左室心肌致密化不全合并长QT综合征1例伴文献复习

目的:探讨儿童左室心肌致密化不全(LVNC)伴长QT综合征(LQTS)的临床特征并进行文献复习。方法:回顾分析1例LVNC伴LQTS患儿的临床资料包括临床症状、辅助检查及治疗情况。结果:本例患儿以阿斯综合征发作为首发症状,发作时心电监测见室性心动过速(VT),包括尖端扭转性室速(Tdp)。病程后期超声心动图才提示左室心肌致密化不全,左室收缩功能减低(LVEF 35%,LVFS 17%)。住我院后异丙肾上腺素静脉维持未能控制阿斯综合征发作,予安装心脏临时起搏器合并利多卡因静脉维持治疗,入院第3周静脉植入埋藏式心脏复律除颤仪(ICD)。结论:LVNC临床表型波动变化且合并LQTS少见;LVNC继发QT间期延长可诱发Tdp可危及患儿生命,药物控制难度大,ICD是有效治疗方法。

Left Ventricular Non-Compaction Cardiomyopathy Associated with Long QT Syndrome: A Case Report and Literature Review

Objective: To probe into the clinical characteristics of left ventricular non-compaction cardiomyopathy (LVNC) associated with long QT syndrome (LQTS) and review the literature. Methods: Clinical data of a child with LVNC associated with LQTS was retrospectively analyzed , including clinical symptoms, assistant examinations and treatment process. Results: Adams-Strokes syndrome (ASS) was the primary symptom in this child. During the onset of ECG monitoring, ventricular tachycardia (VT) was detected, including Torsades de pointes (Tdp). LVNC was observed in the echocardiography in the late stage of the disease, persistently with left ventricular dysfunction (LVEF 35%, LVFS 17%). After hospitalization, isoproterenol intravenous maintenance failed to control the onset of ASS, and temporary cardiac pacemaker combined with lidocaine intravenous maintenance treatment was given. Implantable cardioverter defibrillator (ICD) was implanted at the third week of admission. Conclusion: LVNC manifests as an undulating phenotype and associated with LQTS is relatively rare in clinic. Tdp induced by prolonged QT interval of secondary LVNC is deadly , and ecurrent Tdp is difficult to be controlled by antiarrhythmic agent, while ICD is an effective treatment.