Pub Date : 2023-01-01DOI: 10.21693/1933-088x-22.1.79
S. McDevitt, M. Wilson
Comprehensive serial risk assessment in pulmonary arterial hypertension has shown to determine prognosis, monitor disease progression, and guide treatment decisions. The treatment goal is to achieve a low-risk status, which is associated with lower mortality rate. However, use of formal risk assessment in clinical practice has been inconsistent due to numerous barriers related to the multivariable nature of the scores. This publication reviews strategies to increase risk evaluation in daily clinical practice, while emphasizing the role of the RN and APRN in implementing risk assessment calculation and skillful communication to the patient-family dyad to promote open dialogue with shared decision making and improved patient outcomes.
{"title":"PH Professional Network: Real-World Implementation: Nursing Role in Balancing the Art and Science of PAH Risk Assessment","authors":"S. McDevitt, M. Wilson","doi":"10.21693/1933-088x-22.1.79","DOIUrl":"https://doi.org/10.21693/1933-088x-22.1.79","url":null,"abstract":"Comprehensive serial risk assessment in pulmonary arterial hypertension has shown to determine prognosis, monitor disease progression, and guide treatment decisions. The treatment goal is to achieve a low-risk status, which is associated with lower mortality rate. However, use of formal risk assessment in clinical practice has been inconsistent due to numerous barriers related to the multivariable nature of the scores. This publication reviews strategies to increase risk evaluation in daily clinical practice, while emphasizing the role of the RN and APRN in implementing risk assessment calculation and skillful communication to the patient-family dyad to promote open dialogue with shared decision making and improved patient outcomes.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42799647","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 : 2023-01-01DOI: 10.21693/1933-088x-22.1.62
G. Triantafyllou, B. Maron
For decades, pulmonary hypertension (PH) used to be defined by a mean pulmonary artery pressure (mPAP) ≥25 mm Hg; however, this criterion was not based on data that were systematically collected. With the availability of contemporary datasets however, it was evident that the upper limit of normal mPAP was ∼20 mm Hg, which is also the level of mPAP above which adverse outcomes increase. In addition, it is now evident that the specificity of mPAP >20 mm Hg to denote precapillary pulmonary vascular disease could be enhanced by adding pulmonary vascular resistance (PVR) to the precapillary PH definition. Finally, after characterizing large groups of normal individuals, akin to observations for mPAP, it was recently demonstrated that a PVR of ∼2.0 Wood units (WU) is the upper limit of normal, and the lower level associated with all-cause mortality in at-risk patients. The current hemodynamic criteria for PH are positioned to capture more patients compared to the classical definition, with particular implications for earlier diagnosis. Importantly, pulmonary vasodilator therapies have not been tested adequately in patients with mPAP <25 mm Hg or PVR between 2 to 3 WU and, thus, should not be administered in these patients. Mild PH is an active focus of clinical trial design; at present, these patients should be referred to expert PH centers earlier for individualized therapeutic planning. The revised definition of precapillary PH uses a PVR threshold of >2 WU. This value is evidence-based, and exceeding this threshold is associated with adverse clinical outcomes. This revision places focus on early diagnosis, close monitoring, and consideration for certain treatments. Further studies are needed that test the efficacy and safety of pulmonary arterial hypertension-specific therapy in precapillary PH patients with PVR 2 to 3 WU.
几十年来，肺动脉高压（PH）的定义是平均肺动脉压（mPAP）≥25毫米汞柱；然而，这一标准并不是基于系统收集的数据。然而，随着当代数据集的可用性，很明显，正常mPAP的上限为~20 mm Hg，这也是mPAP的水平，超过该水平，不良后果会增加。此外，现在很明显，mPAP>20mm Hg表示毛细血管前肺血管疾病的特异性可以通过在毛细血管前PH定义中添加肺血管阻力（PVR）来增强。最后，在对大组正常个体进行表征后，类似于mPAP的观察结果，最近证明，约2.0 Wood单位（WU）的PVR是正常的上限，也是与高危患者全因死亡率相关的下限。与经典定义相比，目前的PH血液动力学标准能够捕获更多的患者，对早期诊断具有特殊意义。重要的是，肺血管舒张剂治疗尚未在mPAP 2 WU患者中得到充分测试。这个值是基于证据的，超过这个阈值会导致不良的临床结果。这一修订将重点放在早期诊断、密切监测和考虑某些治疗上。需要进一步的研究来测试肺动脉高压特异性治疗PVR2-3WU的毛细血管前PH患者的有效性和安全性。
