Shah Dipak P, Baez-Escudero Jose L, Weisberg Ian L, Beshai John F, Burke Martin C
Department of Medicine, University of Chicago, Chicago, IL, USA.
Pacing Clin Electrophysiol. 2012 Mar;35(3):e62-4. doi: 10.1111/j.1540-8159.2010.02913.x. Epub 2010 Sep 30.
Long QT eight (LQT8), otherwise known as Timothy syndrome (TS), is a genetic disorder causing hyper-activation of the L-type calcium channel Cav 1.2. This calcium load and the resultant increase in the QT interval provide the substrate for ventricular arrhythmias. We previously presented a case in a patient with TS who had a profound decrease in his burden of ventricular arrhythmias after institution of an L-type calcium channel blocker. Although this patient's arrhythmia burden had decreased, he displayed an increasing burden of atrial fibrillation and still had bouts of ventricular fibrillation requiring defibrillator therapy. Basic research has recently shown that ranolazine, a multipotent ion-channel blocker, may be of benefit in patients with LQT8 syndrome. This case report details the decrease of atrial fibrillation and ventricular fibrillation events in our LQT8 patient with the addition of ranolazine.
长QT综合征8型(LQT8),又称蒂莫西综合征(TS),是一种导致L型钙通道Cav 1.2过度激活的遗传性疾病。这种钙负荷以及随之而来的QT间期延长为室性心律失常提供了基础。我们之前报告过一例蒂莫西综合征患者,在使用L型钙通道阻滞剂后,其室性心律失常负担显著减轻。尽管该患者的心律失常负担有所减轻,但他的房颤负担却在增加,并且仍有需要除颤器治疗的室颤发作。基础研究最近表明,雷诺嗪,一种多效离子通道阻滞剂,可能对LQT8综合征患者有益。本病例报告详细介绍了在我们的LQT8患者中加用雷诺嗪后房颤和室颤事件的减少情况。