Article
Medical Laboratory Technology
Ying-Ju Lai, Winston W-Y Kao, Yung-Hsin Yeh, Wei-Jan Chen, Pao-Hsien Chu
Summary: Lumican deficiency promotes pulmonary arterial remodeling. Administration of lumikine reverses the PAH pathogenesis caused by hypoxia-induced experimental PAH. Lumican is an antiproliferative target that functions to suppress pAKT activation during pathogenesis.
TRANSLATIONAL RESEARCH
(2021)
Article
Cardiac & Cardiovascular Systems
Kae-Woei Liang, Sheng-Kai Chang, Yu-Wei Chen, Wei-Wen Lin, Wan-Jane Tsai, Kuo-Yang Wang
Summary: This study identified the presence of genetic variants associated with pulmonary arterial hypertension (PAH) in a Taiwanese cohort, particularly the BMPR2 gene variant. These findings suggest the importance of genetic factors in the development of PAH.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
Article
Biochemistry & Molecular Biology
Ting He, Junzhi Zhang, Ting Qiao, Zhongjun Zhang, Hui Han, Chao Yang, Yong Chen, Yiwen Ruan, Liukun Meng
Summary: This study found that the compensatory decrease of PRDC in patients with pulmonary arterial hypertension (PAH) may slow down the natural course of the disease and have therapeutic potential.
INTERNATIONAL JOURNAL OF BIOLOGICAL SCIENCES
(2022)
Article
Multidisciplinary Sciences
Gusty R. T. Ryanto, Koji Ikeda, Kazuya Miyagawa, Ly Tu, Christophe Guignabert, Marc Humbert, Tomoyuki Fujiyama, Masashi Yanagisawa, Ken-ichi Hirata, Noriaki Emoto
Summary: The dysregulation of the activin A-bone morphogenetic protein receptor type 2 link in endothelium plays a critical role in the progression of pulmonary arterial hypertension. Inhibin-beta -A/activin-A in endothelial cells could be a potential pharmacotherapeutic target for the treatment of pulmonary arterial hypertension.
NATURE COMMUNICATIONS
(2021)
Article
Critical Care Medicine
Rowena J. Jones, Eckart M. D. D. De Bie, Emily Groves, Kasia Zalewska, Emilia M. Swietlik, Carmen M. Treacy, Jennifer M. Martin, Gary Polwarth, Wei Li, Jingxu Guo, Helen E. Baxendale, Stephen Coleman, Natalia Savinykh, J. Gerry Coghlan, Paul A. Corris, Luke S. Howard, Martin K. Johnson, Colin Church, David G. Kiely, Allan Lawrie, James L. Lordan, Robert V. Mackenzie Ross, Joanna Pepke Zaba, Martin R. Wilkins, S. John Wort, Edoardo Fiorillo, Valeria Orru, Francesco Cucca, Christopher J. Rhodes, Stefan Graf, Nicholas W. Morrell, Eoin F. McKinney, Chris Wallace, Mark Toshner
Summary: This study investigated autoimmunity in idiopathic pulmonary arterial hypertension (IPAH) using a large cross-sectional cohort. The results showed that IPAH is associated with aberrant immune regulation and the presence of autoantibodies, which is linked to clinical outcomes.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2022)
Article
Critical Care Medicine
Athenais Boucly, Laurent Savale, Xavier Jais, Fabrice Bauer, Emmanuel Bergot, Laurent Bertoletti, Antoine Beurnier, Arnaud Bourdin, Helene Bouvaist, Sophie Bulifon, Celine Chabanne, Ari Chaouat, Vincent Cottin, Claire Dauphin, Bruno Degano, Pascal De Groote, Nicolas Favrolt, Yuanchao Feng, Delphine Horeau-Langlard, Mitja Jevnikar, Etienne-Marie Jutant, Zhiying Liang, Pascal Magro, Pierre Mauran, Pamela Moceri, Jean-Francois Mornex, Sylvain Palat, Florence Parent, Francois Picard, Jeremie Pichon, Patrice Poubeau, Gregoire Prevot, Sebastien Renard, Martine Reynaud-Gaubert, Marianne Riou, Pascal Roblot, Olivier Sanchez, Andrei Seferian, Cecile Tromeur, Jason Weatherald, Gerald Simonneau, David Montani, Marc Humbert, Olivier Sitbon
Summary: The study findings suggest that initial triple combination therapy containing parenteral prostacyclin is associated with a higher survival rate in patients with PAH, particularly in younger high-risk patients.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2021)
Article
Respiratory System
Peter Heukels, Odilia B. J. Corneth, Denise van Uden, Jennifer A. C. van Hulst, Leon M. van den Toorn, Annemien E. van den Bosch, Marlies S. Wijsenbeek, Karin A. Boomars, Mirjam Kool, Rudi W. Hendriks
Summary: This study demonstrates that pulmonary injury combined with enhanced B-cell activation can induce PH symptoms in mice, while immune homeostasis is compromised in IPAH patients, as shown by increased BCR signaling and cTfh17 polarization.
