Pulmonary Circulation[JOURNAL]
Sun
200 papers
The Prognostic Accuracy of Three-Dimensional Echocardiography in Patients With Pre-Capillary Pulmonary Hypertension.
Liu BY, Yan WJ, Chen Y … +11 more , Zhang HW, Yang BL, Zeng QX, Liang YR, Niu LL, Tian Y, Zhao Q, Luo Q, Zhao ZH, Wu WC, Xiong CM
Pulm Circ · 2026 Jan · PMID 41766755 · Full text
To investigate the prognostic accuracy of three-dimensional echocardiographic (3DE) right ventricular (RV) data and compare it with that of risk stratification based on 2015 ESC Guidelines in pre-capillary pulmonary hype...
To investigate the prognostic accuracy of three-dimensional echocardiographic (3DE) right ventricular (RV) data and compare it with that of risk stratification based on 2015 ESC Guidelines in pre-capillary pulmonary hypertension (PcPH) patients. We prospectively enrolled PcPH patients from March 2017 to May 2018. 3DE sequences were analyzed by semi-automatic software (TomTec 4D RV-Function 2.0). RV end-diastolic volume (EDV), end-systolic volume (ESV), ejection fraction, longitudinal strain of septum and free wall, tricuspid annular plane systolic excursion were obtained. All participants were classified into low and intermediate-high risk groups based on 2015 ESC Guidelines. Patients were followed-up till May 2019 for death due to RV failure as an end-point. We finally enrolled 112 PcPH patients (average 36 years, 39 males and 73 females) in our study. Mean follow-up time was 18 months, and 11 patients died. Receive operating characteristic curves identified RV-3D-EDV = 150 mL and RV-3D-ESV = 109 mL as optimal cut-offs. Multivariate Cox proportional regression analyses indicated RV-3D-EDV > 150 mL and RV-3D-ESV > 109 mL were independent predictors of mortality after adjusted by Risk stratification. McNemar-Bowker test revealed that compared with risk stratification, RV-3D-EDV > 150 mL (67.3% vs. 44.6%, < 0.01) and RV-3D-ESV > 109 mL (62.4% vs. 44.6%, < 0.01) had better predictive specificities for end-point. RV volumes detected by three-dimensional echocardiography suggested potential prognostic value for risk stratification in PH patients, warranting validation in larger cohorts.
Efficacy and Safety of Low-Dose Prolonged Infusion Thrombolysis With rt-PA for Acute Intermediate-High Risk Pulmonary Embolism.
Qi W, Gong M, Huang H … +2 more , Zhou Y, Su H
Pulm Circ · 2026 Jan · PMID 41756747 · Full text
Thrombolytic therapy alleviates pulmonary embolism (PE) symptoms rapidly but increases bleeding risk, with no consistent consensus on acute intermediate-high risk PE. This study evaluated the efficacy and safety of low-d...
Thrombolytic therapy alleviates pulmonary embolism (PE) symptoms rapidly but increases bleeding risk, with no consistent consensus on acute intermediate-high risk PE. This study evaluated the efficacy and safety of low-dose prolonged infusion thrombolysis for acute intermediate-high risk PE to provide a safer clinical option. A total of 120 patients were collected and divided into anticoagulant (Group A, = 58) and thrombolytic (Group B, = 62) groups. Efficacy outcomes included pulmonary artery thrombus clearance rate, 30-day all-cause mortality, and 3-6 month pulmonary hypertension incidence; the primary safety outcome was treatment-related bleeding. Both groups showed improved SBP, DBP, SpO₂, and RV/LV ratio (all < 0.05) with no inter-group differences, but Group B had higher thrombus clearance rate ([64.85 ± 17.47]% vs. [41.65 ± 16.19]%, < 0.001), with similar 30-day mortality (1.61% vs. 5.17%, = 0.278). At 1 day, post-treatment, Group B had higher D-dimer (26.78 ± 16.57 μg/mL vs. 7.60 ± 7.23 μg/mL) and FDP (91.45 ± 97.37 μg/mL vs. 18.60 ± 26.34 μg/mL, all < 0.001) but comparable FIB ( = 0.091); these differences persisted until discharge (all < 0.05 for D-dimer/FDP). Although the incidence of bleeding events in group B was numerically higher than that in group A (17.74% vs. 6.90%, = 0.073), the difference between the two groups was not statistically significant. No fatal bleeding, intracranial hemorrhage, or recurrent pulmonary embolism occurred in either group. Group B had lower 3-6 month pulmonary artery systolic pressure (PASP) (30.70 ± 9.70 vs. 34.44 ± 10.04 mmHg, = 0.045) and pulmonary hypertension incidence (27.87% vs. 54.55%, = 0.004). Thrombus clearance rate correlated with treatment group ( = 0.57, < 0.001), and D-dimer ( = 0.42) and FDP ( = 0.32) levels at 1 day post-treatment (both < 0.001). Low-dose prolonged infusion can effectively clear pulmonary artery thrombi in patients with acute intermediate-high-risk PE, which may be associated with a reduced incidence of pulmonary hypertension. Patients in the thrombolysis group showed significant dynamic changes in D-dimer and FDP levels, which were significantly correlated with a higher thrombus clearance rate.
Contrast Reflux Into IVC in Acute Pulmonary Embolism Predicts Clinical Deterioration Risk.
