家族性Q—T间期延长综合征是儿童猝死的重要原因,日渐受到儿科医师的关注。目前国内资料尚少,本文对国外近年有关这一综合征的研究做一介绍。历史与概念 1957年Jerveil、lange和Nielsen等报告了一对健康双亲的6个子女中4人患有先天性耳聋、反复发作的晕厥或猝死,心电图上可见显著的Q—T间期延长。1963年Romano、1964年Ward等相继报告了类似的家族性病例,但不伴先天性耳聋。此后,家族性Q—T间期长延的报告日渐增多。一些非家族性散发病例亦屡有发现。 Familial Q-T prolongation syndrome is an important cause of sudden death in children, which is receiving increasing attention from pediatricians. At present, there is still little domestic data. This paper introduces the research on this syndrome abroad in recent years. History and Concepts In 1957 Jerveil, Lange, and Nielsen, et al. Reported that 4 of 6 children of a healthy parent had congenital deafness, recurrent syncope or sudden death, and significant Q-T prolongation was seen on the ECG. Romano in 1963, Ward in 1964, etc. have reported a similar case of familial, but not with deafness. Since then, there has been an increasing number of reports of prolonged Q-T familial duration. Some cases of non-familial dissemination are also frequently found.