Pub Date : 2019-12-01DOI: 10.21693/1933-088x-18.4.110
Natasha A. Vedage, A. Vaidya
Pulmonary hypertension (PH) is a complex, multi-organ system disease that is broadly defined by an elevation in pulmonary artery pressures. There are key biologic differences in the pathophysiology among the different PH subgroups that warrant a targeted approach to clinical care of these patients. The complex geometry of and wide array of clinical tools to assess the right heart in PH add another layer of complexity in patient care. Echocardiography can be used as a powerful tool to discriminate among the subgroups of PH, a critical first step in evaluating a new case of PH. In this article, we will explore novel approaches using echocardiography that go beyond simply estimating pulmonary pressures and provide a readily accessible means of adding diagnostic and prognostic accuracy in the clinical setting.
{"title":"What Echocardiography Can Reliably Tell Us About Our Pulmonary Hypertension Patients","authors":"Natasha A. Vedage, A. Vaidya","doi":"10.21693/1933-088x-18.4.110","DOIUrl":"https://doi.org/10.21693/1933-088x-18.4.110","url":null,"abstract":"Pulmonary hypertension (PH) is a complex, multi-organ system disease that is broadly defined by an elevation in pulmonary artery pressures. There are key biologic differences in the pathophysiology among the different PH subgroups that warrant a targeted approach to clinical care of these patients. The complex geometry of and wide array of clinical tools to assess the right heart in PH add another layer of complexity in patient care. Echocardiography can be used as a powerful tool to discriminate among the subgroups of PH, a critical first step in evaluating a new case of PH. In this article, we will explore novel approaches using echocardiography that go beyond simply estimating pulmonary pressures and provide a readily accessible means of adding diagnostic and prognostic accuracy in the clinical setting.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44137638","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-18.4.134
This fall, Guest Editors Jeffrey D. Edelman, MD, Associate Professor of Medicine at the University of Washington in Seattle, and Harrison W. Farber, MD, Professor of Medicine at Tufts University School of Medicine in Boston, Massachusetts, convened a panel of experts to discuss the role of imaging in pulmonary hypertension. Guests included Benjamin H. Freed, MD, Assistant Professor of Medicine at Northwestern University Feinberg School of Medicine in Chicago, Illinois; Paul Hassoun, MD, Director of the Pulmonary Hypertension Program and Professor of Medicine at Johns Hopkins University Department of Medicine in Baltimore, Maryland; Peter Leary, MD, PhD, Associate Professor of Medicine and Director of the Pulmonary Vascular Disease Program at the University of Washington in Seattle; Sudhakar N.J. Pipavath, MD, Professor of Cardiothoracic Imaging and Adjunct Professor of Pulmonary, Critical Care, Sleep Medicine and Medicine at the University of Washington in Seattle; and Anjali Vaidya, MD, FACC, FASE, FACP, Associate Professor of Medicine and Co-Director, Pulmonary Hypertension, Right Heart Failure and CTEPH Program at Temple University in Philadelphia, Pennsylvania.
今年秋天，客座编辑Jeffrey D. Edelman医学博士(西雅图华盛顿大学医学副教授)和Harrison W. Farber医学博士(马萨诸塞州波士顿塔夫茨大学医学院医学教授)召集了一个专家小组，讨论成像在肺动脉高压中的作用。与会嘉宾包括伊利诺伊州芝加哥市西北大学范伯格医学院医学助理教授Benjamin H. Freed医学博士;Paul Hassoun，医学博士，肺动脉高压项目主任，马里兰州巴尔的摩市约翰霍普金斯大学医学系医学教授;Peter Leary，医学博士，西雅图华盛顿大学医学副教授和肺血管疾病项目主任;Sudhakar N.J. Pipavath，医学博士，西雅图华盛顿大学心胸影像学教授和肺病、重症监护、睡眠医学和医学副教授;Anjali Vaidya，医学博士，FACC, FASE, FACP，宾夕法尼亚州费城坦普尔大学肺动脉高压，右心衰和CTEPH项目的医学副教授和联合主任。
{"title":"Pulmonary Hypertension Roundtable: The Present and Future of Imaging in Pulmonary Hypertension","authors":"","doi":"10.21693/1933-088x-18.4.134","DOIUrl":"https://doi.org/10.21693/1933-088x-18.4.134","url":null,"abstract":"This fall, Guest Editors Jeffrey D. Edelman, MD, Associate Professor of Medicine at the University of Washington in Seattle, and Harrison W. Farber, MD, Professor of Medicine at Tufts University School of Medicine in Boston, Massachusetts, convened a panel of experts to discuss the role of imaging in pulmonary hypertension. Guests included Benjamin H. Freed, MD, Assistant Professor of Medicine at Northwestern University Feinberg School of Medicine in Chicago, Illinois; Paul Hassoun, MD, Director of the Pulmonary Hypertension Program and Professor of Medicine at Johns Hopkins University Department of Medicine in Baltimore, Maryland; Peter Leary, MD, PhD, Associate Professor of Medicine and Director of the Pulmonary Vascular Disease Program at the University of Washington in Seattle; Sudhakar N.J. Pipavath, MD, Professor of Cardiothoracic Imaging and Adjunct Professor of Pulmonary, Critical Care, Sleep Medicine and Medicine at the University of Washington in Seattle; and Anjali Vaidya, MD, FACC, FASE, FACP, Associate Professor of Medicine and Co-Director, Pulmonary Hypertension, Right Heart Failure and CTEPH Program at Temple University in Philadelphia, Pennsylvania.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46862098","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-18.4.141
H. Carmona, Wei Wu, S. Pipavath
INTRODUCTION Pulmonary hypertension (PH) is a disease characterized by elevated mean pulmonary arterial pressure and/or elevated pulmonary vascular resistance, as measured by right heart catheterization. Individuals often present with dyspnea and decreased functional exertion. Imaging features of PH may be present prior to clinical diagnosis. Certain radiographic signs can be useful in identifying the etiology of the PH. We present a variety of imaging features that are aimed at making these etiologies apparent to clinicians, with a focus on those etiologies that do not otherwise usually have distinguishing clinical manifestations.
