Pub Date : 2019-07-29DOI: 10.21693/1933-088X-18.2.74
A. M. Pirfo, E. Chen
{"title":"PH Professional Network: “Just Do It”: Practical Aspects of Pulmonary Rehabilitation Programs","authors":"A. M. Pirfo, E. Chen","doi":"10.21693/1933-088X-18.2.74","DOIUrl":"https://doi.org/10.21693/1933-088X-18.2.74","url":null,"abstract":"","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":"48390045","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-18.2.47
F. Rischard, B. Borlaug
Background: Most pulmonary vascular disease (PVD) is poorly modifiable and incurable even with effective therapy. Therefore, adaptation to stress, the reserve of the cardiopulmonary system, is important for assessment of patient function and prognosis. Methods that assess the adaptation to stress, especially exercise, provide valuable insight into diagnosis, prognosis, and response to therapy. Implications for Clinicians: We provide a comprehensive review of the indications, methodology, and interpretation, as well as practical information of the forms of provocative testing in PVD. We include 6-minute walk testing, noninvasive cardiopulmonary exercise testing (CPET), invasive CPET, and additional forms, including volume loading. Conclusions: Through a clear understanding of the methodology in the assessment of PVD, the clinician can determine which of these “tools of the trade” are best suited to the individual patient and situation.
{"title":"Tools of the Trade: How Do You Perform and Interpret an Exercise Test?","authors":"F. Rischard, B. Borlaug","doi":"10.21693/1933-088X-18.2.47","DOIUrl":"https://doi.org/10.21693/1933-088X-18.2.47","url":null,"abstract":"Background: Most pulmonary vascular disease (PVD) is poorly modifiable and incurable even with effective therapy. Therefore, adaptation to stress, the reserve of the cardiopulmonary system, is important for assessment of patient function and prognosis. Methods that assess the adaptation to stress, especially exercise, provide valuable insight into diagnosis, prognosis, and response to therapy.\u0000 Implications for Clinicians: We provide a comprehensive review of the indications, methodology, and interpretation, as well as practical information of the forms of provocative testing in PVD. We include 6-minute walk testing, noninvasive cardiopulmonary exercise testing (CPET), invasive CPET, and additional forms, including volume loading.\u0000 Conclusions: Through a clear understanding of the methodology in the assessment of PVD, the clinician can determine which of these “tools of the trade” are best suited to the individual patient and situation.","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":"43958862","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-18.2.56
Martin Johnson, A. Peacock
Exercise training as treatment has become well established in many cardiorespiratory conditions. This is also increasingly the case in pulmonary arterial hypertension, where several studies have demonstrated improvements in symptoms, exercise capacity and quality of life. There remains, however, much potential for development. Current research is focused on clarifying the mechanism of benefit in pulmonary hypertension and exploring strategies for both optimizing the treatment effect and widening access to this intervention.
{"title":"Treating Pulmonary Arterial Hypertension With Exercise: The Role of Rehabilitative Medicine","authors":"Martin Johnson, A. Peacock","doi":"10.21693/1933-088X-18.2.56","DOIUrl":"https://doi.org/10.21693/1933-088X-18.2.56","url":null,"abstract":"Exercise training as treatment has become well established in many cardiorespiratory conditions. This is also increasingly the case in pulmonary arterial hypertension, where several studies have demonstrated improvements in symptoms, exercise capacity and quality of life. There remains, however, much potential for development. Current research is focused on clarifying the mechanism of benefit in pulmonary hypertension and exploring strategies for both optimizing the treatment effect and widening access to this intervention.","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":"43618443","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-18.2.68
L. Melendres‐Groves
{"title":"Ask the Expert: What Is the Exercise “Prescription” That You Provide to Your Patients?","authors":"L. Melendres‐Groves","doi":"10.21693/1933-088X-18.2.68","DOIUrl":"https://doi.org/10.21693/1933-088X-18.2.68","url":null,"abstract":"","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":"45478684","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-18.2.70
A. Gupta, C. Dillingham, H. Farber, M. Farmer
{"title":"PH Grand Rounds: A Case of Pulmonary Hypertension Associated With High Cardiac Output State from Arteriovenous Fistula–Or Is It?","authors":"A. Gupta, C. Dillingham, H. Farber, M. Farmer","doi":"10.21693/1933-088X-18.2.70","DOIUrl":"https://doi.org/10.21693/1933-088X-18.2.70","url":null,"abstract":"","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":"44386784","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 : 2019-07-01DOI: 10.21693/1933-088x-18.2.63
On May 20, 2019, Guest Editor Ronald Oudiz, MD, Director of the Pulmonary Hypertension Program at Harbor-UCLA Medical Center in Los Angeles, California, led a discussion with Aaron Waxman, MD, PhD, Director of the Center for Pulmonary Heart Disease at Brigham and Women's Hospital and Harvard Medical School in Boston, Massachusetts, and Robert Naeije, MD, Professor Emeritus at the Free University of Brussels, Belgium.
