Guthlin M, Huettl S, Krieg H J, Nowak F G, Antoni D H
I. Medizinische Abteilung, Stadtisches Krankenhaus Munchen-Bogehausen.
Z Kardiol. 1996 Jan;85(1):53-8.
The long QT syndrome (LQTS) is an inherited disorder associated with recurrent syncope and sudden death from ventricular arrhythmias. It is characterized by a prolonged QT interval on the ECG. A case is discussed in which a patient presented with recurrent syncopal episodes on exertion associated with a slightly prolonged QT interval. Family history revealed an increased incidence of sudden deaths in two generations. Some reports on similar patients suggest that there may be a forme fruste of the long QT syndrome. The additional diagnostic values of exercise stress test, Valsalva maneuver and genetic testing are evaluated. After analysis of the patient's and mother's ECGs the diagnosis of a forme fruste of LQTS is made. This case is presented to emphasize the importance of diagnosis of borderline cases of LQTS because of the high risk of sudden death for untreated patients.
长QT综合征(LQTS)是一种遗传性疾病,与反复晕厥及室性心律失常导致的猝死有关。其特征是心电图上QT间期延长。本文讨论了一例患者,该患者在运动时出现反复晕厥发作,伴有QT间期轻度延长。家族史显示两代人中猝死发生率增加。一些关于类似患者的报告表明,可能存在长QT综合征的顿挫型。评估了运动负荷试验、瓦尔萨尔瓦动作和基因检测的额外诊断价值。通过分析患者及其母亲的心电图,做出了长QT综合征顿挫型的诊断。本文呈现此病例是为了强调诊断长QT综合征临界病例的重要性,因为未经治疗的患者猝死风险很高。