Drago Fabrizio, Fazio Giovanni, Silvetti Massimo Stefano, Oricchio Gianluca, Michelon Guido
Int J Cardiol. 2007 Jun 12;118(3):e108-12. doi: 10.1016/j.ijcard.2007.01.066. Epub 2007 Apr 3.
Beta-blocker is the first line drug therapy for congenital long QT syndrome. However, in some children this drug is ineffective. In a non-responder patient, Shimizu et al. used Mexiletine to suppress the ventricular arrhythmias, obtaining a good result. In the high risk patient, the ICD is necessary. However the implantation of a device in small children can have technical problems. We report a case of a child affected by long QT syndrome with recurrent episodes of syncope due to self-limiting torsade de point/ventricular fibrillation, successfully treated by an association of mexiletin and propanolol, and in whom an ICD was implanted with a new subcutaneous approach.
β受体阻滞剂是先天性长QT综合征的一线药物治疗方法。然而，在一些儿童中这种药物无效。在一名无反应患者中，清水等人使用美西律抑制室性心律失常，取得了良好效果。在高危患者中，植入式心脏复律除颤器（ICD）是必要的。然而，在幼儿中植入设备可能会有技术问题。我们报告了一例患有长QT综合征的儿童病例，该儿童因自限性尖端扭转型室性心动过速/心室颤动反复发作晕厥，通过美西律和普萘洛尔联合治疗成功治愈，并且采用新的皮下植入方法为其植入了ICD。
