Pulmonary Circulation最新文献

{"title":"Pathogenesis and Phenotypes of Pulmonary Vascular Disease in COPD: A Consensus Statement From the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative - PH Group 3 Workstream.","authors":"Lucilla Piccari, Katarina Zeder, Aparna Balasubramanian, Brian W Allwood, Stephen John Wort, Shelsey W Johnson, Reda E Girgis, Oksana A Shlobin, Steven H Abman, Gabor Kovacs, George Washko, Sylvia M Nikkho, Steven D Nathan","doi":"10.1002/pul2.70409","DOIUrl":"10.1002/pul2.70409","url":null,"abstract":"<p><p>Chronic obstructive pulmonary disease (COPD) is frequently associated with pulmonary vascular disease (PVD) yet pulmonary vascular involvement remains underrecognized and incompletely phenotyped. Emerging evidence demonstrates that pulmonary vasculopathy may precede overt emphysema and airflow obstruction, suggesting that vascular remodeling is an early and integral component of COPD pathobiology rather than solely a late consequence of advanced lung disease. Multiple mechanisms contribute to COPD-associated PVD, including genetic susceptibility, abnormal lung development, cigarette smoke and environmental exposures, endothelial dysfunction, hypoxia, inflammation, hyperinflation, and comorbid cardiovascular and infectious diseases, which are discussed in this paper. These processes lead to heterogeneous clinical phenotypes that span a continuum from early pulmonary vascular abnormalities without resting PH to severe hemodynamically defined PH. Conventional spirometry and resting hemodynamics incompletely capture this complexity, whereas advanced imaging, gas-exchange assessment, and exercise testing improve identification of pulmonary vascular involvement. Distinct pulmonary vascular phenotypes may carry important implications for exercise limitation, exacerbation risk, progression, and survival. Recognizing COPD-associated PVD as a continuum rather than a late-stage complication has major implications for screening, phenotyping, and clinical trial design. This article reflects deliberations of the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative (PVRI IDDI) Group 3 Pulmonary Hypertension Workstream on the pathophysiology and phenotypes of PVD in COPD.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70409"},"PeriodicalIF":2.4,"publicationDate":"2026-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13560002/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148945665","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":"Invasive Management for Acute-on-Chronic Thromboembolic Pulmonary Hypertension: Feasibility and Clinical Outcomes in a Single-Center Cohort.","authors":"Jun Yamashita, Ryosuke Ito, Sayo Ikeda, Koki Hasegawa, Yoshikazu Takeuchi, Tomohiro Kasahara, Naotaka Murata, Shun Suzuki, Hideaki Kanazawa, Yusuke Shimahara, Kazuhiro Satomi","doi":"10.1002/pul2.70407","DOIUrl":"10.1002/pul2.70407","url":null,"abstract":"<p><p>Acute-on-chronic thromboembolic pulmonary hypertension (acute-on-CTEPH) is associated with rapid clinical deterioration, yet the role of invasive intervention in the acute phase remains poorly defined. We retrospectively analyzed consecutive patients with acute-on-CTEPH presenting with severe hypoxemia and/or cardiogenic shock at a single center between 2023 and 2026. Acute-on-CTEPH was defined as acute clinical deterioration requiring ventilatory or circulatory support, including ECMO, with imaging evidence of chronic thromboembolic disease and superimposed acute thrombi. Invasive strategies included pulmonary endarterectomy (PEA) and/or balloon pulmonary angioplasty (BPA), selected based on multidisciplinary assessment. The primary outcome was change in pulmonary hemodynamics; secondary outcomes included acute-phase mortality and clinical recovery after stabilization. A total of 15 patients (median age 69 years; 53% male), all in World Health Organization functional class IV, were included. Mechanical ventilation and ECMO were required in 53% and 47% of patients, respectively. Initial treatment consisted of PEA (40%) or BPA (60%), with combined approaches in selected cases. Acute-phase mortality was 20%. Among survivors, mean pulmonary artery pressure decreased from 42 to 17 mmHg, pulmonary vascular resistance from 11.6 to 2.4 WU, and cardiac index increased from 1.7 to 2.7 L/min/m². Among patients requiring ECMO, 5 were successfully weaned and 4 survived to discharge. At follow-up, 11 of the 12 survivors were in WHO functional class I or II, and 8 (67%) had discontinued home oxygen therapy. Despite substantial acute-phase mortality, invasive strategies were associated with marked hemodynamic improvement and may represent a potential therapeutic option in this life-threatening clinical setting.