{"title":"Pulmonary Hypertension Roundtable: The Role of Exercise in Clinical Practice and Clinical Trials","authors":"","doi":"10.21693/1933-088x-18.2.63","DOIUrl":"https://doi.org/10.21693/1933-088x-18.2.63","url":null,"abstract":"On May 20, 2019, Guest Editor Ronald Oudiz, MD, Director of the Pulmonary Hypertension Program at Harbor-UCLA Medical Center in Los Angeles, California, led a discussion with Aaron Waxman, MD, PhD, Director of the Center for Pulmonary Heart Disease at Brigham and Women's Hospital and Harvard Medical School in Boston, Massachusetts, and Robert Naeije, MD, Professor Emeritus at the Free University of Brussels, Belgium.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42893744","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 : 2019-03-29DOI: 10.21693/1933-088X-18.1.31
J. D. Serfas, R. Krasuski
{"title":"Ask the Expert: Transplantation in Eisenmenger Syndrome: Getting to the Heart of the Matter","authors":"J. D. Serfas, R. Krasuski","doi":"10.21693/1933-088X-18.1.31","DOIUrl":"https://doi.org/10.21693/1933-088X-18.1.31","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48432714","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 : 2019-03-01DOI: 10.21693/1933-088X-18.1.4
Vishal R. Kaley, N. Dahdah, A. El-Sisi, J. Grohmann, E. Rosenthal, Matthew O. Jones, G. Morgan, Nicholas Hayes, Ashish H. Shah, C. Karakurt, M. Sadiq, M. Sigler, H. Figulla, M. Becker, N. Haas, E. Onorato, A. Rico, S. Roymanee, A. Uebing, W. Wiebe, Bennett P. Samuel, Z. Hijazi, J. Vettukattil
Unlike other intracardiac shunts, there is no known linear relationship between ostium secundum atrial septal defects (ASD) and development of pulmonary hypertension (PH). PH is observed in 8% to 10% of all ASD patients. Atrial septal defect–associated pulmonary hypertension (ASDAPH) is usually independent of the degree, duration of shunting, and defect size. Complete closure of ASD in these patients can be detrimental due to the potential risk for increase in pulmonary vascular resistance (PVR). Fenestrated closure allows for controlled residual shunt providing adequate cardiac output with a mechanism for decompression in the event of critical increase in PVR. After approval from institutional review boards and agencies protecting human subjects, 42 patients from 29 international centers underwent compassionate use of the Occlutech Fenestrated Atrial Septal Defect (FASD) device. Physician implanters reported outcomes via electronic survey. Follow-up data were available for 25 patients (72% female, n=18) from 18 centers. Symptomatic improvement was observed in a majority of the patients with reduction in New York Heart Association class III symptoms from 68% at baseline to 8% at long-term follow-up. Mean oxygen saturation improved from 93% at baseline to 97% at long-term follow-up (P=0.0066). Reduction in right atrial pressure and mean pulmonary arterial pressure were also noted. During follow-up, one patient had spontaneous occlusion of the fenestration requiring emergency stenting. No other major complications were observed. FASD implantation improves outcomes in patients with ASDAPH; however, further studies are required in a large cohort of patients to determine timing of intervention, optimal fenestration size, and long-term prognosis.
