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Promotion of Cardiometabolic Health: A Personal Journey. 促进心脏代谢健康:个人旅程。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-14 DOI: 10.1007/s11886-024-02165-9
Jean-Pierre Després

PURPOSE OF REVIEW: Narrative review of the author's main contributions to the field of cardiovascular health spanning four decades, with a focus on findings related to 1- the pathophysiology of obesity, insulin resistance, type 2 diabetes and cardiovascular disease, and 2- the management/prevention of these conditions. Particular attention is given to the importance of regular physical activity. RECENT FINDINGS: Because behaviors and their physiological consequences are still not measured in clinical practice, it is proposed to systematically assess and target "lifestyle vital signs" (waist circumference, cardiorespiratory fitness, food-based diet quality and level of leisure-time physical activity) in primary care. However, cardiometabolic health of the population will not be substantially improved until our living and economic conditions are permissive to the adoption of healthy behaviors. Closing the gap between primary care and public health should be a priority to address the socioeconomic determinants of cardiometabolic health.

综述目的:叙述性综述作者四十年来在心血管健康领域做出的主要贡献,重点是 1- 肥胖、胰岛素抵抗、2 型糖尿病和心血管疾病的病理生理学,以及 2- 这些疾病的管理/预防。特别关注定期体育锻炼的重要性。最新发现:由于行为及其生理后果在临床实践中仍未得到测量,因此建议在初级保健中系统地评估 "生活方式生命体征"(腰围、心肺功能、以食物为基础的饮食质量和业余体育锻炼水平)并将其作为目标。然而,只有在我们的生活和经济条件允许人们采取健康行为的情况下,人口的心脏代谢健康才能得到实质性改善。缩小初级保健与公共卫生之间的差距应成为解决心脏代谢健康的社会经济决定因素的优先事项。
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引用次数: 0
Metabolic Dysfunction Associated-Steatotic Liver Disease (MASLD) and Cardiovascular Risk: Embrace All Facets of the Disease. 代谢功能障碍相关的脂肪变性肝病(MASLD)和心血管风险:包括疾病的所有方面。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-13 DOI: 10.1007/s11886-024-02181-9
Niki Katsiki, Genovefa Kolovou, Michal Vrablik

Purpose of review: In recent years, the terms "metabolic associated fatty liver disease-MAFLD" and "metabolic dysfunction-associated steatotic liver disease-MASLD" were introduced to improve the encapsulation of metabolic dysregulation in this patient population, as well as to avoid the negative/stigmatizing terms "non-alcoholic" and "fatty".

Recent findings: There is evidence suggesting links between MASLD and coronary heart disease (CHD), heart failure (HF), atrial fibrillation (AF), stroke, peripheral artery disease (PAD) and chronic kidney disease (CKD), although the data for HF, AF, stroke and PAD are scarcer. Physicians should consider the associations between MASLD and CV diseases in their daily practice. Based on this knowledge and current guidelines, they should also assess and manage CV risk/co-morbidities in such patients. It is important to further investigate the impact of MASLD on CV outcomes, a knowledge that will help to elucidate the clinical implications of this "novel" liver entity.

综述目的:近年来,“代谢性脂肪性肝病- mafld”和“代谢性功能障碍相关脂肪性肝病- masld”这两个术语被引入,以改善对该患者群体代谢失调的概括,并避免“非酒精性”和“脂肪性”这两个负面/污名化的术语。最近的研究发现:有证据表明MASLD与冠心病(CHD)、心力衰竭(HF)、心房颤动(AF)、中风、外周动脉疾病(PAD)和慢性肾脏疾病(CKD)之间存在联系,尽管关于HF、AF、中风和PAD的数据较少。医生应该在日常实践中考虑MASLD和心血管疾病之间的关系。基于这些知识和当前的指南,他们还应该评估和管理这些患者的心血管风险/合并症。进一步研究MASLD对CV结果的影响是很重要的,这将有助于阐明这种“新型”肝脏实体的临床意义。
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引用次数: 0
Role of Trained Immunity in Heath and Disease. 训练免疫在健康和疾病中的作用。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-13 DOI: 10.1007/s11886-024-02167-7
Shibali Das, Kory J Lavine

Purpose of review: This review aims to explore the role of immune memory and trained immunity, focusing on how innate immune cells like monocytes, macrophages, and natural killer cells undergo long-term epigenetic and metabolic rewiring. Specifically, it examines the mechanisms by which trained immunity, often triggered by infection or vaccination, could impact cardiac processes and contribute to both protective and pathological responses within the cardiovascular system.

