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先天性长QT综合征(long QT syndrome,LQTS)是以QTc间期异常延长,临床上反复发作晕厥、致命性心律失常如尖端扭转性室性心动过速(TdP)及心室颤动(室颤),并常导致猝死为特征的遗传性心脏病。LQTS按是否伴耳聋而区分为Jervell-Lange-Nielson综合征和Romano-Ward综合征,前者为常染色体隐性遗传,后者为常染色体显性遗传。LQTS多见于青少年,是青少年心脏病猝死的常见原因。LQTS比较少见,但并不罕见,易被误诊和漏诊。本文对LQTS诊断和治疗的研究进展做一概述。
Long QT syndrome (LQTS) is characterized by abnormal prolongation of QTc interval, recurrent syncope in clinical practice, fatal arrhythmia such as TdP and ventricular fibrillation (VF) , And often leads to sudden death characterized by inherited heart disease. LQTS is classified as Jervell-Lange-Nielson syndrome and Romano-Ward syndrome depending on whether it is deaf or not, the former being autosomal recessive and the latter being autosomal dominant. LQTS more common in adolescents, is a common cause of sudden cardiac death in adolescents. LQTS is relatively rare, but not uncommon, easily misdiagnosed and missed. This article gives an overview of the research progress of LQTS diagnosis and treatment.