Acta Cardiologica Sinica最新文献

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Atlas Drug-Eluting Coronary Stents Inhibit Neointimal Hyperplasia in Sheep Modeling. Atlas 药物洗脱冠状动脉支架在绵羊模型中抑制新内膜增生
IF 1.8 4区 医学
Acta Cardiologica Sinica Pub Date : 2024-09-01 DOI: 10.6515/ACS.202409_40(5).20240618A
Rasit Dinc, Halit Yerebakan
{"title":"Atlas Drug-Eluting Coronary Stents Inhibit Neointimal Hyperplasia in Sheep Modeling.","authors":"Rasit Dinc, Halit Yerebakan","doi":"10.6515/ACS.202409_40(5).20240618A","DOIUrl":"10.6515/ACS.202409_40(5).20240618A","url":null,"abstract":"<p><strong>Background: </strong>Coronary artery disease (CAD) is one of the leading causes of mortality and morbidity worldwide. Many patients with CAD require mechanical revascularization. However, restenosis after minimally invasive interventions is a major problem for these patients. Fortunately, the controlled drug delivery properties of drug-eluting stents seem to be able to overcome this problem. In this study, the pharmacodynamic and pharmacokinetic properties of Atlas Drug-eluting Coronary Stents coated with poly (lactic acid-coglycolic acid) (PLGA) were evaluated.</p><p><strong>Materials and methods: </strong>This study included 20 non-atherosclerotic female sheep divided into 4 groups that included 4 study and 1 control animal randomly assigned to each group. Animals in the study groups were stented with Atlas Drug-eluting Coronary Stents, and the pharmacodynamic and pharmacokinetic properties were evaluated.</p><p><strong>Results: </strong>Sirolimus was shown to have a statistically important effect on the vascular endothelium. With time, the decrease in sirolimus in blood samples was statistically significant. Two animals died after implantation; however no clinically significant side effects were observed in the others.</p><p><strong>Conclusions: </strong>The results in this study showed a significant reduction in neointimal hyperplasia after experimental implantation of Atlas Drug-eluting Coronary Stents coated with PLGA polymer. Pharmacokinetic studies also showed that the stent did not release a significant amount of sirolimus after 28 days.</p>","PeriodicalId":6957,"journal":{"name":"Acta Cardiologica Sinica","volume":"40 5","pages":"585-594"},"PeriodicalIF":1.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11413949/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142278622","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Determinants of Prosthesis-Patient Mismatch after Aortic Valve Replacement-Ten-Year Cohort Data in Single Center of Taiwan. 主动脉瓣置换术后假体与患者不匹配的决定因素--台湾单一中心的十年队列数据。
IF 1.8 4区 医学
Acta Cardiologica Sinica Pub Date : 2024-09-01 DOI: 10.6515/ACS.202409_40(5).20240207B
Po-Hsueh Su, Ting-Hsing Chao, Mu-Shiang Huang, Wei-Chuan Tsai
{"title":"Determinants of Prosthesis-Patient Mismatch after Aortic Valve Replacement-Ten-Year Cohort Data in Single Center of Taiwan.","authors":"Po-Hsueh Su, Ting-Hsing Chao, Mu-Shiang Huang, Wei-Chuan Tsai","doi":"10.6515/ACS.202409_40(5).20240207B","DOIUrl":"10.6515/ACS.202409_40(5).20240207B","url":null,"abstract":"<p><strong>Background: </strong>Patient-prosthesis mismatch (PPM) after surgical aortic valve replacement for severe aortic stenosis has a significant effect on survival. Few studies have identified the risk factors for PPM and related outcomes. This study investigated these risk factors and clarified the outcomes.