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780 results on '"Pepke-Zaba, Joanna"'

1. Blood DNA methylation profiling identifies cathepsin Z dysregulation in pulmonary arterial hypertension

Author

Ulrich, Anna, Wu, Yukyee, Draisma, Harmen, Wharton, John, Swietlik, Emilia M., Cebola, Inês, Vasilaki, Eleni, Balkhiyarova, Zhanna, Jarvelin, Marjo-Riitta, Auvinen, Juha, Herzig, Karl-Heinz, Coghlan, J. Gerry, Lordan, James, Church, Colin, Howard, Luke S., Pepke-Zaba, Joanna, Toshner, Mark, Wort, Stephen J., Kiely, David G., Condliffe, Robin, Lawrie, Allan, Gräf, Stefan, Morrell, Nicholas W., Wilkins, Martin R., Prokopenko, Inga, and Rhodes, Christopher J.

Published
2024
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2. Oral Treprostinil is Associated with Improved Survival in FREEDOM-EV and its Open-Label Extension

Author

Grünig, Ekkehard, Rahaghi, Franck, Elwing, Jean, Vizza, Carmine Dario, Pepke-Zaba, Joanna, Shen, Jieyan, Yao, Hua, Hage, Antoine, Rosenkranz, Stephan, Vonk, Madelon, Balasubramanian, Vijay, Yuanhua, Yang, Yu, Zaixin, Lordan, James, Cadaret, Linda, Grover, Rob, Ousmanou, Aliou, Seaman, Scott, Deng, Chunqin, Broderick, Meredith, and White, R. James

Published
2024
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3. Highlights from the International Chronic Thromboembolic Pulmonary Hypertension Congress 2021

Author

Simonneau, Gérald, Fadel, Elie, Noordegraaf, Anton Vonk, 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, and Mayer, Eckhard

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

5. Seralutinib in adults with pulmonary arterial hypertension (TORREY): a randomised, double-blind, placebo-controlled phase 2 trial

Author

Adir, Yochai, Baillie, Timothy, Baratz, David, Burger, Charles, Chakinala, Murali M., Cifrián Martínez, Jose Manual, Delcroix, Marion, Dwyer, Nathan, Elwing, Jean M., Fisher, Micah, Franco, Veronica, Grünig, Ekkehard, Highland, Kristin, Hill, Nicholas, Hirani, Naushad, Hoeper, Marius, Jansa, Pavel, Keogh, Anne, Kingrey, John, Lopez-Meseguer, Manuel, McConnell, John W., Mehta, Sanjay, Melendres-Groves, Lana, Opitz, Christian, Pepke-Zaba, Joanna, Pillutla, Priya, Rahaghi, Franck, Raina, Amresh, Raviv, Yael, Robinson, Jeffrey, Ryan, John, Sager, Jeffrey, Shapiro, Shelley, Simon, Marc, Smith, Kerri, Sobol, Irina R., Sood, Namita, Spikes, Leslie A., Stadler, Stefan, Stevens, Wendy, Sulica, Roxana, White, R. James, Frantz, Robert P, McLaughlin, Vallerie V, Sahay, Sandeep, Escribano Subías, Pilar, Zolty, Ronald L, Benza, Raymond L, Channick, Richard N, Chin, Kelly M, Hemnes, Anna R, Howard, Luke S, Sitbon, Olivier, Vachiéry, Jean-Luc, Zamanian, Roham T, Cravets, Matt, Roscigno, Robert F, Mottola, David, Osterhout, Robin, Bruey, Jean-Marie, Elman, Erin, Tompkins, Cindy-ann, Parsley, Ed, Aranda, Richard, Zisman, Lawrence S, and Ghofrani, Hossein-Ardeschir

Published
2024
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7. Current strategies for managing chronic thromboembolic pulmonary hypertension: results of the worldwide prospective CTEPH Registry

Author

Guth, Stefan, D'Armini, Andrea M, Delcroix, Marion, Nakayama, Kazuhiko, Fadel, Elie, Hoole, Stephen P, Jenkins, David P, Kiely, David G, Kim, Nick H, Lang, Irene M, Madani, Michael M, Matsubara, Hiromi, Ogawa, Aiko, Ota-Arakaki, Jaquelina S, Quarck, Rozenn, Sadushi-Kolici, Roela, Simonneau, Gérald, Wiedenroth, Christoph B, Yildizeli, Bedrettin, Mayer, Eckhard, and Pepke-Zaba, Joanna

Subjects
Rare Diseases, Lung, Cardiovascular
Abstract

Pulmonary endarterectomy (PEA), pulmonary arterial hypertension (PAH) therapy and balloon pulmonary angioplasty (BPA) are currently accepted therapies for chronic thromboembolic pulmonary hypertension (CTEPH). This international CTEPH Registry identifies clinical characteristics of patients, diagnostic algorithms and treatment decisions in a global context. 1010 newly diagnosed consecutive patients were included in the registry between February 2015 and September 2016. Diagnosis was confirmed by right heart catheterisation, ventilation-perfusion lung scan, computerised pulmonary angiography and/or invasive pulmonary angiography after at least 3 months on anticoagulation. Overall, 649 patients (64.3%) were considered for PEA, 193 (19.1%) for BPA, 20 (2.0%) for both PEA and BPA, and 148 (14.7%) for PAH therapy only. Reasons for PEA inoperability were technical inaccessibility (n=235), comorbidities (n=63) and patient refusal (n=44). In Europe and America and other countries (AAO), 72% of patients were deemed suitable for PEA, whereas in Japan, 70% of patients were offered BPA as first choice. Sex was evenly balanced, except in Japan where 75% of patients were female. A history of acute pulmonary embolism was reported for 65.6% of patients. At least one PAH therapy was initiated in 35.8% of patients (26.2% of PEA candidates, 54.5% of BPA candidates and 54.1% of those not eligible for either PEA or BPA). At the time of analysis, 39 patients (3.9%) had died of pulmonary hypertension-related causes (3.5% after PEA and 1.8% after BPA). The registry revealed noticeable differences in patient characteristics (rates of pulmonary embolism and sex) and therapeutic approaches in Japan compared with Europe and AAO.

