Catecholaminergic polymorphic ventricular tachycardia: disease with different faces.
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چکیده
منابع مشابه
Paradoxical Effect of Increased Diastolic Ca Release and Decreased Sinoatrial Node Activity in a Mouse Model of Catecholaminergic Polymorphic Ventricular Tachycardia
Catecholaminergic polymorphic ventricular tachycardia is a congenital arrhythmogenic disease linked to -adrenergic–induced ventricular arrhythmias that has a high mortality in children and young adults when untreated. As common features of the catecholaminergic polymorphic ventricular tachycardia phenotype, supraventricular arrhythmias have also been described in catecholaminergic polymorphic v...
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 کاملLeft cardiac sympathetic denervation for catecholaminergic polymorphic ventricular tachycardia.
Catecholaminergic polymorphic ventricular tachycardia is a potentially lethal disease characterized by adrenergically mediated ventricular arrhythmias manifested especially in children and teenagers. Beta-blockers are the cornerstone of therapy, but some patients do not have a complete response to this therapy and receive an implantable cardioverter-defibrillator (ICD). Given the nature of cate...
متن کاملCatecholaminergic polymorphic ventricular tachycardia from bedside to bench and beyond.
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 ...
متن کاملMutations in the cardiac ryanodine receptor gene (hRyR2) underlie catecholaminergic polymorphic ventricular tachycardia.
BACKGROUND Catecholaminergic polymorphic ventricular tachycardia is a genetic arrhythmogenic disorder characterized by stress-induced, bidirectional ventricular tachycardia that may degenerate into cardiac arrest and cause sudden death. The electrocardiographic pattern of this ventricular tachycardia closely resembles the arrhythmias associated with calcium overload and the delayed afterdepolar...
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عنوان ژورنال:
- Circulation. Arrhythmia and electrophysiology
دوره 8 3 شماره
صفحات -
تاریخ انتشار 2015