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5. Right Atrial Adaptation to Precapillary Pulmonary Hypertension: Pressure-Volume, Cardiomyocyte, and Histological Analysis

Author

Wessels, Jeroen N., van Wezenbeek, Jessie, de Rover, Jari, Smal, Rowan, Llucià-Valldeperas, Aida, Celant, Lucas R., Marcus, J. Tim, Meijboom, Lilian J., Groeneveldt, Joanne A., Oosterveer, Frank P.T., Winkelman, Toon A., Niessen, Hans W.M., Goumans, Marie-José, Bogaard, Harm Jan, Vonk Noordegraaf, Anton, Strijkers, Gustav J., Handoko, M. Louis, Westerhof, Berend E., and de Man, Frances S.

Published
2023
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6. Long-Term Effects of Pulmonary Endarterectomy on Right Ventricular Stiffness and Fibrosis in Chronic Thromboembolic Pulmonary Hypertension

Author

Braams, Natalia J., Kianzad, Azar, van Wezenbeek, Jessie, Wessels, Jeroen N., Jansen, Samara M.A., Andersen, Stine, Boonstra, Anco, Nossent, Esther J., Marcus, J. Tim, Bayoumy, Ahmed A., Becher, Clarissa, Goumans, Marie-José, Andersen, Asger, Vonk Noordegraaf, Anton, de Man, Frances S., Bogaard, Harm Jan, and Meijboom, Lilian J.

Published
2023
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7. Pulmonary perfusion defects or residual vascular obstruction and persistent symptoms after pulmonary embolism: a systematic review and meta-analysis

Author

Cimini, Ludovica Anna, primary, Luijten, Dieuwke, additional, Barco, Stefano, additional, Ghanima, Waleed, additional, Jervan, Øyvind, additional, Kahn, Susan R., additional, Konstantinides, Stavros, additional, Lachante, Daniel, additional, Nakano, Yoshihisa, additional, Ninaber, Maarten, additional, van Es, Josien, additional, van Mens, Thijs, additional, Vonk Noordegraaf, Anton, additional, Becattini, Cecilia, additional, and Klok, Frederikus A., additional

Published
2024
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8. Genetic determinants of risk in pulmonary arterial hypertension: international genome-wide association studies and meta-analysis.

Author

Rhodes, Christopher J, Batai, Ken, Bleda, Marta, Haimel, Matthias, Southgate, Laura, Germain, Marine, Pauciulo, Michael W, Hadinnapola, Charaka, Aman, Jurjan, Girerd, Barbara, Arora, Amit, Knight, Jo, Hanscombe, Ken B, Karnes, Jason H, Kaakinen, Marika, Gall, Henning, Ulrich, Anna, Harbaum, Lars, Cebola, Inês, Ferrer, Jorge, Lutz, Katie, Swietlik, Emilia M, Ahmad, Ferhaan, Amouyel, Philippe, Archer, Stephen L, Argula, Rahul, Austin, Eric D, Badesch, David, Bakshi, Sahil, Barnett, Christopher, Benza, Raymond, Bhatt, Nitin, Bogaard, Harm J, Burger, Charles D, Chakinala, Murali, Church, Colin, Coghlan, John G, Condliffe, Robin, Corris, Paul A, Danesino, Cesare, Debette, Stéphanie, Elliott, C Gregory, Elwing, Jean, Eyries, Melanie, Fortin, Terry, Franke, Andre, Frantz, Robert P, Frost, Adaani, Garcia, Joe GN, Ghio, Stefano, Ghofrani, Hossein-Ardeschir, Gibbs, J Simon R, Harley, John, He, Hua, Hill, Nicholas S, Hirsch, Russel, Houweling, Arjan C, Howard, Luke S, Ivy, Dunbar, Kiely, David G, Klinger, James, Kovacs, Gabor, Lahm, Tim, Laudes, Matthias, Machado, Rajiv D, MacKenzie Ross, Robert V, Marsolo, Keith, Martin, Lisa J, Moledina, Shahin, Montani, David, Nathan, Steven D, Newnham, Michael, Olschewski, Andrea, Olschewski, Horst, Oudiz, Ronald J, Ouwehand, Willem H, Peacock, Andrew J, Pepke-Zaba, Joanna, Rehman, Zia, Robbins, Ivan, Roden, Dan M, Rosenzweig, Erika B, Saydain, Ghulam, Scelsi, Laura, Schilz, Robert, Seeger, Werner, Shaffer, Christian M, Simms, Robert W, Simon, Marc, Sitbon, Olivier, Suntharalingam, Jay, Tang, Haiyang, Tchourbanov, Alexander Y, Thenappan, Thenappan, Torres, Fernando, Toshner, Mark R, Treacy, Carmen M, Vonk Noordegraaf, Anton, Waisfisz, Quinten, and Walsworth, Anna K

