Pulmonary Circulation最新文献

{"title":"A Congestive Hemodynamic Footprint Is Associated With Worse Clinical Outcomes in Lung Transplant Recipients.","authors":"Mahmoud Kabbani, Varsha Karanam, Vivek Jani, Seamus Hughes, Tess Allan, Ashley Diaz, Arun Rajaratnam, Christina M Tilley, Aditi Sharma, Fatima Dollar, Evan Saenger, Catherine Teng, Mahnoor Liaqat, Shannon Scott, Rodolfo A Estrada, Holly Keyt, Allen S Anderson, M Imran Aslam","doi":"10.1002/pul2.70367","DOIUrl":"10.1002/pul2.70367","url":null,"abstract":"<p><p>Right heart catheterization (RHC) and echocardiography are integral in the assessment of pulmonary hypertension (PH) and cardiac function for lung transplant (LT) candidates. However, the impact of these metrics on post-LT outcomes remains unclear. We performed a single-center retrospective study of adult LT recipients from 2010 to 2022 to evaluate how RHC and echocardiographic variables relate to mortality, length of stay (LOS), duration of mechanical ventilation (MV), and rejection. An unsupervised cluster analysis was used to identify hemodynamic subgroups with adverse outcomes. Among 297 patients undergoing single or bilateral LT, most had interstitial lung disease (ILD; 52%), followed by chronic obstructive pulmonary disease (COPD; 32%) and other diagnoses (16%); mortality did not differ by indication (COPD 51%, ILD 37%, other 45%; <i>p</i> = 0.10), by PH defined as mean pulmonary artery pressure (mPAP) > 20 mmHg or pulmonary vascular resistance (PVR) ≥ 2 Woods Units (<i>p</i> = 0.45 for each), or by PH classification (isolated post-capillary, combined pre/post-capillary, or no PH; <i>p</i> = 0.77). However, in COPD, a higher mPAP, right ventricular (RV) filling pressures, and echocardiographic indices of diastolic function and RV pressure overload were associated with greater mortality. Across the cohort, PH was associated with longer LOS, more MV days, and higher rejection rates. Unsupervised cluster analysis identified a subgroup with elevated biventricular filling pressures and RV dysfunction consistent with a congestive hemodynamic footprint that had worse event-free post-LT survival (<i>p</i> = 0.049). These findings highlight the value of integrated invasive and noninvasive hemodynamics beyond the assessment of PH in LT recipients.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70367"},"PeriodicalIF":2.4,"publicationDate":"2026-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13494671/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148797436","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":"Transitions From Parenteral Prostacyclin Analogues to Selexipag in Patients With Pulmonary Arterial Hypertension.","authors":"Dalton J Kuebel, Adele R Collins, Arun Jose, Maria R Guido, Jean M Elwing","doi":"10.1002/pul2.70383","DOIUrl":"https://doi.org/10.1002/pul2.70383","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a progressive illness that may require therapy with parenteral prostacyclin pathway agents (PPA) (epoprostenol and treprostinil). These parenteral PPA's are continuous ambulatory infusions that require a high level of skill and knowledge to maintain safety and effectiveness. Depending on the clinical context, transition to oral prostacyclin receptor agonist therapy (selexipag) is sometimes pursued. To prevent abrupt withdrawal from parenteral PPA's, overlap transitions are typically used. This report describes 35 inpatient, rapid transitions from parenteral PPA's to oral selexipag. Planned hospital admissions for transition were common (<i>n</i> = 27, 77%), and all patients successfully transitioned without a return to parenteral PPA's in the immediate post-transition period. Most patients transitioned due to difficulties with current parenteral PPA therapy (<i>n</i> = 20, 57%), followed by an improved clinical status on parenteral PPA therapy (<i>n</i> = 8, 23%). The median length of transition was 36 h (IQR 24-48 h) and hospital stay was 3 days (IQR 2-6 days). Following transition, the cohort demonstrated stability in PAH-specific risk scores and functional status. All patients survived to outpatient pulmonary hypertension follow up (median 5.5 weeks, IQR 1.4-9.6 weeks), and the majority were alive at 1-year post-transition (<i>n</i> = 32, 91%). Based on these results, we conclude that transition from parenteral PPA's to selexipag can be done in the inpatient setting safely, quickly, and effectively.