Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ventricular arrhythmias and an increased risk of sudden cardiac death. Although structural abnormalities of the right ventricle predominate, it is well recognized that left ventricular involvement is common, particularly in advanced disease, and that left-dominant forms occur. The pathological characteristic of ARVC is myocyte loss with fibrofatty replacement. Since the first detailed clinical description of the disorder in 1982, significant advances have been made in understanding this disease. Once the diagnosis of ARVC is established, the single most important clinical decision is whether a particular patient’s sudden cardiac death risk is sufficient to justify placement of an implantable cardioverter-defibrillator. The importance of this decision reflects the fact that ARVC is a common cause of sudden death in young people and that sudden death may be the first manifestation of the disease. This decision is particularly important because these are often young patients who are expected to live for many years. Although an implantable cardioverter-defibrillator can save lives in individuals with this disease, it is also well recognized that implantable cardioverter-defibrillator therapy is associated with both short- and long-term complications. Decisions about the placement of an implantable cardioverter-defibrillator are based on an estimate of a patient’s risk of sudden cardiac death, as well as their preferences and values. The primary purpose of this article is to provide a review of the literature that concerns risk stratification in patients with ARVC and to place this literature in the framework of the 3 authors’ considerable lifetime experiences in caring for patients with ARVC. The most important parameters to consider when determining arrhythmic risk include electric instability, including the frequency of premature ventricular contractions and sustained ventricular arrhythmia; proband status; extent of structural disease; cardiac syncope; male sex; the presence of multiple mutations or a mutation in TMEM43; and the patient’s willingness to restrict exercise and to eliminate participation in competitive or endurance exercise.

心律失常性右室心肌病（ARVC）是一种遗传性心肌病，其特征是室性心律不齐和心脏猝死的风险增加。尽管右心室的结构异常占主导，但众所周知，左心室受累是常见的，特别是在晚期疾病中，并且以左为主的形式发生。ARVC的病理特征是纤维脂肪替代引起的心肌细胞丢失。自1982年首次对该疾病进行详细的临床描述以来，在了解该疾病方面已取得了重大进展。一旦确定了ARVC的诊断，最重要的临床决策就是特定患者的突然心脏死亡风险是否足以证明植入式心脏复律除颤器的合理性。该决定的重要性反映了这样一个事实，即ARVC是年轻人突然死亡的常见原因，而突然死亡可能是该疾病的首发表现。该决定尤其重要，因为这些患者通常都是年轻患者，预计可以活很多年。尽管植入式心脏复律除颤器可以挽救患有这种疾病的人的生命，但也众所周知，植入式心脏复律除颤器的治疗与短期和长期并发症有关。关于植入式心脏复律除颤器的放置位置的决定是基于对患者突发性心脏猝死的风险以及他们的喜好和价值观的估计。本文的主要目的是提供有关ARVC患者风险分层的文献的综述，并将该文献置于3位作者在护理ARVC患者方面的大量终生经验的框架内。确定心律不齐风险时要考虑的最重要参数包括电不稳定，包括室性早搏和持续性室性心律失常的频率；先证者身份；结构性疾病的程度；心脏晕厥; 男性; TMEM43中存在多个突变或突变；以及患者限制运动并消除参加竞争性或耐力运动的意愿。确定心律不齐风险时要考虑的最重要参数包括电不稳定，包括室性早搏和持续性室性心律失常的频率；先证者身份；结构性疾病的程度；心脏晕厥; 男性; TMEM43中存在多个突变或突变；以及患者限制运动并消除参加竞争性或耐力运动的意愿。确定心律不齐风险时要考虑的最重要参数包括电不稳定，包括室性早搏和持续性室性心律失常的频率；先证者身份；结构性疾病的程度；心脏晕厥; 男性; TMEM43中存在多个突变或突变；以及患者限制运动并消除参加竞争性或耐力运动的意愿。