Challenges in the diagnosis of arrhythmogenic cardiomyopathy in an adolescent: Case report

Arrhythmogenic cardiomyopathy/right ventricular dysplasia is a rarely diagnosed hereditary cardiomyopathy that seldom debuts in the prepubertal period. Therefore, its diagnosis at this ages certain difficulties. We present a clinical case of this disease, which we have been observing for 6 years. The first symptoms of the disease in a thirteen years old teenager were ventricular premature beats that detected during a routine electrocardiographic examination. The presence of deep negative T waves in leads V1–V3 on the ECG, the presence of stress induced of polymorphic ventricular tachycardia, the appearance of additional markers of electrical instability of the myocardium during Holter monitoring (heart rate turbulence, microvolt alternans) and ventricular late potentials made it possible to suspect and then confirm a rare cardiomyopathy – arrhythmogenic cardiomyopathy during an MRI examination of the heart. The antiarrhythmic therapy allowed stabilizing the patient’s condition. However, at the age of 18, the disease progressed with inadequate intake of drugs, episodes of loss of consciousness appeared, and a cardioverter defibrillator was implanted in the patient. The presented case of observation of a patient with cardiomyopathy, rare for the pediatric population, with a progressive unfavorable course of the disease, the use of modern diagnostic methods made it possible to identify the unfavorable nature of arrhythmia, quickly make a diagnosis and prescribe therapy that prevented the development of syncopal episodes in childhood.