Recent findings: Recent research demonstrates that vaccination and infection not only activate immune responses in circulating monocytes and tissue macrophages but also affect immune progenitor cells within the bone marrow environment, conferring lasting protection against heterologous infections. These protective effects are attributed to epigenetic and metabolic reprogramming, which enable a heightened immune response upon subsequent encounters with pathogens. However, while trained immunity is beneficial in combating infections, it has been linked to exacerbated inflammation, which may increase susceptibility to cardiovascular diseases, including atherosclerosis and heart failure. Our review highlights the dual nature of trained immunity: while it offers protective advantages against infections, it also poses potential risks for cardiovascular health by promoting chronic inflammation. Understanding the molecular mechanisms underlying immune memory's impact on cardiac processes could lead to new therapeutic strategies to mitigate cardiovascular diseases, such as atherosclerosis, heart failure, and diabetes. These insights build the grounds for future research to balance the benefits of trained immunity with its potential risks in cardiovascular disease management.

综述的目的:这篇综述旨在探讨免疫记忆和训练有素的免疫力的作用,重点关注先天性免疫细胞(如单核细胞、巨噬细胞和自然杀伤细胞)如何进行长期的表观遗传和代谢重构。具体来说,该研究将探讨训练有素的免疫(通常由感染或接种疫苗引发)可能影响心脏过程并导致心血管系统内保护性和病理性反应的机制:最近的研究表明,疫苗接种和感染不仅会激活循环单核细胞和组织巨噬细胞的免疫反应,还会影响骨髓环境中的免疫祖细胞,从而对异源感染产生持久的保护作用。这些保护作用归功于表观遗传学和新陈代谢的重编程,从而在以后遇到病原体时能够增强免疫反应。然而,训练有素的免疫力虽然有利于对抗感染,但也与炎症加剧有关,这可能会增加对动脉粥样硬化和心力衰竭等心血管疾病的易感性。我们的综述强调了训练免疫的双重性:它在提供抗感染保护性优势的同时,也通过促进慢性炎症对心血管健康构成潜在风险。了解免疫记忆对心脏过程产生影响的分子机制,可以为缓解动脉粥样硬化、心力衰竭和糖尿病等心血管疾病提供新的治疗策略。这些见解为未来的研究奠定了基础,以平衡训练有素的免疫力在心血管疾病治疗中的益处和潜在风险。
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引用次数: 0
Old and New Biomarkers in Idiopathic Recurrent Acute Pericarditis (IRAP): Prognosis and Outcomes. 特发性复发性急性心包炎(IRAP)的新旧生物标志物:预后和结局。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-11 DOI: 10.1007/s11886-024-02170-y
Ruggiero Mascolo, Emanuele Bizzi, Martina Martelli, Chiara Facoetti, Giulia Colazzo, Fabio Barone, Antonio Brucato

Purpose of review: To outline the latest discoveries regarding the utility and reliability of serum biomarkers in idiopathic recurrent acute pericarditis (IRAP), considering recent findings on its pathogenesis. The study highlights the predictive role of these biomarkers in potential short- (cardiac tamponade, recurrences) and long-term complications (constrictive pericarditis, death).

Recent findings: The pathogenesis of pericarditis has been better defined in recent years, focusing on the autoinflammatory pathway. New studies have demonstrated the pivotal role of the classical inflammatory biomarkers in distinguishing pericarditis phenotypes (high-grade vs. low-grade inflammation) and in defining outcomes of this condition. Pericarditis involves intense inflammatory activity, which causes elevation of different markers, such as C-reactive protein, erythrocyte sedimentation rate, neutrophils and platelets, serum amyloid A and D-Dimer. Conversely, lymphocytes are often reduced, as well as hemoglobin during the acute phase. Cardiac troponins T and I are elevated in up to 30% of cases. A Biomarker for CRP-negative cases is needed. Other markers have been proposed for diagnosis and prognosis in IRAP, such as anti-heart antibodies and anti-intercalated disk antibodies, but we need further studies to validate them.

