Pulmonary Circulation最新文献

{"title":"Inoperable chronic thromboembolic pulmonary hypertension: Evolution of prognosis over 10 years of new emerging therapies.","authors":"Diederik P Staal, Paul M Hendriks, Mitch C J van Thor, Liza D van de Groep, Leon M van den Toorn, Berend-Jan M Mulder, Prewesh P Chandoesing, Robert M Kauling, Sanne Boerman, Annemien E van den Bosch, Johannes J Mager, Karin A Boomars, Martijn C Post","doi":"10.1002/pul2.12419","DOIUrl":"10.1002/pul2.12419","url":null,"abstract":"<p><p>Therapies for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) include balloon pulmonary angioplasty (BPA) and PH-specific medical therapy. This study compares survival and its predictors before and after the introduction of BPA. BPA was independently associated with survival; however, there was no difference in overall survival between the two cohorts.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12419"},"PeriodicalIF":2.2,"publicationDate":"2024-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11340011/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142036788","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":"Novel evaluation of pulmonary hypertension associated with chronic lung disease using perfusion SPECT/CT: A pilot study.","authors":"Kenichiro Atsumi, Yoshimitsu Fukushima, Yosuke Tanaka, Shunichi Nishima, Toru Tanaka, Masahiro Seike, Yoshiaki Kubota, Hiroshi Kimura","doi":"10.1002/pul2.12423","DOIUrl":"10.1002/pul2.12423","url":null,"abstract":"<p><p>In pulmonary hypertension (PH) associated with chronic lung disease (CLD), identifying patients who would benefit from pulmonary vasodilators is a significant clinical challenge because the presence of PH is associated with poorer survival. This study evaluated the severity of pulmonary circulation impairment in patients with CLD-PH using pulmonary perfusion single-photon emission computed tomography/computed tomography (SPECT/CT). This single-center, observational study enrolled patients with CLD-PH who had a mean pulmonary arterial pressure (PAP) ≥ 25 mmHg, as confirmed by right heart catheterization. The primary outcome was to measure the percentage of pulmonary perfusion defect (%PPD), calculated by dividing the perfusion defect volume from perfusion SPECT images by the lung volume from CT scan images. The secondary outcome was to assess the correlation between %PPD and baseline characteristics. The median %PPD was 52.4% (interquartile range, 42.5%-72.3%) in 22 patients. In multivariate linear regression analysis, both forced vital capacity (<i>β</i> = 0.58, <i>p</i> = 0.008) and mean PAP (<i>β</i> = 0.68, <i>p</i> = 0.001) were significantly correlated with %PPD. In conclusion, significant correlation between mean PAP and %PPD in patients with CLD-PH was observed. This noninvasive assessment of %PPD may be useful for evaluating the severity of pulmonary circulation impairment in CLD-PH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12423"},"PeriodicalIF":2.2,"publicationDate":"2024-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11337537/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142018409","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":"Editorial on \"Characteristics and risk profiles of patients with pulmonary arterial or chronic thromboembolic pulmonary hypertension living permanently at >2500 m of high altitude in Ecuador\".","authors":"Samantha Sharma, Naresh Singh","doi":"10.1002/pul2.12428","DOIUrl":"10.1002/pul2.12428","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12428"},"PeriodicalIF":2.2,"publicationDate":"2024-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11332251/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142005125","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":"Impact of selexipag maintenance dose on persistence, adherence, and hospitalization in US patients with pulmonary arterial hypertension.","authors":"Charles D Burger, Wenze Tang, Yuen Tsang, Sumeet Panjabi","doi":"10.1002/pul2.12415","DOIUrl":"10.1002/pul2.12415","url":null,"abstract":"<p><p>Selexipag is an oral selective agonist of the prostacyclin receptor approved to treat adults with pulmonary arterial hypertension (PAH). Selexipag is initiated at a dose of 200 μg twice daily (bid) and usually titrated up by 200 μg bid weekly (per label) or more slowly (e.g., every other week in real-world clinical practice) to the highest tolerated individualized dose (ID) ranging from 200 to 1600 µg bid. In the Phase 3 GRIPHON trial, selexipag delayed disease progression and reduced risk of PAH-related hospitalization compared with placebo; the effect was consistent across three prespecified ID groups: low (200-400 µg bid), medium (600-1000 µg bid), and high (1200-1600 µg bid). This study evaluated patient outcomes across selexipag dose ranges in real-world practice. Data were analyzed from 1186 US adult patients with PAH on selexipag from the Komodo closed-claims database (2015‒2022). Of these, 634 (53.5%) patients completed titration and reached their selexipag ID (43.8% high ID, 29.8% medium ID, 26.3% low ID). Subsequently, 72.4% of patients in the low ID group had dose adjustments compared with 61.9% (medium ID) and 34.5% (high ID; standardized mean difference 0.63). There were no significant differences in patient outcomes, i,e, persistence (time to discontinuation) and risk of all-cause and PAH-related hospitalization across ID groups. The findings in this diverse, real-world population of patients with PAH reinforced an individualized approach to the dosing scheme to maximize benefit-risk and achieve the highest tolerated dose with selexipag similar to findings from the GRIPHON trial and other studies.