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%, p < 0.01) and RV-3D-ESV > 109 mL (62.4% vs. 44.6%, p < 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.

Sleep-disordered breathing (SDB) has heterogeneous cardiovascular risks that are not fully captured by the apnea–hypopnea index (AHI). We aimed to determine whether data-driven sleep–cardiometabolic phenotypes identify long-term cardiovascular risk and support phenotype-based risk stratification. We analyzed 5682 adults from the Sleep Heart Health Study baseline cohort enrolled in 1995-1998 (mean [SD] age, 63.2 [11.2] years; 52.7% female). Fifteen prespecified variables were used for k-means clustering to define SDB phenotypes. Participants with adjudicated follow-up data (n = 4909) underwent time-to-event analyses with a primary composite endpoint of various cardiovascular events. Three phenotypes were identified: Cluster 1 (aging–hypertensive OSA), Cluster 2 (healthy sleep-CVD profile), and Cluster 3 (metabolic–hypoxic OSA). Clusters 1 and 3 showed higher risks of the composite endpoint than Cluster 2 (hazard ratios [HRs], 4.06 [95% confidence interval (CI), 3.50–4.71] and 3.62 [95% CI, 2.94–4.44], respectively) and consistently elevated cardiovascular death, myocardial infarction, and stroke risks. Heart failure risk was pronounced for Clusters 1 and 3 (HRs, 5.46 [95% CI, 4.41–6.76] and 5.55 [95% CI, 4.23–7.29], respectively) compared with Cluster 2. The multinomial classifier demonstrated high accuracy and discrimination in the internal test set. The phenotype-based risk score exhibited a clear stepwise gradient in predicted and observed cardiovascular risk and retained prognostic value in the external cohort, outperforming the AHI with acceptable calibration. Multidimensional phenotyping identifies SDB subgroups with divergent cardiovascular risk. A phenotype-based score using routine variables may improve risk stratification beyond event frequency alone. Sleep-disordered breathing (sleep apnea) is common and can increase the risk of heart disease and stroke. It is usually graded by the apnea–hypopnea index (AHI), but AHI does not fully capture risk. We analyzed 5682 adults in a cohort with sleep testing and long follow-up. A computer method grouped people into three profiles: older with high blood pressure, low-risk healthy profile, and obesity with low oxygen at night. Compared with the group at lower risk, the older and obesity profiles had about four times more cardiovascular events, especially heart failure. A score using routine measures predicted risk better than AHI and also worked in an external cohort. This approach may help identify people at higher-risk earlier and support more personalized prevention and treatment. Huang, Xiang et al. identify sleep-disordered breathing phenotypes using clinical and sleep data from a community cohort. The phenotypes show divergent cardiovascular risks, and a phenotype-based score improves risk stratification beyond the apnea–hypopnea index.

BACKGROUND:Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is characterized by substantial prognostic and therapeutic heterogeneity. Conventional clinical and anatomical-pathophysiological classifications fail to fully capture these intricacies. Unsupervised clustering approaches such as latent class analysis (LCA) have been used to address this limitation. RESEARCH QUESTION:Can LCA-derived phenogroups refine risk stratification and predict PAH-targeted treatment responses in adult patients with PAH-CHD? STUDY DESIGN AND METHODS:Adult patients with PAH-CHD (N = 889) from a multicenter prospective registry were stratified into post- and pre-tricuspid shunt subsets. LCA was performed in each subset using 28 candidate clinical, laboratory, and hemodynamic variables. Ten-year survival and the effect of PAH-targeted therapy across phenogroups were evaluated. RESULTS:LCA identified 4 phenogroups in each subset with distinct phenotyping patterns. In post-tricuspid shunts, Group 1 ("adaptation with open shunt") exhibited preserved right ventricular function and the best survival despite high afterload. Group 3 ("maladaptation and failure") and Group 4 ("closed shunt and failure") faced 2- to 3-fold higher mortality than Group 1. Notably, Group 2 ("hyperkinetic state") uniquely benefited from combination therapy (Pinteraction = .026). In pre-tricuspid shunts, Group 1 ("high shunt and mild resistance") and Group 3 ("young female and low inflammation") exhibited preserved survival despite varying afterloads. However, Group 2 ("old male and comorbidities") had significantly higher mortality and an attenuated treatment response (Pinteraction = .037) despite sharing mild hemodynamics with Group 1. Group 4 ("high resistance and failure") faced severe hypoxemia and the worst prognosis. INTERPRETATION:LCA successfully stratified patients with PAH-CHD into distinct phenogroups with varied clinical profiles, survival, and treatment responses. This data-driven phenotyping system provides a novel approach for risk stratification and may guide personalized clinical decision-making. CLINICAL TRIAL REGISTRATION:ClinicalTrials.gov; No.: NCT01417338; URL: www. CLINICALTRIALS:gov.

