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1,517 results on '"Vonk Noordegraaf, Anton"'

5. Long-term effects of pulmonary endarterectomy on pulmonary hemodynamics, cardiac function, and exercise capacity in chronic thromboembolic pulmonary hypertension

Author

Kianzad, Azar, Baccelli, Andrea, Braams, Natalia J., Andersen, Stine, van Wezenbeek, Jessie, Wessels, Jeroen N., Celant, Lucas R., Vos, Anna E., Davies, Rachel, Lo Giudice, Francesco, Haji, Gulammehdi, Rinaldo, Rocco F., Vigo, Beatrice, Gopalan, Deepa, Symersky, Petr, Winkelman, Jacobus A., Boonstra, Anco, Nossent, Esther J., Tim Marcus, J., Vonk Noordegraaf, Anton, Meijboom, Lilian J., de Man, Frances S., Andersen, Asger, Howard, Luke S., and Bogaard, Harm Jan

Published
2024
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8. Defining the clinical validity of genes reported to cause pulmonary arterial hypertension

Author

Callejo, Emily P., Day, Kristina M., Macaya, Daniela, Maldonado-Velez, Gabriel, Archer, Stephen L., Auckland, Kathryn, Austin, Eric D., Badagliacca, Roberto, Barberà, Joan-Albert, Belge, Catharina, Bogaard, Harm Jan, Bonnet, Sébastien, Boomars, Karin A., Boucherat, Olivier, Chakinala, Murali M., Condliffe, Robin, Damico, Rachel Lynn, Delcroix, Marion, Desai, Ankit A., Doboszynska, Anna, Elliott, C. Greg, Eyries, Melanie, Escribano Subías, Maria Pilar, Gall, Henning, Ghio, Stefano, Ghofrani, Ardeschir-Hossein, Grünig, Ekkehard, Hamid, Rizwan, Harbaum, Lars, Hassoun, Paul M., Hemnes, Anna R., Hinderhofer, Katrin, Howard, Luke S., Humbert, Marc, Kiely, David G., Langleben, David, Lawrie, Allan, Loyd, Jim E., Moledina, Shahin, Montani, David, Morrell, Nichols W., Nichols, William C., Olschewski, Andrea, Olschewski, Horst, Papa, Silvia, Pauciulo, Mike W., Provencher, Steve, Quarck, Rozenn, Rhodes, Christopher J., Scelsi, Laura, Seeger, Werner, Stewart, Duncan J., Sweatt, Andrew, Swietlik, Emilia M., Treacy, Carmen, Trembath, Richard C., Tura-Ceide, Olga, Vizza, Carmine Dario, Vonk Noordegraaf, Anton, Wilkins, Martin R., Zamanian, Roham T., Zateyshchikov, Dmitry, Welch, Carrie L., Aldred, Micheala A., Balachandar, Srimmitha, Dooijes, Dennis, Eichstaedt, Christina A., Gräf, Stefan, Houweling, Arjan C., Machado, Rajiv D., Pandya, Divya, Prapa, Matina, Shaukat, Memoona, Southgate, Laura, Tenorio-Castano, Jair, and Chung, Wendy K.

Published
2023
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9. 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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10. 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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11. 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, Grünig, Ekkehard, Staehler, Gerd, Vizza, Carmine Dario, Gall, Henning, Distler, Oliver, Delcroix, Marion, Ghofrani, Hossain A., Ewert, Ralf, Kabitz, Hans-Joachim, Skowasch, Dirk, Behr, Juergen, Milger, Katrin, Halank, Michael, Wilkens, Heinrike, Seyfarth, Hans-Jürgen, 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, Löffler-Ragg, Judith, Lange, Tobias J., Olsson, Karen M., Hoeper, Marius M., and Opitz, Christian

Published
2023
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12. 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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13. 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

14. Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis

Author

Hoeper, Marius M, Dwivedi, Krit, Pausch, Christine, Lewis, Robert A, Olsson, Karen M, Huscher, Doerte, Pittrow, David, Grünig, Ekkehard, Staehler, Gerd, Vizza, Carmine Dario, Gall, Henning, Distler, Oliver, Opitz, Christian, Gibbs, John Simon R, Delcroix, Marion, Park, Da-Hee, Ghofrani, Hossein Ardeschir, Ewert, Ralf, Kaemmerer, Harald, Kabitz, Hans-Joachim, Skowasch, Dirk, Behr, Juergen, Milger, Katrin, Lange, Tobias J, Wilkens, Heinrike, Seyfarth, Hans-Jürgen, Held, Matthias, Dumitrescu, Daniel, Tsangaris, Iraklis, Vonk-Noordegraaf, Anton, Ulrich, Silvia, Klose, Hans, Claussen, Martin, Eisenmann, Stephan, Schmidt, Kai-Helge, Swift, Andrew J, Thompson, Alfred A Roger, Elliot, Charlie A, Rosenkranz, Stephan, Condliffe, Robin, Kiely, David G, and Halank, Michael