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12415"},"PeriodicalIF":2.2,"publicationDate":"2024-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11330629/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142000533","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":"Carnitine consumption and effect of oral supplementation in human pulmonary arterial hypertension: A pilot study.","authors":"Evan L Brittain, Alisha Lindsey, Kelly Burke, Vineet Agrawal, Ivan Robbins, Meredith Pugh, M Wade Calcutt, Ravi Mallugari, James West, Hui Nian, Anna R Hemnes","doi":"10.1002/pul2.12425","DOIUrl":"10.1002/pul2.12425","url":null,"abstract":"<p><p>Carnitine is required to transport fatty acid across the mitochondrial membrane to undergo beta oxidation. In addition to disorders of fatty acid metabolism, a relative carnitine deficiency has been reported in pulmonary arterial hypertension (PAH). Here we performed an observational study in which food and supplement consumption were collected in an observation period followed by open label administration of a carnitine supplement to determine feasibility of increasing plasma carnitine levels in humans PAH. We confirmed that relative carnitine deficiency in PAH is not due to reduced dietary consumption and that plasma levels of carnitine can be increased in PAH patients with supplementation that is well tolerated.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12425"},"PeriodicalIF":2.2,"publicationDate":"2024-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11327271/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142000604","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":"Pretransplant NT-proBNP levels are associated with mortality among lung transplant recipients.","authors":"Shimon Izhakian, Assaf Frajman, Ariel D Hayat, Alon Gorenshtein, Osnat Shtraichman, Lev Freidkin, Dror Rosengarten, Mordechai R Kramer","doi":"10.1002/pul2.12427","DOIUrl":"10.1002/pul2.12427","url":null,"abstract":"<p><p>The prognostic significance of pretransplant N-terminal pro-brain (B)-type natriuretic peptide (NT-proBNP) level has not been investigated in lung transplant recipients. The electronic files of 173 patients with chronic lung disease who underwent lung transplantation in 2018-2022 at a tertiary medical center were retrospectively reviewed. Right heart catheterization (RHC) and NT-proBNP determination were performed preoperatively in all cases. Pretransplant demographic, clinical, and laboratory data were compared between posttransplant survivors and nonsurvivors. Correlations of NT-proBNP values with lung function and RHC parameters and all-cause mortality were analyzed. NT-proBNP level correlated positively with mean pulmonary artery pressure (<i>R</i> = 0.51, <i>p</i> < 0.001) and pulmonary vascular resistance (PVR) (<i>R</i> = 0.45, <i>p</i> = 0.0013), and negatively with diffusing lung capacity for carbon monoxide (<i>R</i> = -0.25, <i>p</i> = 0.0017), cardiac index (<i>R</i> = -0.26, <i>p</i> = 0.001), and cardiac output (<i>R</i> = -0.23, <i>p</i> = 0.004). Over a median follow-up time of 23.22 months, 74 patients died. On univariate analysis, mortality was significantly associated with higher log-NT-proBNP (hazard ratio [HR] = 0.54, 95% confidence interval [CI] 1.15-2.05, <i>p</i> = 0.016), older age at transplant registration (HR = 1.033, 95% CI 1.009-1.058, <i>p</i> = 0.0068), higher PVR (HR 1.15, 95% CI 1.07-1.23, <i>p</i> = 0.015), and lower cardiac output (HR = 0.62, 95% CI 0.42-0.92, <i>p</i> = 0.045). On multivariate analysis adjusted for age, sex, and body mass index, mortality significance was maintained only for higher log-NT-proBNP (HR = 1.54, 95% CI 1.12-2.11, <i>p</i> = 0.007). Among lung transplant recipients, pretransplant NT-proBNP levels correlated well with RHC parameters and were strongly associated with posttransplantation mortality. Assessment of NT-proBNP may improve risk stratification of lung transplant candidates.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12427"},"PeriodicalIF":2.2,"publicationDate":"2024-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11327270/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142000534","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":"Immature reticulocyte fraction: A novel biomarker of hemodynamic severity in pulmonary arterial hypertension.","authors":"Adam J Brownstein, Jared D Wilkinson, Lloyd L Liang, Richard N Channick, Rajan Saggar, Airie Kim","doi":"10.1002/pul2.12421","DOIUrl":"10.1002/pul2.12421","url":null,"abstract":"<p><p>Various erythropoietic abnormalities are highly prevalent among patients with pulmonary arterial hypertension (PAH) and associated with worse disease severity. Given the poorly understood yet important roles of dysregulated erythropoiesis and iron metabolism in PAH, we sought to further characterize the hematologic and iron profiles in PAH and their relationship to PAH severity. We recruited 67 patients with PAH and 13 healthy controls. Hemodynamics attained within 1 year of blood sample collection were available for 36 patients. Multiple hematologic, iron, and inflammatory parameters were evaluated for their association with hemodynamics. The subset with hemodynamic data consisted of 29 females (81%). The most common etiologies were idiopathic PAH (47%) and connective tissue disease-related PAH (33%). 