Macro T-wave Alternans in Jervell and Lange-Nielsen Syndrome.

Here, we report a rare case of a three-year-old boy with Jervell and Lange-Nielsen (JLN) syndrome who presented with two episodes of nocturnal agonal gasp provoked by fever mimicking syncope in the last six months with a history of sudden cardiac death in one elderly sibling. Interestingly, an electrocardiogram (EKG) revealed macro T-wave alternans (TWA) indicative of a high risk of malignant ventricular arrhythmia in the form of ventricular fibrillation and sudden cardiac death. TWA in JLN syndrome has not been described in the global literature so far. Our case is unique and the first to describe macro TWA in JLN syndrome and is a teaching point to young cardiologists to always look for macro TWA in the EKG of long QT syndrome for risk stratification, management, and, most importantly, avoiding the risk of sudden cardiac death.