Pulmonary Circulation最新文献

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Bioinformatics Analysis Identifies a Potential Key Gene in the Pathogenesis of Pulmonary Hypertension-HSPH1. 生物信息学分析确定肺动脉高压发病机制的潜在关键基因- hsph1。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2025-07-16 eCollection Date: 2025-07-01 DOI: 10.1002/pul2.70127
Yuelong Ji, Xia Li, Rui Wang, Zhongsu Wang, Guangdong Yu, Conghu Yuan, Xiangnan Li
{"title":"Bioinformatics Analysis Identifies a Potential Key Gene in the Pathogenesis of Pulmonary Hypertension-HSPH1.","authors":"Yuelong Ji, Xia Li, Rui Wang, Zhongsu Wang, Guangdong Yu, Conghu Yuan, Xiangnan Li","doi":"10.1002/pul2.70127","DOIUrl":"https://doi.org/10.1002/pul2.70127","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a progressive vascular disease characterized by small artery occlusion, increased pulmonary vascular resistance, and right heart failure. HSPH1, a member of the heat shock protein family, has been shown to inhibit protein aggregation but its role in PAH remains unclear. The purpose of this study was to explore the expression pattern and potential mechanism of HSPH1 in PAH, and to provide new diagnostic markers for PAH. In the study differentially expressed genes from two GEO microarray datasets (GSE53408, GSE113439) were analyzed to identify potential biomarkers for PAH. The expression of HSPH1 in normal lung tissue and pulmonary hypertension tissue was verified by bioinformatics and various experiments. This study also validated the potential mechanism of action of HSPH1 in PAH through transfection techniques. In addition, clinical correlation analysis was used to verify whether HSPH1 was correlated with clinical indicators (age, smoking history, hypertension, SII, NLR, PLR). The results showed that the protein level of HSPH1 was significantly increased in the pulmonary artery tissue of rats with pulmonary hypertension. In the plasma of patients with clinical PAH, the expression of HSPH1 mRNA was also observed to be significantly increased, and its expression was also associated with inflammatory markers such as NLR, PLR and SII. In addition, wet experiments found that HSPH1 could promote the proliferation of pulmonary artery smooth muscle cells, promote epithelial-mesenchymal transformation and inhibit apoptosis. These findings suggest that HSPH1 plays a crucial role in PAH progression and may serve as a potential diagnostic biomarker for the disease.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70127"},"PeriodicalIF":2.2,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12267103/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144660007","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Hemodynamic Patterns in Interstitial Lung Disease: A Snapshot of Clinical Practice and a Roadmap for Future Trials. 间质性肺疾病的血流动力学模式:临床实践的快照和未来试验的路线图。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2025-07-15 eCollection Date: 2025-07-01 DOI: 10.1002/pul2.70108
Ho Cheol Kim, Christopher S King, Christopher A Thomas, Jared Wilkinson, Junad Chowdhury, Behnam Tehrani, Steven D Nathan
{"title":"Hemodynamic Patterns in Interstitial Lung Disease: A Snapshot of Clinical Practice and a Roadmap for Future Trials.","authors":"Ho Cheol Kim, Christopher S King, Christopher A Thomas, Jared Wilkinson, Junad Chowdhury, Behnam Tehrani, Steven D Nathan","doi":"10.1002/pul2.70108","DOIUrl":"https://doi.org/10.1002/pul2.70108","url":null,"abstract":"<p><p>Interstitial lung disease (ILD) complicated by pulmonary hypertension (PH) is associated with poor outcomes. However, real-world data characterizing the hemodynamic profiles of ILD patients undergoing right heart catheterization (RHC) remain limited. We retrospectively analyzed ILD patients who underwent RHC between 2006 and 2024. Hemodynamic profiles were assessed according to the 5th, 6th, and 7th World Symposium on Pulmonary Hypertension (WSPH) definitions and for severe PH (pulmonary vascular resistance [PVR] > 5 Wood units). Correlations between pulmonary function testing (PFT) variables and