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目的 强调先天性长 QT综合征 ( L QTS)患者临床特点的重要性。方法 对 4例 L QTS患者的临床和心电图特点进行分析 ,并对其临床诊断、发病机制、治疗及分子遗传学研究加以讨论 ,并行 4例家系调查分析。结果 4例均为女性 ,家系中除 1例外均为女性 ,QT间期均延长 ,反复发作性晕厥 ;4例均为 Romano- Ward综合征。 结论 L QTS是一个不难诊断的疾病 ,目前已有有效的治疗措施 ,应提高诊断率 ,勿漏诊或误诊
Objective To emphasize the importance of the clinical features of patients with congenital long QT syndrome (LQTS). Methods The clinical and electrocardiographic features of 4 patients with L-QTS were analyzed, and their clinical diagnosis, pathogenesis, treatment and molecular genetics were discussed, and 4 pedigrees were investigated. Results All the 4 cases were female. Except for 1 case, all the family members were female. QT interval prolonged and recurrent syncope occurred. All 4 cases were Romano-Ward syndrome. Conclusion L QTS is a not difficult to diagnose the disease, there are effective treatment measures, should increase the diagnostic rate, do not miss or misdiagnosis