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70407"},"PeriodicalIF":2.4,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13551590/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148913378","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 CT Pulmonary Angiography-Derived Periclot Radiomics for CTEPH Detection.","authors":"Pietro Nardelli, Rubén San José Estépar, Michael Cuttica, Ruben Mylvaganam, Farbod N Rahaghi, Raúl San José Estépar","doi":"10.1002/pul2.70406","DOIUrl":"10.1002/pul2.70406","url":null,"abstract":"<p><p>CTEPH remains underdiagnosed and can be difficult to differentiate on routine CTPA from acute PE and from patients without pulmonary thromboembolism. We developed and evaluated a fully automated CTPA-based machine learning algorithm combining vascular blood volume metrics with clot and periclot imaging features. In this retrospective multicenter study, intraparenchymal pulmonary vessels were segmented using a scale-space particle approach, deep learning models estimated vessel radius, clot probability, and clot area from cross-sectional patches along the vessel axis, and subject-level radiomic and blood volume features were used to train an XGBoost classifier on a Northwestern University (NU) cohort (89 CTEPH; 176 controls). The trained model was then applied (without retraining) to held-out NU data, an independent acute PE cohort (152 patients) as an exploratory generalization test, and an external cohort from Brigham and Women's Hospital (23 CTEPH; 34 controls). Among 474 participants (mean age, 51 years ± 16; 299 men), the model achieved an AUC of 0.87 (95% CI: 0.79, 0.94) for CTEPH versus controls in internal testing, 0.74 (95% CI: 0.61-0.86) in external testing, and 0.89 (95% CI 0.83-0.93) for CTEPH versus acute PE. Excluding periclot features reduced discrimination, particularly for CTEPH versus acute PE (AUC 0.71; 95% CI: 0.63, 0.79). These findings support the feasibility of automated CTPA-derived vascular, clot, and periclot features for differentiating CTEPH from acute PE and controls without evidence of pulmonary thromboembolism, with further validation needed to assess generalizability across institutions.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70406"},"PeriodicalIF":2.4,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13551595/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148913316","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":"Effect of Incident Pulmonary Arterial Hypertension on Risk of Newly-Diagnosed Mental Health Conditions.","authors":"Anna Watzker, Oscar Patterson-Lomba, Cameron Fowler, Yan Song, Alejandro Victores, Alen Dezia, Jiayang Li, Azeem Banatwala, Qingyi Cai, Lana Melendres-Groves","doi":"10.1002/pul2.70398","DOIUrl":"10.1002/pul2.70398","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a life-limiting disease with psychosocial burden, although US population-level estimates of incident mental health (MH) disorders remain limited. In this retrospective cohort study using Komodo Research Data (01/01/2016-03/31/2024), we evaluated the association between PAH diagnosis and risk of newly diagnosed MH conditions. Adults (≥ 18 years) with incident PAH were identified alongside a non-PAH control group. Among patients with PAH (<i>N</i> = 4005), the risks of newly diagnosed MH conditions were compared before and after the PAH diagnosis date (index date). Between-group comparisons of newly diagnosed MH conditions post-index used inverse probability weighting to balance PAH and non-PAH patients. Outcomes included 12-month cumulative incidence and hazard ratios (HRs) from Cox models for any MH condition and prespecified categories (anxiety, mood, sleep-wake, substance use). Post-diagnosis, the 12-month risk of any newly diagnosed MH condition nearly doubled compared with pre-diagnosis (48.1% vs 24.9%; HR: 2.29; <i>p</i> < 0.001). Post-diagnosis risks were also higher for sleep-wake (27.1% vs 11.5%; 2.59), anxiety-spectrum (11.4% vs 8.4%; 1.37), mood (9.7% vs 6.0%; 1.65), and opioid use disorders (1.5% vs 0.7%; 2.07; all <i>p</i> < 0.05). Compared with matched controls (<i>N</i> = 421,987), PAH patients had higher risks of any MH condition (39.5% vs 17.8%; HR: 2.57), anxiety (10.1% vs 6.5%; 1.58), mood (8.7% vs 5.3%; 1.68), and substance use disorders (5.2% vs 3.4%; 1.53; all <i>p</i> < 0.001). Adults with PAH experienced marked increases in newly diagnosed MH conditions after PAH onset and significantly higher risk than matched non-PAH controls, supporting routine MH screening and integrated, proactive MH care in PAH management.