Leverone NA, Singer AG, Lopez AA … +14 more , Ellberg C, Henry PJ, Kabadi AA, Self AA, Leverone DM, Yen A, Hahn L, Karunamuni J, Weihe E, Yang JZ, McGuire WC, Papamatheakis DG, Morris TA, Fernandes TM
Pulm Circ · 2026 Jan · PMID 41756746 · Full text
Reflux of contrast medium into the inferior vena cava (IVC) on computed tomography pulmonary angiogram (CTPA) is an independent risk factor for mortality in patients with acute pulmonary embolism (PE). This study's aim w...
Reflux of contrast medium into the inferior vena cava (IVC) on computed tomography pulmonary angiogram (CTPA) is an independent risk factor for mortality in patients with acute pulmonary embolism (PE). This study's aim was to determine if reflux into the IVC correlates with objective scores of clinical risk in acute PE. Patient-level data were collected for adults diagnosed with acute PE by CTPA at University of California, San Diego Health between January 1 and June 30, 2023. Contrast reflux into the IVC was graded on a 4-point scale. The primary endpoint was the correlation between reflux severity and risk for clinical deterioration as measured by the National Early Warning Score (NEWS). The secondary endpoint was interobserver reliability grading reflux amongst 16 reviewers. Fifty-six subjects with acute PE were included. Extent of contrast reflux into the IVC correlated with NEWS ( 0.2932; = 0.028). Subjects with grade 3 reflux had increased NEWS (7.80) compared to those with reflux grades 0 (3.63; = 0.014) or 1 (4.29; = 0.05). NEWS was elevated in those with contrast below the diaphragm compared to those without when grading was pooled ( = 0.036). Interobserver reliability for reflux grading was "moderate" ( = 0.58), yet increased to "almost perfect" ( = 0.87) for pooled grading. Contrast reflux into the IVC during acute PE correlates with risk for clinical deterioration, as reflected by NEWS. Simplification of grading to contrast above or below the diaphragm improves reliability without compromising this relationship.
Supraventricular Arrhythmias in Pulmonary Hypertension: A Comprehensive Review From Preclinical Evidence to Clinical Practice.
Kiris A, Zhang P, Bronk P … +3 more , Prins KW, Choi BR, Choudhary G
Pulm Circ · 2026 Jan · PMID 41743696 · Full text
Pulmonary Hypertension (PH) is a chronic disease that causes significant structural deterioration in the right atrium and ventricle. In the past 1-2 decades, life expectancy in patients with PH has increased due to impro...
Pulmonary Hypertension (PH) is a chronic disease that causes significant structural deterioration in the right atrium and ventricle. In the past 1-2 decades, life expectancy in patients with PH has increased due to improved awareness, diagnosis and advancements in treatment. Increasingly, PH is recognized in older patients with comorbidities. With the changing demographics, supraventricular arrhythmias (SVAs), especially atrial fibrillation and flutter, are emerging as important outcomes in PH. Here, we comprehensively summarize findings from preclinical and clinical studies to define the mechanistic drivers and clinical implications of SVA. In sum, we propose early recognition and targeted management of SVA is important to improve quality of life, morbidity and mortality in patients with PH.
Pulmonary Venous Vasculopathy in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension With Reduced Diffusion Capacity for Carbon Monoxide.
Fujisaki S, Asano R, Endo H … +8 more , Kuraishi A, Takano R, Hayashi H, Ohta-Ogo K, Hatakeyama K, Tsujita K, Noguchi T, Ogo T
Pulm Circ · 2026 Jan · PMID 41727705 · Full text
Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) has a poorer prognosis than idiopathic PAH, potentially attributable to pulmonary veno-occlusive disease (PVOD)-like involvement. The diffusi...
Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) has a poorer prognosis than idiopathic PAH, potentially attributable to pulmonary veno-occlusive disease (PVOD)-like involvement. The diffusing capacity for carbon monoxide (DLco) may reflect small pulmonary venous obliteration. Whether severely reduced DLco reflects the severity of venous remodelling in CTD-PAH remains incompletely characterised, particularly with quantitative morphometric assessment. Thus, we evaluated the association of reduced DLco with clinical characteristics and venous vasculopathy in patients with CTD-PAH. We retrospectively reviewed patients with CTD-PAH without interstitial pneumonia. Patients were categorised into two groups based on %DLco (< 45%: severely reduced DLco or ≥ 45%: non-severely reduced DLco). Clinical characteristics and survival rates were compared. Histopathological characteristics of the pulmonary vasculature were analysed in eight autopsy cases. Of the 86 patients (mean age, 49.2 ± 16.8 years), 25 (29.1%) were classified into the severely reduced DLco group. They were older, showed a higher prevalence of systemic sclerosis, and exhibited lower arterial oxygen saturation and mean pulmonary arterial pressure compared with the other group. The severely reduced DLco group had a worse survival in unadjusted analysis ( = 0.048). Histopathological analysis revealed that the severity of small pulmonary venous stenosis was inversely correlated with %DLco ( = 0.537, = 0.039; 95% CI, -0.69 to -0.03). Patients with CTD-PAH and a severely reduced DLco demonstrated a poorer prognosis than those without. Reduced DLco was associated with the severity of small pulmonary venous stenosis, consistent with a PVOD-like venous remodelling spectrum in CTD-PAH. DLco may serve as a clinically accessible marker of venous involvement within the PVOD-like spectrum of CTD-PAH.
Plasma GDF-15 and PSP-D Predict the Development of Pulmonary Arterial Hypertension in Systemic Sclerosis.
