转 2,3-烯酰基辅酶 A 还原酶样相关儿茶酚胺多形性室性心动过速伴规则室性心动过速和对氟卡尼的反应。
Trans-2,3-enoyl-CoA reductase-like-related catecholaminergic polymorphic ventricular tachycardia with regular ventricular tachycardia and response to flecainide.
机构信息
Department of Pediatrics, Affiliated with Chest Diseases Hospital, Faculty of Medicine, Kuwait University, Kuwait City, Kuwait.
Faculty of Medicine, Kuwait University, Kuwait City, Kuwait.
出版信息
J Cardiovasc Electrophysiol. 2023 Sep;34(9):1996-2001. doi: 10.1111/jce.16011. Epub 2023 Jul 20.
INTRODUCTION
We describe a unique case of TECRL-CPVT presented with cardiac arrest.
METHODS
Post resuscitation, the patient developed regular ventricular tachycardia featuring a left purkinje system morphology.
RESULTS
There was clear suppression of arrhythmia with the addition of flecainide and isolated ventricular ectopy causing secondary T-wave changes.
CONCLUSION
A high index of suspicion was required to eventually make the diagnosis through whole exome sequencing.
简介
我们描述了一例以心脏骤停为表现的 TECRL-CPVT 独特病例。
方法
复苏后，患者出现了具有左浦肯野系统形态的规则性室性心动过速。
结果
氟卡尼的加入明显抑制了心律失常，孤立性室性异位搏动导致了继发性 T 波改变。
结论
通过全外显子组测序，最终需要高度怀疑才能做出诊断。
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