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- W2028450513 abstract "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." @default.
- W2028450513 created "2016-06-24" @default.
- W2028450513 creator A5020537575 @default.
- W2028450513 creator A5020885257 @default.
- W2028450513 creator A5048890376 @default.
- W2028450513 date "2009-01-01" @default.
- W2028450513 modified "2023-10-15" @default.
- W2028450513 title "Catecholaminergic Polymorphic Ventricular Tachycardia from Bedside to Bench and Beyond" @default.
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