Published
2021

10. 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, Ross, Robert V MacKenzie, 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, Noordegraaf, Anton Vonk, Waisfisz, Quinten, and Walsworth, Anna K

Subjects
Biomedical and Clinical Sciences, Cardiovascular Medicine and Haematology, Clinical Sciences, Genetics, Human Genome, Lung, Aetiology, 2.1 Biological and endogenous factors, Cardiovascular, Female, Genetic Predisposition to Disease, Genetic Variation, Genome-Wide Association Study, Genotyping Techniques, HLA-DP alpha-Chains, HLA-DP beta-Chains, Humans, Male, Middle Aged, Polymorphism, Single Nucleotide, Pulmonary Arterial Hypertension, Risk Assessment, SOXF Transcription Factors, Signal Transduction, Survival Analysis, UK NIHR BioResource Rare Diseases Consortium, UK PAH Cohort Study Consortium, US PAH Biobank Consortium, Public Health and Health Services, Other Medical and Health Sciences, Cardiovascular medicine and haematology, Clinical 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

11. Author Correction: Biological heterogeneity in idiopathic pulmonary arterial hypertension identified through unsupervised transcriptomic profiling of whole blood

Author

Kariotis, Sokratis, Jammeh, Emmanuel, Swietlik, Emilia M., Pickworth, Josephine A., Rhodes, Christopher J., Otero, Pablo, Wharton, John, Iremonger, James, Dunning, Mark J., Pandya, Divya, Mascarenhas, Thomas S., Errington, Niamh, Thompson, A. A. Roger, Romanoski, Casey E., Rischard, Franz, Garcia, Joe G. N., Yuan, Jason X.-J., An, Tae-Hwi Schwantes, Desai, Ankit A., Coghlan, Gerry, Lordan, Jim, Corris, Paul A., Howard, Luke S., Condliffe, Robin, Kiely, David G., Church, Colin, Pepke-Zaba, Joanna, Toshner, Mark, Wort, Stephen, Gräf, Stefan, Morrell, Nicholas W., Wilkins, Martin R., Lawrie, Allan, and Wang, Dennis

Published
2022
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12. 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

13. Shared and Distinct Genomics of Chronic Thromboembolic Pulmonary Hypertension and Pulmonary Embolism

Author

Liley, James, primary, Newnham, Michael, additional, Bleda, Marta, additional, Bunclark, Katherine, additional, Auger, William, additional, Barbera, Joan Albert, additional, Bogaard, Harm, additional, Delcroix, Marion, additional, Fernandes, Timothy M., additional, Howard, Luke, additional, Jenkins, David, additional, Lang, Irene, additional, Mayer, Eckhard, additional, Rhodes, Chris, additional, Simpson, Michael, additional, Southgate, Laura, additional, Trembath, Richard, additional, Wharton, John, additional, Wilkins, Martin R, additional, Gräf, Stefan, additional, Morrell, Nicholas, additional, Pepke Zaba, Joanna, additional, and Toshner, Mark, additional

Published
2024
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14. Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry

Author

Caneva, Jorge, Tuhay, Graciela, Diez, Mirta, Talavera, Maria Lujan, Acosta, Adriana, Vulcano, Norberto, Bosio, Martin, Maldonado, Lorena, Deleo, Sabino, Melatini, Luciano, Keogh, Anne, Kotlyar, Eugene, Feenstra, John, Dwyer, Nathan, Adams, Heath, Stevens, Wendy, Steele, Peter, Proudman, Susanna, Minson, Robert, Reeves, Glenn, Lavender, Melanie, Ng, Benjamin, Mackenzie, Michele, Barry, Lisa, Gruenberger, Margarethe, Huber, Charlotte, Lang, Irene, Tilea, Ioana, Sadushi-Kolici, Roela, Löffler-Ragg, Judith, Feistmantl, Lisa-Theresa, Evrard, Patrick, Louis, Renaud, Guiot, Julien, Naldi, Marco, De Pauw, Michel, Mehta, Sanjay, Camacho, Rafael Conde, Tovar, Patricia Parada, Londoño, Alejandro, Campo, Felipe, Garcia, Paula, Lema, Camila, Orozco-Levi, Mauricio, Martinez, William, Gomez, Juan Esteban, Nielsen-Kudsk, Jens Erik, Mellemkjaer, Soren, Anton, Ly, Altraja, Alan, Vihinen, Tapani, Vasankari, Tuija, Sitbon, Olivier, Cottin, Vincent, Têtu, Laurent, Noël-Savina, Elise, Shearman, Nicole, Tayler, Susanne, Olzik, Ilona, Kulka, Christine, Grimminger, Jan, Simon, Marcel, Nolde, Anna, Oqueka, Tim, Harbaum, Lars, Egenlauf, Benjamin, Ewert, Ralf, Schulz, Christian, Regotta, Sabine, Kramer, Tilmann, Knoop-Busch, Susanne, Gerhardt, Felix, Konstantinides, Stavros, Pitsiou, Georgia, Stanopoulos, Ioannis, Sourla, Evdokia, Mouratoglou, Sofia, Karvounis, Haralambos, Pappas, Athanasios, Georgopoulos, Dimitrios, Fanaridis, Michail, Mitrouska, Ioanna, Michalis, Lampros, Pappas, Konstantinos, Kotsia, Anna, Gaine, Sean, Vizza, Carmine