Sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of Brugada and Long QT3 syndromes
Inherited arrhythmia syndromes are a known, albeit rare, cause of sudden cardiac arrest which may present with characteristic electrocardiogram changes in patients with structurally normal heart. There are a variety of distinct arrhythmogenic syndromes that arise from mutations in voltage gated sodi...
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Elsevier
2016-03-01
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Series: | Indian Pacing and Electrophysiology Journal |
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Online Access: | http://www.sciencedirect.com/science/article/pii/S0972629216313067 |
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author | U. Lakshmanadoss A. Mertens M. Gallagher I. Kutinsky B. Williamson |
author_facet | U. Lakshmanadoss A. Mertens M. Gallagher I. Kutinsky B. Williamson |
author_sort | U. Lakshmanadoss |
collection | DOAJ |
description | Inherited arrhythmia syndromes are a known, albeit rare, cause of sudden cardiac arrest which may present with characteristic electrocardiogram changes in patients with structurally normal heart. There are a variety of distinct arrhythmogenic syndromes that arise from mutations in voltage gated sodium channels, resulting in either gain or loss of function. We describe a patient with a primary inherited arrhythmia syndrome which presented as sudden cardiac arrest. Further workup revealed that her arrest was due to a combination of Brugada syndrome and Long QT3 syndrome secondary to a deleterious mutation of voltage-gated, sodium channel, type V alpha subunit (SCN5A Thr1709Met). |
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format | Article |
id | doaj.art-175c821d57a746dcaaff78ada254477b |
institution | Directory Open Access Journal |
issn | 0972-6292 |
language | English |
last_indexed | 2024-04-13T06:27:03Z |
publishDate | 2016-03-01 |
publisher | Elsevier |
record_format | Article |
series | Indian Pacing and Electrophysiology Journal |
spelling | doaj.art-175c821d57a746dcaaff78ada254477b2022-12-22T02:58:22ZengElsevierIndian Pacing and Electrophysiology Journal0972-62922016-03-01162666910.1016/j.ipej.2016.07.001Sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of Brugada and Long QT3 syndromesU. Lakshmanadoss0A. Mertens1M. Gallagher2I. Kutinsky3B. Williamson4Division of Cardiology, LSUHSC Shreveport, LA, United StatesDepartment of Medicine, William Beaumont Hospital, Oakland University School of Medicine, Royal Oak, MI, United StatesDivision of Cardiology, William Beaumont Hospital, Oakland University School of Medicine, Royal Oak, MI, United StatesDivision of Cardiology, William Beaumont Hospital, Oakland University School of Medicine, Royal Oak, MI, United StatesDivision of Cardiology, William Beaumont Hospital, Oakland University School of Medicine, Royal Oak, MI, United StatesInherited arrhythmia syndromes are a known, albeit rare, cause of sudden cardiac arrest which may present with characteristic electrocardiogram changes in patients with structurally normal heart. There are a variety of distinct arrhythmogenic syndromes that arise from mutations in voltage gated sodium channels, resulting in either gain or loss of function. We describe a patient with a primary inherited arrhythmia syndrome which presented as sudden cardiac arrest. Further workup revealed that her arrest was due to a combination of Brugada syndrome and Long QT3 syndrome secondary to a deleterious mutation of voltage-gated, sodium channel, type V alpha subunit (SCN5A Thr1709Met).http://www.sciencedirect.com/science/article/pii/S0972629216313067Sudden cardiac arrestBrugada syndromeLQT3 syndromeSCN5A mutation |
spellingShingle | U. Lakshmanadoss A. Mertens M. Gallagher I. Kutinsky B. Williamson Sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of Brugada and Long QT3 syndromes Indian Pacing and Electrophysiology Journal Sudden cardiac arrest Brugada syndrome LQT3 syndrome SCN5A mutation |
title | Sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of Brugada and Long QT3 syndromes |
title_full | Sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of Brugada and Long QT3 syndromes |
title_fullStr | Sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of Brugada and Long QT3 syndromes |
title_full_unstemmed | Sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of Brugada and Long QT3 syndromes |
title_short | Sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of Brugada and Long QT3 syndromes |
title_sort | sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of brugada and long qt3 syndromes |
topic | Sudden cardiac arrest Brugada syndrome LQT3 syndrome SCN5A mutation |
url | http://www.sciencedirect.com/science/article/pii/S0972629216313067 |
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