</p><p><strong>Methods: </strong>This study enrolled consecutive patients who underwent aortic valve replacement surgery between January 2010 and June 2020 in our hospital. Data on clinical profiles, prosthesis types, echocardiographic parameters before and after surgery, and clinical outcomes including the composite of all-cause mortality and redo valve replacement were collected. We defined moderate and severe PPM as an effective orifice area index value of ≤ 0.85 and ≤ 0.65 cm<sup>2</sup>/m<sup>2</sup>, respectively, measured postoperatively through echocardiography. Potential risk factors for PPM and clinical outcomes were evaluated.</p><p><strong>Results: </strong>A total of 185 patients were enrolled. Body surface area (BSA; 1.68 ± 0.02 vs. 1.62 ± 0.01 m<sup>2</sup>, p = 0.036), renal insufficiency (32.50% vs. 11.70%, p = 0.026), and aortic annulus diameter (1.99 ± 0.05 vs. 2.17 ± 0.03 cm, p = 0.013) were statistically significant risk factors for severe PPM. The primary outcome was observed in 30.00% and 15.86% of the patients with and without severe PPM, respectively (log-rank p = 0.023). Multivariate Cox proportional hazards analysis indicated that severe PPM was a risk factor for the primary outcome (hazard ratio: 2.688, 95% confidence interval: 1.094-6.622, p = 0.031).</p><p><strong>Conclusions: </strong>Our study demonstrated that large BSA, renal insufficiency, and small annulus diameter were risk factors for severe PPM after aortic valve replacement surgery. Severe PPM was associated with worse clinical outcomes.</p>","PeriodicalId":6957,"journal":{"name":"Acta Cardiologica Sinica","volume":"40 5","pages":"595-607"},"PeriodicalIF":1.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11413942/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142278625","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Feasibility and Effectiveness of Venoarterial Extracorporeal Membrane Oxygenation Plus Intra-Aortic Balloon Pump Assisted High-Risk Percutaneous Coronary Intervention in Complex Coronary Disease. 体外膜肺氧合加主动脉内球囊泵辅助高风险经皮冠状动脉介入治疗复杂冠状动脉疾病的可行性和有效性。
IF 1.8 4区 医学
Acta Cardiologica Sinica Pub Date : 2024-09-01 DOI: 10.6515/ACS.202409_40(5).20240617F
Dong-Tao Li, Yi Cao, Yi-Gang Qiu, Yu Chen, Jian-Yong Zheng
{"title":"Feasibility and Effectiveness of Venoarterial Extracorporeal Membrane Oxygenation Plus Intra-Aortic Balloon Pump Assisted High-Risk Percutaneous Coronary Intervention in Complex Coronary Disease.","authors":"Dong-Tao Li, Yi Cao, Yi-Gang Qiu, Yu Chen, Jian-Yong Zheng","doi":"10.6515/ACS.202409_40(5).20240617F","DOIUrl":"10.6515/ACS.202409_40(5).20240617F","url":null,"abstract":"<p><strong>Background: </strong>Mechanical circulatory support may facilitate high-risk percutaneous coronary intervention (PCI). This study aimed to assess the feasibility, safety and effectiveness of high-risk PCI under the support of venoarterial extracorporeal membrane oxygenation (VA-ECMO) combined with intra-aortic balloon pump (IABP).</p><p><strong>Methods: </strong>We enrolled patients who received VA-ECMO plus IABP-assisted PCI procedures at our center from April 2012 to June 2018. Major adverse cardiac events (MACEs) included all-cause death, myocardial infarction, and target vessel revascularization.</p><p><strong>Results: </strong>A total of 10 patients were included, with a mean age of 71 years, EuroSCORE II of 19.9%, and SYNTAX score of 39.8. Procedural success was achieved in nine (90%) patients. The mean duration of ECMO support was 1.5 hours, and 2.6 stents were implanted per patient. Major complications included contrast-induced nephropathy needing hemodialysis in one (10%) patient, significant hemoglobin drop requiring blood transfusion in two (20%) patients, pulmonary infection in one (10%) patient, and local surgical incision infection in one (10%) patient. The accumulative mortality rates for the nine patients with procedural success were 0, 22.2%, and 44.4% at 1, 3, and 5 years follow-up, respectively. However, cardiac death occurred in only one (11.1%) patient. In addition, two patients received repeat PCI or coronary artery bypass grafting within two years following the index procedure. The overall incidence rates of MACEs were 11.1%, 44.4%, and 66.7% at 1, 3, and 5 years follow-up, respectively.