A Man in His 60s With Severe Heart Failure and Incessant Ventricular Arrhythmias

A man in his 60s presented in cardiogenic shock due to incessant ventricular tachycardia and underwent heart transplant; what is the likely diagnosis of the cardiomyopathy?

The patient, with a history of unexplained ventricular tachycardia requiring implantation of an implantable cardioverter-defibrillator more than 20 years earlier, was referred to our center for cardiogenic shock and incessant ventricular tachycardia. As hemodynamic conditions rapidly worsened, the patient underwent emergency heart transplant. The macroscopic pathological examination revealed an extensive destruction of the right ventricular (RV) myocardium, with massive fibro-fatty replacement (Figure, A). Plurifocal epicardial fat inclusions were also found in the left ventricle (Figure, B), indicating an advanced stage of arrhythmogenic right ventricular cardiomyopathy.