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
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
Cardiac & Cardiovascular Systems
R. Mark Grady, Matthew W. Canter, Fei Wan, Anton A. Shmalts, Ryan D. Coleman, Maurice Beghetti, Rolf M. F. Berger, Maria J. del Cerro Marin, Scott E. Fletcher, Russel Hirsch, Tilman H. Umpl, D. Dunbar Ivy, Edward C. Kirkpatrick, Thomas J. Kulik, Marilyne Levy, Shahin Moledina, Delphine Yung, Pirooz Eghtesady, Damien Bonnet
Summary: Pulmonary-to-systemic arterial shunt surgery provides significant clinical improvement for children with severe PH, with high survival rates after discharge, while children at high risk in-hospital are not suitable candidates for the procedure.
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
(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)
Article
Cardiac & Cardiovascular Systems
Yoichi Sugiyama, Hiromi Matsubara, Hiroto Shimokawahara, Aiko Ogawa
Summary: Lowering mean pulmonary arterial pressure (mPAP) has a favorable impact on the prognosis of patients with pulmonary arterial hypertension (PAH), and it has become a therapeutic goal. By following a treatment algorithm, a significant number of patients achieved the target mPAP, and the survival rate of patients who achieved the therapeutic goal was significantly better than those who did not.
JOURNAL OF CARDIOLOGY
(2022)
Review
Cardiac & Cardiovascular Systems
Matthias Gorenflo, Victoria C. Ziesenitz
Summary: Treatment of pediatric pulmonary arterial hypertension has advanced significantly, with new drug therapies being evaluated. Special attention is needed for the pharmacology of PAH drugs in children and more research is required.
CARDIOVASCULAR DIAGNOSIS AND THERAPY
(2021)
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)
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
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
Pharmacology & Pharmacy
Maria Jose Cristo Ropero, Alejandro Cruz-Utrilla, Maria Pilar Escribano-Subias
Summary: Epoprostenol remains the best treatment option for high-risk PAH patients, but there are many unresolved questions regarding drug dosing, its use in combination therapy with other pulmonary vasodilators, and its utility in PAH associated with connective tissue disease, congenital heart disease, or pulmonary veno-occlusive disease. Its safety and efficacy are supported by clinical trials, observational work, and experience, but it is still underused in clinical practice.
EXPERT REVIEW OF CLINICAL PHARMACOLOGY
(2021)
Article
Cardiac & Cardiovascular Systems
Yinghao Lim, Ting-Ting Low, Siew Pang Chan, Weiqin Lin, Ting Wei Teo, Jin-Hao Justin Jang, Ivandito Kuntjoro, Edgar Lik-Wui Tay, James Wei-Luen Yip
Summary: The study analyzed data from 102 patients with newly diagnosed PAH and found that a low PAPi was associated with mortality, with the low PAPi group having older age, lower pulmonary artery systolic pressure, and higher mean right atrial pressure. These findings suggest that PAPi may be a predictive marker for mortality in PAH.
Article
Respiratory System
Abdullah M. Aldalaan, Sarfraz A. Saleemi, Ihab Weheba, Abeer Abdelsayed, Maha M. Aleid, Fatima Alzubi, Hamdeia Zaytoun, Nadeen Alharbi
Summary: The study presented the initial results of the 'SAUDIPH' registry, focusing on assessing patient characteristics, treatment approach, and clinical outcomes in PAH patients. The most common etiology was Group 1 PH, with improvements in functional class and NT-proBNP observed in most patients. The estimated 1-year survival rate was better than previous studies, possibly due to the wider use of combination therapy and the high prevalence of CHD-PAH.
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
Medicine, General & Internal
Valentina Mercurio, Hussein J. Hassan, Mario Naranjo, Alessandra Cuomo, Jeremy A. Mazurek, Paul R. Forfia, Aparna Balasubramanian, Catherine E. Simpson, Rachel L. Damico, Todd M. Kolb, Stephen C. Mathai, Steven Hsu, Monica Mukherjee, Paul M. Hassoun
Summary: This study found that echocardiographic parameters can improve the risk stratification of intermediate-risk PAH patients and discriminate distinct survival phenotypes. By measuring TAPSE and degree of TR, intermediate-risk patients can be further stratified into different risk groups, which can potentially guide subsequent therapy.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Critical Care Medicine
Hussein J. Hassan, Mario Naranjo, Nour Ayoub, Traci Housten, Steven Hsu, Aparna Balasubramanian, Catherine E. Simpson, Rachel L. Damico, Stephen C. Mathai, Todd M. Kolb, Paul M. Hassoun
Summary: This study compared the characteristics and survival rates of patients with systemic sclerosis-associated pulmonary hypertension (SSc-PH) between the last two decades. The results showed that the transplant-free survival rate has significantly improved for patients with group 1 disease over the past decade, possibly due to earlier detection and better therapeutic management. In contrast, patients with group 2 or 3 disease continue to have a poor prognosis.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Editorial Material
Biochemistry & Molecular Biology
Rizwan Hamid, Eric D. Austin
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
(2019)
Editorial Material
Critical Care Medicine
Eric D. Austin, Rizwan Hamid
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2020)
Editorial Material
Respiratory System
Eric D. Austin, C. Gregory Elliott
EUROPEAN RESPIRATORY JOURNAL
(2020)
Letter
Critical Care Medicine
Elena K. Amin, Eric D. Austin, Claire Parker, Elizabeth Colglazier, Hythem Nawaytou, Peter J. Leary, Anna R. Hemnes, David Teitel, Jeffrey R. Fineman
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2020)
Editorial Material
Critical Care Medicine
Olivier Boucherat, Eric D. Austin, Sebastien Bonnet
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2020)
Article
Pediatrics
Megan Griffiths, Jun Yang, Melanie Nies, Dhananjay Vaidya, Stephanie Brandal, Monica Williams, Elizabeth C. Matsui, Torie Grant, Rachel Damico, Dunbar Ivy, Eric D. Austin, William C. Nichols, Michael W. Pauciulo, Katie Lutz, Erika B. Rosenzweig, Russel Hirsch, Delphine Yung, Allen D. Everett
PEDIATRIC RESEARCH
(2020)
Article
Pediatrics
Joseph M. Collaco, Amit Agarwal, Eric D. Austin, Lystra P. Hayden, Khanh Lai, Jonathan Levin, Winston M. Manimtim, Paul E. Moore, Catherine A. Sheils, Michael C. Tracy, Stamatia Alexiou, Christopher D. Baker, A. Ioana Cristea, Julie L. Fierro, Lawrence M. Rhein, Natalie Villafranco, Leif D. Nelin, Sharon A. McGrath-Morrow
Summary: This study investigated a diverse outpatient cohort of former preterm infants with BPD in geographically distinct clinics, finding variations in management strategies and a majority of nonwhite children. Neither management variation nor BPD severity at 36 weeks influenced outpatient acute care utilization, suggesting that post-neonatal ICU factors may independently modify respiratory outcomes in BPD.
