Article
Medicine, General & Internal
Marc Humbert, Vallerie McLaughlin, J. Simon R. Gibbs, Mardi Gomberg-Maitland, Marius M. Hoeper, Ioana R. Preston, Rogerio Souza, Aaron Waxman, Pilar Escribano Subias, Jeremy Feldman, Gisela Meyer, David Montani, Karen M. Olsson, Solaiappan Manimaran, Jennifer Barnes, Peter G. Linde, Janethe de Oliveira Pena, David B. Badesch
Summary: In this study, patients with pulmonary arterial hypertension receiving Sotatercept treatment showed a significant reduction in pulmonary vascular resistance at 24 weeks, with both Sotatercept groups performing better than the placebo group.
NEW ENGLAND JOURNAL OF MEDICINE
(2021)
Review
Biochemistry & Molecular Biology
Aangi J. Shah, Taylor Beckmann, Mounica Vorla, Dinesh K. Kalra
Summary: Pulmonary arterial hypertension (PAH) is a chronic and progressive disorder that affects the pulmonary vasculature and leads to pulmonary and cardiac remodeling. Despite improved life expectancy through targeted therapies, PAH still remains a progressive disease with significant morbidity and mortality. Current vasodilator therapies do not target the underlying pathogenesis of the disease, highlighting the need for the development of new drugs and interventional therapies. This review focuses on newer targets, drugs, and interventional therapies that modify various pathways involved in the pathogenesis of PAH.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Pharmacology & Pharmacy
Aijun Liu, Bin Li, Ming Yang, Yongying Shi, Junwu Su
Summary: This study developed GlcA-modified liposomes to enhance the delivery of TPS to pulmonary arterial smooth muscle cells (PASMCs) and improve its effectiveness against PAH. The GlcA-liposomes significantly increased TPS bioavailability and sustained release, while also inhibiting PA remodeling and muscularization, decreasing PA medial thickening, suppressing collagen deposition, and attenuating right ventricle hypertrophy. In vitro experiments showed that GlcA-liposomes enhanced the inhibitory effects of TPS on PASMC proliferation and migration.
EUROPEAN JOURNAL OF PHARMACOLOGY
(2022)
Review
Pharmacology & Pharmacy
Adriana Mares, Debabrata Mukherjee, Richard A. A. Lange, Nils P. P. Nickel
Summary: Pulmonary arterial hypertension (PAH) is a devastating cardiovascular disease that can be treated effectively with PAH-targeted therapies. Patients with congenital heart disease (CHD) are at high risk of developing PAH, and evidence shows that PAH-targeted therapy can be beneficial for them. However, treating the PAH-CHD population is challenging due to the complexity of their cardiac lesions and associated comorbidities.
CURRENT VASCULAR PHARMACOLOGY
(2022)
Review
Critical Care Medicine
David F. Condon, Stuti Agarwal, Ananya Chakraborty, Natasha Auer, Rocio Vazquez, Hirai Patel, Roham T. Zamanian, Vinicio A. de Jesus Perez
Summary: Pulmonary arterial hypertension (PAH) is a rare disease characterized by abnormally elevated pulmonary pressures and right heart failure, resulting in high morbidity and mortality. Current therapies are insufficient in preventing small-vessel loss and obstruction, leading to active interest in identifying drugs that target angiogenesis and mechanisms involved in cell growth and fibrosis regulation. Preclinical studies have identified promising compounds that target seven major mechanisms associated with PAH pathogenesis, including bone morphogenetic protein signaling, tyrosine kinase receptors, estrogen metabolism, extracellular matrix, angiogenesis, epigenetics, and serotonin metabolism. This review discusses the prioritization of these mechanisms through preclinical studies, as well as ongoing clinical trials testing novel interventions. The authors anticipate that the next generation of compounds will build upon the current standard of care and improve clinical outcomes and quality of life for PAH patients.
