Pulmonary Circulation最新文献

{"title":"Scimitar Syndrome and Pulmonary Hypertension in Pediatric Population: A Retrospective Analysis of the Prevalence, Clinical Characteristics, Risk Factors, and Outcomes.","authors":"Abdullah Alkhani, Abdulrahman Bendahmash, Albara Arefi, Meshari Alquayt, Raghad Alhuthil, Hanaa Banjar, Mohammed Alhabdan, Dimpna Albert-Brotons","doi":"10.1002/pul2.70284","DOIUrl":"https://doi.org/10.1002/pul2.70284","url":null,"abstract":"<p><p>Pulmonary hypertension is a known complication of scimitar syndrome; however, its risk factors and outcomes are not well understood. This study aimed to assess the prevalence, clinical features, risk factors, and outcomes of pulmonary hypertension in patients with scimitar syndrome. In this retrospective cohort study, patients diagnosed with scimitar syndrome and treated at a tertiary center between June 2015 and June 2024 were reviewed. Pulmonary hypertension was defined as a mean pulmonary arterial pressure > 20 mmHg based on cardiac catheterization. PH developed in 77.5% of pediatric SS patients. PVR and PCWP differed significantly across hemodynamic characteristics (<i>p</i> = 0.038) (<i>p</i> = 0.001), respectively with 67.7% of PH patients exhibiting a pre-capillary phenotype. Patients selected for surgery demonstrated higher baseline severity, including greater Qp:Qs ratio (1.99 vs. 1.49, <i>p</i> = 0.033) and more frequent ASDs (57.1% vs. 8.3%, <i>p</i> = 0.005). Resolution was achieved in 74.2% of patients with longer resolution time found in surgical cases (median 8.1-12.3 years) compared to non-surgical cases (3.2 years). Of the 8 patients with persistent PH, 25% (<i>n</i> = 2) were found to have pathogenic variants associated with non-mechanical factors linked to PH in SS. PH is highly prevalent in patients with scimitar syndrome, particularly in those with infantile type and congenital heart defects. While the majority of patients achieve resolution, the extended timeline in surgical cases reflects the higher baseline severity and complexity, along with advanced vascular remodeling. 25% of persistent PH cases harbored pathogenic variants that may contribute to a biological \"second hit\" that is linked to therapy resistance. Further multicenter and prospective studies are warranted to expand on these findings.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 1","pages":"e70284"},"PeriodicalIF":2.5,"publicationDate":"2026-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13085898/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147723637","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":"Sotatercept in Patients With Eisenmenger Syndrome.","authors":"Oktay Tutarel, Oliver Miera, Felix Berger","doi":"10.1002/pul2.70283","DOIUrl":"https://doi.org/10.1002/pul2.70283","url":null,"abstract":"<p><p>Eisenmenger syndrome represents a complex and severe form of pulmonary arterial hypertension (PAH) associated with congenital heart disease. Sotatercept, a first-in-class activin-signaling inhibitor, offers a novel therapeutic approach in the treatment of PAH, and has emerged as a promising therapeutic agent. However, patients with Eisenmenger syndrome were not included in its pivotal trials. Therefore, data regarding its effect in this patient cohort is lacking. We present two cases of patients with Eisenmenger syndrome treated with sotatercept. Sotatercept improved the subjective and objective exercise capacity in both patients without any significant adverse side effects.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 1","pages":"e70283"},"PeriodicalIF":2.5,"publicationDate":"2026-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12974322/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147435021","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":"Resting Oxygen Consumption Estimates in Scleroderma Can Lead to Underestimation of Cardiac Output.","authors":"Oscar Cullen, Laura Ross, Jessica L Fairley, Luke W Spencer, Amy M Mitchell, Stephanie J Rowe, Kristel Janssens, Youri Bekhuis, Stephen J Foulkes, Paolo D'Ambrosio, Margarita Calvo-Lopez, Kaitlin Newcomb, Andre La Gerche, Andrew T Burns","doi":"10.1002/pul2.70280","DOIUrl":"https://doi.org/10.1002/pul2.70280","url":null,"abstract":"<p><p>Accurate resting oxygen consumption (rVO₂) quantification is critical for Fick-derived cardiac output calculations. Yet, clinical practice predominantly uses empirical estimations, which can be inaccurate. We evaluated the established rVO₂ prediction equations against direct metabolic cart measurements in systemic sclerosis patients, revealing significant discordances with potential diagnostic implications.