INTRODUCTION:Fibrosing mediastinitis (FM) - also known as mediastinal fibrosis or sclerosing mediastinitis - is a rare, benign, but potentially life-threatening disorder of unclear etiology. This study aimed to evaluate the clinical characteristics, geographic distribution, and long-term survival of patients with FM and to identify prognostic factors for all-cause mortality. METHODS:Of 215 patients screened, 192 were diagnosed with FM and included in the final cohort. The clinical characteristics and survival of patients with FM were reported. Prognostic factors were evaluated by Cox regression model. RESULTS:The mean age of FM patients was 66.6 ± 13.4 years. Geographic clustering was evident in northwestern China. Compared with survivors, deceased patients showed higher rates of edema (50.0% vs. 22.3%, p = 0.016), lower serum cholesterol (3.8 ± 0.8 mmol/L vs. 4.5 ± 1.1 mmol/L, p = 0.004), and elevated NT-proBNP (751.8 pg/mL vs. 220.5 pg/mL, p = 0.001). Echocardiography indicated more severe right ventricular remodeling in the deceased group. Chest CT commonly revealed bilateral mediastinal involvement (85.4%), lobar vascular compression, and mediastinal lymphadenopathy (88.5%). Over a median follow-up of 23 months, the 1-, 3-, and 5-year survival rates were 94.5%, 86.3%, and 84.6%, respectively. Right heart failure was the leading cause of death (50%). Multivariable Cox analysis identified smoking (HR 4.32, 95% CI 1.61-11.62, p = 0.004) and elevated systolic pulmonary artery pressure (sPAP) (HR 1.02 per mm Hg, 95% CI 1.01-1.03, p = 0.017) as independent predictors of all-cause mortality. FM-related deaths were more strongly associated with current smoking (HR 15.18, p < 0.001), presence of pleural effusion (HR 5.10, p = 0.03), and elevated sPAP (HR 1.02 per mm Hg, p = 0.011). CONCLUSION:FM predominantly affects older adults and is geographically clustered in northwestern China. Smoking and elevated sPAP are key prognostic indicators for mortality. Despite substantial cardiopulmonary impairment, long-term survival remains relatively favorable.

Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by progressive pulmonary vascular remodeling and inflammation. Current therapies primarily focus on vasodilation and short-term symptom management, with limited efficacy in reversing vascular remodeling, suppressing oxidative stress, or modulating immune-inflammatory responses. To address this, we developed epigallocatechin gallate/manganese dioxide core-shell nanoparticles (EM NPs) as a carrier-free nanomedicine for PAH therapy, designed to simultaneously target key pathological processes for synergistic therapeutic effects. Our results demonstrated that EM NPs effectively scavenged reactive oxygen species, protected pulmonary artery smooth muscle cells (PASMCs) from oxidative damage, considerably improved hemodynamic parameters (reduced right ventricular systolic pressure (RVSP), mean pulmonary arterial pressure (mPAP), and right ventricular hypertrophy index (RVHI)), and attenuated pulmonary vascular remodeling without inducing hepatorenal toxicity. Concurrently, EM NPs markedly modulated inflammatory responses, indicated by the suppression of key pro-inflammatory mediators (CC Motif Chemokine Ligand 5 (CCL5), macrophage inflammatory protein-3 alpha (MIP-3α), monocyte chemoattractant protein-1 (MCP-1), interleukin (IL)-18 and the elevation of anti-inflammatory cytokines (IL-4, IL-13). Furthermore, the core-shell structure significantly enhanced the stability and bioavailability of natural polyphenol, thereby improving its in vivo therapeutic efficacy. Analysis of RNA sequencing data indicated that EM NPs suppressed immune activation in lung tissues by downregulating key pro-inflammatory genes and pathways. With their ability to concurrently target oxidative stress, vascular remodeling, and inflammation in PAH, EM NPs emerge as a promising nanotherapeutic candidate, demonstrating considerable clinical translation potential.