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70383"},"PeriodicalIF":2.4,"publicationDate":"2026-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13470875/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148765190","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":"Patterns and Trends in Vasoactive Medication Use in Patients With Pulmonary Arterial Hypertension Presenting With Shock.","authors":"Viviana Reyes Pinzon, Nicholas A Bosch, Elizabeth S Klings","doi":"10.1002/pul2.70382","DOIUrl":"10.1002/pul2.70382","url":null,"abstract":"<p><p>The optimal use of vasoactive medications in patients with pulmonary arterial hypertension with shock remains unknown. Using a national database, we benchmarked initial vasoactive practices. Twenty-five distinct strategies were identified, most commonly norepinephrine, vasopressin, phenylephrine, and milrinone. Marked practice variation underscores clinical equipoise and the need for comparative effectiveness studies.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70382"},"PeriodicalIF":2.4,"publicationDate":"2026-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13463747/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148727408","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":"The Heterogeneity of Long-Term Cardiopulmonary Outcomes in Children Born Preterm May Benefit from Pulmonary Hypertension-Specialty Outpatient Follow-Up.","authors":"Meaghan C Rousset, Rachel T Sullivan, Anna M Brown, Samantha R Eagle, Eric D Austin","doi":"10.1002/pul2.70380","DOIUrl":"10.1002/pul2.70380","url":null,"abstract":"<p><p>Preterm birth survivors reaching adulthood are a growing population, with bronchopulmonary dysplasia (BPD), the chronic lung disease of prematurity, common among this cohort. Infants with BPD are at increased risk for pulmonary vascular disease (PVD) including the development of pulmonary hypertension (PH). We present three preterm patients with a history of BPD-associated PH (BPD-PH) with monitoring throughout their childhood and young adulthood, highlighting the variation in management and late outcomes of each patient. Resolution of PH is reported on transthoracic echocardiogram (TTE) in all patients early in their life. Long-term follow-up was discontinued for one patient, while long-term monitoring in the other two individuals showed evidence of cardiopulmonary complications requiring PH-specialty management. The burden of cardiopulmonary disease associated with prematurity exists beyond early childhood and further research on the long-term management of this population is necessary to mitigate complications.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70380"},"PeriodicalIF":2.4,"publicationDate":"2026-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13449019/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148689866","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":"Right Ventricular to Left Ventricular (RV/LV) Blood Pool T2 Ratio Is Associated With Invasive Hemodynamics Across Pulmonary Hypertension Subgroups.","authors":"Ipek Buber, Paul Heerdt, Inderjit Singh, Cihan Ilyas Sevgican, Jérôme Lamy, Jie Xiang, Joseph Philip, Steffen Huber, Dana C Peters","doi":"10.1002/pul2.70381","DOIUrl":"10.1002/pul2.70381","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is defined by elevated mean pulmonary arterial pressure (mPAP). Pulmonary vascular resistance (PVR) and SvO₂ (mixed venous oxygenation), measured by right heart catheterization (RHC), are key determinants of disease severity and prognosis. The right-to-left ventricular (RV/LV) blood-pool T2 ratio (T2_RV/LV) by MRI has been proposed as a surrogate of RV-LV blood oxygenation differences and SvO₂. Therefore, we analyzed 31 consecutive patients with PH (precapillary = 13, postcapillary = 6, combined = 12) compared to data from four patients with no PH and 21 healthy subjects. All subjects underwent 1.5 T MRI with cine for RV/LV and atrial volumes and T2 mapping. Blood T2_RV/LV was measured on mid-ventricular short-axis images. Hemodynamic data (including mPAP, PVR, pulmonary arterial wedge pressure (PAWP) and oxygen saturations) were obtained from recent (within 1.5 months) RHC. We found that the T2_RV/LV was