Implantable cardioverter defibrillator therapy for congenital long QT syndrome: A single-center experience

Justin M. Horner, Masayoshi Kinoshita, Tracy L. Webster, Carla M. Haglund, Paul Andrew Friedman, Michael John Ackerman

Research output: Contribution to journalArticle

65 Citations (Scopus)

Abstract

Background Long QT syndrome's (LQTS) marked heterogeneity necessitates both evidence-based and individualized therapeutic approaches. Objective This study sought to analyze a single LQTS specialty center's experience regarding the relationship between risk factors and appropriate ventricular fibrillation (VF)terminating therapies among LQTS patients treated with an implantable cardioverter-defibrillator (ICD). Methods An internal review boardapproved, retrospective analysis of the electronic medical records of 459 patients with genetically confirmed LQTS including the 51 patients (14 LQT1, 22 LQT2, and 15 LQT3) who received an ICD from 2000 to 2010 was performed. Results Twelve patients (24%, 4 LQT1, 8 LQT2) experienced an appropriate, VF-terminating therapy with an average follow-up of 7.3 years, including 7 of 17 LQT2 female patients but none of the 15 LQT3 patients. Conversely, 15 (29%) patients (8 LQT3) have experienced an inappropriate shock. Secondary prevention indications (P = .008), non-LQT3 genotype (P = .02), QTc < 500 ms (P = .0008), documented syncope (P = .05), documented torsades de pointes (P = .003), and a negative family history (P = .0001) were most predictive of an appropriate therapy. Importantly, no LQT-related deaths have occurred among the 408 non-ICDtreated patients. Conclusion The vast majority of LQTS patients can be treated effectively without an ICD. Potentially life-saving therapies were rendered at a 5% to 6% per year rate among those selected for ICD therapy; similar inappropriate shock frequencies were also noted. Secondary prevention, genotype, and QTc predicted those most likely to receive appropriate therapy. Although the ICD implant frequency is greatest among LQT3 patients, the greatest "save" rate has occurred among LQT2 women, who were assessed to be at high risk.

Original languageEnglish (US)
Pages (from-to)1616-1622
Number of pages7
JournalHeart Rhythm
Volume7
Issue number11
DOIs
StatePublished - Nov 2010

Fingerprint

Long QT Syndrome
Implantable Defibrillators
Therapeutics
Ventricular Fibrillation
Secondary Prevention
Shock
Genotype
Torsades de Pointes
Electronic Health Records
Syncope

Keywords

  • Genetic testing
  • Implantable cardioverter defibrillator
  • Ion channels
  • Long QT syndrome
  • Sudden cardiac death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Implantable cardioverter defibrillator therapy for congenital long QT syndrome : A single-center experience. / Horner, Justin M.; Kinoshita, Masayoshi; Webster, Tracy L.; Haglund, Carla M.; Friedman, Paul Andrew; Ackerman, Michael John.

In: Heart Rhythm, Vol. 7, No. 11, 11.2010, p. 1616-1622.

Research output: Contribution to journalArticle

Horner, Justin M. ; Kinoshita, Masayoshi ; Webster, Tracy L. ; Haglund, Carla M. ; Friedman, Paul Andrew ; Ackerman, Michael John. / Implantable cardioverter defibrillator therapy for congenital long QT syndrome : A single-center experience. In: Heart Rhythm. 2010 ; Vol. 7, No. 11. pp. 1616-1622.
@article{a444d6f515f94fa7819b492b9c090e0a,
title = "Implantable cardioverter defibrillator therapy for congenital long QT syndrome: A single-center experience",
abstract = "Background Long QT syndrome's (LQTS) marked heterogeneity necessitates both evidence-based and individualized therapeutic approaches. Objective This study sought to analyze a single LQTS specialty center's experience regarding the relationship between risk factors and appropriate ventricular fibrillation (VF)terminating therapies among LQTS patients treated with an implantable cardioverter-defibrillator (ICD). Methods An internal review boardapproved, retrospective analysis of the electronic medical records of 459 patients with genetically confirmed LQTS including the 51 patients (14 LQT1, 22 LQT2, and 15 LQT3) who received an ICD from 2000 to 2010 was performed. Results Twelve patients (24{\%}, 4 LQT1, 8 LQT2) experienced an appropriate, VF-terminating therapy with an average follow-up of 7.3 years, including 7 of 17 LQT2 female patients but none of the 15 LQT3 patients. Conversely, 15 (29{\%}) patients (8 LQT3) have experienced an inappropriate shock. Secondary prevention indications (P = .008), non-LQT3 genotype (P = .02), QTc < 500 ms (P = .0008), documented syncope (P = .05), documented torsades de pointes (P = .003), and a negative family history (P = .0001) were most predictive of an appropriate therapy. Importantly, no LQT-related deaths have occurred among the 408 non-ICDtreated patients. Conclusion The vast majority of LQTS patients can be treated effectively without an ICD. Potentially life-saving therapies were rendered at a 5{\%} to 6{\%} per year rate among those selected for ICD therapy; similar inappropriate shock frequencies were also noted. Secondary prevention, genotype, and QTc predicted those most likely to receive appropriate therapy. Although the ICD implant frequency is greatest among LQT3 patients, the greatest {"}save{"} rate has occurred among LQT2 women, who were assessed to be at high risk.",
keywords = "Genetic testing, Implantable cardioverter defibrillator, Ion channels, Long QT syndrome, Sudden cardiac death",
author = "Horner, {Justin M.} and Masayoshi Kinoshita and Webster, {Tracy L.} and Haglund, {Carla M.} and Friedman, {Paul Andrew} and Ackerman, {Michael John}",
year = "2010",
month = "11",
doi = "10.1016/j.hrthm.2010.08.023",
language = "English (US)",
volume = "7",
pages = "1616--1622",
journal = "Heart Rhythm",
issn = "1547-5271",
publisher = "Elsevier",
number = "11",