综述的目的:考虑到最近在特发性复发性急性心包炎(IRAP)发病机制方面的发现,概述了关于血清生物标志物在IRAP中的实用性和可靠性的最新发现。该研究强调了这些生物标志物在潜在的短期(心脏填塞、复发)和长期并发症(缩窄性心包炎、死亡)中的预测作用。近年来心包炎的发病机制得到了更好的定义,主要集中在自身炎症途径上。新的研究已经证明了经典炎症生物标志物在区分心包炎表型(高级别与低级别炎症)和确定心包炎结局方面的关键作用。心包炎涉及强烈的炎症活动,引起不同标志物的升高,如c反应蛋白、红细胞沉降率、中性粒细胞和血小板、血清淀粉样蛋白A和d -二聚体。相反,淋巴细胞和血红蛋白在急性期经常减少。心脏肌钙蛋白T和I在高达30%的病例中升高。需要一种crp阴性病例的生物标志物。其他的诊断和预后指标,如抗心脏抗体和抗嵌入盘抗体,已被提出,但我们需要进一步的研究来验证它们。
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引用次数: 0
Atrial Functional Mitral Regurgitation: Definition, Mechanisms, and Treatment Perspectives. 心房功能性二尖瓣返流:定义、机制和治疗前景。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-11 DOI: 10.1007/s11886-024-02188-2
Sebastiaan Dhont, Sébastien Deferm, Philippe B Bertrand, Pieter M Vandervoort

Purpose of review: This review aims to explore the complex interplay between atrial functional mitral regurgitation (AFMR), atrial fibrillation (AF), and heart failure with preserved ejection fraction (HFpEF). The goal is to define these conditions, examine their underlying mechanisms, and discuss treatment perspectives, particularly addressing diagnostic challenges.

Recent findings: Recent research highlights the rising prevalence of AFMR, now accounting for nearly one-third of significant mitral regurgitation cases. Advances in percutaneous treatment options have improved management for vulnerable HFpEF patients, but long-term outcomes remain unclear, and symptom relief is inconsistent. AF and HFpEF share the left atrium as a common pathological substrate, with progressive LA remodeling contributing to AFMR. Diagnostic limitations hinder effective symptom management with current mitral valve interventions. Future research should focus on better diagnostic tools to determine the contributions of valvular disease, arrhythmia, or myocardial dysfunction to clinical outcomes, as we currently lack definitive tests to establish this connection.

综述目的:本综述旨在探讨心房功能性二尖瓣反流(AFMR)、心房颤动(AF)和保留射血分数(HFpEF)心力衰竭之间的复杂相互作用。目的是定义这些疾病,检查其潜在机制,并讨论治疗前景,特别是解决诊断方面的挑战。最近的发现:最近的研究强调了AFMR的患病率上升,现在占近三分之一的重大二尖瓣反流病例。经皮治疗方案的进步改善了易感HFpEF患者的治疗,但长期结局尚不清楚,症状缓解也不一致。房颤和HFpEF都是共同的左心房病理底物,进行性LA重塑导致房颤。诊断的局限性阻碍了当前二尖瓣介入治疗的有效症状管理。未来的研究应侧重于更好的诊断工具,以确定瓣膜性疾病、心律失常或心肌功能障碍对临床结果的影响,因为我们目前缺乏明确的测试来建立这种联系。
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引用次数: 0
Cardiac Regeneration in Adult Zebrafish: A Review of Signaling and Metabolic Coordination. 成年斑马鱼的心脏再生:信号和代谢协调的综述。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-10 DOI: 10.1007/s11886-024-02162-y
Arkadeep Mitra, Subhadeep Mandal, Kalyan Banerjee, Nilanjan Ganguly, Pramit Sasmal, Durba Banerjee, Shreyasi Gupta

Purpose of review: This review investigates how post-injury cellular signaling and energy metabolism are two pivotal points in zebrafish's cardiomyocyte cell cycle re-entry and proliferation. It seeks to highlight the probable mechanism of action in proliferative cardiomyocytes compared to mammals and identify gaps in the current understanding of metabolic regulation of cardiac regeneration.

