Pulmonary CirculationPub Date : 2026-07-18eCollection Date: 2026-07-01DOI: 10.1002/pul2.70370
Anna Smukowska-Gorynia, Sylwia Iwańczyk, Michał Ciurzyński, Marcin Waligóra, Marcin Kurzyna, Piotr Pruszczyk, Grzegorz Kopeć, Katarzyna Mizia-Stec, Ilona Skoczylas, Łukasz Chrzanowski, Piotr Błaszczak, Katarzyna Ptaszyńska, Grzegorz Grześk, Ewa Malinowska, Małgorzata Peregud-Pogorzelska, Michał Tomaszewski, Ewa Mroczek, Miłosz Jaguszewski, Ewa Lewicka, Katarzyna Widejko, Agnieszka Pawlak, Wojciech Jacheć, Zbigniew Gąsior, Katarzyna Betkier-Lipińska, Judyta Winowska-Józwa, Marta Banaszkiewicz, Tatiana Mularek-Kubzdela
{"title":"Effectiveness of PAH Specific Treatment Is Comparable Between PAH Associated With Connective Tissue Disease and Idiopathic PAH: Data From the Multicentre National Registry.","authors":"Anna Smukowska-Gorynia, Sylwia Iwańczyk, Michał Ciurzyński, Marcin Waligóra, Marcin Kurzyna, Piotr Pruszczyk, Grzegorz Kopeć, Katarzyna Mizia-Stec, Ilona Skoczylas, Łukasz Chrzanowski, Piotr Błaszczak, Katarzyna Ptaszyńska, Grzegorz Grześk, Ewa Malinowska, Małgorzata Peregud-Pogorzelska, Michał Tomaszewski, Ewa Mroczek, Miłosz Jaguszewski, Ewa Lewicka, Katarzyna Widejko, Agnieszka Pawlak, Wojciech Jacheć, Zbigniew Gąsior, Katarzyna Betkier-Lipińska, Judyta Winowska-Józwa, Marta Banaszkiewicz, Tatiana Mularek-Kubzdela","doi":"10.1002/pul2.70370","DOIUrl":"10.1002/pul2.70370","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a rare disease with poor prognosis. The same treatment is recommended in patients with PAH associated with connective tissue disease (CTD) and idiopathic PAH (IPAH). However, the effectiveness of PAH-specific treatment in these both groups is inconsistent. The study incorporated data from the prospective, multicentre polish registry (BNP-PL) by comparison of baseline versus 1-year follow-up clinical, laboratory, echocardiographic and hemodynamic parameters in PAH-CTD versus IPAH group. The all-cause 1-year mortality was also analysed. 624 patients were included in the study: 176 (28%) PAH-CTD and 448 (72%) IPAH. 48 (8% of the total 624) patients died during 1-year follow up: 32 (7%) with IPAH and 16 (9%) with PAH-CTD (<i>p</i> = 0.4). The effectiveness of treatment was examined in 421 patients: 115 (27%) PAH-CTD and 306 (73%) IPAH. The improvement of treatment was effective in both groups in terms of WHO FC (<i>p</i> < 0.001 in both groups), 4-strata risk model (<i>p</i> < 0.001 in both groups), 6 min walking test (6MWT, <i>p</i> < 0.001 in IPAH, <i>p</i> = 0.001 in PAH-CTD), Δ6MWT (<i>p</i> = 0.19 between PAH-CTD and IPAH), tricuspid annular plane systolic excursion (<i>p</i> < 0.001 in both groups), NT- proBNP (<i>p</i> < 0.001 in IPAH and <i>p</i> < 0.01 in PAH-CTD), mean pulmonary arterial pressure (<i>p</i> = 0.043 in IPAH and <i>p</i> = 0.01 in PAH-CTD), pulmonary vascular resistance (<i>p</i> < 0.001 in both groups) and cardiac output (<i>p</i> < 0.001 in IPAH and <i>p</i> = 0.034 in PAH-CTD). PAH specific treatment in PAH-CTD patients is as effective as in IPAH patients regarding improvement in WHO FC, 4-strata risk model, 6MWT, Δ6MWT, TAPSE, NT-proBNP, mPAP, PVR and CO at 1-year follow-up. 1-year all-cause mortality was not significantly different between PAH-CTD and IPAH patients.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70370"},"PeriodicalIF":2.4,"publicationDate":"2026-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13404781/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148607004","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}
Pulmonary CirculationPub Date : 2026-07-17eCollection Date: 2026-07-01DOI: 10.1002/pul2.70369
Scott A Barman, Zsuzsanna Bordan, David J R Fulton
{"title":"Evidence for PDZ-Binding Kinase in Lung Disease With an Emphasis on PAH.","authors":"Scott A Barman, Zsuzsanna Bordan, David J R Fulton","doi":"10.1002/pul2.70369","DOIUrl":"10.1002/pul2.70369","url":null,"abstract":"<p><p>Originally named T-cell-originated Lymphokine-activated killer protein kinase (TOPK), PDZ-Binding Kinase (PBK) is a serine/threonine kinase that is a member of the family of mitogen-activated protein kinases (MAPKKs), which is overexpressed in lung cancer and interstitial pulmonary fibrosis (IPF). Along these lines, recent work also shows that PBK expression is upregulated in pulmonary arterial hypertension (PAH) in part