A 14-year-old adolescent without personal or family medical history presented after 4 episodes of syncope. Except for early repolarisation in the inferior ECG leads and ventricular extrasystoles originating from the left ventricular outflow tract, all investigations were negative. Following another syncopal episode, antiepileptic therapy was introduced because of electroencephalographic abnormalities and a Reveal DX Holter was implanted because of early repolarisation recently described as a putative predictor of sudden death.
An event was automatically recorded by the device. The text described an episode detected in the FVT zone, which lasted 5 sec.
This young patient was treated with quinidine and received an ICD. This case illustrates several important points: 1) He was initially treated with an antiepileptic regimen for recurrent syncope, a common observation in case of catecholamine-mediated VT or congenital long QT syndrome; 2) the ECG showed early repolarisation, a common observation in the healthy population, seriously complicating the risk stratification of sudden cardiac death. The place of the ILR in this context is equally delicate. As in this case, it allows to revisit the diagnosis, eliminate a probably useless antiepileptic treatment, consider a cardiac cause and reorganize the treatment. On the other hand, this episode was particularly concerning, as it evoked a clear risk of fatal outcome. The role of the ILR in the risk of sudden death stratification in patients presenting with Brugada syndrome or early repolarisation remains controversial and in need of further investigation.