Pulmonary Circulation最新文献

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Quantitative Inferior T-Wave Inversion as a Marker of High Pulmonary Vascular Resistance and All-Cause Mortality in Patients Evaluated for Pulmonary Hypertension. 定量下t波反转作为肺动脉高压患者高肺血管阻力和全因死亡率的标志。
IF 2.4 4区 医学
Pulmonary Circulation Pub Date : 2026-10-09 eCollection Date: 2026-10-01 DOI: 10.1002/pul2.70420
Hidekazu Ikemiyagi, Ayane Miyagi, Haruno Nagata, Shinya Shiohira, Yuichiro Toma, Takeshi Sasaki, Masashi Iwabuchi, Kenya Kusunose
{"title":"Quantitative Inferior T-Wave Inversion as a Marker of High Pulmonary Vascular Resistance and All-Cause Mortality in Patients Evaluated for Pulmonary Hypertension.","authors":"Hidekazu Ikemiyagi, Ayane Miyagi, Haruno Nagata, Shinya Shiohira, Yuichiro Toma, Takeshi Sasaki, Masashi Iwabuchi, Kenya Kusunose","doi":"10.1002/pul2.70420","DOIUrl":"https://doi.org/10.1002/pul2.70420","url":null,"abstract":"<p><p>Right heart catheterization defines pulmonary hypertension (PH) hemodynamics, but simple markers of pulmonary vascular load remain useful. Inferior-lead T-wave inversion (TWI) is seen in right ventricular pressure overload; its association with pulmonary vascular resistance (PVR) and prognosis is uncertain. We retrospectively studied a cohort of 98 patients who underwent electrocardiography (ECG), echocardiography, and right heart catheterization within 48 h from 2021 to 2023. Inferior TWI amplitudes in leads II, III, and aVF were measured as absolute values and summed. Associations with PVR, performance for PVR > 5 Wood units, and all-cause mortality were evaluated. Twenty-five patients (26%) had PVR > 5 Wood units. The inferior TWI sum correlated with PVR (<i>ρ</i> = 0.326, <i>p</i> = 0.001) and discriminated PVR > 5 Wood units better than conventional ECG markers (area under the curve, 0.78). A cutoff > 0.10 mV yielded 72% sensitivity, 84% specificity, and an odds ratio of 13.1 (95% confidence interval [CI], 4.5-38.1; <i>p</i> < 0.001). During a median follow-up of 731 days, 19 patients died. Inferior TWI sum > 0.15 mV was associated with higher mortality (log-rank <i>p</i> = 0.015) and remained associated after adjustment for age and log<sub>2</sub> N-terminal pro-B-type natriuretic peptide (NT-proBNP) (hazard ratio, 3.83; 95% CI, 1.48-9.90; <i>p</i> = 0.007). Adding this ECG marker increased the global chi-square from 12.0 to 19.3. Quantitative inferior-lead TWI was associated with high PVR and all-cause mortality in patients evaluated for PH. It may complement non-invasive assessment and right heart catheterization but requires external validation.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70420"},"PeriodicalIF":2.4,"publicationDate":"2026-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13650592/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149166084","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}
引用次数: 0
Transcatheter Potts Shunt in Pulmonary Arterial Hypertension After Patent Ductus Arteriosus Closure. 动脉导管未闭闭合后肺动脉高压的经导管分流术。
IF 2.4 4区 医学
Pulmonary Circulation Pub Date : 2026-10-09 eCollection Date: 2026-10-01 DOI: 10.1002/pul2.70422
Zhuoyuan Xu, Yiwei Chen, Xiafeng Yu, Yanjun Sun, Jie Bai, Zhuoming Xu, Lijun Fu, Hao Zhang
{"title":"Transcatheter Potts Shunt in Pulmonary Arterial Hypertension After Patent Ductus Arteriosus Closure.","authors":"Zhuoyuan Xu, Yiwei Chen, Xiafeng Yu, Yanjun Sun, Jie Bai, Zhuoming Xu, Lijun Fu, Hao Zhang","doi":"10.1002/pul2.70422","DOIUrl":"https://doi.org/10.1002/pul2.70422","url":null,"abstract":"<p><p>We report the case of an 18-year-old female with progressive, end-stage pulmonary arterial hypertension (PAH) despite receiving maximal triple combination therapy. The patient had a history of patent ductus arteriosus (PDA) closure at 15 months of age. Due to a pulmonary hypertensive crisis during anesthesia induction, a transcatheter Potts procedure was successfully performed under extracorporeal membrane oxygenation (ECMO) support, involving covered stent implantation just below the previous PDA occluder. At the 3-month follow-up, the patient demonstrated significant clinical improvement, shifting to WHO class II-III, with a dramatic decrease in NT-proBNP (5406-512 pg/ml) and an increased 6-min walk distance (< 300 to 390 m). This case achieved short-term recovery of cardiac function and long-term maintenance of pulmonary vascular stability and demonstrates that the transcatheter Potts shunt is a feasible and effective life-prolonging treatment option for severe PAH, even in patients with a history of prior PDA closure.