Pulmonary Circulation最新文献_第7页

The Impact of Cardiac Output Methods on the Classification of Pulmonary Hypertension. 心输出量测定方法对肺动脉高压分类的影响。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-06-19 eCollection Date: 2025-04-01 DOI: 10.1002/pul2.70112

Léon Genecand, Gaëtan Simian, Mona Lichtblau, Jean-Marc Fellrath, Julian Klug, Hugues Turbé, Christian Lovis, Stéphane Noble, Julie Wacker, Julian Müller, Roberto Desponds, Maurice Beghetti, Benoit Lechartier, David Montani, Olivier Sitbon, Silvia Ulrich, Frédéric Lador

{"title":"The Impact of Cardiac Output Methods on the Classification of Pulmonary Hypertension.","authors":"Léon Genecand, Gaëtan Simian, Mona Lichtblau, Jean-Marc Fellrath, Julian Klug, Hugues Turbé, Christian Lovis, Stéphane Noble, Julie Wacker, Julian Müller, Roberto Desponds, Maurice Beghetti, Benoit Lechartier, David Montani, Olivier Sitbon, Silvia Ulrich, Frédéric Lador","doi":"10.1002/pul2.70112","DOIUrl":"10.1002/pul2.70112","url":null,"abstract":"Cardiac output is essential to calculate pulmonary vascular resistance (PVR) and classify pulmonary hypertension (PH). Recent evidence has shown a lower agreement between thermodilution (COTD) and direct Fick (CODF) methods than historically estimated. The influence of the cardiac output measurement method on the classification of PH is poorly explored. We aimed to estimate the risk of diagnostic error when using COTD instead of CODF. We used a previously published mathematical model to consecutive patients diagnosed with PH at three centers in Switzerland. This model allows an individual estimation of the risk of diagnostic error when using COTD instead of CODF and is based on limits of agreement (LoA) between COTD and CODF of 2 L/min (average estimation) and 2.7 L/min (worst case scenario estimation). One thousand one hundred and forty-two patients with PH were evaluated. The mean risk of diagnostic error using the model with LoA of 2 L/min was 6.0% in the overall population (n = 1142). The mean risk of diagnostic error was 2.9% among the 712 patients with precapillary PH, 15.0% among the 113 patients with isolated postcapillary PH (IpcPH), 7.2% among the 247 patients with combined post- and pre-capillary PH, and 18.8% among the 70 patients with unclassified PH. The estimated diagnostic error when using COTD instead of CODF was generally low, particularly for patients with precapillary PH. Patients with PVR close to the diagnostic threshold of 2 WU (i.e., between 1 and 3 WU), mostly concerning patients with IpcPH and unclassified PH, exhibited a higher risk of diagnostic error.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70112"},"PeriodicalIF":2.2,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12177550/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144333815","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Melatonin Improves Quality of Life, Oxidative Stress, and Cardiovascular Function in Pulmonary Arterial Hypertension. 褪黑素改善肺动脉高压患者的生活质量、氧化应激和心血管功能。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-06-19 eCollection Date: 2025-04-01 DOI: 10.1002/pul2.70109

Alicia de la Fuente, Mónica Zagolín, Víctor Parra, Adolfo A Paz, Alejandro González-Candia, Osvaldo Cabrera, Cristian Olave, Constanza Bahamondes, María José Gaete, Lodz Gudenschwager, Javiera Rivas, Emilio A Herrera