BACKGROUND:Older patients with pulmonary embolism (PE) are at increased risk of bleeding, which is associated with adverse outcomes, making early risk assessment particularly important. This study evaluated the short-term performance of commonly used bleeding risk scores in patients aged ≥65 years. METHODS:An analysis was conducted in a multicenter, prospective study of patients aged ≥65 years with PE receiving anticoagulation (NCT02943343, 2016). The performance of the PE-SARD, Kuijer, RIETE, BACS, and ATRIA scores was evaluated using the time-dependent area under the curve (AUC) analysis. Calibration was assessed with calibration plots. Sensitivity, specificity, and positive predictive values were calculated. The primary outcomes were major bleeding (MB) at 7 and 14 days. RESULTS:Among 3661 anticoagulated patients aged ≥65 years with acute PE, MB occurred in 18 (0.5%) and 36 (1.0%) patients at 7 and 14 days, respectively, and clinically relevant non-major bleeding (CRNMB) in 69 (1.9%) and 105 (2.9%) patients. Patients with bleeding events had higher mortality than those without bleeding. Discrimination for short-term MB was low to moderate across scores, with RIETE (AUC 0.76, 95% CI 0.65-0.87) and PE-SARD (AUC 0.74, 95% CI 0.63-0.85) showing the most consistent performance at 7 days. Overall discrimination declined at 14 days. All scores demonstrated limited ability to predict CRNMB, and no model showed a consistent net clinical benefit. CONCLUSIONS:In older patients with PE, bleeding risk scores showed low-to-moderate discrimination for short-term bleeding, with moderate early performance observed only in selected scores. None of the scores reliably identified CRNMB, highlighting the limitations of baseline risk assessment in acute care settings.

Data on the 10-year survival of incident IPAH in China is lacking. The changing trend of characteristics and survival of IPAH over the past decade has yet been demonstrated. The 4-stratum risk model needs further validation in different cohorts. Incident cases of IPAH identified in the national multicenter prospective registry of PAH from 2009 to 2019 were analyzed. Patients with at least two of the three acquired variables (WHO-FC, 6MWD, NT-pro BNP/BNP) were included for risk stratification. 605 incident patients with IPAH were included (age: 35 ± 12 years; 75.2

