Pub Date : 2020-08-01DOI: 10.21693/1933-088x-19.2.55
{"title":"Pulmonary Hypertension Roundtable: Drug Development and Negative Clinical Trial Results","authors":"","doi":"10.21693/1933-088x-19.2.55","DOIUrl":"https://doi.org/10.21693/1933-088x-19.2.55","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43744536","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-08-01DOI: 10.21693/1933-088x-19.2.62
C. Garnett, N. Stockbridge
Pulmonary arterial hypertension (PAH) is a rare, progressive disease. There are 11 drugs available in the United States to treat adult PAH patients; however, all drugs primarily act through vasodilation and have modest effects on clinical endpoints. None of these drugs can claim survival benefit in their product labels. New drugs are needed that target other mechanisms in the disease to have durable benefits for patients. To demonstrate clinical benefit, new drugs are now tested in large, randomized, placebo-controlled trials evaluating their effect to delay clinical worsening, a composite endpoint of morbidity events and death. Efficient clinical trial designs, such as the use of enrichment strategies, that reduce the number of patients and trial duration would be valuable for this disease. It would also be desirable to have new clinical endpoints that measure improvement in quality of life and allow the use of extrapolation strategies to the pediatric population. Academic, industry, and regulatory partnerships are key to advancing therapies for this disease.
{"title":"Ask the Expert: A Regulatory Perspective on Clinical Trials for Pulmonary Arterial Hypertension","authors":"C. Garnett, N. Stockbridge","doi":"10.21693/1933-088x-19.2.62","DOIUrl":"https://doi.org/10.21693/1933-088x-19.2.62","url":null,"abstract":"Pulmonary arterial hypertension (PAH) is a rare, progressive disease. There are 11 drugs available in the United States to treat adult PAH patients; however, all drugs primarily act through vasodilation and have modest effects on clinical endpoints. None of these drugs can claim survival benefit in their product labels. New drugs are needed that target other mechanisms in the disease to have durable benefits for patients. To demonstrate clinical benefit, new drugs are now tested in large, randomized, placebo-controlled trials evaluating their effect to delay clinical worsening, a composite endpoint of morbidity events and death. Efficient clinical trial designs, such as the use of enrichment strategies, that reduce the number of patients and trial duration would be valuable for this disease. It would also be desirable to have new clinical endpoints that measure improvement in quality of life and allow the use of extrapolation strategies to the pediatric population. Academic, industry, and regulatory partnerships are key to advancing therapies for this disease.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43203118","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-01-01DOI: 10.21693/1933-088x-19.3.72
Christopher H. Chang, J. Robinson
The current understanding of high-altitude pulmonary hypertension (HAPH) is largely attributable to the work of a small cadre of international scientists. The present article discusses the discovery and early investigations into HAPH that now serve as the foundation of our modern understanding of the disease. Further, though HAPH is clearly a distinct entity, we highlight how this early work led to a broader understanding of pulmonary vascular disease—including pulmonary arterial hypertension (PAH)—through the development of translational clinical models of disease, elucidation of hypoxic signaling, and therapeutics applicable to PAH.
{"title":"From the Andes to the Rocky Mountains: A Historical View of High-Altitude Pulmonary Hypertension","authors":"Christopher H. Chang, J. Robinson","doi":"10.21693/1933-088x-19.3.72","DOIUrl":"https://doi.org/10.21693/1933-088x-19.3.72","url":null,"abstract":"The current understanding of high-altitude pulmonary hypertension (HAPH) is largely attributable to the work of a small cadre of international scientists. The present article discusses the discovery and early investigations into HAPH that now serve as the foundation of our modern understanding of the disease. Further, though HAPH is clearly a distinct entity, we highlight how this early work led to a broader understanding of pulmonary vascular disease—including pulmonary arterial hypertension (PAH)—through the development of translational clinical models of disease, elucidation of hypoxic signaling, and therapeutics applicable to PAH.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47772434","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-01-01DOI: 10.21693/1933-088x-19.3.77
W. Cornwell, A. Lovering
Conveniences of modern travel allow for an increasing number of people to sojourn to mountainous, high-altitude locations for work and/or pleasure. Travel to these types of locations places unique stressors on the human body and, more specifically, the cardiovascular and pulmonary systems since ambient oxygen content declines at altitude. The physiologic response to hypoxia is a highly dynamic process that begins immediately and continues to evolve from acute (hours to days) to chronic (days to weeks) time periods. Furthermore, sojourns to hypoxic locations frequently involve exercise, which places additional strain on the heart and lungs. The aim of this review is to emphasize clinically relevant physiologic responses that occur, both acutely and chronically, after travel to high-altitude locations.
{"title":"Impact of Altitude on Cardiopulmonary and Right Ventricular Hemodynamics During Exercise","authors":"W. Cornwell, A. Lovering","doi":"10.21693/1933-088x-19.3.77","DOIUrl":"https://doi.org/10.21693/1933-088x-19.3.77","url":null,"abstract":"Conveniences of modern travel allow for an increasing number of people to sojourn to mountainous, high-altitude locations for work and/or pleasure. Travel to these types of locations places unique stressors on the human body and, more specifically, the cardiovascular and pulmonary systems since ambient oxygen content declines at altitude. The physiologic response to hypoxia is a highly dynamic process that begins immediately and continues to evolve from acute (hours to days) to chronic (days to weeks) time periods. Furthermore, sojourns to hypoxic locations frequently involve exercise, which places additional strain on the heart and lungs. The aim of this review is to emphasize clinically relevant physiologic responses that occur, both acutely and chronically, after travel to high-altitude locations.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45246847","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-01-01DOI: 10.21693/1933-088x-19.1.25
This spring, Guest Editor Richard A. Krasuski, MD, Professor of Medicine at Duke University School of Medicine in Durham, North Carolina, convened a group of experts to discuss the present and future of invasive technologies in the diagnosis and treatment of pulmonary hypertension. The guests included Jamil A. Aboulhosn, MD, Professor of Medicine at the University of California Los Angeles David Geffen School of Medicine in Los Angeles, California; Raymond Benza, MD, Director of the Division of Cardiovascular Medicine and Professor of Medicine at The Ohio State University College of Medicine in Columbus, Ohio; and J. Eduardo Rame, MD, FACC, Louis R. Dinon MD Professor of Cardiovascular Medicine and Physiology and Chief for Advanced Cardiac and Pulmonary Vascular Disease at Thomas Jefferson University Hospital in Philadelphia, Pennsylvania.
