Abstract 3094: Implantable Cardioverter Defibrillator Therapy for Congenital Long QT Syndrome: a Single Center Experience

Background: Long QT syndrome (LQTS) is a potentially lethal yet highly treatable channelopathy underscored by marked heterogeneity that necessitates evidence-based and individualized therapeutic approaches. One such therapy is the implantable cardioverter defibrillator (ICD). Here, we sought to determine the percentage of patients (pts) with LQTS that received an ICD in our LQTS clinic and its short term outcomes. Methods: An IRB-approved, retrospective analysis of the electronic medical records of 167 LQT1, 113 LQT2, and 29 LQT3 pts evaluated in our LQTS Clinic with subset analysis of those implanted with an ICD in our institution from January 2000 to June 2007. Results: Forty-eight pts (15% of total, 33 females, 14 LQT1, 20 LQT2, and 14 LQT3) received an ICD. The average age at implant was 30 ± 12 years (range 8 – 52 years). Nearly two-thirds received an ICD as primary prevention. The QTc exceeded 500 ms in 18 (37.5%). With an average follow-up of 42 ± 32 months, 9 (19%) have had an appropriate, VF-terminating therapy including 3 with LQT1 and 6 with LQT2. Notably, 5 of the 6 LQT2 females have already received an appropriate ICD therapy whereas none of the LQT3 pts have received one. Secondary prevention indications (p = 0.006), LQT2 genotype (p = 0.03), QTc > 500 ms (p = 0.001), and surprisingly a negative family history (p = 0.0005) predicted those ICD pts most likely to receive an appropriate therapy. Importantly, none of the non-ICD treated pts (> 250) have died a LQT-related death during the same follow-up. Conversely, 9 pts have experienced an inappropriate shock including 6 with LQT3 and males were more likely to receive inappropriate therapies (p = 0.002). Conclusions: The majority of pts with LQTS can be treated effectively without an ICD. In this study, potentially life-saving therapies have been rendered so far at a 5– 6%/year rate. Inappropriate shocks occurred with similar frequency. Secondary prevention, genotype, and QTc predicted those ICD recipients most likely to receive appropriate therapy. Although LQT3 status is commonly associated with an ICD recommendation, so far none of the LQT3 positive ICD recipients have needed their device. Instead, the greatest “save” rate has occurred among the LQT2 positive females deemed at high risk.