scholarly journals The natural history of conduction system disease in myotonic muscular dystrophy as determined by serial electrophysiologic studies.

Circulation ◽  
1979 ◽  
Vol 60 (6) ◽  
pp. 1360-1364 ◽  
Author(s):  
E N Prystowsky ◽  
E L Pritchett ◽  
A D Roses ◽  
J Gallagher
Keyword(s):  
Muscular Dystrophy ◽  
Natural History ◽  
Conduction System ◽  
System Disease ◽  
Conduction System Disease ◽  
History Of ◽  
Myotonic Muscular Dystrophy ◽  
Electrophysiologic Studies

Prognosis and Natural History of Conduction System Disease in Patients Undergoing Coronary Angiography

2020 ◽  
Vol 36 (8) ◽  
pp. 1261-1268 ◽  
Author(s):  
Robert J.H. Miller ◽  
Zhi Tan ◽  
Matthew T. James ◽  
Derek V. Exner ◽  
Danielle A. Southern ◽  
...  
Keyword(s):  
Natural History ◽  
Coronary Angiography ◽  
Conduction System ◽  
System Disease ◽  
Conduction System Disease ◽  
History Of

Novel lamin A/C mutations in two families with dilated cardiomyopathy and conduction system disease

2001 ◽  
Vol 7 (3) ◽  
pp. 249-256 ◽  
Author(s):  
Petra M. Jakobs ◽  
Emily L. Hanson ◽  
Kathy A. Crispell ◽  
Warren Toy ◽  
Hugh Keegan ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Conduction System ◽  
Lamin A ◽  
System Disease ◽  
Conduction System Disease

S.P.27 Electrophysiological study with prophylactic pacing and survival in adults with myotonic dystrophy and conduction system disease

2012 ◽  
Vol 22 (9-10) ◽  
pp. 895
Author(s):  
K. Wahbi ◽  
C. Meune ◽  
R. Porcher ◽  
H.M. Bécane ◽  
A. Lazarus ◽  
...  
Keyword(s):  
Myotonic Dystrophy ◽  
Electrophysiological Study ◽  
Conduction System ◽  
System Disease ◽  
Conduction System Disease

scholarly journals Temporal Relationship of Conduction System Disease and Ventricular Dysfunction in LMNA Cardiomyopathy

2013 ◽  
Vol 19 (4) ◽  
pp. 233-239 ◽  
Author(s):  
Chad Brodt ◽  
Jill D. Siegfried ◽  
Mark Hofmeyer ◽  
Jose Martel ◽  
Evadnie Rampersaud ◽  
...  
Keyword(s):  
Temporal Relationship ◽  
Ventricular Dysfunction ◽  
Conduction System ◽  
System Disease ◽  
Conduction System Disease ◽  
Relationship Of

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.

Sign in / Sign up

Export Citation Format

Share Document