Pulmonary Circulation最新文献

{"title":"Automated Bi-Ventricular Segmentation and Regional Cardiac Wall Motion Analysis for Rat Models of Pulmonary Hypertension.","authors":"Marili Niglas, Nicoleta Baxan, Ali Ashek, Lin Zhao, Jinming Duan, Declan O'Regan, Timothy J W Dawes, Chen Nien-Chen, Chongyang Xie, Wenjia Bai, Lan Zhao","doi":"10.1002/pul2.70092","DOIUrl":"10.1002/pul2.70092","url":null,"abstract":"<p><p>Artificial intelligence-based cardiac motion mapping offers predictive insights into pulmonary hypertension (PH) disease progression and its impact on the heart. We proposed an automated deep learning pipeline for bi-ventricular segmentation and 3D wall motion analysis in PH rodent models for bridging the clinical developments. A data set of 163 short-axis cine cardiac magnetic resonance scans were collected longitudinally from monocrotaline (MCT) and Sugen-hypoxia (SuHx) PH rats and used for training a fully convolutional network for automated segmentation. The model produced an accurate annotation in < 1 s for each scan (Dice metric > 0.92). High-resolution atlas fitting was performed to produce 3D cardiac mesh models and calculate the regional wall motion between end-diastole and end-systole. Prominent right ventricular hypokinesia was observed in PH rats (-37.7% ± 12.2 MCT; -38.6% ± 6.9 SuHx) compared to healthy controls, attributed primarily to the loss in basal longitudinal and apical radial motion. This automated bi-ventricular rat-specific pipeline provided an efficient and novel translational tool for rodent studies in alignment with clinical cardiac imaging AI developments.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70092"},"PeriodicalIF":2.2,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12067408/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143977297","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":"Endothelial Features Along the Pulmonary Vascular Tree in Chronic Thromboembolic Pulmonary Hypertension: Distinctive or Shared Facets?","authors":"Janne Verhaegen, Lynn Willems, Allard Wagenaar, Ruben Spreuwers, Nessrine Dahdah, Lucia Aversa, Tom Verbelen, Marion Delcroix, Rozenn Quarck","doi":"10.1002/pul2.70096","DOIUrl":"10.1002/pul2.70096","url":null,"abstract":"<p><p>Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of pulmonary embolism, characterized by the presence of organized fibro-thrombotic material that partially or fully obstructs the lumen of large pulmonary arteries, microvasculopathy, and enlargement of the bronchial systemic vessels. The precise mechanisms underlying CTEPH remain unclear. However, defective angiogenesis and altered pulmonary arterial endothelial cell (PAEC) function may contribute to disease progression. Despite the observation of differences in histological features, shear stress and ischemia along the pulmonary vascular tree, the potential contribution of PAEC phenotype and function to these disparate aspects remains unexplored. Based on these observations, we postulated that angiogenic capacities and endothelial barrier function may contribute to disparities in histological features observed along the pulmonary vascular tree. We thus explored the histological characteristics of the pulmonary vascular tree using pulmonary arterial lesions obtained during pulmonary endarterectomy (PEA). We focused on the angiogenic vascular endothelial growth factor (VEGF)-A/VEGF receptor-2 (VEGFR2) axis and collagen 15A1 (COL15A1), a potential marker of endothelial cells of the systemic circulation. Concurrently, we examined In Vitro angiogenic properties and barrier function of PAECs derived from large and (sub)-segmental pulmonary arterial lesions. (Sub)-segmental pulmonary arterial lesions were abundantly recanalized by neovessels, paralleled by an enriched expression of VEGFR2. VEGF-A expression was more pronounced in large pulmonary arterial lesions. Nevertheless, no significant difference was discerned in In Vitro angiogenic capacities and barrier integrity of PAECs isolated from large and (sub)-segmental pulmonary arterial lesions. Importantly, our findings revealed the presence of endothelial cells (CD31⁺) expressing COL15A1, as well as CD31⁺ cells that did not express COL15A1. This suggests that endothelial cells from both systemic and pulmonary circulation contribute to lesion recanalization. Despite disparate in situ angiogenic cues in VEGF-A/VEGFR2 axis between large and (sub)-segmental pulmonary arterial lesions in CTEPH, In Vitro angiogenic capacities and barrier function remain unaltered.