Pulmonary Circulation最新文献

{"title":"The Emergence of Oral Sildenafil for Pulmonary Hypertension Management.","authors":"Ghazwan Butrous","doi":"10.1002/pul2.70150","DOIUrl":"10.1002/pul2.70150","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70150"},"PeriodicalIF":2.5,"publicationDate":"2025-08-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12321954/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789830","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":"A Call for a Better PoPH Risk Assessment Model, and Treatment Direction.","authors":"Yu Kuang Lai","doi":"10.1002/pul2.70147","DOIUrl":"10.1002/pul2.70147","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70147"},"PeriodicalIF":2.5,"publicationDate":"2025-08-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12321956/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789828","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":"My Quest for Calcium Channel Blockers as the First Medical Treatment for Patients With Pulmonary Hypertension.","authors":"Stuart Rich","doi":"10.1002/pul2.70151","DOIUrl":"10.1002/pul2.70151","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70151"},"PeriodicalIF":2.5,"publicationDate":"2025-08-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12321952/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789829","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":"Characterization of the Population With Intermediate-Risk Status in Pulmonary Arterial Hypertension: Patients in a Latin American Country: The CAPRI Registry.","authors":"R Conde-Camacho, M Orozco-Levi, A Londoño, R Gómez-Palau, E Tuta-Quintero, A Naranjo, K Díaz, J De Luque, S Goldfeder, L F Giraldo-Cadavid, M C Guerrero","doi":"10.1002/pul2.70145","DOIUrl":"10.1002/pul2.70145","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) in intermediate-risk patients poses a challenge for clinicians, particularly in determining the optimal timing for escalating pharmacological treatment. We conducted a multicenter cross-sectional study that analyzed data from the Colombian Pulmonary Hypertension Network (HAPredCO) on patients diagnosed with PAH. Participants were stratified into low- and high-intermediate risk groups using the four-level ESC/ERS score, which incorporates optimized cutoff values for WHO functional class (WHO FC), the 6-min walk test (6MWT), and N-terminal pro-brain natriuretic peptide (NT-proBNP)/brain natriuretic peptide (BNP) concentration. A bivariate analysis was conducted to compare study variables at baseline and during clinical follow-up. Additionally, a survival analysis was performed using Kaplan-Meier curves to compare outcomes between the intermediate-risk groups. A total of 175 patients were analyzed, with a mean age of 44.85 years (SD 15.5), of which 86% (150/175) were women. In the baseline, a total of 75% (131/175) were classified as intermediate-low-risk. In clinical follow-up, 40% (61/154) were classified as intermediate-low-risk, 21% (32/154) were classified as intermediate-low-risk, 23% (35/154) were classified as intermediate-low-risk, and 16% (26/154) as intermediate-high-risk. At baseline, WHO FC III was 61% (107/174), compared to 35% (56/154) at follow-up (<i>p</i> = 0.083). The use of bosentan decreased from 36% (63/175) to 28% (46/154) (<i>p</i> < 0.001), while ambrisentan increased from 18% (31/175) to 30% (47/154), and macitentan increased from 25% (44/175) to 36% (57/154). The use of parenteral prostanoids and selexipag increased by 20% (4% vs. 24%) and 3% (1% vs. 4%) during clinical follow-up, respectively. Overall survival at 1 year of follow-up was 96%, at 2 years 92%, and at 3 years 88%. The survival rates at 1, 2, and 3 years were 98%, 95%, and 90% in the intermediate-low-risk group, and 90%, 80%, and 80% in the intermediate-high-risk group. In this real-world study of PAH in the Colombian HAPredCO registry, the median survival in the intermediate-low-risk group was slightly higher than in the intermediate-high-risk group. At baseline, most patients were classified as intermediate-low-risk. However, during clinical follow-up, risk distribution changed, with a notable proportion shifting across different risk categories.