Cardiovascular diagnosis and therapy最新文献

Background: Gestational diabetes mellitus (GDM) increases the risk of cardiovascular abnormalities in offspring. The objective of this study is to assess changes in left ventricular myocardial work using the left ventricular pressure-strain loop (LVPSL) method in neonates born to mothers with GDM. The aim of the research is to examine early impairments in neonatal left ventricular systolic function and to investigate whether these impairments persist over time.

Methods: In a prospective cohort study, we enrolled 61 neonates born to mothers with GDM and 30 healthy neonates born to mothers without pregnancy complications between August 2021 and March 2023 using a random method. The GDM group was further subdivided based on maternal hemoglobin A1c (HbA1c) levels into those with HbA1c ≤6.5% and those with HbA1c >6.5%. Echocardiographic assessments and left ventricular myocardial work parameters were measured and compared across the three groups using one-way analysis of variance (ANOVA) with multiple comparisons conducted using the Least Significant Difference t (LSD-T) test and multiple correction using the Bonferroni method in terms of data of normal distribution and homoscedasticity. Non-normally distributed data were presented as median (first quartile, third quartile) [M (Q1, Q3)] and compared using the Kruskal-Wallis H test. The correlation between myocardial work parameters in neonates born to mothers with GDM and the maternal HbA1c levels was also analyzed using Pearson correlation analysis or Spearman's rank correlation.

Results: The enrolled 61 neonates born to women with GDM comprised 34 male and 27 female neonates, with a gestational age (GA) of 38.9±1.7 weeks. The control group 30 healthy neonates comprised 17 males and 13 females, with a GA of 39.1±1.8 weeks. Neonates in the HbA1c ≤6.5% and HbA1c >6.5% groups demonstrated increased interventricular septal thickness (IVSD) (P<0.05) compared to the control group. However, no significant differences in IVSD were observed among the groups after a 12-month follow-up (P>0.05). At birth and during the 12-month follow-up, global longitudinal strain (GLS), global work index (GWI), and global constructive work (GCW) values were lower in both HbA1c groups compared to the control group, with the HbA1c >6.5% group revealing significantly reduced GLS, GWI, and GCW (P<0.05). Neonatal GLS exhibited a positive correlation with maternal HbA1c, whereas GWI and GCW revealed negative correlations (r=0.683, r=-0.709, r=-0.688, P<0.001).

Conclusions: The LVPSL method can examine early impairments in left ventricular systolic function in neonates born to mothers with GDM. More severe impairments are associated with poorer glycemic control during pregnancy, as indicated by higher maternal HbA1c levels. These functional impairments persist in the offspring 12 months postpartum.

Background: Apolipoprotein M (ApoM), a protein component of lipoproteins, is closely related to the development of atherosclerosis, but the specific mechanism remains elusive. Mitochondrial DNA damage can contribute to atherosclerosis, so this study was designed to investigate whether ApoM influences the structure and function of mitochondria during the progression of atherosclerosis and to explore the underlying mechanism.

Methods: Atherosclerosis models were established in male ApoM-deficient (ApoM^-/- ) and wild-type (ApoM^+/+ ) C57BL/6 mice fed a high-fat diet (HFD), and the development of atherosclerosis was verified by en face analysis of the aorta and Masson's trichrome staining. We utilized transmission electron microscopy (TEM) to examine the ultrastructure of the aorta, its endothelial cells and EA.hy926 cells. Mass spectrometry-based lipidomics was performed to measure lipidomes in the serum and liver tissue of ApoM^{- /-} mice. In EA.hy926 cells, we modulated the levels of autophagy and ApoM expression, and investigated the mechanism by which ApoM influences the pathogenesis of atherosclerosis through western blotting, JC-1 staining, flow cytometry, and Seahorse extracellular flux analysis.

Results: In ApoM^-/- mice fed an HFD, atherosclerotic markers such as aortic lipid accumulation, fibrosis, endothelial cell oedema, and mitochondrial swelling were observed, indicating early atherosclerotic development. Lipidomic analysis revealed that ApoM deficiency might lead to impaired autophagy and mitochondrial dysfunction. In EA.hy926 cells, overexpression of ApoM not only activated autophagy but also improved mitochondrial structure. Moreover, ApoM decreased the mitochondrial membrane potential (ΔΨm) of EA.hy926 cells, which was further reduced by autophagy activation. Additionally, overexpression of ApoM in EA.hy926 cells, which have a low basal metabolism and primarily rely on glycolysis for energy, significantly reduced basal mitochondrial respiration and adenosine triphosphate (ATP) production, suggesting that ApoM can facilitate mitochondrial fission.

