CARDIOMYOPATHY

心肌病
  • 文章类型: Journal Article
    肝硬化是一个重要的全球健康问题,肝硬化心肌病(CCM)是影响成人和儿童的显着并发症。虽然CCM在成年人中得到了很好的研究,了解其在儿科患者中的表现和诊断标准仍然是一个挑战。这篇综述探讨了肝硬化儿童结构和功能心脏改变的证据。结构异常,包括左心室质量指数(LVMI)增加和左心室壁厚度比改变,在儿科CCM中普遍存在。这些异常甚至在肝移植后仍然存在,强调肝脏疾病的系统性影响。证据表明收缩和舒张功能改变,以及心电图异常,如QT间期延长,常见于儿科CCM。血液生物标志物,包括脑钠肽(BNP)和肌钙蛋白水平,提供对小儿肝硬化患者心功能的见解。BNP水平升高与不良结局相关,表明其作为预后标志物的潜力。然而,需要进一步的研究来阐明这些生物标志物在儿科CCM中的诊断效用.
    结论:本综述提供了有肝硬化和无肝硬化儿童心脏参数之间的标准化平均差异的估计。量身定制的诊断标准和全面的评估方法对于小儿CCM的准确诊断和有效管理至关重要。
    背景:•CCM增加了肝硬化患者的护理负担。•成人的诊断标准正在演变,但是对于儿童CCM没有具体的标准。
    背景:•肝硬化儿童的心脏功能表明成人未考虑的一些参数发生了改变。•对某些参数的效应大小估计为儿科CCM的未来研究提供了指导。
    Liver cirrhosis is a significant global health concern, and cirrhotic cardiomyopathy (CCM) is a notable complication affecting both adults and children. While CCM is well-studied in adults, understanding its manifestation and diagnostic criteria in pediatric patients remains a challenge. This review explores the evidence for structural and functional cardiac alterations in children with liver cirrhosis. Structural abnormalities, including increased left ventricular mass index (LVMI) and altered left ventricular wall thickness ratios, are prevalent in pediatric CCM. These abnormalities persist even after liver transplantation, highlighting the systemic impact of liver disease. Evidence suggests that altered systolic and diastolic function, as well as electrocardiographic abnormalities such as prolonged QT intervals, are common in pediatric CCM. Blood biomarkers, including brain natriuretic peptide (BNP) and troponin levels, offer insights into cardiac function in pediatric cirrhotic patients. Elevated BNP levels correlate with adverse outcomes, indicating its potential as a prognostic marker. However, further research is needed to elucidate the diagnostic utility of these biomarkers in pediatric CCM.
    CONCLUSIONS: This review provides estimates of the standardized mean difference among selected cardiac parameters in children with and without cirrhosis. Tailored diagnostic criteria and comprehensive assessment methods will be essential for accurate diagnosis and effective management of pediatric CCM.
    BACKGROUND: • CCM adds to the burden of care of patients with cirrhosis. • Diagnostic criteria for adults are evolving, but there are no specific criteria for pediatric CCM.
    BACKGROUND: • Cardiac function in children with cirrhosis indicates some parameters not considered in adults are altered. • Effect size estimations for certain parameters provide a guideline for future research into pediatric CCM.
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    文章类型: Journal Article
    Type 2 diabetes mellitus is one of the most common non-infectious diseases in the world. Among people with type 2 diabetes, patients of the older age group. An in understanding of the early cardiovascular manifestations of diabetes occupies an important place in international research and prevention programs, given that cardiac vascular complications are the cause of death in patients with diabetes. Recent studies evaluating left ventricular diastolic dysfunction as a characteristic predictor of diabetic cardiomyopathy by echocardiography. In accordance with the recommendations for diastolic dysfunction, have shown that the algorithm of the informative algorithm is used to determine left ventricular diastolic dysfunction in patients with prognosis in predicting cardiovascular complications.
