Pulmonary CirculationPub Date : 2026-09-15eCollection Date: 2026-07-01DOI: 10.1002/pul2.70411
Miller Giraldo-Sandoval, Fernando Andrés Guerrero-Pinedo, Ángela María Herrera-Peña, Juan David Agrado-Torres, Julián Mauricio Cortés-Colorado, Frank Cañón-Estrada, Carlos Andrés Mejía-Gómez, María Alejandra Londoño-Osorio, Juan Andrés Muñoz-Ordoñez, Valeria Azcarate-Rodríguez, Mario Miguel Barbosa-Rengifo, Brayan Daniel Cordoba-Melo, María Elena Ochoa, Diego Bautista-Rincón, Liliana Fernandez-Trujillo, William Martínez, Fernando Sanabria, Diana Cristina Carrillo-Gómez, Juan David Lopez-Ponce-de-Leon, Julian David Yara-Muñoz, Noel Florez, Paula Andrea Cárdenas-Marín, Edilma Lucy Rivera, Claudia Marcela Rojas-Camejo, Irene Lang, Juan Esteban Gómez-Mesa
{"title":"Clinical Outcomes of Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Hypertension: A Retrospective Cohort Study From a Latin American Care Center.","authors":"Miller Giraldo-Sandoval, Fernando Andrés Guerrero-Pinedo, Ángela María Herrera-Peña, Juan David Agrado-Torres, Julián Mauricio Cortés-Colorado, Frank Cañón-Estrada, Carlos Andrés Mejía-Gómez, María Alejandra Londoño-Osorio, Juan Andrés Muñoz-Ordoñez, Valeria Azcarate-Rodríguez, Mario Miguel Barbosa-Rengifo, Brayan Daniel Cordoba-Melo, María Elena Ochoa, Diego Bautista-Rincón, Liliana Fernandez-Trujillo, William Martínez, Fernando Sanabria, Diana Cristina Carrillo-Gómez, Juan David Lopez-Ponce-de-Leon, Julian David Yara-Muñoz, Noel Florez, Paula Andrea Cárdenas-Marín, Edilma Lucy Rivera, Claudia Marcela Rojas-Camejo, Irene Lang, Juan Esteban Gómez-Mesa","doi":"10.1002/pul2.70411","DOIUrl":"10.1002/pul2.70411","url":null,"abstract":"<p><p>Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct subtype of pulmonary hypertension arising from persistent major pulmonary artery obstruction after pulmonary embolism. Pulmonary endarterectomy (PEA) remains the standard of care, but up to 40% of patients in European and US registries are inoperable; balloon pulmonary angioplasty (BPA) has emerged as a more accessible interventional modality for suitable patients. This retrospective single-center cohort study evaluated functional improvement, hemodynamic response, and mortality in patients with CTEPH ineligible for PEA who underwent BPA at a Colombian institution (May 2022-October 2025). Risk was stratified using COMPERA 2.0. The primary outcome was improvement of at least one New York Heart Association (NYHA) functional class; secondary outcomes were change in mean pulmonary arterial pressure (mPAP) and all-cause mortality during follow-up. Of 33 patients (median age 63 years; 70% female), 5 (15.1%) were high risk at baseline, 16 (48.4%) intermediate-high, 10 (30.3%) intermediate-low, and 2 (6%) low risk. After BPA, 19 patients (58%) improved NYHA class (95% CI, 39.2%-74.5%), and mPAP fell from 60 (IQR 50-68) to 40 mmHg (IQR 37-48) after a median of three sessions (IQR 2-4) (mean paired reduction 14.05 mmHg, 95% credible interval 9.81-18.07; Hedges' g = 1.22, 95% CI 0.77-1.66). Two deaths (6.0%) occurred, both in high-risk patients. BPA improved NYHA class and pulmonary hemodynamics in severe, inoperable CTEPH, with a low complication rate despite advanced baseline disease. COMPERA 2.0 stratified baseline severity but did not predict the magnitude of hemodynamic response to BPA, consistent with the mechanical nature of the disease.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70411"},"PeriodicalIF":2.4,"publicationDate":"2026-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13575860/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148976754","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 : 2026-09-11eCollection Date: 2026-07-01DOI: 10.1002/pul2.70392
Alexandra Bekiaridou, Ibrahim Mughrabi, Nicolas Rios, George Giannakoulas, Betty Diamond, Stavros Zanos
