Magnetic Resonance Imaging Presentation of Arrhythmogenic Right Ventricular Dysplasia: A Case Report

心律失常性右心室發育不良心肌症在磁振造影影像上的表現

邱伯尹(Po-Yin Chiu) ； 呂宜璇(Yi-Hsuan Lu) ； 曾文盛(Wen-Sheng Tzeng) ； 張裕綱(Yu-Kang Chang)

《中華放射線醫學雜誌》 35卷1期 (2010/03) Pp. 39-44

摘要

心律失常性右心室發育不良心肌症是一種主要影響右心室的非缺血性心肌病變。其主要特徵包括右心室壁收縮力低下，右心室壁之心肌層被纖維結締組織或脂肪組織取代，及右心室之心律不整。
心律失常性右心室發育不良心肌症是一種顯性遺傳的心肌病變，其表現非常多樣化，近來已發現其病變基因位置為14q23-24。
本文報告一例有心律失常性右心室發育不良心肌症17歲女性病患，其磁振造影影像上的表現為右心室明顯肥大，右心室壁之心肌層廣泛性變薄並且被纖維結締組織或脂肪組織取代。本文旨在描述磁振造影影像可在此類病例中扮演－有用之輔助角色。

並列摘要

Arrhythmogenic right ventricular dysplasia (ARVD) is a nonischemic cardiomyopathy involving primarily the right ventricle. Its characteristics include hypokinetic areas involving the free wall of the right ventricle, fibrofatty replacement of the right ventricular myocardium, and associated arrhythmia originating in the right ventricle. Genetically, it is a cardiomyopathy with autosomal dominant inheritance, variable expressivity, and incomplete penetrance [1]. The location of the gene was mapped to 14q23-24 recently [1]. The incidence and frequency of this disease are still uncertain till now. Clinically, it is difficult to diagnose ARVD directly and accurately. Here, we report a case of 17 years old female with ARVD; the magnetic resonance imaging (MRI) revealed severe global dilatation of the right ventricle, and diffuse thinning of right ventricular myocardium with transmural replacement by fatty tissue and we conclude that MRI may play an important role in diagnosis of the disease.