Pub Date : 2024-07-01DOI: 10.21693/1933-088x-23.1.4
Aimee M. Layton, Catherine M. Avitabile
{"title":"Guest Editors’ Memo","authors":"Aimee M. Layton, Catherine M. Avitabile","doi":"10.21693/1933-088x-23.1.4","DOIUrl":"https://doi.org/10.21693/1933-088x-23.1.4","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"7 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141696796","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 : 2024-07-01DOI: 10.21693/1933-088x-23.1.21
S. Malenfant, F. Potus, S. Bonnet, S. Provencher
Reduced exercise tolerance stands as the foremost symptom, profoundly impacting the lives of those grappling with pulmonary arterial hypertension (PAH). This decline stems from both pulmonary and cardiac irregularities. Nonetheless, there is a burgeoning recognition that dysfunction within peripheral skeletal muscles (SKMs) significantly contributes to compromised exercise capacity. Consequently, the morphological and functional impairments of SKMs, coupled with microvascular loss, proinflammatory states, and oxidative disorders, play substantial roles in limiting exercise capacity in PAH. Regrettably, these facets have only undergone partial scrutiny. Thus, this review aims to spotlight the current body of literature concerning SKM dysfunctions in PAH and pinpoint knowledge gaps warranting further exploration to deepen our comprehension of SKM dysfunction and exercise intolerance in PAH.
{"title":"Skeletal Muscle Structural and Functional Impairments as Important Peripheral Exercise Intolerance Determinants in Pulmonary Arterial Hypertension","authors":"S. Malenfant, F. Potus, S. Bonnet, S. Provencher","doi":"10.21693/1933-088x-23.1.21","DOIUrl":"https://doi.org/10.21693/1933-088x-23.1.21","url":null,"abstract":"Reduced exercise tolerance stands as the foremost symptom, profoundly impacting the lives of those grappling with pulmonary arterial hypertension (PAH). This decline stems from both pulmonary and cardiac irregularities. Nonetheless, there is a burgeoning recognition that dysfunction within peripheral skeletal muscles (SKMs) significantly contributes to compromised exercise capacity. Consequently, the morphological and functional impairments of SKMs, coupled with microvascular loss, proinflammatory states, and oxidative disorders, play substantial roles in limiting exercise capacity in PAH. Regrettably, these facets have only undergone partial scrutiny. Thus, this review aims to spotlight the current body of literature concerning SKM dysfunctions in PAH and pinpoint knowledge gaps warranting further exploration to deepen our comprehension of SKM dysfunction and exercise intolerance in PAH.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"226 14","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141692765","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 : 2024-07-01DOI: 10.21693/1933-088x-23.1.5
Navneet Singh, Christopher J. Mullin
Exercise intolerance is a common feature of many cardiopulmonary diseases including pulmonary hypertension (PH) and sleep disordered breathing (SDB), which includes obstructive sleep apnea and obesity hypoventilation syndrome. Physiologic abnormalities in both PH and SDB can drive exercise intolerance, and biological mechanisms overlap among the conditions including systemic inflammation, oxidative stress, metabolic dysfunction, and endothelial dysfunction. Despite this understanding, evidence establishing clear causal relationships among PH, SDB, and exercise intolerance is lacking. Data show that treatment of SDB may improve exercise capacity, and exercise training likely improves SDB, although these relationships specifically in PH remain understudied. In this manuscript, we summarize existing data of mechanisms and clinical observations in PH, SDB and exercise and identify gaps and opportunities for future investigation.