significantly lower in PH patients than in healthy controls (0.76 ± 0.09 vs. 0.91 ± 0.05; <i>p</i> < 0.001) and correlated with pulmonary artery oxygen saturations (SvO₂) (<i>r</i> = 0.77; <i>p</i> < 0.001). It also correlated with PVR (<i>ρ</i> = -0.74; <i>p</i> < 0.001), mPAP (<i>r</i> = -0.54; <i>p</i> < 0.001), RVEF (<i>r </i>= -0.61; <i>p</i> < 0.001). In multivariable analysis, T2_RV/LV independently predicted mPAP (<i>p</i> < 0.001; <i>R</i> ²= 0.64 with LA volume index and RVEF) and PVR (<i>p</i> < 0.001; <i>R</i> ²= 0.63 with RVEF). Inter- and intra-observer variability for T2_RV/LV was excellent (<i>R</i> = 0.96-0.97). In conclusion, blood T2_RV/LV independently predicts invasive hemodynamic indices and is a non-invasive biomarker of SvO₂, which might enable noninvasive risk stratification and treatment monitoring in PH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70381"},"PeriodicalIF":2.4,"publicationDate":"2026-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13449024/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148689845","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":"The Causal Role of Gut Microbiota in Pulmonary Embolism and the Mediating Effects of Circulating Metabolites: A Mendelian Randomization Study.","authors":"Yidan Gao, Xuehan Jiang, Yanshuang Lyu, Huiyuan Hu, Shuang Wang, Yufei Hu, Hong Zhang, Zhenguo Zhai, Wanlu Song, Peiran Yang","doi":"10.1002/pul2.70376","DOIUrl":"10.1002/pul2.70376","url":null,"abstract":"<p><p>The gut microbiota has been implicated in pulmonary vascular diseases, yet a direct causal link to pulmonary embolism (PE) remains unestablished. This study aimed to investigate the causal effects of gut microbiota on PE and to explore the mediating role of circulating metabolites in this relationship. We performed a two-sample Mendelian randomization (MR) analysis using summary statistics from large-scale genome-wide association studies. The gut microbiota data were sourced from the MiBioGen consortium, PE data from the FinnGen consortium (R10), and metabolite data from a meta-analysis of 136,016 participants. The inverse variance weighting method was used as the primary analytical approach, supplemented by sensitivity analyzes to ensure robustness. At the nominal level (<i>p</i> < 0.05), 12 gut microbial taxa showed suggestive associations with PE. Among these, genera LachnospiraceaeUCG010 and Defluviitaleaceae showed protective effects, while Subdoligranulum, Lentisphaerae, and Erysipelatoclostridium were associated with increased risk. In contrast, 26 circulating metabolites exhibited robust causal associations with PE, with several surviving Bonferroni correction. Mediation analyzes further revealed that specific metabolites, including histidine, triglycerides in small HDL, and docosahexaenoic acid ratio, significantly mediated the suggestive effects of nominal microbial taxa on PE risk, identifying potential metabolic pathways in the gut-vascular axis. This study generates hypothesis-generating evidence for the gut-pulmonary vascular axis, with identified metabolic mediators warranting further experimental validation. The potential effects of gut microbiota on PE provide exploratory leads for future mechanistic studies. These findings highlight the gut-pulmonary vascular axis as a potential target for novel preventive and therapeutic strategies against PE.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70376"},"PeriodicalIF":2.4,"publicationDate":"2026-08-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13430573/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148670585","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":"Survival Into Adulthood With FOXF1-Associated Pulmonary Vascular Disease After Neonatal Onset.","authors":"Khalifah A Aldawsari, Steven H Abman, David Badesch, David Dunbar Ivy","doi":"10.1002/pul2.70378","DOIUrl":"10.1002/pul2.70378","url":null,"abstract":"<p><p>Neonates and infants with FOXF1 mutation develop alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV), which is typically considered a uniformly fatal neonatal lung disorder due to marked hypoxemic respiratory failure with severe pulmonary hypertension (PH). Survival beyond infancy is exceedingly rare, with limited reports of FOXF1 genetic abnormalities in older children or adult cases of severe PH. Here, we report the case of a male patient with a pathogenic FOXF1 variant who presented with severe PH in early infancy whose disease stabilized and improved with early and aggressive PH therapy. The patient subsequently died at the age of 30 years, however, due to severe right ventricular failure due to the acute onset of pneumonia. This case highlights that survival into adulthood is feasible in patients with FOXF1-related pulmonary vascular disease and supports the inclusion of FOXF1 in pulmonary hypertension genetic panels for subjects who develop PH across the lifespan.