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The congenital long QT syndrome Type 3: An update

Congenital long QT syndrome type 3 (LQT3) is the third in frequency compared to the 15 forms known currently of congenital long QT syndrome (LQTS). Cardiac events are less frequent in LQT3 when compared with LQT1 and LQT2, but more likely to be lethal; the likelihood of dying during a cardiac event...

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Autores principales: Pérez-Riera, Andrés Ricardo, Barbosa-Barros, Raimundo, Daminello Raimundo, Rodrigo, da Costa de Rezende Barbosa, Marianne Penachini, Esposito Sorpreso, Isabel Cristina, de Abreu, Luiz Carlos
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5840852/
https://www.ncbi.nlm.nih.gov/pubmed/29101013
http://dx.doi.org/10.1016/j.ipej.2017.10.011
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author Pérez-Riera, Andrés Ricardo
Barbosa-Barros, Raimundo
Daminello Raimundo, Rodrigo
da Costa de Rezende Barbosa, Marianne Penachini
Esposito Sorpreso, Isabel Cristina
de Abreu, Luiz Carlos
author_facet Pérez-Riera, Andrés Ricardo
Barbosa-Barros, Raimundo
Daminello Raimundo, Rodrigo
da Costa de Rezende Barbosa, Marianne Penachini
Esposito Sorpreso, Isabel Cristina
de Abreu, Luiz Carlos
author_sort Pérez-Riera, Andrés Ricardo
collection PubMed
description Congenital long QT syndrome type 3 (LQT3) is the third in frequency compared to the 15 forms known currently of congenital long QT syndrome (LQTS). Cardiac events are less frequent in LQT3 when compared with LQT1 and LQT2, but more likely to be lethal; the likelihood of dying during a cardiac event is 20% in families with an LQT3 mutation and 4% with either an LQT1 or an LQT2 mutation. LQT3 is consequence of mutation of gene SCN5A which codes for the Nav1.5 Na(+) channel α-subunit and electrocardiographically characterized by a tendency to bradycardia related to age, prolonged QT/QTc interval (mean QTc value 478 ± 52 ms), accentuated QT dispersion consequence of prolonged ST segment, late onset of T wave and frequent prominent U wave because of longer repolarization of the M cell across left ventricular wall.
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spelling pubmed-58408522018-03-07 The congenital long QT syndrome Type 3: An update Pérez-Riera, Andrés Ricardo Barbosa-Barros, Raimundo Daminello Raimundo, Rodrigo da Costa de Rezende Barbosa, Marianne Penachini Esposito Sorpreso, Isabel Cristina de Abreu, Luiz Carlos Indian Pacing Electrophysiol J Review Congenital long QT syndrome type 3 (LQT3) is the third in frequency compared to the 15 forms known currently of congenital long QT syndrome (LQTS). Cardiac events are less frequent in LQT3 when compared with LQT1 and LQT2, but more likely to be lethal; the likelihood of dying during a cardiac event is 20% in families with an LQT3 mutation and 4% with either an LQT1 or an LQT2 mutation. LQT3 is consequence of mutation of gene SCN5A which codes for the Nav1.5 Na(+) channel α-subunit and electrocardiographically characterized by a tendency to bradycardia related to age, prolonged QT/QTc interval (mean QTc value 478 ± 52 ms), accentuated QT dispersion consequence of prolonged ST segment, late onset of T wave and frequent prominent U wave because of longer repolarization of the M cell across left ventricular wall. Elsevier 2017-10-31 /pmc/articles/PMC5840852/ /pubmed/29101013 http://dx.doi.org/10.1016/j.ipej.2017.10.011 Text en Copyright © 2017, Indian Heart Rhythm Society. Production and hosting by Elsevier B.V. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Review
Pérez-Riera, Andrés Ricardo
Barbosa-Barros, Raimundo
Daminello Raimundo, Rodrigo
da Costa de Rezende Barbosa, Marianne Penachini
Esposito Sorpreso, Isabel Cristina
de Abreu, Luiz Carlos
The congenital long QT syndrome Type 3: An update
title The congenital long QT syndrome Type 3: An update
title_full The congenital long QT syndrome Type 3: An update
title_fullStr The congenital long QT syndrome Type 3: An update
title_full_unstemmed The congenital long QT syndrome Type 3: An update
title_short The congenital long QT syndrome Type 3: An update
title_sort congenital long qt syndrome type 3: an update
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5840852/
https://www.ncbi.nlm.nih.gov/pubmed/29101013
http://dx.doi.org/10.1016/j.ipej.2017.10.011
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