Pulmonary Circulation最新文献

{"title":"4D-Flow MRI-Derived Wall Shear Stress as a Non-Invasive Biomarker for the Detection and Hemodynamic Characterization of Pulmonary Hypertension.","authors":"Ben Statton, Chlöe Armour, Kai'en Leong, Jan Sedlacik, Alaine Berry, Ning Jin, Daniel Giese, Francesco Lo Guidice, Luke Howard, Deepa Gopalan","doi":"10.1002/pul2.70426","DOIUrl":"10.1002/pul2.70426","url":null,"abstract":"<p><p>Invasive right heart catheterization (RHC) remains the reference standard for diagnosing pulmonary hypertension (PH), but there is a pressing need for non-invasive techniques to assess pulmonary hemodynamics. This study evaluates 4D-flow MRI-derived wall shear stress (WSS) for PH detection using the updated 2022 hemodynamic criteria, and the prediction of right ventriculo-pulmonary arterial (RV-PA) uncoupling to assess disease severity. 3D WSS was measured in the main (MPA), right (RPA), and left (LPA) pulmonary arteries in a retrospective validation cohort (41 PH patients, 23 non-PH) and then assessed in a prospective test cohort (23 PH patients, 17 healthy controls). For methodological validation, a patient-specific computational fluid dynamics (CFD) was performed in a subset of 16 patients from the validation cohort. RV-PA uncoupling was assessed on the cine MRI, using RV global longitudinal strain (GLS) < -15.3% and RVSV/RVESV < 1. Both 3D peak and time-averaged WSS were significantly reduced in PH patients compared with healthy controls. Global WSS metrics demonstrated the strongest correlations with invasive hemodynamic parameters, including mPAP (<i>r</i> = -0.75, <i>p</i> < 0.001) and PVR (<i>r</i> = -0.76, <i>p</i> < 0.001). ROC curve analysis identified a peak WSS cutoff of < 0.30 Pa (AUC 0.98) for PH detection, a threshold achieving 90% sensitivity and 100% specificity in the test cohort. Furthermore, a peak WSS threshold of < 0.18 Pa demonstrated excellent discriminative ability for RV-PA uncoupling. These findings support the potential integration of WSS analysis into routine CMR protocols as a non-invasive complement to RHC, showing promise for both diagnosis and a longitudinal biomarker for monitoring PH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70426"},"PeriodicalIF":2.4,"publicationDate":"2026-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13678861/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149224793","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":"Identifying a Cardio-Hepatic Phenotype by Integrating the Albumin-Bilirubin Score Into Risk Assessment in Pulmonary Arterial Hypertension.","authors":"Hacer Ceren Tokgoz, Barkin Kultursay, Seda Tanyeri, Dicle Sırma, Cagdas Bulus, Tezel Kovanci, Mustafa Kara, Seyma Zeynep Atici, Seyma Nur Cicek, Caglar Emre Cagliyan, Azmican Kaya, Faruk Akalin, Dogan Sen, Leyla Aliyeva, Nihal Ozdemir, Cihangir Kaymaz","doi":"10.1002/pul2.70412","DOIUrl":"10.1002/pul2.70412","url":null,"abstract":"<p><p>Risk stratification is essential in pulmonary arterial hypertension (PAH), yet the prognostic utility of the albumin-bilirubin (ALBI) score as a marker of hepatic dysfunction remains underexplored. In this single-center study, we evaluated the clinical, neurohumoral, echocardiographic and hemodynamic correlates and prognostic value of the ALBI score, and to assess whether this score offers an incremental benefit over the Registry to EValuate Early And Long-term pulmonary arterial hypertension disease management (REVEAL) 2.0 risk score in patients with PAH. This study evaluated the prognostic value of the ALBI score and its incremental benefit in a cohort of 380 PAH patients (mean age 47.6 ± 18.3 years, 74.3% female). Patients were stratified into low (<= -2.60) and high (> -2.60) ALBI groups. High ALBI scores were significantly associated with worse functional class, reduced exercise capacity, elevated Nt-proBNP, right ventricular systolic dysfunction, and vena cava plethora (all <i>p </i>< 0.001). Hemodynamically, elevated ALBI correlated with increased right atrial pressure (RAP) (<i>p </i>= 0.005), reduced cardiac index (<i>p </i>= 0.002), and a narrowed