To the Editor: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but serious condition characterized by obstruction of the pulmonary arteries due to thromboembolic events and subsequent vascular remodeling, which leads to pulmonary hypertension (PH). Classified as Group 4 in the 2022 PH guidelines by the European Society of Cardiology and the European Respiratory Society, CTEPH poses significant risks, including progressive PH, right ventricular dysfunction, and mortality if left untreated. Despite established diagnostic and treatment guidelines, the exact national prevalence of CTEPH remains largely unknown due to the absence of organized registries, with estimates ranging from 3 to 30 cases per million people. Although CTEPH is commonly thought to follow acute pulmonary embolism (PE), epidemiological data reveal a discrepancy; studies suggest that only 0.1% to 3.8% of PE patients may develop CTEPH.[1,2] Recent reports indicate that 65.6% of CTEPH patients have a history of acute PE, although this proportion varies across regions.[3] Treatment options include pulmonary endarterectomy (PEA), riociguat, and balloon pulmonary angioplasty (BPA). CTEPH significantly impacts patients' quality of life, causing symptoms such as shortness of breath and fatigue, which lead to increased healthcare utilization and economic burden. In China, limited awareness and delayed diagnosis further complicate the management of CTEPH, underscoring the need for studies that address the disease's burden and enhance recognition among healthcare providers. Therefore, this systematic review aimed to estimate the prevalence of CTEPH using data from PE patients and to investigate the characteristics and management patterns of CTEPH in the Chinese population. This study adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement and was registered in the PROSPERO database (Ref. CRD42024510051). An electronic search of databases, including PubMed, EMBASE, Web of Science, Cochrane, the Chinese Biomedical Database (CBM), China National Knowledge Infrastructure (CNKI), and the Wanfang Digital Resource Database, was conducted in August 2023. No restrictions were applied regarding language or publication status. Due to the limited epidemiological evidence, we applied Gall's methodology (2017) to indirectly calculate the prevalence of CTEPH in the general Chinese population.[4] This approach involved estimating overall CTEPH prevalence based on the proportion of CTEPH among PE patients, the prevalence of PE in the general population, and the proportion of CTEPH cases associated with prior PE. Diagnosis of PE and CTEPH was based on the definitions used in the original studies. Inclusion criteria varied between the prevalence and characteristics investigations. For the prevalence study, we included observational studies involving the general population and PE patients in China, which reported PE prevalence, the proportion of CTEPH among PE patients, and the number of CTEPH cases with and without a history of PE. For the characteristics investigation, we included prognostic and cross-sectional studies on CTEPH patients in China, focusing on venous thromboembolism (VTE) history, comorbidities, demographics, risk factors, disability rates, and treatment patterns. Exclusion criteria included interventional studies, case reports, abstracts, reviews, conference proceedings, and literature not published in English or Chinese. Both study selection and data extraction were performed by two independent reviewers, with discrepancies resolved through discussion. For dichotomous outcomes, rates were extracted, and pooled proportions with 95% confidence intervals (CIs) were calculated using a random-effects model in R software (version 4.3.1, https://www.r-project.org/). For continuous outcomes, means and standard deviations (SDs) were pooled similarly. The prevalence of CTEPH was estimated using the following formula: Zprevalence of CTEPH =P1proportion of CTEPH following PE×P2prevalence of PE P3proportion of CTEPH cases with a history of PE These proportions were obtained through meta-analysis. For characteristics related to comorbidities and treatment, the results were described narratively. Heterogeneity was assessed using the Cochrane Q test and I2 statistic, with substantial heterogeneity indicated by an I2 ≥50% and a P-value <0.1 from the Q test. Publication bias was evaluated using a funnel plot and Egger's test, but only when there were more than 10 studies. The risk of bias in studies estimating CTEPH prevalence was independently assessed by two reviewers using the Joanna Briggs Institute (JBI) critical appraisal checklist for prevalence studies. Ultimately, 31 studies on CTEPH and 5 studies on PE were included [Supplementary Figures 1 and 2, https://links.lww.com/CM9/C403]. To estimate the prevalence of CTEPH in China, 18 studies were analyzed [Supplementary reference list 1, https://links.lww.com/CM9/C403]. The quality assessment revealed that 83% (15/18) of studies lacked an appropriate sample frame, 50% (9/18) did not enroll an adequate sample size, and 78% (14/18) failed to measure the condition reliably [Supplementary Table 1, https://links.lww.com/CM9/C403]. The pooled proportion of CTEPH following PE (P1) was found to be 5.36% (95% CI: 3.04–7.67%, Supplementary Figure 3, https://links.lww.com/CM9/C403), based on seven studies involving 1843 patients. Two prevalence representations of PE were identified: P2a (0.09% among hospitalized patients, from five studies, 95% CI: 0.08–0.10%, Supplementary Figure 4, https://links.lww.com/CM9/C403) and P2b (0.0071% among the general population, from one study). The proportion of CTEPH cases with a history of PE (P3) was 56.50% (95% CI: 48.61–64.38%, Supplementary Figure 5, https://links.lww.com/CM9/C403). Using these values, the overall prevalence of CTEPH was estimated at 85 cases per million in hospitalized populations (Za) and 6.74 cases per million in the general population (Zb), translating to approximately 9729 individuals with CTEPH in China [Table 1]. Table 1 - Definitions and values of variables in formula used to calculate prevalence of CTEPH among general population in China. Variable Definition Rate (95% CI) Source Sample size P1 Proportion of CTEPH following PE 5.36% (95% CI 3.04 to 7.67%) Gong 2015, Yang 2015, Bian 2015, Guo 2016, Xi 2016, Xu 2022, Xu 2016 1843 P2a Prevalence of PE among hospitalized population 0.09% (95% CI 0.08 to 0.10%) Yang 2011, Li 2016, Liu 2011, Wang 2017, Zhang 2019 55,028,750 P2b Prevalence of hospitalized PE among general population 0.0071% Zhang 2019 NA P3 Proportion of CTEPH cases with a history of PE 56.50% (95% CI 48.61 to 64.38%) Deng 2020, Du 2019, Han 2013, Shu 2007, Wang 2018, Xie 2019 1221 Za Prevalence of CTEPH among hospitalized population 85 cases per million (95% CI 42 to 128 cases per million) P1×P2a/P3 NA Zb Prevalence of CTEPH among general population 6.74 cases per million P1×P2b/P3 NA CI: Confidence interval; CTEPH: Chronic thromboembolic pulmonary hypertension; NA: not applicable; PE: Pulmonary embolism; SD: Standard deviation; SE: Standard error. For references of studies listed in Source column, please see Supplementary reference list 1, https://links.lww.com/CM9/C403. 