}

TY - JOUR

T1 - Implantable cardioverter defibrillator therapy for congenital long QT syndrome

T2 - A single-center experience

AU - Horner, Justin M.

AU - Kinoshita, Masayoshi

AU - Webster, Tracy L.

AU - Haglund, Carla M.

AU - Friedman, Paul Andrew

AU - Ackerman, Michael John

PY - 2010/11

Y1 - 2010/11

N2 - Background Long QT syndrome's (LQTS) marked heterogeneity necessitates both evidence-based and individualized therapeutic approaches. Objective This study sought to analyze a single LQTS specialty center's experience regarding the relationship between risk factors and appropriate ventricular fibrillation (VF)terminating therapies among LQTS patients treated with an implantable cardioverter-defibrillator (ICD). Methods An internal review boardapproved, retrospective analysis of the electronic medical records of 459 patients with genetically confirmed LQTS including the 51 patients (14 LQT1, 22 LQT2, and 15 LQT3) who received an ICD from 2000 to 2010 was performed. Results Twelve patients (24%, 4 LQT1, 8 LQT2) experienced an appropriate, VF-terminating therapy with an average follow-up of 7.3 years, including 7 of 17 LQT2 female patients but none of the 15 LQT3 patients. Conversely, 15 (29%) patients (8 LQT3) have experienced an inappropriate shock. Secondary prevention indications (P = .008), non-LQT3 genotype (P = .02), QTc < 500 ms (P = .0008), documented syncope (P = .05), documented torsades de pointes (P = .003), and a negative family history (P = .0001) were most predictive of an appropriate therapy. Importantly, no LQT-related deaths have occurred among the 408 non-ICDtreated patients. Conclusion The vast majority of LQTS patients can be treated effectively without an ICD. Potentially life-saving therapies were rendered at a 5% to 6% per year rate among those selected for ICD therapy; similar inappropriate shock frequencies were also noted. Secondary prevention, genotype, and QTc predicted those most likely to receive appropriate therapy. Although the ICD implant frequency is greatest among LQT3 patients, the greatest "save" rate has occurred among LQT2 women, who were assessed to be at high risk.

AB - Background Long QT syndrome's (LQTS) marked heterogeneity necessitates both evidence-based and individualized therapeutic approaches. Objective This study sought to analyze a single LQTS specialty center's experience regarding the relationship between risk factors and appropriate ventricular fibrillation (VF)terminating therapies among LQTS patients treated with an implantable cardioverter-defibrillator (ICD). Methods An internal review boardapproved, retrospective analysis of the electronic medical records of 459 patients with genetically confirmed LQTS including the 51 patients (14 LQT1, 22 LQT2, and 15 LQT3) who received an ICD from 2000 to 2010 was performed. Results Twelve patients (24%, 4 LQT1, 8 LQT2) experienced an appropriate, VF-terminating therapy with an average follow-up of 7.3 years, including 7 of 17 LQT2 female patients but none of the 15 LQT3 patients. Conversely, 15 (29%) patients (8 LQT3) have experienced an inappropriate shock. Secondary prevention indications (P = .008), non-LQT3 genotype (P = .02), QTc < 500 ms (P = .0008), documented syncope (P = .05), documented torsades de pointes (P = .003), and a negative family history (P = .0001) were most predictive of an appropriate therapy. Importantly, no LQT-related deaths have occurred among the 408 non-ICDtreated patients. Conclusion The vast majority of LQTS patients can be treated effectively without an ICD. Potentially life-saving therapies were rendered at a 5% to 6% per year rate among those selected for ICD therapy; similar inappropriate shock frequencies were also noted. Secondary prevention, genotype, and QTc predicted those most likely to receive appropriate therapy. Although the ICD implant frequency is greatest among LQT3 patients, the greatest "save" rate has occurred among LQT2 women, who were assessed to be at high risk.

KW - Genetic testing

KW - Implantable cardioverter defibrillator

KW - Ion channels

KW - Long QT syndrome

KW - Sudden cardiac death

UR - http://www.scopus.com/inward/record.url?scp=78149268639&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=78149268639&partnerID=8YFLogxK

U2 - 10.1016/j.hrthm.2010.08.023

DO - 10.1016/j.hrthm.2010.08.023

M3 - Article

C2 - 20816872

AN - SCOPUS:78149268639

VL - 7

SP - 1616

EP - 1622

JO - Heart Rhythm

JF - Heart Rhythm

SN - 1547-5271

IS - 11

ER -