Conclusions: ApoM exerts atheroprotective effects by promoting autophagy and regulating mitochondrial dynamics, thereby maintaining mitochondrial integrity and function. This study provides novel insights into the mechanisms underlying the protective role of ApoM in atherosclerosis and highlights its potential as a therapeutic target for cardiovascular diseases.

Registries have become pivotal in medical research, offering a robust foundation for understanding disease incidence, treatment patterns, and patient outcomes across diverse populations. By aggregating real-world data (RWD), registries provide invaluable insights into real-world evidence (RWE), shaping clinical guidelines, healthcare policies, and regulatory decisions. Their widespread acceptance underscores their scientific validity and their role in driving evidence-based medicine, ultimately improving healthcare outcomes. In cardiology, particularly within the specialized field of congenital heart disease (CHD), national and international registries have emerged as indispensable tools. They enable the systematic collection of data on patient demographics, disease progression, therapeutic interventions, and long-term outcomes. These datasets support a range of purposes, including observational studies, quality improvement initiatives, and regulatory assessments of medical devices or pharmaceuticals. Establishing a high-quality registry requires meticulous planning and adherence to established guidelines. Professional organizations, such as the European Society of Cardiology (ESC) and the American Heart Association (AHA), offer detailed guidance documents for setting up and managing registries. Additionally, various checklists and frameworks exist to evaluate and ensure registry quality, aiding researchers in optimizing data reliability and utility. With advancements in digital health, the potential of electronic health records (EHRs) to complement or replace traditional registries is increasingly explored. EHRs offer a dynamic, real-time data collection mechanism, reducing redundancy and operational costs while maintaining data accuracy. However, considerations around interoperability, data privacy, and standardization remain critical in leveraging EHRs for registry purposes.

Background: Fabry disease (FD) is a rare hereditary X-linked disorder of glycosphingolipid metabolism caused by a deficiency of α-galactosidase A leading to the accumulation of glycosphingolipids in lysosomes. Clinical manifestations vary widely, involving multiple organs. Delays in diagnosis and deficits in primary care are common due to the limited familiarity with this rare disease in primary care providers [PCPs; including general practitioners (GPs), family physicians, and specialists in internal medicine]. This study aims to assess the real-world healthcare situation of FD patients in Germany through a questionnaire-based approach.

Methods: In this cross-sectional study, 103 FD patients were consecutively recruited from several medical institutions in Germany and Austria between August 2022 and April 2024. The study adhered to ethical guidelines and received approvals from all relevant ethics committees. Inclusion criteria included confirmed FD diagnosis, age 18 years or older, and the ability to provide informed consent. Data were collected using a 32-question survey covering demographics, comorbidities, care structures, information needs, quality of life (QoL), and challenges. Descriptive statistical methods were used for data analysis of the study population.

Results: The analysis included 103 completed questionnaires. The average age of the patient cohort was 49.3±16.2 (range, 18-94) years, with a higher proportion of women (61.2%) participating. The most common symptoms reported were hearing loss/tinnitus (45.6%), followed by thermal sensory disturbances and hypohidrosis (43.7%). A majority of PCP (86.4%) were aware of their patients' FD diagnosis. Overall, 61.2% of patients were not primarily treated by their PCP, but in collaboration with Fabry specialists. Only 63.1% of the patients had received care in a FD competence centre. There was a significant need for advice, especially regarding old-age security (40.0%), nutrition/exercise (39.8%), and resilience in everyday life (37.9%). The awareness among patients, that Fabry competence centers exist which are specialized in Fabry treatment, was limited, with only 64.7% of patients being sufficiently informed. Also, knowledge regarding specific FD self-help groups was limited to 61.2% of patients. FD patients reported a reduced mean QoL score of 78.8±16.7, with pain having the most significant impact on QoL (64.1%).