19 (53%) had functional class 3 or 4 symptomatology, and 12 (33%) were on triple pulmonary vasodilator therapy. Immature reticulocyte fraction (IRF) had significant positive correlations with mean pulmonary artery (PA) pressure (mPAP) (0.59, <i>p</i> < 0.001), pulmonary vascular resistance (0.52, <i>p</i> = 0.001), and right atrial pressure (0.46, <i>p</i> = 0.005), and significant negative correlations with cardiac index (-0.43, <i>p</i> = 0.009), PA compliance (PAC) (-0.60, <i>p</i> < 0.001), stroke volume index (SVI) (-0.57, <i>p</i> < 0.001), and mixed venous oxygen saturation (-0.51, <i>p</i> = 0.003). IRF correlated with markers of iron deficiency (ID) and erythropoiesis. On multivariable linear regression, IRF was associated with elevated mPAP and reduced SVI and PAC independent of EPO levels, transferrin saturation, and soluble transferrin receptor levels. We identified IRF as a novel and potent biomarker of PAH hemodynamic severity, possibly related to its associations with erythropoiesis, ID, and tissue hypoxia.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12421"},"PeriodicalIF":2.2,"publicationDate":"2024-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11298897/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141894205","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":"Air travel in patients suffering from pulmonary hypertension-A prospective, multicentre study.","authors":"Athiththan Yogeswaran, Jan Grimminger, Khodr Tello, Lukas Becker, Werner Seeger, Friedrich Grimminger, Natascha Sommer, Hossein A Ghofrani, Tobias J Lange, Stefan Stadler, Karen Olsson, Jan C Kamp, Stephan Rosenkranz, Felix Gerhardt, Katrin Milger, Michaela Barnikel, Silvia Ulrich, Stéphanie Saxer, Ekkehard Grünig, Satenik Harutynova, Christian Opitz, Hans Klose, Heinrike Wilkens, Michael Halank, Melanie Heberling, Henning Gall, Manuel J Richter","doi":"10.1002/pul2.12397","DOIUrl":"10.1002/pul2.12397","url":null,"abstract":"<p><p>The PEGASUS study is the first multicentric and prospective assessment of the safety of air travel flying in pulmonary hypertension (PH) (NCT03051763). Data of air travel from 60 patients with PH was available. No severe adverse events occurred. Nine patients self-reported mild adverse events during flight (13%), while after landing, 12 patients reported events (20%). Solely one patient (2%) had an adverse event leading to medical consultation. In patients with PH and World Health Organization functional classes II and III, air travel was safe.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12397"},"PeriodicalIF":2.2,"publicationDate":"2024-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11298895/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141894204","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":"Clinical characteristics and anticoagulation patterns of patients with acute pulmonary thromboembolism and hemoptysis.","authors":"Yiyao Li, Peijun Xue, Ting Zhang, Min Peng, Xuefeng Sun, Juhong Shi","doi":"10.1002/pul2.12422","DOIUrl":"10.1002/pul2.12422","url":null,"abstract":"<p><p>Hemoptysis is a frequently encountered manifestation in cases of acute pulmonary thromboembolism (PTE), significantly impacting clinical decision-making. Despite its clinical relevance, studies focusing on patients with acute PTE and hemoptysis are notably scarce. In this retrospective study, we examined data from hospitalized patients with acute PTE at Peking Union Medical College Hospital (PUMCH) between January 2012 and October 2020. Among the 896 patients analyzed, 105 (11.7%) presented with hemoptysis. Patients with hemoptysis were younger, had higher RRs, and frequently reported chest pain, predominantly showing a negative sPESI score. A significant association with autoimmune diseases was observed (39.0% vs. 16.1%; <i>p</i> < 0.001), along with higher occurrences of pulmonary infections (29.5%), lung cancer (21.0%), and chronic heart failure (16.2%). Hemoptysis in PTE is multifactorial; 51.4% of cases were PTE-related, with 85.2% experiencing mild hemoptysis. Among patients with disease-related hemoptysis (13.3%), 90.9% with massive hemoptysis had underlying diseases, predominantly lung cancer. In 35.2% of cases, the cause of hemoptysis remained undetermined, with vasculitis accounting for 29.7%. Anticoagulation strategies varied with the severity of hemoptysis; 82.9% with mild and only 27.3% with massive hemoptysis received therapeutic-dose anticoagulation. Multivariate analysis identified massive hemoptysis as the most significant determinant of anticoagulation decisions. Patients with massive hemoptysis had the poorest outcomes, with an in-hospital mortality rate of 36.4% and 72.7% receiving reduced or no anticoagulation.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12422"},"PeriodicalIF":2.2,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11291552/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141875788","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":"Editorial on \"A systematic review of the impact of pulmonary thromboendarterectomy on health related quality of life\".","authors":"Alison Witkin","doi":"10.1002/pul2.12420","DOIUrl":"10.1002/pul2.12420","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12420"},"PeriodicalIF":2.2,"publicationDate":"2024-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11284448/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141793258","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}