Engel Sällberg A, Ahmed S, Ahmed A … +5 more , Kania K, Carlsen J, Hesselstrand R, Andréasson K, Rådegran G
Pulm Circ · 2026 Jan · PMID 41727704 · Full text
Patients with pulmonary arterial hypertension (PAH) experience long diagnostic delays, high functional class at diagnosis and poor prognosis. We aimed to study the differentiative and predictive value of 90 inflammatory...
Patients with pulmonary arterial hypertension (PAH) experience long diagnostic delays, high functional class at diagnosis and poor prognosis. We aimed to study the differentiative and predictive value of 90 inflammatory and immunomodulatory related proteins in idiopathic and hereditary PAH (IPAH/HPAH) and systemic sclerosis-associated PAH (SSc-APAH). Cohort 1 comprised patients with SSc-APAH ( = 36), IPAH/HPAH ( = 54) and healthy controls ( = 55). Cohort 2 comprised SSc patients without PAH ( = 15) and SSc-APAH ( = 15), with blood samples both ~6 years before, and at PAH diagnosis. This cohort was used for internal validation and to predict future development of PAH in SSc. Cohort 3 comprised connective tissue disease (CTD) APAH ( = 19) and IPAH ( = 20), and was used for external validation. Plasma protein levels were measured with proximity extension assay. In cohort 1, we found that higher IL-27 differentiated PAH patients from controls (odds ratio (OR) = 1.24; area under the curve (AUC) = 0.94), whereas higher TNFRSF4 differentiated SSc-APAH from IPAH/HPAH (OR = 1.14; AUC = 0.82), and controls (OR = 1.30; AUC = 0.99). In cohort 2, GDF-15 and PSP-D were higher in female SSc patients that would develop vs those that wouldn't develop PAH, and predicted PAH-development ~6 years before diagnosis (OR = 1.24; AUC = 0.78 and 1.22; 0.73, respectively). Using equivalence testing, levels of IL-27, GDF-15 and PSP-D were equivalent ( = 0.0072, = 0.0048 and = 0.00076) in cohort 3 and 1. In conclusion, GDF-15 and PSP-D emerged as promising potential biomarkers in early screening and prediction of future PAH development in SSc, whereas IL-27 and TNFRSF4 appeared promising in diagnosis and subtype differentiation of PAH and SSc-APAH.
Right Ventricular Pressure-Volume Loop Analysis in Large Animal Research: A Scoping Review of Current Practices Using Conductance and Admittance Catheters.
Orlitová M, Hellinck J, Dam Lyhne M … +4 more , Granfeldt A, Neyrinck AP, Verbelen T, Claus P
Pulm Circ · 2026 Jan · PMID 41716885 · Full text
Right ventricular (RV) function assessment using catheter-derived pressure-volume (PV) loops is used in translational research, providing detailed insights into cardiac mechanics. Its practical implementation requires me...
Right ventricular (RV) function assessment using catheter-derived pressure-volume (PV) loops is used in translational research, providing detailed insights into cardiac mechanics. Its practical implementation requires methodological accuracy to ensure rigor, reproducibility and transparency. However, current practices for PV loop acquisition are set at individual laboratories, resulting in overall practice remaining largely unknown. This study aimed to explore current practices in RV PV loop acquisition in large animal research. We therefore conducted a scoping review registered on the Open Science Framework and guided by PRISMA Extension for Scoping Reviews, with comprehensive searches in PubMed, Embase, Scopus, and Web of Science (December 2024). Eligible studies included large animal models published after 2014 using conductance or admittance catheters. Out of 5133 identified records, 62 met inclusion criteria. The review revealed underreporting and substantial variability in methodology, particularly in catheter placement, calibration, and ventilatory mode during PV loop recording. Catheter insertion sites included RV inflow (42%) and outflow tracts (19.4%); fluoroscopy was used in 51.6%. Calibration was not reported in one-third of studies. Loop recording during apnea were reported in 23%, mechanical ventilation in 26%, and was not specified in 51%. Overall, RV PV loop protocols show substantial heterogeneity, highlighting the need for standardized methodological reporting, to improve transparency, research reproducibility and translational significance. Expert consensus is warranted to establish methodological guidelines.
Performance of Guideline-Recommended Approaches to Echocardiographic Investigation for Pulmonary Hypertension: Analysis of the CIPHER Study.
Howard LS, Kiely DG, Lawrie A … +10 more , Maron BA, Preston IR, Rosenkranz S, Toshner M, Wilkins MR, Fong YL, Quinn D, Stamatiadis D, Villeneuve M, Chin KM
Pulm Circ · 2026 Jan · PMID 41710256 · Full text
Guidelines recommend different approaches to investigate for pulmonary hypertension (PH) by transthoracic echocardiography (TTE). We used data from the CIPHER study (NCT04193046) to prospectively evaluate TTE detection o...
