Short QT syndrome in a 14-year-old patient: The first pediatric case from Turkey

Short QT syndrome (SQTS) is a cardiac channelopathy associated with sudden cardiac death (SCD) and disposition to atrial-ventricular fibrillation (A-VF) (1). An accelerated ventricular repolarization (VR) abnormality develops in the heart due to an electrical stability disorder secondary to increased extracellular potassium flow in the heart (2, 3). Therefore, ventricular arrhythmias develop, which cause syncope, convulsion, and SD (2). This paper presents a 14-year-old male patient whose elder brother and father had died because of SD and who presented to us with the complaint of syncope. He was identified to have a short QT interval (SQTI) in his electrocardiogram (ECG) result, diagnosed with SQTS following an electrophysiological study (EPS), and implanted with an implantable cardioverter defibrillator (ICD).