{"title":"Melatonin Improves Quality of Life, Oxidative Stress, and Cardiovascular Function in Pulmonary Arterial Hypertension.","authors":"Alicia de la Fuente, Mónica Zagolín, Víctor Parra, Adolfo A Paz, Alejandro González-Candia, Osvaldo Cabrera, Cristian Olave, Constanza Bahamondes, María José Gaete, Lodz Gudenschwager, Javiera Rivas, Emilio A Herrera","doi":"10.1002/pul2.70109","DOIUrl":"10.1002/pul2.70109","url":null,"abstract":"Pulmonary arterial hypertension (PAH) Group 1 from the World Health Organization (WHO) is a rare, severe chronic, and progressive condition. Patients with PAH have increased oxidative stress (OS) and diminished antioxidant capacity. Melatonin is a potent antioxidant hormone with reported benefits in PAH animal models. We aimed to evaluate the functional, hemodynamic, and antioxidant response to a 6-month melatonin therapy in PAH Group 1 patients. Clinical evaluation was done at baseline (BT), as well as at 3 (T3) and 6 (T6) months of melatonin treatment in stable PAH Group 1 (WHO) patients. The principal endpoint was change in walking distance (WD) in the 6-min walking test (6MWT). Secondary endpoints were functional class (FC), quality of life (QoL), performance of right ventricle (RV), and plasma antioxidant capacity. This study included 19 patients. They were mainly women in WHO FC II and III. A significant improvement was noticed in perception of dyspnea, palpitations, and fatigue in concordance with the QoL improvement in the physical domain after 6 months of melatonin. This was represented by a significant fall in the total score of the PAH-SYMPACT questionnaire. In addition, the baseline heart rate was lower at the T6 follow-up. No significant changes were seen in the echocardiographic variables. However, the biochemical analysis showed significative increases in plasma total antioxidant (2.94 ± 0.13 vs. 8.41 ± 0.19) and ferric reducing (191 ± 12 vs. 256 ± 17) capacities. Overall, oral melatonin treatment improved the plasma antioxidant capacity and the QoL in this pilot study.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70109"},"PeriodicalIF":2.2,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12177658/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144333814","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

The Role of Psychological Distress on Health-Related Quality of Life, Fatigue, and Pain in Adults With Pulmonary Hypertension. 心理困扰对成人肺动脉高压患者健康相关生活质量、疲劳和疼痛的影响

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-06-17 eCollection Date: 2025-04-01 DOI: 10.1002/pul2.70101

Gregg H Rawlings, Abbie Stark, Iain Armstrong, Vlad Costin, Andrew R Thompson

{"title":"The Role of Psychological Distress on Health-Related Quality of Life, Fatigue, and Pain in Adults With Pulmonary Hypertension.","authors":"Gregg H Rawlings, Abbie Stark, Iain Armstrong, Vlad Costin, Andrew R Thompson","doi":"10.1002/pul2.70101","DOIUrl":"10.1002/pul2.70101","url":null,"abstract":"While anxiety and depression are commonly reported in pulmonary hypertension (PH), limited evidence exists on how these conditions interact with the pathophysiological symptoms of PH. Fatigue and, to a lesser degree, pain are key symptoms of PH; however, they have rarely been examined as separate experiences associated with PH. Using a cross-sectional research design, 68 adults with PH recruited from global Pulmonary Hypertensions Associations completed a series of self-report measures assessing fatigue, pain self-efficacy, anxiety, depression, and health-related quality of life (HRQoL). Aiming to understand the nuances of PH symptomatology, we first looked at responses on individual items from fatigue and pain measures, respectively. Then, to examine relationships between self-reported symptoms, we tested potential pathways from fatigue and pain to HRQoL through depression and anxiety. All symptoms were correlated, suggesting individuals with greater anxiety and depression also experienced more fatigue, and lower pain self-efficacy and HRQoL. Parallel mediation analyses showed that fatigue and pain had a direct effect on HRQoL, as well as an indirect effect via anxiety and depression. Explorative serial mediation models suggested the indirect path from fatigue to HRQoL was significant when depression was ordered first followed by anxiety; whereas for pain self-efficacy, the path was significant when anxiety was followed by depression. Results add to the evidence demonstrating the high co-occurrence of mental health difficulties in PH and the important role they play in pathophysiological symptomatology. Analyses support providing holistic care for this clinical group to help identify various therapeutic targets suggestive of predicting HRQoL.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70101"},"PeriodicalIF":2.2,"publicationDate":"2025-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12171787/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144317805","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

The Role of Telehealth in PAH Management: Benefits, Barriers, and Solutions. 远程医疗在多环芳烃管理中的作用：益处、障碍和解决方案。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-06-09 eCollection Date: 2025-04-01 DOI: 10.1002/pul2.70079

Susanne McDevitt, Martha Kingman, Lana Melendres Groves, Gabriela Gomez Rendon, Michelle Han, Christina Benninger, Sylvia Georgi, Medi Stone, Daisy Bridge, Richard Perry, Ashley Enstone, Lisa Perrett, Holly Smith, Gurinderpal Doad, Jean M Elwing