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70422"},"PeriodicalIF":2.4,"publicationDate":"2026-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13650509/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149168250","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}
引用次数: 0
Identification and Functional Analysis of tRNA-Derived Small RNAs in the Lung Tissues of Patients With Idiopathic Pulmonary Arterial Hypertension. 特发性肺动脉高压患者肺组织中trna衍生小rna的鉴定和功能分析。
IF 2.4 4区 医学
Pulmonary Circulation Pub Date : 2026-10-09 eCollection Date: 2026-10-01 DOI: 10.1002/pul2.70424
Yuan Chen, Ziqi E, Jintao Zhou, Feifan Ge, Jian Xu, Shugao Ye, Jialong Liang, Bin Ni
{"title":"Identification and Functional Analysis of tRNA-Derived Small RNAs in the Lung Tissues of Patients With Idiopathic Pulmonary Arterial Hypertension.","authors":"Yuan Chen, Ziqi E, Jintao Zhou, Feifan Ge, Jian Xu, Shugao Ye, Jialong Liang, Bin Ni","doi":"10.1002/pul2.70424","DOIUrl":"https://doi.org/10.1002/pul2.70424","url":null,"abstract":"<p><p>Idiopathic pulmonary arterial hypertension (IPAH) is a pulmonary vascular disease with a poor prognosis. Its pathogenesis remains unclear and the treatment methods are limited. The expression of tRNA-derived small RNA (tsRNA) in IPAH tissues remains unknown. tsRNA sequencing was conducted on lung tissue samples obtained from patients with IPAH and healthy controls. Differentially expressed tsRNAs were identified and their expression levels were validated using qRT-PCR. Functional enrichment analysis was performed to explore the potential biological roles of these tsRNAs. Lung tissue samples from three patients with IPAH and three control subjects were sequenced. A total of 382 differential tRFs and tsRNAs were identified (200 upregulated and 182 downregulated). Ten tsRNAs with the most significant differential expression were selected for further investigation. qRT-PCR analysis revealed that all five upregulated tsRNAs exhibited expression trends consistent with the sequencing results, whereas only two of the five downregulated tsRNAs showed consistency. Bioinformatics analysis predicted that five upregulated tsRNAs target the same gene, TENT4A; among them, tiRNA-1:35-Leu-TAG-2 was experimentally confirmed by dual-luciferase reporter assay. Analysis of GSE15197 and GSE113439 indicated that TENT4A expression was significantly lower in IPAH samples compared with that in the controls. Further research indicates that tiRNA-1:35-Leu-TAG-2 directly targets TENT4A and functions dependent on TENT4A. tsRNA dysregulation may play a potential role in the development of IPAH. TENT4A is a potential critical target in the pathogenesis and treatment of IPAH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70424"},"PeriodicalIF":2.4,"publicationDate":"2026-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13650610/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149168865","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}
引用次数: 0
Pulmonary Artery Denervation for Persistent Pulmonary Hypertension After Transcatheter Mitral Edge-to-Edge Repair: A Pilot Study and First Application in the Post M-TEER Setting. 经导管二尖瓣边缘到边缘修复后肺动脉去神经支配治疗持续性肺动脉高压:一项初步研究和M-TEER后的首次应用。
IF 2.4 4区 医学
Pulmonary Circulation Pub Date : 2026-10-06 eCollection Date: 2026-10-01 DOI: 10.1002/pul2.70404
Xiang Chen, Zeying Zhang, Xiaoying Lan, Weibin Chen, Qiuyue Fang, Huaibin Huang, Shaoliang Chen, Yan Wang
