Establishing a System for Viral Delivered Gene Therapy as a Treatment for CPVT Kurtzwald-Josefson, Efrat Catecholaminergic polymorphic ventricular tachycardia (CPVT), a lethal human arrhythmia
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Exercise training improves cardiac function and attenuates arrhythmia in CPVT mice.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a lethal ventricular arrhythmia evoked by physical or emotional stress. Recessively inherited CPVT is caused by either missense or null-allele mutations in the cardiac calsequestrin (CASQ2) gene. It was suggested that defects in CASQ2 cause protein deficiency and impair Ca(2+) uptake to the sarcoplasmic reticulum and Ca(2+)-depende...
متن کاملArrhythmogenic Mechanism of Catecholaminergic Polymorphic Ventricular Tachycardia
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a highly lethal form of inherited arrhythmogenic disease characterized by adrenergically mediated polymorphic VT. The identification of the genetic substrate of the disease has allowed to achieve important milestones in the understanding of the arrhythmogenic mechanisms of the disease. Abnormal calcium leak from the mutant cardiac ...
متن کاملThe Impact of Verapamil on Catecholamine Polymorphic Ventricular Tachycardia
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a disease of mutation of family genes associated with exercise-induced syncope or sudden death. We report a case of a 27-year-old female with CPVT who had experienced several episodes of syncope and had been prescribed beta-blocker regularly. Sudden onset of ventricular arrhythmia occurred and the patient was resuscitated but remai...
متن کاملCurrent topics in catecholaminergic polymorphic ventricular tachycardia
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 include RyR2, CASQ2, CALM2, TRD, and possibly KCNJ2 and ANK2 gene mutations. Hypot...
متن کاملSuccessful treatment of catecholaminergic polymorphic ventricular tachycardia with flecainide: a case report and review of the current literature.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmogenic disease that can cause sudden cardiac death due to ventricular fibrillation (VF). While pharmacological therapy with beta-blockers and/or Ca(2)(+) antagonists is often unreliable, a recent study has demonstrated that flecainide can effectively suppress arrhythmia in a murine model of CPVT as well as clin...
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تاریخ انتشار 2012