Advances in pulmonary hypertension最新文献

{"title":"Genetic Counseling and Testing for Pulmonary Arterial Hypertension in the United States","authors":"Sumathi I. Rachamadugu, M. Emmerson, B. Girerd, D. H. Best","doi":"10.21693/1933-088x-20.5.157","DOIUrl":"https://doi.org/10.21693/1933-088x-20.5.157","url":null,"abstract":"is identified, coordinate segregation patient Consider DNA of uninformative genetic test result. DNA be pretest some circumstances.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41649165","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}

{"title":"PH Professional Network: Genetic Counseling and Pulmonary Arterial Hypertension","authors":"Athena Angelopoulos, R. Farrell","doi":"10.21693/1933-088x-20.5.164","DOIUrl":"https://doi.org/10.21693/1933-088x-20.5.164","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46054993","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}

{"title":"Genotypes and Phenotypes: A Review of Pulmonary Hypertension in Genetic Syndromes","authors":"Rachel T. Sullivan, E. Austin","doi":"10.21693/1933-088x-20.5.150","DOIUrl":"https://doi.org/10.21693/1933-088x-20.5.150","url":null,"abstract":"There has been significant advancement in the understanding of the genetics of pulmonary hypertension (PH), particularly in those with heritable or idiopathic pulmonary arterial hypertension. In addition to genetic variants with a primarily pulmonary vascular disease phenotype, the prevalence of PH in other genetic syndromes is increasingly recognized. We will review the current knowledge of PH associated with multisystem genetic syndromes. There is high prevalence of coexisting cardiac and pulmonary disease, making it challenging to discern whether PH is secondary to these processes or underlying genetic makeup. There is a paucity of data on response to PH-targeted therapy and implications on overall prognosis.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46157429","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}

{"title":"PH Professional Network: 360-degree Care for the Bronchopulmonary Dysplasia Infant with Pulmonary Hypertension: A Comprehensive Review","authors":"N. Villafranco, E. Whalen, N. Varghese","doi":"10.21693/1933-088x-20.4.123","DOIUrl":"https://doi.org/10.21693/1933-088x-20.4.123","url":null,"abstract":"Premature infants are at risk of developing bronchopulmonary dysplasia and associated pulmonary hypertension. These infants make up a complex group of patients with unique considerations regarding development of lung and vascular disease, comorbidities, and care plans. They are high risk for many complications and poor outcomes due to the severity and complexity of disease. Because of this, a comprehensive approach to care with consideration for multiple organ systems and with an interdisciplinary team of experts is the preferred approach. Here we describe in detail the major considerations in care for these infants.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47550924","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}

{"title":"Connective Tissue Disease, Interstitial Lung Disease, and Pulmonary Hypertension (CTD PH-ILD): A Distinct Entity and Potential Opportunity","authors":"D. Sese, K. Highland","doi":"10.21693/1933-088x-20.4.109","DOIUrl":"https://doi.org/10.21693/1933-088x-20.4.109","url":null,"abstract":"Connective tissue diseases are a multisystem disorder that can cause impairments in quality of life, shorten life expectancy, and increase the risk of mortality at a younger age. These patients have an increased risk for the development of pulmonary hypertension through several mechanisms including pulmonary arterial hypertension and pulmonary hypertension associated with interstitial lung disease. This review aims to discuss the various presentations of connective tissue disease associated with pulmonary hypertension associated with interstitial lung disease, demographics, and survival. It gives an overview of accepted mechanisms of disease pathogenesis, discusses advances in diagnostics, and treatment options. Despite a deeper understanding of disease pathogenesis, treatment for this remains limited to prevention of disease progression. The identification of the primary disease driver requires careful evaluation of the disease phenotype and is a potential target for treatment and prevention of death.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48352008","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}

{"title":"Management of PH-ILD: Past, Present, and Future","authors":"E. Harder, A. Waxman","doi":"10.21693/1933-088x-20.4.119","DOIUrl":"https://doi.org/10.21693/1933-088x-20.4.119","url":null,"abstract":"Pulmonary hypertension associated with interstitial lung disease signifies worse outcomes. Given previous negative clinical trials, the use of pulmonary vasodilators in pulmonary hypertension associated with interstitial lung disease has traditionally been on a case-by-case basis; however, the recent INCREASE study has led to the first and milestone approval of inhaled treprostinil for this population. This review discusses the management of pulmonary hypertension associated with interstitial lung disease from the pulmonary vascular perspective, with an emphasis on clinical trials in this population.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44761250","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}

