Ten Harkel Arend D J, Witsenburg Maarten, de Jong Peter L, Jordaens Luc, Wijman Marjolein, Wilde Arthur A M
Department of Pediatric Cardiology, Erasmus MC-Sophia, 3015 GJ Rotterdam, The Netherlands.
Europace. 2005 Jan;7(1):77-84. doi: 10.1016/j.eupc.2004.09.007.
We present a case in which LQTS induced severe prenatal and neonatal arrhythmias. LQT3 was diagnosed (mutation R1623Q). Short-acting beta-blockers were ineffective as well as sotalol and mexiletine in preventing recurrent ventricular arrhythmias. An ICD was implanted at the age of 7 months (weight and length of the infant at implantation 6 kg and 60 cm respectively). Flecainide was prescribed in addition to the ICD implantation. After an appropriate shock the flecainide plasma levels were shown to be subtherapeutic. Readjustment of the flecainide dose resulted in adequate plasma levels. No further shocks occurred during a further 17 months follow-up period. The combination of an active can with a subcutaneous patch proved feasible, and lifesaving shocks occurred at 7 months after implantation.
我们报告了一例长QT综合征（LQTS）引发严重产前和新生儿心律失常的病例。诊断为LQT3型（R1623Q突变）。短效β受体阻滞剂、索他洛尔和美西律在预防室性心律失常复发方面均无效。婴儿7个月大时植入了植入式心律转复除颤器（ICD）（植入时婴儿体重和身长分别为6 kg和60 cm）。除植入ICD外，还开了氟卡尼。在一次适当的电击后，显示氟卡尼血浆水平低于治疗剂量。调整氟卡尼剂量后血浆水平达到了足够的浓度。在接下来的17个月随访期内未再发生电击。有源除颤罐与皮下贴片相结合被证明是可行的，植入后7个月发生了挽救生命的电击。