Dario, Manzi, Giovanna, Poscia, Roberto, Badagliacca, Roberto, Agostoni, Piergiuseppe, Bruno, Noemi, Farina, Stefania, D'Alto, Michele, Argiento, Paola, Correra, Anna, Di Marco, Giovanni Maria, Cresci, Chiara, Vannucchi, Vieri, Torricelli, Elena, Garcea, Alessio, Pesci, Alberto, Sardella, Luca, Paciocco, Giuseppe, Pane, Federico, D'Armini, Andrea Maria, Pin, Maurizio, Grazioli, Valentina, Massola, Giulia, Sciortino, Antonio, Prediletto, Renato, Bauleo, Carolina, Airò, Edoardo, Ndreu, Rudina, Pavlickova, Ivana, Lunardi, Claudio, Mulè, Massimiliano, Farruggio, Silvia, Costa, Serena, Galgano, Giuseppe, Petruzzi, Mario, De Luca, Anna, Lombardi, Francesco, Roncon, Loris, Conte, Luca, Picariello, Claudio, Wirtz, Gil, Alexandre, Myriam, Vonk-Noordegraaf, A., Boogaard, H., Mager, J., Reesink, H., van den Toorn, Leon M., Boomars, Karin, Andreassen, Arne K., Castro, Graça, Tania, Gonçalves, Baptista, Rui, Marinho, António, Shiang, Teresa, Oliveira, Ana, Coutinho, Daniel, Sousa, Joana, Loureiro, Maria José, Repolho, Débora, Martins Jesus, Susana Maria, Capinha, Marta, Agostinho, João, Cardoso, Tania, Rocha, Andreia, Espinha, Mafalda, Ivanov, Kyundyul Ivanovich, Alexeeva, Dalyana Eduardovna, Batalina, Marina Vadimovna, Hegya, Daria Viktorovna, Zvereva, Tatyana Nikolaevna, Avdeev, Sergey Nikolaevich, Tsareva, Natalia Anatolievna, Galyavich, Albert Sarvatovich, Nikolaevich, Bykov Aleksander, Filippov, Evgeny Vladimirovich, Yakovleva, Olga Eduardovna, Pavlova, Olga Borisovna, Skripkina, Elena Sergeevna, Martynyuk, Tamila Vitalievna, Bukatova, Irina Fedorovna, Tregubova, Anna Viktorovna, Platonov, Dmitry Yurievich, Kolomeytseva, Tatyana Mikhaylovna, Al Dalaan, Abdullah, Abdelsayed, Abeer Abeer, Weheba, Ihab, Saleemi, Sarferaz, Sakkijha, Hussam, Bohacekova, Marcela, Valkovicova, Tatiana, Farkasova, Iveta, Quezada, Carlos Andres, Piccari, Lucilla, Blanco, Isabel, Sebastian, Laura, Roman, Antonio, Lopez, Manuel, Otero, Remedios, Elias, Teresa, Jara, Luis, Asencio, Isabel, Arjona, Josefa Jiménez, Almagro, Raúl Menor, Cárdenas, Salvador López, García, Salvador Alcaraz, Rodríguez, Patricia Villanueva, Lopez, Raquel, Garcia, Alberto, Avilés, Francisco Fernandez, De La Pava, Sebastian, Yotti, Raquel, Peñate, Gregorio Pérez, Marrero, Fernando León, Cifrián Martínez, José Manuel, Martinez-Meñaca, Amaya, Alonso, Lecue Pilar, Rozas, Sonia Fernandez, Fernandez, David Iturbe, Cuesta, Victor Mora, Söderberg, Stefan, Bartfay, Sven-Erik, Rundqvist, Bengt, Alfetlawi, Monthir, Wodlin, Peter, Schwarz, Esther Irene, Speich, Rudolf, Lador, Frédéric, Rochat, Thierry, Gasche-Soccal, Paola, Hsu, Chih-Hsin, Lin, Tsung-Hsien, Su, Ho-Ming, Lai, Wen-Ter, Chu, Chun Yuan, Hsu, Po-Chao, Voon, Wen-Chol, Yen, Hsueh-Wei, Yih-Jer Wu, Jacob, Wu, Shu-Hao, Huang, Wen-Pin, Fong, Man-Cai, Huang, Chien-Lung, Kuo, Ping-Hung, Lin, Yen-Hung, Lin, Jiunn-Lee, Hung, Chi-Sheng, Wu, Cho-Kai, Sung, Shih-Hsien, Huang, Wei-Chun, Cheng, Chin-Chang, Kuo, Shu-Hung, Wang, Wen-Hwa, Ho, Wan-Jing, Hsu, Tsu-Shiu, Mutlu, Bülent, Atas, Halil, Ongen, Gul, Un, Zeynep, Okumus, Gulfer, Hanta, Ismail, Corris, Paul, Peacock, Andrew, Church, Colin, Toshner, Mark, Newnham, Michael, Ghofrani, Hossein-Ardeschir, Gomez Sanchez, Miguel-Angel, Humbert, Marc, Pittrow, David, Simonneau, Gérald, Gall, Henning, Grünig, Ekkehard, Klose, Hans, Halank, Michael, Langleben, David, Snijder, Repke J., Escribano Subias, Pilar, Mielniczuk, Lisa M., Lange, Tobias J., Vachiéry, Jean-Luc, Wirtz, Hubert, Helmersen, Douglas S., Tsangaris, Iraklis, Barberá, Joan A., Pepke-Zaba, Joanna, Boonstra, Anco, Rosenkranz, Stephan, Ulrich, Silvia, Steringer-Mascherbauer, Regina, Delcroix, Marion, Jansa, Pavel, Šimková, Iveta, Giannakoulas, George, Klotsche, Jens, Williams, Evgenia, Meier, Christian, and Hoeper, Marius M.

