Catecholergic polymorphic ventricular Tachycardia

The catecholergic polymorphic ventricular tachycardia is a turbid genetics cardiac rhythm characterized by an electric instability of the cardiac muscular cell exacerbated by the stimulation of the Nervous system sympathetic nerve.

The clinical demonstrations are arrhythmia S ventricular polymorphic occurring at the time of the efforts or the intense emotion S in the absence of cardiac anomaly. The ventricular Tachycardie can stop or transform into ventricular Fibrillation responsible for died in the absence of cardiac reanimation (electric shock). Meadows of 60% of the patients present a syncope or a cardiac arrest before the 20 years age.

The diagnosis is made by the description of the turbid of the rate/rhythm at the time of the tests of effort.

Two Gène S are known persons in charge of this disease: the RYR2 which are transmitted in a dominant way autosomic but with a rate of change of novo of 50% and the CASQ2 which transmits in a recessive way autosomic but with a rate of change of novo from 1 to 2%. A third gene is in the course of exploration.

The Syndrome of Brugada, the ventricular Dysplasie right-hand side arythmogene and the catecholergic polymorphic ventricular tachycardia must be systematically required in the event of family antecedent of brutal death at a young subject especially at the time of an effort.

Sources

Carlo Napolitano, Silvia G Priori, Catecholaminergic Polymorphic Ventricular Tachycardia In GeneTests: Medical Genetics Information Resource (database online). Copyright, University off Washington, Seattle. 1993-2005

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