{"title":"POINT: Is It Time to Lower the Cut-off for Increased Pulmonary Vascular Resistance? Yes","authors":"G. Triantafyllou, B. Maron","doi":"10.21693/1933-088x-22.1.62","DOIUrl":"https://doi.org/10.21693/1933-088x-22.1.62","url":null,"abstract":"\u0000 \u0000 For decades, pulmonary hypertension (PH) used to be defined by a mean pulmonary artery pressure (mPAP) ≥25 mm Hg; however, this criterion was not based on data that were systematically collected. With the availability of contemporary datasets however, it was evident that the upper limit of normal mPAP was ∼20 mm Hg, which is also the level of mPAP above which adverse outcomes increase. In addition, it is now evident that the specificity of mPAP >20 mm Hg to denote precapillary pulmonary vascular disease could be enhanced by adding pulmonary vascular resistance (PVR) to the precapillary PH definition. Finally, after characterizing large groups of normal individuals, akin to observations for mPAP, it was recently demonstrated that a PVR of ∼2.0 Wood units (WU) is the upper limit of normal, and the lower level associated with all-cause mortality in at-risk patients.\u0000 \u0000 \u0000 \u0000 The current hemodynamic criteria for PH are positioned to capture more patients compared to the classical definition, with particular implications for earlier diagnosis. Importantly, pulmonary vasodilator therapies have not been tested adequately in patients with mPAP <25 mm Hg or PVR between 2 to 3 WU and, thus, should not be administered in these patients. Mild PH is an active focus of clinical trial design; at present, these patients should be referred to expert PH centers earlier for individualized therapeutic planning.\u0000 \u0000 \u0000 \u0000 The revised definition of precapillary PH uses a PVR threshold of >2 WU. This value is evidence-based, and exceeding this threshold is associated with adverse clinical outcomes. This revision places focus on early diagnosis, close monitoring, and consideration for certain treatments. Further studies are needed that test the efficacy and safety of pulmonary arterial hypertension-specific therapy in precapillary PH patients with PVR 2 to 3 WU.\u0000","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41683960","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 : 2023-01-01DOI: 10.21693/1933-088x-22.1.55
Jennifer L. Keen, N. Al-Naamani, C. Ventetuolo
Pulmonary arterial hypertension (PAH) is a devastating disease mediated by vasoconstriction and vascular remodeling of the pulmonary vasculature. Current therapies target the imbalance of vasoconstrictors and vasorelaxants in 3 pathways: nitric oxide, prostacyclin, and endothelin. While these have extended lifespans for PAH patients, significant morbidity and mortality remains. Notably, the progress in PAH therapy for over a decade has utilized these same 3 pathways. Fortunately, several new treatment options utilizing different mechanisms are emerging and will be reviewed here.
{"title":"The Future of PAH Treatment","authors":"Jennifer L. Keen, N. Al-Naamani, C. Ventetuolo","doi":"10.21693/1933-088x-22.1.55","DOIUrl":"https://doi.org/10.21693/1933-088x-22.1.55","url":null,"abstract":"Pulmonary arterial hypertension (PAH) is a devastating disease mediated by vasoconstriction and vascular remodeling of the pulmonary vasculature. Current therapies target the imbalance of vasoconstrictors and vasorelaxants in 3 pathways: nitric oxide, prostacyclin, and endothelin. While these have extended lifespans for PAH patients, significant morbidity and mortality remains. Notably, the progress in PAH therapy for over a decade has utilized these same 3 pathways. Fortunately, several new treatment options utilizing different mechanisms are emerging and will be reviewed here.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47760932","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 : 2023-01-01DOI: 10.21693/1933-088x-22.1.71
{"title":"PH Roundtable: Pros and Cons of the 2022 ERS/ESC Guidelines: Practicality vs Real World View","authors":"","doi":"10.21693/1933-088x-22.1.71","DOIUrl":"https://doi.org/10.21693/1933-088x-22.1.71","url":null,"abstract":"","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47756570","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 : 2022-10-01DOI: 10.21693/1933-088x-21.4.109
B. Graham
Schistosomiasis is a major cause of group 1 pulmonary arterial hypertension (PAH) worldwide. Schistosomiasis results from a parasitic infection present in over 200 million individuals worldwide. Schistosomiasis-associated PAH was initially thought to be obstructive due to egg embolization but has a pulmonary vascular pathology like other forms of group 1 PAH and can be treated using conventional PAH therapies. Mechanisms that underlie the development of schistosomiasis-associated PAH include type 2 inflammation which triggers TGF-β signaling; importantly, TGF-β signaling is a pathway shared with other PAH etiologies. However, many things which are unknown about this disease remain, including if the lung vascular pathology results from egg embolization causing localized inflammation and vessel remodeling, or if this is a form of portopulmonary hypertension resulting from schistosomiasis liver disease.