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

Author

Caneva, Jorge, Tuhay, Graciela, Diez, Mirta, Talavera, Maria Lujan, Acosta, Adriana, Vulcano, Norberto, Bosio, Martin, Maldonado, Lorena, Deleo, Sabino, Melatini, Luciano, Keogh, Anne, Kotlyar, Eugene, Feenstra, John, Dwyer, Nathan, Adams, Heath, Stevens, Wendy, Steele, Peter, Proudman, Susanna, Minson, Robert, Reeves, Glenn, Lavender, Melanie, Ng, Benjamin, Mackenzie, Michele, Barry, Lisa, Gruenberger, Margarethe, Huber, Charlotte, Lang, Irene, Tilea, Ioana, Sadushi-Kolici, Roela, Löffler-Ragg, Judith, Feistmantl, Lisa-Theresa, Evrard, Patrick, Guiot, Julien, Naldi, Marco, De Pauw, Michel, Louis, Renaud, Mehta, Sanjay, Camacho, Rafael Conde, Tovar, Patricia Parada, Londoño, Alejandro, Campo, Felipe, Garcia, Paula, Lema, Camila, Orozco-Levi, Mauricio, Martinez, William, Gomez, Juan Esteban, Nielsen-Kudsk, Jens Erik, Mellemkjaer, Soren, Anton, Ly, Altraja, Alan, Vihinen, Tapani, Vasankari, Tuija, Sitbon, Olivier, Cottin, Vincent, Têtu, Laurent, Noël-Savina, Elise, Shearman, Nicole, Tayler, Susanne, Olzik, Ilona, Kulka, Christine, Grimminger, Jan, Simon, Marcel, Nolde, Anna, Oqueka, Tim, Harbaum, Lars, Egenlauf, Benjamin, Ewert, Ralf, Schulz, Christian, Regotta, Sabine, Kramer, Tilmann, Knoop-Busch, Susanne, Gerhardt, Felix, Konstantinides, Stavros, Pitsiou, Georgia, Stanopoulos, Ioannis, Sourla, Evdokia, Mouratoglou, Sofia, Karvounis, Haralambos, Pappas, Athanasios, Mitrouska, Ioanna, Georgopoulos, Dimitrios, Fanaridis, Michail, Michalis, Lampros, Pappas, Konstantinos, Kotsia, Anna, Gaine, Sean, Vizza, Carmine Dario, Manzi, Giovanna, Poscia, Roberto, Badagliacca, Roberto, Agostoni, Piergiuseppe, Bruno, Noemi, Farina, Stefania, D'Alto, Michele, Argiento, Paola, Correra, Anna, Di Marco, Giovanni Maria, Cresci, Chiara, Vannucchi, Vieri, Torricelli, Elena, Garcea, Alessio, Pesci, Alberto, Sardella, Luca, Paciocco, Giuseppe, Pane, Federico, D'Armini, Andrea Maria, Pin, Maurizio, Grazioli, Valentina, Massola, Giulia, Sciortino, Antonio, Prediletto, Renato, Bauleo, Carolina, Airò, Edoardo, Ndreu, Rudina, Pavlickova, Ivana, Lunardi, Claudio, Farruggio, Silvia, Costa, Serena, Mulè, Massimiliano, Galgano, Giuseppe, Petruzzi, Mario, De Luca, Anna, Lombardi, Francesco, Roncon, Loris, Conte, Luca, Picariello, Claudio, Wirtz, Gil, Alexandre, Myriam, Vonk-Noordegraaf, A., Boogaard, H., Mager, J., Reesink, H., van den Toorn, Leon M., Boomars, Karin, Andreassen, Arne K., Castro, Graça, Tania, Gonçalves, Baptista, Rui, Marinho, António, Shiang, Teresa, Oliveira, Ana, Coutinho, Daniel, Sousa, Joana, Loureiro, Maria José, Repolho, Débora, Martins Jesus, Susana Maria, Capinha, Marta, Agostinho, João, Cardoso, Tania, Rocha, Andreia, Espinha, Mafalda, Ivanov, Kyundyul Ivanovich, Alexeeva, Dalyana Eduardovna, Batalina, Marina Vadimovna, Hegya, Daria Viktorovna, Zvereva, Tatyana Nikolaevna, Avdeev, Sergey Nikolaevich, Tsareva, Natalia Anatolievna, Galyavich, Albert Sarvatovich, Nikolaevich, Bykov Aleksander, Filippov, Evgeny Vladimirovich, Yakovleva, Olga Eduardovna, Pavlova, Olga Borisovna, Skripkina, Elena Sergeevna, Martynyuk, Tamila Vitalievna, Bukatova, Irina Fedorovna, Tregubova, Anna Viktorovna, Platonov, Dmitry Yurievich, Kolomeytseva, Tatyana Mikhaylovna, Al Dalaan, Abdullah, Abdelsayed, Abeer Abeer, Weheba, Ihab, Saleemi, Sarferaz, Sakkijha, Hussam, Bohacekova, Marcela, Valkovicova, Tatiana, Farkasova, Iveta, Quezada, Carlos Andres, Piccari, Lucilla, Blanco, Isabel, Sebastian, Laura, Roman, Antonio, Lopez, Manuel, Otero, Remedios, Elias, Teresa, Jara, Luis, Asencio, Isabel, Arjona, Josefa Jiménez, Almagro, Raúl Menor, Cárdenas, Salvador López, García, Salvador Alcaraz, Rodríguez, Patricia Villanueva, Lopez, Raquel, Garcia, Alberto, Avilés, Francisco Fernandez, De La Pava, Sebastian, Yotti, Raquel, Peñate, Gregorio Pérez, Marrero, Fernando León, Cifrián Martínez, José Manuel, Martinez-Meñaca, Amaya, Alonso, Lecue Pilar, Rozas, Sonia Fernandez, Fernandez, David Iturbe, Cuesta, Victor Mora, Söderberg, Stefan, Bartfay, Sven-Erik, Rundqvist, Bengt, Alfetlawi, Monthir, Wodlin, Peter, Schwarz, Esther Irene, Speich, Rudolf, Lador, Frédéric, Rochat, Thierry, Gasche-Soccal, Paola, Hsu, Chih-Hsin, Lin, Tsung-Hsien, Su, Ho-Ming, Lai, Wen-Ter, Chu, Chun Yuan, Hsu, Po-Chao, Voon, Wen-Chol, Yen, Hsueh-Wei, Yih-Jer Wu, Jacob, Wu, Shu-Hao, Huang, Wen-Pin, Fong, Man-Cai, Huang, Chien-Lung, Kuo, Ping-Hung, Lin, Yen-Hung, Lin, Jiunn-Lee, Hung, Chi-Sheng, Wu, Cho-Kai, Sung, Shih-Hsien, Huang, Wei-Chun, Cheng, Chin-Chang, Kuo, Shu-Hung, Wang, Wen-Hwa, Ho, Wan-Jing, Hsu, Tsu-Shiu, Mutlu, Bülent, Atas, Halil, Ongen, Gul, Un, Zeynep, Okumus, Gulfer, Hanta, Ismail, Corris, Paul, Peacock, Andrew, Church, Colin, Toshner, Mark, Newnham, Michael, Hoeper, Marius M., Gomez Sanchez, Miguel-Angel, Humbert, Marc, Pittrow, David, Simonneau, Gérald, Gall, Henning, Grünig, Ekkehard, Klose, Hans, Halank, Michael, Langleben, David, Snijder, Repke J., Escribano Subias, Pilar, Mielniczuk, Lisa M., Lange, Tobias J., Vachiéry, Jean-Luc, Wirtz, Hubert, Helmersen, Douglas S., Tsangaris, Iraklis, Barberà, Joan A., Pepke-Zaba, Joanna, Boonstra, Anco, Rosenkranz, Stephan, Ulrich, Silvia, Steringer-Mascherbauer, Regina, Delcroix, Marion, Jansa, Pavel, Šimková, Iveta, Giannakoulas, George, Klotsche, Jens, Williams, Evgenia, Meier, Christian, and Ghofrani, Hossein-Ardeschir

Published
2021
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20. Biological heterogeneity in idiopathic pulmonary arterial hypertension identified through unsupervised transcriptomic profiling of whole blood

Author

Kariotis, Sokratis, Jammeh, Emmanuel, Swietlik, Emilia M., Pickworth, Josephine A., Rhodes, Christopher J., Otero, Pablo, Wharton, John, Iremonger, James, Dunning, Mark J., Pandya, Divya, Mascarenhas, Thomas S., Errington, Niamh, Thompson, A. A. Roger, Romanoski, Casey E., Rischard, Franz, Garcia, Joe G. N., Yuan, Jason X.-J., An, Tae-Hwi Schwantes, Desai, Ankit A., Coghlan, Gerry, Lordan, Jim, Corris, Paul A., Howard, Luke S., Condliffe, Robin, Kiely, David G., Church, Colin, Pepke-Zaba, Joanna, Toshner, Mark, Wort, Stephen, Gräf, Stefan, Morrell, Nicholas W., Wilkins, Martin R., Lawrie, Allan, and Wang, Dennis

Published
2021
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21. 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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22. The Digital 1-Minute Walk Test: A New Patient-centered Cardiorespiratory Endpoint.

Author

Robertson, Lucy, Newman, Joseph, Clayton, Shaun, Ferguson, Mary, Pepke-Zaba, Joanna, Cannon, John, Sheares, Karen, Taboada, Dolores, Bunclark, Katherine, Armstrong, Iain, Ferrer Mallol, Elisa, Davies, Elin Haf, and Toshner, Mark

Subjects
IDIOPATHIC interstitial pneumonias, INTERSTITIAL lung diseases, DUST diseases, PULMONARY arterial hypertension, NOSOLOGY, PULMONARY fibrosis
Abstract

The article focuses on exploring the feasibility and validity of shorter walk tests, particularly the 1-minute walk test (1MWT), as alternatives to the traditional 6-minute walk test (6MWT) in assessing exercise capacity in cardiorespiratory diseases like pulmonary hypertension (PH) and interstitial lung disease (ILD). It challenges the convention of the six minute duration and investigates the practicality of conducting shorter walk tests in a community setting using digital technology.

