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
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)
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
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
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)
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)
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.
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.
Editorial Material
Medicine, General & Internal
Filip Baszkowski, Weronika Pelczar-Plachta, Nikola Pempera, Sylwia Slawek-Szmyt, Marta Kaluzna-Oleksy, Maciej Lesiak, Waldemar Bobkowski
Summary: This paper presents a case of a 9-year-old girl with paediatric idiopathic pulmonary arterial hypertension (IPAH). The management of paediatric IPAH is challenging due to limited comparative data. The patient underwent various treatment methods and showed significant response to treprostinil. The diagnosis of this patient was based mainly on differential diagnosis due to non-specific symptoms.
Article
Cardiac & Cardiovascular Systems
Euy-Myoung Jeong, Mandy Pereira, Eui-Young So, Keith Q. Wu, Michael Del Tatto, Sicheng Wen, Mark S. Dooner, Patrycja M. Dubielecka, Anthony M. Reginato, Corey E. Ventetuolo, Peter J. Quesenberry, James R. Klinger, Olin D. Liang
Summary: This study aimed to determine whether blocking RUNX1 can reverse the development of pulmonary arterial hypertension (PAH) in rats induced by SuHx, and to understand the underlying mechanisms involved.
CARDIOVASCULAR RESEARCH
(2022)
Article
Mathematical & Computational Biology
Fan Zhang, Hongtao Liu
Summary: This study identified ferroptosis-associated genes related to PAH using gene expression data, and explored their regulatory mechanisms and potential therapeutic directions.
MATHEMATICAL BIOSCIENCES AND ENGINEERING
(2021)
Article
Critical Care Medicine
Nicholas A. Kolaitis
Summary: Lung transplantation is a unique and effective treatment option for patients with end-stage pulmonary arterial hypertension. However, these patients face various challenges and inequities in the organ allocation process, resulting in less favorable long-term outcomes.
Review
Biochemistry & Molecular Biology
Carrie L. Welch, Wendy K. Chung
Summary: Pulmonary arterial hypertension (PAH) is a rare and progressive vascular disease with no curative treatments available. Genetic variants are found in a significant portion of PAH patients, and understanding their diagnoses can inform clinical management, risk stratification, and the development of new therapies.
Review
Biochemistry & Molecular Biology
Aangi J. Shah, Mounica Vorla, Dinesh K. Kalra
Summary: Pulmonary arterial hypertension is a chronic disease characterized by abnormalities in the pulmonary vascular system and right ventricular dysfunction. Current treatments have limited effectiveness, highlighting the importance of understanding the molecular pathogenesis of this condition.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Medicine, General & Internal
Nicole F. Ruopp, Barbara A. Cockrill
Summary: Pulmonary arterial hypertension, a subtype of pulmonary hypertension characterized by pulmonary arterial remodeling, can progress to right heart failure and death if left untreated. Approved therapies targeting multiple biological pathways have significantly improved survival rates.
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION
(2022)
Letter
Critical Care Medicine
Ashley Mulchrone, Alessandro Bellofiore, Johannes M. Douwes, Neal Duong, Arij G. Beshish, Gregory P. Barton, Christopher J. Francois, Marlowe W. Eldridge, Kara N. Goss, Naomi C. Chesler
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2020)
Review
Cardiac & Cardiovascular Systems
Anne-Marie C. Koop, Guido P. L. Bossers, Mark-Jan Ploegstra, Quint A. J. Hagdorn, Rolf M. F. Berger, Herman H. W. Sillje, Beatrijs Bartelds
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2019)
Article
Cardiac & Cardiovascular Systems
Benjamin S. Frank, Michal Schafer, Johannes M. Douwes, D. Dunbar Ivy, Steven H. Abman, Jesse A. Davidson, Sandra Burzlaff, Max B. Mitchell, Gareth J. Morgan, Lorna P. Browne, Alex J. Barker, Uyen Truong, Johannes C. von Alvensleben
AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY
(2020)
Article
Pediatrics
Fatema Said, Meindina G. Haarman, Marcus T. R. Roofthooft, Hans L. Hillege, Mark-Jan Ploegstra, Rolf M. F. Berger
JOURNAL OF PEDIATRICS
(2020)
Article
Cardiac & Cardiovascular Systems
Djoeke Wolff, Joost P. van Melle, Tineke P. Willems, Beatrijs Bartelds, Mark-Jan Ploegstra, Hans Hillege, Tjark Ebels, Rolf M. F. Berger
CARDIOLOGY IN THE YOUNG
(2020)
Article
Respiratory System
Meindina G. Haarman, Marilyne Levy, Marcus T. R. Roofthooft, Johannes M. Douwes, Theresia R. Vissia-Kazemier, Isabelle Szezepanski, Rolf M. F. Berger, Damien Bonnet
Summary: This retrospective observational study evaluated the efficacy of upfront triple combination therapy (uTCT) in paediatric PAH, showing that children with idiopathic and heritable PAH had high transplant-free survival rates up to 3 years, despite a significant proportion receiving a Potts shunt during follow-up. The findings suggest that uTCT may be beneficial for children with severe PAH, but further research is needed to determine the role of a Potts shunt in conjunction with uTCT in paediatric PAH.