Recent findings: Metabolic substrate changes after birth correlate with reduced cardiomyocyte proliferation in mammals. Unlike adult mammalian hearts, zebrafish can regenerate cardiomyocytes by re-entering the cell cycle, characterized by a metabolic switch from oxidative metabolism to increased glycolysis. Zebrafish provide a valuable model for studying metabolic regulation during cell cycle re-entry and cardiac regeneration. Proliferative cardiomyocytes have upregulated Notch, hippo, and Wnt signaling and decreased ROS generation, DNA damage in different zebrafish cardiac regeneration models. Understanding the correlation between metabolic switches during cell cycle re-entry of already differentiated zebrafish cardiomyocytes is being increasingly recognized as a critical factor in heart regeneration. Zebrafish studies provide insights into metabolic adaptations during heart regeneration, emphasizing the importance of a metabolic switch. However, there are mechanistic gaps, and extensive studies are required to aid in formulating therapeutic strategies for cardiac regenerative medicine.

综述目的:本文综述了斑马鱼心肌细胞损伤后信号转导和能量代谢在细胞周期再进入和增殖过程中的两个关键环节。它旨在强调与哺乳动物相比,增生性心肌细胞的可能作用机制,并确定目前对心脏再生代谢调节的理解中的空白。最新发现:哺乳动物出生后代谢底物的变化与心肌细胞增殖减少有关。与成年哺乳动物的心脏不同,斑马鱼可以通过重新进入细胞周期来再生心肌细胞,其特征是从氧化代谢到增加糖酵解的代谢转换。斑马鱼为研究细胞周期再进入和心脏再生过程中的代谢调节提供了有价值的模型。在不同的斑马鱼心脏再生模型中,增殖性心肌细胞上调Notch、hippo和Wnt信号,减少ROS的产生和DNA损伤。了解已经分化的斑马鱼心肌细胞在细胞周期再进入过程中代谢开关之间的相关性越来越被认为是心脏再生的关键因素。斑马鱼的研究为心脏再生过程中的代谢适应提供了见解,强调了代谢开关的重要性。然而,存在机制上的差距,需要广泛的研究来帮助制定心脏再生医学的治疗策略。
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引用次数: 0
Tailored Approach to Temporary Mechanical Circulatory Support for Cardiogenic Shock: Strategies to Facilitate Patient Mobilization. 心源性休克临时机械循环支持的量身定制方法:促进患者活动的策略。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-10 DOI: 10.1007/s11886-024-02152-0
Jean-Luc A Maigrot, Brett J Wakefield, Chase M Donaldson, Aaron J Weiss

Purpose of review: This article discusses a tailored approach to managing cardiogenic shock and temporary mechanical circulatory support (tMCS). We also outline specific mobilization strategies for patients with different tMCS devices and configurations, which can be enabled by this tailored approach to cardiogenic shock management.

Recent findings: Safe and effective mobilization of patients with cardiogenic shock receiving tMCS can be accomplished. Appropriate patient selection, tailored device management, and dynamic multidisciplinary approaches to mobilization are critical to success. Cardiogenic shock is a heterogeneous condition characterized by end-organ dysfunction due to hypoperfusion and low cardiac output. Temporary mechanical circulatory support (tMCS) is an increasingly valuable tool in managing these patients, with various devices and configurations available. Critically ill patients receiving tMCS are at risk for complications and deconditioning associated with prolonged bed rest, making it essential to implement strategies that promote mobility when feasible. We advocate for a tailored approach to the selection and management of tMCS in patients with cardiogenic shock. This approach focuses on the early identification of patients who may benefit from tMCS before further deterioration, alongside the selection of devices that provide ventricular-specific support and facilitate upper-body cannulation to enhance mobilization while also considering patients' potential exit strategies from tMCS. Understanding this approach is vital to appropriately facilitating safe and effective mobilization.