{"title":"Pulmonary Artery Denervation for Persistent Pulmonary Hypertension After Transcatheter Mitral Edge-to-Edge Repair: A Pilot Study and First Application in the Post M-TEER Setting.","authors":"Xiang Chen, Zeying Zhang, Xiaoying Lan, Weibin Chen, Qiuyue Fang, Huaibin Huang, Shaoliang Chen, Yan Wang","doi":"10.1002/pul2.70404","DOIUrl":"10.1002/pul2.70404","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is common in patients undergoing transcatheter mitral edge-to-edge repair (M-TEER) and is an established determinant of adverse outcomes after intervention. Importantly, persistent PH after technically successful M-TEER remains associated with ongoing symptoms, right ventricular dysfunction and poor prognosis, yet no dedicated treatment strategy has been established. The aim of this study is to evaluate the safety and efficacy of pulmonary artery denervation (PADN) in patients with persistent PH following successful M-TEER. Six patients (median age 69.0 years; 1 female) with WHO Group 2 PH and persistent symptoms despite successful M-TEER underwent PADN at a median of 583 days after valve repair. All patients had trace to mild residual MR (grade 1+) with median mean mitral valve gradient 3.0 mmHg. Right heart catheterization (RHC) was performed at baseline, immediately post-PADN, and at 6-month follow-up. Immediately post-PADN, mean pulmonary artery pressure (mPAP) decreased from 38.5 (3.8) mmHg to 34.0 (11.5) mmHg (<i>p</i> = 0.028). Pulmonary arterial wedge pressure decreased from 22.5 (7.5) mmHg to 20.0 (6.2) mmHg (<i>p</i> = 0.026). At 6-month follow-up, a directional but statistically non-significant trend toward maintained mPAP reduction was observed [median 32.5 (9.0) mmHg vs. baseline 38.5 (3.8) mmHg; <i>p</i> = 0.075]. Tricuspid regurgitation grade improved in 5 of 6 patients (83%). Median 6-min walk distance increased from 377 (109) m to 436 (109) m (<i>p</i> = 0.046). No procedural complications occurred. PADN appears feasible, safe, and potentially effective in patients with persistent PH following M-TEER, warranting further investigation in controlled studies.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70404"},"PeriodicalIF":2.4,"publicationDate":"2026-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13640058/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149114168","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}
引用次数: 0
Contemporary Economic, Hospitalization, and Mortality Burden Among Pulmonary Arterial Hypertension Patients: A Real-World US Claims Data Analysis. 肺动脉高压患者的当代经济、住院和死亡率负担:一个真实世界的美国索赔数据分析。
IF 2.4 4区 医学
Pulmonary Circulation Pub Date : 2026-10-06 eCollection Date: 2026-10-01 DOI: 10.1002/pul2.70414
Ashwin Ravichandran, Amélie Beaudet, David Lopez, Carolyn R Lew, Helen Varker, Michelle Jerry, Akshay Muralidhar
{"title":"Contemporary Economic, Hospitalization, and Mortality Burden Among Pulmonary Arterial Hypertension Patients: A Real-World US Claims Data Analysis.","authors":"Ashwin Ravichandran, Amélie Beaudet, David Lopez, Carolyn R Lew, Helen Varker, Michelle Jerry, Akshay Muralidhar","doi":"10.1002/pul2.70414","DOIUrl":"10.1002/pul2.70414","url":null,"abstract":"<p><p>This study quantified the recent real-world burden of pulmonary arterial hypertension (PAH) using US real-world data. Adults (18+) with treated PAH were identified from the MarketScan Commercial/Medicare Databases using a claims-based algorithm requiring pulmonary hypertension diagnosis, PAH-specific treatment, and right heart catheterization from January 1, 2017 to August 31, 2024 (earliest treatment claim = index). Patients were followed for ≥ 30 days post-index, overall and stratified by payer and by treatment categories. Subsets of PAH-hospitalized and newly treated patients were also analyzed. Measures included demographics, clinical characteristics, all-cause healthcare utilization/costs, PAH-related hospitalization costs/characteristics, and all-cause mortality. Overall, 2712 treated PAH patients were