</p><p><strong>Conclusions: </strong>VA-ECMO plus IABP-assisted high-risk PCI was feasible in patients with complex coronary disease, with a high procedural success rate and acceptable mid-term clinical outcomes.</p>","PeriodicalId":6957,"journal":{"name":"Acta Cardiologica Sinica","volume":"40 5","pages":"577-584"},"PeriodicalIF":1.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11413948/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142278626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Catheter-Directed Thrombolysis for Superficial Femoral Artery Thrombosis via the Ipsilateral Dorsalis Pedis Artery. 通过同侧足背动脉对股浅动脉血栓形成进行导管引导溶栓治疗。
IF 1.8 4区 医学
Acta Cardiologica Sinica Pub Date : 2024-09-01 DOI: 10.6515/ACS.202409_40(5).20240617B
Hsin-Fu Lee, Hung-Chi Su, Chi Chuang
{"title":"Catheter-Directed Thrombolysis for Superficial Femoral Artery Thrombosis via the Ipsilateral Dorsalis Pedis Artery.","authors":"Hsin-Fu Lee, Hung-Chi Su, Chi Chuang","doi":"10.6515/ACS.202409_40(5).20240617B","DOIUrl":"10.6515/ACS.202409_40(5).20240617B","url":null,"abstract":"","PeriodicalId":6957,"journal":{"name":"Acta Cardiologica Sinica","volume":"40 5","pages":"656-658"},"PeriodicalIF":1.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11413938/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142278623","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Late Cardiac Involvement of the Glycogen Storage Disease Type IIIa with Massive Left Ventricular Hypertrophy and Late Gadolinium Enhancement. 伴有大规模左心室肥大和晚期钆增强的糖原贮积症 IIIa 型晚期心脏受累。
IF 1.8 4区 医学
Acta Cardiologica Sinica Pub Date : 2024-09-01 DOI: 10.6515/ACS.202409_40(5).20240528B
Bedirhan Bozkurt Çimen, Samet Yilmaz
{"title":"Late Cardiac Involvement of the Glycogen Storage Disease Type IIIa with Massive Left Ventricular Hypertrophy and Late Gadolinium Enhancement.","authors":"Bedirhan Bozkurt Çimen, Samet Yilmaz","doi":"10.6515/ACS.202409_40(5).20240528B","DOIUrl":"10.6515/ACS.202409_40(5).20240528B","url":null,"abstract":"","PeriodicalId":6957,"journal":{"name":"Acta Cardiologica Sinica","volume":"40 5","pages":"648-650"},"PeriodicalIF":1.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11413943/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142278629","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Significant Role of Post-Acute Care Programs in Individuals with Heart Failure. 急性期后护理计划在心力衰竭患者中的重要作用。
IF 1.8 4区 医学
Acta Cardiologica Sinica Pub Date : 2024-09-01 DOI: 10.6515/ACS.202409_40(5).20240531A
Ercan Akşit, Hakkı Kaya, Emine Gazi
{"title":"The Significant Role of Post-Acute Care Programs in Individuals with Heart Failure.","authors":"Ercan Akşit, Hakkı Kaya, Emine Gazi","doi":"10.6515/ACS.202409_40(5).20240531A","DOIUrl":"10.6515/ACS.202409_40(5).20240531A","url":null,"abstract":"","PeriodicalId":6957,"journal":{"name":"Acta Cardiologica Sinica","volume":"40 5","pages":"663-664"},"PeriodicalIF":1.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11413951/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142278633","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Look is Worth a Thousand Words!! - Case Series of Forearm Compartment Syndrome after Transradial Coronary Intervention. 一目胜千言!"!- 经桡动脉冠状动脉介入术后前臂室间隔综合征病例系列。
IF 1.8 4区 医学
Acta Cardiologica Sinica Pub Date : 2024-09-01 DOI: 10.6515/ACS.202409_40(5).20240619A
Sriram Veeraraghavan, Bharath Raj Kidambi, Prasanna Subbaraju, Vasundhara Ponnaganti, Abhilasha Munisingh