{"title":"Schistosomiasis-Associated Pulmonary Arterial Hypertension","authors":"B. Graham","doi":"10.21693/1933-088x-21.4.109","DOIUrl":"https://doi.org/10.21693/1933-088x-21.4.109","url":null,"abstract":"Schistosomiasis is a major cause of group 1 pulmonary arterial hypertension (PAH) worldwide. Schistosomiasis results from a parasitic infection present in over 200 million individuals worldwide. Schistosomiasis-associated PAH was initially thought to be obstructive due to egg embolization but has a pulmonary vascular pathology like other forms of group 1 PAH and can be treated using conventional PAH therapies. Mechanisms that underlie the development of schistosomiasis-associated PAH include type 2 inflammation which triggers TGF-β signaling; importantly, TGF-β signaling is a pathway shared with other PAH etiologies. However, many things which are unknown about this disease remain, including if the lung vascular pathology results from egg embolization causing localized inflammation and vessel remodeling, or if this is a form of portopulmonary hypertension resulting from schistosomiasis liver disease.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44800117","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 : 2022-10-01DOI: 10.21693/1933-088x-21.4.123
R. Ruggiero, S. Bartolome
Portopulmonary hypertension (POPH) is a rare complication of liver disease occurring when pulmonary arterial hypertension develops in the setting of portal hypertension. It increases the morbidity and mortality compared to patients with cirrhosis alone. POPH is classified in Group 1 pulmonary arterial hypertension, which has important implications on treatment. After aggressive treatment and in carefully selected patients, liver transplantation can be performed; this can be curative of not only their liver disease but also of their POPH. Treatment and patient selection for optimum results continues to evolve. This article provides updates on the definition, clinical course, and treatment of patients with POPH. We will also discuss the evolving data in treatment and liver transplantation in POPH.
{"title":"Portopulmonary Hypertension: A Review","authors":"R. Ruggiero, S. Bartolome","doi":"10.21693/1933-088x-21.4.123","DOIUrl":"https://doi.org/10.21693/1933-088x-21.4.123","url":null,"abstract":"Portopulmonary hypertension (POPH) is a rare complication of liver disease occurring when pulmonary arterial hypertension develops in the setting of portal hypertension. It increases the morbidity and mortality compared to patients with cirrhosis alone. POPH is classified in Group 1 pulmonary arterial hypertension, which has important implications on treatment. After aggressive treatment and in carefully selected patients, liver transplantation can be performed; this can be curative of not only their liver disease but also of their POPH. Treatment and patient selection for optimum results continues to evolve. This article provides updates on the definition, clinical course, and treatment of patients with POPH. We will also discuss the evolving data in treatment and liver transplantation in POPH.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47599733","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 : 2022-10-01DOI: 10.21693/1933-088x-21.4.130
Wendy Nelson, Mary Miller, Shannon A. Salveson
Advanced practice nurses play an integral role in the management of chronic thromboembolic pulmonary hypertension patients undergoing pulmonary thromboendarterectomy and balloon pulmonary angioplasty. As integral members of the multidisciplinary team, advance practice nurses assist chronic thromboembolic pulmonary hypertension patients in the presurgical, postsurgical, and procedural settings by ensuring appropriate referral, workup, evaluation, and education.
{"title":"PH Professional Network: The Role of the Advanced Practice Nurse With Patients Undergoing Pulmonary Thromboendarterectomy and Balloon Pulmonary Angioplasty for CTEPH","authors":"Wendy Nelson, Mary Miller, Shannon A. Salveson","doi":"10.21693/1933-088x-21.4.130","DOIUrl":"https://doi.org/10.21693/1933-088x-21.4.130","url":null,"abstract":"Advanced practice nurses play an integral role in the management of chronic thromboembolic pulmonary hypertension patients undergoing pulmonary thromboendarterectomy and balloon pulmonary angioplasty. As integral members of the multidisciplinary team, advance practice nurses assist chronic thromboembolic pulmonary hypertension patients in the presurgical, postsurgical, and procedural settings by ensuring appropriate referral, workup, evaluation, and education.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44416056","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-21.3.60
S. Jasuja, Alexander E. Sherman, R. Saggar, R. Channick
The follow-up of patients with acute pulmonary embolism is an essential component of their comprehensive care. This manuscript will discuss the critical components involved in the outpatient follow-up of pulmonary embolism, including the development of post hospitalization follow-up clinics, assessment of functional capacity and residual right ventricular function, anticoagulation, recurrence risk of venous thromboembolism, and retrieval of inferior vena cava filters. In addition to these listed topics, the epidemiology of chronic thromboembolic pulmonary hypertension will be discussed, including the spectrum of postpulmonary embolism syndrome (PPES), risk factors for the development of chronic thromboembolic pulmonary hypertension, and the incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism.
{"title":"Postpulmonary Embolism Follow-Up and Epidemiology of Chronic Thromboembolic Pulmonary Hypertension","authors":"S. Jasuja, Alexander E. Sherman, R. Saggar, R. Channick","doi":"10.21693/1933-088x-21.3.60","DOIUrl":"https://doi.org/10.21693/1933-088x-21.3.60","url":null,"abstract":"The follow-up of patients with acute pulmonary embolism is an essential component of their comprehensive care. This manuscript will discuss the critical components involved in the outpatient follow-up of pulmonary embolism, including the development of post hospitalization follow-up clinics, assessment of functional capacity and residual right ventricular function, anticoagulation, recurrence risk of venous thromboembolism, and retrieval of inferior vena cava filters. In addition to these listed topics, the epidemiology of chronic thromboembolic pulmonary hypertension will be discussed, including the spectrum of postpulmonary embolism syndrome (PPES), risk factors for the development of chronic thromboembolic pulmonary hypertension, and the incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism.","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":"44148870","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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