PDF(Pubmed)
Abstract：
A 76-year-old male patient presented to the emergency room with acute decompensated right heart failure and presyncope episodes. Upon admission, his electrocardiogram (ECG) showed sustained monomorphic ventricular tachycardia at 180 bpm, which was electrically cardioverted, and the patient was subsequently admitted to the intensive care unit. The echocardiography showed a very dilated right ventricle (RV) with global systolic dysfunction and akinetic anterior and lateral walls. The coronary angiography was normal. The cardiac magnetic resonance showed signs of fibro-fatty replacement of the RV myocardium. Furthermore, the ECG after cardioversion showed inverted T waves and an epsilon wave in V1-V3 leads and late potentials by signal-averaged ECG. As such, a diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) was suspected. However, he presented no familial history of ARVC, was 76 years of age at the time of diagnosis and was asymptomatic until now. Given these considerations, we performed a right ventricular angiography which showed dilatation of the RV with akinetic/dyskinetic bulging, creating the \"pile d\'assiettes\" image suggestive of ARVC. In the case of this patient, the RV angiography contributed to establish a diagnosis of ARVC with a very late presentation, to our knowledge the latest presentation in terms of age described in the literature.
摘要：
一名76岁的男性患者因急性失代偿性右心力衰竭和晕厥前发作而被送往急诊室。一被录取,他的心电图（ECG）显示在180bpm持续的单形性室性心动过速，它被电引导，患者随后被送进重症监护室。超声心动图显示右心室（RV）非常扩张，具有整体收缩功能障碍和前后壁运动。冠状动脉造影正常。心脏磁共振显示RV心肌纤维脂肪替代的迹象。此外，心脏复律后的ECG显示V1-V3导联中的T波和ε波倒置，并且通过信号平均ECG显示晚期电位。因此，怀疑诊断为致心律失常性右室心肌病(ARVC).然而,他没有提供ARVC的家族史，诊断时76岁，直到现在都无症状。鉴于这些考虑,我们进行了右心室血管造影，显示右心室扩张伴运动障碍/运动障碍膨出，创建提示ARVC的“packd\'assiettes\”图像。就这个病人而言,RV血管造影有助于确定ARVC的诊断，据我们所知，文献中描述的年龄方面的最新介绍。