Published
2022
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16. Prediction of chronic thromboembolic pulmonary hypertension with standardised evaluation of initial computed tomography pulmonary angiography performed for suspected acute pulmonary embolism

Author

Boon, Gudula J. A. M., Ende-Verhaar, Yvonne M., Beenen, Ludo F. M., Coolen, Johan, Delcroix, Marion, Golebiowski, Marek, Huisman, Menno V., Mairuhu, Albert T. A., Meijboom, Lilian J., Middeldorp, Saskia, Pruszczyk, Piotr, van Rooden, Cornelis J., Vonk Noordegraaf, Anton, Kroft, Lucia J. M., and Klok, Frederikus A.

Published
2022
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20. 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

21. 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

23. Imatinib in patients with severe COVID-19: a randomised, double-blind, placebo-controlled, clinical trial

Author

Aman, Jurjan, Duijvelaar, Erik, Botros, Liza, Kianzad, Azar, Schippers, Job R, Smeele, Patrick J, Azhang, Sara, Bartelink, Imke H, Bayoumy, Ahmed A, Bet, Pierre M, Boersma, Wim, Bonta, Peter I, Boomars, Karin A T, Bos, Lieuwe D J, van Bragt, Job J M H, Braunstahl, Gert-Jan, Celant, Lucas R, Eger, Katrien A B, Geelhoed, J J Miranda, van Glabbeek, Yurika L E, Grotjohan, Hans P, Hagens, Laura A, Happe, Chris M, Hazes, Boaz D, Heunks, Leo M A, van den Heuvel, Michel, Hoefsloot, Wouter, Hoek, Rianne J A, Hoekstra, Romke, Hofstee, Herman M A, Juffermans, Nicole P, Kemper, E Marleen, Kos, Renate, Kunst, Peter W A, Lammers, Ariana, van der Lee, Ivo, van der Lee, E Laurien, Maitland-van der Zee, Anke-Hilse, Mau Asam, Pearl F M, Mieras, Adinda, Muller, Mirte, Neefjes, Elisabeth C W, Nossent, Esther J, Oswald, Laurien M A, Overbeek, Maria J, Pamplona, Carolina C, Paternotte, Nienke, Pronk, Niels, de Raaf, Michiel A, van Raaij, Bas F M, Reijrink, Merlijn, Schultz, Marcus J, Serpa Neto, Ary, Slob, Elise M A, Smeenk, Frank W J M, Smit, Marry R, Smits, A Josien, Stalenhoef, Janneke E, Tuinman, Pieter R, Vanhove, Arthur L E M, Wessels, Jeroen N, van Wezenbeek, Jessie C C, Vonk Noordegraaf, Anton, de Man, Frances S, and Bogaard, Harm J

Published
2021
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28. Switching to riociguat versus maintenance therapy with phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension (REPLACE): a multicentre, open-label, randomised controlled trial