Article
Peripheral Vascular Disease
Fangcheng Luo, Liangmiao Wu, Guoqing Xie, FangFang Gao, Zhixiang Zhang, Guangying Chen, Zheng Liu, Ling Zha, Gaoxiao Zhang, Yewei Sun, Zaijun Zhang, Yuqiang Wang
Summary: PAH is a rare pulmonary vascular disease with limited therapeutic options. MN-08, a dual-functional memantine nitrate derivative, shows promising efficacy in experimental animal models of PAH, potentially serving as a therapeutic agent for the disease.
Article
Critical Care Medicine
Rui-Sheng Wang, Shi Huang, Stephen W. Waldo, Edward Hess, Madhura Gokhale, Shelsey W. Johnson, Katarina Zeder, Gaurav Choudhary, Jane A. Leopold, William M. Oldham, Gabor Kovacs, Matthew S. Freiberg, Ryan J. Tedford, Bradley A. Maron, Evan L. Brittain
Summary: This study aims to identify clinical markers and therapeutic targets that inform survival in patients with pulmonary hypertension (PH). The results suggest that elevated pulmonary arterial compliance (PAC) greater than 3.0 ml/mmHg is associated with improved survival, especially in patients with precapillary PH.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Cardiac & Cardiovascular Systems
G. Jayasekera, A. Macdonald, C. Mccomb, V. Orchard, D. Welsh, C. Church, M. Johnson, M. Brewis, C. Berry, A. Radjenovic, A. Peacock
Summary: This study found that patients with idiopathic pulmonary arterial hypertension (IPAH) have impaired left ventricular function and intraventricular dyssynchrony, which are associated with markers of disease severity. These findings may provide incremental value to the prognosis assessment of IPAH.
INTERNATIONAL JOURNAL OF CARDIOLOGY
(2022)
Article
Critical Care Medicine
Marius M. Hoeper, Krit Dwivedi, Christine Pausch, Robert A. Lewis, Karen M. Olsson, Doerte Huscher, David Pittrow, Ekkehard Gruenig, Gerd Staehler, Carmine Dario Vizza, Henning Gall, Oliver Distler, Christian Opitz, John Simon R. Gibbs, Marion Delcroix, Da-Hee Park, Hossein Ardeschir Ghofrani, Ralf Ewert, Harald Kaemmerer, Hans-Joachim Kabitz, Dirk Skowasch, Juergen Behr, Katrin Milger, Tobias J. Lange, Heinrike Wilkens, Hans-Juergen Seyfarth, Matthias Held, Daniel Dumitrescu, Iraklis Tsangaris, Anton Vonk-Noordegraaf, Silvia Ulrich, Hans Klose, Martin Claussen, Stephan Eisenmann, Kai-Helge Schmidt, Andrew J. Swift, Alfred A. Roger Thompson, Charlie A. Elliot, Stephan Rosenkranz, Robin Condliffe, David G. Kiely, Michael Halank
Summary: This study provides a detailed characterization of patients meeting diagnostic criteria for idiopathic pulmonary arterial hypertension (IPAH) and identifies a distinct lung phenotype characterized by low diffusion capacity for carbon monoxide (DLCO) and smoking history. These patients resemble those with pulmonary hypertension due to lung disease rather than classical IPAH. These findings have important implications for understanding the pathogenesis, diagnosis, and treatment of the disease and warrant further exploration.