included (mean age 61.8 years, 68.9% female, 57.9% commercial insurance). During mean 2.2 years of follow-up, mean (median) per-patient-per-year all-cause healthcare costs were $302,014 ($210,888) for commercially-insured patients and $200,581 ($147,706) for Medicare patients. Over half (56.2%) of patients had ≥ 1 all-cause hospitalization, 44.9% had ≥ 1 PAH-related hospitalization, and 31.0% were hospitalized for ≥ 1 complication (thrombosis [14.1%], hemorrhage [12.5%], sepsis [14.9%]). Among the PAH-hospitalized subset (<i>N</i> = 1079), median time to re-hospitalization was 1.2 years. The mean (median) cost per PAH-related hospitalization was $89,799 ($30,224) for commercially-insured and $37,288 ($15,733) for Medicare patients, and 57.2% of hospitalizations required an ICU stay. Among the newly treated subset (<i>N</i> = 2072), 9.7%, 18.6%, and 25.2% of patients died within 1, 2, and 3 years after treatment initiation. Despite recent therapeutic advancements, PAH patients continue to have a substantial clinical and economic burden. These findings highlight ongoing unmet need and underscore the importance of continued monitoring of disease burden as PAH therapies evolve.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70414"},"PeriodicalIF":2.4,"publicationDate":"2026-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13640061/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149114205","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}
引用次数: 0
Pulmonary Arterial Hypertension Associated With Chronic Mephedrone Use: A Case Report. 肺动脉高压与慢性甲氧麻黄酮使用:1例报告。
IF 2.4 4区 医学
Pulmonary Circulation Pub Date : 2026-10-01 DOI: 10.1002/pul2.70415
Masoud Kashoub, Michael Plunkett, Heba Nashat, Konstantinos Dimopoulos, Colm McCabe, Carl Harries, S John Wort, Paul I Dargan, Laura C Price
{"title":"Pulmonary Arterial Hypertension Associated With Chronic Mephedrone Use: A Case Report.","authors":"Masoud Kashoub, Michael Plunkett, Heba Nashat, Konstantinos Dimopoulos, Colm McCabe, Carl Harries, S John Wort, Paul I Dargan, Laura C Price","doi":"10.1002/pul2.70415","DOIUrl":"https://doi.org/10.1002/pul2.70415","url":null,"abstract":"<p><p>Mephedrone is a synthetic cathinone with sympathomimetic effects, but has not previously been linked to pulmonary arterial hypertension (PAH). We report a 42-year-old woman with no prior cardiopulmonary disease who presented with severe pre-capillary PAH after a decade of near-daily mephedrone use. Extensive evaluation-including autoimmune, infectious, genetic, and imaging studies-excluded recognised causes of PAH. Following cessation of mephedrone and initiation of dual oral PAH-targeted therapy, she demonstrated substantial initial clinical and haemodynamic improvement; however, the response remained incomplete, necessitating further treatment escalation with selexipag. This appears to be the first reported case of PAH linked to mephedrone use.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 4","pages":"e70415"},"PeriodicalIF":2.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13628448/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149114117","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}
引用次数: 0
Global Burden of Pulmonary Arterial Hypertension in Older Adults and Its Causal Association With Body Mass Index. 老年人肺动脉高压的全球负担及其与体重指数的因果关系
IF 2.4 4区 医学
Pulmonary Circulation Pub Date : 2026-09-30 eCollection Date: 2026-07-01 DOI: 10.1002/pul2.70416
Yajuan Fu, Baohong Wang, Fang Li, Xinmin Tian, Jiwei Gu, Yujing Gao
