Pintea Bentea Georgiana, Berdaoui Brahim, Samyn Sophie, Morissens Marielle, Castro Rodriguez Jose
Department of Cardiology, CHU Brugmann, 1020 Brussels, Belgium.
Diagnostics (Basel). 2024 Jul 5;14(13):1434. doi: 10.3390/diagnostics14131434.
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.
一名76岁男性患者因急性失代偿性右心衰竭和晕厥前发作就诊于急诊室。入院时,他的心电图(ECG)显示持续性单形性室性心动过速,心率为180次/分钟,经电复律后,患者随后被收入重症监护病房。超声心动图显示右心室(RV)非常扩张,整体收缩功能障碍,前壁和侧壁运动减弱。冠状动脉造影正常。心脏磁共振显示右心室心肌有纤维脂肪替代的迹象。此外,复律后的心电图显示V1-V3导联T波倒置和ε波,信号平均心电图显示有晚电位。因此,怀疑为致心律失常性右心室心肌病(ARVC)。然而,他没有ARVC家族史,诊断时76岁,此前一直无症状。考虑到这些因素,我们进行了右心室血管造影,结果显示右心室扩张,有运动减弱/运动障碍性膨出,形成了提示ARVC的“盘状堆积”图像。就我们所知,在该患者中,右心室血管造影有助于确诊为表现非常晚的ARVC,这是文献中描述的年龄方面最晚的表现。