中华心血管病杂志最新文献

{"title":"[A 14-year management of an early-onset female Danon disease carrier: analysis of family clinical phenotype and transplant efficacy].","authors":"H Z Zhao, Y Sun, Z H Zhang, Z Zhou, K L Yin","doi":"10.3760/cma.j.cn112148-20250813-00574","DOIUrl":"10.3760/cma.j.cn112148-20250813-00574","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the clinical characteristics, disease progression pattern, genetic variant features, and therapeutic effect of heart transplantation in a family with Danon disease. <b>Methods:</b> This study is a single-center retrospective case analysis. A proband with Danon disease who was diagnosed and treated at Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College between January 2009 and July 2024 and underwent heart transplantation was selected. Retrospective data were collected, including medical history, imaging findings (echocardiography, cardiac magnetic resonance), electrocardiogram, laboratory indicators, pathological examination results, and genetic testing results of the proband and her family members. Regular follow-up was conducted to evaluate the patient's disease progression and post-transplant recovery. <b>Results:</b> The proband was a 23-year-old female with an onset age of 7 years. Initially, the patient presented with non-obstructive hypertrophic cardiomyopathy (maximum left ventricular wall thickness: 29 mm) accompanied by reduced diastolic function. At 19 years old, the disease progressed to dilated cardiomyopathy (left ventricular end-diastolic diameter: 61 mm; left ventricular ejection fraction: 35%). At 22 years old, she was diagnosed with end-stage heart failure (cardiac index: 2.4 L·min^-1·m^-2). Genetic testing revealed that the patient carried a pathogenic heterozygous variant of the LAMP2 gene c.459C>A (p.Cys153Ter). She underwent orthotopic heart transplantation at 22 years old, and during 3 years of post-transplant follow-up, her cardiac function remained normal (left ventricular ejection fraction >60%). The patient's mother carried the same genetic variant and underwent heart transplantation at 29 years old. <b>Conclusion:</b> Despite being female, the Danon disease patient in this case exhibited clinical features of rapidly progressive cardiomyopathy. Heart transplantation is an effective treatment for patients with end-stage Danon disease, with significant long-term therapeutic effects confirmed by post-transplant follow-up. Clinicians should be alert to the possibility of Danon disease in young patients with hypertrophic cardiomyopathy. Early genetic testing helps confirm the diagnosis and provides a basis for clinical intervention.</p>","PeriodicalId":38755,"journal":{"name":"中华心血管病杂志","volume":"54 2","pages":"190-197"},"PeriodicalIF":0.0,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146195833","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Molecular mechanisms of the TRPV4-RhoA/ROCK1 signaling axis in mediating mechanical stress-induced myocardial fibrosis in fetuses with tetralogy of Fallot].","authors":"Y Luo, G Luo, S B Wang, Y Q Tang, S L Pan","doi":"10.3760/cma.j.cn112148-20250228-00149","DOIUrl":"10.3760/cma.j.cn112148-20250228-00149","url":null,"abstract":"&lt;p&gt;&lt;p&gt;&lt;b&gt;Objective:&lt;/b&gt; To explore the role and underlying mechanisms of transient receptor potential vanilloid 4 (TRPV4) in pressure overload-induced myocardial fibrosis in fetal tetralogy of Fallot (TOF). &lt;b&gt;Methods:&lt;/b&gt; Fetal cardiac samples diagnosed by prenatal echocardiography or postmortem examination were retrospectively collected from Qingdao University Affiliated Women and Children's Hospital between May 2022 and July 2024, including 4 cases in the Tetralogy of Fallot (TOF) group and 4 cases in the structurally normal heart control group. Histological analysis of myocardial hypertrophy, fibrosis, and collagen content was performed on collected right ventricular wall tissues using hematoxylin-eosin (HE), Masson＇s trichrome, and Sirius red staining. Immunohistochemistry was used to detect the expression levels of TRPV4, Ras homolog gene family member A (RhoA), and Rho-associated coiled-coil containing protein kinase 1 (ROCK1) in myocardial tissues, while Western blot was applied to measure the expression of Collagen Ⅰ, Collagen Ⅲ, and α-smooth muscle actin (α-SMA). In vitro, human umbilical vein endothelial cells (HUVEC) at passages 3 to 6 were seeded on hydrogel-coated plates with different stiffnesses (8 kPa, 50 kPa) and divided into 8 kPa, 50 kPa, and 50 kPa+TRPV4 inhibitor (pretreated with a TRPV4 antagonist) groups. Small interfering RNA targeting TRPV4 (siTRPV4) and negative control siRNA (siNC) were transfected into HUVECs to establish TRPV4-knockdown models. These cells were then seeded on hydrogel plates of varying stiffness and divided into 8 kPa, 50 kPa, siNC, and siTRPV4 groups. Western blot was used to assess the expression levels of TRPV4, RhoA, ROCK1, Collagen Ⅰ, Collagen Ⅲ, α-SMA, CD31, vascular endothelial cadherin (VE-cadherin), and endothelial-mesenchymal transition (EndMT)-related regulatory proteins (p-Smad2 and the transcription factor Slug). &lt;b&gt;Results:&lt;/b&gt; HE staining revealed cardiomyocyte hypertrophy in the right ventricular wall of the TOF group compared with the control group, accompanied by varying degrees of myocardial disarray. Masson＇s trichrome staining indicated a higher percentage of fibrotic area in the right ventricular myocardium of the TOF group, and Sirius red staining showed a larger collagen deposition area (all &lt;i&gt;P&lt;/i&gt;&lt;0.05). Compared with the control group, the TOF group exhibited significantly higher protein expression of Collagen Ⅰ, Collagen Ⅲ, and α-SMA, along with enhanced positive signal intensity for TRPV4, RhoA, and ROCK1 (all &lt;i&gt;P&lt;/i&gt;&lt;0.05). In vitro experiments, compared with the 50 kPa group, both the 8 kPa group and the 50 kPa+TRPV4 inhibitor group had lower expression levels of Collagen Ⅰ, Collagen Ⅲ, α-SMA, p-Smad2, Slug, TRPV4, RhoA, and ROCK1, while expression levels of CD31 and VE-cadherin were higher (all &lt;i&gt;P&lt;/i&gt;&lt;0.05). Similarly, the siTRPV4 group showed lower expression of Collagen Ⅰ, Collagen Ⅲ, α-SMA, TRPV4, RhoA, ROCK1, Slug and p-Smad2, and higher expression of CD31 and ","PeriodicalId":38755,"journal":{"name":"中华心血管病杂志","volume":"54 2","pages":"204-212"},"PeriodicalIF":0.0,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146195910","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Application of fundus imaging in the diagnosis and treatment of cardiovascular disease comorbid with mental disorders].","authors":"X He, C Li, L Liu, X H Yang","doi":"10.3760/cma.j.cn112148-20260101-00001","DOIUrl":"10.3760/cma.j.cn112148-20260101-00001","url":null,"abstract":"","PeriodicalId":38755,"journal":{"name":"中华心血管病杂志","volume":"54 2","pages":"229-233"},"PeriodicalIF":0.0,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146195935","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Role of glucose transporter 4 in the pathogenesis of diabetic cardiomyopathy].","authors":"Aliyanmu Aheniyazi, F Liu, H Y Jiang, Y N Yang","doi":"10.3760/cma.j.cn112148-20251101-00770","DOIUrl":"10.3760/cma.j.cn112148-20251101-00770","url":null,"abstract":"","PeriodicalId":38755,"journal":{"name":"中华心血管病杂志","volume":"54 2","pages":"244-248"},"PeriodicalIF":0.0,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146195457","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Development of a deep learning model for predicting adverse cardiovascular events in patients with acute coronary syndrome based on retinal fundus images].","authors":"Y D Ding, R X Feng, J Y Han, L Lin, Y Zhang, Z H Zhao, W J Wang, T X Zhao, S J Zhou, J H Xiong, Y Zeng","doi":"10.3760/cma.j.cn112148-20251009-00704","DOIUrl":"10.3760/cma.j.cn112148-20251009-00704","url":null,"abstract":"<p><p><b>Objective:</b> To develop and validate a deep learning model based on retinal fundus images for predicting the risk of long-term adverse cardiovascular events in patients with acute coronary syndrome (ACS). <b>Methods:</b> This multicenter diagnostic study enrolled ACS patients from July 2021 to July 2023 at Beijing Anzhen Hospital, Beijing Daxing