Published
2024
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25. An open-source tool for risk prediction in operable CTEPH

Author

Bunclark, Katherine, primary, Liley, James, additional, Ruggiero, Alessandro, additional, Cannon, John, additional, Coghan, Gerry, additional, Lordan, James, additional, Howard, Luke, additional, Jenkins, David, additional, Johnson, Martin, additional, Kiely, David, additional, Ng, Choo, additional, Screaton, Nicholas, additional, Sheares, Karen, additional, Taboada, Dolores, additional, Tsui, Steven, additional, Wort, John, additional, Pepke-Zaba, Joanna, additional, and Toshner, Mark, additional

Published
2023
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27. Left ventricular diastolic dysfunction attenuates outcomes in CTEPH

Author

Bunclark, Katherine, primary, Fletcher, Andrew, additional, Ruggiero, Alessandro, additional, Cannon, John, additional, Coghlan, Gerry, additional, Lordan, James, additional, Howard, Luke, additional, Jenkins, David, additional, Johnson, Martin, additional, Kiely, David, additional, Ng, Choo, additional, Screaton, Nicholas, additional, Sheares, Karen, additional, Taboada, Dolores, additional, Tsui, Steven, additional, Wort, John, additional, Pepke-Zaba, Joanna, additional, and Toshner, Mark, additional

Published
2023
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30. Balloon pulmonary angioplasty outcomes in patients previously treated by pulmonary endarterectomy surgery are inferior to those of inoperable patients

Author

Kirkby, Louise C., primary, Rodgers, Matthew S., additional, Amaral‐Almeida, Liliana, additional, Sheares, Karen, additional, Toshner, Mark, additional, Bunclark, Katherine, additional, Bartnik, Aleksandra, additional, Taboada, Dolores, additional, Ng, Choo, additional, Taghavi, Fouad J., additional, Tsui, Steven, additional, Cannon, John E., additional, Weir‐McCall, Jonathan R., additional, Coghlan, John G., additional, Jenkins, David P., additional, Pepke‐Zaba, Joanna, additional, and Hoole, Stephen P., additional

Published
2023
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31. CTEPH has shared and distinct genetic associations with pulmonary embolism in a genome-wide association study

Author

Liley, James, primary, Newnham, Michael, additional, Bleda, Marta, additional, Bunclark, Katherine, additional, Auger, William, additional, Barbera, Joan Albert, additional, Bogaard, Harm, additional, Delcroix, Marion, additional, Fernandes, Timothy M., additional, Howard, Luke, additional, Jenkins, David, additional, Lang, Irene, additional, Mayer, Eckhard, additional, Rhodes, Chris, additional, Simpson, Michael, additional, Southgate, Laura, additional, Trembath, Richard, additional, Wharton, John, additional, Wilkins, Martin R, additional, Gräf, Stefan, additional, Morrell, Nicholas, additional, Pepke Zaba, Joanna, additional, and Toshner, Mark, additional

Published
2023
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32. Natural History of Chronic Thromboembolic Pulmonary Disease with no or mild Pulmonary Hypertension

Author

Ashwin Reddy, S, primary, Swietlik, Emilia M, additional, Robertson, Lucy, additional, Michael, Alice, additional, Boyle, Sonja, additional, Polwarth, Gary, additional, Screaton, Nick J, additional, Ruggiero, Alessandro, additional, Nethercott, Sarah L, additional, Taboada, Dolores, additional, Sheares, Karen K, additional, Haddinapola, Charaka, additional, Cannon, John E, additional, Bunclark, Katherine, additional, Jenkins, David, additional, Ng, Choo, additional, Toshner, Mark R, additional, and Pepke-Zaba, Joanna, additional

Published
2023
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33. 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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34. Chronic Thromboembolic Pulmonary Hypertension: A Review of the Multifaceted Pathobiology.

Author

Ghani, Hakim and Pepke-Zaba, Joanna

Subjects
VENOUS thrombosis, THROMBOEMBOLISM, ENDOTHELIUM diseases, PULMONARY hypertension, THROMBOSIS, INFLAMMATORY mediators
Abstract

Chronic thromboembolic pulmonary disease results from the incomplete resolution of thrombi, leading to fibrotic obstructions. These vascular obstructions and additional microvasculopathy may lead to chronic thromboembolic pulmonary hypertension (CTEPH) with increased pulmonary arterial pressure and pulmonary vascular resistance, which, if left untreated, can lead to right heart failure and death. The pathobiology of CTEPH has been challenging to unravel due to its rarity, possible interference of results with anticoagulation, difficulty in selecting the most relevant study time point in relation to presentation with acute pulmonary embolism (PE), and lack of animal models. In this article, we review the most relevant multifaceted cross-talking pathogenic mechanisms and advances in understanding the pathobiology in CTEPH, as well as its challenges and future direction. There appears to be a genetic background affecting the relevant pathological pathways. This includes genetic associations with dysfibrinogenemia resulting in fibrinolysis resistance, defective angiogenesis affecting thrombus resolution, and inflammatory mediators driving chronic inflammation in CTEPH. However, these are not necessarily specific to CTEPH and some of the pathways are also described in acute PE or deep vein thrombosis. In addition, there is a complex interplay between angiogenic and inflammatory mediators driving thrombus non-resolution, endothelial dysfunction, and vascular remodeling. Furthermore, there are data to suggest that infection, the microbiome, circulating microparticles, and the plasma metabolome are contributing to the pathobiology of CTEPH. [ABSTRACT FROM AUTHOR]

Published
2024
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36. Perioperative Management in Pulmonary Endarterectomy.

Author

Jenkins, David P., Martinez, Guillermo, Salaunkey, Kiran, Reddy, S. Ashwin, and Pepke-Zaba, Joanna

Subjects
ENDARTERECTOMY, PULMONARY hypertension, EXTRACORPOREAL membrane oxygenation, WATER-electrolyte balance (Physiology), TRANSLUMINAL angioplasty, POSTOPERATIVE care
Abstract

Pulmonary endarterectomy (PEA) is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (PH), provided lesions are proximal enough in the pulmonary vasculature to be surgically accessible and the patient is well enough to benefit from the operation in the longer term. It is a major cardiothoracic operation, requiring specialized techniques and instruments developed over several decades to access and dissect out the intra-arterial fibrotic material. While in-hospital operative mortality is low (<5%), particularly in high-volume centers, careful perioperative management in the operating theater and intensive care is mandatory to balance ventricular performance, fluid balance, ventilation, and coagulation to avoid or treat complications. Reperfusion pulmonary edema, airway hemorrhage, and right ventricular failure are the most problematic complications, often requiring the use of extracorporeal membrane oxygenation to bridge to recovery. Successful PEA has been shown to improve both morbidity and mortality in large registries, with survival >70% at 10 years. For patients not suitable for PEA or with residual PH after PEA, balloon pulmonary angioplasty and/or PH medical therapy may prove beneficial. Here, we describe the indications for PEA, specific surgical and perioperative strategies, postoperative monitoring and management, and approaches for managing residual PH in the long term. [ABSTRACT FROM AUTHOR]

Published
2023
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37. Predictors of outcomes in mild pulmonary hypertension according to 2022 ESC/ERS Guidelines: the EVIDENCE-PAH UK study.

