Arrhythmogenic right ventricular cardiomyopathy with left ventricular involvement and aortic dissection

Ten years ago, a 59-year-old patient presented with ventricular fibrillations. The resting ECG showed findings typical for ARVC. Echocardiography and ventriculography confirmed the diagnosis of ARVC showing a dilated right ventricle with aneurysms. MRI showed additional fatty replacement of the LV. Furthermore, the diagnosis of a chronic aortic dissection was established. Two years after ICD implantation, the patient died of progressive right heart failure. On autopsy, most of the RV and parts of the LV were replaced by fatty tissue, and the media of the aorta showed degenerative changes. A pathogenetic link between the two diseases remains speculative at this time.