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 1","pages":"e70280"},"PeriodicalIF":2.5,"publicationDate":"2026-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12974325/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147434964","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":"Time to Therapy Activation and Initial Combination Therapy (TITANIC) for Patients With Pulmonary Arterial Hypertension.","authors":"Cyrus Kholdani, Carly J Paoli, Tyler Peck, Wenze Tang, Noah Schoenberg, Sumeet Panjabi, David Furfaro","doi":"10.1002/pul2.70255","DOIUrl":"https://doi.org/10.1002/pul2.70255","url":null,"abstract":"<p><p>Patients with pulmonary arterial hypertension (PAH) experience long delays in diagnosis; however, little is known about the time between diagnosis and initiation of guideline-recommended optimal treatment. Immediate therapy is imperative for optimizing patient outcomes, as much of the pulmonary vasculature has already deteriorated by the time of diagnosis. This study used real-world U.S. data to investigate the time from the most recent right heart catheterization (RHC) to initiation of the first PAH-specific treatment. Adults with PAH were identified in the Komodo Health database using claims for RHC, pulmonary hypertension diagnostic codes, and PAH medications (1/1/2016-4/1/2024). Time from RHC to medication dispensing was examined and categorized by initial treatment regimen (monotherapy, dual therapy, and triple therapy). Baseline characteristics and treatment patterns were assessed. Among 7952 patients, the median time from most recent RHC to first PAH medication was 43.0 days. Most patients (77.7%) initially received monotherapy, with 20.0% and 2.3% receiving upfront dual and triple therapy, respectively. Cardiac comorbidities were present in most patients (85.8%). Among 2965 (48.0%) upfront monotherapy users who added another treatment during follow-up, the median time to dual therapy was 39 days. Of those who escalated to dual therapy, 567 (19.1%) eventually escalated to triple therapy, with a median time from dual therapy to triple therapy of 62 days. In real-world practice, delays between PAH diagnosis and initiation of optimal therapy remain significant, and barriers to access should be addressed.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 1","pages":"e70255"},"PeriodicalIF":2.5,"publicationDate":"2026-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12974550/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147435034","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":"Longitudinal Remote 6-Minute Walk Distance and Cardiac Effort Using Wearable Sensors in Pulmonary Hypertension.","authors":"Daniel Lachant, Nishit Agarwal, Kyle Norton, Deborah Haight, Cameron McCarthy, Sebastian Alphonse, Melissa Ceruolo, R James White","doi":"10.1002/pul2.70278","DOIUrl":"10.1002/pul2.70278","url":null,"abstract":"<p><p>Remote 6-min walk testing (6MWT) has been shown to be safe in pulmonary hypertension, but limited data exist on its longitudinal durability and relationship to supervised in-clinic testing. Cardiac Effort (CE), defined as total heartbeats divided by walk distance, provides physiologic context beyond distance alone. We evaluated the longitudinal performance of a home-based 6MWT and CE performed on a modified indoor walking space using a wearable chest sensor and compared results with in-clinic testing over 8 weeks. This single-center prospective study was conducted with institutional review board approval. Participants with PH performed supervised in-clinic and unsupervised home 6MWTs at baseline and Week 8. A chest-based wearable sensor recorded acceleration and heart rate data to estimate walk distance and calculate CE. Mixed-effects repeated-measures models were used to compare sensor-estimated and directly observed walk distance and CE in the clinic and at home over time. Agreement was assessed using Bland-Altman analyses, intraclass correlation coefficients (ICC), and Spearman correlations. Twenty-nine participants were enrolled, and no adverse events occurred. Sensor-estimated 6MWT distance and CE demonstrated minimal bias and excellent agreement with directly observed in-clinic measurements (6MWD bias 0.04%, ICC 0.995; CE bias -0.04%, ICC 0.995). Home-based 6MWD was approximately 40 m lower than supervised clinic testing. Mixed-effects models showed similar longitudinal changes between home and clinic testing. Changes in home 6MWD and CE correlated with corresponding in-clinic changes (6MWD: <i>r</i> = 0.40, <i>p</i> = 0.045; CE: <i>r</i> = 0.42, <i>p</i> = 0.03). Home-based 6MWT and CE obtained using a wearable chest sensor provide a safe, feasible method for remote functional assessment in PH that tracks with in-clinic testing. Frequent home assessment may facilitate earlier detection of clinical change, support therapeutic titration, and expand access to standardized physiologic monitoring.