myron G., additional, and Bogaard, Harm‐Jan, additional

Published
2023
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56. Efficacy and safety of intravenous imatinib in COVID-19 ARDS: a randomized, double-blind, placebo-controlled clinical trial

Author

Atmowihardjo, Leila N., primary, Schippers, Job R., additional, Duijvelaar, Erik, additional, Bartelink, Imke H., additional, Bet, Pierre M., additional, Swart, Noortje E. L., additional, van Rein, Nienke, additional, Purdy, Keith, additional, Cavalla, David, additional, McElroy, Andrew, additional, Fritchley, Sarah, additional, Vonk Noordegraaf, Anton, additional, Endeman, Henrik, additional, van Velzen, Patricia, additional, Koopmans, Matty, additional, Bogaard, Harm Jan, additional, Heunks, Leo, additional, Juffermans, Nicole, additional, Schultz, Marcus J., additional, Tuinman, Pieter R., additional, Bos, Lieuwe D. J., additional, and Aman, Jurjan, additional

Published
2023
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61. 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, Kim, Nick H, Lang, Irene, Kähler, Christian, Delcroix, Marion, Bshouty, Zoheir, Sepulveda Varela, Pablo, 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, Pulido Zamudio, Tomas Rene, Jerjes Sanchez, Carlos, Vonk Noordegraaf, Anton, 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, and Pham, Nguyen Vinh

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.

Published
2024
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64. How to Incorporate Tricuspid Regurgitation in Right Ventricular-Pulmonary Arterial Coupling

Author

Yoshida, Keimei, Axelsen, Julie Birkmose, Saku, Keita, Andersen, Asger, de Man, Frances S., Sunagawa, Kenji, Vonk Noordegraaf, Anton, and Bogaard, Harm Jan

Subjects
Cardiac Catheterization, Physiology, Physiology (medical), Heart Ventricles, Hypertension, Pulmonary, Ventricular Dysfunction, Right, Ventricular Function, Right, Animals, Stroke Volume, Pulmonary Artery, Tricuspid Valve Insufficiency, Rats
Abstract

Adaptation of the right ventricle (RV) to a progressively increasing afterload is one of the hallmarks of pulmonary arterial hypertension (PAH). Pressure-volume loop analysis provides measures of load-independent RV contractility, i.e., end-systolic elastance, and pulmonary vascular properties, i.e., effective arterial elastance (Ea). However, PAH-induced RV overload potentially results in tricuspid regurgitation (TR). TR makes RV eject to both PA and right atrium; thereby, a ratio of RV end-systolic pressure (Pes) to RV stroke volume (SV) could not correctly define Ea. To overcome this limitation, we introduced a two-parallel compliance model, i.e., Ea = 1/(1/Epa + 1/ETR), while effective pulmonary arterial elastance (Epa = Pes/PASV) represents pulmonary vascular properties and effective tricuspid regurgitant elastance (ETR) represents TR. We conducted animal experiments to validate this framework. First, we performed SV analysis with a pressure-volume catheter in the RV and a flow probe at the aorta in rats with and without pressure-overloaded RV to determine the effect of inferior vena cava (IVC) occlusion on TR. A discordance between the two techniques was found in rats with pressure-overloaded RV, not in sham. This discordance diminished after IVC occlusion, suggesting that TR in pressure-overloaded RV was diminished by IVC occlusion. Next, we performed pressure-volume loop analysis in rats with pressure-overloaded RVs, calibrating RV volume by cardiac magnetic resonance. We found that IVC occlusion increased Ea, suggesting that a reduction of TR increased Ea. Using the proposed framework, Epa was indistinguishable to Ea post-IVC occlusion. We conclude that the proposed framework helps better understanding of the pathophysiology of PAH and associated right heart failure.NEW & NOTEWORTHY This study reveals the impact of tricuspid regurgitation on pressure-volume loop analysis in right ventricle pressure overload. By introducing a novel concept of parallel compliances in the pressure-volume loop analysis, a better description is provided for the right ventricular forward afterload in the presence of tricuspid regurgitation.