Author

Karia, Nina, Howard, Luke, Johnson, Martin, Kiely, David G, Lordan, James, McCabe, Colm, Pepke-Zaba, Joanna, Ong, Rose, Preiss, Michael, Knight, Daniel, Muthurangu, Vivek, and Coghlan, J Gerry

Subjects
PULMONARY hypertension, PULMONARY arterial hypertension, CLINICAL trials, CARDIAC patients, CONNECTIVE tissue diseases
Abstract

Background and Aims Interventional studies in pulmonary arterial hypertension completed to date have shown to be effective in symptomatic patients with significantly elevated mean pulmonary artery pressure (mPAP) (≥25 mmHg) and pulmonary vascular resistance (PVR) > 3 Wood Unit (WU). However, in health the mPAP does not exceed 20 mmHg and PVR is 2 WU or lower, at rest. The ESC/ERS guidelines have recently been updated to reflect this. There is limited published data on the nature of these newly defined populations (mPAP 21–24 mmHg and PVR >2–≤3 WU) and the role of comorbidity in determining their natural history. With the change in guidelines, there is a need to understand this population and the impact of the ESC/ERS guidelines in greater detail. Methods A retrospective nationwide evaluation of the role of pulmonary haemodynamics and comorbidity in predicting survival among patients referred to the UK pulmonary hypertension (PH) centres between 2009 and 2017. In total, 2929 patients were included in the study. Patients were stratified by mPAP (<21 mmHg, 21–24 mmHg, and ≥25 mmHg) and PVR (≤2 WU, > 2–≤3 WU, and >3 WU), with 968 (33.0%) in the mPAP <21 mmHg group, 689 (23.5%) in the mPAP 21–24 mmHg group, and 1272 (43.4%) in the mPAP ≥25 mmHg group. Results Survival was negatively correlated with mPAP and PVR in the population as a whole. Survival in patients with mildly elevated mPAP (21–24 mmHg) or PVR (>2–≤3WU) was lower than among those with normal pressures (mPAP <21 mmHg) and normal PVR (PVR ≤ 2WU) independent of comorbid lung and heart disease [hazard ratio (HR) 1.36, 95% confidence interval (CI) 1.14–1.61, P =.0004 for mPAP vs. HR 1.28, 95% CI 1.10–1.49, P =.0012 for PVR]. Among patients with mildly elevated mPAP, a mildly elevated PVR remained an independent predictor of survival when adjusted for comorbid lung and heart disease (HR 1.33, 95% CI 1.01–1.75, P =.042 vs. HR 1.4, 95% CI 1.06–1.86, P =.019). 68.2% of patients with a mPAP 21–24 mmHg had evidence of underlying heart or lung disease. Patients with mildly abnormal haemodynamics were not more symptomatic than patients with normal haemodynamics. Excluding patients with heart and lung disease, connective tissue disease was associated with a poorer survival among those with PH. In this subpopulation evaluating those with a mPAP of 21–24 mmHg, survival curves only diverged after 5 years. Conclusions This study supports the change in diagnostic category of the ESC/ERS guidelines in a PH population. The newly included patients have an increased mortality independent of significant lung or heart disease. The majority of patients in this new category have underlying heart or lung disease rather than an isolated pulmonary vasculopathy. Mortality is higher if comorbidity is present. Rigorous phenotyping will be pivotal to determine which patients are at risk of progressive vasculopathic disease and in whom surveillance and recruitment to studies may be of benefit. This study provides an insight into the population defined by the new guidelines. [ABSTRACT FROM AUTHOR]

Published
2023
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38. Different Cytokine Patterns in BMPR2-Mutation-Positive Patients and Patients With Pulmonary Arterial Hypertension Without Mutations and Their Influence on Survival

Author

Schwiening, Max, Swietlik, Emilia M, Pandya, Divya, Burling, Keith, Barker, Peter, Feng, Oliver Y, Treacy, Carmen M, Abreu, Susana, Wort, S John, Pepke-Zaba, Joanna, Graf, Stefan, Marciniak, Stefan J, Morrell, Nicholas W, Soon, Elaine, Feng, Oliver [0000-0003-0039-7039], Marciniak, Stefan [0000-0001-8472-7183], Soon, Elaine [0000-0002-5744-5014], and Apollo - University of Cambridge Repository

Subjects
Pulmonary and Respiratory Medicine, Pulmonary Arterial Hypertension, Mutation, Cytokines, Humans, Familial Primary Pulmonary Hypertension, Bone Morphogenetic Protein Receptors, Type II, Cardiology and Cardiovascular Medicine, Critical Care and Intensive Care Medicine
Abstract

Pulmonary arterial hypertension (PAH) covers a range of life-limiting illnesses characterized by increased mean pulmonary arterial pressures, which if untreated, lead to right heart failure and death. This is due to remodeling of the small-to-medium sized pulmonary vessels, which obstruct blood flow. Pulmonary arterial hypertension can be further categorized into idiopathic PAH (without any identifiable cause, 6th World Symposium class 1.11) and heritable PAH , (defined by mutations in specific genes, 6th World Symposium class 1.21), the most common affecting bone morphogenetic protein receptor type II (BMPRII)2 3. It is known that BMPR2-mutation positive patients have worse cardiac indices at presentation and a worse overall outcome compared to PAH without mutations4. Possessing a BMPR2 mutation also creates a pro-inflammatory state, through loss of endothelial barrier function5 and loss of antioxidant capability6. This then begs the question as to whether the mutation-positive groups have different underlying pathogenetic mechanisms and require different biomarkers and treatments, analogous to how EGFR-mutation positive non-small cell lung cancer patients respond to tyrosine kinase inhibition while the majority of NSCLC patients do not.

Published
2022

39. Using the Plasma Proteome for Risk Stratifying Patients with Pulmonary Arterial Hypertension

Author

Rhodes, Christopher J, Wharton, John, Swietlik, Emilia M, Harbaum, Lars, Girerd, Barbara, Coghlan, J Gerry, Lordan, James, Church, Colin, Pepke-Zaba, Joanna, Toshner, Mark, Wort, Stephen J, Kiely, David G, Condliffe, Robin, Lawrie, Allan, Gräf, Stefan, Montani, David, Boucly, Athénaïs, Sitbon, Olivier, Humbert, Marc, Howard, Luke S, Morrell, Nicholas W, Wilkins, Martin R, Rhodes, Christopher J [0000-0002-4962-3204], Wharton, John [0000-0001-8110-2575], Harbaum, Lars [0000-0002-9422-6195], Lawrie, Allan [0000-0003-4192-9505], Gräf, Stefan [0000-0002-1315-8873], Montani, David [0000-0002-9358-6922], Boucly, Athénaïs [0000-0001-6246-5557], Humbert, Marc [0000-0003-0703-2892], Wilkins, Martin R [0000-0003-3926-1171], Apollo - University of Cambridge Repository, British Heart Foundation, and The Academy of Medical Sciences

Subjects
Pulmonary and Respiratory Medicine, Pulmonary Arterial Hypertension, Proteome, Respiratory System, Prognosis, Critical Care and Intensive Care Medicine, clinical outcomes, Peptide Fragments, Area Under Curve, Natriuretic Peptide, Brain, Humans, Familial Primary Pulmonary Hypertension, 11 Medical and Health Sciences, Biomarkers
Abstract