Guidelines recommend different approaches to investigate for pulmonary hypertension (PH) by transthoracic echocardiography (TTE). We used data from the CIPHER study (NCT04193046) to prospectively evaluate TTE detection of PH. Participants newly referred to PH clinics who underwent right heart catheterization (RHC) within 6 weeks and TTE within 60 days of enrolment (blinded central TTE reading) were classified by TTE probability of PH applying (i) the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) TTE algorithm or (ii) right ventricular systolic pressure (RVSP) > 40 mmHg. For calculation of sensitivity and specificity, 'non-assessable' patients (peak tricuspid regurgitation velocity [TRV] missing or ≤ 2.8 m/s with missing data on other echocardiographic signs) and patients with missing RVSP were counted as PH-negative. Performance was measured against RHC-confirmed diagnosis of mean pulmonary artery pressure > 20 mmHg. Of 475 patients included, 345 (73%) had PH. Using the ESC/ERS algorithm, PH probability was high, intermediate, low and non-assessable for 198, 104, 22 and 151 patients and PH prevalence was 98%, 75%, 23%, and 44%, respectively. Seventy-three patients were missing RVSP and 292 had RVSP > 40 mmHg. The two TTE approaches achieved similar results: sensitivity was 79%-77%, specificity was 78%-79%. This prospective study of patients newly referred to PH centres for RHC found similar sensitivity and specificity when using either RVSP > 40 mmHg or the 2015 ESC/ERS TTE algorithm. Among patients who were low-probability or non-assessable by ESC/ERS algorithm, 42% had PH, highlighting the persistent need for additional non-invasive investigative tools.
Association of Immunoglobulin E With Right Ventricular Dysfunction in Pulmonary Arterial Hypertension.
Wu F, Chen X, Chen K … +3 more , Wei J, Gong C, Wu W
Pulm Circ · 2026 Jan · PMID 41695276 · Full text
Pulmonary arterial hypertension (PAH) is characterized by pulmonary vasoconstriction and vascular remodeling, leading to increased pulmonary vascular resistance, pathological right ventricular (RV) remodeling, and ultima...
Pulmonary arterial hypertension (PAH) is characterized by pulmonary vasoconstriction and vascular remodeling, leading to increased pulmonary vascular resistance, pathological right ventricular (RV) remodeling, and ultimately right heart failure and death. RV function and right ventricle-pulmonary artery (RV-PA) coupling are critical determinants of patient prognosis, and echocardiography remains the preferred tool for assessing RV function and predicting RV-PA coupling. Recent studies have revealed that immunoglobulin E(IgE) plays a regulatory role in cardiac and pulmonary vascular remodeling; however, its role in PAH-related RV dysfunction remains unclear. This study aimed to investigate the association between serum IgE levels and RV dysfunction in patients with PAH through echocardiography, serological testing, and right heart catheterization (RHC), to provide potential evidence for novel diagnostic and therapeutic strategies in PAH. A total of 30 patients diagnosed with idiopathic pulmonary arterial hypertension (IPAH) at the First Affiliated Hospital of Chinese Guangxi Medical University between 2024 and 2025 were prospectively enrolled, along with an equal number of age- and sex-matched individuals with normal cardiac function and no cardiopulmonary disease as controls. Clinical baseline data and serum samples were collected from all participants, and serum IgE concentrations were determined using enzyme-linked immunosorbent assay (ELISA). For IPAH patients, serum N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, RHC parameters, and World Health Organization (WHO) functional class were obtained from the electronic medical record system. Transthoracic echocardiography (TTE) was performed to assess RV morphology, structural characteristics, and functional alterations. Statistical analysis was performed using -tests, Mann-Whitney -tests, one-way ANOVA, correlation, and regression analysis to evaluate the differences in IgE levels between groups and the relationship with RV function parameters. The results showed that in the IPAH group, 13 patients (43.3%) were male, with a mean age of 41.27 ± 11.50 years, whereas the control group included 14 males (46.7%) with a mean age of 41.35 ± 10.78 years. Serum IgE levels were significantly higher in IPAH patients compared with controls (859 [702, 1031] vs. 430 [359, 568], ng/mL, < 0.0001). Correlation analyses based on electronic medical record data demonstrated that serum IgE levels in IPAH patients were positively associated with NT-proBNP concentrations ( = 0.79, < 0.0001). Moreover, patients in WHO functional class III with right heart failure exhibited significantly higher serum IgE levels than those in functional class I, and IgE levels showed a positive correlation with WHO functional class. Serum IgE levels were also positively correlated with RV pressure and PA pressure (IgE and mPAP: = 0.37, 95% CI: 0.01-0.64, = 0.04; IgE and mRVP: = 0.43, CI: 0.08-0.68, = 0.01). Echocardiographic analyses further revealed that serum IgE levels were positively correlated with RV hypertrophy and dilation, while being inversely correlated with RV contractile function and TAPSE/sPAP ( = -0.72 [-0.86, -0.49], < 0.0001). Comparisons across groups indicated that serum IgE levels were significantly elevated in IPAH patients whose RV remodeling had progressed to the maladaptive phase compared with those in the adaptive phase (984 [834, 1110] vs. 684 [612, 861], ng/mL, < 0.0001). This study demonstrated that serum IgE levels were significantly elevated in patients with PAH and positively correlated with the severity of RV dysfunction. Notably, the marked increase in IgE levels predominantly occurred during the maladaptive phase of RV remodeling after RV-PA uncoupling. These findings suggest that IgE-mediated immune mechanisms may contribute to the progression of right heart failure in PAH. Serum IgE holds potential as a diagnostic biomarker for PAH-related right heart failure, and anti-IgE therapy may represent a promising therapeutic strategy that warrants further investigation.
Impact of Right Heart Catheterization and the 2022 ESC/ERS Definition of Pulmonary Hypertension in Patients With Mitral Regurgitation Undergoing Valve Repair/Replacement.
Güpfert M, Weber L, Haager PK … +7 more , Baier P, Kopp S, Rigger J, Chronis J, Gerhard M, Rickli H, Maeder MT
Pulm Circ · 2026 Jan · PMID 41695275 · Full text
In patients with mitral regurgitation (MR), the role of invasive hemodynamics is not well defined. We evaluated the value of right heart catheterization (RHC) and the 2022 ESC/ERS pulmonary hypertension (PH) definition p...