{"title":"Ask the Expert: Radiographic Signs and Patterns of Pulmonary Hypertension: A Pictorial Essay","authors":"H. Carmona, Wei Wu, S. Pipavath","doi":"10.21693/1933-088x-18.4.141","DOIUrl":"https://doi.org/10.21693/1933-088x-18.4.141","url":null,"abstract":"INTRODUCTION Pulmonary hypertension (PH) is a disease characterized by elevated mean pulmonary arterial pressure and/or elevated pulmonary vascular resistance, as measured by right heart catheterization. Individuals often present with dyspnea and decreased functional exertion. Imaging features of PH may be present prior to clinical diagnosis. Certain radiographic signs can be useful in identifying the etiology of the PH. We present a variety of imaging features that are aimed at making these etiologies apparent to clinicians, with a focus on those etiologies that do not otherwise usually have distinguishing clinical manifestations.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47207214","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 : 2019-11-27DOI: 10.21693/1933-088x-18.3.92
S. Abman, C. Galambos
In comparison with adult disease, pediatric pulmonary hypertension (PH) and related pulmonary vascular disease (PVD) remain relatively understudied and poorly understood. Despite many advances over the past decades, PH continues to cause significant morbidity and mortality in diverse neonatal, pulmonary, cardiac, hematologic, and other systemic disorders of childhood. Despite some similarities, many aspects of PH in children are distinct from adult PH. Although new drug therapies are available for off-label use in pediatric PH, the long-term outcomes of children with severe PH often remain poor. Most clinical studies have emphasized the results of clinical trials in adult patients, yet PH in pediatrics can be devastating and often contributes to poor outcomes in diverse clinical settings in newborns, infants, and children. Of several major challenges addressed in the recent 6th World Symposium on Pulmonary Hypertension (WSPH), one goal was to explore major issues regarding the pathobiology, diagnostic assessment, management, and outcomes of diverse childhood diseases associated with pediatric PH. There are marked differences in the epidemiology of pediatric and adult PH, as well as very striking differences in function, structure, genetics, and responsiveness to therapies between adults and children with PH. Unfortunately, studies that address the safety and efficacy of PH therapies in children are rare, as most pharmaceutical studies have focused on the adult population and only in patients with a fairly limited range of associated conditions. Except for the use of inhaled nitric oxide therapy for neonates with persistent PH of the newborn (PPHN) as based on multicenter randomized trials, nearly all of the current therapies for children remain almost exclusively based on results from adult clinical trials and small case series of the use of PH-targeted therapies. Thus, pediatric PH has been understudied, and little is understood regarding the natural history, mechanisms of disease, and treatment of childhood PH, especially in the setting of neonatal and genetic developmental lung diseases.