Rationale: NT-proBNP (N-terminal pro-brain natriuretic peptide), a biomarker of cardiac origin, is used to risk stratify patients with pulmonary arterial hypertension (PAH). Its limitations include poor sensitivity to early vascular pathology. Other biomarkers of vascular or systemic origin may also be useful in the management of PAH. Objectives: Identify prognostic proteins in PAH that complement NT-proBNP and clinical risk scores. Methods: An aptamer-based assay (SomaScan version 4) targeting 4,152 proteins was used to measure plasma proteins in patients with idiopathic, heritable, or drug-induced PAH from the UK National Cohort of PAH (n = 357) and the French EFORT (Evaluation of Prognostic Factors and Therapeutic Targets in PAH) study (n = 79). Prognostic proteins were identified in discovery-replication analyses of UK samples. Proteins independent of 6-minute-walk distance and NT-proBNP entered least absolute shrinkage and selection operator modeling, and the best combination in a single score was evaluated against clinical targets in EFORT. Measurements and Main Results: Thirty-one proteins robustly informed prognosis independent of NT-proBNP and 6-minute-walk distance in the UK cohort. A weighted combination score of six proteins was validated at baseline (5-yr mortality; area under the curve [AUC], 0.73; 95% confidence interval [CI], 0.63-0.85) and follow-up in EFORT (AUC, 0.84; 95% CI, 0.75-0.94; P = 9.96 × 10-6). The protein score risk stratified patients independent of established clinical targets and risk equations. The addition of the six-protein model score to NT-proBNP improved prediction of 5-year outcomes from AUC 0.762 (0.702-0.821) to 0.818 (0.767-0.869) by receiver operating characteristic analysis (P = 0.00426 for difference in AUC) in the UK replication and French samples combined. Conclusions: The plasma proteome informs prognosis beyond established factors in PAH and may provide a more sensitive measure of therapeutic response.

Published
2022

40. 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, primary, Gale, Chris P., additional, Brida, Margarita, additional, Giannakoulas, George, additional, Kovacs, Gabor, additional, Adir, Yochai, additional, Benza, Raymond L., additional, Böhm, Michael, additional, Coats, Andrew, additional, D'Alto, Michele, additional, Escribano‐Subias, Pilar, additional, Ferrari, Pisana, additional, Galiè, Nazzareno, additional, Gibbs, J. Simon R., additional, Gin‐Sing, Wendy, additional, Hoeper, Marius M., additional, Humbert, Marc, additional, Lang, Irene M., additional, Maron, Bradley A., additional, Meszaros, Gergely, additional, Noordegraaf, Anton V., additional, Price, Laura C., additional, Pepke‐Zaba, Joanna, additional, Rådegran, Göran, additional, Reis, Abilio, additional, Sitbon, Olivier, additional, Torbicki, Adam, additional, Ulrich, Silvia, additional, Rosenkranz, Stephan, additional, and Delcroix, Marion, additional

Published
2023
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41. Angiopoietin 2 and hsCRP are associated with pulmonary hemodynamics and long-term mortality respectively in CTEPH—Results from a prospective discovery and validation biomarker study

Author

Hadinnapola, Charaka M., primary, Southwood, Mark, additional, Hernández – Sánchez, Jules, additional, Bunclark, Katherine, additional, Newnham, Michael, additional, Swietlik, Emilia M., additional, Cannon, John, additional, Preston, Stephen D., additional, Sheares, Karen, additional, Taboada, Dolores, additional, Screaton, Nicholas, additional, Jenkins, David P., additional, Morrell, Nicholas W., additional, Toshner, Mark, additional, and Pepke-Zaba, Joanna, additional

Published
2023
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42. 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

43. 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

45. Right ventricular-pulmonary artery coupling in chronic thromboembolic pulmonary hypertension

Author

Bartnik, Aleksandra, Pepke-Zaba, Joanna, Hoole, Stephen P, White, Paul, Garbi, Madalina, Coghlan, John G, Taghavi, Fouad, Tsui, Steven, Weir-McCall, Jonathan, Bartnik, Aleksandra [0000-0002-7628-8659], Garbi, Madalina [0000-0001-9520-8186], and Apollo - University of Cambridge Repository

Subjects
cardiac catheterization, Treatment Outcome, pulmonary embolism, Hypertension, Pulmonary, Chronic Disease, Ventricular Function, Right, echocardiography, Humans, Heart, Endarterectomy, Pulmonary Artery, Magnetic Resonance Imaging, Angioplasty, Balloon
Abstract

Chronic thromboembolic pulmonary hypertension occurs in a proportion of patients with prior acute pulmonary embolism and is characterised by breathlessness, persistently raised pulmonary pressures and right heart failure. Surgical pulmonary endarterectomy (PEA) offers significant prognostic and symptomatic benefits for patients with proximal disease distribution. For those with inoperable disease, management options include balloon pulmonary angioplasty (BPA) and medical therapy. Current clinical practice relies on the evaluation of pulmonary haemodynamics to assess disease severity, timing of and response to treatment. However, pulmonary haemodynamics correlate poorly with patient symptoms, which are influenced by right ventricular tolerance of the increased afterload. How best to manage symptomatic patients with chronic thromboembolic pulmonary disease (CTEPD) in the absence of pulmonary hypertension is not resolved.Right ventricular-pulmonary artery coupling (RV-PAC) describes the energy transfer within the whole cardiopulmonary unit. Thus, it can identify the earliest signs of decompensation even before pulmonary hypertension is overt. Invasive measurement of coupling using pressure volume loop technology is well established in research settings. The development of efficient and less invasive measurement methods has revived interest in coupling as a viable clinical tool. Significant improvement in RV-PAC has been demonstrated after both PEA and BPA. Further studies are required to understand its clinical utility and prognostic value, in particular, its potential to guide management in patients with CTEPD. Finally, given the reported differences in coupling between sexes in pulmonary arterial hypertension, further work is required to understand the applicability of proposed thresholds for decoupling in therapeutic decision making.

