Pulmonary Circulation最新文献

{"title":"Digital Inclusion in a Scottish National Pulmonary Hypertension Population.","authors":"Jamie Ingram, Harrison Stubbs, Stephanie Lua, Melanie Brewis, Martin Johnson, Colin Church","doi":"10.1002/pul2.70135","DOIUrl":"10.1002/pul2.70135","url":null,"abstract":"<p><p>To evaluate current digital inclusion in the Scottish pulmonary hypertension population, a paper questionnaire was offered to the entirety of patients with pulmonary arterial hypertension in Scotland. The Scottish Index of Multiple Deprivation was used to stratify patients into deprivation deciles. 464 patients returned questionnaires (86%). 91% had reliable internet access. 89% had access to an internet-enabled device. 71% used the internet daily. The most common barriers to increased internet usage were confidence with technology (19%) and lack of perceived personal benefit (7%). 54% would like virtual healthcare to complement in person review and 58% would like to monitor their health digitally. Older patients were less likely to use the internet and had less desire for virtual healthcare. Rural living did not negatively impact access to the internet. Younger, more rural, and less deprived patients currently use and desire more online exercise. Deprived patients were less likely to have internet access or internet enabled devices, more likely to have no device or a mobile without internet, and had less desire for virtual healthcare or digital health monitoring. Most patients have the means of accessing the internet and support virtual healthcare in addition to direct clinician contact. However, digital engagement was lower in older and more deprived patients. The high response rate supports paper over online survey methodology for future digital inclusion research. Future digital healthcare strategies need to integrate this knowledge to minimize age- and deprivation-related inequity.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70135"},"PeriodicalIF":2.5,"publicationDate":"2025-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12296825/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144732967","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}

{"title":"Melatonin in Pulmonary Arterial Hypertension: A Promising Adjunctive Therapy.","authors":"Shaher Yar, Seyed R Rafieossadat, Taraneh Tadayon, Zahin Shahriar","doi":"10.1002/pul2.70139","DOIUrl":"10.1002/pul2.70139","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70139"},"PeriodicalIF":2.5,"publicationDate":"2025-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12286884/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144708579","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}

{"title":"Risk Assessment Models and Event-Free Survival in Pulmonary Arterial Hypertension.","authors":"Clara Hjalmarsson, Tanvee Thakur, Göran Rådegran, Erik Björklund, Håkan Wåhlander, Magnus Nisell, Joanna-Maria Papageorgiou, Stefan Söderberg, Dominik Lautsch, Barbro Kjellström","doi":"10.1002/pul2.70132","DOIUrl":"10.1002/pul2.70132","url":null,"abstract":"<p><p>Evidence on the predictive ability of risk assessment models for event-free survival (EFS) in patients with pulmonary arterial hypertension is scarce. We aimed to investigate the relationship between risk status at 6 months after diagnosis (6 M) and EFS, by three risk models: Multicomponent Improvement (MCI), ESC/ERS 4-Strata Risk (4SR), and noninvasive French PH Registry Score (FRS). Data collected in the Swedish PAH Registry 2008-2021 were used. The study population was risk-stratified at 6 M according to each model. Information on PAH-related hospitalization (HOSP) was collected from the National Patient Register. EFS was defined as survival without occurrence of: (1) HOSP; (2) initiation of parenteral prostacyclin therapy or dose increase ≥ 10%; (3) lung transplantation. The association between risk and EFS was evaluated by Kaplan-Meier estimates and Cox proportional models. The analysis included 411 incident patients, median age 66 y [50, 73]. Median survival time was 3.5 y [1.7; 5.4], and cumulative EFS was 55%. In a Cox proportional regression adjusted for age, eGFR, obesity, atrial fibrillation, and systemic hypertension, EFS was higher in patients who: (1) achieved two or three MCI criteria compared to one or no MCI criterion (HR 0.58; CI 0.39-0.84, <i>p</i> = 0.005); (2) were assessed as low, intermediate-low, or intermediate-high compared to high risk (HR 0.16; CI 0.09-0.28, <i>p</i> < 0.001); or (3) fulfilled one, two, or three low-risk FRS criteria, compared to no low-risk criterion (HR 0.29; CI 0.19-0.43, <i>p</i> < 0.001). Performing a risk assessment 6 months after diagnosis effectively predicts the likelihood of EFS in the studied population, highlighting its prognostic value.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70132"},"PeriodicalIF":2.2,"publicationDate":"2025-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12272513/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675538","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}

{"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":"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}

{"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":"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}

{"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}

{"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":"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}

{"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":"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}

{"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}

{"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}