Subjects
UK NIHR BioResource Rare Diseases Consortium, UK PAH Cohort Study Consortium, US PAH Biobank Consortium, Humans, Genetic Predisposition to Disease, Risk Assessment, Survival Analysis, Signal Transduction, Polymorphism, Single Nucleotide, Middle Aged, Female, Male, Genetic Variation, Genome-Wide Association Study, SOXF Transcription Factors, HLA-DP beta-Chains, Genotyping Techniques, HLA-DP alpha-Chains, Pulmonary Arterial Hypertension, Clinical Sciences, Public Health and Health Services, Other Medical and Health Sciences
Abstract

BackgroundRare genetic variants cause pulmonary arterial hypertension, but the contribution of common genetic variation to disease risk and natural history is poorly characterised. We tested for genome-wide association for pulmonary arterial hypertension in large international cohorts and assessed the contribution of associated regions to outcomes.MethodsWe did two separate genome-wide association studies (GWAS) and a meta-analysis of pulmonary arterial hypertension. These GWAS used data from four international case-control studies across 11 744 individuals with European ancestry (including 2085 patients). One GWAS used genotypes from 5895 whole-genome sequences and the other GWAS used genotyping array data from an additional 5849 individuals. Cross-validation of loci reaching genome-wide significance was sought by meta-analysis. Conditional analysis corrected for the most significant variants at each locus was used to resolve signals for multiple associations. We functionally annotated associated variants and tested associations with duration of survival. All-cause mortality was the primary endpoint in survival analyses.FindingsA locus near SOX17 (rs10103692, odds ratio 1·80 [95% CI 1·55-2·08], p=5·13 × 10-15) and a second locus in HLA-DPA1 and HLA-DPB1 (collectively referred to as HLA-DPA1/DPB1 here; rs2856830, 1·56 [1·42-1·71], p=7·65 × 10-20) within the class II MHC region were associated with pulmonary arterial hypertension. The SOX17 locus had two independent signals associated with pulmonary arterial hypertension (rs13266183, 1·36 [1·25-1·48], p=1·69 × 10-12; and rs10103692). Functional and epigenomic data indicate that the risk variants near SOX17 alter gene regulation via an enhancer active in endothelial cells. Pulmonary arterial hypertension risk variants determined haplotype-specific enhancer activity, and CRISPR-mediated inhibition of the enhancer reduced SOX17 expression. The HLA-DPA1/DPB1 rs2856830 genotype was strongly associated with survival. Median survival from diagnosis in patients with pulmonary arterial hypertension with the C/C homozygous genotype was double (13·50 years [95% CI 12·07 to >13·50]) that of those with the T/T genotype (6·97 years [6·02-8·05]), despite similar baseline disease severity.InterpretationThis is the first study to report that common genetic variation at loci in an enhancer near SOX17 and in HLA-DPA1/DPB1 is associated with pulmonary arterial hypertension. Impairment of SOX17 function might be more common in pulmonary arterial hypertension than suggested by rare mutations in SOX17. Further studies are needed to confirm the association between HLA typing or rs2856830 genotyping and survival, and to determine whether HLA typing or rs2856830 genotyping improves risk stratification in clinical practice or trials.FundingUK NIHR, BHF, UK MRC, Dinosaur Trust, NIH/NHLBI, ERS, EMBO, Wellcome Trust, EU, AHA, ACClinPharm, Netherlands CVRI, Dutch Heart Foundation, Dutch Federation of UMC, Netherlands OHRD and RNAS, German DFG, German BMBF, APH Paris, INSERM, Université Paris-Sud, and French ANR.