{"title":"The Role of Telehealth in PAH Management: Benefits, Barriers, and Solutions.","authors":"Susanne McDevitt, Martha Kingman, Lana Melendres Groves, Gabriela Gomez Rendon, Michelle Han, Christina Benninger, Sylvia Georgi, Medi Stone, Daisy Bridge, Richard Perry, Ashley Enstone, Lisa Perrett, Holly Smith, Gurinderpal Doad, Jean M Elwing","doi":"10.1002/pul2.70079","DOIUrl":"10.1002/pul2.70079","url":null,"abstract":"Telehealth utilization increased during the coronavirus disease-2019 (COVID-19) pandemic, which encouraged remote patient management. The identification of optimal strategies to enhance telehealth and address barriers to its use for patients with pulmonary arterial hypertension (PAH) may improve patient outcomes. Physicians (n = 11) and advanced practice providers (n = 6) based in the United States with experience with telehealth in PAH were recruited to a double-blinded modified Delphi panel (two survey rounds and a virtual consensus meeting). Consensus was defined as ≥ 80% of panelists rating their agreement or disagreement using a 9-point Likert scale. A consensus in agreement was reached that telehealth is the use of virtual or remote methodologies to interact with, monitor and assess patients and deliver health care. Telehealth methodologies may be used in PAH for returning visits, diagnostic test follow-up and medication management; telehealth is convenient, enables additional visits, and provides patient access to care. There are barriers to telehealth such as socioeconomic status, patient digital literacy, visual and/or hearing impairments, developmental disabilities, geographical location, and reimbursement. Solutions to improve patient access to telehealth include health insurance reimbursements, financial resources for healthcare professionals (HCPs), and improved connectivity infrastructure for patients. There are benefits, barriers and solutions for telehealth in general, and more research is required to evaluate the impact on PAH care. Further research and insights from the present study may improve PAH management and telehealth services. Improvements in telehealth care and delivery may allow better monitoring of patients with PAH and assist in more equitable PAH care.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70079"},"PeriodicalIF":2.2,"publicationDate":"2025-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12147401/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144258905","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Programmatic Approach to Outpatient Cardiac Catheterization in Children With Stable Pulmonary Arterial Hypertension. 稳定期肺动脉高压患儿门诊心导管置入术的程序化方法。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-06-05 eCollection Date: 2025-04-01 DOI: 10.1002/pul2.70105

Gregory T Adamson, Minnie N Dasgupta, Zachary Kleiman, Lynn F Peng, Michelle Ogawa, Vidhya Balasubramanian, Chandra Ramamoorthy, Jeffrey A Feinstein

{"title":"Programmatic Approach to Outpatient Cardiac Catheterization in Children With Stable Pulmonary Arterial Hypertension.","authors":"Gregory T Adamson, Minnie N Dasgupta, Zachary Kleiman, Lynn F Peng, Michelle Ogawa, Vidhya Balasubramanian, Chandra Ramamoorthy, Jeffrey A Feinstein","doi":"10.1002/pul2.70105","DOIUrl":"10.1002/pul2.70105","url":null,"abstract":"When performing cardiac catheterization in pediatric outpatients with pulmonary arterial hypertension (PH), our approach is to allow spontaneous ventilation, minimize procedural length, and evaluate for same-day discharge whenever safe and feasible. We describe our experience with this approach and identify clinical characteristics that influenced procedural safety. Outpatients < 21 years who underwent catheterization for PH from 2009 to 2018 were included in the retrospective cohort. Demographic, clinical, and procedural data were collected. Data were modeled using a mixed effects logistic regression for correlated data, and a patient random effect was included to account for multiple procedures in the same patient. Of 409 catheterizations screened, 250 procedures in 118 outpatients were included. Of the 250 procedures, 185 (74.0%) were discharged on the same day. There were no major adverse events within 48 h of discharge in any of the 185 nor in the 12 (197 total, 78.8%) admitted for medication titration or an unrelated procedure (i.e., could have otherwise been discharged). Median procedural duration was 51.0 (33.0, 76.8) minutes. Endotracheal intubation, younger age, longer procedural duration, and worse functional status were associated with higher odds of admission. In a prospective secondary cohort of 39 procedures in 34 patients, 32 (82%) were discharged same-day without complication, including over 90% of children over 3 years of age who were managed without endotracheal intubation. By prioritizing spontaneous ventilation and procedural efficiency, outpatient pediatric PH patients who undergo catheterization, emergence, and a 4-h observation with no complications may be considered for same-day discharge or observation in a low-acuity bed.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70105"},"PeriodicalIF":2.2,"publicationDate":"2025-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12141513/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144249306","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Prophylactic Effects of Radiofrequency Electromagnetic Field on Pulmonary Ischemia-Reperfusion via HIF-1α/eNOS Pathway and BCL2/BAX Signaling. 射频电磁场通过HIF-1α/eNOS通路和BCL2/BAX信号对肺缺血再灌注的预防作用

