小结
定义
病史和体格检查
关键诊断因素
- 已知基因突变的病史
- 使用已知会延长 QT 间期的药物或存在延长QT间期的情况
- 肾上腺素能增强时发生晕厥
- 觉醒或惊吓过程中发生晕厥
- 产后心律失常症状
- 休息和心动过缓过程中的晕厥
- 心源性晕厥
- 心悸
- 周期性麻痹
- 畸形特征
- 感觉神经性耳聋
其他诊断因素
- 头晕
- 心绞痛
- 疲劳
- 少尿
- 肌无力
- 抽搐
- 麻木
- Chvostek 征
- 特鲁索征
- 肢体寒冷、苍白
- 低血压
- 意识模糊
危险因素
- KCNQ1 基因突变
- KCNH2 基因突变
- SCN5A 基因突变
- QT 间期延长药物
- 低钾血症
- 低镁血症
- 低钙血症
- 缓慢性心律失常
- 中枢神经系统病变
- 女性
- 营养不良
诊断性检查
首要检查
- LQT1 的 ECG
- LQT2 的 ECG
- LQT3 的 ECG
- 低钾血症和低镁血症的 ECG
- 低钙血症的 ECG
- 完全性房室（atrioventricular, AV）传导阻滞的 ECG
- 血钾
- 血清镁
- 血清钙
需考虑的检查
- 霍尔特心电监测仪
- 运动负荷试验
- 超声心动图
- 基因检测
- 肾上腺素试验
治疗流程
获得性 LQTS
无既往心脏事件的先天性 LQTS
有既往心脏事件的先天性 LQTS
撰稿人
作者
Mehmet K. Aktas, MD, MBA
Associate Professor of Medicine
University of Rochester Medical Center
Rochester
NY
利益声明
MKA declares that he has received research grants from Astra Zeneca, Medtronic, and Boston Scientific. MKA has served as consultant for Abbott and Huxley Medical. MKA holds a patent for ‘ECG Clock Electrocardiogram Based Diagnostic Device and Method’ (US patent #10,085,667). He is the author of a reference cited in this topic.
James P. Daubert, MD
Professor of Medicine
Duke University Medical Center
Duke Clinical Research Institute
Durham
NC
利益声明
JPD declares that he has received honoraria for advisory boards, steering committees, data safety monitoring boards, events committees, and lecture fees from Abbott, Acutus Medical, Affera Inc., Biosense Webster, Biotronik, Boston Scientific, Farapulse, Gilead Sciences Inc., Medtronic, Microport, Phillips, and Vytronus; and research grants from Abbott, Biosense Webster, Boston Scientific, Farapulse, and Medtronic. He receives royalty payments from Wiley, Springboard and McGraw Hill for textbook authorship and educational materials. JPD declares that he has no stock, stock options, or other forms of ownership. He is the author of a reference cited in this topic.
同行评议者
Sami Viskin, MD
Director of Cardiac Hospitalization
Department of Cardiology
Tel Aviv Medical Center
Tel Aviv
Israel
利益声明
SV declares that he has no competing interests.
Elizabeth S. Kaufman, MD
Associate Professor
Heart and Vascular Center
MetroHealth Campus
Case Western Reserve University
Cleveland
OH
利益声明
ESK declares that she has no competing interests.
鉴别诊断
- 获得性结构性心脏病
- 神经心源性（血管迷走神经性）晕厥
- 神经性晕厥
更多 鉴别诊断指南
- 欧洲心律协会（EHRA）/心律学会（HRS）/亚太心律学会（APHRS）/拉丁美洲心律学会（LAHRS）心脏病基因检测现状专家共识声明
- 2022 年 ESC 室性心律失常患者管理和心源性猝死预防指南
更多 指南患者教育信息
何为心脏病发作？
如何预防心脏病再次发作
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QT 间期校正
更多 医学计算器
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