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70096"},"PeriodicalIF":2.2,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12067398/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143993768","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":"Inhibiting Serotonin Synthesis for the Treatment of Pulmonary Arterial Hypertension.","authors":"Georg Hansmann, Michael Bader","doi":"10.1002/pul2.70100","DOIUrl":"10.1002/pul2.70100","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70100"},"PeriodicalIF":2.2,"publicationDate":"2025-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12059275/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144014657","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":"Role of MiR-542-3p/Integrin-Linked Kinase/Myocardin Signaling Axis in Hypoxic Pulmonary Hypertension.","authors":"Linqing Li, Weining Zhou, Qingrong Ji, Xianzhao Zhang, Ni Yang, Kaiyou Song, Shunpeng Hu, Cunfei Liu, Zhihong Ou, Fengwei Zhang, Yuda Wei, Jiantong Hou","doi":"10.1002/pul2.70094","DOIUrl":"10.1002/pul2.70094","url":null,"abstract":"<p><p>Phenotypic transition of pulmonary artery smooth muscle cells (PASMCs) under hypoxic conditions, which in turn causes increased proliferation and migration capacity, is an important pathological process in Hypoxic pulmonary hypertension (HPH). Although research on the phenotypic transition of PASMCs has been ongoing, little is known about the specific molecular mechanisms underlying this process. Integrin-linked kinase (ILK) is one of the genes essential for maintaining the contractile phenotype of vascular smooth muscle cells (VSMCs). It has been shown that ILK is a target gene of MiR-542-3p, and overexpression of MiR-542-3p can promote apoptosis of osteosarcoma cells by downregulating the expression of ILK, and inhibit their cell proliferation, migration, and invasion. In this study we found that hypoxia upregulated MiR-542-3p expression, and MiR-542-3p mimics reduced ILK, Myocardin expression, and promote phenotypic transition in PASMCs. And, ILK was a direct target of MiR-542-3p in PASMCs. MiR-542-3p inhibitor reversed hypoxia-induced reduction of ILK and Myocardin expression in PASMCs, and phenotypic transition, proliferation, and migration of PASMCs. MiR-542-3p antagomir reversed hypoxia-induced pulmonary vascular remodeling and also reversed hypoxia-induced reduction in ILK, Myocardin expression, and phenotype transition in rat pulmonary arteries. Thus, our results suggest that hypoxia induced an increase in MiR-542-3p expression, which caused an increase in binding to ILK gene and negatively regulated ILK expression. This in turn, caused a decrease in Myocardin expression leading to phenotypic transition, proliferation, and increased migration of PASMCs, causing hypoxic pulmonary vascular remodeling and ultimately leading to HPH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70094"},"PeriodicalIF":2.2,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12053744/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144064445","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":"Activin-A Regulates Bone Morphogenetic Protein Signaling in Pulmonary Endothelial Cells Without Affecting Bone Morphogenetic Protein Type-II Receptor Expression.","authors":"Benjamin J Dunmore, Nobuhiro Kikuchi, Wei Li, Paul D Upton, Nicholas W Morrell","doi":"10.1002/pul2.70095","DOIUrl":"10.1002/pul2.70095","url":null,"abstract":"<p><p>Activin-A is elevated in pulmonary arterial hypertension (PAH) patients, and reportedly suppresses BMPR-II. This suggests one mechanism of action for PAH drug, sotatercept, an activin-ligand trap. However, we were unable to confirm that activin-A reduces BMPR-II in pulmonary endothelial cells. Thus, it seems unlikely that sotatercept influences BMPR-II or PAH via this mechanism.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70095"},"PeriodicalIF":2.2,"publicationDate":"2025-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12052755/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144053880","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":"PVRI International Conference 2025: Embracing Heterogeneity.","authors":"Navneet Singh, Katarina Zeder","doi":"10.1002/pul2.70093","DOIUrl":"10.1002/pul2.70093","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70093"},"PeriodicalIF":2.