Hospital, Beijing Miyun Hospital, and the Second Affiliated Hospital of Hainan Medical University. Retinal fundus images were acquired. Patients underwent follow-up, with the primary endpoint defined as major adverse cardiovascular and cerebrovascular events (MACCE). Patients were divided into a development set and an external test set. A multimodal deep learning model based on Inception-Resnet-V2 was constructed. Predictive performance for MACCE was evaluated using the area under the receiver operating characteristic curve (<i>AUC</i>). Patients were further stratified into low-and high-risk groups according to the optimal cutoff value derived from the receiver operating characteristic curve, and Kaplan-Meier survival curves were generated. Class activation mapping was used to analyze the retinal regions the model focused on during decision-making. <b>Results:</b> A total of 4 703 ACS patients were included, with a median age of 59 years and 3 504 (74.51%) males. The development set comprised 1 521 patients, and the external validation set included 3 182 patients. The maximum follow-up duration was 883 days. The MACCE incidence rate was 27.42% (417/1 521) in the development cohort and 10.03% (319/3 182) in the external validation cohort. The model's <i>AUC</i> for predicting MACCE was 0.709 (95%<i>CI</i> 0.676-0.742) in the development cohort and 0.644 (95%<i>CI</i> 0.613-0.675) in the external validation cohort. Kaplan-Meier survival curves demonstrated that the MACCE incidence was higher in the high-risk group than in the low-risk group in both the development and external validation cohorts (<i>P</i><0.05 for log-rank tests). Class activation mapping analysis revealed that the model focused on retinal vascular regions during decision-making. <b>Conclusions:</b> A deep learning model based on retinal fundus images effectively predicts long-term adverse cardiovascular event risk in ACS patients, demonstrating robust risk stratification capability and clinical interpretability. It holds promise as a noninvasive risk assessment tool.</p>","PeriodicalId":38755,"journal":{"name":"中华心血管病杂志","volume":"54 2","pages":"172-178"},"PeriodicalIF":0.0,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146195941","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Research progress on acute left ventricular apical ballooning caused by left ventricular outflow tract obstruction with obstructive hypertrophic cardiomyopathy].","authors":"C Z Shen, X F Ouyang, Q You","doi":"10.3760/cma.j.cn112148-20251113-00800","DOIUrl":"10.3760/cma.j.cn112148-20251113-00800","url":null,"abstract":"","PeriodicalId":38755,"journal":{"name":"中华心血管病杂志","volume":"54 2","pages":"238-243"},"PeriodicalIF":0.0,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146194700","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Progress in the study of poor response to proprotein convertase subtilisin/kexin type 9 monoclonal antibodies].","authors":"Z X Guo, Y Zhang, C Y Gao","doi":"10.3760/cma.j.cn112148-20251202-00846","DOIUrl":"10.3760/cma.j.cn112148-20251202-00846","url":null,"abstract":"","PeriodicalId":38755,"journal":{"name":"中华心血管病杂志","volume":"54 2","pages":"234-237"},"PeriodicalIF":0.0,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146195926","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Large-scale electrocardiogram and imaging models in cardiovascular diseases: applications and challenges].","authors":"B Chen, X J Lin, P Zhang","doi":"10.3760/cma.j.cn112148-20251223-00901","DOIUrl":"10.3760/cma.j.cn112148-20251223-00901","url":null,"abstract":"","PeriodicalId":38755,"journal":{"name":"中华心血管病杂志","volume":"54 2","pages":"127-133"},"PeriodicalIF":0.0,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146195932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Cardiovascular age prediction based on large language models and prognostic value analysis in acute coronary syndrome patients].","authors":"W F Liu, Y X Duan, Z Z Qi, K X Wang, M H Qiu, J Li, Y Li, Y L Han","doi":"10.3760/cma.j.cn112148-20251127-00831","DOIUrl":"10.3760/cma.j.cn112148-20251127-00831","url":null,"abstract":"&lt;p&gt;&lt;p&gt;&lt;b&gt;Objective:&lt;/b&gt; To