Turley A J, Thambyrajah J, Harcombe A A
Cardiothoracic Division, The James Cook University Hospital, Marton Road, Middlesbrough TS4 3BW, UK.
Heart. 2005 Jan;91(1):15-7. doi: 10.1136/hrt.2004.039271.
Congenital long QT syndrome (LQTS) is a disorder of prolonged cardiac repolarisation, manifest by a prolonged QT interval and characterised by recurrent presyncope/syncope, polymorphic ventricular tachycardia (PMVT), or sudden cardiac death. A 46 year old woman with no family history of sudden death or deafness presented with recurrent syncope. Physical examination and electrolytes were normal and a 12 lead ECG showed a corrected QT interval of 458 ms. A monitored syncopal episode documented PMVT. Despite potassium and magnesium supplements, beta blockade, implantation of a single then dual chamber implantable cardioverter defibrillator (ICD), amiodarone, nicorandil, and mexiletine, the patient continued to experience arrhythmia storms, receiving more than 700 ICD discharges over seven months. She was ultimately treated successfully with bilateral thoracoscopic cervicothoracic sympathectomies. This is the first reported bilateral thoracoscopic treatment of a patient with LQTS and symptomatic life threatening ventricular tachyarrhythmias refractory to current pharmacological and pacing techniques.
先天性长QT综合征(LQTS)是一种心脏复极延长的疾病,表现为QT间期延长,其特征为反复发作的先兆晕厥/晕厥、多形性室性心动过速(PMVT)或心源性猝死。一名无猝死或耳聋家族史的46岁女性出现反复发作的晕厥。体格检查和电解质正常,12导联心电图显示校正QT间期为458毫秒。一次监测到的晕厥发作记录为PMVT。尽管补充了钾和镁、使用了β受体阻滞剂、植入了单腔然后双腔植入式心律转复除颤器(ICD)、使用了胺碘酮、尼可地尔和美西律,但患者仍持续经历心律失常风暴,在七个月内接受了700多次ICD电击。她最终通过双侧胸腔镜颈胸交感神经切除术成功治愈。这是首次报道采用双侧胸腔镜治疗LQTS且对当前药物和起搏技术难治的有症状的危及生命的室性快速性心律失常患者。