Why the study?
Current treatment for long QT syndrome type 2 relies on symptom management, and no mechanism-based therapies currently exist to treat the underlying hERG channel dysfunction.
This review summarizes the pathophysiology and current management of Long QT Syndrome Type 2, while highlighting the need for and potential of mechanism-based therapies targeting hERG channel dysfunction.
β-blockade remains mainstay for LQT2 without correcting hERG dysfunction; leaves open targeted mechanism-based therapy development.
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.
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Cox et al. (2021) studied this question.
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