95% CI of prevalence of CTEPH among general population calculated using delta method SE=p22p32Var(p1)+p12p32Var(p2)+p12p22Var(p3)p34 95% CI: Z ± 1.96SE. The 30 studies provided insights into the baseline clinical characteristics of CTEPH patients [Supplementary references list 2, https://links.lww.com/CM9/C403]. The average age was 57.55 years, with 47.02% of patients being female. The mean body mass index (BMI) was 23.99 kg/m2, and 39.02% had a history of smoking. Notably, 56.50% had a history of PE, while 61.38% were classified as World Health Organization (WHO) functional class III/IV, with an average time from symptom onset to diagnosis of 24.63 months [Supplementary Table 2, https://links.lww.com/CM9/C403]. Egger's tests for mean age, BMI, female proportion, smoking history, and WHO functional class III/IV showed no significant publication bias across the studies (P >0.05, Supplementary Figures 6–10, https://links.lww.com/CM9/C403). Common comorbidities included diabetes mellitus (2.00–33.33%), hypertension (8.33–75.00%), coronary heart disease (6.35–16.67%), and chronic obstructive pulmonary disease (COPD, 2.78–27.27%). Comorbidities associated with the onset of CTEPH included varicose veins in the lower limbs (63.64%) and deep vein thrombosis (DVT; 31.25% to 96.88%; Supplementary Table 3, https://links.lww.com/CM9/C403). Additionally, common risk factors identified for CTEPH included pulmonary artery systolic pressure (PASP) and intermediate- to high-risk PE [Supplementary Table 4, https://links.lww.com/CM9/C403]. Mortality rates associated with CTEPH were reported in seven studies, ranging from 6.94% to 45.45%. Among these, three studies reported additional estimated survival rates: 1-year survival rate was from 93.3% to 97.1%, 3-year survival rate was from 84.6% to 93.3%, 5-year survival rate was from 73.4% to 86.9%, 7-year survival rate was 82.0%, and 8-year survival rate was 66.6%, respectively [Supplementary Table 5, https://links.lww.com/CM9/C403]. Unfortunately, data on disability rates and socioeconomic burdens were not available. Treatment patterns revealed that 11.11% to 35.14% of patients received PEA, and 10.29% to 40.95% were treated with BPA. Medical therapy alone was documented in 30.29% to 88.89% of cases. Among patients using specific medications, a significant proportion received a combination of conventional anticoagulants and other therapies [Supplementary Table 6, https://links.lww.com/CM9/C403]. Compared to global trends of 3 to 30 cases per million, our study found a higher prevalence of CTEPH among hospitalized patients in China. This discrepancy may stem from prior studies that primarily focused on acute PE cases, while CTEPH can also develop from sub-acute and chronic PE, as well as in individuals without a history of PE. Our analysis included a broader range of PE patients, although it may overestimate the prevalence of CTEPH due to the higher likelihood of inpatient cases. Furthermore, the estimation of prevalence in the general population is based on a single study, which requires further validation through larger epidemiological investigations. Our study also reveals a significant delay in diagnosis, with many patients presenting at advanced stages of the disease. This underscores the urgent need to increase awareness for early detection of CTEPH. The absence of standardized guidelines for multimodal therapy, coupled with limited evidence on approved medications such as riociguat, underscores the need for improved treatment strategies and further research in this area. Several limitations of our study should be noted. First, the imprecision inherent in the calculation formula, along with notable heterogeneity due to diverse patient characteristics and study designs, may have impacted the results. Additionally, the lack of tailored prevalence studies further complicates the accuracy of our findings. Risk factor analysis was based on descriptive data rather than pooled data, which hinders a more precise predictive assessment of these factors. The relatively small sample sizes in the studies examining risk factors may contribute to data instability, emphasizing the need for caution in interpreting the results. This also highlights the importance of future research involving larger sample sizes. In conclusion, our study highlights the complexity of the CTEPH landscape in China, stressing the need for greater awareness, specialized databases, and enhanced clinical standards. Ongoing studies are expected to provide further insights into the management patterns and outcomes of CTEPH in the Chinese population. Acknowledgements The authors would like to acknowledge Miss Xue Zhou, Miss Yang Zhang, and Dr. Sitong Dong from Systematic Review Solutions Ltd for their medical writing and editorial support, which was funded by MSD China. Funding This study was supported by the National Key Research and Development Program of China (No. 2023YFC2507200), Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences (CIFMS) (No. 2021-I2M-1-049), National High Level Hospital Clinical Research Funding (No. 2022-NHLHCRF-LX-01-0203), and National Natural Science Foundation of China (Nos. 82241029, 81970058). Conflicts of interest Y Wu and J Zou are employees of MSD China, while Jean-François Richard is an employee of Merck & Co. The other authors declare no competing interests.