Published
2023
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46. First Genotype-Phenotype Study in TBX4 Syndrome: Gain-of-Function Mutations Causative for Lung Disease

Author

Prapa, Matina, Lago-Docampo, Mauro, Swietlik, Emilia M, Montani, David, Eyries, Mélanie, Humbert, Marc, Welch, Carrie L, Chung, Wendy K, Berger, Rolf MF, Bogaard, Harm Jan, Danhaive, Olivier, Escribano-Subías, Pilar, Gall, Henning, Girerd, Barbara, Hernandez-Gonzalez, Ignacio, Holden, Simon, Hunt, David, Jansen, Samara MA, Kerstjens-Frederikse, Wilhelmina, Kiely, David G, Lapunzina, Pablo, McDermott, John, Moledina, Shahin, Pepke-Zaba, Joanna, Polwarth, Gary J, Schotte, Gwen, Tenorio-Castaño, Jair, Thompson, AA Roger, Wharton, John, Wort, Stephen J, Megy, Karyn, Mapeta, Rutendo, Treacy, Carmen M, Martin, Jennifer M, Li, Wei, Swift, Andrew J, Upton, Paul D, Morrell, Nicholas W, Gräf, Stefan, Valverde, Diana, NIHR BioResource For Translational Research–Rare Diseases, National Cohort Study Of Idiopathic And Heritable PAH, PAH Biobank Enrolling Centers’ Investigators, Prapa, Matina [0000-0002-2717-5463], Lago-Docampo, Mauro [0000-0002-8799-6079], Montani, David [0000-0002-9358-6922], Humbert, Marc [0000-0003-0703-2892], Welch, Carrie L [0000-0003-3521-4458], Chung, Wendy K [0000-0003-3438-5685], Berger, Rolf MF [0000-0002-4385-5784], Bogaard, Harm Jan [0000-0001-5371-0346], Danhaive, Olivier [0000-0002-8208-3086], Escribano-Subías, Pilar [0000-0002-6640-4839], Gall, Henning [0000-0001-7016-7373], Jansen, Samara MA [0000-0003-3493-3573], Kerstjens-Frederikse, Wilhelmina [0000-0003-2528-2203], Lapunzina, Pablo [0000-0002-6324-4825], McDermott, John [0000-0002-5220-8837], Moledina, Shahin [0000-0003-0262-2340], Pepke-Zaba, Joanna [0000-0003-3764-3563], Polwarth, Gary J [0000-0003-0344-3088], Tenorio-Castaño, Jair [0000-0002-5308-2316], Thompson, AA Roger [0000-0002-0717-4551], Wharton, John [0000-0001-8110-2575], Megy, Karyn [0000-0002-2826-3879], Li, Wei [0000-0002-1924-3120], Morrell, Nicholas W [0000-0001-5700-9792], Gräf, Stefan [0000-0002-1315-8873], Valverde, Diana [0000-0002-7024-1657], and Apollo - University of Cambridge Repository

Subjects
interstitial lung disease, Lung Diseases, gain-of-function, Phenotype, Genotype, pulmonary arterial hypertension, Gain of Function Mutation, Mutation, Humans, TBX4, T-Box Domain Proteins, Bone Morphogenetic Protein Receptors, Type II, lung developmental disease
Abstract

Rationale: Despite the increased recognition of TBX4 (T-BOX transcription factor 4)-associated pulmonary arterial hypertension (PAH), genotype-phenotype associations are lacking and may provide important insights. Objectives: To compile and functionally characterize all TBX4 variants reported to date and undertake a comprehensive genotype-phenotype analysis. Methods: We assembled a multicenter cohort of 137 patients harboring monoallelic TBX4 variants and assessed the pathogenicity of missense variation (n = 42) using a novel luciferase reporter assay containing T-BOX binding motifs. We sought genotype-phenotype correlations and undertook a comparative analysis with patients with PAH with BMPR2 (Bone Morphogenetic Protein Receptor type 2) causal variants (n = 162) or no identified variants in PAH-associated genes (n = 741) genotyped via the National Institute for Health Research BioResource-Rare Diseases. Measurements and Main Results: Functional assessment of TBX4 missense variants led to the novel finding of gain-of-function effects associated with older age at diagnosis of lung disease compared with loss-of-function effects (P = 0.038). Variants located in the T-BOX and nuclear localization domains were associated with earlier presentation (P = 0.005) and increased incidence of interstitial lung disease (P = 0.003). Event-free survival (death or transplantation) was shorter in the T-BOX group (P = 0.022), although age had a significant effect in the hazard model (P = 0.0461). Carriers of TBX4 variants were diagnosed at a younger age (P < 0.001) and had worse baseline lung function (FEV1, FVC) (P = 0.009) than the BMPR2 and no identified causal variant groups. Conclusions: We demonstrated that TBX4 syndrome is not strictly the result of haploinsufficiency but can also be caused by gain of function. The pleiotropic effects of TBX4 in lung disease may be in part explained by the differential effect of pathogenic mutations located in critical protein domains.

Published
2022
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50. Acute lung injury after balloon pulmonary angioplasty results in a similar haemodynamic response and possible clinical advantage at follow-up

Author

Rodgers, Matthew S, Kirkby, Louise C, Amaral-Almeida, Liliana, Sheares, Karen, Toshner, Mark, Taboada, Dolores, Ng, Choo, Cannon, John E, D'Errico, Luigia, Ruggiero, Alessandro, Screaton, Nicholas, Jenkins, David, Coghlan, John G, Pepke-Zaba, Joanna, Hoole, Stephen P, Rodgers, Matthew S [0000-0001-9485-2869], Hoole, Stephen P [0000-0002-3530-3808], and Apollo - University of Cambridge Repository

Subjects
RESEARCH ARTICLE, balloon pulmonary angioplasty (BPA), chronic thromboembolic pulmonary hypertension (CTEPH), pulmonary vascular resistance (PVR), acute lung injury (ALI), mean pulmonary artery pressure (mPAP), RESEARCH ARTICLES
Abstract

Funder: None, Acute lung injury (ALI) is a common but poorly defined and understood complication of balloon pulmonary angioplasty (BPA) for chronic thromboembolic pulmonary hypertension (CTEPH). Little data are available on the medium term clinical outcomes of BPA complicated by ALI. We analyzed per-procedure data from 282 procedures in 109 patients and per-patient data from 85 patients. Serial right heart catheterization at baseline, after each BPA and at 3-month follow-up measured pulmonary vascular resistance (PVR), mean pulmonary artery pressure (mPAP), and cardiac output (CO). ALI (ALI+) was identified by chest radiography alone (ALIr+) or in association with hypoxia clinically (ALIcr+). Procedural predictors of ALI and patient outcomes at 3-months were compared no ALI (ALI-). ALI+ occurred in 17/282 (6.0%) procedures (ALIcr+: 2.5%, ALIr+: 3.5%). Prevailing haemodynamics (PVR: p < 0.01; mPAP: p < 0.05) at a procedural and patient level, as well as number of BPA sessions (p < 0.01), total number of vessels (p < 0.05), and occlusions (p < 0.05) treated at a patient level predicted ALI+. Those with ALI had greater percentage improvement in ΔCAMPHOR symptoms score (ALI+: -63.5 ± 35.7% (p < 0.05); ALIcr+: -84.4 ± 14.5% (p < 0.01); ALI-: -27.2 ± 74.2%) and ΔNT-proBNP (ALIcr+: -78.4 ± 11.9% (p < 0.01); ALI-: -42.9 ± 36.0%) at follow-up. There was no net significant difference in haemodynamic changes in ALI+ versus ALI- at follow-up. ALI is predicted by haemodynamic severity, number of vessels treated, number of BPA sessions, and treating occlusive disease. ALI in this cohort was associated with a clinical advantage at follow-up.

Published
2023
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