{"title":"Splenic Focused Ultrasound Improves Experimental Pulmonary Hypertension and Reprograms Immune Responses in Female and Male Rats.","authors":"Alexandra Bekiaridou, Ibrahim Mughrabi, Nicolas Rios, George Giannakoulas, Betty Diamond, Stavros Zanos","doi":"10.1002/pul2.70392","DOIUrl":"10.1002/pul2.70392","url":null,"abstract":"<p><p>Splenic focused ultrasound (sFUS) neuromodulation improves monocrotaline-induced pulmonary hypertension in rats of both sexes, with females achieving near-normalization of right ventricular systolic pressure and males showing partial improvement. In male and female animals, sFUS reduces pulmonary CD8+ T cells and CD68+ macrophages, and reduces splenic monocytes. Splenic single-cell transcriptomic analysis in the SU5416/hypoxia model revealed sex-specific immune responses, including persistent interferon signaling in males.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70392"},"PeriodicalIF":2.4,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13566670/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148956405","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 : 2026-09-11eCollection Date: 2026-07-01DOI: 10.1002/pul2.70397
Ji Zhao, Jiajun Guo, Shichu Liang, Yangjie Li, Juan He, Chen Chen, Xuhan Liu, Lidan Yin, Shoufang Pu, Bi Wen, Yuanwei Xu, Ke Wan, Yuchi Han, Yucheng Chen
{"title":"Prognostic Value of Right Atrial Adverse Remodeling in Patients With Pulmonary Arterial Hypertension.","authors":"Ji Zhao, Jiajun Guo, Shichu Liang, Yangjie Li, Juan He, Chen Chen, Xuhan Liu, Lidan Yin, Shoufang Pu, Bi Wen, Yuanwei Xu, Ke Wan, Yuchi Han, Yucheng Chen","doi":"10.1002/pul2.70397","DOIUrl":"10.1002/pul2.70397","url":null,"abstract":"<p><p>Assessment of right atrial (RA) structure helps monitor right heart (RH) function, critical for prognosis in pulmonary arterial hypertension (PAH). This study aims to assess the prognostic value of RA adverse remodeling (RAAR) with targeted therapy in PAH. Patients diagnosed with PAH between June 2013 and June 2024 were prospectively recruited. Right atrial reverse remodeling (RAAR) was defined by the relative change in right atrial diameter (RAD) on 1-year follow-up echocardiography (ECHO). RAAR was defined as a RAD decrease of < 13% or any increase from baseline, as determined by receiver operating characteristic curve analysis. The primary composite endpoint included heart failure-related readmission, all-cause mortality, and lung transplantation. Of 240 patients enrolled, 234 completed 1-year follow-up, with a median follow-up duration of 37.5 months (interquartile range: 23.3-57.5 months). RAAR was observed in 168 patients (71.8%), and baseline RAD was its sole independent predictor (OR 0.941, <i>p</i> = 0.012). RAAR was associated with significantly poorer event-free survival in both the low-risk group (log-rank <i>p</i> = 0.037) and the intermediate-/high-risk group (log-rank <i>p</i> < 0.001). Adding RAAR improved the ESC model (χ<sup>2</sup>: 18.08-21.83) and the three-strata COMPERA model (χ<sup>2</sup>: 22.65-28.04). These findings suggest that RAAR is a key indicator for evaluating prognosis in PAH patients, holding promise for reflecting treatment response and predicting clinical outcomes in PAH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70397"},"PeriodicalIF":2.4,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13567802/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148956427","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 : 2026-09-11eCollection Date: 2026-07-01DOI: 10.1002/pul2.70408
Michelle M Hall, Seppo T Rinne, A Rani Elwy, Renda Soylemez Wiener, Kari R Gillmeyer
{"title":"Quality of Pulmonary Hypertension Care Within Expert PH Centers: Patient and Provider Perspectives From a Multi-Site Qualitative Study.","authors":"Michelle M Hall, Seppo T Rinne, A Rani Elwy, Renda Soylemez Wiener, Kari R Gillmeyer","doi":"10.1002/pul2.70408","DOIUrl":"10.1002/pul2.70408","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is a progressive disease associated with high morbidity and mortality. Despite advancements, gaps in PH care quality persist, though the drivers of these gaps are incompletely understood. Thus, we sought to explore patient and provider perspectives on high-quality PH care. We conducted a multi-site qualitative study at three expert PH centers in the United States, involving telephone interviews with 20 providers and 22 patients. We conducted a reflexive thematic analysis, using the Institute of Medicine's (IOM) six domains of healthcare quality (safe, effective, patient-centered, timely, efficient, and equitable) as