Noble W E, Chaudhuri P, Qazi M A
Department of Medicine, Robert C. Byrd Health Sciences Center of WVU, Morgantown.
W V Med J. 1994 Apr;90(4):143-4.
Idiopathic long q-t syndrome (LQTS) is an infrequently occurring familial disorder in which affected family members have an abnormally prolonged q-tc interval with syncope, ventricular arrythmias and sudden death. In this article, we present the case of a 54-year-old female admitted for syncope, who was on no medications except for insulin. Her electrocardiogram on admission had prolonged q-tc interval (0.50 ms.). She had repeated episodes of torsades de pointes during her hospitalization, which were later controlled by beta-blockers. Electrocardiograms of her mother and daughter showed asymptomatic prolonged q-tc interval. This syndrome has an autosomal dominant pattern of transmission and it was first described by Romano and Ward in a patient with normal hearing. Our case is unusual because this condition presented so late in life.
特发性长QT综合征(LQTS)是一种罕见的家族性疾病,患病家庭成员的QTc间期异常延长,伴有晕厥、室性心律失常和猝死。在本文中,我们介绍了一名因晕厥入院的54岁女性病例,她除胰岛素外未服用任何药物。入院时她的心电图显示QTc间期延长(0.50毫秒)。她在住院期间反复发作尖端扭转型室速,后来通过β受体阻滞剂得到控制。她母亲和女儿的心电图显示无症状的QTc间期延长。该综合征具有常染色体显性遗传模式,最初由罗曼诺和沃德在一名听力正常的患者中描述。我们的病例不同寻常,因为这种疾病在如此晚的年龄才出现。