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Aims Novel echocardiographic parameters of right ventricular (RV) function, including speckle‐tracking‐derived, three‐dimensional, and RV–pulmonary artery coupling parameters, have emerged for the evaluation of pulmonary arterial hypertension (PAH). The relative role of these parameters in the risk stratification of PAH patients is unclear. We compared the performance of multiple RV parameters and sought to establish an optimal model for identifying the risk profile of patients with PAH. Methods and results Comprehensive risk assessments were performed for 70 patients with PAH. The risk profile of every patient was determined based on the guideline recommendations. Conventional parameters, including fractional area change (FAC) and tricuspid annular plane systolic excursion (TAPSE), novel speckle‐tracking‐derived RV longitudinal strain (RVLS), and three‐dimensional RV ejection fraction (3D‐RVEF), were used to evaluate RV function. Pressure–strain loops were measured for the assessment of RV myocardial work, including RV global wasted work (RVGWW). RV–pulmonary artery coupling was assessed by indexing RV parameters to the estimated pulmonary artery systolic pressure (PASP). The median age was 34 (30–43) years, and 62 (88.6%) patients were female. Forty‐five patients were classified into the low‐risk group, while 25 patients were classified into the intermediate–high‐risk group. Most RV parameters could be used to determine the risk profile and exhibited significantly improved diagnostic performance after indexing to PASP (including FAC/PASP, TAPSE/PASP, and 3D‐RVEF/PASP). RVLS/PASP showed the best performance, with an area under the curve of 0.895. In multivariate analysis (Model 1), only RVGWW (>90.5 mmHg%), RVLS (> −16.7%), and TAPSE (<17.5 mm) remained significant (all P < 0.05). Model 1 outperformed every single RV parameter, with a significantly larger area under the curve (all P < 0.05). With PASP indexing in Model 2, RVLS/PASP > −0.275 [odds ratio (OR) 20.63, 95% confidence interval (CI) 4.62–92.11, P < 0.001] and RVGWW > 90.5 mmHg% (OR 6.17, 95% CI 1.37–27.76, P = 0.018) independently identified a higher risk profile. The addition of RVGWW to two models determined incremental value in identification (continuous net reclassification improvement 1.058, 95% CI 0.639–1.477, P < 0.001). Conclusions The combination models for RV function outperformed any single parameter in identifying the risk profile of patients with PAH. Comprehensive assessment of RV–pulmonary artery coupling using multiparametric methods is clinically meaningful in patients with PAH.

In 2022, new guidelines for the diagnosis and treatment of pulmonary hypertension (PH) revised the hemodynamic definition, reducing the mean pulmonary artery pressure threshold from ≥ 25 to > 20 mmHg. The optimal threshold of transthoracic echocardiography (TTE) parameters and the predictive capability require further validation. This study aims to investigate the diagnostic value of TTE parameters under the new hemodynamic criteria. Retrospective analysis of PH patients who underwent right heart catheterization and TTE examination between 2017 and 2022 in a single center. Logistic regression was employed to ascertain the predictive capacity of parameters across various conditions. Receiver operating characteristic curves were used to determine the optimal cutoff values based on the new criteria. In a cohort of 213 patients, the optimal cutoff values identified were a tricuspid annular plane systolic excursion (TAPSE) to systolic pulmonary arterial pressure (sPAP) ratio of < 0.50 mm/mmHg, a right ventricular outflow tract acceleration time (RVOT-AT) of < 93 ms, and a right atrial area (RAA) > of 14.5 cm . Regardless of the inclusion of tricuspid regurgitation velocity (TRV) and related parameters, RVOT-AT < 93 ms manifested as an effective predictive parameter. A combination of RVOT-AT < 93 ms, main pulmonary artery diameter > 25 mm and RAA > 14.5 cm exhibited better specificity. The threshold values for TAPSE/sPAP, RVOT-AT, and RAA should be adjusted to improve the predictive capacity of PH based on revised criteria in this single-center dataset. RVOT-AT was a promising indirect parameter, and the utilization of combined indirect indicators may enhance diagnostic accuracy, particularly in instances where satisfactory TRV measurements are unavailable.

