Theisen K, Haider M, Jahrmärker H
Dtsch Med Wochenschr. 1975 May 16;100(20):1099-112. doi: 10.1055/s-0028-1106342.
The syndrome of Jervell and Lange-Nielsen with its characteristic combination of Q-T-(U) abnormalities and recurrent episodes of ventricular tachycardia (VT) or ventricular fibrillation can serve as a model for establishing recycling excitation (re-entry) as the likely cause of VT. The ECG abnormality in the Q-T-(U) segment indicates the underlying asynchronous repolarisation, probably localized in the His-Purkinje system. Asynchronous delayed repolarisation facilitates re-entry excitations. A personal case is reported which demonstrated two different patterns of inducing premature beats and VT, indicative of two different fascicular pathways of circus movement. The inducing extrasystole determines the circuit pathway and thus the ECG pattern of VT. The VT shows a fixed time relation depending on conduction delay and refractory period, which is a further indication for an underlying re-entry mechanism. Spontaneous changes in time relation or morphology point toward alterations in circuit pathways and can terminate the VT. Clinical findings in three further cases of the syndrome are reported. Furthermore, the possible general significance of these findings is highlighted by the occurrence of the described phenomena in seven patients in whom Q-T-(U) abnormalities exist merely as transient symptomatic disturbances.
耶尔韦尔和朗格-尼尔森综合征以其特征性的Q-T-(U)异常与室性心动过速(VT)或心室颤动反复发作的组合,可作为确立折返激动(折返)为室性心动过速可能病因的模型。Q-T-(U)段的心电图异常表明存在潜在的异步复极化,可能定位于希氏-浦肯野系统。异步延迟复极化促进折返激动。报告了1例个人病例,该病例展示了诱发早搏和室性心动过速的两种不同模式,提示了两种不同的环形运动束支途径。诱发的期前收缩决定了折返途径,从而决定了室性心动过速的心电图模式。室性心动过速表现出取决于传导延迟和不应期的固定时间关系,这进一步提示存在潜在的折返机制。时间关系或形态的自发变化指向折返途径的改变,并可终止室性心动过速。报告了另外3例该综合征的临床病例。此外,在7例仅存在短暂症状性干扰的Q-T-(U)异常患者中出现了所述现象,这突出了这些发现可能具有的普遍意义。