Corrado D, Buja G, Basso C, Thiene G
Department of Cardiology and Cardiovascular Pathology, University of Padua Medical School, Italy.
J Electrocardiol. 2000;33 Suppl:49-55. doi: 10.1054/jclc.2000.20323.
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.
致心律失常性右室心肌病（ARVC）是一种心肌疾病，通常具有家族性，其特征是右心室（RV）心肌被纤维脂肪组织替代。ARVC最常见的临床表现包括起源于右室的室性心律失常，这可能导致多数年轻人和运动员猝死；心电图去极化/复极化改变主要局限于右胸前导联；以及右室整体和/或局部功能障碍及结构改变。由于心电图异常的特异性存在若干问题、左束支形态的室性心律失常有不同潜在病因、右室结构和功能的评估以及心内膜活检结果的解读等，ARVC的诊断可能较为困难。因此，欧洲心脏病学会ARVC研究组提出了标准化诊断标准。根据这些指南，ARVC的诊断基于包括心电图、心律失常、形态功能、组织病理学和遗传学因素在内的主要和次要标准。由于ARVC患者猝死风险的评估仍未完全明确，尚无精确指南来确定哪些患者需要治疗以及最佳治疗方法是什么。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脏复律除颤器。植入式除颤器是预防心律失常性猝死最有效的保障措施。然而，其通过预防猝死和非猝死来改变ARVC自然病程的确切作用需要通过对大量患者的前瞻性研究来评估。对于ARVC已进展为严重右室或双心室收缩功能障碍且有血栓栓塞并发症风险的患者，治疗包括目前的心力衰竭治疗方法，包括抗凝治疗。对于难治性充血性心力衰竭患者，可能成为心脏移植的候选者。
