Cardiovascular diagnosis and therapy最新文献

{"title":"Non-invasive tissue characterization in children and young adults with aortic coarctation-an MRI-based prospective study.","authors":"Tobias Giertzsch, Michael Jerosch-Herold, Philipp Schneider, Inga Voges, Dominik Daniel Gabbert, Philip Wegner, Götz Müller, Tilo Kölbel, Ida Hüners, Jörg Siegmar Sachweh, Michael Hübler, Jochen Herrmann, Sophie Alt, Anne Schöber, Inka Ristow, Gerhard Adam, Gunnar K Lund, Carsten Rickers","doi":"10.21037/cdt-24-497","DOIUrl":"10.21037/cdt-24-497","url":null,"abstract":"<p><strong>Background: </strong>Aortic coarctation (CoA) necessitates long-term monitoring to identify late complications, including re-stenosis, aneurysms, arrhythmias and heart failure. Nonetheless, there remain gaps in understanding the effects of adverse left-ventricular (LV) remodeling at the myocardial tissue level, which may contribute to incipient heart failure. The aim of this study is to evaluate myocardial tissue characteristics in patients with CoA using advanced cardiac magnetic resonance (CMR) imaging techniques to identify markers of adverse tissue remodeling and their association with disease severity, bicuspid aortic valve (BAV), and clinical management strategies such as blood pressure (BP) medication.</p><p><strong>Methods: </strong>CMR imaging at 3 Tesla was used to determine the myocardial extracellular volume fraction (ECV), native T1, and intracellular water lifetime (τ_ic) by pre- and post-gadolinium contrast T1 mapping in 46 patients (21 male; mean age 20 years) with CoA and 14 age-matched controls. LV volumes, mass, and ejection fraction were obtained from cine CMR. CoA was classified as low grade [\"LG\" = the maximum flow velocity (Vmax) ≤3 m/s and no re-stenosis, nor arterial hypertension or medication], severe CoA (\"sCoA\" = Vmax >3 m/s or one of LG's other variables applies), and \"CoA with BAV\".</p><p><strong>Results: </strong>ECV was significantly higher in sCoA group (0.31±0.04) compared to LG group (0.26±0.02, P=0.002) and healthy controls (0.26±0.02, P=0.001). ECV with BAV (0.31±0.05) was higher than in LG group (P=0.03) and healthy controls (P=0.03). Native T1 values were significantly elevated in sCoA group (T1 =1,391±162 ms) compared to LG group (T1 =1,213±47 ms, P=0.002) and in CoA with BAV (T1 =1,390±127 ms) versus LG group (P=0.002). τ_ic was lower in LG group (0.24±0.03 s), indicative of a smaller cardiomyocyte diameter, compared to sCoA (0.28±0.04 s; P=0.01) and LG CoA with concomitant BAV (0.31±0.05 s; P=0.04). The LV end-systolic volume (ESV) was significantly higher in group with BAV than in LG CoA (P<0.001) and sCoA (P=0.001) groups. Patients who took BP medication had significantly lower values in native T1 (P=0.02) and τ_ic (P=0.03).</p><p><strong>Conclusions: </strong>sCoA is associated with an elevated myocardial ECV and native T1 compared to LG CoAs and healthy controls, reflecting adverse tissue remodeling. Patients with LG CoA and concomitant BAV showed significantly greater diffuse myocardial fibrosis than those with isolated LG CoA. CoA patients, especially those with sCoA and those with concomitant BAV, could be at increased long-term risk for complications related to diffuse myocardial fibrosis, such as diastolic dysfunction and arrhythmias. Patients taking antihypertensive medication may benefit from reduced cardiomyocyte hypertrophy and less interstitial fibrosis.</p>","PeriodicalId":9592,"journal":{"name":"Cardiovascular diagnosis and therapy","volume":"15 2","pages":"375-387"},"PeriodicalIF":2.1,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12082215/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092955","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiovascular magnetic resonance and valvular heart diseases: a suggested protocol for congenital lesions.","authors":"Francesca Baessato, Alessandro Ruzzarin, Christian Meierhofer","doi":"10.21037/cdt-24-470","DOIUrl":"10.21037/cdt-24-470","url":null,"abstract":"<p><p>Valvular heart diseases (VHDs) require definition of anatomy, severity, and risk stratification to best define procedural need, type of intervention and seriate follow-up. Congenital lesions are much rarer and often associated with more complex lesions. Among noninvasive imaging modalities, cardiovascular magnetic resonance (CMR) represents a fundamental tool for complete assessment and quantification of VHDs. CMR can provide wide anatomical views on cardiac and extra-cardiac structures in any plane orientation, flow and volume quantification, as well as information on ventricular remodeling and viability. In the context of valve