Abstract
Congenital Long QT syndrome is a family of conditions that share ion channel disturbances that can lead to severe ventricular arrhythmias. This report describes a rare case of fetal presentation, as well as its subsequent perinatal management. The postnatal electrocardiogram revealed extreme prolongation of QT interval, ventricular arrhythmias, and atrioventricular block. Patient was treated postnatally using propranolol and mexiletine, with pacemaker implantation. Type 2 LQTS was genetically confirmed. At last follow-up the patient remains free of ventricular arrhythmia. Fetal ventricular arrhythmias must raise suspicion of LQTS. This article provides additionally some practical consideration in regard to diagnosis and treatment of fetal arrhythmia.