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-06-03 eCollection Date: 2025-04-01 DOI: 10.1002/pul2.70051

Süleyman Emre Akin, Halil Asci, Muhammet Yusuf Tepebasi, İlter Ilhan, Özlem Ozmen, Selçuk Comlekci, Rümeysa Taner, Hasan Ekrem Camas, Ayşegül Keklik, Rasih Yazkan

{"title":"Prophylactic Effects of Radiofrequency Electromagnetic Field on Pulmonary Ischemia-Reperfusion via HIF-1α/eNOS Pathway and BCL2/BAX Signaling.","authors":"Süleyman Emre Akin, Halil Asci, Muhammet Yusuf Tepebasi, İlter Ilhan, Özlem Ozmen, Selçuk Comlekci, Rümeysa Taner, Hasan Ekrem Camas, Ayşegül Keklik, Rasih Yazkan","doi":"10.1002/pul2.70051","DOIUrl":"10.1002/pul2.70051","url":null,"abstract":"This study aimed to investigate the vascular effects of a radiofrequency electromagnetic field (RF-EMF) applied in the lung ischemia and reperfusion (IR) model on the hypoxia-inducible factor 1 alpha (Hif-1α)/endothelial nitric oxide synthase (eNOS) pathway and B cell lymphoma 2 (BCL2)/BCL-2 associated X protein (BAX) signaling. Forty male Wistar rats were randomly divided into four groups, each consisting of 10 rats: Sham, IR, IR + RF-EMF, and RF-EMF. IR was applied to rats by 60 min of clamping hilus of left lungs and 60 min of reperfusion. Rats were kept in the RF-EMF unit for 60 min with or without activation. After sacrification, lung tissues were excised for histopathological, immunohistochemical, biochemical, and genetic analyses. IR injury led to increased damage-related emphysematous findings, significant hyperemia, and increased septal tissue thickness, as observed histopathologically, and immunoexpression levels of tumor necrosis factor-alpha and caspase-3. In addition, it was noted that the biochemical parameters total oxidant status, oxidative stress index, and genetic parameters Hif 1 α, eNOS, BAX increased, and BCL2 decreased due to IR damage. In the IR-RF-EMF group, improvement has been detected in all parameters. RF-EMF applied in the IR model exerts antioxidant, antiapoptotic, and anti-inflammatory effects on lung tissue damage through the Hif-1α/eNOS pathway and BCL-2/BAX signaling. The use of RF-EMF in IR damage is promising, as models that examine the long-term effects of RF-EMF at different frequencies.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70051"},"PeriodicalIF":2.2,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12133609/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144226428","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Body Mass Index and Right Ventricular Structure: Insights From Observational and Mendelian Randomization Analyses. 体重指数和右心室结构：来自观察性和孟德尔随机化分析的见解。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-06-03 eCollection Date: 2025-04-01 DOI: 10.1002/pul2.70103

Julian Pott, Maximilian Kirchner, Jan K Hennigs, Christoph R Sinning, Hans Klose, Lars Harbaum