PEDIATRIC PULMONOLOGY
(2021)
Article
Respiratory System
Steven H. Abman, Mary P. Mullen, Lynn A. Sleeper, Eric D. Austin, Erika B. Rosenzweig, John P. Kinsella, Dunbar Ivy, Rachel K. Hopper, J. Usha Raj, Jeffrey Fineman, Roberta L. Keller, Angela Bates, Usha S. Krishnan, Catherine M. Avitabile, Alexander Davidson, Marc D. Natter, Kenneth D. Mandl
Summary: The study found a significant proportion of pediatric PH patients with Group 3 disorders, reflecting the growing recognition of PH in diverse developmental lung diseases. Greater precision of clinical phenotyping based on disease-specific characterization may further enhance care and research of pediatric PH.
EUROPEAN RESPIRATORY JOURNAL
(2022)
Article
Pediatrics
Kelsey W. Malloy, Eric D. Austin
Summary: Bronchopulmonary dysplasia (BPD) is the most common chronic lung disease of prematurity, leading to prolonged respiratory support and increased pulmonary morbidity in premature infants. Children with BPD may develop severe complications such as pulmonary hypertension. It is crucial to understand the pathophysiology, screening, diagnosis, and treatment options for pulmonary hypertension in children with BPD.
PEDIATRIC PULMONOLOGY
(2021)
Editorial Material
Critical Care Medicine
Tracy M. Frech, Eric D. Austin
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2022)
Editorial Material
Critical Care Medicine
Rachel T. Sullivan, Eric D. Austin
Article
Respiratory System
Erika B. Rosenzweig, Angela Bates, Mary P. Mullen, Steven H. Abman, Eric D. Austin, Allen Everett, Jeffrey Fineman, Jeffery Feinstein, Rachel K. Hopper, John P. Kinsella, Usha S. Krishnan, Minmin Lu, Kenneth D. Mandl, J. Usha Raj, Nidhy Varghese, Delphine Yung, Stephanie S. Handler, Lynn A. Sleeper
Summary: This study analyzed the hemodynamic findings and safety of catheterization in children with WSPH group 1 and 3 subtypes. The results showed that diagnostic RHC was performed safely in experienced pediatric pulmonary hypertension centers for WSPH group 1 and 3 patients. Additionally, hemodynamic differences were noted between group 1 and 3 subjects.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2022)
Review
Cardiac & Cardiovascular Systems
Nils P. Nickel, Gian M. Galura, Marc J. Zuckerman, M. Nawar Hakim, Haider Alkhateeb, Debabrata Mukherjee, Eric D. Austin, Gustavo D. Heresi
Summary: PAH is a cardiopulmonary disease with high mortality, affecting multiple organ systems including the liver, histology, and systemic metabolism. Right heart failure can lead to congestive hepatopathy, impacting liver biochemistry, histology, and overall glucose and lipid metabolism. This article focuses on summarizing the consequences of congestive hepatopathy in PAH patients, with an emphasis on liver biochemistry, histology, and targeted therapy. Additionally, it discusses specific changes in glucose and lipid metabolism associated with PAH.
PULMONARY CIRCULATION
(2021)
Letter
Biochemistry & Molecular Biology
Ying Cai, Ling Yan, Matthew J. Kielt, Joy D. Cogan, Lora K. Hedges, Bethany Nunley, James West, Eric D. Austin, Rizwan Hamid
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
(2021)
Meeting Abstract
Respiratory System
Catherine Simpson, Rachel L. Damico, Paul M. Hassoun, Lisa J. Martin, Jun Yang, Melanie Nies, R. Dhananjay Vaidya, Stephanie Brandal, Michael Pauciulo, Eric Austin, Dunbar Ivy, William Nichols, Allen D. Everett
EUROPEAN RESPIRATORY JOURNAL
(2019)