    Сахарный диабет 2-го типа (СД2) является одним из самых распространенных неинфекционных заболеваний в мире. Среди лиц с СД2 преобладают пациенты старшей возрастной группы. Углубленное понимание ранних сердечно-сосудистых проявлений диабета занимает важное место в международных исследованиях и программах профилактики, учитывая, что сердечно-сосудистые осложнения являются основной причиной смерти пациентов с диабетом. Последние исследования оценки диастолической дисфункции ЛЖ методом эхо-КГ как характерного предиктора развития кардиомиопатии показали, что обновленный алгоритм более информативен для определения диастолической дисфункции ЛЖ у пациентов при прогнозировании сердечно-сосудистых осложнений.
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  • 文章类型: Journal Article
    2型糖尿病(T2DM)是一种以持续高血糖为特征的广泛存在的慢性疾病,导致严重的并发症,如糖尿病性心肌病和肾病,显著影响患者健康和生活质量。这些并发症的复杂机制包括慢性炎症,氧化应激,和代谢失调。糖尿病心肌病,以心脏结构和功能异常为特征,糖尿病肾病,以进行性肾损伤为特征,是T2DM发病率和死亡率增加的主要原因。AdipoRon,合成的脂联素受体激动剂,在临床前研究中显示了模仿内源性脂联素有益作用的潜力,减少炎症和氧化应激,改善脂质代谢和线粒体功能。这篇系统综述评估了AdipoRon的治疗潜力,关注其对糖尿病心肌病和肾病的影响。通过全面的文献检索和分析,我们强调了AdipoRon在各种动物模型和细胞系统中改善心血管和肾脏并发症的作用。这些发现强调了转化临床研究的迫切需要,以验证AdipoRon在人群中的疗效和安全性。旨在将这种有前途的治疗方法从实验模型推进到临床应用,可能为改善糖尿病并发症的管理提供新的希望。
    Type 2 diabetes mellitus (T2DM) is a widespread chronic disease characterized by persistent hyperglycemia, leading to severe complications such as diabetic cardiomyopathy and nephropathy, significantly affecting patient health and quality of life. The complex mechanisms underlying these complications include chronic inflammation, oxidative stress, and metabolic dysregulation. Diabetic cardiomyopathy, marked by structural and functional heart abnormalities, and diabetic nephropathy, characterized by progressive kidney damage, are major contributors to the increased morbidity and mortality associated with T2DM. AdipoRon, a synthetic adiponectin receptor agonist, has shown potential in preclinical studies for mimicking the beneficial effects of endogenous adiponectin, reducing inflammation and oxidative stress, and improving lipid metabolism and mitochondrial function. This systematic review evaluates the therapeutic potential of AdipoRon, focusing on its impact on diabetic cardiomyopathy and nephropathy. Through a comprehensive literature search and analysis, we highlight AdipoRon\'s role in ameliorating cardiovascular and renal complications in various animal models and cellular systems. The findings underscore the urgent need for translational clinical studies to validate AdipoRon\'s efficacy and safety in human populations, aiming to advance this promising therapeutic approach from experimental models to clinical application, potentially offering new hope for improved management of diabetic complications.
