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原发性遗传性心律失常(Primary Inherited Arrhythmias
PIA)通常是指遗传性编码离子通道和相关蛋白的基因异常导致的疾病,基因突变导致心肌细胞电活动紊乱,临床表现的程度和异常情况跨度很大,可以无症状也可以引起恶性室性心律失常(Ventricular Arrhythmias
VAs)和心源性猝死(Sudden Cardiac Death
SCD)。原发性遗传性心律失常发生SCD通常由于致死性的VAs,包括心室颤动(Ventricular Fibrillation
VF)、尖端扭转型室性心动过速(Torsade de Pointes
Td P)、或者其他的室性心动过速(Ventricular tachycardia
VT)。
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