不整脈性右心室心肌病變主要的病理特徵是脂肪及纖維組織取代了心肌組織。不整脈性右心室心肌病變是一個罕見但重要的疾病，因為它是青年人猝死的原因之一。我們報告一位42 歲的男性因心悸求診，其心電圖發現為具有類似左枝傳導阻斷形態的心室頻脈。經靜注amiodarone 後恢復正常節律後，發現心電圖胸導程V1 至V3 的T 波倒置。心導管檢查發現冠狀動脈正常，而右心室擴大且收縮不良。進一步磁振造影發現右心室壁變薄且內有代表脂肪的高強度訊號。患者經口服amiodarone 追蹤一年並無復發。回顧最近文獻，早期診斷輔以抗心律不整藥及植入自動除顫器，可明顯降低不整脈性右心室心肌病變的死亡率，因此在患者發生猝死前加以正確診斷相當重要。

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a myocardial disease primarilyaffecting the right ventricle (RV) and is pathologically characterized by the replacement ofmyocardial tissue with fatty and fibrous tissues. ARVC is a rare but important disease because ofits association with sudden arrhythmic death in young and otherwise healthy people. We reporton a 42-year-old man who presented with sudden onset of palpitations. His electrocardiogram(ECG) revealed sustained ventricular tachycardia (VT) with left-bundle branch block (LBBB)morphology. The VT reverted to a sinus rhythm following intravenous amiodarone administration.A sinus 12-lead ECG revealed an inverted T wave in the V1 to V3 leads. Cardiac catheterizationshowed normal coronary arteries and a dilated hypokinetic RV. A clinical diagnosis of ARVC wasconfirmed by magnetic resonance imaging (MRI) which showed a thin and dilated RV with ahyperintense signal intheRV freewall suggestive of fatty replacement. The patientwas dischargedwith a normal sinus rhythm on 200 mg/day amiodarone, and there was no recurrent ventriculartachycardia during a follow-up period of 12 months.