Garweg C, D'Orio V, Mélon P, Lancellotti P, Piérard L A
Service de Cardiologie, CHU de Liège, Belgique.
Rev Med Liege. 2010 Nov;65(11):628-33.
The congenital long QT syndrome (LQTS) is a hereditary cardiac disease characterized by a prolongation of the QT interval > 440 ms at rest ECG associated with a high risk of ventricular arrhythmias (torsade de pointe). Clinical manifestations are syncope and sudden cardiac death. The implicated genes encode cardiac ion channel subunits or proteins involved in modulating ionic currents. The diagnosis of LQTS can be complex in borderline cases. Etiology, pathogenesis, diagnosis and treatment are discussed.
先天性长QT综合征（LQTS）是一种遗传性心脏病，其特征是静息心电图时QT间期延长>440毫秒，并伴有室性心律失常（尖端扭转型室速）的高风险。临床表现为晕厥和心源性猝死。相关基因编码参与调节离子电流的心脏离子通道亚基或蛋白质。在临界病例中，LQTS的诊断可能很复杂。本文讨论了其病因、发病机制、诊断和治疗。
