Arrhythmogenic Right Ventricular Dysplasia

致心律失常性右心室发育不良
  • 文章类型: Editorial
    暂无摘要。
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    暂无摘要。
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    特种作战服务人员表现出心悸,晕厥前,在严格的体育锻炼中或劳累性晕厥通常会出现良性状况;然而,应考虑危及生命的病因。我们描述了一位43岁的特殊操作员,他在选择身体评估测试过程中表现出心悸和头晕,随后的检查显示心律失常性右室心肌病(ARVC)。他的初始心电图无异常,无特征性ARVC变化。通过动态心脏监测进行的门诊评估记录了许多非持续性室性心动过速发作。经胸超声心动图显示关于ARVC的发现,随后的心脏MRI通过2020帕多瓦标准确认诊断。管理包括活动修改,III类抗心律失常药物,以及可能放置植入式心律转复除颤器以防止心源性猝死。此病例证明了对表现为劳累性心悸的罕见诊断保持高度临床怀疑的重要性。如致心律失常性右心室心肌病,即使是我们最合适的特殊运营商。
    Special Operations Servicemembers presenting with palpitations, pre-syncope, or exertional syncope during rigorous physical training are often experiencing a benign condition; however, life-threatening etiologies should be considered. We describe a 43-year-old Special Operator who presented to his medics during selection physical assessment testing with palpitations and lightheadedness, with a subsequent workup revealing arrhythmogenic right ventricular cardiomyopathy (ARVC). His initial electrocardiogram was unremarkable without characteristic ARVC changes. Outpatient evaluation with ambulatory cardiac monitoring recorded numerous episodes of non-sustained ventricular tachycardia. Transthoracic echocardiography demonstrated findings concerning for ARVC, with subsequent cardiac MRI confirming the diagnosis via the 2020 Padua criteria. Management includes activity modification, class III anti-arrhythmic medications, and possible placement of an implantable cardioverter defibrillator to prevent sudden cardiac death. This case demonstrates the importance of maintaining high clinical suspicion for rare diagnoses that present with exertional palpitations, such as arrhythmogenic right ventricular cardiomyopathy, in even our fittest Special Operators.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    背景:致心律失常性右心室心肌病(ARVC)是一种罕见的遗传性疾病,其特征是右心室心肌的纤维脂肪替代,这会使个体容易出现危及生命的心律失常。此病例描述了一名ARVC患者,该患者反复发作持续性室性心动过速(VT)。在这种情况下,主要探讨心肌超声造影(MCE)在显示ARVC患者心肌纤维化中的应用。
    方法:一名43岁的男性在8年时间里经历了3次不明原因的VT发作,伴有胸部不适的症状,心悸和头晕。冠状动脉造影显示冠状动脉无明显狭窄。心电图(ECG)结果显示右心前导联的特征性epsilon波,随后的超声心动图发现右心室扩大和右心室收缩功能障碍。MCE进一步公开了在左心室心尖的心外膜的局部心肌缺血。最终,心血管磁共振成像(CMR)证实了ARVC的诊断,在延迟增强期间突出右心室的线性增强。
    结论:及时识别ARVC对于及时干预和管理至关重要。MCE可能为检测ARVC患者的心肌受累提供有效且有价值的技术。
    BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an infrequent hereditary disorder distinguished by fibrofatty replacement of the myocardium in the right ventricular, which predisposes individuals to life-threatening arrhythmias. This case delineates an ARVC patient who suffered recurrent bouts of sustained ventricular tachycardia (VT). In this case, we mainly discuss the application of myocardial contrast echocardiography (MCE) in displaying myocardial fibrosis in patients with ARVC.
    METHODS: A 43-year-old male experienced three episodes of unexplained VT over an eight-year period, accompanied by symptoms of chest discomfort, palpitations and dizziness. Coronary angiography revealed no significant coronary stenosis. The electrocardiogram (ECG) results indicated characteristic epsilon waves in right precordial leads, and subsequent echocardiography identified right ventricular enlargement and right ventricular systolic dysfunction. MCE further disclosed regional myocardial ischemia at the epicardium of the left ventricular apex. Ultimately, cardiovascular magnetic resonance imaging (CMR) corroborated the ARVC diagnosis, highlighting linear intensification in the right ventricle during the delayed enhancement.