Published
2023

68. Automated Quantification of the Pulmonary Vasculature in Pulmonary Embolism and Chronic Thromboembolic Pulmonary Hypertension

Author

Zhai, Zhiwei, primary, J.A.M. Boon, Gudula, additional, Staring, Marius, additional, van Dam, Lisette F., additional, J.M. Kroft, Lucia, additional, Hernández Girón, Irene, additional, K. Ninaber, Maarten, additional, Bogaard, Harm Jan, additional, J. Meijboom, Lilian, additional, Vonk Noordegraaf, Anton, additional, Huisman, Menno V., additional, Klok, Frederikus A., additional, and Stoel, Berend C., additional

Published
2023
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69. Chronic thromboembolic pulmonary hypertension and clot resolution after COVID-19-associated pulmonary embolism

Author

de Jong, Cindy M.M., primary, Visser, Chantal, additional, Bemelmans, Remy H.H., additional, Boersma, Wim G., additional, van den Borst, Bram, additional, Burggraaf, J. Louise I., additional, Cannegieter, Suzanne C., additional, ten Cate-Hoek, Arina J., additional, Croles, F. Nanne, additional, Faber, Harald J., additional, Faber, Laura M., additional, Hellemons, Merel E., additional, Hessels, Lisa M., additional, Huisman, Menno V., additional, Kamphuisen, Pieter W., additional, Koster, Stephanie C.E., additional, Kroft, Lucia J.M., additional, van der Lee, Ivo, additional, Leentjens, Jenneke, additional, Meijer, Karina, additional, Ninaber, Maarten K., additional, Sondermeijer, Brigitte M., additional, Stads, Susanne, additional, Vonk Noordegraaf, Anton, additional, Winckers, Kristien, additional, Kruip, Marieke J.H.A., additional, and Klok, Frederikus A., additional

Published
2023
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71. Right Ventricular Function During Exercise After Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension

Author

Braams, Natalia J., primary, Kianzad, Azar, additional, Meijboom, Lilian J., additional, Westenberg, Jesper, additional, Spruijt, Onno A., additional, Smits, Josien, additional, Vonk Noordegraaf, Anton, additional, Boonstra, Anco, additional, Nossent, Esther J., additional, Oosterveer, Frank, additional, Handoko, M. Louis, additional, Symersky, Petr, additional, de Man, Frances S., additional, and Bogaard, Harm Jan, additional

Published
2023
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72. Highlights from the International Chronic Thromboembolic Pulmonary Hypertension Congress 2021

Author

Simonneau, Gérald, primary, Fadel, Elie, additional, Vonk Noordegraaf, Anton, additional, Toshner, Mark, additional, Lang, Irene M., additional, Klok, Frederikus A., additional, McInnis, Micheal C., additional, Screaton, Nicholas, additional, Madani, Michael M., additional, Martinez, Guillermo, additional, Salaunkey, Kiran, additional, Jenkins, David P., additional, Matsubara, Hiromi, additional, Brénot, Philippe, additional, Hoeper, Marius M., additional, Ghofrani, Hossein A., additional, Jaïs, Xavier, additional, Wiedenroth, Christoph B., additional, Guth, Stefan, additional, Kim, Nick H., additional, Pepke-Zaba, Joanna, additional, Delcroix, Marion, additional, and Mayer, Eckhard, additional

Published
2023
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73. Efficacy and safety of intravenous imatinib in COVID-19 ARDS:a randomized, double-blind, placebo-controlled clinical trial