LANCET RESPIRATORY MEDICINE
(2022)
Article
Biochemistry & Molecular Biology
Michal Tomaszewski, Paulina Malkowska, Olga Sierawska, Rafal Hrynkiewicz, Ewa Mroczek, Szymon Darocha, Anna Hymos, Piotr Blaszczak, Ewelina Grywalska, Paulina Niedzwiedzka-Rystwej
Summary: Pulmonary arterial hypertension (PAH) is a common disease with unclear molecular mechanisms. This study investigated the role of lymphocyte subpopulations in various types of PAH and identified potential biomarkers for differential diagnosis. The study found that CD4+CTLA-4+ T cells were significantly increased in iPAH patients and correlated with the severity of heart failure. Additionally, the percentage of CD19+CTLA-4+ B cells was positively correlated with the concentration of NT-proBNP. CD4+CTLA-4+ and CD8+CTLA-4+ T cells may serve as negative prognostic factors in iPAH.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Immunology
Chen-Yu Jiang, Li-Wei Wu, Yi-Wei Liu, Bei Feng, Lin-Cai Ye, Xu Huang, Yang-Yang He, Yi Shen, Yi-Fan Zhu, Xing-Liang Zhou, Dai-Ji Jiang, Hai-Kun Qi, Hao Zhang, Yi Yan
Summary: This study identified critical chemokines in the progression of pulmonary arterial hypertension (PAH) through transcriptomic analysis. ACKR4 was found to be downregulated in PAH lung tissues and correlated with immune cell infiltration and function, suggesting its potential role as a protective immune checkpoint for PAH.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Su-Gang Gong, Wen-Hui Wu, Chao Li, Qin-Hua Zhao, Rong Jiang, Ci-Jun Luo, Hong-Ling Qiu, Jin-Ming Liu, Lan Wang, Rui Zhang
Summary: The 2015 European PH guidelines and its simplified risk stratification assessment effectively discriminate different risk groups and accurately estimate mortality in Chinese patients with IPAH. Changes in risk proportions at re-evaluation suggest that goal-oriented treatment decisions may need to be reconsidered.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2021)
Article
Immunology
Christoph Tabeling, Carla Gonzalez R. Calera, Jasmin Lienau, Jakob Hoeppner, Thomas Tschernig, Olivia Kershaw, Birgitt Gutbier, Jan Naujoks, Julia Herbert, Bastian Opitz, Achim D. Gruber, Berthold Hocher, Norbert Suttorp, Harald Heidecke, Gerd-R. Burmester, Gabriela Riemekasten, Elise Siegert, Wolfgang M. Kuebler, Martin Witzenrath
Summary: This study provides evidence for the anti-inflammatory role of ETB and its protective effects on Th2-induced pathologies of the cardiovascular system. Autoantibodies against ETB may modulate ETB-mediated immune homeostasis.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Respiratory System
Marc Humbert, Vallerie McLaughlin, J. Simon R. Gibbs, Mardi Gomberg-Maitland, Marius M. Hoeper, Ioana R. Preston, Rogerio Souza, Aaron B. Waxman, Hossein-Ardeschir Ghofrani, Pilar Escribano Subias, Jeremy Feldman, Gisela Meyer, David Montani, Karen M. Olsson, Solaiappan Manimaran, Janethe de Oliveira Pena, David B. Badesch
Summary: In participants with pulmonary arterial hypertension, 24 weeks of sotatercept resulted in a significant reduction in pulmonary vascular resistance. This study reports the longer-term safety and efficacy of sotatercept in the PULSAR open-label extension, showing that sotatercept has good safety and efficacy in patients with pulmonary arterial hypertension.