Article
Critical Care Medicine
Bradley A. Maron, Steven H. Abman, C. Greg Elliott, Robert P. Frantz, Rachel K. Hopper, Evelyn M. Horn, Mark R. Nicolls, Oksana A. Shlobin, Sanjiv J. Shah, Gabor Kovacs, Horst Olschewski, Erika B. Rosenzweig
Summary: The diagnosis and management of pulmonary arterial hypertension have seen advances in early diagnosis, multiple pharmacotherapeutics, and new risk factors becoming focal points. Developmental biology may be useful in predicting incident PAH.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2021)
Article
Medicine, General & Internal
Marius M. Hoeper, David B. Badesch, H. Ardeschir Ghofrani, J. Simon R. Gibbs, Mardi Gomberg-Maitland, Vallerie V. McLaughlin, Ioana R. Preston, Rogerio Souza, Aaron B. Waxman, Ekkehard Gruenig, Grzegorz Kopec, Gisela Meyer, Karen M. Olsson, Stephan Rosenkranz, Yayun Xu, Barry Miller, Marcie Fowler, John Butler, Joerg Koglin, Janethe de Oliveira Pena, Marc Humbert
Summary: This study confirms that in patients with stable background therapy, sotatercept can significantly improve the exercise capacity of patients with pulmonary arterial hypertension compared to placebo.
NEW ENGLAND JOURNAL OF MEDICINE
(2023)
Review
Medicine, Research & Experimental
Carlos Jerjes-Sanchez, Hector Glenn-Valdez, Nayeli Zayas, Guillermo Cueto-Robledo, Liliana Bonola, Baltazar Pech-Alonso, Alicia Ramirez, Fernando Flores-Puente, Humberto Garcia-Aguilar, Guadalupe Espitia-Hernandez, Guillermo Prisciliano Montes, Tomas Pulido
Summary: Pulmonary arterial hypertension is a severe condition that greatly impacts patients' well-being and survival. Combined therapy with drugs like riociguat has shown better results.
ARCHIVES OF MEDICAL RESEARCH
(2022)
Review
Pediatrics
Li Li, Xinyu Zhu, Xiaojie Chen, Jieyun Gao, Chunchun Ding, Min Zhang, Shixun Ma
Summary: Pulmonary arterial hypertension (PAH) is a rare and devastating disease in infants and children. Current treatment approaches are mostly based on adult guidelines and clinical experience, but the advent of targeted drugs has improved the treatment of PAH in children. However, more research is needed to evaluate the combination of drugs, treatment strategies, and clinical endpoints in children PAH.
EUROPEAN JOURNAL OF PEDIATRICS
(2023)
Review
Biochemistry & Molecular Biology
Meng-Chien Willie Hsieh, Wei-Ting Wang, Jwu-Lai Yeh, Chuang-Yu Lin, Yur-Ren Kuo, Su-Shin Lee, Ming-Feng Hou, Yi-Chia Wu
Summary: Pulmonary arterial hypertension (PAH) is a serious progressive disorder that currently has no cure. The existing pharmacological agents for PAH are limited in their ability to reverse disease progression. This review explores potential therapeutic targets and strategies for treating PAH.
Article
Pharmacology & Pharmacy
Wenhai Fu, Wenjun He, Yuexin Li, Yangxiao Chen, Jingyi Liang, Hui Lei, Lin Fu, Yanghang Chen, Ni Ren, Qian Jiang, Yi Shen, Ran Ma, Tao Wang, Xinni Wang, Nuofu Zhang, Dakai Xiao, Chunli Liu
Summary: The study findings indicate that targeted medications show significant advantages in improving symptoms and clinical outcomes in PAH patients, but safety concerns remain, warranting cautious use.
Article
Chemistry, Multidisciplinary
Yoogyeong Oh, Kyungtae Park, Sungwon Jung, Moonhyun Choi, Taihyun Kim, Yoojin Lee, Jae Young Choi, Yang-Hee Kim, Se Yong Jung, Jinkee Hong
Summary: In this study, open porous nitric oxide (NO) inhalers (OPNIs) with highly porous structures are synthesized to deliver nanomolar NO deep into the lungs through a nebulizer. The OPNIs exhibit vasodilatory and anti-inflammatory effects via sustained NO release, suggesting their potential as tools for pulmonary arterial hypertension (PAH) treatment.
