English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools annual

Global: Zendy Free Plan

Global: Zendy Tools monthly

Global: Zendy Plus monthly

In this issue of the Journal, Kawata and colleagues7 evaluate the recurrence rate of fatal cardiac events after initiation of appropriate medical therapy in 34 CPVT patients with RyR2 mutations. During 7.4 years of follow-up, 7 of the 34 patients developed fatal cardiac events, and among them, 6 patients (85.7%) were not compliant with exercise restriction or medication therapy. In other words, the majority of CPVT patients were well controlled by appropriate medical therapy, emphasizing the importance of drug compliance and advice to the atecholaminergic polymorphic ventricular tachycardia (CPVT) is a malignant inherited arrhythmia syndrome characterized by bidirectional or polymorphic ventricular arrhythmias under conditions of increased sympathetic activity in young patients without structural heart disease.1 The common autosomal-dominant form has been linked to mutations in the gene encoding the cardiac calcium-release channel (RYR2).2 To date at least 130 unique, almost exclusively missense, mutations in RYR2 have been reported.3 A less common but more severe autosomal-recessive form is caused by mutations in the gene encoding cardiac calsequestrin (CASQ2), the major calcium-binding protein in the sarcoplasmic reticulum (SR).4 Mutations in these genes have been identified in approximately 60% of patients diagnosed as CPVT,2 and result in inappropriate calcium leakage from the SR, leading to cytosolic calcium overload generating delayed afterdepolarizations, triggered activity, and ventricular arrhythmias, particularly under increased β-adrenergic tone. CPVT has become recognized as C

Long-Term Prognosis of Catecholaminergic Polymorphic Ventricular Tachycardia Patients With Ryanodine Receptor (RYR2) Mutations