Pub Date : 2022-07-01DOI: 10.21693/1933-088x-21.3.101
Abhijit A. Raval, Nancy Edwards, Ravi Kant, V. Verma, M. Antony, D. Raval
{"title":"PH Grand Rounds: COVID-19 Infection Causing Delayed Pulmonary Arterial Hypertension","authors":"Abhijit A. Raval, Nancy Edwards, Ravi Kant, V. Verma, M. Antony, D. Raval","doi":"10.21693/1933-088x-21.3.101","DOIUrl":"https://doi.org/10.21693/1933-088x-21.3.101","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47386832","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 : 2022-07-01DOI: 10.21693/1933-088x-21.3.88
A. Goyanes, G. Heresi
Medical therapy in chronic thromboembolic pulmonary hypertension (CTEPH) has two primary goals- to prevent recurrent thromboembolic events and to reduce right ventricular afterload with targeted medications (vasodilators) for pulmonary hypertension. These medical strategies are used in conjunction with mechanical treatments for CTEPH (pulmonary thromboendarterectomy (PTE) or balloon angioplasty). In the context of this review, we discuss anticoagulation strategies, patient selection for vasodilator therapy with particular focus on hemodynamic and clinically meaningful definitions of residual pulmonary hypertension after PTE and inoperable disease and then summarize the current randomized clinical trials (RCT) which have studied effectiveness of vasodilators in patients with CTEPH.
{"title":"Medical Management of Chronic Thromboembolic Pulmonary Hypertension","authors":"A. Goyanes, G. Heresi","doi":"10.21693/1933-088x-21.3.88","DOIUrl":"https://doi.org/10.21693/1933-088x-21.3.88","url":null,"abstract":"Medical therapy in chronic thromboembolic pulmonary hypertension (CTEPH) has two primary goals- to prevent recurrent thromboembolic events and to reduce right ventricular afterload with targeted medications (vasodilators) for pulmonary hypertension. These medical strategies are used in conjunction with mechanical treatments for CTEPH (pulmonary thromboendarterectomy (PTE) or balloon angioplasty). In the context of this review, we discuss anticoagulation strategies, patient selection for vasodilator therapy with particular focus on hemodynamic and clinically meaningful definitions of residual pulmonary hypertension after PTE and inoperable disease and then summarize the current randomized clinical trials (RCT) which have studied effectiveness of vasodilators in patients with CTEPH.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46988138","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 : 2022-07-01DOI: 10.21693/1933-088x-21.3.93
{"title":"PH Roundtable: Tailoring CTEPH Imaging for Evaluation and Postintervention Assessment—What Works and What’s New?","authors":"","doi":"10.21693/1933-088x-21.3.93","DOIUrl":"https://doi.org/10.21693/1933-088x-21.3.93","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48290838","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 : 2022-07-01DOI: 10.21693/1933-088x-21.3.73
M. Madani, J. Higgins
Chronic ThromboEmbolic Pulmonary Hypertension (CTEPH) is a potentially curative form of pulmonary hypertension, which continues to be underdiagnosed. Pulmonary ThromboEndarterectomy (PTE, also referred to as PEA for Pulmonary Endarterectomy) is a technically challenging procedure that requires careful patient selection, meticulous surgical techniques, and expertise in postoperative care. Over the last decade, there have been significant advances not only in the techniques of the operation, but also in the postoperative management of major complications. Furthermore, advances have been made not only in medical therapy, but also in percutaneous interventions, in the form of balloon pulmonary angioplasty (BPA). BPA and medical therapy are considered to be palliative; they are reserved for patients who are inoperable, or for those who continue to have symptomatic PH postoperatively. PTE remains the gold standard treatment for CTEPH, as long as the patient has evidence of surgically accessible disease, and the patient has acceptable surgical risk. All CTEPH patients should be evaluated and considered for surgery, and no patient should be turned down without consultation with a multidisciplinary team at an expert center. Furthermore, no amount of PH or degree of right heart failure is a contraindication to surgery, as long as there is corresponding level of disease. Excellent short- and long-term results can be achieved with current data suggesting significant advantage with 10-yr survival of 85–90%.