{"title":"PH-ILD: Identification, Evaluation, and Monitoring: A Diagnostic View From Both Sides","authors":"F. Rahaghi, F. Rahaghi","doi":"10.21693/1933-088x-20.4.103","DOIUrl":"https://doi.org/10.21693/1933-088x-20.4.103","url":null,"abstract":"INTRODUCTION Pulmonary hypertension (PH) has long been recognized as a complication of interstitial lung disease (ILD). It contributes significantly to morbidity and mortality and thus is of key importance in prognostication and deciding the timing of referral for lung transplant. There is increasing evidence of the complexity of its pathogenesis beyond simple fibrosis and hypoxemic vasoconstriction. The pathophysiologic overlap with pulmonary arterial hypertension (PAH) has led to trials of pulmonary vasodilatory therapy in PH-ILD. While prior trials of pulmonary vasodilatory therapy in ILD have presented mixed results, a recent trial of inhaled pulmonary vasodilator therapy in this group has shown positive effect. As a result, the early recognition of the development of PH in ILD may have a greater implication for patients than just prognostication and assessment during considerations for transplant, and may contribute to better outcomes. In this paper we review the current understanding of the pathogenesis of PH in patients with ILD and what is known about the clinical impact of PH in the context of ILD. We then review the importance of hemodynamic assessment to the diagnosis of PH in ILD. Lastly, we review different symptoms, physical exam findings and studies that raise the index of suspicion for the presence of PH in ILD and considerations for incorporating these into initial and subsequent evaluations for patients with ILD. DEFINING PH-ILD While PH can occur in many different contexts in a patient who also has ILD, the implications of labeling an individual as having PH-ILD suggests that ILD is the primary driver of the presence of PH. This can be a subtle distinction: many patients with group 1 PH (PAH), and in particular those with connective tissue disease (CTD), may have a mild form of ILD while also having PAH. Similarly, patients with sarcoidosis may have both ILD and PH while still not being considered as group 3 PH. The understanding of these distinctions is crucial for interpretation of results of clinical studies, which often use such definitions for inclusion or exclusion. Significant history exists in classification of patients with ILD into group 3 PH (PH associated with chronic lung disease) using a combination of hemodynamics and the degree of lung disease. The hemodynamic definition of PH, in the context of chronic lung disease (group 3 PH) was updated in the 6 World Symposium on Pulmonary Hypertension to include a resting mean pulmonary artery pressure of >20 mm Hg, a pulmonary artery occlusion pressure ≤15 mm Hg, and a pulmonary vascular resistance of >3 Wood units. It is important, however, to note hemodynamic definitions do not create a distinction between group 3 and group 1 PH, rather the distinction relies on defining chronic lung disease as the primary driver of precapillary PH. This is done through a combination of pulmonary function testing and imaging—evidence of significant decrement in lung volumes or evidence ","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43638239","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}

{"title":"PH Roundtable: Conundrums and Controversies in PH-ILD: To Treat or Not to Treat—Identifying Optimal Treatment Candidates","authors":"J. Edelman, J. Elwing, A. Vaidya, S. Mathai","doi":"10.21693/1933-088x-20.4.132","DOIUrl":"https://doi.org/10.21693/1933-088x-20.4.132","url":null,"abstract":"This fall, Jeffrey Edelman, MD, Associate Professor of Medicine at the University of Washington and at the VA Medical Center in Seattle, Washington, gathered with Jean Elwing, MD, Professor of Medicine and the Director of the Pulmonary Hypertension Program at the University of Cincinnati; Anjali Vaidya, MD, Associate Professor of Medicine and Co-Director of the Pulmonary Hypertension, Right Heart Failure & CTEPH Program at Temple University in Philadelphia; and Steve Mathai, MD, Associate Professor of Medicine at Johns Hopkins School of Medicine and Co-Director of the Ann Dana Kusch Multidisciplinary Research Program for Pulmonary Hypertension and Interstitial Lung Disease, to discuss pulmonary hypertension (PH) in World Health Organization (WHO) Group 3 patients with interstitial lung disease (ILD).","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41664388","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}

{"title":"Pulmonary Hypertension Clinical Trials and COVID-19: A Discussion With John Ryan and Roham Zamanian","authors":"","doi":"10.21693/1933-088x-20.1.26","DOIUrl":"https://doi.org/10.21693/1933-088x-20.1.26","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44284365","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}

{"title":"PH Roundtable: Pulmonary Hypertension and Telehealth in the Time of Coronavirus Disease 2019","authors":"Anonymous","doi":"10.21693/1933-088x-20.1.16","DOIUrl":"https://doi.org/10.21693/1933-088x-20.1.16","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43405648","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}