Left Cardiac Sympathetic Denervation Monotherapy in Patients with Congenital Long QT Syndrome.

Background - Videoscopic left cardiac sympathetic denervation (LCSD) is an effective anti-fibrillatory, minimally invasive therapy for patients with potentially life-threatening arrhythmia syndromes like long QT syndrome (LQTS). Although initially used primarily for treatment intensification following documented LQTS-associated breakthrough cardiac events (BCEs) while on beta-blockers, LCSD as one-time monotherapy for certain patients with LQTS requires further evaluation. We are presenting our early experience with LCSD-monotherapy for carefully selected patients with LQTS. Methods - Among the 1400 patients evaluated and treated for LQTS, a retrospective review was performed on the 204 patients with LQTS who underwent LCSD at our institution since 2005 to identify the patients where the LCSD served as stand-alone, monotherapy. Clinical data on symptomatic status prior to diagnosis, clinical and genetic diagnosis, and BCEs after diagnosis were analyzed to determine efficacy of LCSD-monotherapy. Result - Overall, 64 of 204 patients (31%) were treated with LCSD alone [37 (58%) female, mean QTc 466 ± 30 ms, 16 (25%) patients were symptomatic prior to diagnosis with a mean age at diagnosis 17.3 ± 11.8 years, 5 had (8%) ≥ 1 BCE after diagnosis, and mean age at LCSD was 21.1 ± 11.4 years]. The primary motivation for LCSD-monotherapy was an unacceptable quality of life stemming from beta-blocker related side effects (i.e. beta-blocker intolerance) in 56/64 patients (88%). The underlying LQTS genotype was LQT1 in 36 (56%) and LQT2 in 20 (31%). There were no significant LCSD-related surgical complications. With a mean follow-up of 2.7 ± 2.4 years so far, only 3 patients have experienced a non-lethal, post-LCSD BCE in 180 patient-years. Conclusions - LCSD may be a safe and effective stand-alone therapy for select patients who do not tolerate beta-blockers. However, LCSD is not curative and patient selection will be critical when potentially considering LCSD as monotherapy.