Pulmonary Circulation最新文献

{"title":"The Heterogeneity of Pulmonary Hypertension Nomenclature in Empiric Research Studies: Systematic Findings From Three Western European Countries.","authors":"Armella Santi, Veranyuy Ngah, Eric W Robbins, Bradley A Maron, Katarina Zeder","doi":"10.1002/pul2.70089","DOIUrl":"https://doi.org/10.1002/pul2.70089","url":null,"abstract":"<p><p>This systematic literature review of three Western European Countries identified <i>N</i> = 48 different terms to describe pulmonary arterial pressure and <i>N</i> = 35 thresholds used to define pulmonary hypertension in published empiric research studies. There is an urgent need to standardize pulmonary artery pressure nomenclature and pulmonary hypertension definitions in clinical research reports.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70089"},"PeriodicalIF":2.2,"publicationDate":"2025-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12037687/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144050765","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":"Long-Term Treatment With Subcutaneous Treprostinil in Patients With Severe Inoperable Chronic Thromboembolic Pulmonary Hypertension in the Multimodal Therapy Era (Data From CTREPH Study Open Label Extension).","authors":"Pavel Jansa, Roela Sadushi-Kolici, Nika Skoro-Sajer, Grzegorz Kopec, Iveta Simkova, Regina Steringer-Mascherbauer, Barbara Salobir, Jaroslav Lindner, Irene M Lang","doi":"10.1002/pul2.70080","DOIUrl":"10.1002/pul2.70080","url":null,"abstract":"<p><p>The aim of the open label extension (OLE) of CTREPH study was to characterize multimodal treatment in patients with severe inoperable CTEPH, to describe long-term subcutaneous (SC) treprostinil safety and tolerability, and to evaluate change in functional class and exercise capacity over 24 months since completion of the blinded phase of CTREPH. The target population in the OLE consisted of patients who completed 24 weeks of blinded treatment with either high-dose treprostinil of around 30 ng/kg/min (former high-dose group), or low-dose treprostinil of around 3 ng/kg/min (former low-dose group) in the CTREPH study. From the start of OLE, treprostinil dose and any additional therapy were chosen according to the standard of care and physician's discretion. Out of 47 enrolled patients, 20 patients received other PH drugs during OLE and 17 patients underwent at least 1 BPA session. Number of treprostinil-related AEs was substantially higher in the former low-dose group in comparison to the former high-dose group. Related AEs were also more frequent during the first 6 months of the preceding blinded trial than over 24 months of OLE, especially infusion site pain and all local infusion site reactions. No new safety signal was detected. Evaluated clinical outcomes show sustained benefit from long-term treprostinil treatment. Long-term SC treprostinil is a safe and effective component of multimodal treatment for patients with severe CTEPH. Patients who tolerate treprostinil after initiation are likely to continue tolerating it over time, with the clinical benefit maintained over 24 months.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70080"},"PeriodicalIF":2.2,"publicationDate":"2025-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12034262/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144053882","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":"Morbidity and Mortality Associated With Pulmonary Arterial Hypertension in a Schistosomiasis-Endemic Region of Brazil.","authors":"Ricardo Amorim Correa, Camila Farnese Rezende, Eliane Viana Mancuzo, Claudia Mickael, Camila M C Loureiro, Rudolf K F Oliveira, Joan F Hilton, Brian B Graham","doi":"10.1002/pul2.70086","DOIUrl":"10.1002/pul2.70086","url":null,"abstract":"<p><p>Data about pulmonary arterial hypertension (PAH) patients living in low- and middle-income countries remain scarce. This study assessed prognostic factors associated with time to clinical worsening (CW) or death of a cohort of PAH patients in Minas Gerais, Brazil. This retrospective cohort study describes baseline clinical variables by PAH etiology and estimates time from diagnosis to CW [all-cause death, any-cause hospitalization, or disease progression (decrease of ≥ 15% in 6MWD and need for additional PAH therapy or worsening of functional class (FC)] and time to death. 