PVR were explored, and baseline characteristics were compared across groups stratified by PH severity. There were 3541 ILD patients evaluated of whom 12.2% underwent RHC. Among 371 patients with available RHC data and pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg, 49.6%, 54.4%, and 69.4% met the 5th, 6th, and 7th WSPH criteria for precapillary PH, respectively, while 31.3% exhibited severe PH. Correlations between diffusing capacity for carbon monoxide (DLco)%, and forced vital capacity (FVC)%/DLco% ratio with PVR were weak. Our findings highlight the heterogeneous hemodynamic landscape of ILD-PH in clinical practice and underscore the need for heightened vigilance and lower thresholds for RHC. These real-world data can inform future clinical trial design, screening strategies, and management decisions for ILD-PH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70108"},"PeriodicalIF":2.2,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12263507/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144650304","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Sex Differences Among Patients Treated With Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension. 球囊肺动脉成形术治疗慢性血栓栓塞性肺动脉高压患者的性别差异
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2025-07-15 eCollection Date: 2025-07-01 DOI: 10.1002/pul2.70128
Cze Ci Chan, Bárbara Lacerda Teixeira, Jenny Z Yang, David S Poch, Lawrence Ang, Angela Bautista, Ehtisham Mahmud, Nick H Kim
{"title":"Sex Differences Among Patients Treated With Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension.","authors":"Cze Ci Chan, Bárbara Lacerda Teixeira, Jenny Z Yang, David S Poch, Lawrence Ang, Angela Bautista, Ehtisham Mahmud, Nick H Kim","doi":"10.1002/pul2.70128","DOIUrl":"10.1002/pul2.70128","url":null,"abstract":"<p><p>Studies have shown sex differences in the outcomes of chronic thromboembolic pulmonary hypertension (CTEPH) and in patients who have undergone pulmonary endarterectomy. Limited data exist regarding outcomes of balloon pulmonary angioplasty (BPA) for female versus male subjects. In this analysis cohort, a total of 767 sessions of BPA were performed on 144 patients of which 83 (58%) were female. There were no differences in age, body mass index and comorbidities between males and females. However, females had worse baseline functional class, higher median N-terminal pro-brain natriuretic peptide and a shorter 6-min walk test. At baseline, males were more likely to be on pulmonary hypertension (PH) medical treatment before BPA (92% vs 76%, <i>p</i> = 0.008), and females had significantly higher pulmonary vascular resistance (PVR) (5.5 ± 3.0 wu vs 3.9 ± 1.9 wu, <i>p</i> < 0.001). After BPA, the improvements in mean pulmonary artery pressure and PVR from baseline were comparable in both groups. The requirement for PH medical therapy significantly reduced after BPA for males (92% vs. 80%, <i>p</i> = 0.008) but not for females. Functional class improved in both groups after BPA. There were no significant differences in complication rates per BPA session between male and female patients (9% and 12%, <i>p</i> = 0.104). Female CTEPH patients had a worse baseline clinical profile including higher PVR. Female patients were also more likely to remain on PH medical therapy after BPA. There were no significant differences observed in hemodynamic response to BPA and in complication rates between male and female patients.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70128"},"PeriodicalIF":2.2,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12263098/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144643275","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Randomized, Placebo-Controlled Trial of Peri-Operative Treprostinil in Pediatric Patients Undergoing the Fontan Operation. 小儿Fontan手术患者围手术期treprostiil的随机、安慰剂对照试验。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2025-07-15 eCollection Date: 2025-07-01 DOI: 10.1002/pul2.70122
