本文通过二例及家系发病调查,对家族性 Q—T 间期延长综合征的病因、发病机理、临床、心电图表现及治疗进行了讨论。指出:本病虽多认为是遗传性疾病,但亦有报告认为有的病例为慢性病毒感染或某种非感染性变性所致。病机可能与植物神经系统功能障碍有关。本病多自幼起病,可伴有或不伴有先天性耳聋,家族中常有猝死的病例。临床特点为反复出现晕厥与抽搐,发作时心电图常表现为多形性室速,部分符合尖端扭转型,间有室扑、室颤。发作时宜选用阿托品、异丙肾等心脏兴奋剂及人工心脏起搏治疗,奎尼丁一类心肌抑制剂为禁忌,间歇期可口服心得安、阿托品等预防多形性室速发作。 In this paper, the etiology, pathogenesis, clinical, electrocardiogram and treatment of familial Q-T prolongation syndrome were discussed through two cases and family history of disease. Pointed out: Although the disease is often considered as a genetic disease, but there are also reports that some cases of chronic viral infection or a non-infective degeneration caused. Pathogenesis may be related to autonomic dysfunction. The disease more than childhood, with or without congenital deafness, often sudden death in the family. Clinical features of recurrent syncope and convulsions, electrocardiogram seizures often pleomorphic ventricular tachycardia, in part, meet the tip torsion type, between the room flutter, ventricular fibrillation. Episodes should use atropine, isoprenaline and other cardiac stimulants and artificial cardiac pacing treatment, quinidine a class of myocardial inhibitors is taboo, intermittent period may be propranolol, atropine and other prevention of pleomorphic VT onset.