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70398"},"PeriodicalIF":2.4,"publicationDate":"2026-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549224/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148907120","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 Arterial Hypertension in Latin America: Characteristics, Treatment, and Survival From the LATAM-PAH Meta-Analysis and Systematic Review.","authors":"Marcos Vinicius Fernandes Garcia, Katherine Julian, Evans R Fernández Pérez, Rogerio Souza, Tim Lahm","doi":"10.1002/pul2.70403","DOIUrl":"10.1002/pul2.70403","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) in Latin America and the Caribbean (LAC) remains poorly characterized, with limited regional data on clinical presentation, hemodynamics, treatment patterns, and outcomes. We aimed to characterize demographic features, clinical presentation, hemodynamic severity, treatment patterns, and survival outcomes of right heart catheterization-confirmed PAH cohorts across LAC. We performed a systematic review and meta-analysis of retrospective and prospective cohort studies of adults with right heart catheterization-confirmed PAH in LAC, searching six databases in English, Spanish, and Portuguese without date restrictions. Study-level aggregate data were pooled using random-effects models. Sixteen studies from eight countries (1999-2025) including 2,553 patients were analyzed. Mean age was 43.6 years and 81.5% were female. Etiologies included idiopathic PAH (40.3%), congenital heart disease-associated PAH (25.6%), connective tissue disease-associated PAH (20.6%), and schistosomiasis-associated PAH (15.3% in Brazilian cohorts). At diagnosis, 51% were in functional class III/IV, mean 6-min walk distance was 406 m, mean pulmonary arterial pressure was 55.8 mmHg, and cardiac index was 2.63 L/min/m². Treatment consisted primarily of monotherapy (56%) or dual therapy (33%). Pooled survival was 93.9% at 1 year, 87.5% at 2 years, and 83.0% at 3 years. Predominantly reflecting South American PAH care, our findings establish the first regional benchmark for PAH in LAC. Patients are younger and predominantly female, with a high burden of CHD-PAH and schistosomiasis-associated PAH. Despite delayed diagnosis and limited combination therapy access, survival is comparable to high-income registries, highlighting priorities such as earlier referral, risk assessment, expanded therapy access, and prospective registry development.</p><p><strong>Trail registration: </strong>PROSPERO CRD420251131636.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70403"},"PeriodicalIF":2.4,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13543302/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148897798","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":"Optimized Contrast-Enhanced Micro-Computed Tomography for Simultaneous Imaging of the Ductus Arteriosus and Pulmonary Arteries During the Perinatal Transition.","authors":"Elahe Alizadeh, Patricia D A Lima, Rachel E T Bentley, Ashley Y Martin, Stephen L Archer","doi":"10.1002/pul2.70396","DOIUrl":"10.1002/pul2.70396","url":null,"abstract":"<p><p>Contrast-enhanced micro-computed tomography (micro-CT) provides high-resolution, three-dimensional visualization of small vascular structures in situ, enabling detailed analysis of morphological changes in the perinatal vasculature. The ductus arteriosus (DA) connects the aorta and pulmonary artery (PA), diverting placentally oxygenated blood away from the lungs in utero. Both DA and PA undergo rapid changes in tone with the first breath during the transition from fetal to neonatal oxygen conditions. This study aimed to optimize contrast-enhanced micro-CT protocols to enable simultaneous visualization of the DA and pulmonary vasculature during this transition in fetal rabbits. Two contrast agents, an iodine-based agent (Omnipaque, 300 mg/mL) and two forms of a barium sulphate suspension, were compared to determine which optimally opacified the DA and pulmonary circulation in fetal rabbit kits (<i>n</i> = 22) from new-term New Zealand white rabbits (<i>n</i> = 4). Micro-CT imaging was performed to assess DA patency and changes in PA caliber across a wide range of vessel diameters from ~50 µm