investigate the application value of cardiovascular age prediction based on large language model for prognostic assessment in patients with acute coronary syndrome (ACS). &lt;b&gt;Methods:&lt;/b&gt; This retrospective cohort study included 47 249 ACS patients who were admitted to the Department of Cardiology at the General Hospital of Northern Theater Command and underwent percutaneous coronary intervention between November 2015 and May 2023. Cardiovascular age was predicted using a large language model for cardiovascular critical care developed by our institution, incorporating patients' demographic characteristics, medical history, and laboratory data. The difference between cardiovascular age and chronological age was calculated as the Age-Gap. Based on previous studies, patients were categorized into the high Age-Gap group (Age-Gap≥8 years) and the low Age-Gap group (Age-Gap&lt;8 years). The primary endpoint was major adverse cardiovascular and cerebrovascular events (MACCE), including cardiac death, myocardial infarction, stroke, and target vessel revascularization. Secondary endpoints included the individual components of MACCE and all-cause mortality. Pearson correlation analysis was used to assess the correlation between cardiovascular age and chronological age. Cox proportional hazards models were used to evaluate the association between Age-Gap and clinical outcomes, and restricted cubic spline analyses were applied to explore potential nonlinear relationships between Age-Gap and study endpoints. &lt;b&gt;Results:&lt;/b&gt; The chronological age was (61.1±10.4) years, the cardiovascular age was (69.5±10.8) years. There were 31 775 patients in the high Age-Gap group and 15 474 in the low Age-Gap group. Cardiovascular age was positively correlated with chronological age (&lt;i&gt;r&lt;/i&gt;=0.87, &lt;i&gt;P&lt;/i&gt;&lt;0.000 1). The follow-up duration was 39.6 (24.0, 59.6) months. The incidence of MACCE was significantly higher in the high Age-Gap group than that in the low Age-Gap group (12.99% (4 127/31 775) vs. 7.86% (1 216/15 474), &lt;i&gt;P&lt;/i&gt;&lt;0.000 1). After adjustment for age, sex, smoking status, and other confounders, Cox regression analysis showed that patients in the high Age-Gap group had a significantly increased risk of MACCE compared with those in the low Age-Gap group (&lt;i&gt;HR&lt;/i&gt;=1.47, 95%&lt;i&gt;CI&lt;/i&gt; 1.37-1.58, &lt;i&gt;P&lt;/i&gt;&lt;0.000 1). Higher risks were also observed for cardiac death (&lt;i&gt;HR&lt;/i&gt;=2.72, 95%&lt;i&gt;CI&lt;/i&gt; 2.36-3.15, &lt;i&gt;P&lt;/i&gt;&lt;0.000 1), myocardial infarction (&lt;i&gt;HR&lt;/i&gt;=1.77, 95%&lt;i&gt;CI&lt;/i&gt; 1.46-2.14, &lt;i&gt;P&lt;/i&gt;&lt;0.000 1), stroke (&lt;i&gt;HR&lt;/i&gt;=1.24, 95%&lt;i&gt;CI&lt;/i&gt; 1.05-1.46, &lt;i&gt;P&lt;/i&gt;=0.010 9), and all-cause mortality (&lt;i&gt;HR&lt;/i&gt;=2.01, 95%&lt;i&gt;CI&lt;/i&gt; 1.81-2.23, &lt;i&gt;P&lt;/i&gt;&lt;0.000 1). Restricted cubic spline analyses demonstrated a nonlinear association between Age-Gap and the risks of MACCE, cardiac death and all-cause mortality, with a marked increase in risk as Age-Gap increased (all &lt;i&gt;P-nonlinear&lt;/i&gt;&lt;0.000 1; all &lt;i&gt;P-overall&lt;/i&gt;&lt;0.000 1). &lt;b&gt;Conclusions:&lt;/b&gt; The appl","PeriodicalId":38755,"journal":{"name":"中华心血管病杂志","volume":"54 2","pages":"157-164"},"PeriodicalIF":0.0,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146195983","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Extracorporeal membrane oxygenation-assisted radiofrequency ablation for ventricular tachycardia electrical storm in super-elderly patient: a case report].","authors":"X N Shang, M Y Sun, Z L Wang, Z Y Liang, D H Zhang, M Liang","doi":"10.3760/cma.j.cn112148-20251130-00839","DOIUrl":"10.3760/cma.j.cn112148-20251130-00839","url":null,"abstract":"","PeriodicalId":38755,"journal":{"name":"中华心血管病杂志","volume":"54 2","pages":"221-223"},"PeriodicalIF":0.0,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146195975","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}