due to the inappropriate proliferation of pulmonary arterial smooth muscle cells (PASMC), similar to the hyper-proliferative cellular properties seen in both lung cancer and IPF. Genetic knock-out of PBK as well as pharmacologically selective inhibitors of PBK improve PA remodeling and cardiopulmonary function. Further, PBK bound to the Protein Regulator of Cytokinesis 1 (PRC1) induces PRC1 phosphorylation and cytokinesis in PASMC, and thus, it is conjectured that PBK enhances PASMC proliferation via PRC1-mediated cytokinesis, identifying a key mechanism which contributes to the pathologic pulmonary vascular remodeling that occurs in PAH. Towards this end, along with the already published studies showing that PBK is involved in both lung cancer and IPF, PBK-mediated pulmonary vascular remodeling reveals a new signaling pathway in lung vascular disease and a novel mechanism of PASMC proliferation. Exploration of this signaling pathway will advance the utility of identifying novel therapeutic approaches targeting PBK-mediated cytokinesis to reduce pulmonary vascular remodeling (as seen in PAH) and subsequently improve the morbidity and mortality associated with lung vascular disease. Collectively, the literature strongly suggests that PBK is a worthy and viable therapeutic target to pursue in the context of lung disease.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70369"},"PeriodicalIF":2.4,"publicationDate":"2026-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13404778/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148606980","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}
Pulmonary CirculationPub Date : 2026-07-17eCollection Date: 2026-07-01DOI: 10.1002/pul2.70363
Carolin Kränzle, Vera Bissig, Simon R Schneider, Julian Müller, Kay von Gruenigen, Meret Bauer, Laura Mayer, Lea Lüönd, Tanja Ulrich, Aglaia Forrer, Arcangelo Carta, Ester I Schwarz, Konrad E Bloch, Mona Lichtblau, Michael Furian, Silvia Ulrich
{"title":"Nocturnal Cerebral Oxygenation in Low-Altitude Residents With Pulmonary Vascular Disease Staying Overnight at High Altitude. Data From a Randomized, Controlled, Crossover Trial.","authors":"Carolin Kränzle, Vera Bissig, Simon R Schneider, Julian Müller, Kay von Gruenigen, Meret Bauer, Laura Mayer, Lea Lüönd, Tanja Ulrich, Aglaia Forrer, Arcangelo Carta, Ester I Schwarz, Konrad E Bloch, Mona Lichtblau, Michael Furian, Silvia Ulrich","doi":"10.1002/pul2.70363","DOIUrl":"10.1002/pul2.70363","url":null,"abstract":"<p><p>Whether hypoxia during popular high-altitude travel negatively affects cerebral oxygenation in vulnerable patients with pulmonary vascular disease (PVD) is unknown. We studied overnight cerebral tissue oxygen saturation (CTO) and desaturation index (cODI) in PVD-patients at 2500 m and effects of supplemental oxygen therapy (SOT). In this randomized-controlled crossover trial, stable PVD-patients diagnosed with pulmonary arterial or distal chronic thromboembolic pulmonary hypertension (PAH/CTEPH) had CTO and cODI (decrease in CTO ≥ 4%) assessed overnight at 470 m and 2500 m along with fingertip pulseoximetry (SpO<sub>2</sub>) and desaturation index (aODI). For safety, SOT was given if SpO<sub>2</sub> dropped < 80% for > 30 min and stratified analysis accordingly. Primary endpoint was the difference in CTO between 470 and 2500 m. 16 PVD-patients (7 women; 13 PAH, 3 CTEPH), (mean ± SD) 56 ± 14 years old, were included per-protocol. At 470 m, the mean nocturnal CTO was 66.3 ± 1.6% and SpO<sub>2</sub> 90.3 ± 0.8%. At 2500 m on ambient air, CTO was unchanged 65.9 ± 1.6% (mean difference -0.4%; 95% CI -4.0 to 3.2) despite a lower SpO<sub>2</sub> of 83.6 ± 0.8; -6.7 (-8.7 to -4.6%). At 2500 vs. 470 m, cODI increased by 3.1 events/hour (-0.3 to 6.6). SOT needed by 50% of PH-patients improved SpO<sub>2</sub> by +7.5 (4.9 to 10.0), but not CTO and cODI (+3.6% (-1.0 to 8.2)) and -3.8 events/hour (-8.1 to 0.5). In PVD-patients on ambient air at 2500 m, nocturnal CTO remained unchanged compared to 470 m despite lower SpO<sub>2</sub>. SOT promptly improved SpO<sub>2</sub> without affecting cerebral oxygenation. These findings suggest sufficient nocturnal cerebral protection at 2500 m in PVD.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70363"},"PeriodicalIF":2.4,"publicationDate":"2026-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13404777/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148607013","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}
