Hsieh Y C, Chow L H, Wong T D, Jeng K N, Lui P W, Lee T Y
Department of Anesthesiology, Veterans General Hospital-Taipei, Taiwan, R.O.C.
Zhonghua Yi Xue Za Zhi (Taipei). 1995 Sep;56(3):215-9.
The long QT syndrome (LQTS) is a rare disorder of cardiac conduction in children. It may lead to severe arrhythmia, syncope or sudden death. Electrolyte disturbance and the use of procainamide are closely related to the acquired LQTS. This report concerns a fatality of acquired LQTS in a boy undergoing craniotomy. After induction of anesthesia, his electrocardiogram displayed a frequent torsade de pointes type of ventricular tachycardia with prolonged QT interval. Therefore, congenital LQTS was impressed. However, the arrhythmia was aggravated by intravenous propranolol. Isoproterenol infusion was given under the diagnosis of acquired LQTS. The arrhythmia was successfully abolished and a stable hemodynamic status was maintained. However, the child could not tolerate the anesthesia which lasted for 30 hours, and eventually succumbed, despite aggressive treatment and resuscitation attempts. Differential diagnosis between the types of LQTS should be made promptly. This may be of paramount importance in the outcome since prognosis depends upon proper treatment. This case report discusses the etiology and management of this rare abnormality.
长QT综合征(LQTS)是一种儿童罕见的心脏传导障碍疾病。它可能导致严重心律失常、晕厥或猝死。电解质紊乱和使用普鲁卡因酰胺与获得性LQTS密切相关。本报告涉及一名接受开颅手术男孩发生的获得性LQTS致死病例。麻醉诱导后,他的心电图显示频发尖端扭转型室性心动过速,QT间期延长。因此,最初考虑为先天性LQTS。然而,静脉注射普萘洛尔使心律失常加重。在诊断为获得性LQTS后给予异丙肾上腺素输注。心律失常成功消除,血流动力学状态保持稳定。然而,尽管进行了积极治疗和复苏尝试,该患儿无法耐受长达30小时的麻醉,最终死亡。应及时对LQTS的类型进行鉴别诊断。这对于预后可能至关重要,因为预后取决于恰当的治疗。本病例报告讨论了这种罕见异常的病因及处理。