79 out of 102 participants developed CW and 38 died while under follow-up. The most prevalent etiologies were PAH associated with schistosomiasis (PAH-Sch), idiopathic (IPAH), with congenital heart disease (PAH-CHD), and with connective tissue disease (PAH-CTD). The overall median event-free time to CW was 3.3 (95% CI, 2.3-4.6) years, which was similar across etiologies (log-rank test: <i>p</i> = 0.12). WHO FC III-IV, DLCO < 70%, heart rate recovery in 1 min after the 6-min walk test (HRR1) < 18 beats/minute, and baseline mPAP ≥ 50 mmHg were predictive of CW-free time. The median time to all-cause mortality was 10.2 (95% CI, 6.8 - > 10) years and varied among etiologies (log-rank test: <i>p</i> < 0.001). Time to CW was statistically independent of PAH etiology but depended on baseline WHO FC, DLCO, HRR, and mPAP. After CW events, PAH-Sch and PAH-CTD survived less on average than IPAH and PAH-CHD participants.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70086"},"PeriodicalIF":2.2,"publicationDate":"2025-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12034267/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144009726","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 Subgroup Reanalysis of the Efficacy of Bufei Huoxue Capsules in Patients With \"Long-Covid-19\".","authors":"Chi Hou, Yue Xing, Yuqin Chen, Tingping Wang, Jingjing Qi, Xiaoqing Jia, Xiansheng Zeng, Jianling Bai, Wenju Lu, Yu Deng, Bihua Zhong, Yongxia Lei, Yilin Chen, Zhan Lian, Haohao Zhou, Junping Yan, Xuejiao Yang, Hao Yu, Jiawei Zhou, Lixia Qiu, Yunliang Zhai, Wanli Geng, Nanshan Zhong, Chunli Liu, Jian Wang","doi":"10.1002/pul2.70084","DOIUrl":"10.1002/pul2.70084","url":null,"abstract":"<p><p>Bufei huoxue capsules (BFHX), manufactured products of traditional Chinese medicine, have demonstrated anti-inflammatory properties and efficacy against chronic pulmonary diseases and COVID-19. This study was designed to further determine the clinical efficacy of BFHX in diverse patient subgroups during the convalescent phase of COVID-19, extending upon previously reported findings from a multicenter randomized controlled trial. Patients who had clinically recovered from COVID-19 were blindly assigned to BFHX or placebo groups. All enrolled patients underwent chest computed tomography (CT) imaging, 6-min walking distance (6MWD) test, and fatigue assessment inventory (FAI) at monthly follow-up for 3 months. A post hoc subgroup reanalysis was performed on subgroups of sex, age, severity of acute illness, and positive/negative IgG antibody against S antigen variants. A total of 129 patients were enrolled in BFHX (<i>N</i> = 64) and placebo groups (<i>N</i> = 65). The 6MWD and FAI scores were more significantly improved in females and mild patients than in males and severe patients after BFHX treatment. Lung CT image evaluated by the change in whole lung volume and mean CT value showed that the patients below 60 years gained more therapeutic effects after 3 months of BFHX treatment (<i>p</i> = 0.0008; <i>p</i> = 0.017; <i>p</i> = 0.0313, respectively). The subgroup reanalysis implies that the therapeutic effectiveness of BFHX in managing COVID-19 convalescence could potentially be influenced by factors including gender, age, and disease severity. <b>Trial registration:</b> This study was registered with the China Clinical Trial Registration Center (registration number: ChiCTR2000032573).</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70084"},"PeriodicalIF":2.2,"publicationDate":"2025-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12034259/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144012422","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":"Transcriptional Signatures of the Right Ventricle in End-Stage Heart Failure.","authors":"Jonah D Garry, Giovanni E Davogustto, Vineet Agrawal, Fei Ye, Kelsey Tomasek, Yan Ru Su, Tarek Absi, James D West, Anna Hemnes, Evan L Brittain","doi":"10.1002/pul2.70090","DOIUrl":"10.1002/pul2.70090","url":null,"abstract":"<p><p>The molecular mechanisms driving right ventricular (RV) adaptation to stress and failure in end-stage heart failure (HF) are largely unknown. We aimed to characterize myocardial transcriptional changes in the RV caused by left sided HF and comparing RV compensation to failure. Additionally, we compared transcriptomic changes between right and left