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Background Fibrosing mediastinitis (FM) is a rare yet fatal condition, caused by different triggers and frequently culminating in the obstruction of the pulmonary vasculature and airways, often leading to pulmonary hypertension and right heart failure. Percutaneous transluminal pulmonary venoplasty (PTPV) is an emerging treatment for pulmonary vein stenosis (PVS) caused by FM. Our previous study showed as high as 24% of in-stent restenosis (ISR) in FM. However, the predictors of ISR are elusive. Objectives We sought to identify the predictors of ISR in patients with PVS caused by extraluminal compression due to FM. Methods We retrospectively enrolled patients with PVS-FM who underwent PTPV between July 1, 2018, and December 31, 2022. According to ISR status, patients were divided into two groups: the ISR group and the non-ISR group. Baseline characteristics (demographics and lesions) and procedure-related information were abstracted from patient records and analyzed. Univariate and multivariate analyses were performed to determine the predictors of ISR. Results A total of 142 stents were implanted in 134 PVs of 65 patients with PVS-FM. Over a median follow-up of 6.6 (3.4–15.7) months, 61 of 134 PVs suffered from ISR. Multivariate analysis demonstrated a significantly lower risk of ISR in PVs with a larger reference vessel diameter (RVD) (odds ratio (OR): 0.79; 95% confidence interval [CI]: 0.64 to 0.98; P  = 0.032), and stenosis of the corresponding pulmonary artery (Cor-PA) independently increased the risk of restenosis (OR: 3.41; 95% CI: 1.31 to 8.86; P  = 0.012). The cumulative ISR was 6.3%, 21.4%, and 39.2% at the 3-, 6-, and 12-month follow-up, respectively. Conclusion ISR is very high in PVS-FM, which is independently associated with RVD and Cor-PA stenosis. Trail Registration Chinese Clinical Trials Register; No.: ChiCTR2000033153. URL: http://www.chictr.org.cn . Graphical Abstract

Background: Misdiagnosis and underdiagnosis of pulmonary hypertension caused by fibrosing mediastinitis (PH-FM) are considerably prevalent due to unspecific symptoms and as well as the lack of awareness of this fatal disease. Objectives: The aim of this study was to evaluate the diagnostic accuracy of the chest X-ray (CXR) for screening the patients with PH-FM from those with pulmonary hypertension (PH). Design: This was a retrospective observational cohort study. Methods: The patients with suspected PH were recruited between October 2014 and October 2020. All the clinical data and CXR findings were collected. The sensitivity, specificity, and likelihood ratio of the CXR features were calculated. Logistic regression was used to identify the factors associated with the CXR characteristics and FM and to generate a prediction model. Finally, the diagnostic efficiency of the prediction model was evaluated using nomogram and internal validation. Results: The patients with PH-FM (n = 36) and PH caused by the diseases other than FM (PH-non-FM, n = 62) were enrolled. The CXR features, including atelectasis, pleural effusion, consolidation, nodules, calcification, interlobular septal thickening, and interstitial reticulation, were more prevalent in patients with PH-FM than in those with PH-non-FM (all p < 0.05). Atelectasis had a specificity of 97%, a sensitivity of 50%, and a greater accuracy for diagnosing of PH-FM [area under the curve (AUC) = 0.720; 95% CI: 0.634–0.806] than the other factors did. The combination of tuberculosis, natural logarithmic NT-proBNP (lnBNP), atelectasis, pleural effusion, and prominent right heart border constituted a prediction model to distinguish the PH-FM from the PH-non-FM, with a sensitivity of 91.7% and a specificity of 83.9%. The model demonstrated good prediction performance by showing an AUC of 0.922 (95% CI: 0.861–0.983) in the internal validation. Conclusion: In this study, atelectasis was the most specific and accurate CXR characteristic for identifying PH-FM in the PH patients. The combination of atelectasis, pleural effusion, prominent right heart border, tuberculosis, and lnBNP constituted a prediction model that distinguished the PH-FM patients from the PH-non-FM ones with good performance.

The 6‐min walk test (6MWT) has significant prognostic value, but requires long walking distances and lacks evaluation of exercise speed. This study aimed to investigate the clinical utility of a new walk test, the 18‐meter walk test (18MWT), in patients with pulmonary arterial hypertension (PAH) as a complement to the 6MWT. In summary, a total of 117 patients with PAH from January 2018 to December 2022 were included. Spearman correlation, Cox regression, and Kaplan–Meier analysis were utilized to demonstrate the value of 18MWT in predicting disease severity and clinical worsening. The median time to complete the 18MWT was 12.8 s (interquartile range: 11.3–14.6 s). 18MWT completion time showed significant correlations with indicators such as N‐terminal pro‐brain natriuretic peptide and 6MWT distance. Adjusted Cox regression showed 18MWT time remained an independent predictor of clinical worsening (hazard ratio = 1.10; 95% confidence interval: 1.01–1.21; p = 0.026). A simplified risk stratification using WHO functional class, 6MWT distance, 18MWT time and NT‐proBNP was predictive of 1‐year clinical outcome. These results suggest that the 18MWT provides clinicians with an efficient measure that can be used to evaluate the disease severity of PAH patients and to identify those patients at greater risk for future clinical worsening as a complement to the 6MWT.