{"title":"Pulmonary Thromboendarterectomy: Patient Selection, Techniques, Outcomes, and Recent Advances","authors":"M. Madani, J. Higgins","doi":"10.21693/1933-088x-21.3.73","DOIUrl":"https://doi.org/10.21693/1933-088x-21.3.73","url":null,"abstract":"Chronic ThromboEmbolic Pulmonary Hypertension (CTEPH) is a potentially curative form of pulmonary hypertension, which continues to be underdiagnosed. Pulmonary ThromboEndarterectomy (PTE, also referred to as PEA for Pulmonary Endarterectomy) is a technically challenging procedure that requires careful patient selection, meticulous surgical techniques, and expertise in postoperative care. Over the last decade, there have been significant advances not only in the techniques of the operation, but also in the postoperative management of major complications. Furthermore, advances have been made not only in medical therapy, but also in percutaneous interventions, in the form of balloon pulmonary angioplasty (BPA). BPA and medical therapy are considered to be palliative; they are reserved for patients who are inoperable, or for those who continue to have symptomatic PH postoperatively. PTE remains the gold standard treatment for CTEPH, as long as the patient has evidence of surgically accessible disease, and the patient has acceptable surgical risk. All CTEPH patients should be evaluated and considered for surgery, and no patient should be turned down without consultation with a multidisciplinary team at an expert center. Furthermore, no amount of PH or degree of right heart failure is a contraindication to surgery, as long as there is corresponding level of disease. Excellent short- and long-term results can be achieved with current data suggesting significant advantage with 10-yr survival of 85–90%.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43877682","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 : 2022-07-01DOI: 10.21693/1933-088x-21.3.66
A. Vaidya, P. Forfia
Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct form of pulmonary hypertension, uniquely characterized by pulmonary artery narrowing and occlusion from clot material. With advances in medical education and therapeutic options, awareness of CTEPH has grown significantly in recent years. The diagnostic evaluation remains complex, warranting an integrated assessment of history, physical exam, echocardiogram, chest imaging including computerized tomography with angiography, ventilation–perfusion scanning, right heart catheterization, catheter-based pulmonary angiography, and assessment for medical and mechanical CTEPH risk factors. The diagnostic evaluation of CTEPH is reviewed here.
{"title":"Diagnostic Evaluation of Chronic Thromboembolic Pulmonary Hypertension","authors":"A. Vaidya, P. Forfia","doi":"10.21693/1933-088x-21.3.66","DOIUrl":"https://doi.org/10.21693/1933-088x-21.3.66","url":null,"abstract":"Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct form of pulmonary hypertension, uniquely characterized by pulmonary artery narrowing and occlusion from clot material. With advances in medical education and therapeutic options, awareness of CTEPH has grown significantly in recent years. The diagnostic evaluation remains complex, warranting an integrated assessment of history, physical exam, echocardiogram, chest imaging including computerized tomography with angiography, ventilation–perfusion scanning, right heart catheterization, catheter-based pulmonary angiography, and assessment for medical and mechanical CTEPH risk factors. The diagnostic evaluation of CTEPH is reviewed here.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41933098","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 : 2022-04-01DOI: 10.21693/1933-088x-21.2.49
{"title":"PH Roundtable: Disparities in PH","authors":"","doi":"10.21693/1933-088x-21.2.49","DOIUrl":"https://doi.org/10.21693/1933-088x-21.2.49","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47100756","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 : 2022-04-01DOI: 10.21693/1933-088x-21.2.29
V. A. de Jesus Perez, Arunabh Talwar
{"title":"Guest Editors' Memo: Disparities in Pulmonary Arterial Hypertension Care: Challenges and Solutions","authors":"V. A. de Jesus Perez, Arunabh Talwar","doi":"10.21693/1933-088x-21.2.29","DOIUrl":"https://doi.org/10.21693/1933-088x-21.2.29","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46188139","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 : 2022-04-01DOI: 10.21693/1933-088x-21.2.35
Debbie Drell, Ashanthi De Silva, Cornelia Lee
Compared with chronic diseases affecting larger populations, rare disease (RD) patients experience great inequities in diagnosis, care, and research. Within RDs, health disparities compound these inequities, as marginalized communities experience additional barriers in accessing clinical care and are often underrepresented from participation in research and clinical trials. For almost 40 years, the National Organization for Rare Disorders (NORD), a RD umbrella organization with over 300 nonprofit organizational members, has led efforts to understand and address inequities for the RD community through innovative research, programming, and collaboration with patients, caregivers, practitioners, and external stakeholders. The beginning of the COVID-19 pandemic in 2020 brought to light longstanding disparities and discrimination for marginalized communities as well as pivotal racial justice movements. These events spurred many RD nonprofit organizations’ interest in increasing outreach and engagement with minoritized communities within RDs and diversifying their organization internally. Building on the increased interest in diversity, equity, and inclusion (DEI), NORD has focused on collecting case studies from within NORD and its member organizations to capture current efforts to improve DEI within the RD ecosystem. One way clinicians can work to mitigate the effects of disparities is to collaborate with RD patient organizations; this article provides a means by which clinicians and researchers can understand some of the challenges RD nonprofit organizations face in bridging disparities and learn about solutions to supporting marginalized patients within their communities. Clinicians are encouraged to join NORD in our policy efforts advocating to ensure patient access to health care providers practicing in a different state vis-à-vis telehealth.