an a priori coding scheme, with subcodes developed under each domain. We then compared codes across IOM domains and patient and provider data to identify overarching themes. Our analysis revealed three cross-cutting themes that encompassed all six IOM healthcare quality domains, covered all identified subcodes, and represented both provider and patient perspectives: (1) \"It takes a village,\" that is, the expansive team needed to provide care for a patient with PH; (2) The need for care coordination within a PH center, with specialty pharmacies, and with community providers; and (3) PH centers as a model to deliver high-quality care. Finally, participants offered clear recommendations for addressing gaps in care quality, including expanding village members, such as the addition of social workers, improving care coordination by improving patient follow-up and tracking, and expanding access to PH centers by having a PH center presence in the community. These findings highlight key areas for targeted improvements in PH care quality.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70408"},"PeriodicalIF":2.4,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13567799/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148956432","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 : 2026-09-11eCollection Date: 2026-07-01DOI: 10.1002/pul2.70372
Rongrong Chen, Shadi P Bagherzadeh, Neev Patel, Patrick Khoury, Bettia E Celestin, Frida N Hermansson, Jennifer P Woo, Francois Haddad
{"title":"Validation of Tricuspid Annular Displacement by Tissue Doppler in Pulmonary Hypertension.","authors":"Rongrong Chen, Shadi P Bagherzadeh, Neev Patel, Patrick Khoury, Bettia E Celestin, Frida N Hermansson, Jennifer P Woo, Francois Haddad","doi":"10.1002/pul2.70372","DOIUrl":"10.1002/pul2.70372","url":null,"abstract":"<p><p>Tricuspid annular plane systolic excursion (TAPSE) is primarily measured using motion-mode (M-mode) echocardiography. However, image resolution doesn't always provide clear visualization. In these cases, tissue Doppler imaging (TDI) may offer a valuable alternative for right ventricular assessment. To validate TDI-derived TAPSE (TAPSE-TDI) against M-mode TAPSE and compare their diagnostic utility for right heart catheterization-confirmed pulmonary hypertension(PH). We analyzed 304 healthy volunteers and 310 PH patients in a case-control study. M-mode TAPSE and TAPSE-TDI were measured in the RV-focused apical four-chamber view. TAPSE-TDI was calculated by averaging the systolic and diastolic signals from the lateral tricuspid annulus. Agreement between methods was quantified using bias, percentile precision, and intra-class coefficient (ICC). The dependence of annular motion on body size, sex, and heart rate was evaluated with weighted regression. We assessed PH discrimination using receiver operating curve (ROC) analysis, comparing TAPSE, TAPSE-TDI, and RV TDI peak systolic velocity (s'). Thresholds were further tested using Classification and Regression Tree (CART) analysis. TAPSE-TDI demonstrated a strong linear relationship with TAPSE (<i>r</i> = 0.945, <i>p</i> < 0.001) and a slope of 1.02 in no-intercept regression. TAPSE-TDI demonstrated excellent reproducibility (ICC = 0.937), and its reference range fell within the guideline-recommended values. For PH diagnosis, TAPSE and TAPSE-TDI showed good discrimination (AUC > 0.83), with the TAPSE/RVSP ratio outperforming (both AUC > 0.96). CART analysis identified optimal thresholds of 16.68 mm for TAPSE and 15.88 mm for TAPSE-TDI to discriminate PH. TAPSE measured using TDI was closely related to M-mode and both better discriminated PH than RV s'.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70372"},"PeriodicalIF":2.4,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13567800/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148956475","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 : 2026-09-10eCollection Date: 2026-07-01DOI: 10.1002/pul2.70400
Julia K Munchel, Bess M Flashner, Ryosuke Imai, Cyrus A Kholdani, Rene S Bermea, Anjali Singh, Robert W Hallowell, Mary B Rice, Andrew J Synn
