This review highlights the role of cardiac calsequestrin deficiency in arrhythmia susceptibility, particularly in CPVT, and discusses potential new drug therapies.
Does not support changes in CPVT management; leaves open targeted therapy development pending clinical validation.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited disease characterized by physical or emotional stress-induced ventricular arrhythmias in the absence of any structural heart disease or QT prolongation. Thus far, mutations in genes encoding the sarcoplasmic reticulum Ca(2+) release channel (RYR2) and the sarcoplasmic reticulum Ca(2+) binding protein cardiac calsequestrin (CASQ2) have been identified in CPVT patients. Here, we review the role of cardiac calsequestrin in health and disease, with a particular focus on how calsequestrin deficiency can cause arrhythmia susceptibility. Clinical implications and a promising new drug therapy for CPVT are discussed.
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Chopra et al. (2009) studied this question.
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