trans-systemic pressure gradient (<i>p </i>= 0.001). Multivariable Cox regression demonstrated that ALBI was an independent predictor of mortality [Adjusted HR: 1.80 (95% CI: 1.30-2.49), <i>p </i>< 0.001], outperforming MELD variants and the FIB-4 index. Integrating ALBI into REVEAL 2.0 yielded higher risk discrimination (C-index: 0.756) than adding direct echocardiographic indices of right ventricular function or coupling (C-indices: 0.748-0.749). In sensitivity analyses adjusting for TAPSE/PASP, invasive cardiac index, mean RAP, and background therapy, ALBI maintained its independent prognostic value [Adjusted HR: 1.44 (95% CI: 1.01-2.05), <i>p </i>= 0.044], with the full model achieving a C-index of 0.801. Within the REVEAL 2.0 intermediate-risk subgroup, a high ALBI score identified patients with a three-fold higher mortality risk [HR: 3.04 (95% CI: 1.25-7.38), <i>p </i>= 0.014], improving discriminative performance by 26.3%. The ALBI score independently predicts mortality beyond standard pulmonary hemodynamics and echocardiographic right ventricular parameters. Incorporating ALBI into REVEAL 2.0 enhances survival prediction and aids risk refinement in intermediate-risk PAH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70412"},"PeriodicalIF":2.4,"publicationDate":"2026-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13674166/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149216644","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":"Pulmonary Haemodynamics After Mechanical Therapy With PEA and BPA Predict Survival in Patients With CTEPH - A Prospective Analysis From the National UK CURATE Registry.","authors":"Eilish Turner-Frick, Hakim Ghani, Paula Appenzeller, Liliana Amaral-Almeida, Katherine Bunclark, John Cannon, Karen Sheares, Mark Toshner, Dolores Taboada, Choo Ng, John Taghavi, Steven Tsui, Nick Screaton, Alessandro Rugierro, Luigia D'Errico, Paul Bambrough, J Gerry Coghlan, David P Jenkins, Joanna Pepke-Zaba, Stephen P Hoole","doi":"10.1002/pul2.70419","DOIUrl":"10.1002/pul2.70419","url":null,"abstract":"<p><p>Chronic thromboembolic pulmonary hypertension (CTEPH) can be treated by mechanical therapy with pulmonary endarterectomy (PEA) or balloon pulmonary angioplasty (BPA). Haemodynamic treatment goal is ill-defined. We explored post-procedure mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance (PVR), and its relationship with 5-Year survival and exercise capacity Using 6-min Walk Distance (6MWD). Consecutive PEA and BPA patients from the UK CURATE database, treated between 2007 and 2022 and 2015-2022, respectively, stratified by 3-month follow-up mPAP and PVR by right heart catheter (RHC), had 5-year survival and 6MWD compared, adjusted for age, sex, drug treatment, and comorbidities in the PEA cohort. Those treated by PEA achieving an mPAP ≤ 25 mmHg had significantly better survival than higher mPAP groups (all pairwise comparisons <i>p</i> < 0.001), with no additional survival advantage below mPAP of 25 mmHg. Those with PVR ≤ 300 dyne.s.cm^-5 also had better 5-year survival (<i>p</i> < 0.001). Continuous PVR modelling showed a 17% decrease in 5-year survival for every 100 dyne.s.cm^-5 increase in post-operative PVR (<i>p</i> < 0.001). And these results remained significant after proportional hazard modelling. Those treated by BPA achieving an mPAP ≤ 30 mmHg and PVR ≤ 300 had a significantly better 5-year survival with no additional survival advantage below these values. Continuous PVR modelling showed a 48% decrease in 5-year survival for every 100 dyne.s.cm^-5 increase in post-operative PVR (<i>p</i> = 0.008). In conclusion, post-procedure haemodynamics predict 5-year survival after mechanical therapy for CTEPH. Achieving a mPAP ≤ 25 mmHg for PEA and ≤ 30 mmHg for BPA, or a PVR ≤ 300 dyne.s.cm^-5 for both, conferred a better prognosis.