{"title":"Atrial Septal Defect–Associated Pulmonary Hypertension: Outcomes of Closure With a Fenestrated Device","authors":"Vishal R. Kaley, N. Dahdah, A. El-Sisi, J. Grohmann, E. Rosenthal, Matthew O. Jones, G. Morgan, Nicholas Hayes, Ashish H. Shah, C. Karakurt, M. Sadiq, M. Sigler, H. Figulla, M. Becker, N. Haas, E. Onorato, A. Rico, S. Roymanee, A. Uebing, W. Wiebe, Bennett P. Samuel, Z. Hijazi, J. Vettukattil","doi":"10.21693/1933-088X-18.1.4","DOIUrl":"https://doi.org/10.21693/1933-088X-18.1.4","url":null,"abstract":"Unlike other intracardiac shunts, there is no known linear relationship between ostium secundum atrial septal defects (ASD) and development of pulmonary hypertension (PH). PH is observed in 8% to 10% of all ASD patients. Atrial septal defect–associated pulmonary hypertension (ASDAPH) is usually independent of the degree, duration of shunting, and defect size. Complete closure of ASD in these patients can be detrimental due to the potential risk for increase in pulmonary vascular resistance (PVR). Fenestrated closure allows for controlled residual shunt providing adequate cardiac output with a mechanism for decompression in the event of critical increase in PVR. After approval from institutional review boards and agencies protecting human subjects, 42 patients from 29 international centers underwent compassionate use of the Occlutech Fenestrated Atrial Septal Defect (FASD) device. Physician implanters reported outcomes via electronic survey. Follow-up data were available for 25 patients (72% female, n=18) from 18 centers. Symptomatic improvement was observed in a majority of the patients with reduction in New York Heart Association class III symptoms from 68% at baseline to 8% at long-term follow-up. Mean oxygen saturation improved from 93% at baseline to 97% at long-term follow-up (P=0.0066). Reduction in right atrial pressure and mean pulmonary arterial pressure were also noted. During follow-up, one patient had spontaneous occlusion of the fenestration requiring emergency stenting. No other major complications were observed. FASD implantation improves outcomes in patients with ASDAPH; however, further studies are required in a large cohort of patients to determine timing of intervention, optimal fenestration size, and long-term prognosis.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48870940","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 : 2019-03-01DOI: 10.21693/1933-088X-18.1.33
D. Levine, Emily Slater, U. Krishnan, E. Rosenzweig
{"title":"PH Grand Rounds: Eisenmenger Syndrome: When Less Is More","authors":"D. Levine, Emily Slater, U. Krishnan, E. Rosenzweig","doi":"10.21693/1933-088X-18.1.33","DOIUrl":"https://doi.org/10.21693/1933-088X-18.1.33","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43731732","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 : 2019-03-01DOI: 10.21693/1933-088X-18.1.10
M. Mullen
Atrial septal defects (ASDs) are common congenital heart defects in children and adults. Pulmonary arterial hypertension (PAH) is found in subsets of both pediatric and adult patients with atrial defects under varied clinical contexts. The pulmonary hypertension specialist is often faced with questions surrounding timing and method of defect closure, which may have significant impact on procedural and long-term morbidity and survival. This review highlights important differences in management between children and adults with ASDs associated with PAH, highlighting indications for closure, operability, types of closure, and long-term outcomes.
{"title":"Challenges in the Patient With Pulmonary Hypertension and Atrial Septal Defect: Understanding When and How to Close the Defect","authors":"M. Mullen","doi":"10.21693/1933-088X-18.1.10","DOIUrl":"https://doi.org/10.21693/1933-088X-18.1.10","url":null,"abstract":"Atrial septal defects (ASDs) are common congenital heart defects in children and adults. Pulmonary arterial hypertension (PAH) is found in subsets of both pediatric and adult patients with atrial defects under varied clinical contexts. The pulmonary hypertension specialist is often faced with questions surrounding timing and method of defect closure, which may have significant impact on procedural and long-term morbidity and survival. This review highlights important differences in management between children and adults with ASDs associated with PAH, highlighting indications for closure, operability, types of closure, and long-term outcomes.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47509970","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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