    CONCLUSIONS: Prompt identification of ARVC is crucial for timely intervention and management. MCE may offer an effective and valuable technique for the detection of myocardial involvement in ARVC patient.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    背景:致心律失常性右心室心肌病(ARVC),或最近被称为心律失常性心肌病(ACM),是一种遗传性心肌疾病,其特征是进行性纤维脂肪替代心肌和室性心律失常和心源性猝死(SCD)的风险。我们报告了一个案例研究,以证明基因突变检测在诊断为ARVC的年轻患者的SCD一级预防风险分层中的作用。
    方法:一名15岁的亚洲(越南)男性患者因心悸入院,无快速性心律失常或晕厥病史,有潜在SCD家族史。包括心脏磁共振成像(MRI)在内的临床发现和检查高度提示ARVC。基因测序用于SCD风险分层,发现PKP2基因突变。基于个性化的风险分层,植入ICD用于SCD一级预防.ICD植入后6个月,装置检测到并成功实施了适当的电击,以终止潜在致命性室性心律失常的发作.因此,ICD植入被证明对该患者是合适的。
    结论:虽然根据2010年工作组标准和最近的临床指南,已知基因突变是诊断ARVC的重要因素,它们在SCD危险分层中的作用仍存在争议.我们的案例表明,当与其他临床因素和家族史一起使用时,这些信息可能有助于确定ICD植入的适当适应症.
    BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC), or more recently known as arrhythmogenic cardiomyopathy (ACM), is an heritable disorder of the myocardium characterized by progressive fibrofatty replacement the heart muscle and risk of ventricular arrhythmias and sudden cardiac death (SCD). We report a case study to demonstrate the role of gene mutation detection in risk stratification for primary prevention of SCD in a young patient diagnosed with ARVC.
    METHODS: A 15-year-old Asian (Vietnamese) male patient with no history of documented tachyarrhythmia or syncope and a family history of potential SCD was admitted due to palpitations. Clinical findings and work-up including cardiac magnetic resonance imaging (MRI) were highly suggestive of ARVC. Gene sequencing was performed for SCD risk stratification, during which PKP2 gene mutation was found. Based on the individualized risk stratification, an ICD was implanted for primary prevention of SCD. At 6 months post ICD implantation, the device detected and successfully delivered an appropriate shock to terminate an episode of potentially fatal ventricular arrhythmia. ICD implantation was therefore proven to be appropriate in this patient.
    CONCLUSIONS: While gene mutations are known to be an important factor in the diagnosis of ARVC according to the 2010 Task Force Criteria and recent clinical guidelines, their role in risk stratification of SCD remains controversial. Our case demonstrated that when used with other clinical factors and family history, this information could be helpful in identifying appropriate indication for ICD implantation.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    背景:V1-V3中T波倒置的心电图(ECG)发现,有或没有伴随的ε波,经常引起人们对稀有事物的关注,但可能是致命的,致心律失常性右心室心肌病(ARVC)。然而,这种模式可能在心包发育不全中发现,更罕见的病理学。合并心肌炎可以进一步混淆这种表现。
    方法:我们报告一例以前健康的男性,他出现左侧胸痛,心电图结果提示ARVC,最终诊断为心肌炎并伴有部分心包发育不全。为什么急诊医生应该意识到这一点?:越来越多的病例报告心包发育不全,其心电图改变类似于ARVC。我们讨论了一种诊断挑战性患者的方法。这个案例强调了广泛差异的重要性和过早关闭的危险。
    BACKGROUND: Electrocardiographic (ECG) findings of T-wave inversions in V1-V3, with or without accompanying epsilon waves, often raise concerns for the rare, but potentially lethal, arrhythmogenic right ventricular cardiomyopathy (ARVC). However, this pattern may be found in pericardial agenesis, an even rarer pathology. Concomitant myocarditis can confuse this presentation further.