EUROPEAN RESPIRATORY JOURNAL
(2023)
Review
Critical Care Medicine
Bartolome R. Celli, Leonardo M. Fabbri, Shawn D. Aaron, Alvar Agusti, Robert D. Brook, Gerard J. Criner, Frits M. E. Franssen, Marc Humbert, John R. Hurst, Maria Montes de Oca, Leonardo Pantoni, Alberto Papi, Roberto Rodriguez-Roisin, Sanjay Sethi, Daiana Stolz, Antoni Torres, Claus F. Vogelmeier, Jadwiga A. Wedzicha
Summary: Patients with chronic obstructive pulmonary disease (COPD) may experience acute episodes of worsening dyspnea, accompanied by increased cough, sputum, and/or sputum purulence. These exacerbations impact health status, accelerate lung function decline, and increase the risk of hospitalization. It is important to recognize and address other morbidities that can mimic or aggravate COPD exacerbations to improve outcomes and reduce readmissions.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Rheumatology
Sebastien Sanges, Lisa Rice, Ly Tu, Eleanor Valenzi, Jean-Luc Cracowski, David Montani, Julio C. Mantero, Camille Ternynck, Guillemette Marot, Andreea M. Bujor, Eric Hachulla, David Launay, Marc Humbert, Christophe Guignabert, Robert Lafyatis
Summary: This study examined the serum proteome of patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) and identified two proteins that were significantly correlated with pulmonary vascular resistance (PVR), potentially providing biomarkers for earlier diagnosis and treatment.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Cardiac & Cardiovascular Systems
Emmanuelle Fournier, Maelle Selegny, Myriam Amsallem, Francois Haddad, Sarah Cohen, Estibaliz Valdeolmillos, Jerome Le Pavec, Marc Humbert, Marc -Antoine Isorni, Arshid Azarine, Olivier Sitbon, Xavier Jais, Laurent Savale, David Montani, Elie Fadel, Joy Zoghbi, Emre Belli, Sebastien Hascoet
Summary: This study aimed to explore the accuracy of different echocardiographic parameters in assessing right ventricular function in patients with pretricuspid shunt and pulmonary arterial hypertension (PAH). The results showed that right ventricular global longitudinal strain, right atrial area, and right ventricular end-systolic remodeling index were strongly correlated with right ventricular ejection fraction. These parameters are important markers for evaluating right ventricular dysfunction in patients with pretricuspid shunt and PAH.
REVISTA ESPANOLA DE CARDIOLOGIA
(2023)
Review
Respiratory System
J. Le Pavec, L. Savale, G. Prevot, D. Montani, O. Sitbon, E. Fadel, M. Humbert, O. Mercier
REVUE DES MALADIES RESPIRATOIRES
(2023)
Article
Rheumatology
Benjamin Chaigne, Kevin Chevalier, Athenais Boucly, Christian Agard, Antoine Baudet, Arnaud Bourdin, Celine Chabanne, Vincent Cottin, Pierre Fesler, Francois Goupil, Patrick Jego, David Launay, Herve Levesque, Arnaud Maurac, Shirine Mohamed, Cecile Tromeur, Laurence Rottat, Olivier Sitbon, Marc Humbert, Luc Mouthon
Summary: Objective of this study was to describe PAH in well-characterized MCTD patients. Results showed that pericarditis, polyarthritis, thrombocytopenia, ILD and anti-Sm antibodies were independent predictive factors of PAH in MCTD. Survival rates of MCTD-PAH patients did not differ from SLE-PAH and SSc-PAH patients. Tobacco exposure was an independent predictor of mortality in MCTD-PAH.