{"title":"Pediatric Pulmonary Hypertension on the World Stage: Do We Need Separate Neonatal Guidelines?","authors":"S. Abman, C. Galambos","doi":"10.21693/1933-088x-18.3.92","DOIUrl":"https://doi.org/10.21693/1933-088x-18.3.92","url":null,"abstract":"In comparison with adult disease, pediatric pulmonary hypertension (PH) and related pulmonary vascular disease (PVD) remain relatively understudied and poorly understood. Despite many advances over the past decades, PH continues to cause significant morbidity and mortality in diverse neonatal, pulmonary, cardiac, hematologic, and other systemic disorders of childhood. Despite some similarities, many aspects of PH in children are distinct from adult PH. Although new drug therapies are available for off-label use in pediatric PH, the long-term outcomes of children with severe PH often remain poor. Most clinical studies have emphasized the results of clinical trials in adult patients, yet PH in pediatrics can be devastating and often contributes to poor outcomes in diverse clinical settings in newborns, infants, and children. Of several major challenges addressed in the recent 6th World Symposium on Pulmonary Hypertension (WSPH), one goal was to explore major issues regarding the pathobiology, diagnostic assessment, management, and outcomes of diverse childhood diseases associated with pediatric PH. There are marked differences in the epidemiology of pediatric and adult PH, as well as very striking differences in function, structure, genetics, and responsiveness to therapies between adults and children with PH. Unfortunately, studies that address the safety and efficacy of PH therapies in children are rare, as most pharmaceutical studies have focused on the adult population and only in patients with a fairly limited range of associated conditions. Except for the use of inhaled nitric oxide therapy for neonates with persistent PH of the newborn (PPHN) as based on multicenter randomized trials, nearly all of the current therapies for children remain almost exclusively based on results from adult clinical trials and small case series of the use of PH-targeted therapies. Thus, pediatric PH has been understudied, and little is understood regarding the natural history, mechanisms of disease, and treatment of childhood PH, especially in the setting of neonatal and genetic developmental lung diseases.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42961269","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 : 2019-11-27DOI: 10.21693/1933-088x-18.3.103
S. McDevitt, C. Walter
INTRODUCTION Pulmonary hypertension (PH) is a chronic, progressive heterogeneous disease that affects many individuals worldwide. Despite extensive research and therapeutic advances in the treatment of PH, the morbidity and mortality rates remain high, and this bears a significant economic burden on the health care system. PH-related morbidity is associated with disease progression and often requires hospitalization, which may be considered an important measure of clinical worsening. Current US third-party reimbursement systems can penalize hospitals for hospital readmission. A coordinated multidisciplinary approach to hospital discharge is required for patients with PH.
{"title":"PH Professional Network: Bridging the Gap: A Multidisciplinary Approach to Transitions of Care","authors":"S. McDevitt, C. Walter","doi":"10.21693/1933-088x-18.3.103","DOIUrl":"https://doi.org/10.21693/1933-088x-18.3.103","url":null,"abstract":"INTRODUCTION Pulmonary hypertension (PH) is a chronic, progressive heterogeneous disease that affects many individuals worldwide. Despite extensive research and therapeutic advances in the treatment of PH, the morbidity and mortality rates remain high, and this bears a significant economic burden on the health care system. PH-related morbidity is associated with disease progression and often requires hospitalization, which may be considered an important measure of clinical worsening. Current US third-party reimbursement systems can penalize hospitals for hospital readmission. A coordinated multidisciplinary approach to hospital discharge is required for patients with PH.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44545529","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 : 2019-11-27DOI: 10.21693/1933-088x-18.3.87
T. Thenappan
Pulmonary hypertension (PH) due to left heart disease (LHD) is the most common cause of PH in clinical practice. The definition and classification of PH-LHD has evolved in the last 5 years from the 5th World Symposium on PH (WSPH) in 2013 to the most recent 6th WSPH in 2018. Differentiation of PH-LHD, especially PH due to heart failure with preserved ejection from pulmonary arterial hypertension and chronic thromboembolic PH, can be very challenging. Finally, there is unclarity on the role of pulmonary vasodilators in the treatment of PH-LHD. The 6th WSPH consensus proceedings addresses all these topics in a detailed manner. In this article, we review the changes proposed by the 6th WSPH consensus proceedings in the definition, classification, diagnostic evaluation, and treatment of PH-LHD.