Published
2019

10. Assessment of Right Ventricular Function in the Research Setting: Knowledge Gaps and Pathways Forward. An Official American Thoracic Society Research Statement

Author

Lahm, Tim, Douglas, Ivor S, Archer, Stephen L, Bogaard, Harm J, Chesler, Naomi C, Haddad, Francois, Hemnes, Anna R, Kawut, Steven M, Kline, Jeffrey A, Kolb, Todd M, Mathai, Stephen C, Mercier, Olaf, Michelakis, Evangelos D, Naeije, Robert, Tuder, Rubin M, Ventetuolo, Corey E, Vieillard-Baron, Antoine, Voelkel, Norbert F, Vonk-Noordegraaf, Anton, and Hassoun, Paul M

Subjects
Cardiovascular, Heart Disease, Lung, Rare Diseases, Animals, Humans, Research, Societies, Medical, United States, Ventricular Dysfunction, Right, Ventricular Function, Right, right ventricle, pulmonary hypertension, pulmonary embolism, acute respiratory distress syndrome, pulmonary circulation, American Thoracic Society Assembly on Pulmonary Circulation, Medical and Health Sciences, Respiratory System
Abstract

BackgroundRight ventricular (RV) adaptation to acute and chronic pulmonary hypertensive syndromes is a significant determinant of short- and long-term outcomes. Although remarkable progress has been made in the understanding of RV function and failure since the meeting of the NIH Working Group on Cellular and Molecular Mechanisms of Right Heart Failure in 2005, significant gaps remain at many levels in the understanding of cellular and molecular mechanisms of RV responses to pressure and volume overload, in the validation of diagnostic modalities, and in the development of evidence-based therapies.MethodsA multidisciplinary working group of 20 international experts from the American Thoracic Society Assemblies on Pulmonary Circulation and Critical Care, as well as external content experts, reviewed the literature, identified important knowledge gaps, and provided recommendations.ResultsThis document reviews the knowledge in the field of RV failure, identifies and prioritizes the most pertinent research gaps, and provides a prioritized pathway for addressing these preclinical and clinical questions. The group identified knowledge gaps and research opportunities in three major topic areas: 1) optimizing the methodology to assess RV function in acute and chronic conditions in preclinical models, human studies, and clinical trials; 2) analyzing advanced RV hemodynamic parameters at rest and in response to exercise; and 3) deciphering the underlying molecular and pathogenic mechanisms of RV function and failure in diverse pulmonary hypertension syndromes.ConclusionsThis statement provides a roadmap to further advance the state of knowledge, with the ultimate goal of developing RV-targeted therapies for patients with RV failure of any etiology.

Published
2018

11. Identification of rare sequence variation underlying heritable pulmonary arterial hypertension.

Author

Gräf, Stefan, Haimel, Matthias, Bleda, Marta, Hadinnapola, Charaka, Southgate, Laura, Li, Wei, Hodgson, Joshua, Liu, Bin, Salmon, Richard M, Southwood, Mark, Machado, Rajiv D, Martin, Jennifer M, Treacy, Carmen M, Yates, Katherine, Daugherty, Louise C, Shamardina, Olga, Whitehorn, Deborah, Holden, Simon, Aldred, Micheala, Bogaard, Harm J, Church, Colin, Coghlan, Gerry, Condliffe, Robin, Corris, Paul A, Danesino, Cesare, Eyries, Mélanie, Gall, Henning, Ghio, Stefano, Ghofrani, Hossein-Ardeschir, Gibbs, J Simon R, Girerd, Barbara, Houweling, Arjan C, Howard, Luke, Humbert, Marc, Kiely, David G, Kovacs, Gabor, MacKenzie Ross, Robert V, Moledina, Shahin, Montani, David, Newnham, Michael, Olschewski, Andrea, Olschewski, Horst, Peacock, Andrew J, Pepke-Zaba, Joanna, Prokopenko, Inga, Rhodes, Christopher J, Scelsi, Laura, Seeger, Werner, Soubrier, Florent, Stein, Dan F, Suntharalingam, Jay, Swietlik, Emilia M, Toshner, Mark R, van Heel, David A, Vonk Noordegraaf, Anton, Waisfisz, Quinten, Wharton, John, Wort, Stephen J, Ouwehand, Willem H, Soranzo, Nicole, Lawrie, Allan, Upton, Paul D, Wilkins, Martin R, Trembath, Richard C, and Morrell, Nicholas W

