Garg A, Finneran W, Feld G K
Department of Medicine, Division of Cardiology, University of California, San Diego, USA.
J Cardiovasc Electrophysiol. 1998 Jun;9(6):642-7. doi: 10.1111/j.1540-8167.1998.tb00947.x.
A case is presented of an 18-year-old male who had been resuscitated following an episode of sudden death due to ventricular fibrillation. The patient was noted to have an abnormal deflection in the terminal QRS on surface ECG and an abnormal signal-averaged ECG demonstrating a late potential coincident with the terminal QRS abnormality on the ECG. The patient had easily inducible polymorphic ventricular tachycardia during electrophysiologic study, which was suppressed by quinidine but not by procainamide or beta blockers. The surface ECG and signal-averaged ECG also were normalized by quinidine but not by procainamide or beta blockers. The patient had no further arrhythmias on quinidine for 6 years until he inexplicably discontinued his medication and died suddenly shortly thereafter. The present case may represent a unique familial sudden death syndrome or possibly a variant of the sudden death syndrome associated with right bundle branch block and ST elevation in V1 through V3. Currently available data suggest that, in such patients, an implantable cardioverter defibrillator may provide better protection from sudden death than does antiarrhythmic drug therapy.
本文报告一例18岁男性，该患者因室颤导致心搏骤停后经复苏成功。体表心电图显示患者终末QRS波有异常偏移，信号平均心电图异常，显示与心电图终末QRS波异常同时出现的晚电位。在电生理研究中，该患者易于诱发多形性室性心动过速，可被奎尼丁抑制，但不能被普鲁卡因胺或β受体阻滞剂抑制。体表心电图和信号平均心电图也可被奎尼丁恢复正常，但不能被普鲁卡因胺或β受体阻滞剂恢复正常。该患者服用奎尼丁6年未再发生心律失常，直至他不明原因停药，此后不久突然死亡。本病例可能代表一种独特的家族性猝死综合征，或者可能是与右束支传导阻滞及V1至V3导联ST段抬高相关的猝死综合征的一种变异型。目前可得的数据表明，在此类患者中，植入式心脏复律除颤器可能比抗心律失常药物治疗能更好地预防猝死。