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70145"},"PeriodicalIF":2.5,"publicationDate":"2025-08-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12318366/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144776061","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Role of the Pulmonary Vascular Microenvironment in Chronic Thromboembolic Pulmonary Hypertension.","authors":"Lu Sun, Han Tian, Jixiang Liu, Min Liu, Yuhan Li, Haobo Li, Ran Miao, Yunxia Zhang, Wanmu Xie, Zhu Zhang, Shiqing Xu, Peiran Yang, Zhenguo Zhai","doi":"10.1002/pul2.70118","DOIUrl":"10.1002/pul2.70118","url":null,"abstract":"<p><p>Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by persistent obstruction and vascular remodeling of the pulmonary arteries following pulmonary thromboembolism (PTE), diagnosed after a minimum of 3 months of therapeutic anticoagulation. Disease progression from PTE to CTEPH takes place in the pulmonary circulation, where the vascular microenvironment is composed of a fluid portion that includes blood cells and components of the fibrinolytic system, and various vascular cells, including endothelial and smooth muscle cells. Following PTE, the homeostasis of the pulmonary vascular microenvironment is disrupted, leading to the accumulation of inflammatory mediators and immune cells at the site of thrombosis. Platelets are also involved in the regulation of coagulation and inflammation, and functional changes such as impaired fibrinolysis are observed. Subsequently, endothelial cell dysfunction and smooth muscle cell dysregulation lead to delayed thrombus resolution and pulmonary vascular remodeling, eventually resulting in CTEPH. Early intervention targeting the aberrant vascular microenvironment may thwart or mitigate the transition from PTE to CTEPH. Here, we discuss the development of CTEPH from the perspective of the pulmonary vascular microenvironment and examine its related biomarkers and therapeutic methods for CTEPH diagnosis and treatment.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70118"},"PeriodicalIF":2.5,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12314654/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144776062","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":"Pharmacologic Management of Segmental Pulmonary Hypertension in Children After Unifocalization and Pulmonary Artery Reconstruction: Initial Experience.","authors":"Julian E Cameron, Doff B McElhinney, Esther Liu, Rachel K Hopper, Ritu Asija, Manchula Navaratnam, Frank L Hanley, Jeffrey A Feinstein","doi":"10.1002/pul2.70134","DOIUrl":"10.1002/pul2.70134","url":null,"abstract":"<p><p>Segmental pulmonary hypertension (PH) in congenital heart disease remains poorly understood with data limited to case studies. We performed a retrospective, single center study in children treated with PH medications after unifocalization/pulmonary artery reconstruction for major aortopulmonary collaterals (MAPCA). Drug response was determined by hemodynamic changes across at least two cardiac catheterizations. Mechanical properties of the segmental arteries were quantified by distensibility, stiffness, and augmentation indices. Twenty-five patients were included (8 surgical shunt, 17 complete repair), with 76% considered responsive to PH medications based on the relative decrease in maximum segmental mean pulmonary artery pressure (mPAP). At a median duration of 14 months (Q1-Q3 9.5-29), mPAP decreased from 33 mmHg (28-38) to 23 mmHg (21-32) (<i>p</i> < 0.001) with no significant change in blood flow distribution by lung perfusion scintigraphy. Subgroup analysis demonstrated a trend towards a larger percent decrease in mPAP of 35% (18-45) on dual therapy compared to 23% (Q1-Q3 3-36) on monotherapy (<i>p</i> = 0.16). In repaired patients, arterial distensibility at initial catheterization correlated with residual elevation in mPAP at follow-up (<i>R</i> ² 0.687, <i>p</i> < 0.001), with distensibility < 1.7%/mmHg associated with treatment failure. Among the lowest distensibility values were patients with <i>JAG1</i> mutations, and among patients with extended follow-up, progressive increase in mPAP was identified only in those with hereditary PH-associated mutations. Children with segmental PH following pulmonary artery reconstruction can be successfully treated with PH medications. Although treatment efficacy may be limited in patients with high vascular stiffness and those with pathologic vascular mutations.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70134"},"PeriodicalIF":2.5,"publicationDate":"2025-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12309978/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144754130","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":"Development of a Novel, Pulmonary Endovascular Device to Treat Patients With Pulmonary Hypertension.","authors":"Karl Vollmers, John Scandurra, Joshua R Woolley, Guruprasad A Giridharan, Alexander M K Rothman, Marc Pritzker, E Kenneth Weir, Christian Gerges, Irene Lang","doi":"10.1002/pul2.70131","DOIUrl":"10.1002/pul2.70131","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) carries a poor prognosis and a high mortality. Loss of pulmonary arterial compliance (PAC) plays a significant role in the development of PH and is an early predictor of mortality. Currently, there are no therapeutic options