{"title":"Global Burden of Pulmonary Arterial Hypertension in Older Adults and Its Causal Association With Body Mass Index.","authors":"Yajuan Fu, Baohong Wang, Fang Li, Xinmin Tian, Jiwei Gu, Yujing Gao","doi":"10.1002/pul2.70416","DOIUrl":"https://doi.org/10.1002/pul2.70416","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a severe cardiopulmonary disease associated with substantial morbidity and mortality. However, the global burden, future trajectory, and potentially modifiable risk factors for PAH among older adults remain insufficiently characterized. This study aimed to quantify the global burden and projected trends of PAH among adults aged ≥ 60 years and investigate the causal association between body mass index (BMI) and PAH risk using Mendelian randomization (MR). We extracted PAH burden estimates from the Global Burden of Disease (GBD) 2021 study across 204 countries and territories, 21 GBD regions, and five socio-demographic index (SDI) strata. Temporal trends were assessed using age-standardized rates and average annual percent changes (AAPCs), while demographic decomposition and Bayesian age-period-cohort (BAPC) models were applied to evaluate drivers of burden changes and future trajectories. Two-sample MR analyses were performed using genome-wide significant BMI-associated genetic variants as instrumental variables and PAH genome-wide association study summary statistics from FinnGen. Between 1990 and 2021, global incident cases of PAH among adults aged ≥ 60 years increased from 6771.01 (95% uncertainty interval [UI]: 4577.81-9656.55) to 15,622.29 (95% UI: 10,546.44-22,296.65), and deaths increased from 6812.93 (95% UI: 5305.00-8473.04) to 15,443.42 (95% UI: 11,746.25-18,388.02). Although the age-standardized mortality, prevalence, and disability-adjusted life year (DALY) rates declined, the age-standardized incidence rate showed a slight increase (AAPC = 0.07%, 95% CI: 0.05-0.09). Marked geographic and SDI-related disparities were observed, with population growth and aging identified as the predominant contributors to the rising absolute burden. BAPC modeling projected continued increases in absolute PAH cases through 2050 despite declining age-standardized mortality and DALY rates. In addition, MR analyses provided suggestive evidence for a positive association between genetically predicted higher BMI and increased PAH risk (inverse-variance weighted odds ratio [IVW OR] = 1.84, 95% CI: 1.11-3.05, <i>p</i> = 0.018). Among older adults, the global absolute burden of PAH has increased substantially over the past three decades and is expected to continue rising, largely driven by population aging and growth. Genetic evidence further suggests that elevated BMI may represent a potentially modifiable risk factor for PAH. These findings highlight the need for enhanced surveillance, improved diagnostic strategies, equitable healthcare access, and targeted metabolic risk management to address the growing burden of PAH in aging populations.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70416"},"PeriodicalIF":2.4,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13627979/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149114139","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}
引用次数: 0
Pulmonary Arterial Hypertension in Octogenarians: The Pulmonary Hypertension Association Registry. 80岁老人肺动脉高压:肺动脉高压协会登记。
IF 2.4 4区 医学
Pulmonary Circulation Pub Date : 2026-09-22 eCollection Date: 2026-07-01 DOI: 10.1002/pul2.70387
Renuka Reddy, Jude Moutchia, Ashwin Ravichandran, James Runo, David B Badesch, John Moss, Charles Burger, Teresa De Marco, Evelyn Horn, Ali Ataya, Andrew Bryant, Nadine Al-Naamani, Steven M Kawut
{"title":"Pulmonary Arterial Hypertension in Octogenarians: The Pulmonary Hypertension Association Registry.","authors":"Renuka Reddy, Jude Moutchia, Ashwin Ravichandran, James Runo, David B Badesch, John Moss, Charles Burger, Teresa De Marco, Evelyn Horn, Ali Ataya, Andrew Bryant, Nadine Al-Naamani, Steven M Kawut","doi":"10.1002/pul2.70387","DOIUrl":"10.1002/pul2.70387","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) was historically viewed as a disease of young adults; however, emerging evidence suggests rising prevalence in the elderly, which is an insufficiently characterized population. We evaluated clinical and sociodemographic features of PAH patients over 80 years and compared their outcomes with younger patients. We performed a prospective cohort study utilizing the