EUROPEAN RESPIRATORY JOURNAL
(2021)
Review
Cardiac & Cardiovascular Systems
Mark-Jan Ploegstra, Rolf M. F. Berger
Summary: Children with pulmonary arterial hypertension (PAH) require reliable prognostic biomarkers to guide their treatment decisions, and there are many types of potential prognostic biomarkers available for the management of PAH. A multi-marker approach may be the most effective in guiding treatment decisions and improving outcomes.
CARDIOVASCULAR DIAGNOSIS AND THERAPY
(2021)
Review
Cardiac & Cardiovascular Systems
Chantal Lokhorst, Sjoukje van der Werf, Rolf M. F. Berger, Johannes M. M. Douwes
Summary: This systematic review examines the variables and prognostic value of risk stratification models in adult and pediatric pulmonary arterial hypertension. The study finds that the most commonly used variables in current risk stratification models are WHO functional class, 6-minute walk distance, and BNP/NT-proBNP. The c-statistics of risk stratification models range from 0.56 to 0.83 in adults and 0.69 to 0.78 in children.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
Article
Pediatrics
Meindina G. G. Haarman, Iris Coenraad, Quint A. J. Hagdorn, Hans L. L. Hillege, Tineke P. P. Willems, Rolf M. F. Berger, Johannes M. M. Douwes
Summary: Cardiac magnetic resonance (CMR)-derived measures of right ventricular (RV) morphology and function can predict the outcome of children with pulmonary arterial hypertension (PAH), which is crucial for treatment strategies.
Article
Cardiac & Cardiovascular Systems
Mark-Jan Ploegstra, David Dunbar Ivy, Maurice Beghetti, Damien Bonnet, Dursun Alehan, Laszlo Ablonczy, Sandra Mattos, David Bowers, Tilman Humpl, Rolf M. F. Berger
Summary: The study describes the long-term outcomes and predictors for newly diagnosed pediatric patients with pulmonary arterial hypertension (PAH). The results showed that among the 242 patients who underwent long-term follow-up, 17.4% died, 3.7% underwent lung transplantation, 1.2% underwent atrial septostomy, and 3.7% underwent Potts shunt palliation. Younger age, worse World Health Organization functional class, and higher pulmonary vascular resistance index were identified as independent predictors of long-term adverse outcome.
EUROPEAN HEART JOURNAL-QUALITY OF CARE AND CLINICAL OUTCOMES
(2023)
Article
Cardiac & Cardiovascular Systems
Emmanouil Mavrogiannis, Quint A. J. Hagdorn, Venetia Bazioti, Johannes M. Douwes, Diederik E. van der Feen, Silke U. Oberdorf-Maass, Marit Westerterp, Rolf M. F. Berger
Summary: Pulmonary arterial hypertension (PAH) is a fatal disease characterized by increased pulmonary arterial pressure, inflammation, and neointimal remodeling of pulmonary arterioles. Serum levels of interleukin (IL)-1 beta and IL-18 are elevated in PAH patients and may enhance proinflammatory neointimal remodeling. Pirfenidone (PFD), an antifibrotic and anti-inflammatory drug, has been suggested to inhibit NLRP3 inflammasome activation, delaying the progression of PAH.
PULMONARY CIRCULATION
(2022)
Article
Respiratory System
Johannes M. Douwes, Willemijn M. H. Zijlstra, Erika B. Rosenzweig, Mark-Jan Ploegstra, Usha S. Krishnan, Meindina G. Haarman, Marcus T. R. Roofthooft, Douwe Postmus, Hans L. Hillege, D. Dunbar Ivy, Rolf M. F. Berger
Summary: This study retrospectively analyzed data from 275 children with PAH and found that early initiation and higher doses of IV/SC prostanoid therapy, as well as combination with other targeted drugs, were associated with better outcomes. Transition from IV/SC prostanoid therapy to oral or inhaled therapies was found to be safe in selected patients who met specific hemodynamic criteria.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2022)
Article
Respiratory System
Baukje M. Zaaijer, Nienke Duppen, Brigitte W. M. Willemse, Martijn V. Verhagen, Marcus T. R. Roofthooft, Wim Timens, Rolf M. F. Berger, Johannes M. Douwes
Summary: This case illustrates the diversity of diseases causing PAH and underscores the importance of questioning the original diagnosis when there is an unexpected treatment response. The presence of PAH and PAVM's in the patient does not necessarily indicate a confirmed link to HHT.
RESPIRATORY MEDICINE CASE REPORTS
(2021)
Article
Cardiac & Cardiovascular Systems
Melanie J. Dufva, Dunbar Ivy, Kristen Campbell, Aimee Lam, Adam Rauff, Karel T. N. Breeman, Johannes M. Douwes, Rolf M. F. Berger, Vitaly Oleg Kheyfets, Kendall Hunter
Summary: The ventricular-vascular coupling ratio (E-es/E-a) derived from pressure alone is a strong independent predictor of adverse outcome in pediatric pulmonary arterial hypertension (PAH), serving as a potentially powerful prognostic tool.