to overcome the loss of PAC. Aria CV (Saint Paul, MN) has developed a device to augment PAC. The device consists of a 20-cc balloon and anchor that can be implanted in the pulmonary artery using a minimally invasive procedure, a catheter, and a gas reservoir. Computed tomography imaging of 46 patients from the ASPIRE database and cadaver studies (<i>n</i> = 7) were used to ascertain device fit and optimize surgical procedure. Aria CV devices (<i>n</i> = 6) were tested for simulated use, durability, and PAC augmentation. Animal studies were conducted to demonstrate device safety in the deflated state (<i>n</i> = 9), and gas embolism due to simulated balloon rupture (<i>n</i> = 5). A chronic bovine model of PH was used to demonstrate PAC augmentation (<i>n</i> = 3). Chronic animal studies (<i>n</i> = 8, 30-days) were conducted to demonstrate long-term device safety and biocompatibility per ISO 10993 standards. In-silico fit and cadaver studies demonstrated that the device could be successfully implanted in the PA for a wide range of patients. In vitro and bovine models of PH demonstrated that the chronic Aria CV device enhanced PAC by > 0.4 ml/mmHg, which matched the PAC enhancement observed in 28 human patients with a short-term Aria CV device. The device passed all required durability, safety, and biocompatibility testing and is enrolling patients in a Food and Drug Administration (FDA) approved clinical trial (ASPIRE PH, NCT04555161).</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70131"},"PeriodicalIF":2.5,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307243/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144754129","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":"Reassessment of the Current Iron Deficiency Definition in Pulmonary Hypertension.","authors":"Aurelia E Reiser, Markus Thiersch, Max Gassmann, Martina U Muckenthaler, Thomas Geiser, Mona Lichtblau, Silvia Ulrich","doi":"10.1002/pul2.70142","DOIUrl":"10.1002/pul2.70142","url":null,"abstract":"<p><p>Iron deficiency (ID) is prevalent in pulmonary hypertension(PH), but there is no consensus on ID definition and its possible correlation to prognostic markers. Hence, in this study, PH-patients were recruited at the University Hospital Zurich from May 2019 to April 2021. Clinical and hemodynamic characteristics were recorded at inclusion and venous blood samples were taken. ID was defined as: (i) ferritin-ID: ferritin< 100 µg/L or 100-299 µg/L plus a transferrin saturation (TSAT) < 20%; (ii) TSAT-ID: a TSAT < 20% (males)/< 15% (females) and (iii) TFRI-ID: a transferrin receptor index (TFRI) > 3.2/ > 2.0 depending on CRP < / > 5 mg/L. 94 patients (52% female, mean age 62.9 ± 14.6 years) with pulmonary arterial hypertension(48%), PH associated with lung disease (20%) or chronic thromboembolic PH (32%) were included. Sixty-seven percent fulfilled criteria for ferritin-ID, 35% for TSAT-ID, and 13% for TFRI-ID. Mean pulmonary arterial pressure (mPAP) was elevated in TFRI-ID patients compared to non-ID (50 ± 12.2 mmHg vs. 35.9 ± 11.7 mmHg); however, after correction for age, sex, PH-type, and anticoagulation, the difference was nonsignificant (<i>p</i> = 0.085). NT-proBNP was significantly higher in TFRI-ID-positive (1237 ± 1166 pg/mL vs. 334 ± 417 pg/mL, <i>p</i> = 0.004). No significant differences were found for ferritin-ID and TSAT-ID (<i>p</i> > 0.05). Six-minute walk distance (6MWD) was reduced for both TSAT-ID (402 ± 133 m vs. 469 ± 152 m, <i>p</i> = 0.006) and TFRI-ID (370 ± 112 m vs. 459 ± 151 m, <i>p</i> = 0.052), but not for ferritin-ID (<i>p</i> > 0.05). In conclusion, TFRI-ID is seemingly associated with clinical markers of right heart parameters and disease severity. This could not be seen with the currently recommended ferritin-ID-definition or TSAT-ID. More data is needed to assess the use of the TFRI-ID instead of the ferritin-ID-definition as a method to identify PH-patients at risk and as a threshold for iron substitution.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70142"},"PeriodicalIF":2.5,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12305916/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144744484","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":"Important Differences in Disease Severity, Echocardiography Findings, and Referral Delays in Non-Hispanic Black Patients With Pulmonary Arterial Hypertension.","authors":"Kelsey Holbert, Ashar Usmani, Solomon Krow, Benjamin Follman, Kumar Lal, Dustin R Fraidenburg","doi":"10.1002/pul2.70138","DOIUrl":"10.1002/pul2.70138","url":null,"abstract":"<p><p>Awareness of health disparities that exist across different self-identified racial and ethnic groups are essential to developing interventions that improve the quality of care of patients with rare diseases such as pulmonary arterial hypertension (PAH). We sought to determine whether there are important differences in clinical characteristics and illness severity at the time of PAH diagnosis among different racial/ethnic groups. 