Tachycardia in Mice and in Patients Accelerated Sinus Rhythm Prevents Catecholaminergic Polymorphic Ventricular

نویسندگان

  • Björn C. Knollmann
  • Michela Faggioni
  • Hyun Seok Hwang
  • Christian van der Werf
  • Ineke Nederend
چکیده

Michela Faggioni, Hyun Seok Hwang, Christian van der Werf, Ineke Nederend, Prince J. Tachycardia in Mice and in Patients Accelerated Sinus Rhythm Prevents Catecholaminergic Polymorphic Ventricular Print ISSN: 0009-7330. Online ISSN: 1524-4571 Copyright © 2013 American Heart Association, Inc. All rights reserved. is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Circulation Research doi: 10.1161/CIRCRESAHA.111.300076 2013;112:689-697; originally published online January 7, 2013; Circ Res. http://circres.ahajournals.org/content/112/4/689 World Wide Web at: The online version of this article, along with updated information and services, is located on the http://circres.ahajournals.org/content/suppl/2013/01/07/CIRCRESAHA.111.300076.DC1.html Data Supplement (unedited) at:

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Accelerated sinus rhythm prevents catecholaminergic polymorphic ventricular tachycardia in mice and in patients.

RATIONALE Catecholaminergic polymorphic ventricular tachycardia (CPVT) is caused by mutations in cardiac ryanodine receptor (RyR2) or calsequestrin (Casq2) genes. Sinoatrial node dysfunction associated with CPVT may increase the risk for ventricular arrhythmia (VA). OBJECTIVE To test the hypothesis that CPVT is suppressed by supraventricular overdrive stimulation. METHODS AND RESULTS Using ...

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A Case of Catecholaminergic Polymorphic Ventricular Tachycardia

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a familial cardiac arrhythmia that is related to RYR2 or CASQ2 gene mutation. It occurs in patients with structurally normal heart and causes exercise-emotion-triggered syncope and sudden cardiac death. We experienced a case of CPVT in an 11 year-old female patient who was admitted for sudden cardiovascular collapse. The initial el...

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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...

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Paradoxical effect of increased diastolic Ca(2+) release and decreased sinoatrial node activity in a mouse model of catecholaminergic polymorphic ventricular tachycardia.

BACKGROUND Catecholaminergic polymorphic ventricular tachycardia is characterized by stress-triggered syncope and sudden death. Patients with catecholaminergic polymorphic ventricular tachycardia manifest sinoatrial node (SAN) dysfunction, the mechanisms of which remain unexplored. METHODS AND RESULTS We investigated SAN [Ca(2+)](i) handling in mice carrying the catecholaminergic polymorphic ...

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Just Sinus Bradycardia or Something More Serious?

An asymptomatic 5-year-old girl presented with bradycardia during a routine well-child visit. Further evaluation revealed profound sinus bradycardia, exercise-induced bidirectional ventricular tachycardia, and supraventricular tachycardia. An echocardiogram showed heavy trabeculations in the left ventricular myocardium. This patient's presentation suggested catecholaminergic polymorphic ventric...

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تاریخ انتشار 2013