{"title":"Body Mass Index and Right Ventricular Structure: Insights From Observational and Mendelian Randomization Analyses.","authors":"Julian Pott, Maximilian Kirchner, Jan K Hennigs, Christoph R Sinning, Hans Klose, Lars Harbaum","doi":"10.1002/pul2.70103","DOIUrl":"10.1002/pul2.70103","url":null,"abstract":"Overweight and obesity have emerged as modifiable risk factors for right ventricular (RV) phenotypic changes, but their genetic relationship remains unclear. This study examined RV phenotypes using cardiac magnetic resonance imaging in European participants from the UK Biobank without overt heart disease. Observational and Mendelian randomization approaches, based on individual- and summary-level genetic data, were integrated to assess the effects of BMI on RV imaging phenotypes. Among 33,801 individuals with a mean age of 64 years, 52% were women, 41% were overweight, and 18% were obese. Overweight and obese participants exhibited larger RV volumes and lower RV ejection fractions compared to normal-weight participants, even after adjusting for left heart parameters, cardiometabolic risk factors, and diseases. One-sample Mendelian randomization revealed that higher lifetime BMI was related to larger RV end-diastolic volume (3.4 mL per standard deviation BMI increase, 95% CI 2.8-4.0 mL), RV end-systolic volume (1.6 mL, 95% CI 1.3-1.9 mL), and stroke volume (1.8 mL, 95% CI 1.4-2.2 mL). Adjustment for left ventricular measures reduced these effect sizes by 51%-67%, but relationships remained statistically significant. Two-sample Mendelian randomization confirmed these findings using robust methods and correction for pleiotropic outliers. While the observational associations were more pronounced in women than in men, the genetic effects were similar across sexes. In conclusion, the relationships between BMI and RV volumes were generally consistent across observational and genetic analyses. Genetic predisposition to higher lifetime BMI influenced RV volumes in a population with a low prevalence of cardiopulmonary diseases, an effect not fully explained by left ventricular measures. These findings suggest that managing overweight and obesity may help prevent structural RV remodeling.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70103"},"PeriodicalIF":2.2,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12130632/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144216730","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Ectopic Expansion of Pulmonary Vasculature in Fibrotic Lung Disease and Lung Adenocarcinoma Marked by Proangiogenic COL15A1+ Endothelial Cells. 以促血管生成COL15A1+内皮细胞为标志的纤维化肺病和肺腺癌肺血管异位扩张

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-06-03 eCollection Date: 2025-04-01 DOI: 10.1002/pul2.70102

Eric Engelbrecht, Tristan Kooistra, Nathalie Burg, Lida Hariri, Trong Nguyen, Patricia Brazee, Timothy Hla, Bernadette R Gochuico, Rachel S Knipe

{"title":"Ectopic Expansion of Pulmonary Vasculature in Fibrotic Lung Disease and Lung Adenocarcinoma Marked by Proangiogenic COL15A1+ Endothelial Cells.","authors":"Eric Engelbrecht, Tristan Kooistra, Nathalie Burg, Lida Hariri, Trong Nguyen, Patricia Brazee, Timothy Hla, Bernadette R Gochuico, Rachel S Knipe","doi":"10.1002/pul2.70102","DOIUrl":"10.1002/pul2.70102","url":null,"abstract":"Lung vasculature arises from both pulmonary and systemic (bronchial) circulations. Remodeling and structural changes in lung vasculature have been recognized in end-stage fibrotic lung diseases such as idiopathic pulmonary fibrosis (IPF) but have not been well characterized. The vasculature that expands and supplies lung cancers is better described, with the recent recognition that systemic bronchial circulation expands to be the main blood supply to primary lung tumors. Here, we use publicly available single-cell RNA-sequencing (scRNA-seq) data to compare vascular endothelial cell (EC) populations in multiple progressive interstitial lung diseases (ILD) and non-small cell lung cancer (NSCLC) to identify common and distinct features. Lung tissue specimens were collected from healthy lung tissue (n = 59), ILD (n = 97), chronic obstructive pulmonary disease (n = 22), and NSCLC (n = 8). We identify two subtypes of expanded EC populations in both ILD and NSCLC, \"Bronch-1\" and \"Bronch-2\", expressing transcripts associated with venules and angiogenic tip/stalk cells, respectively. Relative to pulmonary capillary and arterial ECs, bronchial ECs show low expression of transcripts associated with vascular barrier integrity. The pan-bronchial EC marker COL15A1 showed positive staining in lung parenchyma from patients with IPF, SSc-ILD, and NSCLC, whereas positive staining was limited to subpleural and peri-bronchial regions in non-fibrotic controls. In conclusion, expansion of a subset of ECs expressing markers of the bronchial circulation is one of the most pronounced changes in vascular cell composition across multiple ILDs and NSCLC. These data support additional studies to determine the role of the bronchial vasculature in ILD progression.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70102"},"PeriodicalIF":2.2,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12130637/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144216731","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome. 以严重肺血管疾病为表现的青少年系统性硬化症：积极治疗期间ECMO稳定导致有利结果

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-06-03 eCollection Date: 2025-04-01 DOI: 10.1002/pul2.70106

Stephanie M Tsoi, Shannon Cheung, Roberto Alejandro Valdovinos, Elena Amin, Hythem Nawaytou, Elizabeth Colglazier, Claire Parker, April Edwell, Susan Kim, Jeffrey R Fineman