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  • 文章类型: Case Reports
    由于PNPLA2突变引起的脂肪甘油三酯脂肪酶(ATGL)的缺乏导致中性脂质贮积病伴肌病(NLSDM),常染色体隐性遗传疾病(MIM:#610717)。NLSDM患者主要受进行性肌病的影响,心肌病,还有肝肿大.据报道,40%-50%的NLSDM患者有心脏受累。心脏受累的患者有成人发作的进行性心力衰竭,模仿扩张型或肥厚型心肌病。临床特点,基因型-表型相关性,并且不了解PNPLA2突变继发心肌病的预后。我们报道了两名男性患者在PNPLA2中携带纯合剪接突变NM_020376.4(c.7571G>T),表现为严重的扩张型心肌病和轻度骨骼肌受累。通过文献综述,总结了49例PNPLA2突变引起的心肌病的心电图和影像学特征以及预后.这项研究表明,NLSDM应被视为心肌病的原因,尤其是肌酸激酶(CK)水平升高的患者,无论是否存在肌肉无力或萎缩等症状。
    Deficiency of adipose triglyceride lipase (ATGL) due to mutation in PNPLA2 causes neutral lipid storage disease with myopathy (NLSDM), an autosomal recessive disorder (MIM: #610717). NLSDM patients are mainly affected by progressive myopathy, cardiomyopathy, and hepatomegaly. Cardiac involvement was reported in 40%-50% of NLSDM patients. Patients with cardiac involvement have adult-onset progressive heart failure, mimicking dilated or hypertrophic cardiomyopathy. The clinical characteristics, genotype-phenotype correlation, and prognosis of cardiomyopathy secondary to PNPLA2 mutation are not understood. We reported two male patients carrying a homozygous splicing mutation NM_020376.4 (c.757 + 1G>T) in PNPLA2, presenting with severe dilated cardiomyopathy and mild skeletal muscle involvement. Through the literature review, the ECG and imaging features and the prognosis of 49 previously reported cases of cardiomyopathy caused by the PNPLA2 mutation were summarized. This study suggests that NLSDM should be considered a cause of cardiomyopathy, especially in those with elevated creatine kinase (CK) levels, regardless of whether symptoms such as muscle weakness or atrophy are present.
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  • 文章类型: Journal Article
    多柔比星(DOX)是一种化疗药物,已知会引起心脏代谢变化,导致潜在的心脏毒性。这些变化影响各种细胞功能和途径,例如破坏雷帕霉素(mTOR)信号传导途径的机械靶标。本研究旨在通过体内系统评价探讨DOX对mTOR通路的影响。在2023年9月11日搜索了数据库。我们最终纳入了30项体内研究,这些研究检查了心脏组织样品中的mTOR表达。本研究表明,PI3K/AKT/mTOR,AMPK/MTOR,p53/mTOR信号,mTOR/TFEB通路,p38MAPK/mTOR,sestrins/mTOR,KLF15/eNOS/mTORC1信号通路在DOX诱导的心脏毒性的发展中起着至关重要的作用。这些途径的抑制或失调可导致氧化应激增加,凋亡,以及其他对心脏的不良影响。靶向和调节mTOR途径的策略,例如使用mTOR抑制剂如雷帕霉素,有可能增强DOX的抗癌作用,同时减轻其心脏毒性副作用。
    Doxorubicin (DOX) is a chemotherapy drug known to induce metabolic changes in the heart, leading to potential heart toxicity. These changes impact various cellular functions and pathways such as disrupting the mechanistic target of rapamycin (mTOR) signaling pathway. The study aimed to investigate the effect of DOX on the mTOR pathway through an in vivo systematic review. Databases were searched on September 11, 2023. We finally included 30 in vivo studies that examined the mTOR expression in cardiac tissue samples. The present study has shown that the PI3K/AKT/mTOR, the AMPK/mTOR, the p53/mTOR signaling, the mTOR/TFEB pathway, the p38 MAPK/mTOR, the sestrins/mTOR, and the KLF15/eNOS/mTORC1 signaling pathways play a crucial role in the development of DOX-induced cardiotoxicity. Inhibition or dysregulation of these pathways can lead to increased oxidative stress, apoptosis, and other adverse effects on the heart. Strategies that target and modulate the mTOR pathways, such as the use of mTOR inhibitors like rapamycin, have the potential to enhance the anticancer effects of DOX while also mitigating its cardiotoxic side effects.