{"title":"A Look is Worth a Thousand Words!! - Case Series of Forearm Compartment Syndrome after Transradial Coronary Intervention.","authors":"Sriram Veeraraghavan, Bharath Raj Kidambi, Prasanna Subbaraju, Vasundhara Ponnaganti, Abhilasha Munisingh","doi":"10.6515/ACS.202409_40(5).20240619A","DOIUrl":"10.6515/ACS.202409_40(5).20240619A","url":null,"abstract":"","PeriodicalId":6957,"journal":{"name":"Acta Cardiologica Sinica","volume":"40 5","pages":"651-655"},"PeriodicalIF":1.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11413954/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142278620","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Impact of Abnormal Ankle Brachial Index on Sepsis Survival: One-Year Prospective Study Results. 踝臂指数异常对败血症存活率的影响:一年前瞻性研究结果。
IF 1.8 4区 医学
Acta Cardiologica Sinica Pub Date : 2024-09-01 DOI: 10.6515/ACS.202409_40(5).20240528A
Hsinyu Tseng, Min-Tsun Liao, Li-Ta Keng, Chia-Hao Chang, Ya-Zih Zeng, Mu-Yang Hsieh
{"title":"Impact of Abnormal Ankle Brachial Index on Sepsis Survival: One-Year Prospective Study Results.","authors":"Hsinyu Tseng, Min-Tsun Liao, Li-Ta Keng, Chia-Hao Chang, Ya-Zih Zeng, Mu-Yang Hsieh","doi":"10.6515/ACS.202409_40(5).20240528A","DOIUrl":"10.6515/ACS.202409_40(5).20240528A","url":null,"abstract":"<p><strong>Background: </strong>Lower extremity peripheral artery disease (LE-PAD) has been linked to unfavorable cardiovascular outcomes. The impact of potentially undiagnosed LE-PAD, suspected by abnormal ankle-brachial index (ABI), on the survival of sepsis patients admitted to the intensive care unit (ICU) remains uncertain.</p><p><strong>Methods: </strong>We conducted a prospective cohort study and recruited adult patients admitted to the ICU with a primary diagnosis of sepsis (defined by a quick Sepsis-Related Organ Failure Assessment score of ≥ 2) between November 23, 2017 and July 22, 2018. ABI measurements were obtained within 24 hours of admission. The study compared the 30-day and 1-year all-cause mortality rates as well as the incidence of major adverse cardiovascular events (MACEs) between the groups with normal and abnormal ABI values.</p><p><strong>Results: </strong>Of the 102 sepsis patients admitted to the ICU, 38 (37%) were diagnosed with LE-PAD based on their ABI measurements. The overall 30-day mortality rate was 30.0% in patients with LE-PAD and 25.8% in those with normal ABI (p = 0.56). At 1 year, the overall mortality rate was 52.6% in the patients with abnormal ABI and 40.6% in those with normal ABI (p = 0.24). Additionally, the incidence of MACEs was significantly higher in the patients with abnormal ABI compared to those with normal ABI at 1-year follow-up (21.1% vs. 3.1%, respectively; p = 0.003).</p><p><strong>Conclusions: </strong>The patients with abnormal ABI had a higher incidence of MACEs within one year following hospital discharge. Future studies are needed to improve cardiovascular outcomes among sepsis survivors (ClinicalTrials.gov number, NCT03372330).</p>","PeriodicalId":6957,"journal":{"name":"Acta Cardiologica Sinica","volume":"40 5","pages":"627-634"},"PeriodicalIF":1.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11413945/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142278627","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Subacute Bioprosthetic Mitral Valve and Inter-Atrial Septum Mural Thrombosis. 亚急性生物人工二尖瓣和房间隔壁间血栓形成。
IF 1.8 4区 医学
Acta Cardiologica Sinica Pub Date : 2024-09-01 DOI: 10.6515/ACS.202409_40(5).20240617C
Ping-Ping Wang, Chuan-Chih Hsu, Chih-Wei Chen, Yi-Cheng Lin, Chun-Yao Huang
