Takayasu arteritis (TA) is a rare large-vessel vasculitis, and pulmonary artery involvement (PAI) can cause severe stenosis and pulmonary hypertension (PH), markedly impairing exercise capacity and quality of life. Although balloon pulmonary angioplasty (BPA) has emerged as a promising interventional option, its preliminary efficacy and safety in TA-PAI—particularly in patients without PH—remain insufficiently defined. Therefore, this study aimed to evaluate the preliminary safety and differential clinical outcomes of BPA in patients with TA-PAI, stratified by the presence or absence of baseline PH. We retrospectively analyzed TA-PAI patients undergoing BPA. Hemodynamics, N-terminal pro-B-type natriuretic peptide (NT-proBNP), exercise capacity, and Quality of Life (QoL) were assessed pre- and post-BPA. Lesion anatomy and procedural complications were recorded. Statistical analyses compared PH and without PH subgroups. Among 33 TA-PAI patients, including 22 with PH and 11 without PH, BPA significantly reduced mPAP (25.41 ± 8.86 to 22.82 ± 7.94 mmHg, P = 0.005) and Pulmonary Vascular Resistance (PVR) from ( 3.06 [1.37,6.04] to 2.17 [1.35, 4.28] WU, P=0.039) in the overall population. In the PH subgroup, significant hemodynamic and functional improvements were observed, including reduced NT-proBNP (P=0.024), increased 6-minute walk distance (6MWD) (P=0.012), and enhanced QoL (all P <0.001). Conversely, the without PH group showed no significant hemodynamic changes (P > 0.05) but demonstrated modest 6MWD increases and significant QoL improvements. Left pulmonary artery lesions were more prevalent in the PH group (42.0

BACKGROUND:Chronic thromboembolic pulmonary hypertension is characterized by proximal pulmonary artery obstruction and distal microvasculopathy. However, the mechanisms driving this dual-compartment pulmonary vascular remodeling remain unclear. METHODS:Male Sprague-Dawley rats were injected with gelatin sponge combined with SU5416 as a secondary insult. Hemodynamics, echocardiography, and pulmonary vascular remodeling were evaluated to investigate the development of chronic thromboembolic pulmonary hypertension. Single-cell RNA sequencing of rat lung tissue was conducted to elucidate the molecular mechanisms underlying pulmonary vascular remodeling. The results were validated by immunofluorescence and cell-based experiments. RESULTS:The optimal size range of gelatin sponge for large pulmonary artery obstruction was 710 to 1000 µm, which synergized with a low dose of SU5416 (10 mg/kg) to induce significant increases in right ventricular systolic pressure and right ventricular hypertrophy at 5 weeks. The model exhibited persistent elastic pulmonary artery obstruction and remodeling, along with significant wall thickening and muscularization of pulmonary microvessels. Single-cell transcriptomic analysis revealed a significant reduction in microvascular endothelial cells and an increase in smooth muscle cells in the chronic thromboembolic pulmonary hypertension rats. STAT3, EGR1, and HIF1A were identified as key genes driving aberrant smooth muscle cell proliferation. The Sell (encoding L-selectin)-Podxl (encoding PODXL, podocalyxin) ligand-receptor pair was found specifically in diseased rats and mediated immune cell-endothelial cell interactions. L-selectin promoted neutrophil adhesion and dysfunction in pulmonary arterial and microvascular endothelial cells, both of which were reversed by PODXL knockdown. CONCLUSIONS:Our new model recapitulates human chronic thromboembolic pulmonary hypertension pathophysiology and is useful for understanding pulmonary microvasculopathy. Sell-Podxl is a previously unrecognized link between inflammation and vascular remodeling, offering a potential therapeutic target.

