Literature DB >> 32882399

Long QT syndrome type 1 and 2 patients respond differently to arrhythmic triggers: The TriQarr in vivo study.

Peter Marstrand1, Kasim Almatlouh2, Jørgen K Kanters3, Claus Graff4, Alex Hørby Christensen2, Henning Bundgaard5, Juliane Theilade2.   

Abstract

BACKGROUND: In patients with long QT syndrome (LQTS), swimming and loud noises have been identified as genotype-specific arrhythmic triggers in LQTS type 1 (LQTS1) and LQTS type 2 (LQTS2), respectively.
OBJECTIVE: The purpose of this study was to compare LQTS group responses to arrhythmic triggers.
METHODS: LQTS1 and LQTS2 patients were included. Before and after beta-blocker intake, electrocardiograms were recorded as participants (1) were exposed to a loud noise of ∼100 dB; and (2) had their face immersed into cold water.
RESULTS: Twenty-three patients (9 LQTS1, 14 LQTS2) participated. In response to noise, LQTS groups showed similarly increased heart rate, but LQTS2 patients had corrected QT interval (Fridericia formula) (QTcF) prolonged significantly more than LQTS1 patients (37 ± 8 ms vs 15 ± 6 ms; P = .02). After intake of beta-blocker, QTcF prolongation in LQTS2 patients was significantly blunted and similar to that of LQTS1 patients (P = .90). In response to simulated diving, LQTS groups experienced a heart rate drop of ∼28 bpm, which shortened QTcF similarly in both groups. After intake of beta-blockers, heart rate dropped to 28 ± 2 bpm in LQTS1 patients and 20 ± 3 bpm in LQTS2, resulting in a slower heart rate in LQTS1 compared with LQTS2 (P = .01). In response, QTcF shortened similarly in LQTS1 and LQTS2 patients (57 ± 9 ms vs 36 ± 7 ms; P = .10).
CONCLUSION: When exposed to noise, LQTS2 patients had QTc prolonged significantly more than did LQTS1 patients. Importantly, beta-blockers reduced noise-induced QTc prolongation in LQTS2 patients, thus demonstrating the protective effect of beta-blockers. In response to simulated diving, LQTS groups responded similarly, but a slower heart rate was observed in LQTS1 patients during simulated diving after beta-blocker intake.
Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

Entities:  

Keywords:  Auditory stimuli; Diving reflex; Loud noise; Ventricular arrhythmia

Mesh:

Substances:

Year:  2020        PMID: 32882399     DOI: 10.1016/j.hrthm.2020.08.017

Source DB:  PubMed          Journal:  Heart Rhythm        ISSN: 1547-5271            Impact factor:   6.343


  1 in total

1.  Effect of moderate potassium-elevating treatment in long QT syndrome: the TriQarr Potassium Study.

Authors:  Peter Marstrand; Kasim Almatlouh; Jørgen K Kanters; Claus Graff; Alex Hørby Christensen; Henning Bundgaard; Juliane Theilade
Journal:  Open Heart       Date:  2021-09
  1 in total

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