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70378"},"PeriodicalIF":2.4,"publicationDate":"2026-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13425634/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148654261","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":"The Role of Medical Therapy Before and After Balloon Pulmonary Angioplasty: CTEPH AC Registry Across Japan.","authors":"Miki Sakamoto-Iwata, Yu Taniguchi, Kohei Masaki, Kazuya Hosokawa, Shiro Adachi, Takumi Inami, Jun Yamashita, Hitoshi Ogino, Masaru Hatano, Ichizo Tsujino, Nobuhiro Yaoita, Nobutaka Ikeda, Hiroto Shimokawahara, Nobuhiro Tanabe, Kayoko Kubota, Ayako Shigeta, Yoshito Ogihara, Koshin Horimoto, Yoshihiro Dohi, Takahiro Hiraide, Takashi Kawakami, Yuichi Tamura, Yoshihiro Fukumoto, Kohtaro Abe","doi":"10.1002/pul2.70377","DOIUrl":"10.1002/pul2.70377","url":null,"abstract":"<p><p>We aimed to evaluate the role of medical therapy before and after balloon pulmonary angioplasty (BPA) for non-operable chronic thromboembolic pulmonary hypertension (CTEPH) in the modern management era. This Japanese nationwide, multicenter, prospective observational registry, including 37 centers, analyzed data newly registered from August 2018-December 2023. Pretreatment before BPA was analyzed in patients who were treatment naïve or received medical therapy only at registration (<i>n</i> = 557). De-escalation or escalation of medical therapy after BPA was analyzed in patients who underwent BPA (<i>n</i> = 966). The BPA pretreatment analysis in 557 patients showed that approximately 70% of the patients received medical therapy, of which 18% received combination therapy. Patients who received monotherapy or combination therapy had more severe baseline hemodynamics than those who did not. The 3-year survival did not significantly differ between the no, mono, and combination pretreatment groups (94%, 98%, and 97%, respectively; <i>p</i> = 0.852). The BPA post-treatment analysis in 966 patients showed that 20% of patients underwent de-escalation of medical therapy. Patients with de-escalation of medical therapy had lower mean pulmonary artery pressure (PAP) (20.9 ± 5.8 vs. 24.1 ± 7.3 mmHg, <i>p</i> < 0.01); however, they had a lower cardiac index at last follow-up compared to patients without de-escalation (2.66 ± 0.61 vs. 2.84 ± 0.70 L/min/m², <i>p</i> = 0.01). Patients with severe disease tended to undergo pre-treatment before BPA; however, patient survival did not differ across pretreatment types. De-escalation of medical therapy after BPA can affect cardiac index. Therefore, de-escalation or continuation of medical therapy should be carefully considered even after mean PAP normalizes after BPA.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70377"},"PeriodicalIF":2.4,"publicationDate":"2026-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13425626/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148654231","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 Practice of Pulmonary Arterial Hypertension (PAH) and Potential Barriers to Intensive Treatment Among PAH-Specialized and Non-PAH-Specialized Centers in Japan.","authors":"Yoko Arai, Wen Zhang, Shohei Yamauchi, Shigeru Tokita, Shunta Akutsu","doi":"10.1002/pul2.70351","DOIUrl":"10.1002/pul2.70351","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a rare and progressive disease characterized by remodeling of the pulmonary vasculature, which leads to increased pulmonary artery pressure and ultimately right ventricular failure. Although Japanese guidelines recommend combination therapy, real-world data show inconsistencies in its implementation. We thus conducted a cross-sectional, web-based questionnaire survey to understand the recent real-world