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70419"},"PeriodicalIF":2.4,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13669816/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149216627","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":"Comparison of Clinical, Hemodynamic and Imaging Profiles Between Isolated Pulmonary Vasculitis and Chronic Thromboembolic Pulmonary Hypertension.","authors":"Xuesheng Fang, Hanwen Zhang, Yu Chen, Qixian Zeng, Wenjie Yan, Bingyang Liu, Beilan Yang, Qin Luo, Zhihui Zhao, Tao Yang, Qin Zhao, Yan Wu, Li Deng, Zhihong Liu, Tianli Wang, Xuejing Duan, Li Li, Changming Xiong","doi":"10.1002/pul2.70421","DOIUrl":"10.1002/pul2.70421","url":null,"abstract":"<p><p>Isolated pulmonary vasculitis (IPV) and chronic thromboembolic pulmonary hypertension (CTEPH) are important causes of pulmonary artery stenosis-related pulmonary hypertension. Differentiating between them remains challenging. Therefore, this study aims to compare the clinical, hemodynamic and imaging profiles of patients diagnosed with IPV and CTEPH by pathological diagnosis, so as to improve the accuracy of clinical diagnosis. Patients with IPV and CTEPH confirmed by pathological diagnosis were retrospectively enrolled. The general information, clinical manifestations, laboratory indicators, imaging features, hemodynamics, and echocardiographic data before and after pulmonary endarterectomy (PEA) were collected. There were no significant differences in general information, comorbidities, and symptoms. Compared with the IPV group, the CTEPH group had worse oxygenation status, hemodynamic conditions and right ventricular function. And those in the IPV group had higher levels of ESR. The imaging manifestations showed that IPV is mainly characterized by thickening of the vessel walls, while the filling defects of the lumen account for the majority of CTEPH. Moreover, echocardiography showed improvement in right ventricular function and hemodynamics after PEA in both groups, remarkably in the CTEPH group. Comprehensive evaluation of laboratory indicators, imaging features, and hemodynamic parameters help improve the accuracy of differential diagnosis between IPV and CTEPH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70421"},"PeriodicalIF":2.4,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13665635/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149195408","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":"Macrophages Contribute to Pulmonary Hypertension Associated With Sickle Cell Disease.","authors":"Christina Lisk, Francesca Cendali, Natalie Westover, Delaney Swindle, Daniel Colon Hidalgo, Caitlin Lewis, Melissa J Lucero, Nishant K Rana, Gemlyn George, Saini Setua, Monika Dzieciatkowska, Kathryn Hassel, Christy S Niemeyer, Kurt Stenmark, Eva Nozik, Angelo D'Alessandro, Paul W Buehler, David C Irwin","doi":"10.1002/pul2.70413","DOIUrl":"10.1002/pul2.70413","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is a fatal sequela in sickle cell disease (SCD) patients and occurs with greater prevalence compared to the general population. Macrophages (Mϕ) are one of the first immune cells to be recruited to the lungs in mouse models of PH, including SCD-PH, and a key cell type that contribute toward disease progression. Here, we first determined if phenotypic difference exist between control (C57BL/6) and Berkeley-SS (Berk-SS, SCD) mouse pulmonary Mϕs and, if so, how chronic moderate hypoxic exposure (~8,000 ft, Pb ~568 mmHg for 10 weeks) altered molecular and metabolic pathways. Next, we aimed to determine if the microenvironment of the Berk-SS lung is primed for Mϕs recruitment compared to the C57BL/6 lung. Using precision cut lung slice (PCLS), we determined PCLS from Berk-SS mice have significant increases in adhesion molecules associate with Mϕ recruitment and recruit splenic Mϕs at a greater rate compared to control mice. We confirmed these results in an acute hypoxic exposure (18,000 ft for 3 days) experiment and our results suggest that Berk-SS mice recruit more interstitial Mϕ with a pro-inflammatory phenotype when compared to C57BL/6 mice. Lastly, the depletion of Mϕ in our Berk-SS PH model improved right ventricular function, decreased pulmonary vascular muscularization, and improved exercise tolerance. Together, these data emphasize a critical role of Mϕ pulmonary recruitment in SCD-PH pathophysiology that is driven by inflammation.