Background:The impact of cardiometabolic multimorbidity (CMM), defined as the coexistence of multiple cardiometabolic diseases (CMD), on acute pulmonary embolism (PE) outcomes remains understudied. We investigated the burden of CMM and its association with short-term outcomes, treatment patterns, and potential mediators in PE. Methods:We analyzed 7284 patients with acute PE from a nationwide multicenter prospective registry. CMM was defined as the presence of ≥2 CMDs (hypertension, diabetes, dyslipidemia, pre-existing cardiovascular disease, or stroke). The primary objective was to evaluate the association between CMM and 30-day all-cause death and bleeding events. Cox regression models were used to estimate hazard ratios (HR). Sensitivity and subgroup analyses confirmed the robustness of findings, while causal mediation analysis explored underlying pathways. Results:Overall, 54.2% of patients had ≥1 CMD, and 24.8% had CMM. After multivariable adjustment, CMM was independently associated with increased risks of 30-day all-cause death (HR = 1.28, 95% CI 1.02-1.65) and bleeding (HR = 1.45, 95% CI 1.04-2.01), with mortality rising stepwise with increasing CMD burden. In intermediate-high risk PE, the presence of CMM was linked to lower use of reperfusion therapy. Mediation analysis identified body mass index, blood glucose, and renal function markers as partial drivers of these associations. Conclusion:CMM is prevalent in PE and not only independently increases the risk of 30-day all-cause death and bleeding but also may influence reperfusion treatment choices. Future studies should test adding CMM to risk assessment to improve stratification and decision-making.

Background: Systemic inflammation plays a critical role in the progression of heart failure (HF). Aggregate Index of Systemic Inflammation (AISI) is a new composite biomarker, whose prognostic value in critically ill HF patients are still poorly characterized. Methods: 4, 936 HF patients in MIMIC-IV database were extracted in this retrospective cohort study. AISI was calculated on ICU admission [AISI = (neutrophils×monocytes×platelets)/lymphocytes]. Outcomes were 30-day and 1-year all-cause mortality. Multivariable Cox regression, restricted cubic splines (RCS), time-dependent receiver operating characteristic (ROC) curves, subgroup analysis and causal mediation analysis were used to assess independent prognostic value of AISI and mediation role of heart rate (HR). Findings: Multivariate Cox models revealed that patients who were in the highest AISI quartile (Q4) had a much higher risk of 30-day (HR: 1.47; 95% CI: 1.21-1.78) and 1-year mortality (HR: 1.27; 95% CI: 1.10-1.45). RCS analysis revealed a J-shaped dose-response relationship (P-non-linear≤0.001), with risk nadirs at AISI values of 225.97 (30-day) and 256.64 (1-year). The analysis of ROC indicated the strong acute prognostic performance of AISI. Mediation analysis showed that HR was a significant mediator in the relationship between AISI and survival with mediation of 16.7% and 22.7%, respectively, of the total effect on the 30-day mortality and 1-year mortality. Conclusion: The AISI is a strong and independent predictor of mortality in critically ill patients with HF, characterized by a non-linear, J-shaped association. HR acts as a significant partial mediator in this pathway.

Background Pressure‐wire fractional flow reserve (FFR) can guide balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension, but lesion crossing with a distal wire increases cost and procedural risk. We developed an angiography‐derived physiologic index—pulmonary artery flow reserve (PFR)—computed from selective pulmonary angiography using fluid‐dynamic modeling without distal pressure‐wire advancement. Methods In this prospective observational study, selective pulmonary angiography was acquired in 2 projections for type A (ring‐like), type B (web lesion) and type C (subtotal) pulmonary artery lesions. Proximal pressure (Pa) was measured at the target vessel ostium using a pressure‐sensor catheter; flow velocity was estimated by frame counting; pressure drop (ΔP) was computed by a proprietary computational fluid dynamics approach, and PFR was derived as (Pa − ΔP)/Pa. Pressure‐wire FFR (Pd/Pa) served as the reference standard (FFR ≤0.80). Diagnostic performance was assessed by receiver operating characteristic analysis and agreement by Bland–Altman analysis. Results Fifty‐two vessels in 29 patients were analyzed. Mean FFR and PFR were 0.57 ± 0.23 and 0.58 ± 0.24; 44 vessels had FFR ≤0.80. PFR showed excellent discrimination for FFR ≤0.80 (area under the curve 0.981, 95% CI 0.935–1.000), with sensitivity 94.1% (95% CI 80.9%–98.7%) and specificity 87.5% (95% CI 64.0%–97.0%) at an optimal PFR cutoff of 0.77. Agreement was high (bias 0.0076; 95% limits of agreement −0.1037 to 0.1190), and correlation was strong (r=0.970, P<0.001). At 3‐month follow‐up (n=45 vessels), PFR maintained high performance (area under the curve 0.985; bias 0.0071; r=0.967; all P<0.001). Conclusions Angiography‐derived PFR demonstrated strong agreement with pressure‐wire FFR for physiologic assessment of pulmonary artery stenoses in chronic thromboembolic pulmonary hypertension and may provide a simpler alternative to distal pressure‐wire measurements to support physiology‐guided balloon pulmonary angioplasty.