clinical practices in Japan and identify factors associated with treatment decisions, with a particular focus on the use of combination therapies. The findings indicated that oral PAH medications were widely available and commonly prescribed, whereas inhaled and parenteral infusion therapy were less accessible and used less frequently. Moreover, physicians working in PAH-specialized centers were significantly more likely to prescribe parenteral infusion therapies (adjusted odds ratio [aOR] = 2.6, 95% confidence interval [CI]: 1.5-4.3, <i>p</i> < 0.001) and adopt dual (aOR = 3.0, 95% CI: 1.4-6.5, <i>p</i> = 0.01) or triple combination regimens (aOR = 2.7, 95% CI: 1.5-2.7, <i>p</i> < 0.001). Physicians with experience treating high risk patients at PAH-specialized centers were also significantly more likely to prescribe parenteral infusion therapies (aOR = 3.5, 95% CI: 1.8-6.6, <i>p</i> < 0.001) and adopt dual (aOR = 5.9, 95% CI: 2.5-14.2, <i>p</i> < 0.001) or triple combination regimens (aOR = 3.4, 95% CI: 1.7-6.7, <i>p</i> < 0.001). This study revealed differences in clinical practice between PAH-specialized and non-specialized centers in real-world settings. Management of PAH at PAH-specialized centers was associated with increased treatment intensification, indicating the need for enhanced collaboration between PAH-specialized and non-specialized centers to ensure optimal care for patients with PAH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70351"},"PeriodicalIF":2.4,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13410739/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148621305","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":"Noninvasive Gas Exchange Determinants of Exercise Hemodynamics in Non-Severe Pulmonary Hypertension Associated With Interstitial Lung Disease.","authors":"Chebly Dagher, Niala Moallem, Garrett Fiscus, Brett Carollo, Kristen Swanson, Harrison W Farber, Raj Parikh","doi":"10.1002/pul2.70358","DOIUrl":"10.1002/pul2.70358","url":null,"abstract":"<p><p>Level 3 cardiopulmonary exercise testing (CPET) can unmask the dynamic burden of pulmonary vascular disease (PVD), while submaximal exercise testing offers a simpler, noninvasive alternative. Emerging markers such as a gas-exchange derived estimate of pulmonary vascular capacitance (GXCAP) and ventilatory efficiency (VE/VCO₂) may reflect PVD and correlate with invasive hemodynamics. Level 3 CPET has been used to stratify PH-ILD patients into those with exercise-induced increases in pulmonary vascular resistance (PVR_D) and those with static PVR (PVR_S). This study evaluated whether GXCAP and VE/VCO₂ measured during submaximal testing could identify non-severe PH-ILD patients with exercise-induced increases in PVR. GXCAP and VE/VCO₂ were retrospectively analyzed in 16 non-severe PH-ILD patients who underwent both submaximal exercise testing and invasive Level 3 CPET. Patients were previously categorized as having dynamic (PVR_D) or stable (PVR_S) PVR responses. Between-group differences were assessed using unpaired t-tests, and receiver operating characteristic (ROC) analysis evaluated the ability of each variable to identify the PVR_D phenotype. Among the 16 patients included in the analysis, 6 were classified as PVR_S and 10 as PVR_D. Baseline characteristics, including peak VO₂, were similar between groups. Mean GXCAP was significantly higher in PVR_S (471 ± 85 mL·mmHg) than in PVR_D (311 ± 38 mL·mmHg, <i>p</i> < 0.01), while VE/VCO₂ was lower in PVR_S (33 ± 2 vs 37 ± 2.5, <i>p</i> < 0.01). ROC analysis demonstrated excellent discriminatory performance for GXCAP (AUC 0.98) and good discrimination for VE/VCO₂ (AUC 0.90). The GXCAP/VE/VCO₂ ratio performed similarly (AUC 0.98). These findings suggest that GXCAP and VE/VCO₂ obtained during submaximal testing may serve as practical, noninvasive tools for identifying exercise-induced pulmonary vascular impairment and aiding risk stratification in patients with non-severe PH-ILD. However, validation in larger prospective cohorts is needed.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70358"},"PeriodicalIF":2.4,"publicationDate":"2026-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13404787/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148607103","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}