to > 1 mm under hypoxia versus normoxia in anesthetized kits. Omnipaque provided adequate opacification of the DA but limited visualization of the pulmonary vasculature. However, unfiltered 25% barium sulfate in 3% gelatin achieved robust, simultaneous visualization of the DA and pulmonary circulation. This agent optimally enhanced contrast in both large vessels and small PAs, enabling simultaneous assessment of DA constriction and pulmonary arterial vasodilation within 20 min of breathing oxygen. This study established an optimal contrast agent and micro-CT imaging strategy for post-mortem assessment of DA and pulmonary vascular morphology during the perinatal transition.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70396"},"PeriodicalIF":2.4,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13543299/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148897788","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":"Observed Weight Gain in Patients With Pulmonary Arterial Hypertension Treated With Sotatercept.","authors":"Rebecca A Greene, Darlene Tat, Allyson Sherman-Roe, Tiffaney Cayton, Jacqueline Lopes, Alexander Jorrin, James Klinger, Christopher J Mullin, Kathryn Needle, William M Oldham, Crossandra Osgood, Navneet Singh, Corey E Ventetuolo","doi":"10.1002/pul2.70391","DOIUrl":"10.1002/pul2.70391","url":null,"abstract":"<p><p>Sotatercept, an activin signaling inhibitor for pulmonary arterial hypertension (PAH), has demonstrated significant improvements in clinical outcomes though its off-target effects are still an area for discovery. Anecdotal increases in total body weight not attributable to fluid retention have been observed. Understanding the potential for weight gain and body composition changes is important as excessive weight gain in the PAH population is deleterious whereas increases in lean body mass may favorably affect exercise capacity. Therefore, we conducted a single-center retrospective observational cohort study of adult Group 1 PAH patients seen at a Pulmonary Hypertension Association-accredited Comprehensive Care Center treated with sotatercept for a minimum of 12 weeks. The primary endpoint was the proportion of patients who experienced clinically significant weight gain of ≥ 5% with sotatercept treatment. Secondary endpoints were the frequency of sotatercept dose adjustments required due to changes in body weight. Exploratory analyses were conducted to identify potential relationships between weight change and covariates of interest. Of 23 patients included, clinically significant weight gain was observed in 43.5% (<i>n</i> = 10). The median absolute change from baseline weight was 3.18 kg (Q1-Q3, -0.80-7.43 kg), and the median percent change was 4.43 (Q1-Q3, -0.78-9.01). Dosage adjustments based on dosing weight changes (increase or decrease) occurred at an incidence of 1.56 per patient-year. These results suggest the importance of ongoing monitoring of body weight throughout sotatercept treatment and that weight gain is commonly associated with treatment, although not previously observed or evaluated in clinical trials to date.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70391"},"PeriodicalIF":2.4,"publicationDate":"2026-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13540266/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148887269","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":"Pressure-Derived Right Ventricular Ejection Fraction During Initial Dual Combination Therapy in Pulmonary Arterial Hypertension.","authors":"Tharsiga Kanagasingam, Jonas Lund, Christoph R Sinning, Gunnar K Lund, Hans Klose, Lars Harbaum","doi":"10.1002/pul2.70394","DOIUrl":"10.1002/pul2.70394","url":null,"abstract":"<p><p>In patients with pulmonary arterial hypertension, pressure-based right ventricular ejection fraction (RVEF) estimated from routine right heart catheterisation correlated with RVEF from magnetic resonance imaging. A framework combining Weibull distribution-derived theoretical isovolumic pressure and time-varying elastance-derived end-systolic pressure showed the closest correlation and was responsive to initial oral combination therapy.