Subjects
Humans, Genetic Predisposition to Disease, Transforming Growth Factor beta, Membrane Transport Proteins, Prognosis, Case-Control Studies, Signal Transduction, Gene Expression Regulation, Base Sequence, Mutation, Models, Molecular, Adult, Female, Male, Aquaporin 1, Bone Morphogenetic Protein Receptors, Type II, Adenosine Triphosphatases, Growth Differentiation Factors, SOXF Transcription Factors, HEK293 Cells, Familial Primary Pulmonary Hypertension, Whole Genome Sequencing, Bone Morphogenetic Protein Receptors, Type II, Models, Molecular
Abstract

Pulmonary arterial hypertension (PAH) is a rare disorder with a poor prognosis. Deleterious variation within components of the transforming growth factor-β pathway, particularly the bone morphogenetic protein type 2 receptor (BMPR2), underlies most heritable forms of PAH. To identify the missing heritability we perform whole-genome sequencing in 1038 PAH index cases and 6385 PAH-negative control subjects. Case-control analyses reveal significant overrepresentation of rare variants in ATP13A3, AQP1 and SOX17, and provide independent validation of a critical role for GDF2 in PAH. We demonstrate familial segregation of mutations in SOX17 and AQP1 with PAH. Mutations in GDF2, encoding a BMPR2 ligand, lead to reduced secretion from transfected cells. In addition, we identify pathogenic mutations in the majority of previously reported PAH genes, and provide evidence for further putative genes. Taken together these findings contribute new insights into the molecular basis of PAH and indicate unexplored pathways for therapeutic intervention.

Published
2018

19. Patients with CTEPH and mild hemodynamic severity of disease improve to a similar level of exercise capacity after pulmonary endarterectomy compared to patients with severe hemodynamic disease

Author

van Kan, Coen, primary, Tramper, Jelco, additional, Bresser, Paul, additional, J. Meijboom, Lilian, additional, Symersky, Petr, additional, Winkelman, Jacobus A., additional, Nossent, Esther J., additional, Aman, Jurjan, additional, Bogaard, Harm Jan, additional, Vonk Noordegraaf, Anton, additional, and van Es, Josien, additional

Published
2024
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20. Comparison of cardiac magnetic resonance imaging, functional and haemodynamic variables in pulmonary arterial hypertension: insights from REPAIR

Author

Kiely, David G., primary, Channick, Richard, additional, Flores, Dayana, additional, Galiè, Nazzareno, additional, MacDonald, Gwen, additional, Marcus, J. Tim, additional, Mitchell, Lada, additional, Peacock, Andrew, additional, Rosenkranz, Stephan, additional, Tawakol, Ahmed, additional, Torbicki, Adam, additional, Vonk Noordegraaf, Anton, additional, and Swift, Andrew J., additional

Published
2024
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21. ERS International Congress 2023: highlights from the Pulmonary Vascular Diseases Assembly

Author

Cullivan, Sarah, primary, Boucly, Athénaïs, additional, Jevnikar, Mitja, additional, Lechartier, Benoit, additional, Ulrich, Silvia, additional, Bertoletti, Laurent, additional, Sitbon, Olivier, additional, Vonk-Noordegraaf, Anton, additional, Bokan, Aleksandar, additional, Park, Da-Hee, additional, Genecand, Leon, additional, Guiot, Julien, additional, Jutant, Etienne-Marie, additional, Piccari, Lucilla, additional, and Lichtblau, Mona, additional

Published
2023
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22. 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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23. Right Ventricular and Right Atrial Function Are Less Compromised in Pulmonary Hypertension Secondary to Heart Failure With Preserved Ejection Fraction: A Comparison With Pulmonary Arterial Hypertension With Similar Pressure Overload

Author

van Wezenbeek, Jessie, Kianzad, Azar, van de Bovenkamp, Arno, Wessels, Jeroen, Mouratoglou, Sophia A., Braams, Natalia J., Jansen, Samara M.A., Meulblok, Eva, Meijboom, Lilian J., Marcus, J. Tim, Vonk Noordegraaf, Anton, José Goumans, Marie, Jan Bogaard, Harm, Handoko, M. Louis, and de Man, Frances S.