stenosis, quantification by CMR is based primarily on direct measurement of valve orifice at maximal valve opening, although CMR data remain less reliable than standard echocardiography due to reduced temporal resolution. Definition of great vessels anatomy by CMR can allow differentiation of valvular, subvalvular or supravalvular lesions. For valve regurgitation, CMR is the gold standard for quantification of ventricular volumes and function and for direct calculation of regurgitation of the semilunar valves with through-plane phase-contrast images. Additional flow measurements can be integrated to cross-check quantitative data on great vessels flow and stroke volumes. A standardized approach is recommended in CMR studies. A minimum CMR dataset should include two-dimensional cine and phase-contrast sequences, and three-dimensional whole heart imaging. This should be applied in the clinical practice to assess VHDs, including most complex congenital lesions.</p>","PeriodicalId":9592,"journal":{"name":"Cardiovascular diagnosis and therapy","volume":"15 2","pages":"441-454"},"PeriodicalIF":2.1,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12082240/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092837","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Applications of multi-modality imaging in the diagnosis of infective endocarditis: a real-life case based contemporary narrative review.","authors":"Alice Haouzi, Mohamed Khayata, Wael A Jaber, Bo Xu","doi":"10.21037/cdt-2024-605","DOIUrl":"10.21037/cdt-2024-605","url":null,"abstract":"<p><strong>Background and objective: </strong>Recent major international society guidelines have highlighted the utility of multi-modality imaging in the evaluation of infective endocarditis (IE). This article aims to discuss the contemporary applications of multimodality imaging in IE through real-life cases, demonstrating how emerging imaging modalities, including cardiac computed tomography (CCT) and nuclear imaging techniques can be used.</p><p><strong>Methods: </strong>A literature search of the PubMed database was performed between Jan 01, 2024 and Oct 01, 2024. Relevant articles on the subjects of \"infective endocarditis\" and \"multi-modality imaging\" were used in our review. Four clinical cases from the Cleveland Clinic Foundation were incorporated to supplement this literature review with real-world examples.</p><p><strong>Key content and findings: </strong>This literature review encompasses international cardiology guidelines, as well as investigational studies, meta-analyses, and dedicated reviews highlighting the specific roles, strengths, and weaknesses of different imaging modalities in the evaluation of IE, including transthoracic and transesophageal echocardiography (TEE), CCT, ¹⁸F-fluorodeoxyglucose positron emission tomography (¹⁸F-FDG PET), and white blood cell single-photon emission computed tomography (WBC SPECT). This review demonstrates the emerging role for these multi-modality imaging tools in light of an increasingly complex patient population with growing numbers of prosthetic valves and devices.</p><p><strong>Conclusions: </strong>The current literature and guidelines are discussed with reference to complex clinical cases, with the aim of illustrating the relative advantages and disadvantages, and appropriate utility of multimodality cardiac imaging in IE.</p>","PeriodicalId":9592,"journal":{"name":"Cardiovascular diagnosis and therapy","volume":"15 2","pages":"465-479"},"PeriodicalIF":2.1,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12082189/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092616","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessment of left ventricular systolic function using pressure-strain loops in offspring of women with gestational diabetes mellitus: a prospective cohort study.","authors":"Xu-Feng Cao, Li-Li Su, Ying-Chao Fan, Jun Li, Ning Zhang, Hai-Yan Niu, Rui-Fang Wang, Hui-Fang Liu","doi":"10.21037/cdt-24-450","DOIUrl":"10.21037/cdt-24-450","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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 <i>H</i> 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.</p><p><strong>Results: </strong>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).