BACKGROUND:Two-dimensional (2D) speckle-tracking echocardiography (STE) for fibrosis detection has been validated against cardiac magnetic resonance late gadolinium enhancement (CMR-LGE) in hypertrophic cardiomyopathy (HCM), whereas 3-dimensional speckle-tracking echocardiography (3D-STE) may overcome limitations of 2D imaging. OBJECTIVES:The authors aimed to evaluate whether 3D-STE can quantify replacement fibrosis as a surrogate for CMR-LGE. METHODS:Two-dimensional speckle-tracking echocardiography, 3D-STE, and the extent of late gadolinium enhancement (%LGE) were prospectively measured in patients with HCM within ≤72 hours. RESULTS:Among 511 patients with HCM (age 49.0 ± 14.3 years; men 340/511, 66.5%), strain parameters were significantly lower in patients with HCM and LGE [LGE (+)] than those in patients without LGE [LGE (-)]. Three-dimensional global longitudinal strain (GLS-3D) correlated moderately with GLS-2D (r = 0.622; 95% CI: 0.565-0.672; P < 0.001) and most strongly with %LGE (r = -0.698; 95% CI: -0.741 to -0.649; P < 0.001). A quadratic model explained the relationship between GLS-3D and %LGE better than the linear model (R2 = 0.448 vs 0.381). GLS-3D was independently associated with extensive LGE (%LGE ≥15%) (OR = 0.629; 95% CI: 0.553-0.716; P < 0.001), and showed the highest AUC for detecting extensive LGE (AUC = 0.894; 95% CI: 0.854-0.934; P < 0.001). At a cutoff value of 15.25%, GLS-3D yielded 87.3% sensitivity (95% CI: 75.5%-94.7%) and 78.3% specificity (95% CI: 74.4-82.2%). CONCLUSIONS:GLS-3D showed the strongest correlation with %LGE among strain parameters and may serve as a surrogate marker for CMR-LGE in HCM.