{"title":"Beyond IPF: Pulmonary Function Test Predictors of Echocardiographic Pulmonary Hypertension Across the Spectrum of ILD.","authors":"Julia K Munchel, Bess M Flashner, Ryosuke Imai, Cyrus A Kholdani, Rene S Bermea, Anjali Singh, Robert W Hallowell, Mary B Rice, Andrew J Synn","doi":"10.1002/pul2.70400","DOIUrl":"10.1002/pul2.70400","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is a common complication of interstitial lung disease (ILD), yet data on screening in heterogenous ILD populations are limited. We evaluated whether routinely measured pulmonary function test (PFT) parameters identify individuals with intermediate-to-high echocardiographic risk of PH. 432 ILD patients met inclusion criteria with an echocardiogram reporting peak tricuspid regurgitant velocity (TRV) and a PFT reporting diffusion impairment (DLCO) within 1 year (median interval, 39 days). Of these, 126 underwent right heart catheterization (RHC). Age- and sex- adjusted logistic regression assessed associations between %predicted forced vital capacity (FVC), %predicted DLCO, and FVC/DLCO ratio with intermediate-to-high echocardiographic PH probability (TRV > 2.8 m/s). Receiver operating characteristic curves evaluated diagnostic performance. The cohort included 28% systemic autoimmune rheumatic disease-associated ILD, 27% interstitial pneumonia with autoimmune features, and 6% idiopathic pulmonary fibrosis, with a mean age of 72% and 54% female. Lower FVC and DLCO were associated with higher odds of echocardiographic PH (OR 1.35, 95%CI 1.21-1.50; OR 1.50, 95%CI 1.36-1.73) and increases in FVC/DLCO ratio similarly predicted risk (OR 1.83, 95%CI 1.44-2.32). DLCO had the highest predictive ability (AUC 0.74, 95%CI 0.69-0.79) with an optimal cut-off of 42% (sensitivity 67%, specificity 74%). In patients who underwent RHC, TRV > 2.8 m/s correlated with mean pulmonary artery pressure (r = 0.55, 95%CI 0.42-0.66) and tripled the odds of confirmed PH (OR 3.75, 95%CI 2.25-6.24). In sum, severe diffusion impairment, regardless of fibrosis extent, may detect patients with ILD who warrant echocardiographic screening, specialty PH referral, and consideration of RHC.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70400"},"PeriodicalIF":2.4,"publicationDate":"2026-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13560502/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148945706","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 : 2026-09-10eCollection Date: 2026-07-01DOI: 10.1002/pul2.70399
Nelson Villasmil Hernandez, Maria Jose Maestre, Meagan Chavarria, Pujita Munnangi, Neelam Lakhani, Laura Valecillos Loukidis, Sandeep Sahay
{"title":"Referral Patterns and Clinical Characteristics of Patients With Pulmonary Hypertension at a Comprehensive Care Center.","authors":"Nelson Villasmil Hernandez, Maria Jose Maestre, Meagan Chavarria, Pujita Munnangi, Neelam Lakhani, Laura Valecillos Loukidis, Sandeep Sahay","doi":"10.1002/pul2.70399","DOIUrl":"10.1002/pul2.70399","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are life-threatening forms of pulmonary hypertension (PH) that require early recognition and referral to expert centers. In 2014, the Pulmonary Hypertension Association (PHA) launched the Pulmonary Hypertension Care Centers (PHCC) initiative to improve access to specialized evaluation and guideline-directed therapy. A decade later, real-world data evaluating referral patterns and time to diagnosis remain limited. We conducted a retrospective analysis of patients referred to Houston Methodist PHCC between January 2022 and December 2023 for suspected or confirmed PH. Of 274 referred patients, 97 were confirmed to have PAH or CTEPH and included in this study. Demographics, referral sources, presenting symptoms, diagnostic and treatment timelines, and risk stratification by REVEAL Lite 2 score were analyzed. At the time of referral, 66% patients lacked a defined group classification, 18% were labeled as PAH, and 13% as CTEPH. Following a comprehensive evaluation, 76 (28%) were confirmed as PAH and 21 (8%) as CTEPH, whereas 14% were determined not to have PH. At presentation, 58% of patients were in WHO FC III or IV, and 63% of patients with PAH were categorized as intermediate or high risk. Median time from symptom onset to diagnosis was 13 months (IQR: 4-29), to treatment 16 months (IQR: 6-35), and to PHCC referral 24 months (IQR: 10-54). 