Changming Xiong

The ratio of tricuspid annular plane systolic excursion to systolic pulmonary artery pressure (TAPSE/sPAP) is a noninvasive surrogate of right ventricular–pulmonary arterial coupling, but its value for dynamic risk stratification in chronic thromboembolic pulmonary hypertension (CTEPH) remains uncertain. This multicenter prospective cohort enrolled adults with CTEPH between 2016 and 2023. Patients were stratified by quartile‐based baseline TAPSE/sPAP cutoffs into < 0.15, 0.15 to 0.30, and > 0.30. The primary outcome was clinical worsening (CW) within 12 months, comprising all‐cause death, hospitalization for worsening pulmonary hypertension, escalation of pulmonary hypertension‐targeted therapy, or a > 30% increase in pulmonary vascular resistance. Associations were examined using Kaplan–Meier, Cox regression, and time‐dependent Cox analyses. Among 949 patients, 868 had follow‐up and 212 (24.4%) experienced CW during a median follow‐up of 10.3 months (interquartile range, 5.4–12.2 months). Lower baseline TAPSE/sPAP was associated with worse functional and hemodynamic profiles, and CW‐free survival differed across strata (log‐rank p = 0.004). Each 0.1‐unit increase in baseline TAPSE/sPAP was associated with lower CW risk (adjusted HR, 0.89; 95% CI, 0.79–0.99; p = 0.039). Among 593 patients with serial measurements, higher time‐updated TAPSE/sPAP (adjusted HR, 0.66; 95% CI, 0.52–0.83; p < 0.001) and greater improvement from baseline (adjusted HR, 0.81; 95% CI, 0.71–0.92; p = 0.002) were associated with lower subsequent CW risk. Lower TAPSE/sPAP identifies greater disease severity and CW risk in CTEPH. Serial TAPSE/sPAP may support noninvasive dynamic risk stratification and residual risk assessment during follow‐up.

Isolated pulmonary vasculitis (IPV) and chronic thromboembolic pulmonary hypertension (CTEPH) are important causes of pulmonary artery stenosis‐related pulmonary hypertension. Differentiating between them remains challenging. Therefore, this study aims to compare the clinical, hemodynamic and imaging profiles of patients diagnosed with IPV and CTEPH by pathological diagnosis, so as to improve the accuracy of clinical diagnosis. Patients with IPV and CTEPH confirmed by pathological diagnosis were retrospectively enrolled. The general information, clinical manifestations, laboratory indicators, imaging features, hemodynamics, and echocardiographic data before and after pulmonary endarterectomy (PEA) were collected. There were no significant differences in general information, comorbidities, and symptoms. Compared with the IPV group, the CTEPH group had worse oxygenation status, hemodynamic conditions and right ventricular function. And those in the IPV group had higher levels of ESR. The imaging manifestations showed that IPV is mainly characterized by thickening of the vessel walls, while the filling defects of the lumen account for the majority of CTEPH. Moreover, echocardiography showed improvement in right ventricular function and hemodynamics after PEA in both groups, remarkably in the CTEPH group. Comprehensive evaluation of laboratory indicators, imaging features, and hemodynamic parameters help improve the accuracy of differential diagnosis between IPV and CTEPH.

Background: Hepatic dysfunction is increasingly recognized as a contributor to adverse outcomes in patients with pulmonary arterial hypertension (PAH). However, current prognostic models for PAH do not incorporate hepatic function parameters. Objectives: To develop and validate a novel risk prediction model that integrates hepatic function indicators with established PAH severity markers, and to compare its performance against the COMPERA 2.0 model. Design: Prospective cohort study. Methods: Three liver fibrosis scores (LFSs)—FIB-4, NFS, and HUI—were evaluated for their prognostic significance. A total of 715 PAH patients from Fuwai Hospital were used to develop the FW-HP model. The model’s predictive performance was assessed and compared with COMPERA 2.0. Risk stratification of the PAH cohort was subsequently conducted based on model scores. Results: All three LFSs were independently associated with mortality in PAH. The FW-HP model, incorporating albumin, total bilirubin, N-terminal pro-B-type natriuretic peptide, and World Health Organization functional class, provided superior risk prediction with area under the receiver operating characteristic curve of 0.86, 0.89, and 0.83 at 1-, 2-, and 3-year intervals, respectively—markedly exceeding COMPERA 2.0. The FW-HP score demonstrated higher net clinical benefit and enabled earlier identification of non-low-risk patients requiring more aggressive management. Conclusions: The FW-HP model offers improved prognostic accuracy by incorporating hepatic function into PAH risk stratification. It represents a practical tool for early risk identification with potential to guide clinical decision-making in PAH patients.

Background: Occlusive pulmonary arterial lesions represent a major technical challenge for balloon pulmonary angioplasty (BPA) in patients with Takayasu arteritis–associated pulmonary artery involvement. However, evidence and procedural guidance regarding BPA for occlusive lesions in this population remain limited. Objectives: This study aimed to summarize our center experience with BPA for occlusive pulmonary arterial lesions in patients with Takayasu arteritis and to identify potential risk factors associated with restenosis after BPA. Design: Retrospective cohort. Methods: We retrospectively enrolled patients with Takayasu arteritis who had occlusive pulmonary arterial lesions. Lesions were morphologically categorized as subtotal or total occlusions. Total occlusions were further subclassified as cone-shaped, pouch-like, or ostial occlusions. Results: We included 66 patients with Takayasu arteritis, in whom 182 occlusive lesions were identified. In terms of locations, most ostial occlusions (56.52%) located lobar, while other kinds of lesions more common located segmentally. Total occlusions more frequently required guide-extension catheter support than subtotal occlusions (70.21% vs. 40.63%, P <0.001). Hard-tip guidewires were required for revascularization in 53.85% of ostial occlusions, compared with only 3.13% of subtotal occlusions. Lesion-level technical success rates among attempted lesions decreased with increasing lesion complexity: 100% for subtotal occlusions, 96.0% for cone-shaped occlusions, 68.4% for pouch-like occlusions, and 43.8% for ostial occlusions. The corresponding recanalization rates among all identified lesions were 78.69%, 88.9%, 52.04%, and 10.14%, respectively. In exploratory univariable Firth penalized logistic regression, irregular anticoagulation was associated with a higher likelihood of restenosis (OR 8.51, 95% CI 1.39–60.91; P=0.021), whereas baseline hemodynamics were not associated with restenosis. Conclusion: The techniques and devices illustrated in this study might guide the BPA performance in addressing occlusive lesions.

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.

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.

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.

Background 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. Methods 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. Results 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% 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) had the potential to predict non-low-risk stratification. Multivariate logistic regression analyses identified RV-SD6 (odds ratio [OR]: 1.02, 95% CI: 1.01–1.03, p = 0.009) and QRS duration (OR: 1.07, 95% CI: 1.00–1.15, p = 0.045) as independent predictors of non-low-risk PcPH. Conclusion Mechanical dyssynchrony presented by RV-SD6 correlates with QRS duration and better predicts risk stratification in PcPH patients without complete bundle branch block.