Atypical ST segment elevation and ventricular fibrillation without structural heart disease: a new electrocardiographic presentation of a channelopathy?

Primary electrical syndromes are a group of rare inherited diseases that predispose to arrhythmias in the absence of structural abnormalities of the heart, and are associated with several ion channel mutations. Extrinsic factors, such as fever, may contribute to the development of electrical instability in these patients. We report the case of a 52-year-old patient who was admitted for syncope and had an in-hospital episode of ventricular fibrillation, who presented with an admission ECG showing marked precordial ST segment elevation (maximum 5 mm in V2). The patient did not have structural heart disease and during the hospital stay there was progressive ST segment normalization, with features suggestive of Brugada pattern. An automated defibrillator was implanted for secondary prevention of sudden cardiac arrest. We believe that these findings may represent a new form of presentation of a genetic electrical syndrome.