Pulmonary CirculationPub Date : 2026-07-17eCollection Date: 2026-07-01DOI: 10.1002/pul2.70354
Emmanouil Mavrogiannis, Rebeca Weldeghebreal, Iris R Schilthuis, Zain K Fal, Niels J Kloosterhuis, Mirjam H Koster, Wim Timens, Johannes M Douwes, Rolf M F Berger, Marit Westerterp
{"title":"The NLRP3 Inflammasome Increases Pulmonary Vascular Remodeling in Experimental Pulmonary Arterial Hypertension.","authors":"Emmanouil Mavrogiannis, Rebeca Weldeghebreal, Iris R Schilthuis, Zain K Fal, Niels J Kloosterhuis, Mirjam H Koster, Wim Timens, Johannes M Douwes, Rolf M F Berger, Marit Westerterp","doi":"10.1002/pul2.70354","DOIUrl":"10.1002/pul2.70354","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a progressive vasculopathy leading to right-sided heart failure. We have shown previously that the (NOD-like-receptor-3) NLRP3 inflammasome is activated in end-stage disease of the monocrotaline and aortocaval shunt (MCT/ACS) neointimal PAH rat model. The NLRP3 and absent-in-melanoma 2 (AIM2) inflammasomes mediate the secretion of interleukin (IL)-1β and IL-18. We investigated the role of the NLRP3 inflammasome in PAH rats and the presence of NLRP3 and AIM2 in PAH rat and pediatric patient lungs. For a natural history study, we assessed inflammasome activation by Western blot at different disease stages in the MCT/ACS neointimal PAH rat model. To assess the role of the inflammasome in PAH, we treated PAH rats with or without the NLRP3 inflammasome inhibitor MCC950 and assessed vascular remodeling employing hematoxylin and eosin staining on rat lungs. Lung sections from patients who had undergone lung transplantation were stained for NLRP3 and AIM2 using immunofluorescence. We found that inflammasome activation in lungs of PAH rats, reflected by cleaved caspase-1, IL-1β, and IL-18, increased over time, reaching significance for cleaved caspase-1 and IL-18 in end-stage PAH. The NLRP3 inflammasome inhibitor MCC950 suppressed vascular remodeling but not hemodynamic variables compared to control PAH rats. We detected NLRP3<sup>+</sup> and AIM2<sup>+</sup> cells in end-stage pediatric PAH lungs. In conclusion, inflammasome activation increases over time in the MCT/ACS neointimal PAH rat model, and NLRP3 inflammasome activation increases vascular remodeling. NLRP3 and AIM2 positive cells are present in end-stage pediatric patient PAH lungs, suggesting human relevance.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70354"},"PeriodicalIF":2.4,"publicationDate":"2026-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13404779/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148607110","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}
Pulmonary CirculationPub Date : 2026-07-17eCollection Date: 2026-07-01DOI: 10.1002/pul2.70340
Karla Cruz Morel, Namita Sood, Roberto Bernardo, Kimberly Jackson, Gillian P Leung, Charles Burger, Jean M Elwing
{"title":"Navigating the Complexities of Pulmonary Hypertension Care: Unique Challenges in PAH Management.","authors":"Karla Cruz Morel, Namita Sood, Roberto Bernardo, Kimberly Jackson, Gillian P Leung, Charles Burger, Jean M Elwing","doi":"10.1002/pul2.70340","DOIUrl":"10.1002/pul2.70340","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) comprises a heterogeneous group of disorders characterized by elevated pressure in the pulmonary arteries. Despite advances in awareness, utilization of non-invasive screening with echocardiography, increasing number of targeted therapies, and evolving clinical guidelines emphasizing referral to PH specialty centers, barriers to timely diagnosis and treatment persist. Key challenges span patient-related and system-level domains that hinder the effective diagnosis, management, and treatment of PH, particularly pulmonary arterial hypertension (PAH). In this article, the obstacles to PH care are examined and actionable recommendations are proposed. By integrating patient-related and system-level perspectives, this review provides a framework to bridge persistent gaps in PH care delivery in the United States.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70340"},"PeriodicalIF":2.4,"publicationDate":"2026-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13404776/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148607074","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}
