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Automated external defibrillator rescues among children with diagnosed and treated long QT syndrome
Kavitha N Pundi1, J Martijn Bos2, Bryan C Cannon1
1Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
Insights
Automated external defibrillator (AED) rescues are rare in children with Long QT syndrome (LQTS) when treated at a specialty center. Despite their infrequent need, AEDs remain a vital, cost-effective tool for sudden cardiac death prevention in LQTS patients.
Area of Science:
- Cardiology
- Genetics
- Pediatrics
Background:
- Long QT syndrome (LQTS) is a life-threatening but treatable cardiac condition.
- Comprehensive LQTS management often incorporates automated external defibrillators (AEDs).
Purpose of the Study:
- To determine the frequency of AED rescues in children evaluated and treated at an LQTS specialty center.
Main Methods:
- Retrospective review of 1665 patients evaluated between 1999 and 2013.
- Subset analysis of 291 children managed without an implantable cardioverter-defibrillator (ICD).
Main Results:
- The incidence of cardiac arrest with appropriate AED rescue was 1% (3 of 291 children).
- Rescues occurred in both symptomatic and asymptomatic children with various LQTS genotypes.
- The average age at diagnosis was 8.3 years, with 17% having QTc ≥500 ms.
Conclusions:
- AEDs are seldom required in appropriately treated pediatric LQTS patients.
- AEDs are a potentially lifesaving and cost-effective component of sudden cardiac death prevention strategies for LQTS.
- The study highlights the importance of specialized care centers for managing LQTS.
Background:
Long 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).
Objective:
The purpose of this study was to determine the incidence of AED rescues among children evaluated, risk-stratified, and treated in an LQTS specialty center.
Methods:
We 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).
Results:
The 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.
Conclusion:
An 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.
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