110 patients followed at the University of Illinois Health Pulmonary Hypertension Clinic diagnosed with PAH between 2010 and 2019 were enrolled in our retrospective cohort study. Self-reported race, ethnicity, ZIP code, and standard clinical measures were obtained from the electronic medical record. Comparisons of clinical severity, hemodynamic measurements, social vulnerability, income, and timing of diagnostic testing were made between non-Hispanic Black and non-Black subjects. Our data shows that PAH is more severe at the time of diagnosis in non-Hispanic Black patients compared to non-Black patients, by both clinical and hemodynamic assessments. Tricuspid regurgitant velocity correlated poorly with invasive hemodynamics in non-Hispanic Black patients, yet measures of RV performance were worse than non-Black counterparts. Increased social vulnerability and income inequality was evident between the groups. When compared to non-Black patients, there were significant delays between abnormal echocardiogram findings and completion of diagnostic catheterization. These results implicate concerning health disparities in non-Hispanic Black patients with PAH. More severe disease at time of diagnosis and longer delays from time of symptom onset to PAH diagnosis have both been associated with increased mortality in this population and future work should be aimed at comprehensive strategies to reduce this disparity.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70138"},"PeriodicalIF":2.5,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12302280/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144732968","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":"Pericardial Effusion and Prostacyclin Analog Toxicity After Initiation of Sotatercept.","authors":"Dany Tager, Kristin B Highland, Kulwant S Aulak, Leora Haber, Adriano R Tonelli","doi":"10.1002/pul2.70141","DOIUrl":"10.1002/pul2.70141","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a disorder characterized by progressive remodeling of small pulmonary arteries, leading to increased pulmonary vascular resistance, right ventricular failure and premature death (1-2). Over the past 30 years, significant advancements have been made in the treatment of PAH, including the recent approval of sotatercept, a first-in-class fusion protein that acts as a ligand trap for activins and growth differentiation factors, which are key players in the transforming growth factor β (TGF-β) superfamily (3-4). Sotatercept improves exercise capacity, as assessed by 6-min walk distance and World Health Organization (WHO) functional class, reduces pulmonary vascular resistance and NT-pro brain natriuretic peptide, and improves the simplified French risk score while extending the time to death or nonfatal clinical worsening (3). The 7th World Symposium in pulmonary hypertension recommends the addition of sotatercept as an option in PAH patients who have not achieved low risk despite combination therapy with at least an endothelin receptor antagonist and phosphodiesterase type-5 inhibitor, in intermediate or high-risk patients. The STELLAR study of sotatercept in PAH patients demonstrated the efficacy of this medication in patients receiving background therapy (3). In fact, 34% of the patients were on double and 60% of the patients on triple therapy. Interestingly 40% were on prostacyclin infusion therapy (3). Post-hoc analysis of the stellar study showed a beneficial effect for those on double or triple background PAH therapy as well as those receiving prostacyclin infusion at baseline (1,5). It remains unclear if the addition of sotatercept to other PAH treatments may have unexpected complications. It is possible that by rebalancing the proliferative/antiproliferative effects in the pulmonary circulation, the effect of other treatments for PAH may become excessive, particularly when parenteral prostacyclin is used at high doses. This phenomenon may manifest with the typical characteristics of prostacyclin overdose, including enhanced side effects and high cardiac output heart failure. Hereby we describe a patient with PAH on triple PAH-specific therapy, who after the initiation of sotatercept developed a large pericardial effusion and high cardiac output heart failure.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70141"},"PeriodicalIF":2.5,"publicationDate":"2025-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12296709/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144732969","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}