ventricular (LV) failure. Paired right and left ventricular myocardial tissue samples were obtained from 33 human subjects with end stage HF referred for transplantation and 8 control donors with unused transplant hearts. RV samples from end stage HF subjects were subdivided into compensated (<i>n</i> = 25) and failing (<i>n</i> = 8) categories based on pulmonary artery pulsatility index of < 1.85. All samples underwent bulk tissue RNA-sequencing. We compared gene expression between groups and performed pathway enrichment analysis. Pathways related to fatty acid metabolism and mitochondrial function were negatively enriched, while extracellular structure-related pathways were positively enriched in stressed RVs (compensated and failing) compared to controls. Compensated and failing RVs were differentiated by transcriptional changes in protein production/processing and immune system pathways. PPAR signaling and fatty acid metabolism pathways were consistently enriched in the RV compared to the LV. The RV has a distinct transcriptional signature under stress and in failure. Overlapping molecular mechanisms may underlie RV failure in pulmonary arterial hypertension and HF. Fatty Acid metabolism and associated signaling pathways appear enriched in the RV compared to the LV.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70090"},"PeriodicalIF":2.2,"publicationDate":"2025-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12020025/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144042086","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":"Role of a New Walk Test in Pulmonary Arterial Hypertension: A Retrospective Cohort Study.","authors":"Junhao Jin, Yao Mi, Aqian Wang, Bo Li, Kaiyu Jiang, Hai Zhu, Ting Liang, Hongling Su, Yunshan Cao","doi":"10.1002/pul2.70087","DOIUrl":"10.1002/pul2.70087","url":null,"abstract":"<p><p>The 6-min walk test (6MWT) has significant prognostic value, but requires long walking distances and lacks evaluation of exercise speed. This study aimed to investigate the clinical utility of a new walk test, the 18-meter walk test (18MWT), in patients with pulmonary arterial hypertension (PAH) as a complement to the 6MWT. In summary, a total of 117 patients with PAH from January 2018 to December 2022 were included. Spearman correlation, Cox regression, and Kaplan-Meier analysis were utilized to demonstrate the value of 18MWT in predicting disease severity and clinical worsening. The median time to complete the 18MWT was 12.8 s (interquartile range: 11.3-14.6 s). 18MWT completion time showed significant correlations with indicators such as N-terminal pro-brain natriuretic peptide and 6MWT distance. Adjusted Cox regression showed 18MWT time remained an independent predictor of clinical worsening (hazard ratio = 1.10; 95% confidence interval: 1.01-1.21; <i>p</i> = 0.026). A simplified risk stratification using WHO functional class, 6MWT distance, 18MWT time and NT-proBNP was predictive of 1-year clinical outcome. These results suggest that the 18MWT provides clinicians with an efficient measure that can be used to evaluate the disease severity of PAH patients and to identify those patients at greater risk for future clinical worsening as a complement to the 6MWT.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70087"},"PeriodicalIF":2.2,"publicationDate":"2025-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12021992/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143991235","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":"Approach to Lung Transplantation in Pulmonary Arterial Hypertension: A Delphi Consensus on Behalf of the Transplant Task Force of the Pulmonary Vascular Research Institute.","authors":"Nicholas A Kolaitis, Hayley Barnes, Deborah J Levine, Howard Castillo, Selim M Arcasoy, Matthew Bacchetta, Luke Benvenuto, Erika Berman-Rosenzweig, Marisa Cevasco, Caitlin T Demarest, Celine Dewachter, Michiel E Erasmus, Allan R Glanville, John Granton, Shaf Keshavjee, Vikramjit Khangoora, Sheila Krishnan, Olaf Mercier, Andrea N Miltiades, David Montani, Edward Murphy, Ivan Robbins, Franck F Rahaghi, Sahar A Saddoughi, Laurent Savale, Marc A Simon, Jean-Luc Vachiery, Corey E Ventetuolo, Helen M Whitford, Reda E Girgis","doi":"10.1002/pul2.70088","DOIUrl":"10.1002/pul2.70088","url":null,"abstract":"<p><p>Lung transplantation is indicated for selected patients