Background: The six-minute walk test (6MWT) is a simpler, safer, and more cost-effective measure of functional capacity than cardiopulmonary exercise testing (CPET) and can be used for risk stratification of several cardiovascular diseases. However, there are no data on the prognostic value of the 6MWT in hypertrophic cardiomyopathy (HCM). Objective: To investigate the prognostic value of the 6MWT in HCM and determine whether it can be used as an alternative to CPET. Methods: A total of 534 patients with HCM were prospectively recruited to undergo both 6MWT and CPET. The endpoint of this study was major adverse cardiovascular events (MACE, a composite of cardiovascular death, heart failure, aborted sudden cardiac death, and stroke). The associations of the 6MWT with MACE were investigated using survival analyses, and the ability of 6MWT and CPET parameters to predict MACE were compared. Results: Four hundred and ninety-six patients with HCM who had complete follow-up data available were included. The mean age was 46.8 years, and 69.1% were male. The 6MWT distance correlated significantly with peak VO 2 on CPET (r=0.328, P<0.001). Thirty-eight patients had MACE during a median follow-up of 2.7 years. A shorter 6MWT distance was independently associated with a higher risk of MACE (per 100-m decrease, hazard ratio 1.755, P=0.003) after adjusting for covariables. The association between peak VO 2 and MACE was also determined (per 1 mL/min/kg decrease, hazard ratio 1.102, P=0.044). The ability of the 6MWT distance to predict MACE was not significantly different from that of peak VO 2 (C-statistic, 0.643 vs 0.616). The optimal cutoff value of the 6MWT distance was identified as 423 m. Addition of the 6MWT distance (as a dichotomous variable) to the adjusted Cox model with clinical and demographic covariables significantly improved the ability of the model to predict MACE (C-statistic, 0.791 vs. 0.731, P=0.038), while addition of peak VO 2 did not improve the prediction performance of the model (C-statistic, 0.754 vs. 0.731, P=0.346). Conclusions: Poor performance on the 6MWT was an independent risk factor for MACE in patients with HCM. The 6MWT distance provided additional prognostic discrimination beyond conventional covariables when compared with peak VO 2 . These findings suggest that the 6MWT not only serves as an alternative to CPET but has greater clinical value, highlighting the importance of routine 6MWT to assess functional capacity in patients with HCM.

BACKGROUND:The 6-minute walk test (6MWT) is a practical and cost-effective measure of functional capacity in cardiovascular diseases, yet its prognostic utility in hypertrophic cardiomyopathy (HCM) remains unknown. In this study we aimed to evaluate the prognostic value of the 6MWT and compare it with cardiopulmonary exercise testing (CPET) in HCM. METHODS:A total of 496 patients with HCM who underwent both 6MWT and CPET were included in this study. The endpoint was major adverse cardiovascular events (MACEs). RESULTS:During a median follow-up of 2.7 years, 38 patients developed MACEs. The 6MWT distance was an independent prognostic factor for MACEs in HCM (per 100-m decrease, adjusted hazard ratio [HR] 1.646, P = 0.006), and it correlated significantly with CPET parameters such as peak oxygen consumption (V˙O2; r = 0.328, P < 0.001). The ability of the 6MWT distance to predict MACEs was comparable with peak V˙O2 (C-statistic = 0.643 vs 0.616), with 423 m identified as the optimal cutoff value. Adding the 6MWT distance to the adjusted Cox model significantly improved predictive performance (C-statistic = 0.791 vs 0.731, P = 0.038), whereas adding peak V˙O2 did not (C-statistic = 0.754 vs 0.731, P = 0.346). The 6MWT distance remained an independent predictor of MACEs in patients at increased risk of adverse events during CPET (per 100-m decrease, adjusted HR 1.848, P = 0.037). CONCLUSIONS:The 6MWT serves as a practical and cost-effective alternative to CPET for risk stratification in HCM, particularly when CPET is unavailable or poses risks.

BACKGROUND:Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe condition resulting from unresolved thrombi in the pulmonary arteries, leading to increased pulmonary vascular resistance and right heart failure. Currently, the scarcity of clinically relevant animal models of CTEPH significantly hampers mechanistic studies and drug development. METHODS:This study aimed to establish a rat model of CTEPH by combining splenectomy with thrombus injection, simulating key clinical risk factors associated with the disease. Rats underwent splenectomy and subsequent intravenous administration of thrombi, followed by hemodynamic and histological measurements as well as lung tissue RNA sequencing. RESULTS:Splenectomized rats exhibited significant increases in platelets and delayed thrombolysis. Five weeks after splenectomy and thrombus injection, the rats exhibited thrombus retention in large pulmonary arteries, increased right ventricular systolic pressure, and pulmonary vascular remodeling, which were characteristic of CTEPH. Transcriptomic analysis revealed increased expression of inflammatory cytokines Ccl2 and Ccl3, as well as the B cell marker Cd79a, which was confirmed as an increase in CD79A+ B cells in the lung tissue. CONCLUSIONS:Overall, this novel approach of combining splenectomy with thrombus injection provides a clinically relevant model for studying CTEPH pathophysiology and evaluating potential therapeutic interventions.