Pulmonary hypertension (PH) often leads to poor survival outcomes and encompasses diverse subtypes with distinct underlying causes. Specifically, PH resulting from fibrosing mediastinitis (FM‐PH) presents significant diagnostic challenges due to nonspecific symptoms and overlap of clinical characterization with other PH subtypes, leading to frequent misdiagnosis and delayed treatment. Moreover, the complex diagnostic procedures impose a significant burden on FM‐PH patients, many of whom already experience mobility difficulties. This study represents a single test‐based diagnosis of FM‐PH, using the plasma metabolites obtained through ferric particle‐enhanced laser desorption/ionization mass spectrometry analysis. Distinct metabolic alterations in FM‐PH are identified compared to healthy controls and other PH subtypes, achieving an area under the curve (AUC) of 0.987 for FM‐PH diagnosis and 0.728 for differentiating FM‐PH from other subtypes. By addressing existing gaps in diagnostic strategies, this research highlights the potential of metabolic analysis in elucidating the metabolic landscape of PH. Pulmonary hypertension caused by fibrosing mediastinitis has a five‐year survival rate of just 56%. Current diagnostic approaches are complex for patients with mobility limitations and can lead to misdiagnoses due to their nonspecific clinical features. This work presents a straightforward and precise strategy based on metabolic analysis—a single test with an accuracy of 93.6%—to complement existing diagnostic modalities.

Background: Right ventricular function (RVF) is an independent predictor of prognosis for patients undergoing aortic valve replacement: transcatheter aortic valve replacement (TAVR) or surgical aortic valve replacement (SAVR). The effect of transfemoral aortic valve replacement (TF-TAVR) on RVF is uncertain. We aimed to perform a meta-analysis of the effect of TF-TAVR on RVF in patients with aortic stenosis (AS) and compare the effect of TF-TAVR with SAVR. Methods: We searched relevant studies from PubMed, Embase, Cochrane Library databases, and Web of Science. Furthermore, two reviewers (Wang AQ and Cao YS) extracted all relevant data, which were then double checked by another two reviewers (Zhang M and Qi GM). We used the forest plot to present results. Tricuspid annular plane systolic excursion (TAPSE) was the primary outcome. Results: This meta-analysis included 11 studies. There were 353 patients who underwent TF-TAVR, and 358 patients who were subjected to SAVR. There was no significant difference in TAPSE at 1 week and 6 months as well as right ventricular ejection fraction (RVEF) at <2 weeks and 6 months after TF-TAVR. For the SAVR group, TAPSE at 1 week and 3 months as well as fractional area change (FAC) at 3 months post procedure were significantly aggravated, while RVEF did not change significantly. Moreover, TAPSE post-TF-TAVR was significantly improved as compared with post-SAVR. The △TAPSE, the difference between TAPSE post-procedure and TAPSE prior to procedure, was also significantly better in the TF-TAVR group than in the SAVR group. Conclusion: RVF was maintained post TF-TAVR. For SAVR, discrepancy in the measured parameters exists, as reduced TAPSE indicates compromised longitudinal RVF, while insignificant changes in RVEF implicate maintained RVF post procedure. Collectively, our study suggests that the baseline RV dysfunction and the effect of TF-TAVR versus SAVR on longitudinal RVF may influence the selection of aortic valve intervention.

Familial hypercholesterolemia (FH) can lead to premature coronary heart disease. Anticardiolipin antibody may be a contributor for thrombosis. Here, we report an adult with possible FH suffered from premature myocardial infarction that may be triggered by transient increased anticardiolipin antibody. Transient elevated anticardiolipin antibody may be a trigger or biomarker of cardiac thrombotic events in younger atherosclerotic patients.

Lymphomatoid granulomatosis is a rare, vascular‐centric, and vessel‐destroying lymphoproliferative disease that hardly involves the pulmonary arteries. Herein, we report a case with severe right heart failure and pulmonary arterial stenosis caused by pulmonary artery lymphomatoid granulomatosis. This case was diagnosed by percutaneous transluminal pulmonary artery biopsy and was effectively treated with stent implantation and steroid administration.

Background ST‐segment elevation (STE) in lead aVR is a useful tool in recognizing patients with left main or left anterior descending coronary obstruction during acute coronary syndrome (ACS). The prognostic implication of STE in lead aVR on outcomes has not been established. Methods We performed a systematic search for clinical studies about STE in lead aVR in four databases including PubMed, EMBASE, Cochrane Library, and Web of Science. Primary outcome was in‐hospital mortality. Secondary outcomes included in‐hospital (re)infarction, in‐hospital heart failure, and 90‐day mortality. Results We included 7 studies with a total of 7,700 patients. The all‐cause in‐hospital mortality of patients with STE in lead aVR during ACS was significantly higher than that of patients without STE (OR: 4.37, 95% CI 1.63 to 11.68, p = .003). Patients with greater STE (>0.1 mV) in lead aVR had a higher in‐hospital mortality when compared to lower STE (0.05–0.1 mV) (OR: 2.00, 95% CI 1.11–3.60, p = .02), However, STE in aVR was not independently associated with in‐hospital mortality in ACS patients (OR: 2.72, 95% CI 0.85–8.63, p = .09). The incidence of in‐hospital myocardial (re)infarction (OR: 2.77, 95% CI 1.30–5.94, p = .009), in‐hospital heart failure (OR: 2.62, 95% CI 1.06–6.50, p = .04), and 90‐day mortality (OR: 10.19, 95% CI 5.27–19.71, p < .00001) was also noted to be higher in patients STE in lead aVR. Conclusions This contemporary meta‐analysis shows STE in lead aVR is a poor prognostic marker in patients with ACS with higher in‐hospital mortality, reinfarction, heart failure and 90‐day mortality. Greater magnitude of STE portends worse prognosis. Further studies are needed to establish an independent predictive role of STE in aVR for these adverse outcomes.