Author

Atmowihardjo, Leila N., Schippers, Job R., Duijvelaar, Erik, Bartelink, Imke H., Bet, Pierre M., Swart, Noortje E.L., van Rein, Nienke, Purdy, Keith, Cavalla, David, McElroy, Andrew, Fritchley, Sarah, Vonk Noordegraaf, Anton, Endeman, Henrik, van Velzen, Patricia, Koopmans, Matty, Bogaard, Harm Jan, Heunks, Leo, Juffermans, Nicole, Schultz, Marcus J., Tuinman, Pieter R., Bos, Lieuwe D.J., Aman, Jurjan, Atmowihardjo, Leila N., Schippers, Job R., Duijvelaar, Erik, Bartelink, Imke H., Bet, Pierre M., Swart, Noortje E.L., van Rein, Nienke, Purdy, Keith, Cavalla, David, McElroy, Andrew, Fritchley, Sarah, Vonk Noordegraaf, Anton, Endeman, Henrik, van Velzen, Patricia, Koopmans, Matty, Bogaard, Harm Jan, Heunks, Leo, Juffermans, Nicole, Schultz, Marcus J., Tuinman, Pieter R., Bos, Lieuwe D.J., and Aman, Jurjan

Abstract

Purpose: A hallmark of acute respiratory distress syndrome (ARDS) is hypoxaemic respiratory failure due to pulmonary vascular hyperpermeability. The tyrosine kinase inhibitor imatinib reversed pulmonary capillary leak in preclinical studies and improved clinical outcomes in hospitalized COVID-19 patients. We investigated the effect of intravenous (IV) imatinib on pulmonary edema in COVID-19 ARDS. Methods: This was a multicenter, randomized, double-blind, placebo-controlled trial. Invasively ventilated patients with moderate-to-severe COVID-19 ARDS were randomized to 200 mg IV imatinib or placebo twice daily for a maximum of seven days. The primary outcome was the change in extravascular lung water index (∆EVLWi) between days 1 and 4. Secondary outcomes included safety, duration of invasive ventilation, ventilator-free days (VFD) and 28-day mortality. Posthoc analyses were performed in previously identified biological subphenotypes. Results: 66 patients were randomized to imatinib (n = 33) or placebo (n = 33). There was no difference in ∆EVLWi between the groups (0.19 ml/kg, 95% CI − 3.16 to 2.77, p = 0.89). Imatinib treatment did not affect duration of invasive ventilation (p = 0.29), VFD (p = 0.29) or 28-day mortality (p = 0.79). IV imatinib was well-tolerated and appeared safe. In a subgroup of patients characterized by high IL-6, TNFR1 and SP-D levels (n = 20), imatinib significantly decreased EVLWi per treatment day (− 1.17 ml/kg, 95% CI − 1.87 to − 0.44). Conclusions: IV imatinib did not reduce pulmonary edema or improve clinical outcomes in invasively ventilated COVID-19 patients. While this trial does not support the use of imatinib in the general COVID-19 ARDS population, imatinib reduced pulmonary edema in a subgroup of patients, underscoring the potential value of predictive enrichment in ARDS trials. Trial registration NCT04794088 , registered 11 March 2021. European Clinical Trials Database (EudraCT number: 2020-005447-23).

Published
2023

74. Chronic thromboembolic pulmonary hypertension and clot resolution after COVID-19-associated pulmonary embolism

Author

de Jong, Cindy M.M., Visser, Chantal, Bemelmans, Remy H.H., Boersma, Wim G., van den Borst, Bram, Louise, J., Cannegieter, Suzanne C., ten Cate-Hoek, Arina J., Nanne Croles, F., Faber, Harald J., Faber, Laura M., Hellemons, Merel E., Hessels, Lisa M., Huisman, Menno V., Kamphuisen, Pieter W., Koster, Stephanie C.E., Kroft, Lucia J.M., van der Lee, Ivo, Leentjens, Jenneke, Meijer, Karina, Ninaber, Maarten K., Sondermeijer, Brigitte M., Stads, Susanne, Vonk Noordegraaf, Anton, Winckers, Kristien, Kruip, Marieke J.H.A., Klok, Frederikus A., de Jong, Cindy M.M., Visser, Chantal, Bemelmans, Remy H.H., Boersma, Wim G., van den Borst, Bram, Louise, J., Cannegieter, Suzanne C., ten Cate-Hoek, Arina J., Nanne Croles, F., Faber, Harald J., Faber, Laura M., Hellemons, Merel E., Hessels, Lisa M., Huisman, Menno V., Kamphuisen, Pieter W., Koster, Stephanie C.E., Kroft, Lucia J.M., van der Lee, Ivo, Leentjens, Jenneke, Meijer, Karina, Ninaber, Maarten K., Sondermeijer, Brigitte M., Stads, Susanne, Vonk Noordegraaf, Anton, Winckers, Kristien, Kruip, Marieke J.H.A., and Klok, Frederikus A.