Background: Hypertrophic cardiomyopathy (HCM) lacks targeted pharmacotherapy. We employed RNA-seq of human HCM myocardium combined with Connectivity Map screening to identify repurposable drugs modulating hypertrophy-associated pathways. Hypothesis: L-canavanine(LCA), identified through HCM transcriptome screening, directly binds CDC42 to inhibit GTP-loading and downstream JNK signaling, thereby reversing pathological hypertrophy. Goals/Aims (1) Determine in vivo therapeutic efficacy of LCA in pressure-overload cardiac hypertrophy (2) Elucidate direct molecular target and binding mechanism of LCA (3) Delineate the CDC42-JNK signaling pathway in hypertrophy regression Methods: We identified L-canavanine (LCA) through Connectivity Map analysis of dysregulated pathways in human hypertrophic cardiomyopathy (HCM) myocardium (RNA-seq; n=15 HCM vs. 8 controls). In vivo , C57BL/6J mice undergoing transverse aortic constriction (TAC) were randomized to sham, vehicle, or LCA treatment (4 weeks; n=6/group). Cardiac hypertrophy was monitored by serial echocardiography and validated by histology (fibrosis/cross-sectional area). In vitro , target engagement was confirmed by cellular thermal shift assay (CETSA) coupled with surface plasmon resonance and mutagenesis. CDC42-GTP activity and JNK signaling were assessed in human HCM samples and phenylephrine-stimulated cardiomyocytes. Rescue experiments employed CDC42 overexpression and JNK activation to investigate underlying mechanisms. Results: L-canavanine ameliorated pressure overload-induced cardiac pathology in mice, reducing left ventricular hypertrophy (LVPWd), myocardial fibrosis, and cardiomyocyte size post-TAC. LCA directly bound CDC42 with high affinity (SPR), stabilizing its structure (CETSA). Critical CDC42 mutations (Thr3Ala/Pro73Ala/Gln74Ala/Asp76Ala) abolished binding. Mechanistically, LCA suppressed CDC42-JNK signaling, decreasing GTP-CDC42 and JNK phosphorylation (p-JNK/JNK). Rescue experiments confirmed causality: CDC42 overexpression reversed LCA-mediated JNK inhibition, and JNK activation (anisomycin) blocked LCA's anti-hypertrophic effects.Thus, LCA attenuates cardiac remodeling via direct CDC42 targeting and JNK pathway disruption. Conclusion: LCA ameliorates pathological hypertrophy by binding CDC42 at Thr3/Pro73/Gln74/Asp76, inhibiting GTP loading and JNK signaling. This establishes CDC42 as a novel therapeutic target for HCM.

BACKGROUND: Hypertrophic cardiomyopathy (HCM), affecting approximately 1 in 500 individuals globally, is a leading cause of sudden cardiac death in young adults. Current treatments are largely symptomatic and fail to alter disease progression. This study addresses the critical need for disease-modifying interventions by elucidating molecular mechanisms underlying HCM and identifying therapeutic candidates through a drug repurposing strategy. METHODS: We employed a systems biology framework, integrating transcriptomic profiles from human HCM cardiac tissue with drug-response signatures from the Connectivity Map (CMAP) database to predict compounds capable of reversing disease-associated gene expression patterns. Atovaquone emerged as a top candidate. Its therapeutic efficacy was evaluated using in vitro and in vivo models, including established genetic HCM mouse models and cardiomyocyte hypertrophy assays. Target deconvolution and mechanistic analyses were conducted to define the molecular basis of its action. RESULTS: Atovaquone markedly ameliorated pathological features in HCM models, including reduced left ventricular wall thickness, heart weight-to-body weight ratios, myocardial fibrosis, cardiomyocyte size, and expression of hypertrophy markers (Nppa/Nppb). CETSA-based thermal proteome profiling identified the membrane-associated E3 ubiquitin ligase MARCH3 as a direct target of Atovaquone. This interaction was supported by molecular docking and validated by CETSA and DARTS assays. Functional studies demonstrated that MARCH3 knockdown abrogated Atovaquone’s anti-hypertrophic effects in phenylephrine-stimulated neonatal rat ventricular myocytes, evidenced by increased hypertrophic gene expression and cell size. Mechanistically, Atovaquone enhanced MARCH3-mediated polyubiquitination of GP130, facilitating its lysosomal degradation and suppressing STAT3 phosphorylation at Y705 and S727. The use of leupeptin, but not MG132, reversed GP130 downregulation, confirming lysosomal pathway specificity. CONCLUSION: This study identifies Atovaquone as a repurposed, disease-modifying candidate for HCM therapy. By targeting MARCH3 and inducing lysosomal degradation of GP130, it disrupts pathological STAT3 signaling. These findings provide both mechanistic insight and translational potential for a novel therapeutic approach in HCM.

Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease, often caused by sarcomere gene mutations, though many sporadic cases remain genetically unexplained. Here we show that the somatic variant NAP1L1 p.D349E was involved in cardiac hypertrophy in sporadic HCM patients. Through next generation sequencing, we found that somatic variant NAP1L1 p.D349E was recurrent in the cardiomyocytes of gene-elusive sporadic HCM patients. Subsequent in vivo and in vitro functional analysis confirmed that NAP1L1 p.D349E contributes to HCM by triggering an innate immunity response. This mutation destabilizes nucleosome formation, causing DNA to leak into the cytoplasm. This leakage activates a key immune pathway, cGAS-STING, which leads to the release of inflammatory molecules and promotes heart muscle thickening. Our findings reveal a new mechanism driving HCM and suggest that somatic variants could be important in understanding and management of HCM.

Most genome-wide association studies (GWAS) of Venous Thromboembolism (VTE) have used data from individuals of European descent, however, genetic factors for VTE have not been fully identified in Chinese populations, which causes the limited use of existing polygenic risk scores (PRS) to identify subpopulations at high risk of VTE for prevention. We, therefore, aimed to curate all the potential VTE-related single-nucleotide polymorphisms (SNPs) for the construction of a new improved PRS model based on the self-adapting method, and then evaluate its utility and effectiveness in the stratification of VTE risk in Chinese populations. We comprehensively analyzed the mutation spectrum of VTE-associated SNPs in the Chinese cohort, and ranked their individual risk effects independently using risk ratio, logistic regression coefficient, and penalty regression coefficient as evaluation criteria. By integrating various algorithms and evaluating their performance, we trained the optimal prediction model of VTE risk in the Chinese population with the least SNP features, established an adaptive PRS model with progressive SNP overlay, and tested it on an independent Chinese population cohort. Self-adaptive polygenic risk score model based on all 318 SNPs or on the 44 most strongly associated SNPs performed similarly (areas under receiver-operating characteristic curves (AUCs) of 0.739 and 0.709, respectively) on the testing dataset of the Chinese VTE cohort, and that achieve the overall best level of the AUC from a conventional PRS model based on known genetic risk factors (0.620–0.718). In addition, we observed the self-adaptive PRS model was an independent effective risk stratification indicator beyond other clinical characteristics including age and smoking status. Our data revealed that only 44 SNPs-derived PRS model can be effectively used in discriminating subpopulations at high risk of VTE. To become clinically useful, our model could benefit from a practically feasible VTE screening program for precision prevention in Chinese populations.

