Clinical diagnosis and management strategies in arrhythmogenic right ventricular cardiomyopathy.
Clinical diagnosis and management strategies in arrhythmogenic right ventricular cardiomyopathy.
复制标题
致心律失常性右心室心肌病的临床诊断和治疗策略。
DOI：
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发表时间：
2000
影响因子：
1.3
通讯作者：
G. Thiene
中科院分区：
文献类型：
--
作者：
D. Corrado;G. Buja;C. Basso;G. Thiene
致心律失常性右心室心肌病（ARVC）是一种以右心室（RV）心肌纤维脂肪替代为特征的心肌疾病，通常具有家族性。ARVC最常见的临床表现包括RV起源的室性心律失常，这可能导致主要在年轻人和运动员中的猝死，心电图去极化/复极化变化主要局限于右胸导联，以及RV的整体和/或区域功能障碍和结构改变。由于心电图异常的特异性、左束分支形态的室性心律失常的不同潜在病因、RV结构和功能的评估以及肌内膜活检结果的解释等问题，ARVC的诊断可能很困难。因此，欧洲心脏病学会ARVC研究小组提出了标准化的诊断标准。根据这些指南，ARVC的诊断是基于主要和次要标准的存在，包括心电图，血液动力学，形态功能，组织病理学和遗传因素。由于对ARVC患者猝死风险的评估还没有很好地建立，因此没有确切的指南来确定哪些患者需要治疗，哪些是最佳的管理方法。治疗选择包括β受体阻滞剂、抗心律失常药物、导管消融和植入式心律转复除颤器。植入式除颤器是预防心脏猝死最有效的安全措施。然而，它在通过预防猝死和非猝死来改变ARVC自然史中的确切作用需要通过对大量患者的前瞻性研究来评估。在ARVC进展为重度RV或双心室收缩功能障碍伴血栓栓塞并发症风险的患者中，治疗包括当前心力衰竭治疗，包括抗凝治疗。在顽固性充血性心力衰竭的情况下，患者可能成为心脏移植的候选人。
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a myocardial disease, often familial, that is characterized by fibro-fatty replacement of the right ventricular (RV) myocardium. The most common clinical manifestations of ARVC consists of ventricular arrhythmias of RV origin, which may lead to sudden death mostly in young people and athletes, electrocardiograph depolarization/repolarization changes mostly localized to right precordial leads, and global and/or regional dysfunction and structural alterations of the RV. The diagnosis of ARVC may be difficult due to several problems with the specificity of the electrocardiograph abnormalities, the different potential etiologies of ventricular arrhythmias with a left bundle branch morphology, the assessment of the RV structure and function, and the interpretation of endomyocardial biopsy findings. Therefore, standardized diagnostic criteria have been proposed by the Study Group on ARVC of the European Society of Cardiology. According to these guidelines, the diagnosis of ARVC is based on the presence of major and minor criteria encompassing electrocardiograph, arrhythmic, morphofunctional, histopathologic, and genetic factors. Because the assessment of sudden death risk in patients with ARVC is still not well established, there are no precise guidelines to determine which are the patients who need to be treated and which is the best management approach. The therapeutic options include beta blockers, antiarrhythmic drugs, catheter ablation, and implantable cardioverter defibrillator. The implantable defibrillator is the most effective safe-guard against arrhythmic sudden death. However, its precise role in changing natural history of ARVC by preventing sudden and nonsudden death needs to be evaluated by a prospective study of a large series of patients. In patients in whom ARVC has progressed to severe RV or biventricular systolic dysfunction with risk of thromboembolic complications, treatment consists of current therapy for heart failure including anticoagulant therapy. In case of refractory congestive heart failure, the patients may become candidates for heart transplantation.
影响因子：
37.8
作者：
Ahmad, F;Li, DX;Roberts, R
通讯作者：
Roberts, R