Pulmonary Hypertension Association Registry to compare clinical characteristics, functional capacity, and quality of life measures between octogenarians (≥ 80 years) and non-octogenarians (< 80 years) at enrollment and follow-up visits. Associations between age subgroups and outcomes were assessed using linear, binomial, and Cox regression. The cohort comprised 113 octogenarians and 3,094 non-octogenarians with PAH. Smoking history was more common in octogenarians, who also more frequently had idiopathic or connective tissue disease-related PAH. Octogenarians had lower right atrial pressure and mean pulmonary artery pressure (<i>p</i> < 0.001) than younger patients. At baseline, octogenarians had a lower absolute 6-min walk distance and higher World Health Organization functional class (<i>p</i> < 0.001). Octogenarians were more often initially treated with monotherapy and less likely to receive a prostacyclin analog. Octogenarians had a 57% higher risk of all-cause hospitalization and twice the mortality of younger adults after covariate adjustment (IRR 1.57, 95%CI 1.06-2.33, <i>p</i> < 0.05 and HR 2.38, 95%CI 1.62-3.52, <i>p</i> = 0.001, respectively). The oldest PAH patients exhibit distinct clinical profiles, therapies, and outcomes compared to younger adults, though it remains uncertain whether these differences reflect a unique phenotype, reduced therapy response, or the cumulative effects of aging and comorbid conditions.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70387"},"PeriodicalIF":2.4,"publicationDate":"2026-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13595173/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149025901","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}
引用次数: 0
Long-Term Subcutaneous Treprostinil in Pediatric Pulmonary Arterial Hypertension at a Latin American Referral Center: A Retrospective Cohort Study. 拉丁美洲转诊中心长期皮下注射曲前列地尼治疗小儿肺动脉高压:一项回顾性队列研究。
IF 2.4 4区 医学
Pulmonary Circulation Pub Date : 2026-09-21 eCollection Date: 2026-07-01 DOI: 10.1002/pul2.70395
Angelo Valencia Salazar, Ernesto León Vallejo Mondragón
{"title":"Long-Term Subcutaneous Treprostinil in Pediatric Pulmonary Arterial Hypertension at a Latin American Referral Center: A Retrospective Cohort Study.","authors":"Angelo Valencia Salazar, Ernesto León Vallejo Mondragón","doi":"10.1002/pul2.70395","DOIUrl":"10.1002/pul2.70395","url":null,"abstract":"<p><p>Pediatric pulmonary arterial hypertension is a progressive vasculopathy with substantial early mortality. Subcutaneous treprostinil is an established prostacyclin-pathway therapy for higher-risk disease, but long-term pediatric data from Latin American referral centers remain limited. This retrospective cohort included all nine consecutive children with World Symposium on Pulmonary Hypertension Group 1 pulmonary arterial hypertension who initiated subcutaneous treprostinil at a Colombian referral center and were followed from diagnosis until death or last known contact. Outcomes included survival, pediatric risk category transitions, N-terminal pro-B-type natriuretic peptide levels, tricuspid annular plane systolic excursion, systolic pulmonary artery pressure, eccentricity index, and structured patient-/caregiver-reported domains. Exploratory comparisons were performed by maintenance dose (≤ 60 vs. > 60 ng/kg/min) and therapy duration. Kaplan-Meier survival at 1, 3, and 5 years was 88.9%, 55.6%, and 55.6%, respectively. Eccentricity index decreased from a median of 2.8 at diagnosis to 1.5 at 5 years, with paired comparisons showing reductions at 1 and 3 years. By Year 5, four of five children with paired risk data had transitioned to a lower pediatric risk category. Doses > 60 ng/kg/min were associated descriptively with more favorable final risk profiles, whereas structured patient-/caregiver-reported domains showed heterogeneous patterns; schooling was the only domain with a statistically detectable association by both dose and duration. In this small Latin American referral-center cohort, subcutaneous treprostinil treatment was accompanied by favorable risk transitions and stabilization of right ventricular geometry among long-term survivors. These findings are exploratory and should be interpreted in light of the small sample size, retrospective design, missing longitudinal data, and potential confounding by disease severity, survival, and treatment escalation.