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  • 文章类型: Journal Article
    反向takotsubo心肌病是一种罕见疾病的罕见变种,其特征是基底球囊扩张和左心室功能障碍。虽然它会使患者严重不适并依赖重症监护,这是一个短暂的现象,最严重的症状在2-3天后消退。在学期,一名年轻女性在计划再次剖宫产前自发分娩.在分娩过程中经历身体和心理上的痛苦和真空抽取后,她因逆转塔克替诺心肌病而发生心源性休克,快速诊断为经胸超声心动图。她需要2天的重症监护支持,并取得了良好的恢复。这种非常罕见的情况应该在围产期全身不适的妇女中考虑,因为它可以快速诊断,为患者提供最佳的护理。
    Reverse takotsubo cardiomyopathy is a rare variant of a rare disease characterized by basal ballooning and dysfunction of the left ventricle. While it can render patients profoundly unwell and reliant on intensivist care, it is a transient phenomenon, with the worst symptoms subsiding after 2-3 days. At term, a young woman spontaneously entered labor prior to a planned repeat cesarean section. After experiencing physical and psychological distress during labor and a vacuum extraction, she developed cardiogenic shock from reverse takotsubo cardiomyopathy, quickly diagnosed with transthoracic echocardiogram. She required 2 days of intensive care support and made an excellent recovery. This very rare condition should be considered in systemically unwell women in the peripartum as it can be quickly diagnosed, providing patients with the best appropriate care.
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  • 文章类型: Journal Article
    左西孟旦,一种新型药物,一种钙致敏剂,已成为心力衰竭(HF)的潜在治疗调节剂。本文综述了左西孟旦治疗HF的有效性和安全性。在不同的临床环境中。该研究旨在检查选定试验中报告的临床结果,以确定左西孟旦改善与HF相关的关键参数的有效性。从三个数据库中确定了包括1200名参与者的七项相关研究。纳入标准包括研究左西孟旦治疗HF疗效的临床试验,以及涉及成人和儿科参与者的研究。排除标准涉及数据不足的研究,临床试验以外的研究,病例报告,给编辑的信,会议文件,灰色文学,以及以英语以外的语言发表的研究。在评估纳入的研究后,结果发现,左西孟旦可改善严重脓毒性心肌病患者的血流动力学和临床疗效.左西孟旦可增强二尖瓣(MV)手术后右心室功能(RV)功能,并降低非ST段抬高型心肌梗死(NSTEMI)患者的N末端B型利钠肽前体(NT-ProBNP)含量NT-proBNP升高,所有这些都不会增加总费用或住院时间。尽管研究设计和参与者特征有所不同,有证据表明,左西孟旦可显著改善左心室射血分数(LVEF)和6分钟步行距离测量的运动耐量.值得注意的是,其安全性表现良好,心律失常事件发生率最低,不良反应发生率与安慰剂相当.这篇系统综述强调了左西孟旦在HF管理方面的潜力,保证进一步的研究,以巩固其临床作用。
    Levosimendan, a novel drug, a calcium-sensitizing inotrope, has emerged as a potential therapeutic modulator for heart failure (HF). This review appraises the efficacy and safety of levosimendan in managing HF, in different clinical settings. The study aims to examine the clinical outcomes reported in the selected trials to determine the effectiveness of levosimendan in improving key parameters related to HF. Seven relevant studies encompassing 1200 participants were identified from three databases. Inclusion criteria included clinical trials that investigated the therapeutic efficacy of levosimendan in the treatment of HF, and studies involving both adult and pediatric participants. Exclusion criteria involved studies with insufficient data, studies other than clinical trials, case reports, letters to the editor, conference papers, grey literature, and studies published in a language other than English. Upon evaluating the included studies, it was found that levosimendan shows improved hemodynamics and clinical efficacy in patients with severe septic cardiomyopathy. Levosimendan enhanced right ventricular (RV) function in patients with RV dysfunction after mitral valve (MV) surgeries and decreased the amount of N-terminal pro-B-type natriuretic peptide (NT-ProBNP) in non-ST elevated myocardial infarction (NSTEMI) patients with elevated NT-proBNP, all without increasing the overall cost or duration of hospitalization. Despite variations in study designs and participant characteristics, evidence suggests levosimendan significantly improves left ventricular ejection fraction (LVEF) and exercise tolerance measured by a six-minute walk distance. Notably, its safety profile appears favorable with minimal arrhythmic events and comparable rates of adverse effects to a placebo. This systematic review highlights levosimendan\'s promising potential for HF management, warranting further research to solidify its clinical role.