    METHODS: We report a case of a previously healthy man who presented with left-sided chest pain, ECG findings suggestive of ARVC, and a final diagnosis of myocarditis with underlying partial pericardial agenesis. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: A growing number of cases have reported pericardial agenesis demonstrating ECG changes similar to ARVC. We discuss an approach to a diagnostically challenging patient. This case emphasizes the importance of a broad differential and the danger of premature closure.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Letter
    暂无摘要。
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    心律失常性心肌病是一种罕见的遗传性结构性心脏病,具有各种表型,主要影响心脏的右心室,导致心肌纤维脂肪替代,并倾向于产生自发性恶性心律失常,可能导致猝死。以前的大多数报告都是关于年轻人的。我们报告了一名61岁的重型卡车司机的双心室表型病例,该司机目前有糖尿病和吸烟史,并在向医院提出头晕和晕厥的投诉后根据帕多瓦标准被偶然诊断。他最终有了植入式心脏复律除颤器,从而防止死亡。通过采取必要的管理措施,我们能够正确诊断病例并预防心脏猝死。
    Arrhythmogenic cardiomyopathy is a rare hereditary structural heart disease, with various phenotypes, which mostly affects the right ventricle of the heart, resulting in fibrofatty replacement of the heart muscles and a proclivity to create spontaneous malignant cardiac arrhythmias that may lead to sudden death. Most previous reports were noted on young people. We report a case of its biventricular phenotype in a 61-year-old heavy truck driver who has a current medical history of diabetes mellitus and smoking and was incidentally diagnosed based on the Padua criteria after presenting to the hospital with complaints of lightheadedness and syncope. He eventually had an implantable cardioverter defibrillator, hence preventing death. We were able to correctly diagnose the case and prevent sudden cardiac death by instituting the necessary management.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • DOI:
    文章类型: Journal Article
    致心律失常性右心室发育不良(ARVD)是由脂肪和纤维化构成的右心室(RV)心肌组织的进行性变性和替换而产生的临床状况。桥粒基因突变只是ARVD遗传性疾病的致病状态。致心律失常性右心室心肌病的发生率约为1/1000-5000。大多数年轻人和运动员的临床表现包括晕厥前,晕厥,室性心动过速或室颤导致心脏骤停。我们报告了首例心脏磁共振(CMR)成像诊断一例Savar一名34岁男性的心律失常性右心室发育不良(ARVD),达卡,孟加拉国因评估复发性头晕而被称为心脏急诊。
    Arrhythmogenic right ventricular dysplasia (ARVD) is a progressive degeneration and replacement of the right ventricular (RV) myocardial tissue by fat and fibrosis and produce clinical condition. Desmosome gene mutations are only the causative state for ARVD hereditary disorder. The arrhythmogenic right ventricular cardiomyopathy incidence is about 1/1000-5000. Mostly young people and athletes are bearing the clinical presentations include presyncope, syncope, ventricular tachycardias or ventricular fibrillation leading to cardiac arrest. We report about the first case of Cardiac magnetic resonance (CMR) imaging to diagnose a case Arrhythmogenic right ventricular dysplasia (ARVD) of a 34-year-old male from Savar, Dhaka, Bangladesh who was referred to cardiac emergency for the evaluation recurrent dizzy spells.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    本文提供了致心律失常性右心室心肌病的广泛概述,包括评估,诊断,和治疗选择。通过案例研究的镜头回顾了护理注意事项和临床管理。早期诊断预防心源性猝死对于致心律失常性右室心肌病患者至关重要。
    This article provides a broad overview of arrhythmogenic right ventricular cardiomyopathy, including evaluation, diagnosis, and treatment options. Nursing considerations and clinical management are reviewed through the lens of a case study. Early diagnosis to prevent sudden cardiac death is essential for patients with arrhythmogenic right ventricular cardiomyopathy.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

公众号