scholarly journals Analysis of a Case of Brugada Syndrome Through Numerical Simulation of Ventricular Action Potential

2020 ◽  
Author(s):  
Giulia Guidi ◽  
Chiara Bartolucci ◽  
Anthony Frosio ◽  
Procolo Marchese ◽  
Annalisa Bucchi ◽  
...  
Keyword(s):  
Numerical Simulation ◽  
Action Potential ◽  
Brugada Syndrome ◽  
Ventricular Action Potential

Steep restitution of ventricular action potential duration and conduction slowing in human Brugada syndrome

Heart Rhythm ◽  
2007 ◽  
Vol 4 (8) ◽  
pp. 1087-1089 ◽  
Author(s):  
Sanjiv M. Narayan ◽  
Jason Kim ◽  
Charles Tate ◽  
Brett J. Berman
Keyword(s):  
Action Potential ◽  
Brugada Syndrome ◽  
Action Potential Duration ◽  
Ventricular Action Potential

Sodium channel kinetic changes that produce Brugada syndrome or progressive cardiac conduction system disease

2007 ◽  
Vol 292 (1) ◽  
pp. H399-H407 ◽  
Author(s):  
Zhu-Shan Zhang ◽  
Joseph Tranquillo ◽  
Valentina Neplioueva ◽  
Nenad Bursac ◽  
Augustus O. Grant
Keyword(s):  
Action Potential ◽  
Sodium Channel ◽  
Brugada Syndrome ◽  
Sodium Current ◽  
Conduction System ◽  
Cardiac Conduction ◽  
Cardiac Conduction System ◽  
Wild Type ◽  
System Disease ◽  
Conduction System Disease

Some mutations of the sodium channel gene NaV1.5 are multifunctional, causing combinations of LQTS, Brugada syndrome and progressive cardiac conduction system disease (PCCD). The combination of Brugada syndrome and PCCD is uncommon, although they both result from a reduction in the sodium current. We hypothesize that slow conduction is sufficient to cause S-T segment elevation and undertook a combined experimental and theoretical study to determine whether conduction slowing alone can produce the Brugada phenotype. Deletion of lysine 1479 in one of two positively charged clusters in the III/IV inter-domain linker causes both syndromes. We have examined the functional effects of this mutation using heterologous expression of the wild-type and mutant sodium channel in HEK-293-EBNA cells. We show that ΔK1479 shifts the potential of half-activation, V1/2m, to more positive potentials ( V1/2m = −36.8 ± 0.8 and −24.5 ± 1.3 mV for the wild-type and ΔK1479 mutant respectively, n = 11, 10). The depolarizing shift increases the extent of depolarization required for activation. The potential of half-inactivation, V1/2h, is also shifted to more positive potentials ( V1/2h = −85 ± 1.1 and −79.4 ± 1.2 mV for wild-type and ΔK1479 mutant respectively), increasing the fraction of channels available for activation. These shifts are quantitatively the same as a mutation that produces PCCD only, G514C. We incorporated experimentally derived parameters into a model of the cardiac action potential and its propagation in a one dimensional cable (simulating endo-, mid-myocardial and epicardial regions). The simulations show that action potential and ECG changes consistent with Brugada syndrome may result from conduction slowing alone; marked repolarization heterogeneity is not required. The findings also suggest how Brugada syndrome and PCCD which both result from loss of sodium channel function are sometimes present alone and at other times in combination.

scholarly journals Action potential changes due to Y1795H mutation in Brugada syndrome patients: a simulation study

2003 ◽  
Author(s):  
S. Severi ◽  
S. Vecchietti ◽  
I. Rivolta ◽  
C. Napolitano ◽  
S.G. Priori ◽  
...  
Keyword(s):  
Action Potential ◽  
Simulation Study ◽  
Brugada Syndrome

scholarly journals Inhibition of HERG K + Current and Prolongation of the Guinea‐Pig Ventricular Action Potential by 4‐Aminopyridine

2003 ◽  
Vol 549 (3) ◽  
pp. 667-672 ◽  
Author(s):  
J. M. Ridley ◽  
J. T. Milnes ◽  
Y. H. Zhang ◽  
H. J. Witchel ◽  
J. C. Hancox
Keyword(s):  
Guinea Pig ◽  
Action Potential ◽  
K Current ◽  
Ventricular Action Potential

Mathematical model of the ventricular action potential and effects of isoproterenol-induced cardiac hypertrophy in rats

2020 ◽  
Vol 49 (5) ◽  
pp. 323-342
Author(s):  
Sevgi Şengül Ayan ◽  
Ahmet K. Sırcan ◽  
Mohamedou Abewa ◽  
Ahmet Kurt ◽  
Uğur Dalaman ◽  
...  
Keyword(s):  
Mathematical Model ◽  
Action Potential ◽  
Cardiac Hypertrophy ◽  
Ventricular Action Potential
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