DOI: 10.36347/sjmcr.2020.v08i07.022

Pages: 738-740

Congenital long QT syndrome (SQTL) is a group of genetically heterogeneous heart diseases characterized by repolarization disorders secondary to QT prolongation, leading to an increased risk of ventricular arrhythmia. The genes involved in these mutations encode for subunits of the ion channels responsible for cardiac electrical activity. SQTL should be evoked during the onset of syncope or sudden death in young subjects in the context of adrenergic stimulation. There is often a delay in diagnosis in this age group, because the symptoms are, wrongly, attributed to an epilepsy attack. The diagnosis is based on the electrocardiogram, a family survey and a genetic study. The treatment is based on beta-blockers and the implantable defibrillator. We report through an observation of a 3 years old girl who presented loss of consciousness following exertion. We will discuss her diagnosis andtreatment.