{"title":"What Rare Disease Patient Advocacy Groups Are Doing to Mitigate the Effects of Disparities","authors":"Debbie Drell, Ashanthi De Silva, Cornelia Lee","doi":"10.21693/1933-088x-21.2.35","DOIUrl":"https://doi.org/10.21693/1933-088x-21.2.35","url":null,"abstract":"Compared with chronic diseases affecting larger populations, rare disease (RD) patients experience great inequities in diagnosis, care, and research. Within RDs, health disparities compound these inequities, as marginalized communities experience additional barriers in accessing clinical care and are often underrepresented from participation in research and clinical trials. For almost 40 years, the National Organization for Rare Disorders (NORD), a RD umbrella organization with over 300 nonprofit organizational members, has led efforts to understand and address inequities for the RD community through innovative research, programming, and collaboration with patients, caregivers, practitioners, and external stakeholders.\u0000 The beginning of the COVID-19 pandemic in 2020 brought to light longstanding disparities and discrimination for marginalized communities as well as pivotal racial justice movements. These events spurred many RD nonprofit organizations’ interest in increasing outreach and engagement with minoritized communities within RDs and diversifying their organization internally. Building on the increased interest in diversity, equity, and inclusion (DEI), NORD has focused on collecting case studies from within NORD and its member organizations to capture current efforts to improve DEI within the RD ecosystem. One way clinicians can work to mitigate the effects of disparities is to collaborate with RD patient organizations; this article provides a means by which clinicians and researchers can understand some of the challenges RD nonprofit organizations face in bridging disparities and learn about solutions to supporting marginalized patients within their communities. Clinicians are encouraged to join NORD in our policy efforts advocating to ensure patient access to health care providers practicing in a different state vis-à-vis telehealth.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42367096","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 : 2022-04-01DOI: 10.21693/1933-088x-21.2.30
A. Talwar, K. C. Morel, V. D. Perez, Arunabh Talwar
Health disparities have a major impact in the quality of life and level of clinical care received in minority populations in the United States. Underrepresented patients with pulmonary arterial hypertension (PAH) may be at risk for worse outcomes. Furthermore, advances in biomedical research have provided extensive knowledge on the genetic role in the pathogenesis of PAH but whether these also impact minorities is incompletely understood. Health disparities in patients with PAH create an enormous barrier in health care delivery. Understanding the contributors to health disparity represent a fundamental step towards personalized medicine and further improvement in PAH care.
{"title":"The Impact of Socioeconomic, Racial, and Ethnic Disparities on Pulmonary Hypertension Diagnosis and Treatment","authors":"A. Talwar, K. C. Morel, V. D. Perez, Arunabh Talwar","doi":"10.21693/1933-088x-21.2.30","DOIUrl":"https://doi.org/10.21693/1933-088x-21.2.30","url":null,"abstract":"Health disparities have a major impact in the quality of life and level of clinical care received in minority populations in the United States. Underrepresented patients with pulmonary arterial hypertension (PAH) may be at risk for worse outcomes. Furthermore, advances in biomedical research have provided extensive knowledge on the genetic role in the pathogenesis of PAH but whether these also impact minorities is incompletely understood. Health disparities in patients with PAH create an enormous barrier in health care delivery. Understanding the contributors to health disparity represent a fundamental step towards personalized medicine and further improvement in PAH care.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41889910","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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