Open AccessLong QT syndrome type 2: mechanism-based therapies
Author(s) -
Kofi Cox,
Brian Wang
Publication year - 2021
Publication title -
future cardiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.392
H-Index - 33
eISSN - 1744-8298
pISSN - 1479-6678
DOI - 10.2217/fca-2020-0234
Subject(s) - medicine , sudden cardiac death , long qt syndrome , disease , herg , qt interval , cardiology , cardiac arrhythmia , sudden death , intensive care medicine , neuroscience , atrial fibrillation , potassium channel , biology
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 hERG 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.