Pharmacological Targeting in Inherited Arrhythmia Syndromes
S. Zumhagen, N. Strutz-Seebohm, G. Seebohm and E. Schulze-Bahr
Affiliation: Institut für Genetik von Herzerkrankungen (IfGH), Universitätsklinikum Münster (UKM) Albert- Schweitzer-Campus 1, Gebäude D3 D-48149 Münster, Germany.
The development of pharmacologic agents for the treatment of diseases is still challenging, especially in rare
inherited arrhythmia syndromes like long and short QT syndrome, Brugada syndrome and catecholaminergic polymorphic
ventricular tachycardia. The underling pathophysiologic mechanism of these inherited diseases is in most cases either a
gain- or a loss-of-function due to mutations in ion channel genes. Although the biophysical properties of mutant channel
subunits are well studied, little is known about the targeting effect of specific pharmacologic agents. In this review, we focus
on the therapeutic (in vivo) and the experimental (in vitro) approaches in the most common inherited forms.
Keywords: BrS, brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, CPVT, Long QT syndrome,
LQTS, review, short QT syndrome, SQTS, therapeutic approach.
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