Patients with pulmonary arterial hypertension (PAH) exhibit exertional dyspnea and decreased exercise capacity, which are not solely attributable to right heart dysfunction. Numerous studies have aimed to elucidate pulmonary function in PH patients and its correlation with disease severity and prognosis; however, the findings remain inconsistent. The impairment of ventilation and diffusion function may partially account for the occurrence of exertional dyspnea in PAH patients. This was a single-center prospective observational study. Pulmonary function tests, right heart catheterization, and four-strata risk status stratification were performed in PAH patients. The PAH patients were followed up for 12 months. A total of 181 PAH patients were enrolled in the study, comprising 62 with idiopathic pulmonary arterial hypertension (IPAH) and heritable PAH (HPAH), 69 with PAH associated with congenital heart disease (CHD-PAH), and 50 with PAH associated with connective tissue disease (CTD-PAH). Forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), single-breath diffusion capacity for carbon monoxide (DLCO), DLCO% predicted (% pred), and reactance at 5 Hz (X5) were significantly reduced, while residual volume (RV)% pred increased in PAH patients. CHD-PAH exhibited more pronounced ventilation impairment. Six-minute walking distance (6MWD) demonstrated a positive correlation with FEV (r = 0.353,  < 0.01) and FVC (r = 0.373,  < 0.01), respectively. A total of 104 patients finished the follow-up. Patients exhibiting FVC% pred values below 82% demonstrated a diminished response to PAH-targeted therapy (OR = 10.553,  = 0.000, 95% CI: 2.580-43.165). PAH patients exhibited impairment in both ventilation and diffusion capacity, while patients with diverse etiologies demonstrated distinct characteristics. FVC and FEV were positively correlated with 6MWD, respectively. PAH patients with FVC% pred values below 82% demonstrated a diminished response to PAH-targeted therapy.

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive pulmonary vascular disorder with substantial morbidity and mortality, also a disease underdiagnosed and undertreated. It is potentially curable by pulmonary endarterectomy (PEA) in patients with surgically accessible thrombi. Balloon pulmonary angioplasty (BPA) and targeted medical therapy are options for patients with distal lesions or persistent/recurrent pulmonary hypertension after PEA. There is an urgent need to increase the awareness of CTEPH. Qualified CTEPH centers are still quite limited. Baseline characteristics, management pattern and clinical outcome of CTEPH in China needs to be reported. Methods and design The CHinese reAl-world study to iNvestigate the manaGEment pattern and outcomes of chronic thromboembolic pulmonary hypertension (CHANGE) study is designed to provide the multimodality treatment pattern and clinical outcomes of CTEPH in China. Consecutive patients who are ≥ 14 year-old and diagnosed with CTEPH are enrolled. The diagnosis of CTEPH is confirmed in right heart catheterization and imaging examinations. The multimodality therapeutic strategy, which consists of PEA, BPA and targeted medical therapy, is made by a multidisciplinary team. The blood sample and tissue from PEA are stored in the central biobank for further research. The patients receive regular follow-up every 3 or 6 months for at least 3 years. The primary outcomes include all-cause mortality and changes in functional and hemodynamic parameters from baseline. The secondary outcomes include the proportion of patients experiencing lung transplantation, the proportion of patients experiencing heart and lung transplantation, and changes in health-related quality of life. Up to 31 December 2023, the study has enrolled 1500 eligible patients from 18 expert centers. Conclusions As a real-world study, the CHANGE study is expected to increase our understanding of CTEPH, and to fill the gap between guidelines and the clinical practice in the diagnosis, assessment and treatment of patients with CTEPH. Registration Number in ClinicalTrials.gov NCT05311072.

