Wearable cardioverter defibrillators for patients with long QT syndrome

Heidi J. Owen, J. Martijn Bos, Michael John Ackerman

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Background: Long QT syndrome (LQTS) is a potentially lethal cardiac channelopathy, but with the appropriate treatment strategy, such as beta-blockers, left cardiac sympathetic denervation (LCSD), and/or an implantable cardioverter defibrillator (ICD), most LQTS-triggered tragedies can be avoided. Since 2001, wearable cardioverter defibrillators (WCD:LifeVest™) have been available clinically. Objective: Herein, we evaluated the use and outcome of WCDs in patients with LQTS. Methods: We performed a retrospective review of 1027 patients with LQTS to identify patients who received a WCD, and collected pertinent clinical information regarding their LQTS diagnosis as well as indication and experience regarding use of the WCD. Results: Overall, 10 LQTS patients (1%, 8 females, age at diagnosis 29 ± 18 years, mean QTc 488 ± 34 ms) were prescribed a WCD. Most common indication for WCD was as bridge to treatment during (temporary) situation of assessed high risk of sudden cardiac arrest (SCA; n = 6). The mean time of WCD use was 24 days (range 0 to 114 days). One patient (female, age 42, LQT2) received an appropriate VF-terminating shock 2 days after receiving her WCD. No inappropriate treatments or adverse events from wearing the WCD have occurred. Conclusions: A WCD can be considered in patients with LQTS deemed to be at high risk for SCA while up-titrating beta blockers, considering ICD therapy, or when navigating short term periods of increased SCA-risk, like the post-partum period in LQT2 women, ICD revision or temporary inactivation, or during short term administration of known QT prolonging medications.

Original languageEnglish (US)
JournalInternational Journal of Cardiology
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Long QT Syndrome
Defibrillators
Implantable Defibrillators
Romano-Ward Syndrome
Channelopathies
Sympathectomy
Sudden Cardiac Death
Therapeutics
Shock

Keywords

  • Long QT syndrome
  • LQTS
  • Sudden cardiac death
  • Syncope
  • Wearable cardioverter defibrillator

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Wearable cardioverter defibrillators for patients with long QT syndrome. / Owen, Heidi J.; Bos, J. Martijn; Ackerman, Michael John.

In: International Journal of Cardiology, 01.01.2018.

Research output: Contribution to journalArticle

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abstract = "Background: Long QT syndrome (LQTS) is a potentially lethal cardiac channelopathy, but with the appropriate treatment strategy, such as beta-blockers, left cardiac sympathetic denervation (LCSD), and/or an implantable cardioverter defibrillator (ICD), most LQTS-triggered tragedies can be avoided. Since 2001, wearable cardioverter defibrillators (WCD:LifeVest™) have been available clinically. Objective: Herein, we evaluated the use and outcome of WCDs in patients with LQTS. Methods: We performed a retrospective review of 1027 patients with LQTS to identify patients who received a WCD, and collected pertinent clinical information regarding their LQTS diagnosis as well as indication and experience regarding use of the WCD. Results: Overall, 10 LQTS patients (1{\%}, 8 females, age at diagnosis 29 ± 18 years, mean QTc 488 ± 34 ms) were prescribed a WCD. Most common indication for WCD was as bridge to treatment during (temporary) situation of assessed high risk of sudden cardiac arrest (SCA; n = 6). The mean time of WCD use was 24 days (range 0 to 114 days). One patient (female, age 42, LQT2) received an appropriate VF-terminating shock 2 days after receiving her WCD. No inappropriate treatments or adverse events from wearing the WCD have occurred. Conclusions: A WCD can be considered in patients with LQTS deemed to be at high risk for SCA while up-titrating beta blockers, considering ICD therapy, or when navigating short term periods of increased SCA-risk, like the post-partum period in LQT2 women, ICD revision or temporary inactivation, or during short term administration of known QT prolonging medications.",
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AB - Background: Long QT syndrome (LQTS) is a potentially lethal cardiac channelopathy, but with the appropriate treatment strategy, such as beta-blockers, left cardiac sympathetic denervation (LCSD), and/or an implantable cardioverter defibrillator (ICD), most LQTS-triggered tragedies can be avoided. Since 2001, wearable cardioverter defibrillators (WCD:LifeVest™) have been available clinically. Objective: Herein, we evaluated the use and outcome of WCDs in patients with LQTS. Methods: We performed a retrospective review of 1027 patients with LQTS to identify patients who received a WCD, and collected pertinent clinical information regarding their LQTS diagnosis as well as indication and experience regarding use of the WCD. Results: Overall, 10 LQTS patients (1%, 8 females, age at diagnosis 29 ± 18 years, mean QTc 488 ± 34 ms) were prescribed a WCD. Most common indication for WCD was as bridge to treatment during (temporary) situation of assessed high risk of sudden cardiac arrest (SCA; n = 6). The mean time of WCD use was 24 days (range 0 to 114 days). One patient (female, age 42, LQT2) received an appropriate VF-terminating shock 2 days after receiving her WCD. No inappropriate treatments or adverse events from wearing the WCD have occurred. Conclusions: A WCD can be considered in patients with LQTS deemed to be at high risk for SCA while up-titrating beta blockers, considering ICD therapy, or when navigating short term periods of increased SCA-risk, like the post-partum period in LQT2 women, ICD revision or temporary inactivation, or during short term administration of known QT prolonging medications.

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