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12045786/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143996294","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 Artery Stent Implantation for Fibrosing Mediastinitis: Our Clinical Experience.","authors":"Cheng Hong, Daibing Zhou, Haiming Chen, Xiaofeng Wu, Wenliang Guo, Jiangyu Cui, Weijie Guan, Nanshan Zhong, Jielong Lin","doi":"10.1002/pul2.70076","DOIUrl":"10.1002/pul2.70076","url":null,"abstract":"<p><p>Fibrosing mediastinitis (FM) can block pulmonary vessels and airways, hindering treatment efficacy. Pulmonary artery (PA) stenting might provide a solution in such cases. This study involved 30 patients who had 49 PA stenting procedures for FM. Data on baseline characteristics, CT pulmonary angiography images, stent patency, and hemodynamics were collected. Patients with FM often had a history of chronic obstructive pulmonary disease (15/30), tuberculosis (12/30), and pneumoconiosis (11/30). Patients exhibited typical symptoms such as dyspnea, exercise intolerance, and cough. FM appeared as multiple bilateral shadows with enlarged hilar and mediastinal lymph nodes. Our study found that the PA involvement alone was predominantly in the left and right lower basilar trunk, with the left lower pulmonary arteries (LLPA) involved in 80% of cases and the right lower pulmonary arteries (RLPA) in 100%. Moreover, over 2/3 of patients showed involvement of both PA and pulmonary vein (PV), mainly in the bilateral upper lung lobes, then in the right middle lobe and left lingual lobe. After PA stent implantation, patients showed enhanced tricuspid annular plane systolic excursion (20.6 vs. 18.5, <i>p</i> < 0.001) and reduced right atrial diameter (35.5 vs. 37.3, <i>p</i> = 0.042), along with significant gains in 6-min walk distance (465.2 vs. 392.7, <i>p</i> = 0.002) and improved World Health Organization functional class (<i>p</i> < 0.001). Hemodynamic parameters improved after PA stent placement with significant reductions in systolic pulmonary artery pressure (PAP) (51.1 vs. 64.2, <i>p</i> < 0.001), mean PAP (28.4 vs. 35.2, <i>p</i> < 0.001), pulmonary vascular resistance (4.7 vs. 5.9, <i>p</i> = 0.004), and stent gradient (11.2 vs. 33.4, <i>p</i> < 0.001), along with increased patency (84.8% vs. 28%, <i>p</i> < 0.001), and fractional flow reserve (0.84 vs. 0.44, <i>p</i> < 0.001). Over a median follow-up of 331 days (range 45-980), no significant stent stenosis occurred (<i>p</i> = 0.287). Mild adverse events like cough and mild hemoptysis were noted during the procedure. Secondary intervention was needed for 5 of 49 stents. PA stents placement, especially the LLPA and RLPA, improved pulmonary vascular patency, hemodynamics, and symptoms.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70076"},"PeriodicalIF":2.2,"publicationDate":"2025-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12040597/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144029307","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":"The Heterogeneity of Pulmonary Hypertension Nomenclature in Empiric Research Studies: Systematic Findings From Three Western European Countries.","authors":"Armella Santi, Veranyuy Ngah, Eric W Robbins, Bradley A Maron, Katarina Zeder","doi":"10.1002/pul2.70089","DOIUrl":"https://doi.org/10.1002/pul2.70089","url":null,"abstract":"<p><p>This systematic literature review of three Western European Countries identified <i>N</i> = 48 different terms to describe pulmonary arterial pressure and <i>N</i> = 35 thresholds used to define pulmonary hypertension in published empiric research studies. There is an urgent need to standardize pulmonary artery pressure nomenclature and pulmonary hypertension definitions in clinical research reports.