Published
2022
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24. 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

25. Mechanical stimulation of induced pluripotent stem derived cardiac fibroblasts.

Author

Bekedam, Fjodor T., Smal, Rowan, Smit, Marisa C., Aman, Jurjan, Vonk-Noordegraaf, Anton, Bogaard, Harm Jan, Goumans, Marie José, De Man, Frances S., and Llucià-Valldeperas, Aida

Subjects
TRANSFORMING growth factors-beta, INDUCED pluripotent stem cells, FIBROBLASTS, HEART fibrosis, HEART failure
Abstract

Cardiac fibrosis contributes to the development of heart failure, and is the response of cardiac fibroblasts (CFs) to pressure or volume overload. Limiting factors in CFs research are the poor availability of human cells and the tendency of CFs to transdifferentiate into myofibroblasts when cultured in vitro. The possibility to generate CFs from induced pluripotent stem cells (iPSC), providing a nearly unlimited cell source, opens new possibilities. However, the behaviour of iPSC-CFs under mechanical stimulation has not been studied yet. Our study aimed to assess the behaviour of iPSC-CFs under mechanical stretch and pro-fibrotic conditions. First, we confirm that iPSC-CFs are comparable to primary CFs at gene, protein and functional level. Furthermore, iPSC-derived CFs adopt a pro-fibrotic response to transforming growth factor beta (TGF-β). In addition, mechanical stretch inhibits TGF-β-induced fibroblast activation in iPSC-CFs. Thus, the responsiveness to cytokines and mechanical stimulation of iPSC-CFs demonstrates they possess key characteristics of primary CFs and may be useful for disease modelling. [ABSTRACT FROM AUTHOR]

Published
2024
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27. 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

28. 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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29. 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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30. 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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36. ERS International Congress 2021: highlights from the Pulmonary Vascular Diseases Assembly

Author

Lichtblau, Mona; https://orcid.org/0000-0003-4485-1758, Piccari, Lucilla; https://orcid.org/0000-0002-2241-7523, Ramjug, Sheila, Bokan, Aleksandar; https://orcid.org/0000-0003-0549-3919, Lechartier, Benoit, Jutant, Etienne-Marie; https://orcid.org/0000-0002-1374-1890, Barata, Margarida, Garcia, Agustin Roberto, Howard, Luke S; https://orcid.org/0000-0003-2822-210X, Adir, Yochai, Delcroix, Marion; https://orcid.org/0000-0001-8394-9809, Jara-Palomares, Luis; https://orcid.org/0000-0002-4125-3376, Bertoletti, Laurent; https://orcid.org/0000-0001-8214-3010, Sitbon, Olivier; https://orcid.org/0000-0002-1942-1951, Ulrich, Silvia; https://orcid.org/0000-0002-5250-5022, Vonk Noordegraaf, Anton; https://orcid.org/0000-0002-4057-758X, Lichtblau, Mona; https://orcid.org/0000-0003-4485-1758, Piccari, Lucilla; https://orcid.org/0000-0002-2241-7523, Ramjug, Sheila, Bokan, Aleksandar; https://orcid.org/0000-0003-0549-3919, Lechartier, Benoit, Jutant, Etienne-Marie; https://orcid.org/0000-0002-1374-1890, Barata, Margarida, Garcia, Agustin Roberto, Howard, Luke S; https://orcid.org/0000-0003-2822-210X, Adir, Yochai, Delcroix, Marion; https://orcid.org/0000-0001-8394-9809, Jara-Palomares, Luis; https://orcid.org/0000-0002-4125-3376, Bertoletti, Laurent; https://orcid.org/0000-0001-8214-3010, Sitbon, Olivier; https://orcid.org/0000-0002-1942-1951, Ulrich, Silvia; https://orcid.org/0000-0002-5250-5022, and Vonk Noordegraaf, Anton; https://orcid.org/0000-0002-4057-758X

Abstract

This article aims to summarise the latest research presented at the virtual 2021 European Respiratory Society (ERS) International Congress in the field of pulmonary vascular disease. In light of the current guidelines and proceedings, knowledge gaps are addressed and the newest findings of the various forms of pulmonary hypertension as well as key points on pulmonary embolism are discussed.Despite the comprehensive coverage of the guidelines for pulmonary embolism at previous conferences, discussions about controversies in the diagnosis and treatment of this condition in specific cases were debated and are addressed in the first section of this article.We then report on an interesting pro–con debate about the current classification of pulmonary hypertension.We further report on presentations on Group 3 pulmonary hypertension, with research exploring pathogenesis, phenotyping, diagnosis and treatment; important contributions on the diagnosis of post-capillary pulmonary hypertension are also included.Finally, we summarise the latest evidence presented on pulmonary vascular disease and COVID-19 and a statement on the new imaging guidelines for pulmonary vascular disease from the Fleischner Society.