Author

Olschewski, H, Delcroix, M, Andrade-Lima, M, de Amorim Corrêac, R, Figueiredo Campos, F, Ota Arakaki, J, Meyer, G, De Souza, R, Langleben, D, Al-Hiti, H, Jansa, P, Mellemkjær, S, Bauer, F, Montani, D, Simonneau, G, Drömann, D, Ghofrani, H-A, Grünig, E, Halank, M, Held, M, Hoeper, MM, Klose, H, Kneidinger, N, Leuchte, H, Opitz, C, Rosenkranz, S, Wilkens, H, Wirtz, H, Karvounis, H, Pitsiou, G, Orfanos, S, D'Alto, M, Ghio, S, Vizza, CD, Vitulo, P, Nakayama, T, Maki, H, Tatebe, S, de los Rios Ibarra, M, Pulido, T, Van Dijk, A, Vonk-Noordegraaf, A, Roleder, T, Castro, G, Loureiro, MJ, Robalo-Martins, S, Barberá, JA, Lázaro, M, Perez-Penate, GM, Román, A, Cheng, C-C, Hsu, C-H, Hsu, H-H, Atahan, E, Mogulkoc Bishop, N, Okumus, NG, Onen, Z, Chang, H-J, Chang, S-A, Lee, J-S, Kim, H-K, Coghlan, JG, Corris, PA, Church, AC, Condliffe, R, Gibbs, JSR, Peacock, AJ, Wort, S, Allen, R, Allen, S, Awdish, R, Benza, RL, DeSouza, S, Feldman, J, Johri, S, Klinger, JR, Layish, D, McConnell, J, McLaughlin, VV, Migliore, C, Rahaghi, F, Rischard, F, Robbins, I, Satterwhite, L, Shah, T, Sulica, R, White, RJ, Hoeper, Marius M, Al-Hiti, Hikmet, Benza, Raymond L, Chang, Sung-A, Corris, Paul A, Gibbs, J Simon R, Grünig, Ekkehard, Jansa, Pavel, Klinger, James R, Langleben, David, McLaughlin, Vallerie V, Meyer, Gisela M B, Ota-Arakaki, Jaquelina, Peacock, Andrew J, Pulido, Tomás, Rosenkranz, Stephan, Vizza, Carmine Dario, Vonk-Noordegraaf, Anton, White, R James, Chang, Mikyung, Kleinjung, Frank, Meier, Christian, Paraschin, Karen, Ghofrani, Hossein Ardeschir, and Simonneau, Gérald

Published
2021
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30. Contributors

Author

Aikawa, Elena, primary, Anderson, S.G., additional, Araújo Passos, Livia Silva, additional, Arefin, Samsul, additional, Arvidsson, Per M., additional, Avolio, Alberto, additional, Bachler, Martin, additional, Bäck, Magnus, additional, Bashline, Michael J., additional, Becker-Greene, Dakota, additional, Bellinge, Jamie, additional, Bennasroune, Amar, additional, Blaise, Sébastien, additional, Borlaug, Barry A., additional, Boutouyrie, Pierre, additional, Breet, Y., additional, Breslin, Jerome W., additional, Budoff, Matthew J., additional, Butlin, Mark, additional, Cecelja, Marina, additional, Chen, Chen-Huan, additional, Cheng, Hao-Min, additional, Cheng, Yi-Bang, additional, Chirinos, Julio A., additional, Chowienczyk, Phil, additional, Chuang, Shao-Yuan, additional, Clavel, Marie-Annick, additional, Cohen, Jordana B., additional, Corcoran, Alexis M., additional, Cornwell, William K., additional, Corrales–Medina, Vicente F., additional, Côté, Nancy, additional, Coutinho, Thais, additional, Cox, James, additional, Cruickshank, J.K., additional, Dai, Lu, additional, Daskalopoulou, Stella S., additional, Davy, Kevin P., additional, De Buyzere, Marc L., additional, Dieffenbach, Paul B., additional, Duca, Laurent, additional, Dwivedi, Girish, additional, Edwards, David G., additional, Farquhar, William B., additional, Fernhall, Bo, additional, Floras, John S., additional, Fredenburgh, Laura E., additional, Fukumitsu, Masafumi, additional, Gafane-Matemane, L., additional, Gahungu, Nestor, additional, Ghanem, Ahmed K., additional, Gillebert, Thierry C., additional, Gillery, Philippe, additional, Gomez, Delphine, additional, Guzzetti, Ezequiel, additional, Hametner, Bernhard, additional, Hashimoto, Junichiro, additional, Heffernan, Kevin S., additional, Hibner, Brooks A., additional, Hobson, Sam, additional, Hu, Nien-Wen, additional, Hughes, T.M., additional, Humphrey, Jay D., additional, Jaisson, Stéphane, additional, Kachenoura, Nadjia, additional, Kario, Kazuomi, additional, Katakam, Prasad V.G., additional, Katsuumi, Goro, additional, Kondiboyina, Avinash, additional, Kovács, Sándor J., additional, Kruger, R., additional, Kublickiene, Karolina, additional, Lacolley, Patrick, additional, Laffargue, Muriel, additional, Lampejo, Arinola O., additional, Laucyte-Cibulskiene, Agne, additional, Laurent, Stéphane, additional, Lee, Hae-Young, additional, Lefferts, Wesley K., additional, Lefferts, Elizabeth C., additional, Leite-Moreira, Adelino F., additional, Liew, Chee H., additional, Lima, Joao A.C., additional, Lourenço, André P., additional, Maki-Petaja, Kaisa, additional, Maracle, Marcy, additional, Martiny, Laurent, additional, Maurice, Pascal, additional, Mayer, Christopher C., additional, McDonnell, Barry J., additional, McEvoy, John W., additional, Meyer, M.L., additional, Michel, Jean-Baptiste, additional, Millar, Philip J., additional, Minamino, Tohru, additional, Mitchell, Gary F., additional, Murfee, Walter L., additional, Mynard, Jonathan P., additional, Nardone, Massimo, additional, Nilsson, Peter M., additional, O'Gallagher, Kevin, additional, Ohyama, Yoshiaki, additional, Omote, Kazunori, additional, Park, Jeong Bae, additional, Peirce, Shayn M., additional, Pibarot, Philippe, additional, Pierce, Gary L., additional, Prenner, Stuart B., additional, Protogerou, Athanase, additional, Pyeritz, Reed E., additional, Quail, Michael A., additional, Reddy, Yogesh N.V., additional, Redheuil, Alban, additional, Regnault, Véronique, additional, Rehman, Rakhshinda, additional, Rietzschel, Ernst R., additional, Romier-Crouzet, Béatrice, additional, Rooprai, Jasjit, additional, Salvi, Lucia, additional, Salvi, Paolo, additional, Sartelet, Hervé, additional, Schmelzer, Christian E.H., additional, Schutte, A.E., additional, Schwarz, Angelina, additional, Segers, Patrick, additional, Sharman, James E., additional, Shimizu, Ippei, additional, Simon, Marc A., additional, Sosa, Piera, additional, Spronck, Bart, additional, Stenvinkel, Peter, additional, Stöhr, Eric J., additional, Strauss-Kruger, M., additional, Suarez-Martinez, Ariana, additional, Suda, Masayoshi, additional, Sung, Shih-Hsien, additional, Tan, Isabella, additional, Terentes-Printzios, Dimitrios, additional, Townsend, Raymond R., additional, Tran, Andrew H., additional, Urbina, Elaine M., additional, Venkatesh, Bharath Ambale, additional, Vlachopoulos, Charalambos, additional, Vonk Noordegraaf, Anton, additional, Wahart, Amandine, additional, Wang, Ji-Guang, additional, Wassertheurer, Siegfried, additional, Webb, Andrew James, additional, Weber, Thomas, additional, Westerhof, Berend E., additional, Wilkinson, Ian B., additional, and Yoshida, Yohko, additional