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70089"},"PeriodicalIF":2.2,"publicationDate":"2025-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12037687/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144050765","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":"Long-Term Treatment With Subcutaneous Treprostinil in Patients With Severe Inoperable Chronic Thromboembolic Pulmonary Hypertension in the Multimodal Therapy Era (Data From CTREPH Study Open Label Extension).","authors":"Pavel Jansa, Roela Sadushi-Kolici, Nika Skoro-Sajer, Grzegorz Kopec, Iveta Simkova, Regina Steringer-Mascherbauer, Barbara Salobir, Jaroslav Lindner, Irene M Lang","doi":"10.1002/pul2.70080","DOIUrl":"10.1002/pul2.70080","url":null,"abstract":"<p><p>The aim of the open label extension (OLE) of CTREPH study was to characterize multimodal treatment in patients with severe inoperable CTEPH, to describe long-term subcutaneous (SC) treprostinil safety and tolerability, and to evaluate change in functional class and exercise capacity over 24 months since completion of the blinded phase of CTREPH. The target population in the OLE consisted of patients who completed 24 weeks of blinded treatment with either high-dose treprostinil of around 30 ng/kg/min (former high-dose group), or low-dose treprostinil of around 3 ng/kg/min (former low-dose group) in the CTREPH study. From the start of OLE, treprostinil dose and any additional therapy were chosen according to the standard of care and physician's discretion. Out of 47 enrolled patients, 20 patients received other PH drugs during OLE and 17 patients underwent at least 1 BPA session. Number of treprostinil-related AEs was substantially higher in the former low-dose group in comparison to the former high-dose group. Related AEs were also more frequent during the first 6 months of the preceding blinded trial than over 24 months of OLE, especially infusion site pain and all local infusion site reactions. No new safety signal was detected. Evaluated clinical outcomes show sustained benefit from long-term treprostinil treatment. Long-term SC treprostinil is a safe and effective component of multimodal treatment for patients with severe CTEPH. Patients who tolerate treprostinil after initiation are likely to continue tolerating it over time, with the clinical benefit maintained over 24 months.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70080"},"PeriodicalIF":2.2,"publicationDate":"2025-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12034262/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144053882","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":"Morbidity and Mortality Associated With Pulmonary Arterial Hypertension in a Schistosomiasis-Endemic Region of Brazil.","authors":"Ricardo Amorim Correa, Camila Farnese Rezende, Eliane Viana Mancuzo, Claudia Mickael, Camila M C Loureiro, Rudolf K F Oliveira, Joan F Hilton, Brian B Graham","doi":"10.1002/pul2.70086","DOIUrl":"10.1002/pul2.70086","url":null,"abstract":"<p><p>Data about pulmonary arterial hypertension (PAH) patients living in low- and middle-income countries remain scarce. This study assessed prognostic factors associated with time to clinical worsening (CW) or death of a cohort of PAH patients in Minas Gerais, Brazil. This retrospective cohort study describes baseline clinical variables by PAH etiology and estimates time from diagnosis to CW [all-cause death, any-cause hospitalization, or disease progression (decrease of ≥ 15% in 6MWD and need for additional PAH therapy or worsening of functional class (FC)] and time to death. 79 out of 102 participants developed CW and 38 died while under follow-up. The most prevalent etiologies were PAH associated with schistosomiasis (PAH-Sch), idiopathic (IPAH), with congenital heart disease (PAH-CHD), and with connective tissue disease (PAH-CTD). The overall median event-free time to CW was 3.3 (95% CI, 2.3-4.6) years, which was similar across etiologies (log-rank test: <i>p</i> = 0.12). WHO FC III-IV, DLCO < 70%, heart rate recovery in 1 min after the 6-min walk test (HRR1) < 18 beats/minute, and baseline mPAP ≥ 50 mmHg were predictive of CW-free time. The median time to all-cause mortality was 10.2 (95% CI, 6.8 - > 10) years and varied among etiologies (log-rank test: <i>p</i> < 0.001). Time to CW was statistically independent of PAH etiology but depended on baseline WHO FC, DLCO, HRR, and mPAP. After CW events, PAH-Sch and PAH-CTD survived less on average than IPAH and PAH-CHD participants.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70086"},"PeriodicalIF":2.2,"publicationDate":"2025-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12034267/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144009726","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}