</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":9592,"journal":{"name":"Cardiovascular diagnosis and therapy","volume":"15 2","pages":"388-397"},"PeriodicalIF":2.1,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12082216/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092697","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Apolipoprotein M delays the development of atherosclerosis by regulating autophagy and mitochondrial function.","authors":"Yuanping Shi, Shuang Yao, Binhua Jiang, Jun Zhang, Mingjun Cao, Jing Wu, Lu Zheng, Ning Xu, Xiaoying Zhang, Guanghou Shui, Guanghua Luo","doi":"10.21037/cdt-2024-614","DOIUrl":"10.21037/cdt-2024-614","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>Atherosclerosis models were established in male ApoM-deficient (<i>ApoM^-/-</i> ) and wild-type (<i>ApoM^+/+</i> ) 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 <i>ApoM^-</i> ^/- 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.</p><p><strong>Results: </strong>In <i>ApoM^-/-</i> 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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":9592,"journal":{"name":"Cardiovascular diagnosis and therapy","volume":"15 2","pages":"423-440"},"PeriodicalIF":2.1,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12082226/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092596","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Establishing a cardiology registry: navigating quality and regulatory challenges with a focus on congenital heart disease.","authors":"Robert David Pittrow, Oliver Dewald, Frank Harig, Ann-Sophie Kaemmerer-Suleiman, Mathieu Suleiman, Leonard Bernhard Pittrow, Stephan Achenbach, Annika Freiberger, Sebastian Freilinger, Benjamin Alexander Pittrow, Renate Kaulitz, Harald Kaemmerer","doi":"10.21037/cdt-2024-579","DOIUrl":"10.21037/cdt-2024-579","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":9592,"journal":{"name":"Cardiovascular diagnosis and therapy","volume":"15 2","pages":"455-464"},"PeriodicalIF":2.1,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12082178/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092893","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development of peripheral biomarker-based prognostic nomograms for short-term and long-term survival in immune checkpoint inhibitor-associated myocarditis.","authors":"Zhengkun Guan, Tiezhu Yao, Guang Liu, Jing Liu, Ling Guo, Shaoyan Du, Zhenli Li, Ruipu Gao, Yansong Wang, Jingtao Ma","doi":"10.21037/cdt-24-556","DOIUrl":"10.21037/cdt-24-556","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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).</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":9592,"journal":{"name":"Cardiovascular diagnosis and therapy","volume":"15 2","pages":"277-290"},"PeriodicalIF":2.1,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12082187/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092879","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"\"Real world\" medical care of patients with Fabry disease by primary care physicians, internists or general practitioners.","authors":"Oliver Mayer, Harald Kaemmerer, Peter Ewert, Annika Freiberger, Florian B Lagler, Claudia Regenbogen, Teresa Trenkwalder, Fabian von Scheidt, Christine Kurschat, Sebastian Freilinger","doi":"10.21037/cdt-24-483","DOIUrl":"10.21037/cdt-24-483","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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%).</p><p><strong>Conclusions: </strong>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","PeriodicalId":9592,"journal":{"name":"Cardiovascular diagnosis and therapy","volume":"15 2","pages":"350-361"},"PeriodicalIF":2.1,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12082224/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Influencing factors of hypotension during plasma exchange in patients with neuroimmunological diseases: a retrospective cohort study.","authors":"Sulian Gu, Dandan Wei, Ling Yu","doi":"10.21037/cdt-24-456","DOIUrl":"10.21037/cdt-24-456","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":9592,"journal":{"name":"Cardiovascular diagnosis and therapy","volume":"15 2","pages":"414-422"},"PeriodicalIF":2.1,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12082250/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Beta-blockers in post-myocardial infarction with preserved ejection fraction: systematic review and meta-analysis.","authors":"Rafael Alessandro Ferreira Gomes, Ludmila Cristina Camilo Furtado, Marcela Vasconcelos Montenegro, Dário Celestino Sobral Filho","doi":"10.21037/cdt-24-368","DOIUrl":"10.21037/cdt-24-368","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":9592,"journal":{"name":"Cardiovascular diagnosis and therapy","volume":"15 2","pages":"398-413"},"PeriodicalIF":2.1,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12082196/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144092831","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}