{"title":"Subacute Bioprosthetic Mitral Valve and Inter-Atrial Septum Mural Thrombosis.","authors":"Ping-Ping Wang, Chuan-Chih Hsu, Chih-Wei Chen, Yi-Cheng Lin, Chun-Yao Huang","doi":"10.6515/ACS.202409_40(5).20240617C","DOIUrl":"10.6515/ACS.202409_40(5).20240617C","url":null,"abstract":"","PeriodicalId":6957,"journal":{"name":"Acta Cardiologica Sinica","volume":"40 5","pages":"659-662"},"PeriodicalIF":1.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11413946/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142278632","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
2024 Update of the TSOC Expert Consensus of Fabry Disease. 2024 法布里病 TSOC 专家共识更新。
IF 1.8 4区 医学
Acta Cardiologica Sinica Pub Date : 2024-09-01 DOI: 10.6515/ACS.202409_40(5).20240731A
Chung-Lieh Hung, Yen-Wen Wu, Ling Kuo, Kuo-Tzu Sung, Heng-Hsu Lin, Wei-Ting Chang, Chia-Hsiu Chang, Chih-Hung Lai, Chun-Yao Huang, Chun-Li Wang, Chih-Chan Lin, Jyh-Ming Jimmy Juang, Po-Sheng Chen, Chao-Yung Wang, Hao-Chih Chang, Chun-Yuan Chu, Wen-Hwa Wang, Hsinyu Tseng, Yung-Ta Kao, Tzung-Dau Wang, Wen-Chung Yu, Wen-Jone Chen
{"title":"2024 Update of the TSOC Expert Consensus of Fabry Disease.","authors":"Chung-Lieh Hung, Yen-Wen Wu, Ling Kuo, Kuo-Tzu Sung, Heng-Hsu Lin, Wei-Ting Chang, Chia-Hsiu Chang, Chih-Hung Lai, Chun-Yao Huang, Chun-Li Wang, Chih-Chan Lin, Jyh-Ming Jimmy Juang, Po-Sheng Chen, Chao-Yung Wang, Hao-Chih Chang, Chun-Yuan Chu, Wen-Hwa Wang, Hsinyu Tseng, Yung-Ta Kao, Tzung-Dau Wang, Wen-Chung Yu, Wen-Jone Chen","doi":"10.6515/ACS.202409_40(5).20240731A","DOIUrl":"10.6515/ACS.202409_40(5).20240731A","url":null,"abstract":"<p><p>As an X-linked inherited lysosomal storage disease that is caused by α-galactosidase A gene variants resulting in progressive accumulation of pathogenic glycosphingolipid (Gb3) accumulation in multiple tissues and organs, Fabry disease (FD) can be classified into classic or late-onset phenotypes. In classic phenotype patients, α-galactosidase A activity is absent or severely reduced, resulting in a more progressive disease course with multi-systemic involvement. Conversely, late-onset phenotype, often with missense variants (e.g., IVS4+919G>A) in Taiwan, may present with a more chronic clinical course with predominant cardiac involvement (cardiac subtype), as they tend to have residual enzyme activity, remaining asymptomatic or clinically silent during childhood and adolescence. In either form, cardiac hypertrophy remains the most common feature of cardiac involvement, potentially leading to myocardial fibrosis, arrhythmias, and heart failure. Diagnosis is established through α-galactosidase enzyme activity assessment or biomarker analyisis (globotriaosylsphingosine, Lyso-Gb3), advanced imaging modalities (echocardiography and cardiac magnetic resonance imaging), and genotyping to differentiate FD from other cardiomyopathy. Successful therapeutic response relies on early recognition and by disease awareness from typical features in classic phenotype and cardiac red flags in cardiac variants for timely therapeutic interventions. Recent advances in pharmacological approach including enzyme replacement therapy (agalsidase alfa or beta), oral chaperone therapy (migalastat), and substrate reduction therapy (venglustat) aim to prevent from irreversible organ damage. Genotype- and gender-based monitoring of treatment effects through biomarker (Lyso-Gb3), renal assessment, and cardiac responses using advanced imaging modalities are key steps to optimizing patient care in FD.</p>","PeriodicalId":6957,"journal":{"name":"Acta Cardiologica Sinica","volume":"40 5","pages":"544-568"},"PeriodicalIF":1.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11413953/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142278619","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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