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70394"},"PeriodicalIF":2.4,"publicationDate":"2026-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13540265/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148887217","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 Function and Exercise-Based Assessments for the Detection of Pulmonary Hypertension in Fibrotic Interstitial Lung Disease.","authors":"Vaida Averjanovaitė, Lina Gumbienė, Diana Zakarkaitė, Monika Laukytė-Slėnienė, Eglė Grigonienė, Virginija Rudienė, Taida Ivanauskienė, Virginija Šileikienė","doi":"10.1002/pul2.70402","DOIUrl":"10.1002/pul2.70402","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is a frequent complication of fibrotic interstitial lung diseases (ILDs), but its detection remains challenging due to overlapping clinical features. We aimed to evaluate the discriminatory power of physiological and exercise-based markers that differentiate right heart catheterization (RHC)-confirmed PH from low-probability ILD cases. In this prospective observational study of 59 patients with ILD, we evaluated pulmonary function tests (PFTs), cardiopulmonary exercise testing (CPET), and the 6-min walk test (6MWT). Patients deemed at high probability for clinically relevant PH following a multidisciplinary assessment were referred for RHC. Receiver operating characteristic (ROC) analysis was used to compare RHC-confirmed PH cases (<i>n</i> = 18) with a clinically low-probability ILD cohort (<i>n</i> = 19). Patients with RHC-confirmed PH had significantly lower diffusing capacity for carbon monoxide (DLCO) (43.44 ± 13.73% vs. 59.11 ± 13.50%, <i>p</i> = 0.002) and higher ventilatory inefficiency, as measured by the VE/VCO₂ slope (47.00 ± 7.90 vs. 40.71 ± 8.48, <i>p</i> = 0.035). DLCO showed moderate discriminatory performance (AUC 0.799; optimal cut-off 50.5% predicted; sensitivity 72%; specificity 72%), whereas VE/VCO₂ slope showed fair discrimination (AUC 0.711; cut-off ≈ 41.5; sensitivity 75%; specificity 59%). Although 6MWT distance did not differentiate the groups, post-exercise oxygen desaturation and heart rate were significant indicators. In fibrotic ILD, gas exchange and ventilatory inefficiency parameters-specifically DLCO and VE/VCO₂ slope-effectively discriminate RHC-confirmed PH from clinically low-probability cases. These markers offer a practical screening approach to identify patients who should be prioritized for further PH testing.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70402"},"PeriodicalIF":2.4,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13536034/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148881447","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":"Diffusing Capacity of the Lung for Carbon Monoxide and the Risk of All-Cause Mortality in Patients With Pulmonary Hypertension Caused by Fibrosing Mediastinitis: A Multicenter Cohort Study.","authors":"Mengyu Wu, Hongling Su, Xuechun Sun, Sugang Gong, Wei Ding, Yuhong Li, Yuting Li, Wenjie Dong, Bowen Jin, Liming Zhao, Qian Jiang, Xincao Tao, Ou Xu, Wande Yu, Jie Cheng, Xintian Cai, Yunshan Cao","doi":"10.1002/pul2.70401","DOIUrl":"10.1002/pul2.70401","url":null,"abstract":"<p><p>Pulmonary hypertension caused by fibrosing mediastinitis (PH-FM) is a high-risk condition with limited validated tools for prognostic assessment. Although diffusing capacity of the lung for carbon monoxide (DLCO) predicts outcomes in several cardiopulmonary diseases, its prognostic value in PH-FM remains unclear. In this multicenter retrospective cohort study, we included 106 patients with confirmed PH-FM from 14 centers across China. The primary endpoint was all-cause mortality. Cox proportional hazards models were used to assess the association between baseline percent-predicted DLCO and mortality, with sequential adjustment for clinical, hemodynamic, and spirometric variables. The final cohort included 106 patients, of whom 60 had DLCO ≥ 60% predicted and 46 had DLCO < 60% predicted. Over a median follow-up of 36 months (interquartile range [IQR], 14.0-50.8), 27 deaths occurred. Across Cox models, each 10-percentage-point increase in percent-predicted DLCO was associated with a 30% lower risk of all-cause mortality. DLCO < 60% predicted was associated with higher all-cause mortality compared with DLCO ≥ 60% predicted, with hazard ratios ranging from 6.90 to 7.80 across models. Lower baseline percent-predicted DLCO was independently associated with a higher risk of all-cause mortality in patients with PH-FM. DLCO may provide a simple, noninvasive measure to complement current risk assessment in this population.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70401"},"PeriodicalIF":2.4,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13535974/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148881410","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}