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.
Article
Multidisciplinary Sciences
Hanqiang Deng, Yanying Xu, Xiaoyue Hu, Zhen W. Zhuang, Yuzhou Chang, Yewei Wang, Aglaia Ntokou, Martin A. Schwartz, Bing Su, Michael Simons
Summary: Arterial inward remodeling plays a critical role in the progression of common diseases such as hypertension and atherosclerosis. This study revealed the unexpected involvement of endothelial MEKK3 in regulating TGF-beta R1-Smad2/3 signaling and arterial remodeling in artery diseases.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Critical Care Medicine
Farbod N. Rahaghi, Pietro Nardelli, Eileen Harder, Inderjit Singh, Gonzalo Vegas Sanchez-Ferrero, James C. Ross, Ruben San Jose Estepar, Samuel Y. Ash, Andetta R. Hunsaker, Bradley A. Maron, Jane A. Leopold, Aaron B. Waxman, Raul San Jose Estepar, George R. Washko
Summary: Pruning and vascular tortuosity were observed in both arterial and venous pulmonary vessels of patients with pulmonary arterial hypertension. Lower small vessel volume and higher arterial volume and tortuosity were identified in pulmonary arteries.
Review
Biochemistry & Molecular Biology
Bernardo Rodriguez-Iturbe, Richard J. Johnson, Laura Gabriela Sanchez-Lozada, Hector Pons
Summary: Heat shock protein 70 (HSP70) plays a role in both inflammation and immune reactivity, depending on the context. It is associated with hypertension, which is an important risk factor for cardiovascular disease mortality. The HSP70 response can either drive inflammatory immune reactivity or protect against hypertension through immunomodulatory responses.
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)
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)
Review
Cardiac & Cardiovascular Systems
Iona Cuthbertson, Nicholas W. Morrell, Paola Caruso
Summary: Pulmonary arterial hypertension (PAH) is the most severe type of pulmonary hypertension, characterized by progressive remodeling of peripheral pulmonary arteries. Endothelial cell dysfunction is a key trigger, leading to vascular remodeling, increased pressure, and right ventricular hypertrophy. Abnormal cellular molecular signatures and loss-of-function mutations in the BMPR2 gene contribute to PAH pathophysiology. Metabolic abnormalities, including glycolytic reprogramming and mitochondrial dysfunction, are observed in various tissues and may affect treatment response. This review critically discusses the mechanisms linking metabolic abnormalities with dysfunctional BMPR2 signaling and their relevance to PAH pathogenesis and potential therapies.
CIRCULATION RESEARCH
(2023)
Review
Biochemistry & Molecular Biology
Julia Krzeminska, Magdalena Wronka, Ewelina Mlynarska, Beata Franczyk, Jacek Rysz
Summary: Arterial hypertension is a major cause of cardiovascular diseases, and oxidative stress and inflammation play important roles in its development, influenced by factors such as diet, supplementation, and pharmacotherapy.
Review
Medicine, General & Internal
Max Wagener, Eamon Dolan, Samer Arnous, Joseph Galvin, Andrew W. Murphy, Ivan Casserly, Joseph Eustace, Stephen O'Connor, Charles Mccreery, James Shand, Catherine Wall, Saijad Matiullah, Faisal Sharif
Summary: This article discusses the evidence base for the efficacy and safety of catheter-based renal denervation in lowering blood pressure, and highlights its potential in addressing hypertension and its adverse consequences.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Medicine, General & Internal
Tomasz Stacel, Magdalena Latos, Maciej Urlik, Miroslaw Necki, Remigiusz Antonczyk, Tomasz Hrapkowicz, Marcin Kurzyna, Marek Ochman
Summary: Although pharmacological treatments have advanced significantly, interventional and surgical options remain viable for treating pulmonary arterial hypertension (PAH), especially in the case of idiopathic PAH. Veno-arterial extracorporeal membrane oxygenation (V-A ECMO) serves as the ultimate bridging therapy for severe PAH patients, with lung transplantation (Ltx) as the final surgical option for refractory cases. Creating artificial defects to obtain Eisenmenger physiology can improve survival, but risks associated with shunting and closure of these defects must be carefully considered.
JOURNAL OF CLINICAL MEDICINE
(2021)
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
Shia Vang, Phillip Cochran, Julio Sebastian Domingo, Stefanie Krick, Jarrod Wesley Barnes
Summary: Pulmonary arterial hypertension (PAH) is a complex pulmonary vascular disease with low survival rate without treatment. Idiopathic PAH (IPAH) is associated with metabolic dysregulation, including altered nitric oxide (NO) availability and dysregulated glucose metabolism. Metabolic changes may regulate multiple processes in PAH. Recent studies have highlighted similarities between PAH and cancer in terms of metabolic abnormalities.
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)
Article
Biochemistry & Molecular Biology
Shuxin Liang, Manivannan Yegambaram, Ting Wang, Jian Wang, Stephen M. Black, Haiyang Tang
Summary: This review focuses on the alterations in mitochondrial metabolism that occur in deranged pulmonary vessels and the right ventricle in pulmonary arterial hypertension (PAH), including abnormalities in glycolysis and glucose oxidation, fatty acid oxidation, glutaminolysis, redox homeostasis, as well as iron and calcium metabolism. Further understanding of these mitochondrial metabolic mechanisms could provide viable therapeutic approaches for PAH patients.
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)