{"title":"Sleep Disordered Breathing and Exercise in Pulmonary Hypertension","authors":"Navneet Singh, Christopher J. Mullin","doi":"10.21693/1933-088x-23.1.5","DOIUrl":"https://doi.org/10.21693/1933-088x-23.1.5","url":null,"abstract":"Exercise intolerance is a common feature of many cardiopulmonary diseases including pulmonary hypertension (PH) and sleep disordered breathing (SDB), which includes obstructive sleep apnea and obesity hypoventilation syndrome. Physiologic abnormalities in both PH and SDB can drive exercise intolerance, and biological mechanisms overlap among the conditions including systemic inflammation, oxidative stress, metabolic dysfunction, and endothelial dysfunction. Despite this understanding, evidence establishing clear causal relationships among PH, SDB, and exercise intolerance is lacking. Data show that treatment of SDB may improve exercise capacity, and exercise training likely improves SDB, although these relationships specifically in PH remain understudied. In this manuscript, we summarize existing data of mechanisms and clinical observations in PH, SDB and exercise and identify gaps and opportunities for future investigation.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"5 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141703093","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 : 2024-07-01DOI: 10.21693/1933-088x-23.1.27
Catherine M. Avitabile, Nicola Benjamin, Erika S. Berman Rosenzweig, Karen Chia, Prof. Dr. med. Ekkehard Grünig, Aimee M. Layton
{"title":"PH Roundtable: Recommending Exercise in Pulmonary Hypertension: Adult and Pediatric Perspectives","authors":"Catherine M. Avitabile, Nicola Benjamin, Erika S. Berman Rosenzweig, Karen Chia, Prof. Dr. med. Ekkehard Grünig, Aimee M. Layton","doi":"10.21693/1933-088x-23.1.27","DOIUrl":"https://doi.org/10.21693/1933-088x-23.1.27","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"186 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141692537","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 : 2024-07-01DOI: 10.21693/1933-088x-23.1.3
Richard A. Krasuski
{"title":"Editor’s Memo","authors":"Richard A. Krasuski","doi":"10.21693/1933-088x-23.1.3","DOIUrl":"https://doi.org/10.21693/1933-088x-23.1.3","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"71 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141691374","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 : 2023-11-01DOI: 10.21693/1933-088x-22.3.134
William F. Patten, Usha S. Krishnan
Pulmonary hypertension (PH) from left-sided heart disease (group-II PH) is an increasingly recognized cause of PH in pediatrics. Group-II PH can result from obstruction at any level of the left heart, and can progress over time. Management can be particularly difficult, as targeted PH therapy in the setting of a fixed obstruction carries a risk of pulmonary vascular congestion and pulmonary edema. Based on existing evidence, the use of pulmonary vasodilators in group II PH is not recommended, and management centers around early identification and correction of the underlying left-sided lesion. In this review, we highlight the pathophysiology of group-II PH, the diagnostic evaluation of left heart pathology, and a general approach to both medical and surgical management, with particular attention to relevant left-sided lesions. Group-II PH is a multifaceted and progressive disease process that poses a difficult challenge to clinicians, and requires a thoughtful and individualized approach to management.
{"title":"Pediatric Pulmonary Hypertension in Left-Sided Heart Disease","authors":"William F. Patten, Usha S. Krishnan","doi":"10.21693/1933-088x-22.3.134","DOIUrl":"https://doi.org/10.21693/1933-088x-22.3.134","url":null,"abstract":"Pulmonary hypertension (PH) from left-sided heart disease (group-II PH) is an increasingly recognized cause of PH in pediatrics. Group-II PH can result from obstruction at any level of the left heart, and can progress over time. Management can be particularly difficult, as targeted PH therapy in the setting of a fixed obstruction carries a risk of pulmonary vascular congestion and pulmonary edema. Based on existing evidence, the use of pulmonary vasodilators in group II PH is not recommended, and management centers around early identification and correction of the underlying left-sided lesion. In this review, we highlight the pathophysiology of group-II PH, the diagnostic evaluation of left heart pathology, and a general approach to both medical and surgical management, with particular attention to relevant left-sided lesions. Group-II PH is a multifaceted and progressive disease process that poses a difficult challenge to clinicians, and requires a thoughtful and individualized approach to management.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"38 11","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135615867","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 : 2023-11-01DOI: 10.21693/1933-088x-22.3.130
Yogesh N. V. Reddy
Heart failure with preserved ejection fraction (HFpEF) is now the most common cause of pulmonary hypertension (PH), and the diagnosis of HFpEF should be considered in any patient with a preserved left ventricular systolic function being evaluated for PH. Accurately diagnosing HFpEF as compared with pulmonary arterial hypertension has critical treatment implications, given the vastly different treatment options available, and can be accurately guided using exercise right heart catheterization. In this review, the diagnostic approach and treatment implications of PH in patients at risk for HFpEF will be discussed.
