Yanagida H, Kemi C, Suwa K
Anesth Analg. 1976 Nov-Dec;55(6):782-7. doi: 10.1213/00000539-197611000-00007.
The association of a prolonged Q-T interval, congenital deafness, and syncopal attacks due to ventricular fibrillation following emotional or physical stress is known as the Jervell-Lange-Nielsen syndrome. Absence of the congenital deafness characterizes the otherwise identical Romano-Ward syndrome. Both conditions have a high mortality rate and are recognized as contributing to sudden death in children. Although the pathogenetic factors are not yet completely elucidated, the condition is well enough understood to permit effective treatment. The authors performed a left stellate ganglion block in a 12-year-old girl with a mild form of the Romano-Ward syndrome, shortening the Q-T interval and eliminating the ventricular tachyarrhythmia. The left stellete ganglion block should be considered by anesthesiologists as an emergency measure, effective in interrupting the attack.
长Q-T间期、先天性耳聋以及情绪或身体应激后因室颤导致的晕厥发作之间的关联被称为杰韦尔-朗格-尼尔森综合征。先天性耳聋的缺失是与之相似的 Romano-Ward 综合征的特征。这两种病症的死亡率都很高,被认为是导致儿童猝死的原因。虽然发病机制尚未完全阐明,但对该病症的了解已足以进行有效治疗。作者对一名患有轻度 Romano-Ward 综合征的12岁女孩实施了左侧星状神经节阻滞,缩短了Q-T间期并消除了室性快速心律失常。麻醉医生应将左侧星状神经节阻滞视为一种紧急措施,对中断发作有效。