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70413"},"PeriodicalIF":2.4,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13666554/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149195644","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 Perceptions of a Video-Based Educational Platform for Pulmonary Arterial Hypertension: A Qualitative Study of the SMART-PAH Course.","authors":"Denise G Sese, Purnadeo Persaud, Lauryn Durham, Kathleen Neuendorf, Logan J Harper, Kristin B Highland","doi":"10.1002/pul2.70427","DOIUrl":"https://doi.org/10.1002/pul2.70427","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a chronic and progressive disease with substantial physiological and psychosocial burden. Effective patient education is essential for self-management and improved outcomes, yet access to reliable information remains limited by brief clinic visits and variable-quality external resources. Although video-assisted learning has been used in other chronic diseases, patient perceptions of video education in PAH have not been studied. To address this gap, we developed the SMART-PAH Library, a web-based learning management platform featuring 12 concise, expert-developed videos on key aspects of PAH management. This qualitative pilot explored patient perceptions, feasibility, and perceived impact of the SMART-PAH library. After completing the SMART-PAH course, 13 PAH patients participated in semi-structured virtual interviews exploring user experience, unmet educational needs, and the impact of the content on knowledge and self-management. Transcripts were analyzed iteratively by multiple reviewers using an inductive grounded theory-informed approach until thematic saturation was reached. Transcript analysis identified four domains in which patients sought support from a PAH-focused educational platform: emotional, physical, educational, and structural. Patients valued credible, relatable content, practical disease-management guidance, and education on under-addressed topics such as sexual health and palliative care. The platform helped address barriers including limited specialist access, financial complexity, and provider trust through accessible, repeatable reinforcement of clinical guidance. The SMART-PAH video library was perceived as a feasible and impactful approach to PAH education. Video-assisted learning can complement traditional PAH care by addressing informational gaps and supporting self-management. Larger studies are needed to evaluate effects on mental health, patient knowledge, and outcomes.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70427"},"PeriodicalIF":2.4,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13660719/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149183738","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":"β-sitosterol Alleviates Pulmonary Arterial Hypertension in Rats With Involvement of the VEGF/PI3K/AKT Signaling Pathway.","authors":"Xiaobing Wu, Chen Gong, Zhiyu Zeng","doi":"10.1002/pul2.70428","DOIUrl":"https://doi.org/10.1002/pul2.70428","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a progressive and lethal disorder with limited therapeutic options. β-sitosterol, a plant compound, has shown potential in managing PAH, but its protective mechanisms remain unclear. We evaluated the protective effects of β-sitosterol against monocrotaline (MCT)-induced PAH in rats and explored the underlying mechanisms. First, network pharmacology was used to predict potential targets of β-sitosterol in PAH. A rat model of PAH was established by a single subcutaneous injection of MCT (60 mg/kg). From day 28 after MCT injection, PAH rats were treated with β-sitosterol (100 mg/kg/day) by gavage for 28 days. Histopathological analysis, RT-qPCR, and Western blotting were performed to evaluate inflammatory infiltration, cellular proliferation, apoptosis, and the activity of the VEGF/PI3K/AKT pathway in lung tissues. Network pharmacology analysis identified the VEGF/PI3K/AKT pathway as centrally implicated. <i>In