In patients with mitral regurgitation (MR), the role of invasive hemodynamics is not well defined. We evaluated the value of right heart catheterization (RHC) and the 2022 ESC/ERS pulmonary hypertension (PH) definition prior to a mitral valve procedure for the prediction of PH several months thereafter. We studied 274 patients (mean age 68 ± 11 years) with at least moderate MR (84% degenerative, 11% functional, 5% combined) undergoing RHC (baseline) followed by surgical (70%) or transcatheter (30%) valve repair/replacement who had an echocardiogram after a median follow-up of 3 months. PH was defined as a mean pulmonary artery pressure (mPAP) > 20 mmHg and was subclassified by mean pulmonary artery wedge pressure (mPAWP) and pulmonary vascular resistance (PVR). The PH follow-up probability was assessed by peak tricuspid regurgitant velocity and indirect PH signs. At baseline, 143/274 (52%) patients had any PH: 40 had isolated post-capillary, 65 had combined pre- and post-capillary (CpcPH), 27 had pre-capillary, and 11 had unclassified PH. Follow-up PH probability was low in 149, intermediate in 73, or high in 42 patients. There was a progressive increase in baseline mPAP, mPAWP, and PVR ( < 0.001 for all) in patients with low, intermediate or high follow-up PH probability. Patients with baseline CpcPH had a nearly 11-fold higher risk (odds ratio 10.7) for a high follow-up PH probability compared to those without PH. In conclusion, in MR patients the hemodynamic constellation according to the 2022 ESC/ERS PH definition predicts the PH probability several months after a mitral valve procedure.
Echocardiographic Assessment After Pulmonary Thromboendarterectomy for Chronic Thromboembolic Pulmonary Hypertension: Which Parameters Reflect Improved Right Ventricular Function?
Tager D, Collier P, Akintoye EO … +7 more , Wang Y, Wang X, Goyanes A, Elgharably H, Tong M, Mehta A, Heresi GA
Pulm Circ · 2026 Jan · PMID 41674543 · Full text
Traditional echocardiographic measures of right ventricular (RV) function, such as tricuspid annular plane systolic excursion (TAPSE) and peak systolic velocity at the tricuspid annulus (S'), may be unreliable after card...
Traditional echocardiographic measures of right ventricular (RV) function, such as tricuspid annular plane systolic excursion (TAPSE) and peak systolic velocity at the tricuspid annulus (S'), may be unreliable after cardiac surgery due to changes in loading conditions and myocardial mechanics. This study aimed to evaluate RV function following pulmonary thromboendarterectomy (PTE) for chronic thromboembolic pulmonary hypertension (CTEPH) and to identify alternative echocardiographic indices that better reflect postoperative RV recovery. This retrospective, single-center study included CTEPH patients who underwent PTE at Cleveland Clinic between January 2020 and March 2021. Echocardiographic evaluations were performed preoperatively and within 3 months postoperatively. Parameters assessed included RV size, function, volumes, pulmonary pressures, and tricuspid regurgitation (TR) severity. TAPSE and RV S' significantly decreased postoperatively (mean changes -0.75 cm and -2.8 cm/s, respectively; < 0.001). RV systolic pressure, end-diastolic volume (RVEDV), and end-systolic volume (RVESV) also declined ( < 0.05). RV diameter decreased (mean 4.57 cm; = 0.012), and 2D RV ejection fraction (RVEF) increased significantly (mean change +7%; = 0.003). TR severity improved, with 97.1% of patients showing only mild TR ( = 0.003). Fractional area change (FAC) did not change significantly. After PTE, RV size, volume, RVEF, and TR severity are more reliable indicators of RV recovery than TAPSE or S'. These findings reflect reverse RV remodeling due to reduced afterload and underscore the limitations of traditional longitudinal RV function metrics in the postoperative setting.
LIN28B Aggravates the Inflammation in Sepsis-Induced Lung Injury by Stabilizing GATA6 mRNA and Upregulating TRAF6.
Chen L, Chen J, Wang Z … +5 more , Jiang X, Cao J, Yang H, Wen X, Li J
Pulm Circ · 2026 Jan · PMID 41674542 · Full text
Sepsis affects more than 19 million people globally each year, and approximately 50% of sepsis patients develop lung injury. Sepsis-triggered lung injury is characterized by over-activation of inflammatory response and t...
Sepsis affects more than 19 million people globally each year, and approximately 50% of sepsis patients develop lung injury. Sepsis-triggered lung injury is characterized by over-activation of inflammatory response and the production of large amounts of inflammatory cytokines. However, the underlying mechanisms remain unclear. A cellular model of sepsis-induced lung injury was constructed using lipopolysaccharide (LPS)-exposed human pulmonary microvascular endothelial cells (HPMECs). Cell viability was checked with CCK-8. Apoptosis was checked utilizing flow cytometry and TUNEL staining. TNF-α, IL-1β, and IL-6 levels were measured through ELISA assay. Intermolecular interaction relationships were confirmed by chromatin immunoprecipitation (ChIP), RNA immunoprecipitation (RIP), RNA pull down, and dual luciferase reporter assays. LIN28B was increased in the LPS-induced HPMEC. LIN28B knockdown alleviated inflammation and cell injury in LPS-induced HPMEC. LIN28B was bound to GATA6 mRNA and enhanced its mRNA stability, consequently exacerbating LPS-triggered HPMEC inflammation and injury. GATA6 transcriptionally upregulated TRAF6 to promote LPS-induced HPMEC inflammation and injury. The manipulation of the GATA6/TRAF6 axis by LIN28B amplified inflammation and injury in HPMEC prompted by LPS. Our results revealed that LIN28B induced sepsis-triggered lung injury by stabilizing GATA6 mRNA and increasing TRAF6 expression, which might provide novel therapeutic targets for the treatment of sepsis-mediated lung injury.