Published
2022

37. 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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38. 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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40. 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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41. 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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42. 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

43. 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

44. Risk stratification and response to therapy in patients with pulmonary arterial hypertension and comorbidities: A COMPERA analysis

Author

Rosenkranz, Stephan, Pausch, Christine, Coghlan, John G., Huscher, Doerte, Pittrow, David, Gruenig, Ekkehard, Staehler, Gerd, Vizza, Carmine Dario, Gall, Henning, Distler, Oliver, Delcroix, Marion, Ghofrani, Hossain A., Ewert, Ralf, Kabitz, Han -Joachim, Skowasch, Dirk, Behr, Juergen, Milger, Katrin, Halank, Michael, Wilkens, Heinrike, Seyfarth, Hans-Juergen, Held, Matthias, Scelsi, Laura, Neurohr, Claus, Vonk-Noordegraaf, Anton, Ulrich, Silvia, Klose, Hans, Claussen, Martin, Eisenmann, Stephan, Schmidt, Kai-Helge, Remppis, Bjoern Andrew, Skride, Andris, Jureviciene, Elena, Gumbiene, Lina, Miliauskas, Skaidrius, Loeffler-Ragg, Judith, Lange, Tobias J., Olsson, Karen M., Hoeper, Marius M., Opitz, Christian, Rosenkranz, Stephan, Pausch, Christine, Coghlan, John G., Huscher, Doerte, Pittrow, David, Gruenig, Ekkehard, Staehler, Gerd, Vizza, Carmine Dario, Gall, Henning, Distler, Oliver, Delcroix, Marion, Ghofrani, Hossain A., Ewert, Ralf, Kabitz, Han -Joachim, Skowasch, Dirk, Behr, Juergen, Milger, Katrin, Halank, Michael, Wilkens, Heinrike, Seyfarth, Hans-Juergen, Held, Matthias, Scelsi, Laura, Neurohr, Claus, Vonk-Noordegraaf, Anton, Ulrich, Silvia, Klose, Hans, Claussen, Martin, Eisenmann, Stephan, Schmidt, Kai-Helge, Remppis, Bjoern Andrew, Skride, Andris, Jureviciene, Elena, Gumbiene, Lina, Miliauskas, Skaidrius, Loeffler-Ragg, Judith, Lange, Tobias J., Olsson, Karen M., Hoeper, Marius M., and Opitz, Christian

Abstract

BACKGROUND: A diagnosis of idiopathic pulmonary arterial hypertension (IPAH) is frequently made in elderly patients who present with comorbidities, especially hypertension, coronary heart disease, diabe-tes mellitus, and obesity. It is unknown to what extent the presence of these comorbidities affects the response to PAH therapies and whether risk stratification predicts outcome in patients with comorbidities.METHODS: We assessed the database of COMPERA, a European pulmonary hypertension registry, to determine changes after initiation of PAH therapy in WHO functional class (FC), 6-minute walking distance (6MWD), brain natriuretic peptide (BNP) or N-terminal fragment of probrain natriuretic pep-tide (NT-pro-BNP), and mortality risk assessed by a 4-strata model in patients with IPAH and no comorbidities, 1-2 comorbidities and 3-4 comorbidities.RESULTS: The analysis was based on 1,120 IPAH patients (n = 208 [19%] without comorbidities, n = 641 [57%] with 1-2 comorbidities, and n = 271 [24%] with 3-4 comorbidities). Improvements in FC, 6MWD, BNP/NT-pro-BNP, and mortality risk from baseline to first follow-up were significantly larger in patients with no comorbidities than in patients with comorbidities, while they were not signifi-cantly different in patients with 1-2 and 3-4 comorbidities. The 4-strata risk tool predicted survival in patients without comorbidities as well as in patients with 1-2 or 3-4 comorbidities.CONCLUSIONS: Our data suggest that patients with IPAH and comorbidities benefit from PAH medica-tion with improvements in FC, 6MWD, BNP/NT-pro-BNP, and mortality risk, albeit to a lesser extent than patients without comorbidities. The 4-strata risk tool predicted outcome in patients with IPAH irrespective of the presence of comorbidities.J Heart Lung Transplant 2023;42:102-114 (c) 2022 The Author(s). Published by Elsevier Inc. on behalf of International Society for Heart and Lung Transplantation. This is an open access article under the CC BY-NC-ND license

Published
2023

45. 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

48. 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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50. 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

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