title: Current topics in catecholaminergic polymorphic ventricular tachycardia.
abstract: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by emotions or exercise in patients without organic heart disease and may be polymorphic or bidirectional in nature. The prognosis of CPVT is not good, and therefore prevention of sudden death is of utmost importance. Genetic variants of CPVT includeRyR2,CASQ2,CALM2,TRD, and possiblyKCNJ2andANK2gene mutations. Hypotheses that suggest the causes of CPVT include weakened binding of FKBP12.6 andRyR2, a store overload-induced Ca2+release (SOICR), unzipping of intramolecular domain interactions inRyR2, and molecular and functional abnormalities caused by mutations in theCASQ2gene. The incidence of anRyR2anomaly in CPVTs is about 35-79%, whereas anomalies in theCASQ2gene account for 3-5% CPVTs. The ping-pong theory, suggesting that reciprocating delayed after depolarization induces bigeminy of the right and left bundle branches, may explain the pathogenesis of bidirectional ventricular tachycardia. Flecainide, carvedilol, left sympathetic nerve denervation, and catheter ablation of the PVC may serve as new therapeutic strategies for CPVT while gene-therapy may be applied to some types of CPVT in the future. Although, not all sudden cardiac deaths in CPVT patients are currently preventable, new medical and interventional therapies may improve CPVT prognosis.
authors.affiliation: Department of Pediatric Cardiology, Saitama Medical University International Medical Center, 1397-1 Yamane, Hidaka-City, Saitama 350-1298, Japan.
authors.forename: Naokata
authors.initials: N
authors.lastname: Sumitomo
keywords: Calsequestrine (CASQ2); Catecholaminergic polymorphic ventricular tachycardia (CPVT); Delayed after depolarization; Left cardiac sympathetic denervation; Ryanodine (RyR2)