Publication:

Arrhythmogenic cardiomyopathy: from concealed phase to refractory failure: a nine-year case study illustrating diagnostic evolution and biventricular progression

Arrhythmogenic cardiomyopathy (ACM) is a type of cardiomyopathy characterized by fibrofatty replacement of the right ventricular myocardium and associated with life-threatening arrhythmias. The criteria of the diagnosis of ACM are based on the estimates of structural changes in the right ventricular myocardium, electrocardiographic changes, fibrofatty dystrophy in the course of a histological investigation, and a family history of the disease. The diagnosis can be a challenging task as there are no pathognomonic clinical markers, which makes differential diagnosis of ACM with secondary myocardial pathology more difficult to perform. The article presents a nine-year-old case study of a young male patient with ACM, dominated by life-threatening ventricular arrhythmias and episode of resuscitated sudden cardiac death despite optimal medical therapy and cardioverter-defibrillator implantation.