Pulmonary CirculationPub Date : 2026-07-16eCollection Date: 2026-07-01DOI: 10.1002/pul2.70356
Gregg H Rawlings, Iain Armstrong, Andrew R Thompson, David G Kiely
{"title":"Getting the Wheels in Motion to Deal With Unmet Psychological Need: A Reply to \"The Pulmonary Hypertension Global Patient Survey: Physical and Psychosocial Impacts on Health-Related Quality of Life\".","authors":"Gregg H Rawlings, Iain Armstrong, Andrew R Thompson, David G Kiely","doi":"10.1002/pul2.70356","DOIUrl":"10.1002/pul2.70356","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70356"},"PeriodicalIF":2.4,"publicationDate":"2026-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13404774/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148606950","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}
Pulmonary CirculationPub Date : 2026-07-16eCollection Date: 2026-07-01DOI: 10.1002/pul2.70348
Athiththan Yogeswaran, Meike Fünderich, Katharina Wendlandt, Jeffrey S Annis, Evan Brittain, Christina A Eichstaedt, Ekkehard Grünig, Hector R Cajigas, Robert Frantz, Andrew J Sweatt, Roham T Zamanian, Friedrich Grimminger, Jochen Wilhelm, Hossein-Ardeschir Ghofrani, Khodr Tello, Werner Seeger
{"title":"Angiotensin Converting Enzyme Inhibition and Mortality in Pulmonary Hypertension Associated With Chronic Obstructive Pulmonary Disease (PH-COPD).","authors":"Athiththan Yogeswaran, Meike Fünderich, Katharina Wendlandt, Jeffrey S Annis, Evan Brittain, Christina A Eichstaedt, Ekkehard Grünig, Hector R Cajigas, Robert Frantz, Andrew J Sweatt, Roham T Zamanian, Friedrich Grimminger, Jochen Wilhelm, Hossein-Ardeschir Ghofrani, Khodr Tello, Werner Seeger","doi":"10.1002/pul2.70348","DOIUrl":"10.1002/pul2.70348","url":null,"abstract":"<p><p>In 567 PH-COPD patients from the PVRI GoDeep Meta-Registry, ACE inhibitor use was associated with improved survival only in severe PH (PVR > 5 WU), supporting prospective trials targeting this high-risk subgroup.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70348"},"PeriodicalIF":2.4,"publicationDate":"2026-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13404775/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148606816","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}
Pulmonary CirculationPub Date : 2026-07-15eCollection Date: 2026-07-01DOI: 10.1002/pul2.70361
Hannah K Robertson, Emma Yuguchi, Ian Gurholt, William C Nichols, Nick H Kim, Timothy Fernandes, Atul Malhotra, Mona Alotaibi
{"title":"Divergent Inflammatory and Bioenergetic Metabolomic Signatures Distinguish Methamphetamine-Associated From Idiopathic Pulmonary Arterial Hypertension.","authors":"Hannah K Robertson, Emma Yuguchi, Ian Gurholt, William C Nichols, Nick H Kim, Timothy Fernandes, Atul Malhotra, Mona Alotaibi","doi":"10.1002/pul2.70361","DOIUrl":"10.1002/pul2.70361","url":null,"abstract":"<p><p>Methamphetamine-associated pulmonary arterial hypertension (Meth-APAH) is an increasingly recognized cause of pulmonary arterial hypertension (PAH) and is associated with worse outcomes than idiopathic PAH (IPAH). Although both share histopathologic features of pulmonary vascular remodeling, the molecular basis for their clinical divergence remains unclear, particularly at the metabolic level. We performed high-throughput, non-targeted plasma metabolomic profiling in patients with Meth-APAH and matched IPAH controls enrolled from the Pulmonary Arterial Hypertension Biobank. Multivariate and regression analyses adjusted for demographics, hemodynamic severity, and PAH therapies were used to identify distinguishing metabolites. Among 98 patients (49 Meth-APAH and 49 IPAH), baseline hemodynamics and functional status were similar, although Meth-APAH patients had higher smoking prevalence and lower PAH therapy use. Of 757 metabolites passing quality control, 55 differed significantly between subtypes, including 22 enriched in Meth-APAH. Meth-APAH was characterized by elevations in amino acid-derived metabolites and pro-inflammatory eicosanoids, implicating dysregulation of the kynurenine pathway and arachidonic acid metabolism. IPAH demonstrated relative enrichment of branched-chain amino acids, antioxidant metabolites, and specialized pro-resolving mediators. Exploratory survival analyses identified associations between subtype-discriminating metabolites and outcomes. These findings reveal metabolic heterogeneity across PAH etiologies and support biologically distinct pathobiology in Meth-APAH and IPAH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70361"},"PeriodicalIF":2.4,"publicationDate":"2026-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13404770/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148606985","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}