A Case of IgG4-Related Disease Developing During Long-Term High-Dose Intravenous Epoprostenol Therapy in a Patient With Idiopathic Pulmonary Arterial Hypertension.
Inao T, Nakayama K, Yasui S … +7 more , Shite N, Shiraki E, Ohbayashi C, Otsuka K, Onishi H, Ohta S, Iwahashi M
Pulm Circ · 2026 Jan · PMID 41658488 · Full text
Long-term intravenous (IV) epoprostenol is a key therapy for pulmonary arterial hypertension (PAH); however, rare immune-mediated conditions occurring during therapy remain incompletely characterised. We report a 34-year...
Long-term intravenous (IV) epoprostenol is a key therapy for pulmonary arterial hypertension (PAH); however, rare immune-mediated conditions occurring during therapy remain incompletely characterised. We report a 34-year-old man with idiopathic PAH who developed low-grade fever and bilateral pulmonary infiltrates after 11 years of IV epoprostenol. Despite empirical antibiotics, his condition persisted, and a lip biopsy confirmed IgG4-related disease (IgG4-RD). Systemic corticosteroids were initiated, and the patient was transitioned from IV epoprostenol to a combination of IV and inhaled treprostinil; IgG4-RD has remained in remission to date, with normalisation of serum IgG4. This case highlights a practical, minimally invasive diagnostic approach for suspected IgG4-RD in severe PAH and demonstrates the feasibility of prostacyclin modification to sustain PAH control while reducing immunosuppression.
Impact of Preoperative Hemodynamic Values on Development of Reperfusion Edema After Pulmonary Endarterectomy.
Torregroza C, Roth S, Meermann K … +10 more , M'Pembele R, Tsimpoura P, Kuschka D, Lafioniatis N, Ganceva N, Adameit MSD, Mayer E, Wiedenroth CB, Huhn R, Guth S
Pulm Circ · 2026 Jan · PMID 41640630 · Full text
Reperfusion pulmonary edema (RPE) is a severe complication after pulmonary endarterectomy (PEA) and is associated with prolonged mechanical ventilation, organ dysfunction, and worse outcome. While residual pulmonary hype...
Reperfusion pulmonary edema (RPE) is a severe complication after pulmonary endarterectomy (PEA) and is associated with prolonged mechanical ventilation, organ dysfunction, and worse outcome. While residual pulmonary hypertension after PEA is associated with RPE, studies on preoperative risk factors for RPE after PEA are scarce. We investigated a potential association between preoperative hemodynamic values and development of RPE after PEA surgery. All adult CTEPH-patients who underwent PEA surgery at the Kerckhoff-Clinic Bad Nauheim, Germany, between 2018 and 2021 were included. The primary endpoint was development of postoperative RPE. Preoperative hemodynamic values and patient characteristics were compared between patients with and without RPE. Receiver Operating Characteristic curve analysis and logistic regression models were used for identification of risk factors for RPE. A total of 483 patients were included in this analysis (mean age 61 ± 13 years, 57% male). 75 patients developed postoperative RPE. ROC analysis revealed a significant discrimination for RPE by preoperative mean pulmonary artery pressure (mPAP) [AUC = 0.7, 95% CI: 0.622-0.779]. According to the Youden Index, the cut-off for preoperative mPAP was 54.5 mmHg. Multivariable logistic regression identified preoperative mPAP [OR: 2.684 95% CI: 1.220-5.904, = 0.014] as independent risk factor for development of RPE after PEA surgery. Our study determines preoperative mPAP value as an independent risk factor for development of RPE after PEA surgery. These results might help to identify patients with an increased risk for RPE and adapt perioperative therapy accordingly.
Prospective Evaluation of Serial Biomarkers in Patients With Intermediate High Risk Acute Pulmonary Embolism: A Single Center Proof-of-Concept Study.
Subba H, McKenna A, Gilboy J … +5 more , Gelman J, Evans J, Smith A, Kerney J, Wirth JA
Pulm Circ · 2026 Jan · PMID 41640629 · Full text
Patients diagnosed with intermediate high-risk pulmonary embolism (IHRPE) are at significant risk for clinical deterioration during hospitalization; however, clinical tools to identify which patients will worsen are impr...