Background The efficacy and safety of ambrisentan has been previously evaluated in Chinese patients with pulmonary arterial hypertension (PAH). This post-hoc analysis assessed the efficacy and safety of ambrisentan in a subgroup of connective tissue disease (CTD) patients with PAH. Methods In this open-label, single-arm study, patients received ambrisentan 5 mg once daily for 12 weeks, followed by 12-week dose titration period (dose up to 10 mg). Efficacy endpoints included change from baseline in exercise capacity (measured by 6-min walk test [6MWT]), N-terminal pro B type natriuretic peptide (NT-proBNP) plasma levels, WHO Functional Class (FC) and Borg Dyspnoea Index (BDI) scores from baseline to weeks 12 and 24. Safety endpoints included time to clinical worsening and incidence of adverse events (AEs). Results In total, 71 Chinese patients with CTD-PAH were included in this analysis. Ambrisentan treatment significantly improved exercise capacity (6MWT) from baseline (mean: 366.4 m) to week 12 (63.8 m, p  < 0.001) and week 24 (73.2 m, p <  0.001). A significant reduction in NT-proBNP levels was observed from baseline (mean: 1837.5 ng/L) to week 12 (− 1156.8 ng/L, p  < 0.001) and week 24 (− 1095.5 ng/L, p <  0.001). BDI scores decreased significantly at week 12 (− 0.6, p <  0.001) and week 24 (− 0.4, p  = 0.002) from baseline (mean: 2.7). The WHO FC improved in 29 (40.8%) and 34 (47.9%) patients at weeks 12 and 24, respectively. Adverse events were reported in 52 (73.2%) patients. One patient (1.4%) experienced clinical worsening at week 24. Conclusion Ambrisentan showed significant improvement in exercise capacity and no clinical worsening in the majority of Chinese patients with CTD-PAH in the 24-week treatment period. The AEs observed in the CTD-PAH subgroup were consistent with the known safety profile of ambrisentan in the overall Chinese PAH population. Trial registration ClinicalTrial.gov Identifier, https://clinicaltrials.gov/, NCT01808313 Registration date (first time): February 28, 2013.

Abstract Background The quadricuspid pulmonary valve (QPV) is a relatively rare heart congenital anomaly. It is usually asymptomatic and incidentally detected. Case summary A 52-year-old woman presented with paroxysmal palpitations and chest tightness after exertion. After a series of examinations, we finally diagnosed her with QPV and pulmonary hypertension. The symptoms have improved significantly with medications, and subsequently, the patient was discharged. Discussion This case demonstrated the crucial role of multimodality imaging in evaluating the non-invasive depiction of pulmonary valve disease.

Objective To clarify the value of plasma levels of high sensitivity C reactive protein (hs-CRP), soluble CD40 ligand (sCD40L), monocyte chemoattractant protein-1 (MCP-1), interleukin 8 (IL-8), interleukin 6 (IL-6), P-selectin, tissue-type plasminogen activator (tPA) in detecting the existence of coronary lesions. Materials and methods Total of 170 subjects were enrolled in this study, including 129 cases of confirmed coronary heart disease by coronary angiography from February 2009 to January 2010 at Qilu Hospital of Shandong University, and 41 cases without coronary heart disease as the control group. There were 39 cases of SAP, 43 cases of UAP and 47 cases of AMI. sCD40L, MCP-1, IL-8, IL-6, P-selectin and tPA were measured by flow cytometric method in EDTA anticoagulated blood. Hs-CRP was measured by nephelometry in plasma content. The performance was strictly in accordance with the instructions. Results Plasma levels of the tested biomarkers in each group Concentrations of plasma hs-CRP in SAP patients had no significant changes compared with the control (p=0.085). They were significantly higher in UAP group and AMI group than in the control and SAP (p<0.00001, p<0.00001). Plasma levels of sCD40L and MCP-1 in SAP, UAP and AMI group all were significantly higher than those in control group (p=0.006, p<0.00001, p<0.00001), and they were higher in AMI group than in SAP group (p<0.05). Plasma levels of MCP-1, IL-8, IL-6, P-selectin, tPA in each CHD group was higher than those in control group (all p<0.05). The differences in the three CHD groups were not significant (p=NS). Areas under ROC curve of the six factors were obtained by taking the plasma values of each biomarker in the stable and unstable angina as the test variables, and coronary heart disease as the state variables. The result showed that the areas under ROC curve for sCD40L was 0.743 (p<0.00001), for MCP-1 was 0.674 (p=0.008), for IL-8 was 0.641 (p=0.014), for IL-6 was 0.779 (p<0.00001), for tPA was 0.759 (p<0.00001) and for P-selectin was 0.675 (p=0.003). Univariate binary Logistic regression analysis of each variable for CHD showed that each had predictive value, however, multivariate logistic regression analysis showed only IL-6 had the role of predicting CHD, OR=1.857 (p=0.007). Conclusion The plasma levels of sCD40L, MCP-1, IL-8, IL-6, tPA and P-selectin in CHD can reflect the existence of coronary atherosclerosis. Of the biomarkers, IL-6 had the greatest value for diagnosing CHD and had great clinical significance.