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长QT1型综合征(LQT1)患者的外显率较低,存在20%~40%静息心电图QTc间期正常的隐匿性LQT1患者,其发生机制可能与复极储备及特定的突变基因相关。隐匿性LQT1患者仍然具有发生恶性心脏事件的风险,需在临床中引起重视,对LQT1的家族成员进行必要的基因筛查,同时可根据其在运动或肾上腺素激发后表现出的QT间期矛盾性延长的特点帮助诊断。
Patients with long QT1 syndrome (LQT1) have a lower penetrance, and 20% -40% of patients with occult LQT1 with normal QTc interval resting electrocardiogram may be related to repolarization reserve and mutation-specific genes. Patients with occult LQT1 still have the risk of developing a malignant cardiac event that needs to be highlighted clinically to screen for the necessary genetic screening of LQT1 family members and based on their QT interval contradictions after exercise or adrenergic challenge The characteristics of sexual extension to help diagnose.