30% of patients with PAH were not receiving PH-specific therapy, and 35% were on suboptimal regimens based on their risk status. Following PHCC evaluation, dual and triple therapy use increased to 50% and 36%. Despite the establishment of the PHCC network, diagnostic delays and therapeutic gaps persist, emphasizing the need for enhanced awareness, standardized referral pathways, and stronger collaboration between community providers and PHCCs.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70399"},"PeriodicalIF":2.4,"publicationDate":"2026-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13560503/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148930732","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 : 2026-09-10eCollection Date: 2026-07-01DOI: 10.1002/pul2.70409
Lucilla Piccari, Katarina Zeder, Aparna Balasubramanian, Brian W Allwood, Stephen John Wort, Shelsey W Johnson, Reda E Girgis, Oksana A Shlobin, Steven H Abman, Gabor Kovacs, George Washko, Sylvia M Nikkho, Steven D Nathan
{"title":"Pathogenesis and Phenotypes of Pulmonary Vascular Disease in COPD: A Consensus Statement From the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative - PH Group 3 Workstream.","authors":"Lucilla Piccari, Katarina Zeder, Aparna Balasubramanian, Brian W Allwood, Stephen John Wort, Shelsey W Johnson, Reda E Girgis, Oksana A Shlobin, Steven H Abman, Gabor Kovacs, George Washko, Sylvia M Nikkho, Steven D Nathan","doi":"10.1002/pul2.70409","DOIUrl":"10.1002/pul2.70409","url":null,"abstract":"<p><p>Chronic obstructive pulmonary disease (COPD) is frequently associated with pulmonary vascular disease (PVD) yet pulmonary vascular involvement remains underrecognized and incompletely phenotyped. Emerging evidence demonstrates that pulmonary vasculopathy may precede overt emphysema and airflow obstruction, suggesting that vascular remodeling is an early and integral component of COPD pathobiology rather than solely a late consequence of advanced lung disease. Multiple mechanisms contribute to COPD-associated PVD, including genetic susceptibility, abnormal lung development, cigarette smoke and environmental exposures, endothelial dysfunction, hypoxia, inflammation, hyperinflation, and comorbid cardiovascular and infectious diseases, which are discussed in this paper. These processes lead to heterogeneous clinical phenotypes that span a continuum from early pulmonary vascular abnormalities without resting PH to severe hemodynamically defined PH. Conventional spirometry and resting hemodynamics incompletely capture this complexity, whereas advanced imaging, gas-exchange assessment, and exercise testing improve identification of pulmonary vascular involvement. Distinct pulmonary vascular phenotypes may carry important implications for exercise limitation, exacerbation risk, progression, and survival. Recognizing COPD-associated PVD as a continuum rather than a late-stage complication has major implications for screening, phenotyping, and clinical trial design. This article reflects deliberations of the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative (PVRI IDDI) Group 3 Pulmonary Hypertension Workstream on the pathophysiology and phenotypes of PVD in COPD.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70409"},"PeriodicalIF":2.4,"publicationDate":"2026-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13560002/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148945665","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":"Invasive Management for Acute-on-Chronic Thromboembolic Pulmonary Hypertension: Feasibility and Clinical Outcomes in a Single-Center Cohort.","authors":"Jun Yamashita, Ryosuke Ito, Sayo Ikeda, Koki Hasegawa, Yoshikazu Takeuchi, Tomohiro Kasahara, Naotaka Murata, Shun Suzuki, Hideaki Kanazawa, Yusuke Shimahara, Kazuhiro Satomi","doi":"10.1002/pul2.70407","DOIUrl":"10.1002/pul2.70407","url":null,"abstract":"<p><p>Acute-on-chronic thromboembolic pulmonary hypertension (acute-on-CTEPH) is associated with rapid clinical deterioration, yet the role of invasive intervention in the acute phase remains poorly defined. We retrospectively analyzed consecutive patients