Rachel T Sullivan, Stephanie S Handler, Alisa A Arunamata, Michelle T Ogawa, Esther Liu, Di Lu, Elisabeth Martin, Chandra Ramamoorthy, Rebecca Kameny, Jeffrey A Feinstein
{"title":"Randomized, Placebo-Controlled Trial of Peri-Operative Treprostinil in Pediatric Patients Undergoing the Fontan Operation.","authors":"Rachel T Sullivan, Stephanie S Handler, Alisa A Arunamata, Michelle T Ogawa, Esther Liu, Di Lu, Elisabeth Martin, Chandra Ramamoorthy, Rebecca Kameny, Jeffrey A Feinstein","doi":"10.1002/pul2.70122","DOIUrl":"https://doi.org/10.1002/pul2.70122","url":null,"abstract":"<p><p>The objective of this study is to assess the effect of subcutaneous treprostinil (TRE) administered peri-operatively after Fontan operation on chest tube duration (CTD), hospital length of stay (LOS), and post-operative hemodynamics. This is a single center randomized, blinded, placebo-controlled study of pediatric patients with single ventricle congenital heart disease undergoing Fontan operation between September 2015 and September 2019. Patients were randomized to receive subcutaneous TRE (target dose 10 ng/kg/min) or saline placebo starting intraoperatively through post-operative day 7. Baseline demographics, pre-operative hemodynamics, and peri-operative clinical details were collected. The primary outcome was CTD. Secondary outcomes included hospital LOS and hemodynamics. Thirty-four patients were randomized, 16 to TRE and 18 to saline placebo. Baseline characteristics were similar between groups, including pre-operative hemodynamics. Patients receiving TRE had increased risk for longer CTD (median CTD 8 vs. 7 days compared to placebo [IQR: 7-12.5, 5-8 days, respectively] with a relative risk (RR) of 1.29 (95% CI: 1.02, 1.64; <i>p</i> = 0.03)) and increased risk for longer hospital LOS (median LOS 11 vs. 9 days compared to placebo [IQR: 9.5-14.5, 8-10 days, respectively] with a RR of 1.23 (95% CI: 1.00, 1.51; <i>p</i> = 0.05)). Patients receiving TRE had higher median Fontan pressure at post-operative hour 12 (13 mmHg [IQR: 12.5-15.0] vs. 10.5 mmHg [IQR: 8.0-12.5]; <i>p</i> < 0.01 on repeated measure analysis model) and greater transpulmonary gradient at post-operative hour 12 (7.0 mmHg [IQR: 5.0-9.5] vs. 4.0 mmHg [IQR: 3.5-5.0]; <i>p</i> < 0.01 on repeated measure analysis model) compared to placebo. Peri-operative subcutaneous TRE did not reduce CTD or hospital LOS after Fontan operation and did not exert significant beneficial effects on post-operative hemodynamics. Accordingly, TRE at a dose of 10 ng/kg/min is not recommended for routine use in immediate post-operative Fontan management.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70122"},"PeriodicalIF":2.2,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12263512/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144650305","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Enhancing Drug Development for Paediatric Pulmonary Hypertension-An Integrative Perspective. 加强小儿肺动脉高压药物开发:综合视角。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2025-07-15 eCollection Date: 2025-07-01 DOI: 10.1002/pul2.70126
Steven H Abman, Sylvia M Nikkho, Rolf M F Berger, Maria Jesus Del Cerro, Eric D Austin, Maurice Beghetti, Dunbar Ivy, Megan Griffiths, Anne Hilgendorff, Steven H Kawut, Usha S Krishnan, Mary P Mullen, Shahin Moledina, Bernard Thébaud, Norman Stockbridge
{"title":"Enhancing Drug Development for Paediatric Pulmonary Hypertension-An Integrative Perspective.","authors":"Steven H Abman, Sylvia M Nikkho, Rolf M F Berger, Maria Jesus Del Cerro, Eric D Austin, Maurice Beghetti, Dunbar Ivy, Megan Griffiths, Anne Hilgendorff, Steven H Kawut, Usha S Krishnan, Mary P Mullen, Shahin Moledina, Bernard Thébaud, Norman Stockbridge","doi":"10.1002/pul2.70126","DOIUrl":"https://doi.org/10.1002/pul2.70126","url":null,"abstract":"<p><p>As with adult pulmonary hypertension (PH), high morbidity and mortality persist with diverse types of paediatric PH. Despite major advances in pharmacologic therapies based on extensive studies in adult PH, few drugs have been comprehensively studied in neonates, infants, and children, leaving current