Article
Cardiac & Cardiovascular Systems
Christophe Guignabert, Laurent Savale, Athenais Boucly, Raphael Thuillet, Ly Tu, Mina Ottaviani, Christopher J. Rhodes, Pascal De Groote, Gregoire Prevot, Emmanuel Bergot, Arnaud Bourdin, Luke S. Howard, Elie Fadel, Antoine Beurnier, Anne Roche, Mitja Jevnikar, Xavier Jais, David Montani, Martin R. Wilkins, Olivier Sitbon, Marc Humbert
Summary: Activin A and FSTL3 serum levels are associated with transplant-free survival in PAH. They can serve as prognostic biomarkers for PAH, and their diagnostic values have been confirmed in an independent validation cohort. The activin signaling system in PAH involves phosphorylated Smad2/3 nuclear accumulation and differential immunoreactivities of various proteins in vascular endothelial and smooth muscle layers.
Letter
Respiratory System
Marcos V. F. Garcia, Rogerio Souza, Pedro Caruso
Review
Pharmacology & Pharmacy
Mithum Kularatne, Athenais Boucly, Laurent Savale, Sabina Solinas, Celine Cheron, Anne Roche, Mitja Jevnikar, Xavier Jais, David Montani, Marc Humbert, Olivier Sitbon
Summary: Despite advances in treatment, patients with CTD-associated PAH still have poorer survival compared to those with idiopathic PAH. This review discusses the factors contributing to disparate outcomes and explores potential improvements for increasing quality of life and survival in CTD-associated PAH patients.
EXPERT OPINION ON PHARMACOTHERAPY
(2023)
Review
Respiratory System
Sabina Solinas, Athenais Boucly, Antoine Beurnier, Mithum Kularatne, Julien Grynblat, Melanie Eyries, Peter Dorfmueller, Olivier Sitbon, Marc Humbert, David Montani
Summary: Pulmonary veno-occlusive disease (PVOD), characterized by substantial involvement of small pulmonary veins and capillaries, is an uncommon etiology of pulmonary arterial hypertension (PAH). Recent advancements include the discovery of biallelic mutations in the EIF2AK4 gene causing heritable PVOD. Differentiating PVOD from PAH is vital due to the worse prognosis and poor response of PVOD to PAH-approved drugs.
EXPERT REVIEW OF RESPIRATORY MEDICINE
(2023)
Meeting Abstract
Respiratory System
Chin Kook Rhee, Jonathan Corren, Mario Castro, Jorge F. Maspero, Marc Humbert, David M. G. Halpin, Arman Altincatal, Nami Pandit-Abid, Xavier Soler, Shahid Siddiqui, Juby A. Jacob-Nara, Yamo Deniz, Paul J. Rowe
Meeting Abstract
Cardiac & Cardiovascular Systems
E. Valdeolmillos, J. Le Pavec, M. Audie, L. Savale, X. Jais, S. Feuillet, O. Sitbon, O. Mercier, J. Petit, M. Humbert, E. Fadel, E. Belli, S. Hascoet
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)
Article
Respiratory System
Bruna Piloto, Caio Julio Cesar dos Santos Fernandes, Carlos Jardim, Marcela Castro, Jose Leonidas Alves-, Rogerio Souza
Summary: Idiopathic pulmonary arterial hypertension (PAH) patients who initially respond positively to acute vasodilator challenge and show clinical response to calcium channel blockers (CCBs) for at least one year can lose their response to CCBs after long-term treatment. This highlights the importance of constant multidimensional reevaluation to assess the need for targeted PAH therapies and correctly classify these patients.
JORNAL BRASILEIRO DE PNEUMOLOGIA
(2023)
Letter
Critical Care Medicine
David Montani, Jason Weatherald, Marc Humbert, Olivier Sitbon
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Correction
Cardiac & Cardiovascular Systems
[Anonymous]
EUROPEAN HEART JOURNAL
(2023)