Ventricular noncompaction and long QT syndrome – A deadly double hit for the foetus

Congenital long QT syndrome [LQTS] is a channelopathy characterized by QT prolongation and polymorphic VT. LQTS however need not be a purely electrical disease. Defects in ion channels may cause myocardial architectural disruption leading to ventricular non compaction [VNC]. It is defined as the pre...

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Autores principales: Cherian, Anne George, Lankala, Pramitha, Krupa, Jesu, Roshan, John
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2021
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8116813/
https://www.ncbi.nlm.nih.gov/pubmed/33774159
http://dx.doi.org/10.1016/j.ipej.2021.03.001
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author Cherian, Anne George
Lankala, Pramitha
Krupa, Jesu
Roshan, John
author_facet Cherian, Anne George
Lankala, Pramitha
Krupa, Jesu
Roshan, John
author_sort Cherian, Anne George
collection PubMed
description Congenital long QT syndrome [LQTS] is a channelopathy characterized by QT prolongation and polymorphic VT. LQTS however need not be a purely electrical disease. Defects in ion channels may cause myocardial architectural disruption leading to ventricular non compaction [VNC]. It is defined as the presence of prominent ventricular trabeculations and deep intertrabecular recesses within the endomyocardium. We describe the in-utero management of a foetus who was later found to have LQTS with VNC. The detection of ventricular tachycardia and complete heart block in utero should arouse the suspicion of LQTS. It would be wise to avoid QT prolonging antiarrhythmics in this subset of patients.
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spelling pubmed-81168132021-05-18 Ventricular noncompaction and long QT syndrome – A deadly double hit for the foetus Cherian, Anne George Lankala, Pramitha Krupa, Jesu Roshan, John Indian Pacing Electrophysiol J Case Report Congenital long QT syndrome [LQTS] is a channelopathy characterized by QT prolongation and polymorphic VT. LQTS however need not be a purely electrical disease. Defects in ion channels may cause myocardial architectural disruption leading to ventricular non compaction [VNC]. It is defined as the presence of prominent ventricular trabeculations and deep intertrabecular recesses within the endomyocardium. We describe the in-utero management of a foetus who was later found to have LQTS with VNC. The detection of ventricular tachycardia and complete heart block in utero should arouse the suspicion of LQTS. It would be wise to avoid QT prolonging antiarrhythmics in this subset of patients. Elsevier 2021-03-24 /pmc/articles/PMC8116813/ /pubmed/33774159 http://dx.doi.org/10.1016/j.ipej.2021.03.001 Text en © 2021 Indian Heart Rhythm Society. Production and hosting by Elsevier B.V. https://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 Case Report
Cherian, Anne George
Lankala, Pramitha
Krupa, Jesu
Roshan, John
Ventricular noncompaction and long QT syndrome – A deadly double hit for the foetus
title Ventricular noncompaction and long QT syndrome – A deadly double hit for the foetus
title_full Ventricular noncompaction and long QT syndrome – A deadly double hit for the foetus
title_fullStr Ventricular noncompaction and long QT syndrome – A deadly double hit for the foetus
title_full_unstemmed Ventricular noncompaction and long QT syndrome – A deadly double hit for the foetus
title_short Ventricular noncompaction and long QT syndrome – A deadly double hit for the foetus
title_sort ventricular noncompaction and long qt syndrome – a deadly double hit for the foetus
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8116813/
https://www.ncbi.nlm.nih.gov/pubmed/33774159
http://dx.doi.org/10.1016/j.ipej.2021.03.001
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AT krupajesu ventricularnoncompactionandlongqtsyndromeadeadlydoublehitforthefoetus
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