paediatric PH care largely dependent on small observational studies and extrapolation of evidence from adult clinical trials. Challenges in developing successful clinical trials in children include the need to define distinct disease phenotypes with well-characterised natural history and outcomes, the lack of established age- and disease-specific study endpoints, small and heterogeneous paediatric populations, and the common off-label use of PH-targeted drug therapies without regulatory approval. From a regulatory perspective, sufficient studies of safety, pharmacokinetics, and pharmacodynamics in neonates and young children are often lacking, and the potential role for bridging biomarkers has been underexplored. Additional opportunities include developing innovative trial designs, employing real-world data from existing registries, and fostering collaborations among sponsors, regulatory authorities, physicians, patients, and their families. By reducing reliance on off-label drug use and leveraging paediatric PH registry data, this approach offers a path toward more effective and evidence-based treatment protocols for paediatric patients. This review provides an overview of integrated international perspectives from an interprofessional platform that includes academia, the pharmaceutical industry, and regulatory agencies surrounding the future design of clinical trials for paediatric PH. Ongoing evaluation and adaptation of these strategies will be essential for ensuring that paediatric PH patients receive the highest standard of care.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70126"},"PeriodicalIF":2.2,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12263513/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144650303","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Pulmonary Hypertension Global Patient Survey: A Call to Action 2025. 肺动脉高压全球患者调查:行动呼吁2025。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2025-07-13 eCollection Date: 2025-07-01 DOI: 10.1002/pul2.70130
Joseph Newman, Hakim Ghani, Shiv Munagala, Eva Otter, Gerald Fischer, Marcin Kurzyna, Gergely Meszaros, Millicent Stone, Lynsay MacDonald, Shahin Moledina, Luke Howard, Wendy Gin-Sing, Pisana Ferrari, Maurice Beghetti, Katherine Bunclark, Mark Toshner, Matt Granato, Joanna Pepke-Zaba
{"title":"Pulmonary Hypertension Global Patient Survey: A Call to Action 2025.","authors":"Joseph Newman, Hakim Ghani, Shiv Munagala, Eva Otter, Gerald Fischer, Marcin Kurzyna, Gergely Meszaros, Millicent Stone, Lynsay MacDonald, Shahin Moledina, Luke Howard, Wendy Gin-Sing, Pisana Ferrari, Maurice Beghetti, Katherine Bunclark, Mark Toshner, Matt Granato, Joanna Pepke-Zaba","doi":"10.1002/pul2.70130","DOIUrl":"10.1002/pul2.70130","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70130"},"PeriodicalIF":2.2,"publicationDate":"2025-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12256269/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144637841","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Patient With Pulmonary Hypertension Carrying FLNA Loss-of-Function Variant. 携带FLNA功能丧失变异的肺动脉高压患者。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2025-07-09 eCollection Date: 2025-07-01 DOI: 10.1002/pul2.70110
Zongye Cai, Shuangxiang Lin, Nan Jin, Yahong Fu, Ying Zhang, Jifang Cheng, Yue Mao, Yuanshi Li, David Montani, Jun Jiang
{"title":"A Patient With Pulmonary Hypertension Carrying <i>FLNA</i> Loss-of-Function Variant.","authors":"Zongye Cai, Shuangxiang Lin, Nan Jin, Yahong Fu, Ying Zhang, Jifang Cheng, Yue Mao, Yuanshi Li, David Montani, Jun Jiang","doi":"10.1002/pul2.70110","DOIUrl":"10.1002/pul2.70110","url":null,"abstract":"<p><p>In a recent study conducted by Laura Stourm et al., the authors identified that patients with pulmonary hypertension (PH) harboring loss-of-function variants in the <i>Filamin A (FLNA)</i> gene exhibit a unique spectrum of phenotypes. These include pulmonary involvement with lung parenchymal abnormalities and emphysema, as well as a range of extrapulmonary manifestations such as dysmorphic facial features, epilepsy, congenital heart defects, valvular and aortic diseases, thrombocytopenia, and periventricular nodular heterotopia (PVNH). Based on these findings, the study advocates for <i>FLNA</i> genetic screening in patients with PH who present with these specific phenotypic features. Here, we describe a clinical case that aligns closely with the observations reported in their study.