vivo</i>, β-sitosterol treatment significantly improved hemodynamic parameters and right ventricular function, reducing pulmonary vascular remodeling. Furthermore, β-sitosterol suppressed vascular inflammation and proliferation while promoting apoptosis. RT-qPCR analysis revealed that β-sitosterol significantly reduced mRNA levels of VEGF, <i>TNF-α, IL-6, IL-1β, Collagen I, and TGF-β1</i> compared with the MCT group. Western blotting demonstrated that β-sitosterol decreased the phosphorylation of VEGFR2, PI3K and AKT, upregulated Bax expression, and downregulated PCNA and Bcl-2. In conclusion, β-sitosterol may alleviate PAH with the involvement of the VEGF/PI3K/AKT signaling pathway. These findings suggest the therapeutic potential of β-sitosterol against PAH and offer preliminary insights into its molecular mechanisms.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70428"},"PeriodicalIF":2.4,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13660722/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149183952","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 Team-Based Patient Engagement Approach to Selexipag Initiation and Maintenance in Pulmonary Arterial Hypertension (TEAM PAH): Real-World Dosing Patterns and Persistence Among Patients With and Without a History of Methamphetamine Use.","authors":"Samuel B Brusca, Amanda Schnell Heringer, Mark Joshua Lacsamana, Anya Thakur, Jonathan Thomas, Christina Benninger, Gurinderpal Doad, Paul Strachan, Sarah Walden, Ambika Satija, Gayatri Marathe, Henrietta Blinder, Marjolaine Gauthier-Loiselle, Teresa De Marco","doi":"10.1002/pul2.70384","DOIUrl":"https://doi.org/10.1002/pul2.70384","url":null,"abstract":"<p><p>Selexipag is an oral prostacyclin receptor agonist approved for pulmonary arterial hypertension (PAH). This single-center, retrospective study described oral selexipag dosing patterns and persistence, overall and stratified by history of methamphetamine (meth) use, at an expert center in California, USA, using a multidisciplinary, nurse-directed protocol entailing frequent follow-up and individualized side effect management. Medical charts of patients with PAH initiating oral selexipag between January 2016 and March 2023 were abstracted. Patient characteristics and dosing patterns were described up to 14 months post-initiation. The individualized maintenance dose (IMD) was the first dose maintained for ≥ 14 days after reaching the maximum tolerated dose. Persistence was described using Kaplan-Meier analysis. A total of 200 patients initiated oral selexipag (median age 52 years; 69.5% female; 54.5% White; 70.5% on triple therapy; 41% with a history of meth use). Most patients (89.0%) achieved maintenance (median IMD: 900 μg twice daily; median time to maintenance: 2.5 months). Selexipag persistence at 1 year was 76.2%, with most discontinuations occurring within 2 months of initiation. Similar proportions of patients with and without a history of meth use achieved maintenance (85.4% vs. 91.5%; <i>p</i> = 0.3) and persisted on selexipag at 1 year (74.0% vs. 77.5%; <i>p</i> = 0.9). Using a multidisciplinary nurse-directed protocol, most patients with PAH were titrated to their IMD and persisted on oral selexipag, including those with a history of meth use. These findings highlight the importance of frequent patient contact for dose adjustment and side effect management, particularly earlier in the titration process.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70384"},"PeriodicalIF":2.4,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13660724/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149184209","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":"Antithrombotic Therapy Use in Children With Pulmonary Hypertension.","authors":"Meredith J O'Neil, Lynn A Sleeper, Nidhy P Varghese, Paul J Critser, Eric D Austin, Catherine M Avitabile, Angela Bates, Erika B Rosenzweig, Carly E Byrd, Patrick D Evers, Stephanie S Handler, Rachel K Hopper, Emma O Jackson, Roberta L Keller, Usha S Krishnan, Minmin Lu, Jessica Maclean, Raysa Morales-Demori, Mary P Mullen, Meredith Riker, Rachel