{"title":"Pulmonary Hypertension Due to Left Heart Disease—Combine or Not Combined? DPG In or Out? A Practical Approach to the Patient With Suspected Left Heart Disease","authors":"T. Thenappan","doi":"10.21693/1933-088x-18.3.87","DOIUrl":"https://doi.org/10.21693/1933-088x-18.3.87","url":null,"abstract":"Pulmonary hypertension (PH) due to left heart disease (LHD) is the most common cause of PH in clinical practice. The definition and classification of PH-LHD has evolved in the last 5 years from the 5th World Symposium on PH (WSPH) in 2013 to the most recent 6th WSPH in 2018. Differentiation of PH-LHD, especially PH due to heart failure with preserved ejection from pulmonary arterial hypertension and chronic thromboembolic PH, can be very challenging. Finally, there is unclarity on the role of pulmonary vasodilators in the treatment of PH-LHD. The 6th WSPH consensus proceedings addresses all these topics in a detailed manner. In this article, we review the changes proposed by the 6th WSPH consensus proceedings in the definition, classification, diagnostic evaluation, and treatment of PH-LHD.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48779389","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 : 2019-11-01DOI: 10.21693/1933-088x-18.3.97
{"title":"Pulmonary Hypertension Roundtable: Behind the Scenes at the World Symposium on PH 2018","authors":"","doi":"10.21693/1933-088x-18.3.97","DOIUrl":"https://doi.org/10.21693/1933-088x-18.3.97","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46416255","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 : 2019-11-01DOI: 10.21693/1933-088x-18.3.84
I. Preston
At the 6th World Symposium on Pulmonary Hypertension, the task force on clinical risk stratification and medical therapy in pulmonary arterial hypertension (PAH) reviewed the latest developments published in the field of therapeutics since the previous meeting and presented their consensus opinions to an audience of 1376 participant attendees between February 27 and March 1, 2018, in Nice, France. After participants’ input was incorporated, the final recommendations were published in the European Respiratory Journal. In the past several years, treatment for PAH was based on several parameters to determine the severity of the disease and risk of progression and poor outcome. These parameters included New York Heart Association Functional Class (NYHA FC), exercise capacity represented by the 6-minute walk distance (6MWD), and echocardiographic and hemodynamic measurements. Until recently, the guidelines for initiation and escalation of therapy relied mostly upon NYHA FC. However, data from 3 independent registries demonstrate the importance of a methodical risk assessment and treatment strategy in PAH patients. All registries prove that, in order to obtain a good outcome (assessed as event-free survival at 1 year), patients need to achieve a low-risk status.
{"title":"Risk Stratification—What's My Risk? A Practitioner's Tool","authors":"I. Preston","doi":"10.21693/1933-088x-18.3.84","DOIUrl":"https://doi.org/10.21693/1933-088x-18.3.84","url":null,"abstract":"At the 6th World Symposium on Pulmonary Hypertension, the task force on clinical risk stratification and medical therapy in pulmonary arterial hypertension (PAH) reviewed the latest developments published in the field of therapeutics since the previous meeting and presented their consensus opinions to an audience of 1376 participant attendees between February 27 and March 1, 2018, in Nice, France. After participants’ input was incorporated, the final recommendations were published in the European Respiratory Journal. In the past several years, treatment for PAH was based on several parameters to determine the severity of the disease and risk of progression and poor outcome. These parameters included New York Heart Association Functional Class (NYHA FC), exercise capacity represented by the 6-minute walk distance (6MWD), and echocardiographic and hemodynamic measurements. Until recently, the guidelines for initiation and escalation of therapy relied mostly upon NYHA FC. However, data from 3 independent registries demonstrate the importance of a methodical risk assessment and treatment strategy in PAH patients. All registries prove that, in order to obtain a good outcome (assessed as event-free survival at 1 year), patients need to achieve a low-risk status.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45902064","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 : 2019-11-01DOI: 10.21693/1933-088x-18.3.80
{"title":"Hemodynamic Definitions and Updated Classification—Impact on Clinical Practice: A Conversation With Erika Berman Rosenzweig and Nicholas Hill","authors":"","doi":"10.21693/1933-088x-18.3.80","DOIUrl":"https://doi.org/10.21693/1933-088x-18.3.80","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46538937","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 : 2019-07-29DOI: 10.21693/1933-088X-18.2.42
D. Dumitrescu, R. Oudiz
Pulmonary arterial hypertension (PAH) is a chronic disease that is associated with a significant and progressive limitation of exercise tolerance. The pathophysiological mechanisms of exercise intolerance during exercise are complex, multifactorial, and in fact not limited to the pulmonary circulation and the right ventricle. Disturbance of autonomic nervous function leads to an enhanced chemosensitivity, as well as respiratory and peripheral muscle weakness, and systemic endothelial dysfunction, which together play important roles in PAH pathophysiology and symptomatology. This article is focused on the different pathophysiological mechanisms of exercise intolerance in PAH, their interactions, and their relevance for clinical practice.
{"title":"Exercise Pathophysiology in Pulmonary Arterial Hypertension—The Physiologic Explanation for Why Pulmonary Arterial Hypertension Does What It Does","authors":"D. Dumitrescu, R. Oudiz","doi":"10.21693/1933-088X-18.2.42","DOIUrl":"https://doi.org/10.21693/1933-088X-18.2.42","url":null,"abstract":"Pulmonary arterial hypertension (PAH) is a chronic disease that is associated with a significant and progressive limitation of exercise tolerance. The pathophysiological mechanisms of exercise intolerance during exercise are complex, multifactorial, and in fact not limited to the pulmonary circulation and the right ventricle. Disturbance of autonomic nervous function leads to an enhanced chemosensitivity, as well as respiratory and peripheral muscle weakness, and systemic endothelial dysfunction, which together play important roles in PAH pathophysiology and symptomatology. This article is focused on the different pathophysiological mechanisms of exercise intolerance in PAH, their interactions, and their relevance for clinical practice.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42760214","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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