Conclusions: Using a novel patient-reported questionnaire, this study reveals for the first time the state of medical care of FD-patients under "real life" conditions. This study highlights significant deficiencies in the medical care of FD patients in Germany, including insufficient knowledge of specialized centers and patient organizations, as well as unmet counselling needs. Despite the availability of at least 24 FD competence centers in Germany, many patients remain under the care of

Background: Immune checkpoint inhibitor-associated myocarditis (ICI myocarditis) is a rare but highly fatal immune-related adverse reaction. This study aimed to develop nomogram prognostic models for both short-term and long-term survival outcomes in patients with ICI myocarditis based on key biomarkers in peripheral blood.

Methods: In this single-center retrospective study, we included 90 patients with ICI myocarditis at the Fourth Hospital of Hebei Medical University. Critical peripheral biomarkers associated with 40-day and 1-year overall survival (OS) were identified. Two prognostic models were developed and evaluation of the models were performed with receiver operating characteristic (ROC) curves, C-index, calibration curves, and decision curve analysis (DCA).

Results: A total of 24 patients (26.7%) succumbed within 40 days, while 40 patients (44.4%) died within one year. Cardiac troponin-I (cTnI), N-terminal pro-brain natriuretic peptide (NTBNP) and lactic dehydrogenase-to-albumin ratio (LAR) were identified as critical prognostic factors for 40-day OS in patients with ICI myocarditis and utilized to develop a nomogram model. The model demonstrates an area under the curve (AUC) of 0.867 [95% confidence interval (CI): 0.774-0.960] and a C-index of 0.824. Another predictive model for the 1-year OS was developed based on cTnI, NTBNP, LAR and systemic inflammatory response index (SIRI) with an AUC of 0.765 (95% CI: 0.664-0.866) and a C index of 0.742. The calibration curve demonstrates that both models exhibit strong consistency. The results of the DCA further indicate that both nomograms possess substantial clinical utility.

Conclusions: These two prediction models will enable clinicians to more effectively utilize readily available peripheral blood biomarkers for the convenient and efficient identification of high-risk patients with poor prognoses, thereby facilitating early intervention.

Background: Hypotension frequently occurs in patients who are undergoing plasma exchange procedures. However, the prevalence, clinical manifestations, and determinants of hypotension during plasma exchange in patients with neuroimmunological disorders have not been fully elucidated. The aim of this retrospective cohort study was to evaluate the current status and influencing factors of hypotension during plasma exchange in patients with neuroimmunological diseases, to provide insights for clinical care.

Methods: This study encompasses patients diagnosed with neuroimmunological disorders who received plasma exchange treatment at a tertiary hospital in Nanjing, China, over a period from February 1, 2023, to April 30, 2024. The demographic characteristics and clinical profiles of these patients were subjected to a comprehensive analysis. To ascertain the factors influencing the development of hypotension during plasma exchange, a logistic regression analysis was performed.

Results: A total of 206 patients with neuroimmunological diseases were included, the incidence of hypotension during plasma exchange in patients with neuroimmunological diseases was 33.01% (68/206). There were statistical differences in the age, use of sedative drugs during plasma exchange, pre-plasma exchange systolic blood pressure and serum calcium between hypotension and control group. Logistic regression analysis indicated that age [odds ratio (OR) =2.851, 95% confidence interval (CI): 1.978-3.194], use of sedative drugs during plasma exchange (OR =3.175, 95% CI: 2.363-4.425), pre-plasma exchange systolic blood pressure (OR =0.857, 95% CI: 0.410-0.932), and serum calcium (OR =0.791, 95% CI: 0.340-0.895) were the influencing factors of hypotension during plasma exchange in patients with neuroimmune diseases.

Conclusions: The incidence of hypotension during plasma exchange in patients with neuroimmune diseases is relatively high. Health care providers should actively take measures against factors associated with hypotension to reduce its occurrence during plasma exchange.

Background: Myocardial infarction (MI) remains one of the main causes of mortality worldwide. Beta-blockers (BBs) are an essential component in the pharmacological treatment for MI. The long-term role of BB in patients with preserved left ventricular ejection fraction (LVEF) is not yet well established. Thus, we performed a systematic review and meta-analysis to synthesize the impact of long-term use of BB on reducing mortality in patients with preserved LVEF after MI.

