Pulmonary Circulation最新文献

{"title":"A Prospective Analysis of Vasoreactivity and Mortality in WHO Group 3 Pulmonary Hypertension.","authors":"Daniel J Strick, Carl Tanba, Meredith A Kaplan, Nicholas S Hill, Harrison W Farber, David Condon, Ioana R Preston","doi":"10.1002/pul2.70078","DOIUrl":"https://doi.org/10.1002/pul2.70078","url":null,"abstract":"<p><p>Prognostic markers of Group 3 pulmonary hypertension (PH) remain largely unknown. In this study, we evaluate clinical data to provide a comprehensive profile of patients with Group 3 PH and evaluate the potential use of vasoreactivity testing as a prognostic tool within this population. We hypothesized that patients with a stronger vasoconstrictive component of their pulmonary vascular disease would have a more favorable prognosis. Patients were given inhaled nitric oxide during their right heart catheterization to determine if they met the European Respiratory Society guidelines for having a positive vasoreactivity test as defined for patients with Group 1 pulmonary arterial hypertension (PAH). While vasoreactivity response is proven to predict survival in subgroups of PAH, there was no significant relationship between change in mean pulmonary artery pressure (mPAP) during acute vasodilator challenge and survival within our cohort. On the contrary, patients with larger decreases in pulmonary vascular resistance (PVR) during the acute vasodilator challenge were at a significantly higher risk of mortality. The data suggests that the change in PVR during acute vasodilator challenge may be a better indicator of survival in patients with WHO Group 3 PH than the change in mPAP.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70078"},"PeriodicalIF":2.2,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12006029/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144036396","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":"Physical Activity, Sleep, and Quality of Life in Pulmonary Arterial Hypertension: Novel Insights From Wearable Devices.","authors":"Andrew M Hughes, Alisha Lindsey, Jeffrey Annis, Kelly Burke, Hiral Master, Luke G Silverman-Lloyd, Jonah D Garry, Michael J Blaha, Erika S Berman Rosenzweig, Robert P Frantz, Paul M Hassoun, Evelyn M Horn, Jane A Leopold, Franz P Rischard, Brett Larive, Nicholas S Hill, Serpil C Erzurum, Gerald J Beck, Anna R Hemnes, Evan L Brittain","doi":"10.1002/pul2.70069","DOIUrl":"10.1002/pul2.70069","url":null,"abstract":"<p><p>Reduced functional capacity and poor sleep quality are common in pulmonary arterial hypertension (PAH). Wearable devices are an emerging, user-friendly tool to capture activity and sleep information. We aimed to determine whether Fitbit-derived activity and sleep trends provide clinically meaningful information in patients with PAH. Our prospective observational study recruited patients with PAH from across the United States using remote enrollment strategies and in-person efforts. Participants wore a Fitbit device for 12 weeks at baseline and a subgroup with 1-year follow-up. A matched control cohort was generated from the <i>All of Us</i> Research Program and we evaluated changes in patients with PAH compared to matched controls. Among 110 patients with baseline monitoring, average daily steps correlated with 6MWD (<i>r</i> = 0.61, <i>p</i> < 0.001) and percent rapid eye movement (REM) sleep (<i>r</i> = 0.28, <i>p</i> = 0.008). In 44 PAH participants who completed baseline and 1-year monitoring, there was a group-time interaction for percent light sleep (<i>p</i> = 0.024) and percent REM sleep (<i>p</i> = 0.034), which demonstrated that sleep quality worsened in patients with PAH over 1 year compared to matched controls. Average daily steps decreased in patients with PAH from 5200 [IQR 3212-7458] at baseline to 4651 [IQR 2912-6827] at 1 year (<i>p</i> = 0.008). In conclusion, our study demonstrated the potential clinical value of wearable devices by showing that activity and sleep quality are reduced in PAH compared to matched controls and these measures decline over time. Future studies should investigate if monitoring these health behaviors detects early functional decline and whether targeted interventions may improve outcomes.