Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a primary 
electrical myocardial disease characterized by exercise- and stress-related 
ventricular tachycardia manifested as syncope and sudden death. The disease has 
a heterogeneous genetic basis, with mutations in the cardiac Ryanodine Receptor 
channel (RyR2) gene accounting for an autosomal-dominant form (CPVT1) in 
approximately 50% and mutations in the cardiac calsequestrin gene (CASQ2) 
accounting for an autosomal-recessive form (CPVT2) in up to 2% of CPVT cases. 
Both RyR2 and calsequestrin are important participants in the cardiac cellular 
calcium homeostasis. We review the physiology of the cardiac calcium 
homeostasis, including the cardiac excitation contraction coupling and myocyte 
calcium cycling. The pathophysiology of cardiac arrhythmias related to myocyte 
calcium handling and the effects of different modulators are discussed. The 
putative derangements in myocyte calcium homeostasis responsible for CPVT, as 
well as the clinical manifestations and therapeutic options available, are 
described.