This study focused on exploring the correlation between the electrical and mechanical dyssynchrony of the right ventricle (RV) in patients with pre-capillary pulmonary hypertension (PcPH). It also aimed to compare the predictive capabilities of these dyssynchronies for risk stratification. From April 2017 to March 2018, PcPH patients at Fuwai Hospital were consecutively enrolled. They were divided into low-risk and non-low-risk groups according to the 2015 European Society of Cardiology Guidelines. Off-line software (GE EchoPAC version 201) was used to measure RV mechanical dyssynchrony (standard deviation of the time from QRS onset to peak strain for the six RV segments [RV-SD6]), while QRS duration representing electrical dyssynchrony was measured manually. In total, 66 PcPH patients (average 35 years, 19 males and 47 females) were enrolled, 37 in the low-risk group and 29 in the non-low-risk group. QRS duration was significantly correlated with RV-SD6 (r = 0.25, p = 0.047). Both RV-SD6 and QRS duration were significantly correlated with N‑terminal pro-brain natriuretic peptide levels (r = 0.44, p < 0.001 vs. r = 0.26, p = 0.039). Furthermore, RV-SD6 (area under the curve [AUC]: 0.75, 95

BACKGROUND:Fibrosing mediastinitis (FM) is a rare condition that may be complicated by pulmonary hypertension (PH). This multicenter study aimed to investigate the clinical features, long-term survival outcomes, and prognostic factors in patients with fibrosing mediastinitis associated pulmonary hypertension (FM-PH). METHODS:A total of 85 FM-PH patients were enrolled across seven centers in China between January 2007 and July 2024. Patients were classified into two groups based on the occurence of clinical worsening (CW): FM-PH with CW and FM-PH without CW. Clinical worsening was defined as a composite of all-cause mortality, rehospitalization for heart failure, or deterioration in World Health Organization functional class (WHO-FC) compared with baseline. RESULTS:Of the 85 FM-PH patients, 37 were classified as FM-PH without CW and 48 as FM-PH with CW. The FM-PH with CW patients had significantly higher levels of systemic inflammation, elevated N-terminal pro-brain natriuretic peptide (NT-proBNP), higher mean pulmonary artery pressure (mPAP), and worse right heart function compared to the FM-PH without CW patients. Multivariate Cox regression analysis showed that high-sensitivity C-reactive protein (hs-CRP), mPAP, and peripheral edema were independently associated with clinical worsening in FM-PH patients. Over a median follow-up of 27 months [IQR 11-55], 48 patients experienced clinical worsening events, including five deaths. The 1-, 3-, and 5-year overall survival rates were 94.5%, 86.3%, and 84.6%, respectively. However, the 5-year clinical worsening-free rate was only 26.6%. CONCLUSION:Although the 1-, 3-, and 5-year overall survival rates were relatively favorable, the 5-year clinical worsening-free rate was not satisfying. Elevated hs-CRP, mPAP and the presence of peripheral edema were independently associated with worse clinical outcomes.

Infective endocarditis (IE) is an inflammatory disease caused by the infection of the endocardium or heart valves by pathogenic microorganisms. It is characterized by diagnostic challenges, difficult treatment, and high mortality. Multimodal imaging techniques, including echocardiography, computed tomography (CT), magnetic resonance imaging (MRI), and nuclear medicine imaging, play a crucial role in the diagnosis of IE. Echocardiography is the first-line imaging modality for suspected IE. Cardiac CT, with its excellent spatial resolution and three-dimensional (3D) reconstruction capabilities, is helpful in detecting paravalvular abscesses, fistulas, and pseudoaneurysms. MRI has advantages in identifying neurological complications and assessing myocardial involvement. Nuclear imaging demonstrates high specificity in detecting prosthetic valve IE and device-related infections. These imaging techniques are important in detecting perivalvular complications, evaluating local and distant spread of infection, and guiding therapeutic interventions, thereby enhancing the diagnostic and therapeutic management of IE.