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70110"},"PeriodicalIF":2.2,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12240676/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144609172","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
2025 SELECT ABSTRACTS FROM THE 18th ANNUAL INTERNATIONAL CONFERENCE ON NEONATAL AND CHILDHOOD PULMONARY VASCULAR DISEASE. 2025选自第18届新生儿和儿童肺血管疾病国际年会的摘要。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2025-07-08 eCollection Date: 2025-07-01 DOI: 10.1002/pul2.70114
{"title":"2025 SELECT ABSTRACTS FROM THE 18<sup>th</sup> ANNUAL INTERNATIONAL CONFERENCE ON NEONATAL AND CHILDHOOD PULMONARY VASCULAR DISEASE.","authors":"","doi":"10.1002/pul2.70114","DOIUrl":"https://doi.org/10.1002/pul2.70114","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70114"},"PeriodicalIF":2.2,"publicationDate":"2025-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12237822/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144601360","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Survival Outcomes and Impact of Targeted PAH Therapy in Portopulmonary Hypertension in the PVRI GoDeep Meta-Registry. PVRI GoDeep Meta-Registry中靶向PAH治疗门脉性肺动脉高压的生存结局和影响
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2025-07-08 eCollection Date: 2025-07-01 DOI: 10.1002/pul2.70121
Arun Jose, Athiththan Yogeswaran, Meike Fuenderich, David Kiely, Andrew J Sweatt, Roham T Zamanian, Paul M Hassoun, Antoine Mouawad, Aparna Balasubramanian, Martin Wilkins, Allan Lawrie, Luke Howard, Sandeep Sahay, Horst Olschewski, Gabor Kovacs, Khaled Saleh, Hani Sabbour, Christina A Eichstaedt, Ekkehard Grünig, George Giannakoulas, Alexandra Arvanitaki, Yuriy Sirenko, Olena Torbas, Hector Cajigas, Robert Frantz, Laura Scelsi, Stefano Ghio, Raphael W Majeed, Jochen Wilhelm, Hossein Ardeschir Ghofrani, Friedrich Grimminger, Khodr Tello, Jean Elwing, Werner Seeger
{"title":"Survival Outcomes and Impact of Targeted PAH Therapy in Portopulmonary Hypertension in the PVRI GoDeep Meta-Registry.","authors":"Arun Jose, Athiththan Yogeswaran, Meike Fuenderich, David Kiely, Andrew J Sweatt, Roham T Zamanian, Paul M Hassoun, Antoine Mouawad, Aparna Balasubramanian, Martin Wilkins, Allan Lawrie, Luke Howard, Sandeep Sahay, Horst Olschewski, Gabor Kovacs, Khaled Saleh, Hani Sabbour, Christina A Eichstaedt, Ekkehard Grünig, George Giannakoulas, Alexandra Arvanitaki, Yuriy Sirenko, Olena Torbas, Hector Cajigas, Robert Frantz, Laura Scelsi, Stefano Ghio, Raphael W Majeed, Jochen Wilhelm, Hossein Ardeschir Ghofrani, Friedrich Grimminger, Khodr Tello, Jean Elwing, Werner Seeger","doi":"10.1002/pul2.70121","DOIUrl":"10.1002/pul2.70121","url":null,"abstract":"<p><p>Portopulmonary hypertension (PoPH), a type of pulmonary arterial hypertension (PAH) in patients with liver disease, is associated with high morbidity and mortality. The relationship between cardiopulmonary hemodynamics, PAH therapy, and survival in PoPH remains unclear. We performed a retrospective cohort study of PoPH patients from the international pulmonary hypertension (PH) meta-registry, PVRI GoDeep. PAH was defined by a mean pulmonary arterial pressure > 20 mmHg, pulmonary arterial wedge pressure ≤ 15 mmHg, and a pulmonary vascular resistance (PVR) > 2 Wood Units. PoPH diagnoses were assigned by each center's PH specialist based on international guidelines at the time of enrollment. 