Lebreiro Ana, Sousa Alexandra, Silva Anabela, Campos Júlio, Maciel Maria Júlia
Cardiology Department, Hospital S. João, Porto, Portugal.
Cardiology. 2010;115(3):232-5. doi: 10.1159/000297686. Epub 2010 Mar 19.
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.
原发性心电综合征是一组罕见的遗传性疾病，在心脏无结构异常的情况下易发生心律失常，并与多种离子通道突变有关。诸如发热等外在因素可能促使这些患者发生电不稳定。我们报告一例52岁因晕厥入院的患者，其在住院期间发生心室颤动，入院心电图显示胸前导联ST段明显抬高（V2导联最大抬高5mm）。该患者无结构性心脏病，住院期间ST段逐渐恢复正常，具有Brugada波型特征。植入自动除颤器用于心脏骤停的二级预防。我们认为这些发现可能代表了一种遗传性心电综合征的新表现形式。
