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The pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Histopathologic studies revealed that pulmonary vasculature lesions similar to idiopathic pulmonary arterial hypertension (PAH) existed in CTEPH patients as well. It's well-known that genetic predisposition plays an important role in the mechanism of PAH. So we hypothesized that PAH-causing gene mutation might exist in some CTEPH patients and act as a background to facilitate the development of CTEPH. In this study, we analyzed 7 PAH-causing genes including BMPR2, ACVRL1, ENG, SMAD9, CAV1, KCNK3, and CBLN2 in 49 CTEPH patients and 17 patients recovered from pulmonary embolism (PE) but without pulmonary hypertension(PH). The results showed that the nonsynonymous mutation rate in CTEPH patients is significantly higher than that in PE without PH patients (25 out of 49 (51%) CTEPH patients vs. 3 out of 17 PE without PH patients (18%); p = 0.022). Four CTEPH patients had the same point mutation in ACVRL1 exon 10 (c.1450C>G), a mutation approved to be associated with PH in a previous study. In addition, we identified two CTEPH associated SNPs (rs3739817 and rs55805125). Our results suggest that PAH-causing gene mutation might play an important role in the development of CTEPH.

Background Insulin resistance (IR) plays an important role in the pathophysiology of cardiovascular disease. Recent studies have shown that diabetes mellitus and impaired lipid metabolism are associated with the severity and prognosis of idiopathic pulmonary arterial hypertension (IPAH). However, the relationship between IR and pulmonary hypertension is poorly understood. This study explored the association between four IR indices and IPAH using data from a multicenter cohort. Methods A total of 602 consecutive participants with IPAH were included in this study between January 2015 and December 2022. The metabolic score for IR (METS-IR), triglyceride to high-density lipoprotein cholesterol (TG/HDL-C) ratio, triglyceride and glucose (TyG) index, and triglyceride-glucose-body mass index (TyG-BMI) were used to quantify IR levels in patients with IPAH. The correlation between non-insulin-based IR indices and long-term adverse outcomes was determined using multivariate Cox regression models and restricted cubic splines. Results During a mean of 3.6 years’ follow-up, 214 participants experienced all-cause death or worsening condition. Compared with in low to intermediate-low risk patients, the TG/HDL-C ratio (2.9 ± 1.7 vs. 3.3 ± 2.1, P = 0.003) and METS-IR (34.5 ± 6.7 vs. 36.4 ± 7.5, P < 0.001) were significantly increased in high to intermediate-high risk patients. IR indices correlated with well-validated variables that reflected the severity of IPAH, such as the cardiac index and stroke volume index. Multivariate Cox regression analyses indicated that the TyG-BMI index (hazard ratio [HR] 1.179, 95% confidence interval [CI] 1.020, 1.363 per 1.0-standard deviation [SD] increment, P = 0.026) and METS-IR (HR 1.169, 95% CI 1.016, 1.345 per 1.0-SD increment, P = 0.030) independently predicted adverse outcomes. Addition of the TG/HDL-C ratio and METS-IR significantly improved the reclassification and discrimination ability beyond the European Society of Cardiology (ESC) risk score. Conclusions IR is associated with the severity and long-term prognosis of IPAH. TyG-BMI and METS-IR can independently predict clinical worsening events, while METS-IR also provide incremental predictive performance beyond the ESC risk stratification.

Background Hyperglycemia upon admission is associated with poor prognosis of many cardiovascular diseases. However, the relationship of stress hyperglycemia ratio (SHR), admission blood glucose (ABG), and hemoglobin A1c (HbA1c) with pulmonary hypertension has not been reported. This study aimed to explore the association of hyperglycemia indices with disease severity and long-term adverse outcomes in patients with idiopathic pulmonary arterial hypertension (IPAH). Methods This multi-center cohort study included 625 consecutive patients diagnosed with or treated for IPAH between January 2015 and June 2023. SHR was calculated using the followings: ABG (mmol/L)/(1.59 × HbA1c [%] − 2.59). The primary endpoint was defined as clinical worsening events. Multivariable Cox regression and restricted cubic spline analyses were employed to evaluate the association of SHR, ABG, and HbA1c with endpoint events. The mediating effect of pulmonary hemodynamics was evaluated to investigate the potential mechanism between hyperglycemia and clinical outcomes. Results During a mean follow-up period of 3.8 years, 219 (35.0%) patients experienced all-cause death or clinical worsening events. Hyperglycemia indices correlated with well-validated variables that reflected the severity of IPAH, such as the World Health Organization functional class, 6-min walk distance, and N-terminal pro-brain natriuretic peptide levels. Multivariable Cox regression analyses indicated that SHR (hazard ratio [HR] 1.328, 95% confidence intervals [CI]: 1.185, 1.489 per 0.1-unit increment, P  < 0.001) and ABG (HR 1.317, 95% CI: 1.134, 1.529 per 1.0-unit increment, P  < 0.001) were independent predictors of primary endpoint events. Mediation analysis indicated that pulmonary vascular resistance mediated 5.65% and 14.62% of the associations between SHR and ABG and clinical worsening events, respectively. The addition of SHR significantly improved reclassification, discrimination ability, and model fit beyond the clinical risk prediction model. Conclusions SHR is positively associated with clinical worsening in patients with IPAH. The association appeared to be partially mediated through the pathway of pulmonary vascular remodeling, indicating that SHR may serve as a valuable indicator for providing additional risk information.