with advanced pulmonary arterial hypertension (PAH). We used a modified Delphi process to develop recommendations on care of patients with PAH undergoing lung transplantation. This Delphi panel was recruited from the Pulmonary Vascular Research Institute's Innovative Drug Discovery Initiative - Lung Transplantation Workstream, consisting of clinical and research experts in PAH and lung transplantation. In this process, 29 panelists were given open-ended questions, querying topics related to lung transplantation in PAH. A steering group converted the responses into discrete statements. Panelists then rated agreement using a Likert scale in two further survey rounds: -5 (strongly disagree) to 5 (strongly agree). Consensus was defined as mean ≥ 2.5 or ≤ -2.5, with a standard deviation not crossing zero. Consensus was reached on 141 of 223 statements. Notable areas of consensus were for early discussions about transplantation, and agreement with previously published referral and listing criteria. There was agreement that lung transplantation could be offered in sick candidates, including those with concurrent renal or hepatic insufficiency. Bilateral lung transplantation was considered the procedure of choice for most patients, with rare indications for heart-lung transplantation. Consensus on bridging strategies included use of veno-arterial extracorporeal membrane oxygenation and preemptive awake cannulation in those with severe right ventricular dysfunction. Consensus was also achieved on intraoperative use of invasive hemodynamic monitoring, and prolonged postoperative circulatory support guided by hemodynamic response and echocardiography. Patients with PAH undergoing transplantation require specialized management, which differs somewhat from other candidates.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70088"},"PeriodicalIF":2.2,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12018530/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144028801","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":"Outcomes of Multidisciplinary Care at a Chronic Thromboembolic Pulmonary Hypertension Center.","authors":"S Christopher Malaisrie, Stephen Chiu, Daniel Schimmel, Maanasi Samant, Ryan Avery, Amir Rahsepar, Bradley Allen, Yasmin Raza, Benjamin Freed, Ruben Mylvaganam, Michael J Cuttica","doi":"10.1002/pul2.70085","DOIUrl":"https://doi.org/10.1002/pul2.70085","url":null,"abstract":"<p><p>Recent international guidelines recommend a multidisciplinary evaluation and care model for patients with chronic thromboembolic pulmonary hypertension (CTEPH), but there is a paucity of supporting data. The aim of this study was to describe the outcomes of a multidisciplinary team approach to the comprehensive care of CTEPH patients. This single-center cohort study enrolled 166 consecutive adult patients undergoing CTEPH treatment evaluation from 2016 to 2022 at a tertiary care, academic regional referral and comprehensive CTEPH center with pulmonary thromboendarterectomy (PTE) and balloon pulmonary angioplasty (BPA) capabilities. Patients underwent PTE, BPA, or medical management after consensus evaluation by a multidisciplinary team including pulmonary hypertension physicians, surgeons, interventional cardiologists, and radiologists. 86% (142/166) of patients underwent interventional therapies; 100 (60%) underwent PTE and 42 (25%) BPA. Of the 24 (14%) medically treated patients, 13 patients were offered but deferred intervention; 11 patients had non-intervenable disease. 30-day mortality in both PTE and BPA was 0%. 1- and 3-year survival was 99% and 96% for PTE, 100% and 93% for BPA, 79% and 79% for medical management. Patients who underwent PTE had the best hemodynamic response (∆PVR: PTE -278.8 ± 366.9 dyne/sec/cm⁵; BPA -15.9 ± 171.8 dyne/sec/cm⁵; medical -60.2 ± 233.1 dyne/sec/cm⁵; <i>p</i> = 0.001), largest improvement in Borg Dyspnea Scale; [PTE -1.0 (-2.8 to 0.0), BPA + 0.5 (-0.8 to 5.0), medical +1.0 (0.75 to 3.0), <i>p</i> = 0.01], and most improvement in NYHA functional class [% improving at least 1 functional class: PTE 64% (47/73), BPA 18% (5/28), medical 21% (4/19), <i>p</i> = 0.0004].