• Heart Rhythm · Apr 2015

    Automated external defibrillator rescues among children with diagnosed and treated long QT syndrome.

    • Kavitha N Pundi, J Martijn Bos, Bryan C Cannon, and Michael J Ackerman.
    • Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
    • Heart Rhythm. 2015 Apr 1; 12 (4): 776-81.

    BackgroundLong QT syndrome (LQTS) is a potentially lethal yet highly treatable cardiac channelopathy. A comprehensive LQTS-directed treatment program often includes an automated external defibrillator (AED).ObjectiveThe purpose of this study was to determine the incidence of AED rescues among children evaluated, risk-stratified, and treated in an LQTS specialty center.MethodsWe performed a retrospective review of the electronic medical records to identify 1665 patients evaluated in our Genetic Heart Rhythm Clinic (1999-2013). Subset analysis was performed on 291 children managed without an implantable cardioverter-defibrillator (ICD).ResultsThe average age at diagnosis was 8.3 ± 5.7 years with an average. QTc of 463 ± 40 ms (17% ≥500 ms). The represented LQTS genotypes included type 1 (LQT1) in 52%, type 2 (LQT2) in 35%, and type 3 (LQT3) in 7%. During follow-up, 3 of 291 children (1%) had a cardiac arrest with an appropriate AED rescue (2/51 symptomatic, 1/240 asymptomatic). The first AED rescue occurred during exercise in a symptomatic 3-year-old boy with compound LQT1 treated with beta-blocker and videoscopic left cardiac sympathetic denervation (LCSD). The second AED rescue occurred in a remotely symptomatic 14-year-old boy with high-risk LQT2 (QTc >550 ms) on a beta-blocker who previously declined a prophylactic ICD. The third AED rescue involved an asymptomatic 17-year-old girl with LQT3 on mexiletine who collapsed in school.ConclusionAn AED should seldom be necessary in an appropriately treated child with LQTS. Nevertheless, despite only 3 AED rescues in more than 1700 patient-years, an AED can be a lifesaving and cost-effective part of an LQTS patient's comprehensive sudden death prevention program.Copyright © 2015 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

      Pubmed     Full text   Copy Citation     Plaintext  

      Add institutional full text...

    Notes

     
    Knowledge, pearl, summary or comment to share?
    300 characters remaining
    help        
    You can also include formatting, links, images and footnotes in your notes
    • Simple formatting can be added to notes, such as *italics*, _underline_ or **bold**.
    • Superscript can be denoted by <sup>text</sup> and subscript <sub>text</sub>.
    • Numbered or bulleted lists can be created using either numbered lines 1. 2. 3., hyphens - or asterisks *.
    • Links can be included with: [my link to pubmed](http://pubmed.com)
    • Images can be included with: ![alt text](https://bestmedicaljournal.com/study_graph.jpg "Image Title Text")
    • For footnotes use [^1](This is a footnote.) inline.
    • Or use an inline reference [^1] to refer to a longer footnote elseweher in the document [^1]: This is a long footnote..

    hide…