{"title":"Pulmonary Hypertension Roundtable: New Invasive Technologies in Pulmonary Hypertension","authors":"","doi":"10.21693/1933-088x-19.1.25","DOIUrl":"https://doi.org/10.21693/1933-088x-19.1.25","url":null,"abstract":"This spring, Guest Editor Richard A. Krasuski, MD, Professor of Medicine at Duke University School of Medicine in Durham, North Carolina, convened a group of experts to discuss the present and future of invasive technologies in the diagnosis and treatment of pulmonary hypertension. The guests included Jamil A. Aboulhosn, MD, Professor of Medicine at the University of California Los Angeles David Geffen School of Medicine in Los Angeles, California; Raymond Benza, MD, Director of the Division of Cardiovascular Medicine and Professor of Medicine at The Ohio State University College of Medicine in Columbus, Ohio; and J. Eduardo Rame, MD, FACC, Louis R. Dinon MD Professor of Cardiovascular Medicine and Physiology and Chief for Advanced Cardiac and Pulmonary Vascular Disease at Thomas Jefferson University Hospital in Philadelphia, Pennsylvania.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47393228","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-01-01DOI: 10.21693/1933-088x-19.3.83
{"title":"A Life at High Altitude: A Conversation With Todd Bull and Peter Hackett","authors":"","doi":"10.21693/1933-088x-19.3.83","DOIUrl":"https://doi.org/10.21693/1933-088x-19.3.83","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45227627","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-12-27DOI: 10.21693/1933-088x-18.4.152
Nicholas Fox, V. Test, Vinod Jona, R. Harley, R. Argula
{"title":"PH Grand Rounds: When a Clot Is Not a Clot: An Unusual Cause of Progressively Worsening Dyspnea in a Previously Healthy Woman","authors":"Nicholas Fox, V. Test, Vinod Jona, R. Harley, R. Argula","doi":"10.21693/1933-088x-18.4.152","DOIUrl":"https://doi.org/10.21693/1933-088x-18.4.152","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48757691","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-12-27DOI: 10.21693/1933-088x-18.4.157
J. Brewer, S. Allen
{"title":"PH Professional Network: Facilitating and Improving Adherence: The Development of a Pulmonary Arterial Hypertension Self-Care Management Agreement","authors":"J. Brewer, S. Allen","doi":"10.21693/1933-088x-18.4.157","DOIUrl":"https://doi.org/10.21693/1933-088x-18.4.157","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46766073","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-12-01DOI: 10.21693/1933-088x-18.4.115
S. Kligerman, M. Horowitz, L. Hahn, A. Hsiao, E. Weihe
PULMONARY HYPERTENSION Elevated pulmonary arterial pressures are the result of a spectrum of diseases that have been classified into 5 categories by the World Symposium on Pulmonary Hypertension. The finding of pulmonary hypertension (PH) is usually the first step in a multidisciplinary workup to diagnose the underlying cause, as different etiologies have different treatment algorithms and outcomes. Diagnostic imaging plays a key role in not only the initial evaluation of a patient with PH, but also to assess disease progression or treatment response. In trying to discover the underlying cause of PH, the diagnostic radiologist often must act as a detective. While some findings, such as varying degrees of enlargement of the pulmonary trunk and remodeling of the right heart, are ubiquitous in patients with PH, it is often the more subtle findings that can help elucidate the cause. These findings may be isolated to the lung parenchyma or may involve the pulmonary or systemic vasculature, heart, or mediastinum. The purpose of this article is to review the various findings of PH on computed tomography (CT) and ventilation/perfusion (V/Q) scans that can help one to differentiate between the various etiologies.
{"title":"Multimodality Imaging of Pulmonary Hypertension","authors":"S. Kligerman, M. Horowitz, L. Hahn, A. Hsiao, E. Weihe","doi":"10.21693/1933-088x-18.4.115","DOIUrl":"https://doi.org/10.21693/1933-088x-18.4.115","url":null,"abstract":"PULMONARY HYPERTENSION Elevated pulmonary arterial pressures are the result of a spectrum of diseases that have been classified into 5 categories by the World Symposium on Pulmonary Hypertension. The finding of pulmonary hypertension (PH) is usually the first step in a multidisciplinary workup to diagnose the underlying cause, as different etiologies have different treatment algorithms and outcomes. Diagnostic imaging plays a key role in not only the initial evaluation of a patient with PH, but also to assess disease progression or treatment response. In trying to discover the underlying cause of PH, the diagnostic radiologist often must act as a detective. While some findings, such as varying degrees of enlargement of the pulmonary trunk and remodeling of the right heart, are ubiquitous in patients with PH, it is often the more subtle findings that can help elucidate the cause. These findings may be isolated to the lung parenchyma or may involve the pulmonary or systemic vasculature, heart, or mediastinum. The purpose of this article is to review the various findings of PH on computed tomography (CT) and ventilation/perfusion (V/Q) scans that can help one to differentiate between the various etiologies.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41612883","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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