Published
2022
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34. Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry

Author

Hoeper, Marius M., Pausch, Christine, Grünig, Ekkehard, Klose, Hans, Staehler, Gerd, Huscher, Doerte, Pittrow, David, Olsson, Karen M., Vizza, Carmine Dario, Gall, Henning, Benjamin, Nicola, Distler, Oliver, Opitz, Christian, Gibbs, J. Simon R., Delcroix, Marion, Ghofrani, H. Ardeschir, Rosenkranz, Stephan, Ewert, Ralf, Kaemmerer, Harald, Lange, Tobias J., Kabitz, Hans-Joachim, Skowasch, Dirk, Skride, Andris, Jureviciene, Elena, Paleviciute, Egle, Miliauskas, Skaidrius, Claussen, Martin, Behr, Juergen, Milger, Katrin, Halank, Michael, Wilkens, Heinrike, Wirtz, Hubert, Pfeuffer-Jovic, Elena, Harbaum, Lars, Scholtz, Werner, Dumitrescu, Daniel, Bruch, Leonhard, Coghlan, Gerry, Neurohr, Claus, Tsangaris, Iraklis, Gorenflo, Matthias, Scelsi, Laura, Vonk-Noordegraaf, Anton, Ulrich, Silvia, and Held, Matthias

Published
2020
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38. 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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39. 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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40. 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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41. 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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42. 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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43. Usefulness of standard computed tomography pulmonary angiography performed for acute pulmonary embolism for identification of chronic thromboembolic pulmonary hypertension: results of the InShape III study

Author

Ende-Verhaar, Yvonne M., Meijboom, Lilian J., Kroft, Lucia J.M., Beenen, Ludo F.M., Boon, Gudula J.A.M., Middeldorp, Saskia, Nossent, Esther J., Symersky, Petr, Huisman, Menno V., Bogaard, Harm Jan, Vonk Noordegraaf, Anton, and Klok, Frederikus A.