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70069"},"PeriodicalIF":2.2,"publicationDate":"2025-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12003557/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144042571","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":"Editorial on \"Developing Benchmarks in the Diagnosis and Treatment of Pulmonary Arterial Hypertension in a Tertiary, Academic Medical Center\".","authors":"Robert P Frantz","doi":"10.1002/pul2.70081","DOIUrl":"10.1002/pul2.70081","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70081"},"PeriodicalIF":2.2,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12000534/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144043522","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":"Importance of age at diagnosis of pulmonary hypertension in children living at high altitude: Longitudinal follow-up of 86 patients.","authors":"Gabriel F Diaz, Carlos E Diaz-Castrillon, Alicia Marquez Garcia, Rachel K Hopper, Vinicio de J Perez","doi":"10.1002/pul2.70017","DOIUrl":"10.1002/pul2.70017","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) at high altitudes presents unique characteristics due to hypobaric hypoxia. We aimed to evaluate the association between early diagnosis and clinical outcomes among children with severe PH living at high altitudes. A retrospective analysis was conducted on 86 children, divided into three age groups at the time of diagnosis: ≤3, 3-10, and ≥10 years. The median age at diagnosis was 4 years, with 48% of patients under 3 years old. Over a median follow-up of 6 years (interquartile ranges 2.5-8), 10% had a follow-up exceeding 15 years. Mortality rates were lowest in those diagnosed before age 3 (22% vs. 48% vs. 25%, <i>p</i> = 0.06). Responders to the prolonged hyperoxia test (PHT) were younger and had a significantly lower mortality hazard ratio (0.23, 95% confidence interval 0.08-0.70; <i>p</i> = 0.01). Early detection of PH in children living at high altitudes is associated with a higher likelihood of having a positive response to the PHT and subsequently, lower mortality rates. These findings underscore the importance of early diagnosis in improving long-term outcomes for this population.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70017"},"PeriodicalIF":2.2,"publicationDate":"2025-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11994476/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144049884","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":"Resolution of Severe PAH After Late VSD Closure in an 8-Year-Old: Clinical Prudence or Adventurism?","authors":"Saurabh Kumar Gupta, Shyam S Kothari","doi":"10.1002/pul2.70077","DOIUrl":"10.1002/pul2.70077","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70077"},"PeriodicalIF":2.2,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11978727/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144021546","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":"Assessment of the Relationship Between the Six-Minute Walk Test (6MWT) With Serum Chloride Level and Mean Pulmonary Arterial Pressure in Patients With Pulmonary Arterial Hypertension.","authors":"Sepideh Hejazi, Maryam Masinaei, Maryam Emadzadeh, Shima Baniassad, Sara Amini, Soroush Attaran, Mohammad Javad Najafzadeh, Davood Attaran, Amir Baniasad","doi":"10.1002/pul2.70066","DOIUrl":"10.1002/pul2.70066","url":null,"abstract":"<p><p>Pulmonary artery hypertension (PAH) is a fatal disease associated with high mortality, especially in countries with limited health resources in terms of lack of access to diagnostic and therapeutic evaluations. Therefore, it is necessary to discover inexpensive and available serum biomarkers for examining patients. This study investigates the relationship between PAH patients' six-minute walk (6MWT) distance, serum chloride levels, and mean pulmonary arterial pressure (mPAP). In this cross-sectional study, patients with PAH referring to the pulmonary hypertension clinic of our tertiary hospital were included. Then, the patient's demographic information and clinical findings were recorded, and the serum level of chloride and the 6MWT were examined in the patients. In the present study, 70 PAH patients were evaluated. All patients were female, and the mean age of the patients was 39.44 ± 8.33 years old. Hypochloremia was considered as serum chloride < 97 mmol/L in our study. The mPAP of patients with hypochloremia was significantly higher than non-hypochloremia patients (<i>p</i> < 0.001). The serum chloride levels had a significant positive correlation with the 6MWT distance (<i>r</i> = 0.634, <i>p</i> < 0.001). According to the linear regression analysis results, serum chloride level was a significant predictor of 6MWT distance even after adjustment for age and creatinine (<i>β</i> = 0.48; <i>p</i> = 0.002). Serum chloride level can be used as an inexpensive method for the evaluation of disease severity in PAH patients, especially in patients with higher time since the diagnosis of PAH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70066"},"PeriodicalIF":2.2,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11973496/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143804204","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":"Shedding Light on the Dark Side of the Moon-Understanding Pediatric Right Ventricular Work in Pulmonary Hypertension.","authors":"Taylor P Saley, Ryan D Coleman","doi":"10.1002/pul2.70070","DOIUrl":"10.1002/pul2.70070","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70070"},"PeriodicalIF":2.2,"publicationDate":"2025-04-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11972546/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143796083","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":"Editorial