Pulmonary Circulation最新文献

{"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":"<p><p>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 (<i>n</i> = 11) and advanced practice providers (<i>n</i> = 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.</p>","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}

{"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":"<p><p>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.</p>","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}

{"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":"<p><p>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<i>)</i> 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.</p>","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}

{"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":"<p><p>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.</p>","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}

{"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":"<p><p>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 (<i>n </i>= 59), ILD (<i>n </i>= 97), chronic obstructive pulmonary disease (<i>n </i>= 22), and NSCLC (<i>n </i>= 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.</p>","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}

{"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":"<p><p>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.</p>","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}

{"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":"<p><p>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.</p>","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}

{"title":"History of Adverse Pregnancy Outcomes in Women With Pulmonary Arterial Hypertension: A Single-Center Pilot Study.","authors":"Jessica B Badlam, Renee D Stapleton, Peter W Callas, Ira M Bernstein, Eric D Austin","doi":"10.1002/pul2.70104","DOIUrl":"10.1002/pul2.70104","url":null,"abstract":"<p><p>Single-center pilot study suggests a threefold increase in self-reported history of adverse pregnancy outcomes (APO) among women with subsequent diagnosis of pulmonary arterial hypertension (PAH). Females with PAH and a history of APO were younger and had higher mean pulmonary artery pressures at diagnosis compared to those without a history of APO.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70104"},"PeriodicalIF":2.2,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12126851/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144199920","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":"Patient Perspectives on Fixed Dose Combination Therapy for Pulmonary Arterial Hypertension: Exploratory Focus Group Research.","authors":"Jean M Elwing, Stacey Barta, Tim Smith, Gabriela Gomez Rendon, David Lopez, Wesley Peters, Marinella Sandros, Akshay Muralidhar","doi":"10.1002/pul2.70098","DOIUrl":"10.1002/pul2.70098","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) requires lifelong medication, with patients taking an average of 12 tablets/day. Several chronic diseases can be treated with a fixed-dose combination (FDC) tablet, decreasing patient pill burden and increasing adherence. This exploratory research, conducted via focus groups, asked 13 patients with PAH for their insights about the use of FDC (2 PAH treatments in a single tablet), its potential benefits, and challenges to its use. At the time of the focus groups (July 2023), no FDC medications were available for PAH and the discussions were therefore hypothetical. Focus group transcripts were analyzed by a qualitative research specialist to identify key themes. Most participants were unfamiliar with FDC prior to taking part in the focus groups; however, during the engagement, 12/13 participants expressed interest in taking FDC as single-tablet therapy for their PAH. In general, participants saw the potential benefits as improved convenience, less time spent navigating insurance coverage, and improved adherence. Participants felt that reducing their tablet count by just one tablet would be meaningful to them. Concerns were raised about the potential difficulty with medication titration and challenges distinguishing between the side effects of two combined medications. This exploratory research provides insight into the perceptions of US patients on the utility of FDC in PAH and highlights an unmet need for patient education on medication adherence in PAH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70098"},"PeriodicalIF":2.2,"publicationDate":"2025-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12117475/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144174655","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":"Characteristics of Daily Walking Bouts as Valid and Reliable Indicators of Exercise Capacity in Pulmonary Arterial Hypertension: Insights From the Randomized Controlled Study With Selexipag (TRACE).","authors":"Rana Zia Ur Rehman, Federico Parisi, Noman Ashraf, Meenakshi Chatterjee, Robert P Frantz, Anna R Hemnes, Nikolay V Manyakov, Oscar M Carrasco-Zevallos, Tarik Yardibi, Mona Selej, Tommaso Mansi, Preston Dunnmon, Dzmitry A Kaliukhovich","doi":"10.1002/pul2.70097","DOIUrl":"10.1002/pul2.70097","url":null,"abstract":"<p><p>There is a need for objective, continuous and remote assessment of exercise capacity in patients with pulmonary arterial hypertension (PAH). Using data from the TRACE study, in which 108 adult patients with PAH were continuously monitored with a wrist-worn accelerometer, we evaluated whether actigraphy can facilitate continuous monitoring of exercise capacity. Distributions of step rate, distance and duration of patient's walking bouts were estimated at baseline, Week 16 and Week 24 using 2-week periods of actigraphy data. Twenty-one metrics per walking bout characteristic were described (mean, standard deviation, 19 percentiles [5th-95th]). The relationships between these metrics and the 6-min walk distance (6MWD), Borg dyspnea index (BDI), and the PAH Symptoms and Impact questionnaire (PAH-SYMPACT) Physical Impact domain score were assessed at the three timepoints. Test-retest reliability, and discriminant and known-group validity of each metric were also evaluated. All metrics of step rate and bout distance were significantly correlated with 6MWD (Pearson's correlation coefficients: 0.34-0.67; <i>p</i> < 0.005) and the PAH-SYMPACT Physical Impact domain score (Pearson's correlation coefficients: -0.51 to -0.32; <i>p</i> < 0.05) at all timepoints. Negative correlations were also observed with the BDI and the actigraphy-derived metrics, with the majority reaching significance. Strong test-retest reliability was demonstrated (intra-class correlation coefficient ≥ 0.70). The metrics differentiated well between patients with varying disease severity levels. In conclusion, actigraphy-derived metrics of patients' walking bouts correlate significantly with 6MWD, BDI and physical impacts of PAH, indicating the utility of actigraphy in facilitating continuous monitoring of exercise capacity in adult patients with PAH.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov: NCT03078907; URL: clinicaltrials.gov.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70097"},"PeriodicalIF":2.2,"publicationDate":"2025-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12106039/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144161960","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}