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
Critical Care Medicine
Breanne E. McCarthy, Robyn L. McClelland, Dina H. Appleby, Jude S. Moutchia, Jasleen K. Minhas, Jeff Min, Jeremy A. Mazurek, K. Akaya Smith, Jason S. Fritz, Steven C. Pugliese, Ryan J. Urbanowicz, John H. Holmes, Harold I. Palevsky, Steven M. Kawut, Nadine Al-Naamani
Summary: Overweight and obese patients with PAH have lower baseline 6MWD and worse WHO functional class compared to patients with normal weight. Higher BMI did not modify the treatment response for change in 6MWD, but attenuated the treatment response for WHO functional class.
Review
Chemistry, Medicinal
Naoyuki Otani, Takashi Tomoe, Atsuhiko Kawabe, Takushi Sugiyama, Yasuto Horie, Hiroyuki Sugimura, Takanori Yasu, Takaaki Nakamoto
Summary: Pulmonary arterial hypertension (PAH) is a disease that leads to right-sided heart failure and death. Basic research has identified the decrease in levels of endogenous vasodilators and increase in levels of endogenous vasoconstrictors in PAH patients, leading to the development of therapeutic agents. Current treatments target specific pathways to improve prognosis.
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
Critical Care Medicine
David Montani, Pierre Thore, Xavier Mignard, Xavier Jais, Athenais Boucly, Mitja Jevnikar, Andrei Seferian, Etienne-Marie Jutant, Vincent Cottin, Elie Fadel, Gerald Simonneau, Laurent Savale, Olivier Sitbon, Marc Humbert
Summary: Precapillary pulmonary hypertension (PH) is a rare and often overlooked complication of myeloproliferative neoplasms (MPNs). This study describes the characteristics and outcomes of MPN-associated PH. Patients with MPN often present with severe hemodynamic impairment and impaired clinical conditions. Chronic thromboembolic PH and group 5 PH are common diagnoses. Thromboendarterectomy can improve survival rates.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Editorial Material
Cardiac & Cardiovascular Systems
Alexander Moiroux-Sahraoui, Justin Issard, Jean-Baptiste Menager, Delphine Mitilian, Dominique Fabre, Olaf Mercier, Xavier Jais, Elie Fadel
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)
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
Fida Charif, Fatima Dakroub, Imad Bou Akl, Mithum Kularatne, David Montani
Summary: COVID-19 remains a healthcare concern even after the end of the pandemic. Patients with cardiovascular disease (CVD) are at higher risk for severe COVID-19 complications. Studies on the clinical characteristics of COVID-19 in patients with pulmonary arterial hypertension (PAH) have produced conflicting results. In this review, we summarize the literature on the clinical presentation of COVID-19 in PAH patients and discuss common pathological aspects and disease mechanisms between PAH and COVID-19. We also provide an overview of PAH-approved therapies and their potential use in COVID-19 treatment, as well as summarize clinical trials on the safety and efficacy of PAH-approved drugs in COVID-19 patients. Finally, we propose future research studies.
RESPIRATORY MEDICINE AND RESEARCH
(2023)
Review
Respiratory System
David Montani, Fabrice Antigny, Etienne-Marie Jutant, Marie-Camille Chaumais, Helene Le Ribeuz, Julien Grynblat, Charles Khouri, Marc Humbert
Summary: The ATP-sensitive potassium channels and their regulatory subunits, SUR1 and SUR2, have a significant role in the pathophysiology of pulmonary hypertension. Loss-of-function variants in ABCC8 gene, which encodes for SUR1, are associated with heritable pulmonary arterial hypertension, while activation of SUR1 and SUR2 leads to relaxation of pulmonary arteries and reduces cell proliferation and migration. Diazoxide, an activator of SUR1, has shown potential as a therapeutic option for pulmonary hypertension. However, there are contradictory reports of diazoxide-induced pulmonary hypertension in infants.
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)
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)