Ranolazine Safely Decreases Ventricular and Atrial Fibrillation in Timothy Syndrome (LQT8)
Ranolazine Safely Decreases Ventricular and Atrial Fibrillation in Timothy Syndrome (LQT8)
复制标题
DOI：
10.1111/j.1540-8159.2010.02913.x
复制
发表时间：
2012-03-01
影响因子：
1.8
通讯作者：
Burke, Martin C.
中科院分区：
文献类型：
--
作者：
Shah, Dipak P.;Baez-Escudero, Jose L.;Burke, Martin C.
长QT8 (LQT8)，也被称为Timothy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。这种钙负荷和由此导致的QT间期延长为室性心律失常提供了基础。我们之前报告了一例TS患者，在使用l型钙通道阻滞剂后，他的室性心律失常负担明显减轻。虽然该患者的心律失常负担减轻，但心房颤动负担增加，仍有室性颤动发作，需要除颤器治疗。最近的基础研究表明，雷诺嗪是一种多能离子通道阻滞剂，可能对LQT8综合征患者有益。本病例报告详细介绍了我们的LQT8患者添加雷诺嗪后房颤和室颤事件的减少。（2010; 13）
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. (PACE 2010; 13)