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.

Transthoracic echocardiography (TTE), commonly used for initial screening of pulmonary hypertension (PH), often lacks sufficient accuracy. To address this gap, we developed and validated a multimodal fusion model for improved PH screening (MMF-PH). The study was registered in the ClinicalTrials.gov (NCT05566002, 09/30/2022). The MMF-PH underwent extensive training, validation, and testing, including comparisons with TTE and evaluations across various patient subgroups to assess robustness and reliability. We analyzed 2451 patients who underwent right heart catheterization, supplemented by a prospective dataset of 477 patients and an external dataset. The MMF-PH demonstrated robust performance across different datasets. The model outperformed TTE in terms of specificity and negative predictive value across all test datasets. An ablation study using the external test dataset confirmed the essential role of each module in the MMF-PH. The MMF-PH significantly advances PH detection, offering robust and reliable diagnostic accuracy across diverse patient populations and clinical settings.

Pulmonary artery (PA) dilatation is commonly observed in patients with pulmonary hypertension (PH). However, the clinical aspects of PA dilatation in various etiology of PH remain unknown. In this study, we investigated the clinical and imaging characteristics of 1018 patients with different subtypes of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). The independent determinants for all‐cause death were identified using univariate and multivariate Cox proportional hazard models. PA dilatation was identified in 88.8% of the patients, and 27.2% had a PA diameter/ascending aorta diameter ratio greater than 1.5. PA diameter was shown to be significantly correlated with hemodynamic parameters and symptom duration in idiopathic PAH patients. PA diameter only correlated with pulmonary circulation volume in patients with PAH associated with congenital heart disease. PA diameter correlated with symptom duration and right ventricular end‐diastolic dimension in CTEPH patients. PA diameter correlated with right ventricular end‐diastolic dimension in patients with PAH associated with connective tissue disease. Only 6‐min walk distance, but not PA dilatation, predicts all‐cause death independently. In conclusion, PA dilatation is a common finding in PH patients. The clinical feature of PA dilatation varies greatly between PH types. PA dilatation is not associated with all‐cause death.

Purpose Obstructive sleep apnea (OSA) is prevalent in patients with cardiovascular disease (CVD), and is linked to worsened outcomes. Screening tools are essential for early detection and intervention. We aimed to investigate the role of ascending aorta diameter (AAD) obtained from echocardiography in identifying OSA in CVD patients. Methods We examined the correlation between AAD and sleep apnea test parameters in 721 hospitalized CVD (including hypertension, coronary artery disease, chronic heart failure, atrial fibrillation, and pulmonary hypertension) patients. Multivariate analyses were performed on all findings. Receiver operator characteristic curve (ROC) analyses were used to determine the role of AAD in detecting OSA. Results AAD was significantly higher in patients with OSA than those without OSA. AAD significantly correlated with apnea-hypopnea index (AHI) and saturation-related parameters. In comparison, pulmonary artery diameter did not correlate with AHI but had significant negative correlations with the saturation-related parameters. The area under the ROC of AAD for detecting OSA in CVD patients was 0.70 (95% CI, 0.65–0.74; p  < 0.001). In the chronic heart failure subgroup, the area under the curve for AAD was 0.75 (95% CI 0.67–0.82, p  < 0.001). The optimal cut-off value of AAD was 31.5 mm, resulting in a sensitivity of 73%, a specificity of 55% in CVD patients, and 29.5 mm with a sensitivity of 82% and a specificity of 64% in chronic heart failure patients. Conclusions AAD was associated with the severity of OSA. AAD could be a valuable tool for identifying OSA in CVD patients, particularly in patients with chronic heart failure.

The pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Histopathologic studies revealed that pulmonary vasculature lesions similar to idiopathic pulmonary arterial hypertension (PAH) existed in CTEPH patients as well. It's well-known that genetic predisposition plays an important role in the mechanism of PAH. So we hypothesized that PAH-causing gene mutation might exist in some CTEPH patients and act as a background to facilitate the development of CTEPH. In this study, we analyzed 7 PAH-causing genes including BMPR2, ACVRL1, ENG, SMAD9, CAV1, KCNK3, and CBLN2 in 49 CTEPH patients and 17 patients recovered from pulmonary embolism (PE) but without pulmonary hypertension(PH). The results showed that the nonsynonymous mutation rate in CTEPH patients is significantly higher than that in PE without PH patients (25 out of 49 (51%) CTEPH patients vs. 3 out of 17 PE without PH patients (18%); p = 0.022). Four CTEPH patients had the same point mutation in ACVRL1 exon 10 (c.1450C>G), a mutation approved to be associated with PH in a previous study. In addition, we identified two CTEPH associated SNPs (rs3739817 and rs55805125). Our results suggest that PAH-causing gene mutation might play an important role in the development of CTEPH.