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  • 文章类型: Journal Article
    目的:这篇综述的目的有两个方面:(1)研究他汀类药物对蒽环类所致心脏毒性的保护作用机制;(2)全面综述现有临床文献,探讨他汀类药物对蒽环类所致心脏毒性的一级预防作用。
    结果:与他汀类药物相关的潜在心脏保护机制尚未完全阐明。已经提出了与抑制Ras同源(Rho)GTP酶相关的关键机制。来自观察性研究的数据支持他汀类药物在蒽环类药物引起的心脏毒性的一级预防中的有益作用。最近,几项研究他汀类药物在蒽环类药物引起的心脏毒性一级预防中的作用的随机对照试验产生了对比结果.在接受蒽环类药物的癌症患者中,他汀类药物与心脏功能障碍的风险较低相关。需要进行更大的随机对照试验和更长的随访期的进一步研究,以更好地评估他汀类药物治疗的长期作用,并确定从他汀类药物治疗中受益最大的亚组。
    OBJECTIVE: The aim of this review is two-fold: (1) To examine the mechanisms by which statins may protect from anthracycline-induced cardiotoxicity and (2) To provide a comprehensive overview of the existing clinical literature investigating the role of statins for the primary prevention of anthracycline-induced cardiotoxicity.
    RESULTS: The underlying cardioprotective mechanisms associated with statins have not been fully elucidated. Key mechanisms related to the inhibition of Ras homologous (Rho) GTPases have been proposed. Data from observational studies has supported the beneficial role of statins for the primary prevention of anthracycline-induced cardiotoxicity. Recently, several randomized controlled trials investigating the role of statins for the primary prevention of anthracycline-induced cardiotoxicity have produced contrasting results. Statins have been associated with a lower risk of cardiac dysfunction in cancer patients receiving anthracyclines. Further investigation with larger randomized control trials and longer follow-up periods are needed to better evaluate the long-term role of statin therapy and identify the subgroups who benefit most from statin therapy.
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  • 文章类型: Journal Article
    背景:淀粉样变性是由不溶性错误折叠蛋白的细胞外沉积引起的一组异质性疾病,导致终末器官损伤。转甲状腺素蛋白淀粉样心肌病(ATTR-CM)是一种亚型,其中称为转甲状腺素蛋白的蛋白质在心脏组织内积累,逐渐导致限制性心肌病和心力衰竭。由于ATTR-CM的渐进性,临床管理需要有效的治疗方案来控制衰弱状况,Tafamidis在这方面显示出有希望的结果.
    方法:ATTR-CM由于其性质和有限的治疗选择而提出了重大挑战。Tafamidis是一种新颖的疗法,旨在稳定运甲状腺素蛋白四聚体,抑制淀粉样纤维的形成。它已成为一种有前途的治疗方法,也是唯一获得FDA批准的ATTR-CM药物。Tafamidis在减缓ATTR-CM患者的疾病进展和改善预后方面的作用已在主要的随机对照试验ATTR-ACT中得到证实,具有有希望的开放标签扩展研究。一些仍在进行中。此外,真实世界的证据支持它在临床实践中的使用,显示其在降低与这种情况相关的发病率和死亡率方面的作用。临床证据表明其在改善患者症状和心功能方面的功效。案例研究还揭示了对患者的显着益处,例如减少心肌损伤,心房颤动逆转,和心力衰竭症状的解决。真实世界的结果和临床试验显示淀粉样蛋白沉积持续减少,心血管相关的住院,以及Tafamidis治疗的全因死亡率。
    结论:Tafamidis是ATTR-CM治疗的重要组成部分,本叙述性综述综合了目前有关安全性的证据,功效,以及在实际实践中的利用。虽然它显示出有希望的效果,其有效性也可能有所不同,高成本阻碍了现实世界的大规模研究。总的来说,Tafamidis成为管理ATTR-CM的有价值的治疗选择。
    BACKGROUND: Amyloidosis is a heterogeneous group of disorders caused by the extracellular deposition of insoluble misfolded proteins, leading to end-organ damage. Transthyretin amyloid cardiomyopathy (ATTR-CM) is a subtype in which a protein known as transthyretin accumulates within the heart tissue, progressively resulting in restrictive cardiomyopathy and heart failure. Due to the progressive nature of ATTR-CM, clinical management requires efficacious regimens to manage the debilitating condition and Tafamidis shows promising results in this regard.
