Nikos Protonotarios Medical Centre, Naxos 84300, Greece.
Heart Department, University Hospital, Salerno, Italy.
Int J Cardiol. 2018 Apr 15;257:371-377. doi: 10.1016/j.ijcard.2018.01.021.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is the most common phenotype described within the spectrum of arrhythmogenic cardiomyopathies. It usually presents in early adolescence with severe ventricular arrhythmias along with cardiac structural and functional alterations mainly of the right ventricular myocardium. Though the estimated prevalence of ARVC in the general population is only 1:5000, it represents one of the most common causes of juvenile sudden death. However, detection of early RV dysfunction in ARVC may be challenging requiring high clinical suspicion and an algorithmic approach. A thorough family history of juvenile sudden death, ventricular arrhythmias and ICD implants should always be sought. Diagnosis usually requires electrocardiographic interpretation as well as cardiac imaging. In this article, the key diagnostic steps in the assessment of ARVC and diagnostic red flags that aid its differential diagnosis are discussed.
致心律失常性右室心肌病（ARVC）是心律失常性心肌病谱中描述的最常见表型。它通常在青少年早期出现，伴有严重的室性心律失常以及主要发生在右心室心肌的心脏结构和功能改变。尽管 ARVC 在普通人群中的估计患病率仅为 1:5000，但它是青少年猝死的最常见原因之一。然而，在 ARVC 中检测到早期 RV 功能障碍可能具有挑战性，需要高度的临床怀疑和算法方法。应始终寻找青少年猝死、室性心律失常和 ICD 植入的详细家族史。诊断通常需要心电图解读以及心脏成像。本文讨论了评估 ARVC 的关键诊断步骤和有助于其鉴别诊断的诊断警示标志。