Published
2023

75. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Author

Humbert, Marc, Kovacs, Gabor, Hoeper, Marius M., Badagliacca, Roberto, Berger, Rolf M.F., Brida, Margarita, Carlsen, Jørn, Coats, Andrew J.S., Escribano-Subias, Pilar, Ferrari, Pisana, Ferreira, Diogenes S., Ghofrani, Hossein Ardeschir, Giannakoulas, George, Kiely, David G., Mayer, Eckhard, Meszaros, Gergely, Nagavci, Blin, Olsson, Karen M., Pepke-Zaba, Joanna, Quint, Jennifer K., Rådegran, Göran, Simonneau, Gerald, Sitbon, Olivier, Tonia, Thomy, Toshner, Mark, Vachiery, Jean Luc, Vonk Noordegraaf, Anton, Delcroix, Marion, Rosenkranz, Stephan, Humbert, Marc, Kovacs, Gabor, Hoeper, Marius M., Badagliacca, Roberto, Berger, Rolf M.F., Brida, Margarita, Carlsen, Jørn, Coats, Andrew J.S., Escribano-Subias, Pilar, Ferrari, Pisana, Ferreira, Diogenes S., Ghofrani, Hossein Ardeschir, Giannakoulas, George, Kiely, David G., Mayer, Eckhard, Meszaros, Gergely, Nagavci, Blin, Olsson, Karen M., Pepke-Zaba, Joanna, Quint, Jennifer K., Rådegran, Göran, Simonneau, Gerald, Sitbon, Olivier, Tonia, Thomy, Toshner, Mark, Vachiery, Jean Luc, Vonk Noordegraaf, Anton, Delcroix, Marion, and Rosenkranz, Stephan

Published
2023

76. European Society of Cardiology quality indicators for the care and outcomes of adults with pulmonary arterial hypertension. Developed in collaboration with the Heart Failure Association of the European Society of Cardiology

Author

Aktaa, Suleman; https://orcid.org/0000-0002-9854-481X, Gale, Chris P, Brida, Margarita, Giannakoulas, George, Kovacs, Gabor, Adir, Yochai, Benza, Raymond L, Böhm, Michael, Coats, Andrew, D'Alto, Michele, Escribano‐Subias, Pilar, Ferrari, Pisana, Galiè, Nazzareno, Gibbs, J Simon R, Gin‐Sing, Wendy, Hoeper, Marius M, Humbert, Marc, Lang, Irene M, Maron, Bradley A, Meszaros, Gergely, Vonk Noordegraaf, Anton, Price, Laura C, Pepke‐Zaba, Joanna, Rådegran, Göran, Reis, Abilio, Sitbon, Olivier, Torbicki, Adam, Ulrich, Silvia, Rosenkranz, Stephan, Delcroix, Marion, Aktaa, Suleman; https://orcid.org/0000-0002-9854-481X, Gale, Chris P, Brida, Margarita, Giannakoulas, George, Kovacs, Gabor, Adir, Yochai, Benza, Raymond L, Böhm, Michael, Coats, Andrew, D'Alto, Michele, Escribano‐Subias, Pilar, Ferrari, Pisana, Galiè, Nazzareno, Gibbs, J Simon R, Gin‐Sing, Wendy, Hoeper, Marius M, Humbert, Marc, Lang, Irene M, Maron, Bradley A, Meszaros, Gergely, Vonk Noordegraaf, Anton, Price, Laura C, Pepke‐Zaba, Joanna, Rådegran, Göran, Reis, Abilio, Sitbon, Olivier, Torbicki, Adam, Ulrich, Silvia, Rosenkranz, Stephan, and Delcroix, Marion