Pulmonary arterial compliance (PAC) reflects the pulsatile load and predicts outcome in pulmonary arterial hypertension (PAH). The prognostic role of PAC in the heterogeneous patient population of PAH associated with congenital heart disease (PAH-CHD) is poorly defined. This study aimed to explore the prognostic value of PAC in patients with PAH-CHD. Adult patients diagnosed with PAH-CHD were collected from a PAH multicenter prospective registry between August 2009 and December 2019. The primary endpoint was all-cause mortality. Multivariable Cox regression and restricted cubic spline (RCS) analysis were used to evaluate the association between PAC and the primary endpoint. Subgroup and interaction analysis between PAC and shunts or defect characteristics were explored. Incremental predictive performance was evaluated by calculating the C-index, continuous net reclassification improvement, and integrated discrimination improvement. A total of 434 adult PAH-CHD patients were enrolled. The median follow-up time was 52.2 months. The survival rate of patients in the lower PAC group was significantly worse than those in the higher PAC group (Log-rank P < 0.001). Multivariable Cox regression analysis showed that PAC independently predicted all-cause mortality after adjustment for other prognostic factors, whether as a continuous variable (HR = 0.665, 95

Background:Chronic thromboembolic pulmonary hypertension (CTEPH), a progressive disease, can lead to decompensation of the right half of the heart and death. Studies on CTEPH have increased in number over the last two decades. This study aimed to perform a bibliometric analysis of the global scientific output on CTEPH in the last 24 years to identify research trends and hot topics. Methods:We searched the Web of Science Core Collection (WoSCC) Science Citation Index Expanded (Science Citation Index Expanded and Social Sciences Citation Index) for literature on CTEPH published from January 1, 2000 to January 31, 2024, and recorded their information. The Bibliometrix software package was used for bibliometric indicator analysis, and VOSviewer and CiteSpace were used to visualize trends and hotspots of CTEPH research. Results:Overall, 2,264 publications were included in the bibliometric analysis. The number of annual publications increased drastically from 14 in 2000 to 199 in 2023. The number of publications on CTEPH has increased over the last two decades, with 24.4% (n=553) of the publications published in top academic journals. The United States ranked first in publications and had the best CTEPH-investigating institutions; it also had the highest level of international cooperation. Distinctively, 7 of the top 10 institutions and 7 of the top 10 researchers in the field were from Europe with the most influential scientists. A relatively high percentage of the publications were published in top-rank academic journals, especially in respirology and cardiology journals. Pulmonary endarterectomy (PEA) was the main therapy during the first decade, while balloon pulmonary angioplasty (BPA) and pulmonary arterial hypertension (PAH)-targeted medicine have recently gained interest. Dual-energy computed tomography (DECT), right ventricular function, and the role of pulmonary endothelium in microvasculopathy have become the focus of CTEPH research. Conclusions:The number of publications on CTEPH has increased over the last two decades, and CTEPH has become a significant topic in both respirology and cardiology. Our results indicate that future research in this field will continue to focus on elucidating the pathophysiological mechanisms underlying this disorder, improving diagnostic accuracy, and developing novel imaging techniques. The integration of existing therapeutic modalities and the elucidation of right ventricular dysfunction are key areas of research.