Patients diagnosed with intermediate high-risk pulmonary embolism (IHRPE) are at significant risk for clinical deterioration during hospitalization; however, clinical tools to identify which patients will worsen are imprecise. We designed a proof-of-concept, single-center prospective study to assess IHRPE patients (using 2019 ESC criteria), measuring blood concentrations of N-terminal pro-B-type natriuretic peptide (NT-proBNP), troponin T (TnT), uric acid, and plasma lactate serially during the first 72 h to better understand their kinetics and associations with in-hospital adverse clinical events. Twenty subjects (mean age 62.0 ± 12.6 years) diagnosed by computed tomography angiogram were enrolled. Central pulmonary embolism was seen in 18/20, and lower extremity deep vein thrombosis in 14/20. On presentation, the mean Bova and PESI scores were 5 ± 0.7 and 105 ± 25.2, respectively. At baseline, TnT was elevated in 20/20, NT-proBNP in 18/20, uric acid in 10/20, and lactate in 8/20 subjects. Clinical outcomes included ICU admission in 7/20, clinical deterioration in 10/20, and death in 2/20. Clinical deterioration was associated with persistent elevations of TnT, NT-proBNP, uric acid and lactate (all < 0.05). The NT-proBNP time from baseline to peak concentration was highly associated with clinical deterioration (ROC AUC = 0.82 [95% CI: 0.62-0.97, < 0.01, RR = 2.8 at 24 h). The baseline PESI score ROC AUC for clinical deterioration was 0.75 (95% CI: 0.515-0.952, = NS). Persistently elevated biomarkers show an association with in-hospital adverse clinical events in IHRPE and warrant further study to assist clinical management.
Transmembrane Protein 100 Expression on Endothelial Cells Vascularizing Thrombi in Chronic Thromboembolic Pulmonary Hypertension Modulates TGFβ1-ALK1 Signaling During Angiogenesis.
Bochenek ML, Ghasemi I, Wiedenroth CB … +8 more , Bikou O, Karampinis I, Roessner ED, Hobohm L, Guth S, Lurz P, Konstantinides S, Schäfer K
Pulm Circ · 2026 Jan · PMID 41625184 · Full text
Endothelial cells within chronic pulmonary artery thrombi in CTEPH overexpress transmembrane protein 100 (TMEM100), an activin A receptor-like kinase 1 (ACVRL1 or ALK1) signaling-dependent gene, and TGFβ1 upregulated TME...
Endothelial cells within chronic pulmonary artery thrombi in CTEPH overexpress transmembrane protein 100 (TMEM100), an activin A receptor-like kinase 1 (ACVRL1 or ALK1) signaling-dependent gene, and TGFβ1 upregulated TMEM100 transcription in healthy lung ECs. TMEM100 permitted the TGFβ1-induced increase of ALK1, while repressing ALK5, and preventing ALK1-TMEM100 signaling impaired angiogenesis ex vivo. Our data indicate that TGFβ1-ALK1-TMEM100 signaling is active during CTEPH thrombus revascularization.
Sotatercept in Pulmonary Langerhans Cell Histiocytosis-Associated Pulmonary Hypertension: A Case Series and Systematic Review.
Chilingarashvili G, Mylvaganam RJ, Bernardo RJ … +5 more , Shah A, Cuttica M, Roberts T, Marchetti N, Rali P
Pulm Circ · 2026 Jan · PMID 41613828 · Full text
Pulmonary Langerhans cell histiocytosis (PLCH) frequently complicated by pulmonary hypertension (PH), which markedly worsens prognosis. We retrospectively reviewed three institutional PLCH-PH cases treated with off-label...
Pulmonary Langerhans cell histiocytosis (PLCH) frequently complicated by pulmonary hypertension (PH), which markedly worsens prognosis. We retrospectively reviewed three institutional PLCH-PH cases treated with off-label Sotatercept added to background triple therapy and performed a systematic review of published PLCH-PH reports (PubMed/Embase through May 2025). Our three patients (ages 69, 62, 49; all female) had progressive vascular disease despite optimized vasodilator therapy. Following Sotatercept, all experienced ≥ 3-fold increases in 6-min walk distance and improved functional class. Hemodynamics improved substantially: right-atrial pressure -78.6%, pulmonary vascular resistance -75.5%, pulmonary artery systolic pressure -58.5%, mean PAP - 56.0%, PA diastolic pressure -51.0%, PAWP - 47.1%; cardiac output and index rose +75.9% and +73.8%, respectively. BNP/NT-proBNP normalized. Systematic review identified 34 published cases (2010-2025): mean age 37.2 ± 13.7 years, 44.1% female, 45.7% current/former smokers. Reported management strategies included targeted vasodilators, cytotoxic PLCH therapies (e.g., cladribine), smoking cessation, and selective surgery/transplant. In this small series, Sotatercept added to background therapy produced marked clinical and hemodynamic gains in refractory PLCH-PH. These effects of Sotatercept in Group 5 PH-are encouraging but limited by sample size and retrospective design. Prospective, collaborative studies are needed to define safety, efficacy, and optimal patient selection.
Percutaneous Reperfusion Therapies vs. Anticoagulation in Patients With Acute Intermediate-High-Risk Pulmonary Embolism: The PRETHA Randomized Clinical Trial.
Ivanauskienė T, Berūkštis A, Burneikaitė G … +7 more , Daubaraitė A, Ivanauskaitė K, Matačiūnas M, Navickas G, Kūgienė R, Kurzyna M, Glaveckaitė S
Pulm Circ · 2026 Jan · PMID 41608297 · Full text
The optimal treatment strategy for patients with acute intermediate-high-risk pulmonary embolism (PE) remains uncertain. This randomized clinical trial (PRETHA) aimed to evaluate the efficacy and safety of percutaneous r...