Abstract

AimsTo develop a suite of quality indicators (QIs) for the evaluation of the care and outcomes for adults with pulmonary arterial hypertension (PAH).Methods and resultsWe followed the European Society of Cardiology (ESC) methodology for the development of QIs. This included (i) the identification of key domains of care for the management of PAH, (ii) the proposal of candidate QIs following systematic review of the literature, and (iii) the selection of a set of QIs using a modified Delphi method. The process was undertaken in parallel with the writing of the 2022 ESC/European Respiratory Society (ERS) guidelines for the diagnosis and treatment of pulmonary hypertension and involved the Task Force chairs, experts in PAH, Heart Failure Association (HFA) members and patient representatives. We identified five domains of care for patients with PAH: structural framework, diagnosis and risk stratification, initial treatment, follow‐up, and outcomes. In total, 23 main and one secondary QIs for PAH were selected.ConclusionThis document presents the ESC QIs for PAH, describes their development process and offers scientific rationale for their selection. The indicators may be used to quantify and improve adherence to guideline‐recommended clinical practice and improve patient outcomes.

Published
2023

81. The thromboxane receptor antagonist NTP42 promotes beneficial adaptation and preserves cardiac function in experimental models of right heart overload

Author

Mulvaney, Eamon P., primary, Renzo, Fabiana, additional, Adão, Rui, additional, Dupre, Emilie, additional, Bialesova, Lucia, additional, Salvatore, Viviana, additional, Reid, Helen M., additional, Conceição, Glória, additional, Grynblat, Julien, additional, Llucià-Valldeperas, Aida, additional, Michel, Jean-Baptiste, additional, Brás-Silva, Carmen, additional, Laurent, Charles E., additional, Howard, Luke S., additional, Montani, David, additional, Humbert, Marc, additional, Vonk Noordegraaf, Anton, additional, Perros, Frédéric, additional, Mendes-Ferreira, Pedro, additional, and Kinsella, B. Therese, additional

Published
2022
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84. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension:Developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG)

Author

Humbert, Marc, Kovacs, Gabor, Hoeper, Marius M, Badagliacca, Roberto, Berger, Rolf M F, Brida, Margarita, Carlsen, Jørn, Coats, Andrew J S, Escribano-Subias, Pilar, Ferrari, Pisana, Ferreira, Diogenes S, Ghofrani, Hossein Ardeschir, Giannakoulas, George, Kiely, David G, Mayer, Eckhard, Meszaros, Gergely, Nagavci, Blin, Olsson, Karen M, Pepke-Zaba, Joanna, Quint, Jennifer K, Rådegran, Göran, Simonneau, Gerald, Sitbon, Olivier, Tonia, Thomy, Toshner, Mark, Vachiery, Jean Luc, Vonk Noordegraaf, Anton, Delcroix, Marion, Rosenkranz, Stephan, and Nielsen, Jens Cosedis

Subjects
Endothelin Receptor Antagonists, Hypertension, Pulmonary/diagnosis, Humans
Published
2022

85. Riociguat and the right ventricle in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Author

Benza, Raymond L., primary, Langleben, David, additional, Hemnes, Anna R., additional, Vonk Noordegraaf, Anton, additional, Rosenkranz, Stephan, additional, Thenappan, Thenappan, additional, Hassoun, Paul M., additional, Preston, Ioana R., additional, Ghio, Stefano, additional, Badagliacca, Roberto, additional, Vizza, Carmine D., additional, Lang, Irene M., additional, Meier, Christian, additional, and Grünig, Ekkehard, additional

