scholarly journals The Role of Flecainide in the Management of Catecholaminergic Polymorphic Ventricular Tachycardia

2016 ◽  
Vol 5 (1) ◽  
pp. 45 ◽  
Author(s):  
Krystien VV Lieve ◽  
◽  
Arthur A Wilde ◽  
Christian van der Werf ◽  
◽  
...  
Keyword(s):  
Heart Disease ◽  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Structural Heart Disease ◽  
Current Data ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Β Blockers

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare but severe genetic cardiac arrhythmia disorder, with symptoms including syncope and sudden cardiac death due to polymorphic VT or ventricular fibrillation typically triggered by exercise or emotions in the absence of structural heart disease. The cornerstone of medical therapy for CPVT is β -blockers. However, recently flecainide has been added to the therapeutic arsenal for CPVT. In this review we summarise current data on the efficacy and role of flecainide in the treatment of CPVT.

The Role of Flecainide in the Management of Catecholaminergic Polymorphic Ventricular Tachycardia

2016 ◽  
pp. 1a
Author(s):  
Krystien VV Lieve ◽  
Arthur A Wilde ◽  
Christian van der Werf ◽  
◽  
◽  
...  
Keyword(s):  
Heart Disease ◽  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Structural Heart Disease ◽  
Current Data ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Β Blockers

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare but severe genetic cardiac arrhythmia disorder, with symptoms including syncope and sudden cardiac death due to polymorphic VT or ventricular fibrillation typically triggered by exercise or emotions in the absence of structural heart disease. The cornerstone of medical therapy for CPVT is β -blockers. However, recently flecainide has been added to the therapeutic arsenal for CPVT. In this review we summarise current data on the efficacy and role of flecainide in the treatment of CPVT.

Short Term Variability of Repolarization Predicts Ventricular Tachycardia and Sudden Cardiac Death in Patients with Structural Heart Disease

Heart Rhythm ◽  
2010 ◽  
Vol 7 (11) ◽  
pp. 1720-1721
Author(s):  
Peter Oosterhoff ◽  
Larisa G. Tereshchenko ◽  
Marcel A.G. van der Heyden ◽  
Raja N. Ghanem ◽  
Paul J. De Groot ◽  
...  
Keyword(s):  
Heart Disease ◽  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Structural Heart Disease ◽  
Short Term ◽  
Short Term Variability

Sustained monomorphic ventricular tachycardia: the role of catheter ablation

ESC CardioMed ◽  
2018 ◽  
pp. 2279-2288
Author(s):  
Tilman Maurer ◽  
William G. Stevenson ◽  
Karl-Heinz Kuck
Keyword(s):  
Heart Disease ◽  
Ventricular Tachycardia ◽  
Catheter Ablation ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Therapeutic Option ◽  
Structural Heart Disease ◽  
Premature Ventricular Contractions ◽  
Monomorphic Ventricular Tachycardia ◽  
Premature Beats

Monomorphic ventricular tachycardia (VT) may occur in the presence or absence of structural heart disease. The standard therapy for patients with structural heart disease at high risk of sudden cardiac death due to VT is the implantable cardioverter defibrillator (ICD). While ICDs effectively terminate VT and prevent sudden cardiac death, they do not prevent recurrent episodes of VT, since the underlying arrhythmogenic substrate remains unchanged. However, shocks from an ICD increase mortality and impair quality of life. These limitations as well as continuous advancements in technology have made catheter ablation an important treatment strategy for patients with structural heart disease presenting with VT. Idiopathic ventricular arrhythmias include premature ventricular contractions and VT occurring in the absence of overt structural heart disease. In this setting, catheter ablation has evolved as the primary therapeutic option for symptomatic ventricular premature beats and sustained VTs and is curative in most cases. This chapter presents an overview of the principles of invasive diagnosis and treatment of monomorphic VTs in patients with and without structural heart disease and delineates the clinical outcome of catheter ablation. Finally, the chapter provides an outlook to the future, discussing potential directions and upcoming developments in the field of catheter ablation of monomorphic VT.

scholarly journals Human RyR2 (Ryanodine Receptor 2) Loss-of-Function Mutations

2021 ◽  
Vol 14 (9) ◽  
Author(s):  
Yanhui Li ◽  
Jinhong Wei ◽  
Wenting Guo ◽  
Bo Sun ◽  
John Paul Estillore ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Stress Testing ◽  
Functional Characterization ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Exercise Stress ◽  
Polymorphic Ventricular Tachycardia ◽  
Loss Of Function

Background: The overall objective of the present study is to extend our understanding of the clinical phenotype and underlying mechanism of a newly discovered cardiac arrhythmia syndrome through a multicenter study. Gain-of-function mutations in the cardiac Ca 2+ release channel (RyR2 [ryanodine receptor 2]) cause catecholaminergic polymorphic ventricular tachycardia, whereas loss-of-function RyR2 mutations are linked to a new cardiac arrhythmia disorder termed Ca 2+ -release deficiency syndrome (CRDS). Catecholaminergic polymorphic ventricular tachycardia is an inherited arrhythmia disorder characterized by stress-induced bidirectional and polymorphic ventricular tachyarrhythmias and is routinely diagnosed by using exercise stress testing. Conversely, RyR2-CRDS is characterized by ventricular arrhythmias and sudden cardiac death but a negative exercise stress testing for catecholaminergic polymorphic ventricular tachycardia. There are currently no clinical diagnostic tests for CRDS and affected patients may manifest with sudden cardiac death as their first symptom. In the absence of effective clinical diagnostic tools, in vitro functional characterization of associated RyR2 mutations provides an alternative means to identify potential cases of CRDS. Methods: We searched for patients presenting with phenotypes compatible with CRDS that have RyR2 mutations and performed in vitro functional characterization. Results: We found that 3 novel (G570D, R4147K, and A4203V) and 2 previously reported (M4109R and A4204V) RyR2 mutations associated with CRDS phenotypes markedly reduced caffeine-induced Ca 2+ release and store overload-induced Ca 2+ release. We also characterized 2 additional loss-of-function RyR2 mutations previously reported (Q3925E and L4769S) that are located in the central and channel pore-forming domains critical for Ca 2+ activation and channel gating. Q3925E was identified through postmortem genetic testing in an individual who died suddenly, while L4769S is a variant of uncertain significance reported in ClinVar, suggesting that RyR2 CRDS may be under detected. Conclusions: These findings provide further support for the existence of an emerging RyR2 loss-of-function associated arrhythmia syndrome (CRDS) and shed new insights into the disease mechanism.

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