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 1","pages":"e70278"},"PeriodicalIF":2.5,"publicationDate":"2026-03-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12967498/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147390826","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":"Sotatercept for Decompensated Pulmonary Arterial Hypertension Requiring VA ECMO: First Canadian ICU Experience.","authors":"Jonathan Taylor, Eddy Fan, John Thenganatt, John Granton","doi":"10.1002/pul2.70276","DOIUrl":"https://doi.org/10.1002/pul2.70276","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a rare and progressive disease resulting in increased workload of the right ventricle (RV). Despite advances in therapies, PAH remains highly morbid from a progressive vasculopathy and RV failure. For patients admitted to hospital with RV failure, transplant remains the only option for a select few. Sotatercept, a novel activin signaling inhibitor approved for the treatment of PAH, has demonstrated significant improvement in clinical outcomes across a wide spectrum of disease severity. However, descriptions of its use to treat acute RV failure in an intensive care unit (ICU) setting are limited. We report a case of cardiogenic shock and respiratory failure secondary to decompensated PAH requiring veno-arterial extracorporeal membrane oxygenation (VA ECMO), with rapid improvement following sotatercept initiation.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 1","pages":"e70276"},"PeriodicalIF":2.5,"publicationDate":"2026-03-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12967482/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147390839","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":"Industry Payments and Prescribing Patterns of Pulmonary Hypertension Therapies in the United States.","authors":"Elizabeth S Tarras, Anju Murayama, Inderjit Singh, Lina M Freeman, Hinari Kugo, Deborah C Marshall","doi":"10.1002/pul2.70279","DOIUrl":"https://doi.org/10.1002/pul2.70279","url":null,"abstract":"<p><p>This study analyzed the US Open Payments and Medicare Part D databases from 2014 to 2022 to assess associations between industry-physician payments and PAH therapy prescribing. Among 5502 pulmonologist-, cardiologist- and pediatrician-prescribers of PAH therapies, drug-specific industry payments were associated with dose-response increases in Medicare spending.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 1","pages":"e70279"},"PeriodicalIF":2.5,"publicationDate":"2026-03-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12967594/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147390801","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 Pulmonary Hypertension Global Patient Survey: Physical and Psychosocial Impacts on Health-Related Quality of Life.","authors":"Katherine Bunclark, Joseph Newman, Hakim Ghani, Shiv Munagala, Eva Otter, Gerald Fischer, Marcin Kurzyna, Gergely Meszaros, Millicent Stone, Shahin Moledina, Luke Howard, Wendy Gin-Sing, Pisana Ferrari, Maurice Beghetti, Mark Toshner, Matt Granato, Joanna Pepke-Zaba","doi":"10.1002/pul2.70264","DOIUrl":"https://doi.org/10.1002/pul2.70264","url":null,"abstract":"<p><p>The Pulmonary Hypertension (PH) Global Patient Survey provides the first truly international description of the symptomatic burden of PH and its treatment on patient's health-related quality of life (HRQoL). From 3329 adult responses across 88 countries, the \"invisible\" nature of PH is an over-arching theme, with repercussions on socialisation, relationships and employment. Emotional burden is common with 35.6% feeling misunderstood, 33.4% angry or frustrated and 32.0% isolated. A reduced capacity for paid employment, in combination with healthcare/medication costs and difficulty in accessing social and financial support, compound societal exclusion and isolation. Physical limitations are almost universally reported (78.6%) with poor sleep quality (42.3%) the greatest factor in physical well-being. Treatment side-effects are common (46.7%), are greatest with parenteral therapies, but improve with age. Women of child-bearing age bear the brunt of PH physical and psychological burden, compounded by inconsistent messages from