Pulmonary Circulation最新文献

{"title":"Arteriovenous Oxygen Content Difference: A Diagnostic Predictor for Preselecting Invasive Treatment in Congenital Heart Disease-Related Pulmonary Arterial Hypertension.","authors":"Ashfaq Ahmad, Songlin Zhang, Lingling Li, Xiaoyu Wang, Yajuan Du, Ting Liu, Fenling Fan","doi":"10.1002/pul2.70091","DOIUrl":"10.1002/pul2.70091","url":null,"abstract":"<p><p>Patients with congenital heart disease-related pulmonary arterial hypertension (CHD-PAH) often require regular follow-up through invasive right heart catheterization (RHC) to assess disease progression and potential interventions. This study aims to evaluate the relationship between arteriovenous oxygen content difference (Ca-vO₂) and RHC parameters to identify blood gas parameters that can aid in a clue about preselecting patients with CHD-PAH for follow-up RHC and potential surgical or percutaneous shunt closure. In this study, a total of 137 adult CHD-PAH patients were retrospectively enrolled between September 2019 and May 2024. The patients were divided into two groups based on their Qp/Qs ratio (< 1.5 or ≥ 1.5). Key parameters such as Ca-vO₂, 6-min walk distance (6MWD), TAPSE, and IVC diameter were correlated with RHC parameters such as mean pulmonary artery pressure (mPAP), pulmonary vascular resistance (PVR), and pulmonary capillary wedge pressure (PCWP) and compared across groups with Qp/Qs < 1.5 and ≥ 1.5. Statistical analysis included Pearson's correlation, logistic regression analysis, and receiver operator characteristic (ROC) curve to determine the predictors for shunt severity. The study enrolled 80 patients with CHD-related PAH in the final evaluation, with a mean age of 41 ± 15 years. Ca-vO₂ exhibits a significant positive correlation with RHC parameters, notably with mPAP (<i>r</i> = 0.524, <i>p</i> < 0.0001) and a negative correlation with Qp/Qs (<i>r</i> = -0.463, <i>p</i> = 0.04). Moreover, Ca-vO₂ emerged as a significant diagnostic predictor with an optimal cutoff value of < 4.3 mmol/L (AUC = 0.71, sensitivity 88.8%, specificity 53.4%). Other noninvasive parameters such as 6MWD, TAPSE, and IVC diameter with AUCs of 0.87, 0.83, and 0.85, respectively, also demonstrated a strong predictive value. Ca-vO₂ correlates well with CHD-PAH severity and can serve as a preselecting marker for invasive follow-up in CHD-related PAH. Other noninvasive measures such as 6MWD, TAPSE, and IVC diameter show stronger predictive value for assessing shunt severity.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70091"},"PeriodicalIF":2.2,"publicationDate":"2025-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12099216/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144143290","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":"Prognosis and Treatment With Phosphodiesterase 5 Inhibitors in Combined Post- and Precapillary Pulmonary Hypertension: A Propensity Score-Matched Analysis From the Hellenic Pulmonary Hypertension Registry.","authors":"Georgios E Papadopoulos, Alexandra Arvanitaki, Sophia-Anastasia Mouratoglou, Panagiotis Gourgiotis, Thomas Chrysochoidis-Trantas, Athena Mpatsouli, Aris Bechlioulis, Aikaterini Naka, Eftychia Demerouti, Panagiotis Karyofyllis, Dimitrios Tsiapras, Anastasia Anthi, Athanasios Manginas, Antonios Ziakas, Stephan Rosenkranz, George Giannakoulas","doi":"10.1002/pul2.70099","DOIUrl":"https://doi.org/10.1002/pul2.70099","url":null,"abstract":"<p><p>Combined post- and precapillary pulmonary hypertension (CpcPH) comprises the most severe form of postcapillary PH. A severe precapillary component (pulmonary vascular resistance [PVR] > 5 WU) is critical for therapeutic decisions. Current treatment guidelines focus on optimizing underlying cardiac disease, while there are conflicting data regarding the efficacy and safety of pulmonary arterial hypertension (PAH) drugs in selected patients. This study examines the impact of PVR > 5 WU on survival in heart failure with preserved ejection fraction (HFpEF) and CpcPH and evaluates the effect of treatment with phosphodiesterase 5 inhibitors (PDE5is) on clinical and hemodynamic parameters and on prognosis. The Hellenic Pulmonary Hypertension Registry (HOPE) enrolls patients from all PH groups in Greece. This study focuses on Group 2 CpcPH patients with HFpEF. Propensity score matching was performed to reduce the risk of bias in the treatment selection and potential confounders. Kaplan-Meier curve was used to estimate 5-year survival, and the log-rank test was used for the comparisons. A total of 98 patients were included, with a median follow-up of 2.9 years. PVR > 5 WU and age were independently associated with worse survival ([HR 2.15, 95% CI 1.13-4.83, <i>p</i> = 0.04], [HR 1.07, 95% CI 1.03-1.13, <i>p</i> = 0.003], respectively). Propensity-matched cohort analysis indicated that PDE5i treatment was associated with a significant reduction in PVR at follow-up (from median [IQR] 4.89 [1.9] WU to 3.1 [2.0] WU, <i>p</i> = 0.04) and a trend towards improved survival. Severe precapillary component is associated with impaired prognosis in CpcPH. While PDE5i treatment shows promise in improving hemodynamic outcomes, its effect on long-term survival requires further investigation.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70099"},"PeriodicalIF":2.2,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12067402/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144031088","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":"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}