Department of Cardiology, Yonsei University Health System, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea.
Yonsei Med J. 2013 Mar 1;54(2):529-33. doi: 10.3349/ymj.2013.54.2.529.
Long QT syndrome (LQTs) is an uncommon genetic disease causing sudden cardiac death with Torsade de Pointes (TdP). The first line drug treatment has been known to be β-blocker. We encountered a 15-year-old female student with LQTs who had prolonged QTc and multiple episodes of syncope or agonal respiration during sleep. Although her T wave morphology in surface electrocardiography resembled LQTs type 1, her clinical presentation was unusual. During the epinephrine test, TdP was aggravated during β-blocker medication, but alleviated by sodium channel blocker (mexiletine). Therefore, she underwent implantable cardioverter defibrillator implantation.
长 QT 综合征（LQTs）是一种罕见的遗传性疾病，可导致尖端扭转型室性心动过速（TdP）引起的心脏性猝死。已知的一线药物治疗是β受体阻滞剂。我们遇到了一位 15 岁的女性学生，患有 LQTs，她的 QTc 延长，并且在睡眠中多次出现晕厥或濒死性呼吸。尽管她的体表心电图 T 波形态类似于 LQTs 1 型，但她的临床表现不同寻常。在肾上腺素试验中，β受体阻滞剂治疗期间 TdP 加重，但钠通道阻滞剂（美西律）可缓解。因此，她接受了植入式心脏复律除颤器植入。
