Abstract
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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 776-781 |
| Number of pages | 6 |
| Journal | Heart Rhythm |
| Volume | 12 |
| Issue number | 4 |
| DOIs | |
| State | Published - Apr 1 2015 |
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Keywords
- Automated externaldefibrillator
- LongQTsyndrome
- Out-of-hospital cardiacarrest
- Suddencardiacdeath
- Ventricular fibrillation
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
- Physiology (medical)
Cite this
Automated external defibrillator rescues among children with diagnosed and treated long QT syndrome. / Pundi, Kavitha N.; Bos, J. Martijn; Cannon, Bryan C.; Ackerman, Michael John.
In: Heart Rhythm, Vol. 12, No. 4, 01.04.2015, p. 776-781.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Automated external defibrillator rescues among children with diagnosed and treated long QT syndrome
AU - Pundi, Kavitha N.
AU - Bos, J. Martijn
AU - Cannon, Bryan C.
AU - Ackerman, Michael John
PY - 2015/4/1
Y1 - 2015/4/1
N2 - 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.
AB - 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.
KW - Automated externaldefibrillator
KW - LongQTsyndrome
KW - Out-of-hospital cardiacarrest
KW - Suddencardiacdeath
KW - Ventricular fibrillation
UR - http://www.scopus.com/inward/record.url?scp=84925296569&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84925296569&partnerID=8YFLogxK
U2 - 10.1016/j.hrthm.2015.01.002
DO - 10.1016/j.hrthm.2015.01.002
M3 - Article
C2 - 25576780
AN - SCOPUS:84925296569
VL - 12
SP - 776
EP - 781
JO - Heart Rhythm
JF - Heart Rhythm
SN - 1547-5271
IS - 4
ER -