on \"Elective Thoracic Surgical Resections for Pulmonary Arteriovenous Malformations-A 16 Year Single-Center Experience\".","authors":"Raghav Murthy","doi":"10.1002/pul2.70073","DOIUrl":"10.1002/pul2.70073","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70073"},"PeriodicalIF":2.2,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11964944/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143781108","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":"Pediatric Pulmonary Hypertension is Associated With Increased Circulating Levels of BMP 7 and CHIP.","authors":"Edward C Kirkpatrick, Stephanie Handler, Melodee Liegl, Amy Y Pan, G Ganesh Konduri, Todd M Gudausky, Adeleye J Afolayan","doi":"10.1002/pul2.70068","DOIUrl":"10.1002/pul2.70068","url":null,"abstract":"<p><p>Pulmonary arterial endothelial and smooth muscle cell homeostasis is regulated through the bone morphogenetic protein (BMP) and transforming growth factor beta (TGF-β) receptor pathways. Pathway imbalance results in pulmonary hypertension (PH). Each pathway has ligands and modulators influencing this balance. How these pathways differ in pediatric PH patients is unknown. Ten PH and 20 control subjects (ages 2-17 years) were prospectively enrolled. Pulmonary artery serum BMP 2, 4, 6, 7, 9, 10, activin A, TGF-β1, carboxyl terminus of Hsc70-interating protein (CHIP), NT Pro BNP, and CRP were measured by ELISA. Analyses were made using the Fisher's exact test, the Mann-Whitney test, ROC analysis, and Pearson and Spearman correlations as appropriate. PH subjects were group 1 (four with simple shunts) or group 3 PH. Control subjects had shunts scheduled for catheter closure but no PH. Only BMP 7 and CHIP levels were statistically elevated in PH patients versus controls; (BMP 7 0.081(0.076-0.084) vs. 0.074(0.069-0.08) OD, <i>p</i> = 0.044), (CHIP 0.17(0.14-0.24) vs. 0.13(0.12-0.15) OD, <i>p</i> = 0.007) respectively. BMP 7 levels correlated with RV systolic pressure (0.431, <i>p</i> = 0.02) and pulmonary resistance (0.446, <i>p</i> = 0.013). CHIP correlated with mean pulmonary artery pressure (0.449, <i>p</i> = 0.013) and resistance ratios (Rp/Rs) (0.419, <i>p</i> = 0.02). BMP 7 OD of 0.077 had sensitivity/specificity of 80% and 70% for PH. CHIP OD of 0.136 had sensitivity/specificity of 90% and 65% for PH. BMP 7 and CHIP levels are heightened in pediatric PH patients which correlate with catheterization values. BMP 7 and CHIP could provide sensitive markers for PH to aid in diagnosis and disease monitoring.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70068"},"PeriodicalIF":2.2,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11964942/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143781135","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":"Hank the Heart and Animation-Based Education for Pulmonary Hypertension.","authors":"Michelle Cash, Barbara Giambra, Lara Stone, Melissa Magness, Kimberly Luebbe, Samantha Moore, Ryan Moore, Russel Hirsch, Kimberley Miles, Michael Kim, Paul J Critser","doi":"10.1002/pul2.70072","DOIUrl":"10.1002/pul2.70072","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is a rare, complex disease affecting both children and adults. Efforts to provide health education are imperative as health literacy impacts patient and caregiver capacity to acquire, process, and understand health information and make informed health decisions. The current study sought to understand patient and family perception of \"Hank the Heart\", an animation used for enhancing health literacy, and preferred learning methods for future educational preferences. A qualitative study was conducted to assess the \"Hank the Heart\" animation using focus groups of family caregivers and patients. Focus group and interview transcripts underwent inductive thematic analysis. Patients and families have complicated health and educational needs that require a personalized approach, and they identified with the animated character as a representation of their reality. We identified three primary themes: (1) An animated video was found to be helpful but not sufficient to address all caregiver and patient educational needs, (2) Parents and children resonated strongly with elements of the video that represent their own experiences, and (3) Patient and family centered ongoing and tailored education is needed. The use of animation videos as a form of education was well received. Patient and family insights and suggestions were offered for improving future animation development.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70072"},"PeriodicalIF":2.2,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11964943/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143781130","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}