Published
2022
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87. Clinical Characteristics of Patients Undergoing Right Heart Catheterizations in Community Hospitals

Author

Jansen, Samara M. A., primary, Huis in ’t Veld, Anna. E., additional, Tolen, Peter Hans C. G., additional, Jacobs, Wouter, additional, Willemsen, H. M., additional, Grotjohan, Hans P., additional, Waskowsky, Marc, additional, van der Maten, Jan, additional, van der Weerdt, Arno, additional, Hoekstra, Romke, additional, Pérez Matos, Ana J., additional, Overbeek, Maria J., additional, Mollema, Sjoerd A., additional, El Bouazzaoui, Lahssan H. Hassan, additional, Vriend, Joris W. J., additional, Roorda, J. Milena M., additional, de Nooijer, Ramon, additional, van der Lee, Ivo, additional, Voogel, A. J., additional, Post, Johannes C., additional, Macken, Thomas, additional, Aerts, Jacqueline M., additional, van de Ven, Marjo J. T., additional, Bergman, Heidi, additional, Bakker‐de Boo, Mirjam, additional, de Boer, Roline C., additional, Vonk Noordegraaf, Anton, additional, de Man, Frances S., additional, and Bogaard, Harm Jan, additional

Published
2022
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88. Diagnostic efficacy of ECG-derived ventricular gradient for the detection of chronic thromboembolic pulmonary hypertension in patients with acute pulmonary embolism

Author

Luijten, Dieuwke, primary, Meijer, Fleur M.M., additional, Boon, Gudula J.A.M., additional, Ende-Verhaar, Yvonne M., additional, Bavalia, Roisin, additional, El Bouazzaoui, Lahassan H., additional, Delcroix, Marion, additional, Huisman, Menno V., additional, Mairuhu, Albert T.A., additional, Middeldorp, Saskia, additional, Pruszcyk, Piotr, additional, Ruigrok, Dieuwertje, additional, Verhamme, Peter, additional, Vonk Noordegraaf, Anton, additional, Vriend, Joris W.J., additional, Vliegen, Hubert W., additional, and Klok, Frederikus A., additional

Published
2022
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92. 3′-Deoxy-3′-[18F]Fluorothymidine Positron Emission Tomography Depicts Heterogeneous Proliferation Pathology in Idiopathic Pulmonary Arterial Hypertension Patient Lung: A Potential Biomarker for Pulmonary Arterial Hypertension

Author

Ashek, Ali, Spruijt, Onno A., Harms, Hendrik J., Lammertsma, Adriaan A., Cupitt, John, Dubois, Olivier, Wharton, John, Dabral, Swati, Pullamsetti, Soni Savai, Huisman, Marc C., Frings, Virginie, Boellaard, Ronald, de Man, Frances S., Botros, Lisa, Jansen, Samara, Vonk Noordegraaf, Anton, Wilkins, Martin R., Bogaard, Harm J., and Zhao, Lan

Published
2018
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93. Contribution of Impaired Parasympathetic Activity to Right Ventricular Dysfunction and Pulmonary Vascular Remodeling in Pulmonary Arterial Hypertension

Author

da Silva Gonçalves Bós, Denielli, Van Der Bruggen, Cathelijne E. E., Kurakula, Kondababu, Sun, Xiao-Qing, Casali, Karina R., Casali, Adenauer G., Rol, Nina, Szulcek, Robert, dos Remedios, Cris, Guignabert, Christophe, Tu, Ly, Dorfmüller, Peter, Humbert, Marc, Wijnker, Paul J.M., Kuster, Diederik W.D., van der Velden, Jolanda, Goumans, Marie-José, Bogaard, Harm-Jan, Vonk-Noordegraaf, Anton, de Man, Frances S., and Handoko, M. Louis

Published
2018
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96. Abstract 14753: Loss of Function ABCC8 Mutations Are Associated With Pulmonary Arterial Hypertension

