Introduction: Commonly accepted clinical classification of arrhythmogenic right ventricular cardiomyopathy (ARVC) is still not developed. Objective: To study the clinical forms of ARVC. Methods: Fifty-four patients (38.7 ± 14.1 years, 42.6% men) with ARVC. Follow-up period: 21 (6–60) months. All patients underwent electrocardiography, 24 h-Holter monitoring, echocardiography, and DNA diagnostic. Magnetic resonance imaging was performed in 49 patients. Results: According to the features of clinical course of ARVC, 4 clinical forms were identified. (I) Latent arrhythmic form (n = 27) – frequent premature ventricular contractions and/or nonsustained ventricular tachycardia (VT) in the absence of sustained VT and syncope; characterized by absence of fatal arrhythmic events. (II) Manifested arrhythmic form (n = 11) – sustained VT/ventricular fibrillation; the high incidence of appropriate implantation of cardioverter-defibrillator (ICD) interventions (75%) registered. (III) ARVC with progressive chronic heart failure (CHF, n = 8) as the main manifestation of the disease; incidence of appropriate ICD interventions was 50%, mortality rate due to CHF was 25%. (IV) Combination of ARVC with left ventricular noncompaction (n = 8); characterized by mutations in desmosomal or sarcomere genes, aggressive ventricular arrhythmias, appropriate ICD interventions in 100% patients. Described 4 clinical forms are stable in time, do not transform into each other, and they are genetically determined. Conclusions: The described clinical forms of ARVC are determined by a combination of genetic and environmental factors and do not transform into each other. The proposed classification could be used in clinical practice to determine the range of diagnostic and therapeutic measures and to assess the prognosis of the disease in a particular patient.

中文翻译：

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致心律失常性右室心肌病的临床分型

引言：公认的致心律失常性右心室心肌病（ARVC）的临床分类仍未制定。目的：研究ARVC的临床表现。方法：54 名 ARVC 患者（38.7 ± 14.1 岁，42.6% 男性）。随访期：21（6-60）个月。所有患者均接受了心电图、24 小时动态心电图监测、超声心动图和 DNA 诊断。对 49 名患者进行了磁共振成像。结果：根据ARVC临床病程特点，确定了4种临床形式。(I) 潜在心律失常形式 (n = 27) – 在没有持续性 VT 和晕厥的情况下，频繁发生室性早搏和/或非持续性室性心动过速 (VT)；特征是没有致命的心律失常事件。(II) 表现性心律失常形式 (n = 11) – 持续 VT/心室颤动；登记了适当植入心律转复除颤器 (ICD) 干预措施的高发生率 (75%)。（三）以进行性慢性心力衰竭（CHF，n=8）为主要表现的ARVC；适当 ICD 干预的发生率为 50%，因 CHF 导致的死亡率为 25%。(IV) ARVC 与左心室致密化不全的组合 (n = 8)；以桥粒或肌节基因突变、侵袭性室性心律失常、100% 患者适当的 ICD 干预为特征。所述4种临床形式时间稳定，不相互转化，由基因决定。结论：所描述的 ARVC 临床形式是由遗传和环境因素共同决定的，不会相互转化。提出的分类可用于临床实践，以确定诊断和治疗措施的范围，并评估特定患者的疾病预后。