Background Left ventricular (LV) trabeculation are increasingly observed in patients with hypertrophic cardiomyopathy (HCM), but its clinical significance remains controversial. This study aims to clarify the characteristics of LV trabeculation and evaluate its prognostic value in HCM. Methods We evaluated 1028 patients with HCM undergoing cardiac magnetic resonance. For each patient, thickness of compacted and trabeculated myocardium was measured at 16 segments of LV. The extent of LV trabeculation was expressed as the maximal trabeculation/compaction (T/C) ratio in any of 16 segments. The primary endpoint was major adverse cardiovascular events (MACEs). The secondary endpoints were heart failure, thromboembolic events, and ventricular arrhythmias. There were 689 patients undergoing whole-exome sequencing. Results LV trabeculation predominantly located in midventricular-to-apical area, anterior, and lateral free walls in HCM. A greater extent of LV trabeculation was correlated with a higher prevalence of female, lower LV ejection fraction, and higher prevalence of extensive late gadolinium enhancement. During a median 4.8-year follow-up, a greater extent of LV trabeculation was associated with an increased risk of MACEs (adjusted hazard ratio [HR] 1.214, P = 0.005), heart failure (adjusted HR 1.372, P = 0.006), thromboembolic events (adjusted HR 1.242, P = 0.032), and ventricular arrhythmias (adjusted HR 1.240, P = 0.047). No gene was significantly associated with trabeculation at genome-wide level. Conclusions The distribution pattern of LV trabeculation was inhomogeneous and asymmetric in HCM. A greater extent of LV trabeculation was associated with poor prognosis. The progression of LV trabeculation might be the natural course of HCM. Graphical Abstract The distribution pattern of LV trabeculation in patients with HCM and the association between the extent of LV trabeculation and poor prognosis. CMR, cardiac magnetic resonance; HCM, hypertrophic cardiomyopathy; MACEs, major adverse cardiovascular events; LGE, late gadolinium enhancement; LV, left ventricular; LVEF, LV ejection fraction; WES, whole-exome sequencing

Balloon pulmonary angioplasty (BPA) is now a widely accepted treatment for inoperable chronic thromboembolic pulmonary hypertension (CTEPH), but it still faces the problem of high complications. Herein, we report a rare case of severe vagal response during the BPA of a total occlusion lesion in a patient with CTEPH. The patient experienced acute chest pain and dyspnea, accompanied by a significant decrease in heart rate and blood pressure. After administering atropine, the patient's symptoms rapidly subsided. This case highlights the potential for vascular and pleural injury with chest pain, which can lead to severe vagal response during the BPA procedures. To minimize this risk, avoiding guide wire-induced injury to the pleura situated distal to the target vessel is crucial.

BackgroundThe public health burden of cardiomyopathies and competency in their management by health agencies in China are not well understood.MethodsThis study adopted a multi-stage sampling method for hospital selection. In the first stage, nationwide tertiary hospital recruitment was performed. As a result, 88 hospitals with the consent of the director of cardiology and access to an established electronic medical records system, were recruited. In the second stage, we sampled 66 hospitals within each geographic-economic stratification through a random sampling process. Data on (1) the outpatient and inpatient visits for cardiomyopathies between 2017 and 2021 and (2) the competency in the management of patients with cardiomyopathies, were collected. The competency of a hospital to provide cardiomyopathy care was evaluated using a specifically devised scale.FindingsThe outpatient and inpatient visits for cardiomyopathies increased between 2017 and 2021 by 38.6% and 33.0%, respectively. Most hospitals had basic facilities for cardiomyopathy assessment. However, access to more complex procedures was limited, and the integrated management pathway needs improvement. Only 4 (6.1%) of the 66 participating hospitals met the criteria for being designated as a comprehensive cardiomyopathy center, and only 29 (43.9%) could be classified as a primary cardiomyopathy center. There were significant variations in competency between hospitals with different administrative and economic levels.InterpretationThe health burden of cardiomyopathies has increased significantly between 2017 and 2021 in China. Although most tertiary hospitals in China can offer basic cardiomyopathy care, more advanced facilities are not yet universally available. Moreover, inconsistencies in the management of cardiomyopathies across hospitals due to differing administrative and economic levels warrants a review of the nation allocation of medical resources.FundingThis work was supported by the Chinese Academy of Medical Sciences (CAMS) Innovation Fund for Medical Sciences (2023-I2M-1-001) and the National High Level Hospital Clinical Research Funding (2022-GSP-GG-17).