Author

Bohnen, Michael S, Ma, Lijiang, Zhu, Na, Qi, Hongjian, McClenaghan, Conor, Gonzaga-Jauregui, Claudia, Dewey, Frederick E, Overton, John D, Reid, Jeffrey G, Shuldiner, Alan R, Baras, Aris, Sampson, Kevin J, Bleda, Marta, Hadinnapola, Charaka, Haimel, Matthias, Bogaard, Harm J, Church, Colin, Coghlan, Gerry, Corris, Paul A, Eyries, Mélanie, Gibbs, J. Simon R, Girerd, Barbara, Houweling, Arjan C, Humbert, Marc, Kiely, David G, Lawrie, Allan, MacKenzie Ross, Rob V, Martin, Jennifer M, Montani, David, Peacock, Andrew J, Pepke-Zaba, Joanna, Soubrier, Florent, Suntharalingam, Jay, Toshner, Mark, Treacy, Carmen M, Trembath, Richard C, Vonk Noordegraaf, Anton, Wharton, John, Wilkins, Martin R, Wort, Stephen J, Yates, Katherine, Gräf, Stefan, Morrell, Nicholas W, Krishnan, Usha, Rosenzweig, Erika B, Shen, Yufeng, Nichols, Colin G, Kass, Robert S, and Chung, Wendy K

Published
2017

98. Distinct Platelet RNA Signatures in Patients with Pulmonary Hypertension

Author

Smits, A Josien, Arkani, Mohammad, In 't Veld, Sjors G J G, Huis In 't Veld, Anna E, Sol, Nik, Groeneveldt, Joanne A, Botros, Liza, Braams, Natalia J, Jansen, Samara Ma, Ramaker, Jip, Zwaan, Kenn, Post, Edward, Nossent, Esther J, Boonstra, Anco, de Man, Frances S, Vonk Noordegraaf, Anton, Gomez-Arroyo, Jose, Best, Myron G, Wurdinger, Tom, Bogaard, Harm Jan, Pulmonology, and Neurosurgery

Abstract

RATIONALE: Pulmonary hypertension encompasses progressive disorders leading to right ventricular dysfunction and early death. Late detection is an important cause of poor clinical outcomes. However, biomarkers that accurately predict the presence of pulmonary hypertension are currently lacking. OBJECTIVES: In this study we provide evidence that blood platelets contain a distinctive RNA profile that may be exploited for detection of pulmonary hypertension. METHODS: Blood platelet RNA was isolated prospectively from 177 prevalent patients with different subtypes of pulmonary hypertension as well as 195 controls clinically not suspected of pulmonary hypertension. Sequencing libraries were created using SMARTer cDNA amplification, and sequenced on the Illumina HiSeq platform. RNA-sequencing reads were mapped to the human reference genome, and intron-spanning spliced RNA reads were selected. Differential spliced RNA panels were calculated by ANOVA-statistics. A particle swarm optimisation (PSO)-enhanced classification algorithm was built employing a development (n=213 samples) and independent validation series (n=159 samples). RESULTS: We detected a total of 4014 different RNAs in blood platelets from pulmonary hypertension patients (n=177) and asymptomatic controls (n=195). GSEA gene ontology analysis revealed enriched RNA levels for genes related to RNA-processing, translation and mitochondrial function. A PSO-selected RNA panel of 408 distinctive differentially spliced RNAs mediated detection of pulmonary hypertension with 93% sensitivity, 62% specificity, 77% accuracy, 0.89 (95%CI 0.83-0.93) area under the curve and a negative predictive value of 91% in the independent validation series. Prediction score was independent of age, sex, smoking, pulmonary hypertension subtype, and the use of pulmonary hypertension-specific medication or anti-coagulants. CONCLUSION: A platelet RNA-panel may accurately discriminate patients with pulmonary hypertension from asymptomatic controls. In the light of current diagnostic delays, this study is the starting point for further development and evaluation of a platelet RNA-based blood test, to ultimately improve early diagnosis and clinical outcomes in patients with pulmonary hypertension.

Published
2022

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