{"title":"Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome.","authors":"Stephanie M Tsoi, Shannon Cheung, Roberto Alejandro Valdovinos, Elena Amin, Hythem Nawaytou, Elizabeth Colglazier, Claire Parker, April Edwell, Susan Kim, Jeffrey R Fineman","doi":"10.1002/pul2.70106","DOIUrl":"10.1002/pul2.70106","url":null,"abstract":"Juvenile systemic sclerosis (jSSc) associated pulmonary hypertension (PH) is rare, but, the leading cause of morbidity and mortality in jSSc. This is a case of a 10-year-old girl whose initial presentation of positive U3-RNP antibody jSSc included diffuse skin findings, severe pulmonary arterial hypertension, and right ventricular failure. Veno-arterial extracorporeal membranous oxygenation (VA-ECMO) and atrial stent placement facilitated treatment with pulse-dose steroids, mycophenolate mofetil, and B-cell depleting therapy to treat the underlying autoimmune inflammation and triple therapy with treprostinil, ambrisentan, and tadalafil for her pulmonary hypertension. At 9-month follow-up, her jSSc is well-controlled with complete resolution of her PH. This case demonstrates that multi-disciplinary treatment, including upfront multi-drug therapy for jSSC and PAH, that included VA-ECMO, may improve outcomes, particularly when treatment for underlying causes (in this case, jSSc) is just being initiated.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70106"},"PeriodicalIF":2.2,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12130638/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144216732","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Lung Transplant Outcomes in Patients With Preoperative Catheterization Indicating Group 2 Pulmonary Hypertension. 2组肺动脉高压术前插管患者的肺移植预后。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-06-02 eCollection Date: 2025-04-01 DOI: 10.1002/pul2.70107

Víctor M Mora-Cuesta, Amaya Martínez-Meñaca, David Iturbe-Fernández, Sandra Tello-Mena, Sheila Izquierdo-Cuervo, Tamara García-Camarero, Aritz Gil-Ongay, Pilar Alonso-Lecue, José M Cifrián-Martínez, Diego Agustín Rodríguez-Chiaradia, Pilar Escribano-Subías, Esther Barreiro

{"title":"Lung Transplant Outcomes in Patients With Preoperative Catheterization Indicating Group 2 Pulmonary Hypertension.","authors":"Víctor M Mora-Cuesta, Amaya Martínez-Meñaca, David Iturbe-Fernández, Sandra Tello-Mena, Sheila Izquierdo-Cuervo, Tamara García-Camarero, Aritz Gil-Ongay, Pilar Alonso-Lecue, José M Cifrián-Martínez, Diego Agustín Rodríguez-Chiaradia, Pilar Escribano-Subías, Esther Barreiro","doi":"10.1002/pul2.70107","DOIUrl":"10.1002/pul2.70107","url":null,"abstract":"Lung transplantation (LT) is a well-established therapeutic option for patients with advanced chronic respiratory diseases. This study aims to assess the prevalence and clinical impact of Group 2 pulmonary hypertension (PHg2) in LT recipients, comparing it with Group 3 pulmonary hypertension (PHg3). This retrospective cohort study analyzed LT recipients from 2015 to 2024 at a single center. Patients were categorized into three groups based on hemodynamic measurements: no PH, PHg2, and PHg3. Hemodynamic data were acquired via right heart catheterization. Perioperative complications, including primary graft dysfunction (PGD), and long-term survival were compared across the groups. Of the 412 LT recipients, 40 (10.9%) were diagnosed with PHg2, while 62.5% had PHg3. Statistical analysis revealed no significant differences in perioperative outcomes, including the incidence of PGD, between patients with PHg2 and those with PHg3. Additionally, there were no differences in long-term survival between the groups. Within the PHg2 subgroup, patients with isolated PHg2 and those with combined PHg2 exhibited similar post-transplant outcomes. PHg2 is identified in a notable fraction of LT recipients, yet it does not appear to adversely affect perioperative complications or long-term survival when compared to PHg3 or patients without PH. These findings suggest that PHg2, despite its prevalence, does not significantly alter transplant outcomes. Future multicenter studies are needed to further explore the impact of subtle left ventricular dysfunction on LT results.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70107"},"PeriodicalIF":2.2,"publicationDate":"2025-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12130283/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144216733","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

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