    METHODS: ATTR-CM poses a significant challenge due to its nature and limited therapeutic options. Tafamidis is a novel therapy designed to stabilize the transthyretin tetramers, inhibiting the formation of amyloid fibrils. It has emerged as a promising treatment and the only FDA-approved drug for ATTR-CM. Tafamidis\' role in slowing disease progression and improving outcomes in patients with ATTR-CM has been demonstrated in the major randomized control trial ATTR-ACT with promising open-label extension studies, some still ongoing. Additionally, real-world evidence supports its use in clinical practice, showing its role in reducing morbidity and mortality associated with this condition. Clinical evidence shows its efficacy in improving symptoms and cardiac function in patients. Case studies also reveal significant benefits to patients like reducing myocardial damage, reversal of atrial fibrillation, and resolution of heart failure symptoms. Real-world outcomes and clinical trials show a consistent reduction in amyloid deposition, cardiovascular-related hospitalizations, and all-cause mortality with Tafamidis therapy.
    CONCLUSIONS: Tafamidis is an essential component of the treatment of ATTR-CM and this narrative review synthesizes the current evidence regarding safety, efficacy, and utilization in real practice. While it shows promising effects, its effectiveness may also vary and high cost precludes real-world large-scale studies. Overall, Tafamidis emerges as a valuable therapeutic option for managing ATTR-CM.
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  • 文章类型: Journal Article
    心血管和癌症结果在心脏肿瘤生存护理领域相交,以种族差异为标志,种族,社会,和地理景观。尽管临床界越来越意识到这个复杂的问题,有效的解决方案正在落后。为了实现重大的公共卫生影响,癌症类型的检查和心血管风险缓解需要互补的方法来引出患者的观点,将其扩展到人口水平,并专注于可行的人群健康干预措施。采用这种多学科方法将加深我们对患者意识的理解,动机,健康素养,和社区资源,以解决心脏肿瘤学的独特挑战。地理空间分析有助于在粒度和更广泛的背景下确定需要的关键社区。在这次审查中,我们描绘了一条从个人到社区层面的障碍。从这些角度收集的数据对于告知干预措施至关重要,这些干预措施可以增强不同社区内的个人能力并改善心脏肿瘤的生存率。
    Cardiovascular and cancer outcomes intersect within the realm of cardio-oncology survivorship care, marked by disparities across ethnic, racial, social, and geographical landscapes. Although the clinical community is increasingly aware of this complex issue, effective solutions are trailing. To attain substantial public health impact, examinations of cancer types and cardiovascular risk mitigation require complementary approaches that elicit the patient\'s perspective, scale it to a population level, and focus on actionable population health interventions. Adopting such a multidisciplinary approach will deepen our understanding of patient awareness, motivation, health literacy, and community resources for addressing the unique challenges of cardio-oncology. Geospatial analysis aids in identifying key communities in need within both granular and broader contexts. In this review, we delineate a pathway that navigates barriers from individual to community levels. Data gleaned from these perspectives are critical in informing interventions that empower individuals within diverse communities and improve cardio-oncology survivorship.
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