Taşar Mehmet, Yaman Nur Dikmen, Dursin Huseyin, Şimşek Murat, Özgür Senem
Pediatric Cardiovascular Surgery Department, Dr. Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital, Ankara, Turkey.
Pediatric Cardiology Department, Dr. Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital, Ankara, Turkey.
Cardiol Young. 2020 Dec;30(12):1880-1881. doi: 10.1017/S1047951120003108. Epub 2020 Sep 30.
Congenital Long QT Syndrome (LQTS) is a dangerous arrhythmic disorder that can be diagnosed in children with bradycardia. It is characterised by a prolonged QT interval and torsades de pointes that may cause sudden death. Long QT syndrome is an ion channelopathy with complex molecular and physiological infrastructure. Unlike the acquired type, congenital LQTS has a genetic inheritance and it may be diagnosed by syncope, stress in activity, cardiac dysfunction, sudden death or sometimes incidentally. Permanent pacemaker implantation is required for LQTS with resistant bradycardia even in children to resolve symptoms and avoid sudden death.
先天性长QT综合征（LQTS）是一种危险的心律失常性疾病，可在心动过缓的儿童中诊断出来。其特征是QT间期延长和尖端扭转型室速，可能导致猝死。长QT综合征是一种具有复杂分子和生理基础的离子通道病。与后天性类型不同，先天性LQTS具有遗传遗传性，可通过晕厥、活动时的应激、心脏功能障碍、猝死或有时偶然诊断出来。即使是儿童，对于伴有难治性心动过缓的LQTS，也需要植入永久性起搏器以缓解症状并避免猝死。
