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Short QT syndrome: The current evidences of diagnosis and management
There are many cardiac arrhythmias and sudden cardiac death (SCD) related to channelopathies or ion channel disorders. Short QT syndrome (SQTS) is an inherited cardiac channelopathy principally caused by defective functioning of both potassium–calcium ion channel that lead to abnormal shortening of...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7733558/ https://www.ncbi.nlm.nih.gov/pubmed/33335610 http://dx.doi.org/10.1002/joa3.12439 |
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author | Dewi, Ivana P. Dharmadjati, Budi B. |
author_facet | Dewi, Ivana P. Dharmadjati, Budi B. |
author_sort | Dewi, Ivana P. |
collection | PubMed |
description | There are many cardiac arrhythmias and sudden cardiac death (SCD) related to channelopathies or ion channel disorders. Short QT syndrome (SQTS) is an inherited cardiac channelopathy principally caused by defective functioning of both potassium–calcium ion channel that lead to abnormal shortening of QT interval, and an increased risk of ventricular and atrial arrhythmias. Tall T waves in all lead electrocardiogram (ECG), peaked T waves, and narrow‐based T waves that are reminiscent of the typical “desert tent” T waves of hyperkalemia are frequently associated with SQTS. Diagnosis is based on patient's family history, evaluation of symptoms (palpitations and cardiac arrest), and 12‐lead ECG. It can be time challenging because of the wide range of QT interval in healthy subjects. Implantable cardioverter defibrillator (ICD) is the first‐line therapy in SQTS. Quinidine has the potential to be an effective pharmacological therapy for SQTS patients, especially in young children who are not feasible in ICD implantation, because of the ability to prolong QT interval. |
format | Online Article Text |
id | pubmed-7733558 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | John Wiley and Sons Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-77335582020-12-16 Short QT syndrome: The current evidences of diagnosis and management Dewi, Ivana P. Dharmadjati, Budi B. J Arrhythm Clinical Reviews There are many cardiac arrhythmias and sudden cardiac death (SCD) related to channelopathies or ion channel disorders. Short QT syndrome (SQTS) is an inherited cardiac channelopathy principally caused by defective functioning of both potassium–calcium ion channel that lead to abnormal shortening of QT interval, and an increased risk of ventricular and atrial arrhythmias. Tall T waves in all lead electrocardiogram (ECG), peaked T waves, and narrow‐based T waves that are reminiscent of the typical “desert tent” T waves of hyperkalemia are frequently associated with SQTS. Diagnosis is based on patient's family history, evaluation of symptoms (palpitations and cardiac arrest), and 12‐lead ECG. It can be time challenging because of the wide range of QT interval in healthy subjects. Implantable cardioverter defibrillator (ICD) is the first‐line therapy in SQTS. Quinidine has the potential to be an effective pharmacological therapy for SQTS patients, especially in young children who are not feasible in ICD implantation, because of the ability to prolong QT interval. John Wiley and Sons Inc. 2020-10-06 /pmc/articles/PMC7733558/ /pubmed/33335610 http://dx.doi.org/10.1002/joa3.12439 Text en © 2020 The Authors. Journal of Arrhythmia published by John Wiley & Sons Australia, Ltd on behalf of Japanese Heart Rhythm Society This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Clinical Reviews Dewi, Ivana P. Dharmadjati, Budi B. Short QT syndrome: The current evidences of diagnosis and management |
title | Short QT syndrome: The current evidences of diagnosis and management |
title_full | Short QT syndrome: The current evidences of diagnosis and management |
title_fullStr | Short QT syndrome: The current evidences of diagnosis and management |
title_full_unstemmed | Short QT syndrome: The current evidences of diagnosis and management |
title_short | Short QT syndrome: The current evidences of diagnosis and management |
title_sort | short qt syndrome: the current evidences of diagnosis and management |
topic | Clinical Reviews |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7733558/ https://www.ncbi.nlm.nih.gov/pubmed/33335610 http://dx.doi.org/10.1002/joa3.12439 |
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