Yin Chunlin, Zhang Ping, Yang Jing, Zhang Li
Department of Cardiology, Xuanwu Hospital, Capital University of Medical Sciences, Beijing, China.
Department of Cardiology, Beijing Tsinghua Changgung Hospital, Beijing, China.
J Electrocardiol. 2018 Jan-Feb;51(1):111-116. doi: 10.1016/j.jelectrocard.2017.08.022. Epub 2017 Aug 17.
A 26-year-old woman, 12 days in postpartum, developed recurrent syncope and cardiac arrest. Her ECG revealed QT-prolongation associated with LQT2-specific T-U wave patterns, T wave alternans, long QT-dependent torsade de pointes (TdP) and ventricular fibrillation (VF). She also had intermittent LBBB (80bpm) on alternate beats and RBBB at sinus tachycardia (113bpm). Family genotyping revealed a novel de novo missense mutation G604C of KCNH2. Propranolol slowed heart rate and further prolonged QT interval (610ms) that caused TdP recurrence. Mexiletine combined with magnesium and potassium supplements prevented TdP/VF recurrence. This patient has remained event-free after 9-month follow-up.
一名26岁产后12天的女性出现反复晕厥和心脏骤停。她的心电图显示QT延长，伴有LQT2特异性T-U波形态、T波交替、长QT依赖性尖端扭转型室速（TdP）和心室颤动（VF）。她还交替出现间歇性左束支传导阻滞（80次/分）和窦性心动过速（113次/分）时的右束支传导阻滞。家族基因分型显示KCNH2有一个新的新发错义突变G604C。普萘洛尔减慢心率并进一步延长QT间期（610毫秒），导致TdP复发。美西律联合补充镁和钾可预防TdP/VF复发。该患者在9个月的随访后未再出现相关事件。