246 incident PoPH patients met eligibility criteria and were included in the analysis, equally split between males (51%) and females (49%), with a median age of 54 years. When compared to both patients with IPAH and those with other subtypes of PAH (not classified as PoPH or IPAH), those with PoPH had significantly lower 5-year survival rates (46% vs. 68% vs. 65%, log-rank <i>p</i> < 0.001). Amongst the PoPH patients, however, there was no significant difference in 5-year survival when dichotomized by disease severity, either by a PVR of 5 Wood Units or a CI of 2.5 L/min/m<sup>2</sup>. Treatment of the PoPH patients with PAH-targeted therapies was associated with significantly higher 5-year survival rates compared to those not receiving such treatments, as shown by Kaplan-Meier analysis. This survival benefit was observed for PDE5i (50% vs. 34%, log-rank <i>p</i> = 0.029), ERA (58% vs. 34%, log-rank <i>p</i> < 0.001), and the combination of PDE5i and/or ERA (51% vs. 22%, log-rank <i>p</i> < 0.001), as well as any PAH-targeting treatment (50% vs. 26%, log-rank <i>p</i> = 0.007). Corresponding survival advantage was noted when including only PoPH patients with MELD Score ≥ 13. PoPH is a disease with significantly worse long-term survival than other PAH subtypes, but targeted PAH therapy is associated with a robust survival benefit. Survival did not differ across high-risk PVR and cardiac index thresholds, suggesting the factors that influence prognosis and survival in PoPH may be unique as compared to other PAH subtypes, and warrant further investigation.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70121"},"PeriodicalIF":2.2,"publicationDate":"2025-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12237829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144601361","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Practical Considerations for Managing Patients on Tyvaso DPI (Treprostinil Inhalation Powder). 替瓦索DPI(曲前列地尼吸入粉)患者管理的实际考虑。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2025-07-03 eCollection Date: 2025-07-01 DOI: 10.1002/pul2.70119
Jennifer H Keeley, Jacqueline Skarre, Lori Reed, Loida A Johnson, Catherine Falardeau, Brittany N J Davis, Manisit Das, Howard Castillo, Susanne McDevitt
{"title":"Practical Considerations for Managing Patients on Tyvaso DPI (Treprostinil Inhalation Powder).","authors":"Jennifer H Keeley, Jacqueline Skarre, Lori Reed, Loida A Johnson, Catherine Falardeau, Brittany N J Davis, Manisit Das, Howard Castillo, Susanne McDevitt","doi":"10.1002/pul2.70119","DOIUrl":"10.1002/pul2.70119","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease (PH-ILD) require a multifaceted, guideline-directed management approach. This includes active patient participation in partnership with their healthcare team to minimize disease impact and improve survival. Inhaled treprostinil, a prostacyclin analog, has been approved for both PAH and PH-ILD in two formulations: Tyvaso (treprostinil nebulizer) and the more recent Tyvaso DPI (treprostinil dry powder inhaler) [United Therapeutics]. Both formulations deliver therapy directly to the lung vasculature, minimizing the risk of ventilation-perfusion mismatch, reducing systemic exposure, and decreasing the incidence of adverse events commonly associated with parenteral and oral prostacyclin formulations. While practical recommendations for the treprostinil nebulizer have been previously published, Tyvaso DPI provides a well-tolerated, convenient administration option for patients requiring prostacyclin therapy. This review provides an overview of inhaled prostacyclin therapy with a focus on practical considerations for managing PAH and PH-ILD patients treated with Tyvaso DPI. Recommendations from a panel of pulmonary hypertension advanced practice providers include patient selection, education, communication, onboarding and monitoring, transition and titration, side effect mitigation, and the availability of clinician- and patient-facing resources.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70119"},"PeriodicalIF":2.2,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12224286/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144560964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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