specialist services surrounding pregnancy and contraception. Patient-reported outcome measures (PROM) accurately reflect impacts from PH but are underutilised in clinical practice. Many respondents would like to be better able to discuss their emotional symptoms with their PH specialist (but often find a lack of empathy). Psychological support is a highly requested, but frequently unmet, need reinforcing the importance of holistic care models and multiagency working in managing patients with PH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 1","pages":"e70264"},"PeriodicalIF":2.5,"publicationDate":"2026-03-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12951358/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147348969","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 Pulmonary Hypertension Global Patient Survey: Understanding the Invisible Burden of Paediatric Pulmonary Hypertension.","authors":"Sadia Quyam, Sarah Drumm, Shiv Mungala, Katherine Bunclark, Gerald Fischer, Eva Otter, Hakim Ghani, Wendy Gin-Sing, Luke Howard, Marcin Kurzyna, Pisana Ferrari, Lynsay Macdonald, Joseph Newman, Millicent Stone, Mark Toshner, Joanna Pepke-Zaba, Maurice Beghetti, Shahin Moledina","doi":"10.1002/pul2.70269","DOIUrl":"10.1002/pul2.70269","url":null,"abstract":"<p><p>The Pulmonary Hypertension Global Patient Survey (PH GPS) provides the first international examination of experiences among children with pulmonary hypertension (PH) and their caregivers. Through an online survey distributed via PH associations across 32 countries, we collected responses from 136 caregivers about diagnostic journeys, treatment experiences, healthcare access and research participation. Qualitative analysis revealed challenges around the 'invisible' nature of PH, with caregivers describing how schools and communities sometimes doubted their child's limitations in the absence of visible symptoms, adding to family burden. Quantitative findings demonstrated extensive caregiver burden with 40% providing constant care while facing significant employment consequences, including leaving work altogether, reduced hours, or career changes. Although 78.8% of children accessed specialist centres, significant barriers persisted: 41.2% encountered diagnostic delays exceeding 6 months, 34.8% travelled over 2 h for appointments and 27.7% received no healthcare cost reimbursement. Treatment experiences revealed 37.7% of children experienced side effects, yet only one-third discussed changes with their clinical teams, reflecting pragmatic decisions within limited therapeutic options. Research participation was low (19.2%), though families with trial experience showed greater willingness for future participation, compared to those without experience (70.8% vs. 49.5%). Barriers centred on awareness gaps (16.4%), safety concerns (14.5%), and age-related considerations (10.9%), distinct from adult PH populations where logistical challenges predominate. These findings demonstrate that paediatric care must extend beyond medical management to address family needs including social-work assistance for benefits navigation, flexible care delivery models incorporating telemedicine, pro-active communication about treatment tolerability and research protocols designed with families from inception.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 1","pages":"e70269"},"PeriodicalIF":2.5,"publicationDate":"2026-03-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12951355/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147348973","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 Pulmonary Hypertension Global Patient Survey: Groups 2 and 3.","authors":"Katherine Bunclark, Joseph Newman, Hakim Ghani, Shiv Munagala, Eva Otter, Gerald Fischer, Marcin Kurzyna, Gergely Meszaros, Millicent Stone, Shahin Moledina, Luke Howard, Wendy Gin-Sing, Pisana Ferrari, Maurice Beghetti, Mark Toshner, Matt Granato, Joanna Pepke-Zaba","doi":"10.1002/pul2.70256","DOIUrl":"https://doi.org/10.1002/pul2.70256","url":null,"abstract":"<p><p>In this first global study of patient perspectives in Group 2 and 3 PH, we demonstrate that those with Group 3 PH report significantly greater adverse impacts on physical and mental wellbeing than those in Group 2 or Group 1, despite a lower treatment side-effect profile.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 1","pages":"e70256"},"PeriodicalIF":2.5,"publicationDate":"2026-03-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12951357/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147348940","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}