综述目的:本文讨论了一种治疗心源性休克和临时机械循环支持(tMCS)的量身定制的方法。我们还概述了不同tMCS设备和配置的患者的具体动员策略,这可以通过这种量身定制的方法来实现心源性休克管理。最近研究发现:接受tMCS的心源性休克患者可以安全有效地动员。适当的患者选择、量身定制的器械管理和动态的多学科方法是成功的关键。心源性休克是一种异质性疾病,其特征是由于灌注不足和心输出量低而引起的终末器官功能障碍。临时机械循环支持(tMCS)是管理这些患者越来越有价值的工具,有各种设备和配置可用。接受tMCS治疗的危重患者存在与长时间卧床休息相关的并发症和身体状况恶化的风险,因此在可行的情况下实施促进活动的策略至关重要。我们提倡对心源性休克患者的tMCS进行量身定制的选择和管理。该方法侧重于在tMCS进一步恶化之前早期识别可能受益于tMCS的患者,同时选择提供心室特异性支持和促进上肢插管的设备以增强活动能力,同时考虑患者从tMCS退出的潜在策略。了解这一方法对于适当促进安全和有效的动员至关重要。
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引用次数: 0
Hot Phases Cardiomyopathy: Pathophysiology, Diagnostic Challenges, and Emerging Therapies. 热期心肌病:病理生理学、诊断挑战和新兴疗法。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-09 DOI: 10.1007/s11886-024-02168-6
Giulia Bassetto, Federico Angriman, Carola Pio Loco Detto Gava, Alessia Paldino, Maria Perotto, Luca Bordignon, Marta Gigli, Matteo Dal Ferro, Laura Massa, Alessandro Altinier, Antonio De Luca, Gianfranco Sinagra, Marco Merlo

Purpose of review: Hot phases are a challenging clinical presentation in arrhythmogenic cardiomyopathy (ACM), marked by acute chest pain and elevated cardiac troponins in the absence of obstructive coronary disease. These episodes manifest as myocarditis and primarily affect young patients, contributing to a heightened risk of life-threatening arrhythmias and potential disease progression. This review aims to synthesize recent research on the pathophysiology, diagnostic challenges, and therapeutic management of hot phases in ACM.

Recent findings: Hot phases have been linked to genetic mutations, particularly in desmosomal proteins such as Desmoplakin (DSP). Diagnostic challenges include differentiating hot phases from isolated acute myocarditis, through identification of red flags and a multimodal approach, including CMR, FDG-PET, endomyocardial biopsy and genetic testing. Emerging therapies, such as immunosuppressive and anti-inflammatory treatments, show promise in managing hot-phase episodes. Hot phases in ACM present a significant risk for arrhythmias and disease progression, necessitating a comprehensive diagnostic and therapeutic management. A multimodal diagnostic approach is essential for accurate diagnosis, but further research is needed to refine these strategies and improve prognosis for affected patients.

回顾目的:热期是心律失常性心肌病(ACM)的一个具有挑战性的临床表现,在没有阻塞性冠状动脉疾病的情况下,以急性胸痛和心肌肌钙蛋白升高为特征。这些发作表现为心肌炎,主要影响年轻患者,增加了危及生命的心律失常和潜在疾病进展的风险。本文综述了ACM热期的病理生理学、诊断挑战和治疗管理方面的最新研究。最近的发现:热期与基因突变有关,特别是在桥粒蛋白中,如桥粒蛋白(DSP)。诊断挑战包括通过识别危险信号和多模式方法(包括CMR、FDG-PET、心内膜活检和基因检测),从孤立的急性心肌炎中区分热期。新兴疗法,如免疫抑制和抗炎治疗,在治疗热期发作方面显示出希望。ACM的热期存在心律失常和疾病进展的重大风险,需要全面的诊断和治疗管理。多模式诊断方法对于准确诊断至关重要,但需要进一步研究来完善这些策略并改善受影响患者的预后。
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引用次数: 0
Right Ventricle Function: The Role of the Forgotten Chamber in Mitral Valve Surgery. 右心室功能:遗忘腔在二尖瓣手术中的作用。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-09 DOI: 10.1007/s11886-024-02151-1
Beatrice Bacchi, Andrea Stefanini, Giulia Elena Mandoli, Federica Lorusso, Gianmarco Toto, Maria Concetta Pastore, Francesco Cabrucci, Massimo Bonacchi, Matteo Cameli, Gianluigi Bisleri

Background: Referred to as the "forgotten chamber," the right ventricle (RV) is now widely acknowledged as a significant factor, particularly in certain cardiovascular pathologies. Despite historically being undervalued in comparison to the left ventricle, the RV function is deemed crucial in determining patient outcomes following mitral valve (MV) surgery. In the context of MV surgery, it is important to note that the RV is highly susceptible to dysfunction before, during, and after the surgical procedure. This vulnerability is also partly compounded by a lack of precise preoperative assessment, appropriate intraoperative management, and sufficient postoperative care for the RV. Moreover, it is notable that the current preoperative risk-score evaluation does not encompass considerations for the RV.