Background: This study focused on exploring the correlation between the electrical and mechanical dyssynchrony of the right ventricle (RV) in pre-capillary pulmonary hypertension (PcPH) patients. It also aimed to compare their predictive capabilities for risk stratification. Methods: From April 2017 to March 2018, PcPH patients at Fuwai Hospital were consecutively enrolled. They were grouped as low-risk and non-low-risk according to 2015 European Society of Cardiology Guidelines. RV mechanical dyssynchrony (RV-SD6) was measured by off-line software (GE EchoPAC version 201), and QRS duration representing electrical dyssynchrony was manually measured. Results: Sixty-six PcPH patients (average 35 years, 19 males and 47 females) were finally enrolled, 37 in low-risk group, while 29 in non-low-risk group. QRS duration was significantly correlated with RV-SD6 (r = 0.25, P = 0.047). Both RV-SD6 and QRS duration significantly correlated with N-terminal pro-brain natriuretic peptide (r = 0.44, P＜0.001 vs r = 0.26, P = 0.039). Furthermore, RV-SD6 (area under curve, AUC 0.75, 95% confidence interval, CI 0.64–0.87, P ＜ 0.001) and QRS duration (AUC 0.65, 95% CI 0.52–0.78, P = 0.036) both had the potential to predict non-low risk stratification. Multivariate Logistic regression analyses identified RV-SD6 (OR: 1.02, 95% CI: 1.01–1.03, P = 0.009) and QRS duration (odds ratio, OR: 1.07, 95% CI: 1.00–1.15, P = 0.045) as independent predictors of non-low risk of PcPH patients. Conclusion: RV-SD6-presented mechanical dyssynchrony correlates with QRS duration and better predicts risk stratification in PcPH patients without complete bundle branch block.

RATIONALE:The transcatheter closure and atrioseptopexy are the main treatment methods for atrial septal defect (ASD). However, persistent hypoxemia due to iatrogenic diversion of inferior vena cava (IVC) to the left atrium (LA) is reported as a rare complication after ASD closure. Contrast echocardiology is a reliable and powerful tool to detect iatrogenic diversion and identify the etiology accurately. PATIENT CONCERNS:We reported 2 patients (a 11-year-old boy [Patient 1] and a 39-year-old female [Patient 2]) with persistent hypoxemia and exertional dyspnea after ASD transcatheter closure and robotic atrioseptopexy, respectively. DIAGNOSES:Contrast echocardiography confirmed the presence of a right-to-left shunt at the atrial level which was presented only in femoral venous contrast injection instead of upper extremity venous contrast injection. INTERVENTIONS AND OUTCOMES:Subsequent surgical exploration found that the occluder straddling the entry of IVC and the fibrous membrane proliferating along the lower edge of the occlude in the first patient, and the patch improperly linked to the Eustachian valve in the second patient. The misoperation led to IVC partially draining into LA. After the surgeries, both of them had their hypoxic symptoms relieved. LESSONS:Persistent unexplained hypoxemia after ASD closure might be considered to result from a right-to-left shunt. An iatrogenic right-to-left shunt flow from the IVC to the LA was usually caused by the misplaced interatrial occluder or patch. Transthoracic agitated saline contrast echocardiography via combined peripheral venous access has the well-performed capability to hint some insidious right-to-left shunts and guides clinical therapy as soon as possible.

AIMS:The prevalence of heart failure (HF) continues to rise with a growing impact on younger populations, while epidemiological comprehensive data on HF in adolescents and young adults remain scarce. This study aimed to report population shifts and HF burden among individuals aged 15-49 years from 1990 to 2021, with projections through 2035. METHODS AND RESULTS:Data were retrieved from the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2021 for individuals aged 15-49 years. We investigated the population shift and analysed the temporal trend in HF prevalence and years lived with disability (YLDs) from 1990 to 2021 using the estimated annual percentage change (EAPC) and age-period-cohort (APC) model. We also assessed the underlying causes of HF and examined the associations between Sociodemographic Index (SDI) and the prevalence and YLDs of HF using Pearson correlation. Finally, the Bayesian age-period-cohort (BAPC) model was employed to project HF burdens through 2035. Between 1990 and 2021, global HF prevalence among individuals aged 15-49 years increased by 76.76% (95% uncertainty interval: 71.02, 83.33), with the most substantial growth observed in the African region (2.67-fold), Eastern Mediterranean region (2.63-fold), and South-East Asia region (2.00-fold). By 2021, global HF prevalence reached 7048.1 thousand (5968.4, 8317.3). Males consistently demonstrated higher HF burdens than females across all World Health Organization (WHO) regions. Ischaemic heart disease emerged as the primary contributor to HF prevalence and YLDs worldwide. SDI showed positive correlations with both HF prevalence rate in 2021 and their percentage changes from 1990 to 2021 in specific WHO regions. Projections suggest global HF prevalence will rise to 8115.4 thousand cases (5754.7, 10476.1) by 2035. CONCLUSION:From 1990 to 2021, the youths and young adults of 15-49 years worldwide experienced escalating HF burdens marked by significant spatial heterogeneities and sexual differences. The imperative of taking action to reduce the HF burden among the younger population should be tailored to countries characterized by different socio-economic development levels.