with acute-on-CTEPH presenting with severe hypoxemia and/or cardiogenic shock at a single center between 2023 and 2026. Acute-on-CTEPH was defined as acute clinical deterioration requiring ventilatory or circulatory support, including ECMO, with imaging evidence of chronic thromboembolic disease and superimposed acute thrombi. Invasive strategies included pulmonary endarterectomy (PEA) and/or balloon pulmonary angioplasty (BPA), selected based on multidisciplinary assessment. The primary outcome was change in pulmonary hemodynamics; secondary outcomes included acute-phase mortality and clinical recovery after stabilization. A total of 15 patients (median age 69 years; 53% male), all in World Health Organization functional class IV, were included. Mechanical ventilation and ECMO were required in 53% and 47% of patients, respectively. Initial treatment consisted of PEA (40%) or BPA (60%), with combined approaches in selected cases. Acute-phase mortality was 20%. Among survivors, mean pulmonary artery pressure decreased from 42 to 17 mmHg, pulmonary vascular resistance from 11.6 to 2.4 WU, and cardiac index increased from 1.7 to 2.7 L/min/m<sup>2</sup>. Among patients requiring ECMO, 5 were successfully weaned and 4 survived to discharge. At follow-up, 11 of the 12 survivors were in WHO functional class I or II, and 8 (67%) had discontinued home oxygen therapy. Despite substantial acute-phase mortality, invasive strategies were associated with marked hemodynamic improvement and may represent a potential therapeutic option in this life-threatening clinical setting.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70407"},"PeriodicalIF":2.4,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13551590/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148913378","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 : 2026-09-08eCollection Date: 2026-07-01DOI: 10.1002/pul2.70406
Pietro Nardelli, Rubén San José Estépar, Michael Cuttica, Ruben Mylvaganam, Farbod N Rahaghi, Raúl San José Estépar
{"title":"Automated CT Pulmonary Angiography-Derived Periclot Radiomics for CTEPH Detection.","authors":"Pietro Nardelli, Rubén San José Estépar, Michael Cuttica, Ruben Mylvaganam, Farbod N Rahaghi, Raúl San José Estépar","doi":"10.1002/pul2.70406","DOIUrl":"10.1002/pul2.70406","url":null,"abstract":"<p><p>CTEPH remains underdiagnosed and can be difficult to differentiate on routine CTPA from acute PE and from patients without pulmonary thromboembolism. We developed and evaluated a fully automated CTPA-based machine learning algorithm combining vascular blood volume metrics with clot and periclot imaging features. In this retrospective multicenter study, intraparenchymal pulmonary vessels were segmented using a scale-space particle approach, deep learning models estimated vessel radius, clot probability, and clot area from cross-sectional patches along the vessel axis, and subject-level radiomic and blood volume features were used to train an XGBoost classifier on a Northwestern University (NU) cohort (89 CTEPH; 176 controls). The trained model was then applied (without retraining) to held-out NU data, an independent acute PE cohort (152 patients) as an exploratory generalization test, and an external cohort from Brigham and Women's Hospital (23 CTEPH; 34 controls). Among 474 participants (mean age, 51 years ± 16; 299 men), the model achieved an AUC of 0.87 (95% CI: 0.79, 0.94) for CTEPH versus controls in internal testing, 0.74 (95% CI: 0.61-0.86) in external testing, and 0.89 (95% CI 0.83-0.93) for CTEPH versus acute PE. Excluding periclot features reduced discrimination, particularly for CTEPH versus acute PE (AUC 0.71; 95% CI: 0.63, 0.79). These findings support the feasibility of automated CTPA-derived vascular, clot, and periclot features for differentiating CTEPH from acute PE and controls without evidence of pulmonary thromboembolism, with further validation needed to assess generalizability across institutions.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70406"},"PeriodicalIF":2.4,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13551595/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148913316","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}