{"title":"Heart Failure With Preserved Ejection Fraction and the Diagnosis of Pulmonary Hypertension","authors":"Yogesh N. V. Reddy","doi":"10.21693/1933-088x-22.3.130","DOIUrl":"https://doi.org/10.21693/1933-088x-22.3.130","url":null,"abstract":"Heart failure with preserved ejection fraction (HFpEF) is now the most common cause of pulmonary hypertension (PH), and the diagnosis of HFpEF should be considered in any patient with a preserved left ventricular systolic function being evaluated for PH. Accurately diagnosing HFpEF as compared with pulmonary arterial hypertension has critical treatment implications, given the vastly different treatment options available, and can be accurately guided using exercise right heart catheterization. In this review, the diagnostic approach and treatment implications of PH in patients at risk for HFpEF will be discussed.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"25 6","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135614570","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 : 2023-11-01DOI: 10.21693/1933-088x-22.3.140
Traci Stewart
Self-care abilities in patients with heart failure (HF) are directly related to quality of life and outcomes such as hospitalizations and mortality. Patient education is essential in helping patients gain knowledge and skills to become successful in self-care. As the trajectory of the patient’s course changes, the HF team members identify barriers, help the patient adapt, and work toward desired goals. Communication and shared decisions about prognosis, symptom management, and treatment options require the HF team to connect with patients and have difficult conversations that can be facilitated with palliative care consultations.
{"title":"Nonpharmacological Management of Heart Failure","authors":"Traci Stewart","doi":"10.21693/1933-088x-22.3.140","DOIUrl":"https://doi.org/10.21693/1933-088x-22.3.140","url":null,"abstract":"Self-care abilities in patients with heart failure (HF) are directly related to quality of life and outcomes such as hospitalizations and mortality. Patient education is essential in helping patients gain knowledge and skills to become successful in self-care. As the trajectory of the patient’s course changes, the HF team members identify barriers, help the patient adapt, and work toward desired goals. Communication and shared decisions about prognosis, symptom management, and treatment options require the HF team to connect with patients and have difficult conversations that can be facilitated with palliative care consultations.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"38 14","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135615865","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 : 2023-11-01DOI: 10.21693/1933-088x-22.3.122
Jonathan Kusner, Richard A. Krasuski
Pulmonary hypertension (PH) is characterized by increased right ventricular (RV) afterload, which is accommodated early by dramatic increases in RV contractility to maintain right ventriculoarterial coupling. Related to its tissue biology, characteristics of RV contractility differ from those of the left ventricle (LV). As the RV undergoes adaptation in PH, echocardiographic signs emerge which can help identify PH and can be reassessed to noninvasively prognosticate outcomes in PH. Many of these indices can be calculated from standard echocardiographic views without significant modification to scanning procedures. This review will discuss contemporary diagnosis of PH, highlighting the role of echocardiography in this process. We will describe the differences between the LV and RV, including adaptations of the RV in PH, and how these factors impact echocardiographic assessment. We will conclude with a discussion of specific echocardiographic parameters and describe their role in diagnosis and reassessment. Routine assessment of the right heart improves noninvasive risk stratification in PH, may reduce delays in diagnosis, and ultimately may impact the significant and potentially modifiable disease burden in this patient population.
{"title":"Echocardiographic Evaluation of the Right Heart in Pulmonary Hypertension","authors":"Jonathan Kusner, Richard A. Krasuski","doi":"10.21693/1933-088x-22.3.122","DOIUrl":"https://doi.org/10.21693/1933-088x-22.3.122","url":null,"abstract":"Pulmonary hypertension (PH) is characterized by increased right ventricular (RV) afterload, which is accommodated early by dramatic increases in RV contractility to maintain right ventriculoarterial coupling. Related to its tissue biology, characteristics of RV contractility differ from those of the left ventricle (LV). As the RV undergoes adaptation in PH, echocardiographic signs emerge which can help identify PH and can be reassessed to noninvasively prognosticate outcomes in PH. Many of these indices can be calculated from standard echocardiographic views without significant modification to scanning procedures. This review will discuss contemporary diagnosis of PH, highlighting the role of echocardiography in this process. We will describe the differences between the LV and RV, including adaptations of the RV in PH, and how these factors impact echocardiographic assessment. We will conclude with a discussion of specific echocardiographic parameters and describe their role in diagnosis and reassessment. Routine assessment of the right heart improves noninvasive risk stratification in PH, may reduce delays in diagnosis, and ultimately may impact the significant and potentially modifiable disease burden in this patient population.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"26 5","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135614566","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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