T Sullivan, Delphine Yung, Benjamin S Frank","doi":"10.1002/pul2.70418","DOIUrl":"https://doi.org/10.1002/pul2.70418","url":null,"abstract":"<p><p>The role of antithrombotic therapy in pulmonary hypertension (PH) remains uncertain, with conflicting adult data and limited pediatric evidence. This study aimed to describe real-world antithrombotic use in pediatric PH utilizing Pediatric Pulmonary Hypertension Network (PPHNet) registry data and a survey of PPHNet centers. We analyzed data of 1,716 patients from the PPHNet registry (2014-2024), comparing characteristics of those who received aspirin or warfarin with those who did not. A survey on real-world antithrombotic use was also distributed to 12 North American PPHNet centers in 2023. Eighteen percent of subjects received aspirin and 7% received warfarin. Patients who received aspirin or warfarin were more likely to have idiopathic/heritable PH. Aspirin or warfarin-treated patients demonstrated higher-risk features as evidenced by use of PH-targeted triple therapy (aspirin vs. no aspirin: 22% vs. 9%, <i>p</i> < 0.01; warfarin vs. no warfarin: 31% vs. 10%; <i>p</i> < 0.01) and higher mortality over a median follow-up of 3.2 years (aspirin vs. no aspirin: 14% vs. 9%, <i>p</i> = 0.008; warfarin vs. no warfarin: 19% vs. 9%, <i>p</i> = 0.001). Center-specific aspirin and warfarin use varied widely. Survey data demonstrated general agreement that antithrombotic therapy was not indicated for patients at lowest risk and probably indicated for patients at highest risk, but management of intermediate-risk patients varied considerably. Antithrombotic therapy is used infrequently in pediatric PH and primarily reserved for high-risk patients, with significant variation across centers. These findings highlight the need for further research to define which patients may benefit and guide evidence-based practice.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70418"},"PeriodicalIF":2.4,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13660712/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149183071","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":"Acute Cor Pulmonale Caused by Pulmonary Tumor Thrombotic Microangiopathy as the Initial Presentation of Multiple Myeloma: A Case Report.","authors":"Jianwei Cui, Bingzhen Zhao, Xin Ding","doi":"10.1002/pul2.70425","DOIUrl":"https://doi.org/10.1002/pul2.70425","url":null,"abstract":"<p><p>Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare cause of acute cor pulmonale, typically associated with advanced solid tumors; its occurrence as the initial presentation of multiple myeloma (MM) has not been described. A 45-year-old woman developed acute right heart failure on postoperative day 1 after uterine myomectomy. Echocardiography showed a pulmonary artery systolic pressure of 86 mmHg with a d-shaped left ventricle and right ventricular dysfunction (TAPSE 10 mm); CTPA excluded pulmonary embolism but revealed bilateral ground-glass opacities. Platelet count fell from 115 × 10⁹/L preoperatively to 30 × 10⁹/L. An overlooked preoperative globulin of 67 g/L prompted serum protein electrophoresis and immunofixation, which identified an IgG κ M-protein; bone marrow aspiration revealed 14% morphologically abnormal plasma cells. Presumed MM-associated PTTM was treated with heparin and sildenafil. The platelet count recovered, and a follow-up echocardiogram on day 7 showed normalized cardiac structure and function. The patient self-discontinued rivaroxaban 1 week after discharge and died 3 months later from progression of untreated MM. In CTPA-negative acute cor pulmonale with unexplained thrombocytopenia, an elevated globulin on routine biochemistry may be the only early clue to a plasma cell disorder, and timely anticoagulation with pulmonary vasodilation may buy time for definitive therapy.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70425"},"PeriodicalIF":2.4,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13660714/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149184130","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}