Sudden cardiac death (SCD) is defined as unexpected death due to circulatory shock, mainly because of cardiac arrhythmias, within one hour of symptom onset. Although coronary heart disease is the most common cause, in young people is mainly due to a congenital structural or arrhythmogenic diseases. Idiopathic ventricular fibrillation (IVF) is a rare cause of SCD. Its incidence is not fully known. Over the past time, with a clearer definition of the diagnosis of primary arrhythmogenic syndromes (Brugada syndrome, long and short QT syndrome, early repolarization, catecholaminergic polymorphic ventricular tachycardia), its incidence decreased, and the definition was changed. The diagnosis of IVF is made by exclusion of known causes of VF. VF without proven structural heart disease or primary arrhythmic syndromes (with its clear phenotypic characteristics) is defined as IVF. It is characterized by rare individual ventricular premature beats (VES) with a narrow interval of binding (R / T phenomenon) and a high propensity for the development of polymorphic ventricular tachycardia (PVT) and VF. The cellular mechanism linked to the Ito potassium current in the His-Purkinje fibers, which causes a strong potential repolarization with surrounding myocardium and leads to VES with short coupling interval bonding with subsequent circular movement in phase II and the emergence of PVT and FK. It is not clear whether it is a monogenic or polygenic entity, and so far has seen the involvement of mutations in genes CALM1, DPP6, RyR and IRX3. Relapse rate is high and ranges (according to the now rare trials) of 11% to 45% annually. (1)

In the lecture we will show case of a patient who is at a younger age survived arrhythmic storm and who we follow up for many years.