Published
2019
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44. 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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45. Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis.

Author

Distler, Oliver, Ofner, Christian, Huscher, Dörte, Jordan, Suzana, Ulrich, Silvia, Stähler, Gerd, Grünig, Ekkehard, Held, Matthias, Ghofrani, H Ardeschir, Claussen, Martin, Lange, Tobias J, Klose, Hans, Rosenkranz, Stephan, Vonk-Noordegraaf, Anton, Vizza, C Dario, Delcroix, Marion, Opitz, Christian, Pausch, Christine, Scelsi, Laura, and Neurohr, Claus

Subjects
COMBINATION drug therapy, SURVIVAL rate, RESEARCH funding, LONG-term health care, FISHER exact test, TREATMENT effectiveness, DESCRIPTIVE statistics, SYSTEMIC lupus erythematosus, MANN Whitney U Test, CHI-squared test, CONNECTIVE tissue diseases, PHOSPHODIESTERASE inhibitors, SYSTEMIC scleroderma, PULMONARY arterial hypertension, COMPARATIVE studies, DATA analysis software, ENDOTHELINS, CELL receptors, CHEMICAL inhibitors
Abstract

Objectives Pulmonary arterial hypertension (PAH) occurs in various connective tissue diseases (CTDs). We sought to assess contemporary treatment patterns and survival of patients with various forms of CTD-PAH. Methods We analysed data from COMPERA, a European pulmonary hypertension registry, to describe treatment strategies and survival in patients with newly diagnosed PAH associated with SSc, SLE, MCTD, UCTD and other types of CTD. All-cause mortality was analysed according to the underlying CTD. For patients with SSc-PAH, we also assessed survival according to initial therapy with endothelin receptor antagonists (ERAs), phosphodiesterase type 5 inhibitors (PDE5is) or a combination of these two drug classes. Results This analysis included 607 patients with CTD-PAH. Survival estimates at 1, 3 and 5 years for SSc-PAH (n  = 390) were 85%, 59% and 42%; for SLE-PAH (n  = 34) they were 97%, 77% and 61%; for MCTD-PAH (n  = 33) they were 97%, 70% and 59%; for UCTD-PAH (n  = 60) they were 88%, 67% and 52%; and for other CTD-PAH (n  = 90) they were 92%, 69% and 55%, respectively. After multivariable adjustment, the survival of patients with SSc-PAH was significantly worse compared with the other conditions (P  = 0.001). In these patients, the survival estimates were significantly better with initial ERA–PDE5i combination therapy than with initial ERA or PDE5i monotherapy (P  = 0.016 and P  = 0.012, respectively). Conclusions Mortality remains high in patients with CTD-PAH, especially for patients with SSc-PAH. However, for patients with SSc-PAH, our results suggest that long-term survival may be improved with initial ERA–PDE5i combination therapy compared with initial monotherapy. [ABSTRACT FROM AUTHOR]

Published
2024
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47. CTEPH patients with 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, Meijboom, Lilian, additional, Symersky, Petr, additional, Winkelman, Toon, additional, Nossent, Esther, additional, Aman, Jurjan, additional, Bogaard, Harm Jan, additional, Vonk Noordegraaf, Anton, additional, and Van Es, Josien, additional

Published
2023
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48. Long-term effects of pulmonary endarterectomy on cardiopulmonary hemodynamics and exercise capacity in chronic thromboembolic pulmonary hypertension: London and Amsterdam experience

Author

Kianzad, Azar, primary, Baccelli, Andrea, additional, Braams, Natalia J., additional, Andersen, Stine, additional, Van Wezenbeek, Jessie, additional, Wessels, Jeroen N., additional, Celant, Lucas R., additional, Vos, Anna E., additional, Davies, Rachel, additional, Lo Giudice, Francesco, additional, Haji, Gulammehdi, additional, Rinaldo, Rocco F., additional, Vigo, Beatrice, additional, Symersky, Petr, additional, Winkelman, Toon A., additional, Boonstra, Anco, additional, Nossent, Esther J., additional, Marcus, J. Tim, additional, Vonk Noordegraaf, Anton, additional, Meijboom, Lilian J., additional, De Man, Frances S., additional, Andersen, Asger, additional, Howard, Luke S., additional, and Bogaard, Harm Jan, additional

Published
2023
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