The optimal treatment strategy for patients with acute intermediate-high-risk pulmonary embolism (PE) remains uncertain. This randomized clinical trial (PRETHA) aimed to evaluate the efficacy and safety of percutaneous reperfusion therapies-trans-catheter thrombectomy and trans-catheter thrombolysis-compared with standard anticoagulation therapy. In this single-center, prospective trial conducted between April 2020 and April 2022, 39 patients with acute intermediate-high-risk PE were randomly assigned (1:1:1) to receive trans-catheter thrombectomy, trans-catheter thrombolysis, or conservative medical therapy with anticoagulation. Echocardiographic, hemodynamic, and biomarker parameters were assessed at baseline, 48 h, and at 1-, 6-, and 12-month follow-up. At 48 h, both interventional groups demonstrated significant improvement in right ventricular (RV) function and pulmonary pressures. The RV/LV ratio decreased by 0.3 (95% CI: 0.13-0.69; < 0.0002) in the thrombectomy group and by 0.4 (95% CI: 0.12-0.96; < 0.0002) in the thrombolysis group. Noninvasively measured systolic pulmonary artery pressure decreased by 29% in the thrombectomy group and by 39% in the thrombolysis group (both < 0.001). Significant reductions in direct systolic and mean pulmonary artery pressures were also observed ( = 0.0002). However, at longer (1 to 12 months) follow-up, all three treatment groups represent similar positive changes of echocardiographic parameters and cardio-specific biomarkers independent of the treatment tactic chosen in the acute period. Functional capacity and quality of life were superior in the interventional groups compared with anticoagulation alone. The incidence of adverse events was highest in the thrombolysis group (38%), whereas thrombectomy and medical therapy demonstrated more favorable safety profiles. Percutaneous reperfusion therapies were associated with earlier improvements in hemodynamic and functional surrogate parameters compared with anticoagulation alone; however, at 1-year follow-up, echocardiographic measures and biomarkers of cardiac function were similar across all treatment groups. These findings should be interpreted as mechanistic and hypothesis-generating.
Chronic Obstructive Pulmonary Disease and Pulmonary Hypertension: A Comparative Study of Biomarkers and Clinical Indicators.
Huang Y, Qin B, Shen B … +5 more , Zhu J, Zheng Q, Zheng X, Hu J, Song Y
Pulm Circ · 2026 Jan · PMID 41586302 · Full text
A major consequence of acute exacerbation of chronic obstructive pulmonary disease (AECOPD) is pulmonary hypertension (PH), which raises morbidity and mortality rates. This study assessed biomarker profiles, clinical cha...
A major consequence of acute exacerbation of chronic obstructive pulmonary disease (AECOPD) is pulmonary hypertension (PH), which raises morbidity and mortality rates. This study assessed biomarker profiles, clinical characteristics and diagnostic efficacy of important coagulation and inflammatory markers in AECOPD patients who have varying degrees of PH severity. A total of 248 AECOPD patients were included and divided into three groups according to the severity of their PH. Analysis of biomarker levels, such as B-type natriuretic peptide (BNP), troponin I (TNT-I), d-dimer, C-reactive protein (CRP), and coagulation markers, besides pulmonary artery systolic pressure was done. These biomarkers' diagnostic precision for PH was evaluated by receiver operating characteristic curve analysis. PH patients were much older than those without PH. The moderate to severe PH group had highest BNP levels (619.92 ± 945.81 pg/mL), which rose gradually with PH severity. TNT-I, CRP and D-dimer showed similar patterns. The most dependable biomarker for PH, according to ROC analysis, was BNP (AUC = 0.803), followed by TNT-I (AUC = 0.712) and D-dimer (AUC = 0.694). Prothrombin and Activated Partial Thromboplastin had a considerable predictive value, although fibrinogen's diagnostic utility was restricted (AUC = 0.559). BNP showed the highest predictive value with an AUC, suggested BNP is most important biomarker for PH in AECOPD patients, TNT-I and D-dimer serve as useful secondary markers. The observed elevations in coagulation and inflammatory markers indicate their potential role in PH pathogenesis.
Beta-Alanine Supplementation Ameliorates Right Ventricular Remodeling Caused by Monocrotaline-Induced Pulmonary Hypertension.
Su H, Li B, Guo Z … +9 more , Zhang F, Wang A, Jiang K, Zhu H, Yang Y, Zeng J, Li W, Cao Y, Liu J
Pulm Circ · 2026 Jan · PMID 41586301 · Full text
Pulmonary hypertension (PH) causes progressive pulmonary vascular resistance and right heart failure. We investigated whether beta-alanine (β-Ala) improves right ventricular (RV) remodeling and dysfunction in a monocrota...
Pulmonary hypertension (PH) causes progressive pulmonary vascular resistance and right heart failure. We investigated whether beta-alanine (β-Ala) improves right ventricular (RV) remodeling and dysfunction in a monocrotaline (MCT)-induced PH rat model. Male Wistar rats were assigned to control, MCT-PH, and β-Ala-treated PH groups. RV function was assessed by RVSP and RVHI; molecular changes were examined by western blotting and qPCR; histology evaluated RV hypertrophy and fibrosis. β-Ala significantly improved RVSP and RVHI versus MCT. Mechanistically, β-Ala reduced ERK and p38 MAPK signaling while enhancing AKT activation. It decreased proapoptotic Bax and cleaved Caspase-3 and increased antiapoptotic Bcl-2. qPCR showed downregulation of ANP, BNP, β-MHC, and TGF-β, with upregulation of β-MHC. Histological analyses confirmed attenuation of RV hypertrophy and fibrosis. Overall, β-Ala mitigates RV remodeling and dysfunction in MCT-induced PH, likely via modulation of MAPK/AKT pathways and apoptosis, supporting its potential as a therapy for PH-related right heart dysfunction.
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