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70395"},"PeriodicalIF":2.4,"publicationDate":"2026-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13591332/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149017912","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}
引用次数: 0
Association Between Serum Uric Acid and Pulmonary Hypertension in Takayasu Arteritis With Pulmonary Artery Involvement: A Cross-Sectional Study. 横断面研究:高须动脉炎伴肺动脉受累患者血清尿酸与肺动脉高压的关系
IF 2.4 4区 医学
Pulmonary Circulation Pub Date : 2026-09-15 eCollection Date: 2026-07-01 DOI: 10.1002/pul2.70393
Jinzhi Wang, Shuwen Zheng, Ruzetuoheti Yiminniyaze, Hanyu Zheng, Shuai Zhang, Han Tian, Yanhua Kong, Wanmu Xie, Qiang Huang, Zhenguo Zhai
{"title":"Association Between Serum Uric Acid and Pulmonary Hypertension in Takayasu Arteritis With Pulmonary Artery Involvement: A Cross-Sectional Study.","authors":"Jinzhi Wang, Shuwen Zheng, Ruzetuoheti Yiminniyaze, Hanyu Zheng, Shuai Zhang, Han Tian, Yanhua Kong, Wanmu Xie, Qiang Huang, Zhenguo Zhai","doi":"10.1002/pul2.70393","DOIUrl":"10.1002/pul2.70393","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is a severe complication of Takayasu arteritis (TAK) with pulmonary artery involvement (PAI), associated with poor prognosis. Serum uric acid (UA) has been linked to both TAK severity and PH in other settings, but its role specifically in TAK-PAI-associated PH remains unclear. This study aimed to investigate the relationship between serum UA and the presence and severity of PH in TAK patients with confirmed PAI. This cross-sectional study enrolled 90 consecutive TAK patients with PAI documented by CT pulmonary angiography. All patients underwent echocardiography; those with echocardiographic signs of PH underwent confirmatory right heart catheterization (RHC). PH was defined as mean pulmonary artery pressure > 20 mmHg with pulmonary artery wedge pressure ≤ 15 mmHg (2022 ESC/ERS criteria). Serum UA was measured at baseline. Multivariable logistic regression, subgroup analyses, and restricted cubic spline (RCS) were performed. Among 90 patients, 42 (46.7%) met the PH criteria. Patients with PH had significantly higher serum UA levels than those without PH (400.5 ± 140.8 vs. 305.2 ± 91.6 μmol/L, <i>p</i> < 0.001). After adjusting for age, sex, eGFR, and Kerr score (disease activity), each 10 μmol/L increase in UA was associated with a 10% higher odds of PH risk (OR = 1.10, 95% CI 1.04-1.16, <i>p</i> = 0.001). Compared with the lowest tertile (< 284 μmol/L), the highest tertile (≥ 377 μmol/L) had an 8-fold increased odds of PH risk (OR = 8.03, 95% CI 2.03-31.79, <i>p</i> = 0.003). Subgroup analyses showed consistent associations across sex, disease activity, and PAI pattern. RCS analysis revealed no significant nonlinearity (<i>p</i> = 0.729), indicating a predominantly linear dose-response relationship. Elevated serum uric acid is independently associated with higher odds of PH in TAK patients with PAI, exhibiting a linear dose-response pattern. A uric acid level ≥ 377 μmol/L may identify patients at substantially increased odds of PH. Serum UA could serve as a simple, noninvasive biomarker for PH risk stratification in this high-risk population. <b>Trial Registration:</b> ClinicalTrials.gov: NCT07184814.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70393"},"PeriodicalIF":2.4,"publicationDate":"2026-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13575858/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148977940","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}
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