Department of Medicine, St George's, University of London, London, UK.
Department of Medicine, Imperial College London, London, UK.
Future Cardiol. 2021 Nov;17(8):1453-1463. doi: 10.2217/fca-2020-0234. Epub 2021 Mar 19.
Long QT syndrome type 2 is a life-threatening disorder of cardiac electrophysiology. It can lead to sudden cardiac death as a result of QT prolongation and can remain undetected until it presents clinically in the form of life-threatening cardiac arrythmias. Current treatment relies on symptom management largely through the use of β-adrenergic blockade and presently no mechanism-based therapies exist to treat the dysfunction in the channels responsible for the rapid delayed rectifier K current which is the pathological source of long QT syndrome type 2. We review the pathophysiology, diagnosis and current management of this life-threatening condition and also analyze some promising potential mechanism-based therapies.
长 QT 综合征 2 型是一种危及生命的心脏电生理学疾病。它可导致 QT 延长导致的心脏性猝死，并且在以危及生命的心律失常形式出现之前，可能一直未被发现。目前的治疗主要依赖于症状管理，主要通过β肾上腺素能阻滞，目前还没有针对负责快速延迟整流钾电流的通道功能障碍的机制治疗方法，而该通道是长 QT 综合征 2 型的病理来源。我们回顾了这种危及生命的疾病的病理生理学、诊断和当前的治疗方法，并分析了一些有前途的潜在机制治疗方法。