Methods: This study adhered to the guidelines outlined by the Cochrane Collaboration and the PRISMA statement. The predefined research protocol was registered in PROSPERO under the ID CRD42024554630. A systematic search was conducted in Embase, the Cochrane Central Register of Controlled Trials, and PubMed for studies published in English up to September 1, 2024, using the succeeding medical subject terms: 'myocardial infarction', 'preserved ejection fraction', and 'beta-blockers'. Data were extracted for: (I) death from any cause; (II) death from cardiovascular causes; (III) MI; (IV) stroke; and (V) hospitalization for heart failure (HF). The risk of bias of each article was analyzed using the tool risk of bias in non-randomized studies of interventions (ROBINS-I) and risk-of-bias tool for randomized trials (RoB2). These outcomes were compared using pooled hazard ratios (HRs) to maintain the integrity of time-to-event data from individual studies.

Results: A total of 85,607 patients from 11 studies were included in this meta-analysis, of whom 65,790 (76.8%) were using BBs after MI with preserved ejection fraction. The use of BBs demonstrated a significant reduction in all-cause mortality in the global analysis of the included studies [HR =0.81; 95% confidence interval (CI): 0.67-0.98; P=0.03]. However, when performing sensitivity analyses to assess the impact of methodological biases and the robustness of the results, this reduction was no longer significant (HR =0.79; 95% CI: 0.62-1.02; P=0.07). Regarding reinfarction, there was no difference between BB users and non-users (HR =1.00; 95% CI: 0.92-1.09; P>0.99). Similarly, hospitalization for HF showed no significant variation between groups (HR =1.05; 95% CI: 0.89-1.24; P=0.55). Stroke incidence was also comparable between the groups, though with substantial heterogeneity (I²=60%). Heterogeneity was otherwise low for the outcomes of reinfarction, and hospitalization for HF (I²<25%). Subgroup analyses revealed no differences in outcomes when stratified by age, sex, hypertension, or diabetes.

Conclusions: Long-term BB use in patients with preserved LVEF after MI did not decrease all-cause mortality, cardiovascular mortality, or major adverse cardiac events (MACEs). There was also no identified reduction in hospitalizations for HF, MI, or stroke in the average follow-up of 3 years.

Background: Congenital right ventricular aneurysm (CVA) and diverticulum (CVD) are rare heart diseases that can be diagnosed prenatally. Data on the natural progression of ventricular aneurysms or diverticula identified during fetal life remains scarce, with insights primarily derived from a limited number of case reports and case series. This case report aims to highlight the diagnosis, management, and outcomes of a rare right ventricular free wall aneurysm progressing to hypoplastic right heart syndrome (HRHS), utilizing advanced fetal imaging and prenatal therapy. These findings provide valuable perspective on the underlying cause, diagnostic approaches, and treatment strategy for rare cardiac anomaly.

Case description: The aneurysm of the right ventricular free wall was diagnosed 13 weeks of gestation during prenatal ultrasound of the fetus in the first trimester. At the time of diagnosis, severe tricuspid valve stenosis with severe tricuspid regurgitation, critical pulmonary stenosis, pericardial effusion, ascites, and secondary flow reversal in the ductus venosus was found. Genetic testing using microarray-based comparative genomic hybridization (aCGH) on amniotic fluid samples showed no genomic imbalance. Digoxin treatment was initiated at 13 weeks of gestation, starting with a loading dose of 1,200 micrograms, followed by a maintenance dose of 375 micrograms daily, to address signs of fetal heart failure. The fetalHQ^® software was utilized to assess the size and contractility of both the right and left ventricles, focusing on global and segmental strain measurements. The gradual reduction in right ventricular cardiac output led to the progression of HRHS with pulmonary atresia. At 39 weeks of gestation, a female newborn weighing 2,480 g was delivered in good condition, with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. Prostaglandin E1 treatment was initiated postnatally to maintain ductal patency. Postnatal echocardiography and angio-computed tomography (CT) confirmed the prenatal diagnosis, revealing a right ventricle-to-right coronary artery (RCA) fistula and interruption of RCA perfusion continuity. The newborn underwent successful first-stage palliation with a Blalock-Taussig shunt to establish systemic-pulmonary circulation. Follow-up during the interstage period showed the child remained in good clinical condition, with no significant complications reported.

Conclusions: CVAs are rare but significant anomalies with potential for severe hemodynamic consequences. Multidisciplinary approaches integrating advanced imaging techniques, prenatal counseling, and tailored postnatal management are essential for optimizing outcomes.