Pulmonary CirculationPub Date : 2026-07-15eCollection Date: 2026-07-01DOI: 10.1002/pul2.70357
Sally E Prins, Jacob Sternbach, Jenna B Mendelson, Todd Markowski, LeeAnn Higgins, John P Carney, Walt Tollison, Gaurav Choudhary, Kurt W Prins
{"title":"Proteomics Defines Shared and Divergent Alterations in the Right Atrium and Right Ventricle in Porcine Right Heart Failure.","authors":"Sally E Prins, Jacob Sternbach, Jenna B Mendelson, Todd Markowski, LeeAnn Higgins, John P Carney, Walt Tollison, Gaurav Choudhary, Kurt W Prins","doi":"10.1002/pul2.70357","DOIUrl":"10.1002/pul2.70357","url":null,"abstract":"<p><p>Right heart failure is due to both right atrial and right ventricular dysfunction. While the two chambers are distinct, the proteomic response to pressure overload is undefined. Here, we used quantitative proteomics to evaluate changes in both chambers in pulmonary artery banded pigs. We found common alterations in oxidative phosphorylation, ribosome regulation, integrin-mediated adhesion, and glycolysis enzymes. However, the right atrium had more pronounced deficits in the TCA cycle and branched-chain amino acid metabolism. Thus, these data highlight metabolic pathways that could be evaluated to selectively enhance right atrial function.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70357"},"PeriodicalIF":2.4,"publicationDate":"2026-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13404772/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148607065","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}
Pulmonary CirculationPub Date : 2026-07-15eCollection Date: 2026-07-01DOI: 10.1002/pul2.70342
Sarah McGarrity, Pär I Johansson, Jørn Carlsen
{"title":"Metabolic Markers as Potential Risk Factors in Pulmonary Hypertension.","authors":"Sarah McGarrity, Pär I Johansson, Jørn Carlsen","doi":"10.1002/pul2.70342","DOIUrl":"10.1002/pul2.70342","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is a condition characterized by elevated mean pulmonary arterial pressure confirmed by right heart catheterization. PH involves both structural and functional changes in the pulmonary vascular bed. Since 2022, a four-level risk stratification has been used to predict 1-year mortality at follow-up. This system is based on the World Health Organization Functional Classification (WHO-FC), 6-min walk distance, and the pro-Brain Natriuretic Peptide level combined. This study included 47 patients with suspected PH. Of these, 40 were diagnosed with PH while 7 was not, and 6 were included as similarly presenting controls. Untargeted metabolomics using ultra-high pressure liquid chromatography mass spectrometry (UHPLC-MS), and gas chromatography mass spectrometry (GC-MS) showed correlations with both risk strata and their component measurements: N-terminal pro-brain natriuretic peptide (NT-proBNP), World Health Organization Functional Class (WHO-FC), and the 6-min walk distance (6MWD). There was a greater overlap in metabolites associated with WHO-FC or 6MWD than between either of them and NT-proBNP. Several of these metabolites showed the same pattern in a verification data set of 35 PH patients. In conclusion, metabolic patterns in pulmonary hypertension (PH) are related to the predicted risk of disease progression. The clinical variables that contribute to prognostic stratification have overlapping but distinct metabolic associations. Further research is needed to verify how these patterns relate to disease progression in a longitudinal cohort. Metabolomics provides a potential new tool for defining risk in PH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70342"},"PeriodicalIF":2.4,"publicationDate":"2026-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13404769/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148607026","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}