</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70085"},"PeriodicalIF":2.2,"publicationDate":"2025-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12011997/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144045971","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":"Societal Costs Associated With Pulmonary Arterial Hypertension Subgroups: A Study Utilizing Linked National Registries.","authors":"Barbro Kjellström, Bodil Ivarsson, Magnus Husberg, Lars-Åke Levin, Lars Bernfort","doi":"10.1002/pul2.70074","DOIUrl":"10.1002/pul2.70074","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a heterogenic diagnosis including idiopathic and hereditary PAH (IPAH/HPAH) and groups associated to connective tissue disease (APAH-CTD) and congenital heart disease (APAH-CHD). Pre- and post-diagnosis societal costs in PAH subgroups are not well known. By linking Swedish national databases, societal costs in a national PAH cohort 5 years before and 5 years after diagnosis were estimated and compared to an age, sex, and geographically matched control group (1:5 match). Incident patients diagnosed 2008-2019 were included (patient/control; IPAH/HPAH = 393/1965, APAH-CTD = 261/1305, APAH-CHD = 89/445). Pre-diagnosis mean societal costs were 2.9, 3.4, and 4.3 times higher for IPAH/HPAH, APAH-CTD and APAH-CHD patients, respectively, than controls. Post-diagnosis, mean costs had increased 3.1, 2.0, and 1.6 times further for IPAH/HPAH, APAH-CTD and APAH-CHD respectively, while it decreased in all control groups. Main cost driver pre-diagnosis were indirect costs (productivity loss) in both patient and control groups, however, 2.7-4.5 times higher in the patient groups. Post-diagnosis, the main cost driver for all groups were health care costs (in- and outpatient-care, drugs) that had increased 7.8, 5.4 and 6.8 times for IPAH/HPAH, APAH-CTD and APAH-CHD, respectively. Corresponding increase for controls were 17%-48%. For the PAH groups, drug treatment accounted for 70%-81% of the direct costs, while hospitalizations were the main driver for the control groups. In conclusion, PAH was associated with large societal costs. Pre-diagnosis, APAH-CHD had the highest societal costs, both in relation to their control group and compared to the other patient groups. Post-diagnosis, highest societal costs were seen in IPAH/HPAH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70074"},"PeriodicalIF":2.2,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12005055/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144012428","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":"Alterations in ECG and Right Heart Catheterization Data in PAH Patients Who Died From Sudden Death Compared With Right Heart Failure.","authors":"Alexandra B Flemington, Jeffery Annis, Evan L Brittain, Anna R Hemnes","doi":"10.1002/pul2.70082","DOIUrl":"10.1002/pul2.70082","url":null,"abstract":"<p><p>A meaningful number of patients with PAH die suddenly, and there is little data to understand the events surrounding sudden death in PAH. We tested the hypothesis that sudden death is associated with pre-mortem ECG or hemodynamics changes compared to those who died of RHF. We extracted data from the Vanderbilt University Medical Center Synthetic Derivative. Patients 18 years of age and older with Group 1 PAH secondary to any etiology who died between 2009 and 2017 with both ECG and RHC data from the inpatient and outpatient setting were included in the study. Continuous variables were compared using the Wilcoxon rank-sum test while categorical variables were compared using the <i>χ</i> ² test. Logistic regression models, adjusted for age and sex, were then used to evaluate the association between death and specific ECG or RHC measurements. Comparing the final ECG before death, those who died of SD had significantly shorter terminal 40 ms interval of the QRS than those who died of RHF, which became nonsignificant when adjusted for age and sex. We observed differences in baseline RHC data between SD and RHF including higher RV systolic pressure which remained significant when adjusted for age and sex. Using this data, we hope to find clinical data that can be used to predict increased risk of sudden death and aid in stratifying Group I PAH patients to earlier and more aggressive interventions.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70082"},"PeriodicalIF":2.2,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12005591/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143993762","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}