Observations: Sophisticated assessment methodologies, including echocardiography, cardiac magnetic resonance imaging, and invasive hemodynamic procedures, play a pivotal role in accurately evaluating the RV function in patients undergoing MV surgery. These methodologies offer invaluable insights into the extent of RV dysfunction both pre- and postoperatively. By furnishing precise measurements of RV performance, these techniques contribute to risk stratification, guide perioperative management, and may enhance surgical outcomes. Their integration into routine clinical practice is essential for optimizing patient care in the context of MV surgery.

Conclusions: This review highlights the importance of evaluating the RV before surgery, ensuring proper perioperative care, and utilizing advanced imaging to monitor RV function in order to predict the outcomes. The goal is to enhance surgical outcomes by thoroughly assessing and supporting RV function during the surgical process.

背景:右心室(RV)被称为“被遗忘的心室”,现在被广泛认为是一个重要的因素,特别是在某些心血管疾病中。尽管与左心室相比,右心室功能历来被低估,但在二尖瓣手术后,右心室功能被认为是决定患者预后的关键。在中心室手术的背景下,重要的是要注意右心室在手术前、手术中和手术后极易发生功能障碍。由于缺乏精确的术前评估、适当的术中管理和足够的右心室术后护理,这种脆弱性也在一定程度上加剧了。此外,值得注意的是,目前的术前风险评分评估并没有考虑到RV。观察:复杂的评估方法,包括超声心动图、心脏磁共振成像和侵入性血流动力学检查,在准确评估中心室手术患者的右心室功能方面起着关键作用。这些方法为术前和术后右心室功能障碍的程度提供了宝贵的见解。通过精确测量右心室功能,这些技术有助于风险分层,指导围手术期管理,并可能提高手术效果。将它们整合到常规临床实践中对于优化中压手术患者护理至关重要。结论:本综述强调了术前评估右心室的重要性,确保适当的围手术期护理,并利用先进的成像监测右心室功能,以预测预后。目的是通过在手术过程中全面评估和支持右心室功能来提高手术效果。
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引用次数: 0
Desmoplakin Cardiomyopathy: Role of Inflammation and Potential Role of Disease-Modifying Therapies. Desmoplakin心肌病:炎症的作用和疾病改善治疗的潜在作用。
IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-09 DOI: 10.1007/s11886-024-02183-7
Alessio Gasperetti, Richard T Carrick, Steven Muller, Brittney Murray, Luigi Adamo, Barbara Bauce, Elizabeth McNally, Adam Helms

Purpose of review: To summarize the available data on the use of immunosuppression therapies for the management of hot phases of disease and recurrent myocarditis in patients with desmoplakin cardiomyopathy (DSP-CMP).

Recent findings: Occurrence of myocarditis episodes has been associated with worsening of outcomes in DSP-CMP. Multiple case reports and small case series have described potential benefit in using anti-inflammatory and immunosuppressive medications for the treatment of those episodes. Recently published translational data has shown a clear link between DSP variants and a propensity to inflammation. The presence of acute myocarditis episodes have been clearly demonstrated as a manifestation of DSP-CMP. These episodes are marked by myocardial inflammation and subsequent fibrosis, and, moreover, contribute to a heightened future risk of subsequent arrhythmias and heart failure. Identifying optimal strategies to prevent and/or interrupt myocardial inflammation is therefore a critical priority of patients with DSP-CMP.

综述目的:总结利用免疫抑制疗法治疗desmoplakin心肌病(DSP-CMP)患者疾病热期和复发性心肌炎的现有资料。最近发现:心肌炎发作的发生与spd - cmp预后的恶化有关。多个病例报告和小病例系列描述了使用抗炎和免疫抑制药物治疗这些发作的潜在益处。最近发表的翻译数据显示了DSP变异和炎症倾向之间的明确联系。急性心肌炎发作的存在已被清楚地证明是DSP-CMP的表现。这些发作的标志是心肌炎症和随后的纤维化,而且,有助于提高未来发生心律失常和心力衰竭的风险。因此,确定预防和/或中断心肌炎症的最佳策略是DSP-CMP患者的关键优先事项。
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引用次数: 0
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