Wearable cardioverter defibrillators for patients with long QT syndrome

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.