TY - JOUR
T1 - Bilateral cardiac sympathetic denervation in patients with congenital long QT syndrome
AU - Tobert, Kathryn E.
AU - Bos, J. Martijn
AU - Moir, Christopher
AU - Polites, Stephanie F.
AU - Ackerman, Michael J.
N1 - Publisher Copyright:
© 2023 Heart Rhythm Society
PY - 2023/7
Y1 - 2023/7
N2 - Background: Long QT syndrome (LQTS) is a potentially lethal yet treatable genetic heart disease for which left cardiac sympathetic denervation (LCSD) is a class I recommendation. Recent reports have suggested bilateral cardiac sympathetic denervation (BiCSD) as the initial surgical denervation therapy in LQTS. Objective: The purpose of this study was to determine the frequency and settings in which BiCSD was used in a tertiary referral center with expertise in LCSD. Methods: We performed a retrospective review of 234 out of 1638 patients with LQTS who underwent sympathetic denervation (14%) at our institution to identify the subset of patients who underwent BiCSD. Cardiac events (CEs) before LCSD, after LCSD, and after the completion of BiCSD were recorded and defined as being an appropriate implantable cardioverter-defibrillator shock, arrhythmic syncope, or sudden cardiac arrest. Results: Only 11 patients (4.7%; 6 females [55%]) had BiCSD at our institution. Patients who received BiCSD trended toward being younger at diagnosis (6 ± 15 years vs 14 ± 13 years; P = .06) and being more likely to be symptomatic (73% vs 53%; P = .07) than the larger LCSD-only cohort. Continued CEs post-LCSD (3.8 CEs per patient on average) was the predominant determinant to return for BiCSD. Over 60 combined years of follow-up, 4 patients have not had a CE post-BiCSD while the other 7 patients average 3.6 nonlethal CEs. Conclusion: Less than 5% of all patients receiving denervation therapy underwent BiCSD. When BiCSD was chosen, it was almost always done in a staged sequential manner beginning with LCSD first and when driven by the arrhythmogenicity of the LQTS substrate, despite otherwise optimized guideline-directed therapies.
AB - Background: Long QT syndrome (LQTS) is a potentially lethal yet treatable genetic heart disease for which left cardiac sympathetic denervation (LCSD) is a class I recommendation. Recent reports have suggested bilateral cardiac sympathetic denervation (BiCSD) as the initial surgical denervation therapy in LQTS. Objective: The purpose of this study was to determine the frequency and settings in which BiCSD was used in a tertiary referral center with expertise in LCSD. Methods: We performed a retrospective review of 234 out of 1638 patients with LQTS who underwent sympathetic denervation (14%) at our institution to identify the subset of patients who underwent BiCSD. Cardiac events (CEs) before LCSD, after LCSD, and after the completion of BiCSD were recorded and defined as being an appropriate implantable cardioverter-defibrillator shock, arrhythmic syncope, or sudden cardiac arrest. Results: Only 11 patients (4.7%; 6 females [55%]) had BiCSD at our institution. Patients who received BiCSD trended toward being younger at diagnosis (6 ± 15 years vs 14 ± 13 years; P = .06) and being more likely to be symptomatic (73% vs 53%; P = .07) than the larger LCSD-only cohort. Continued CEs post-LCSD (3.8 CEs per patient on average) was the predominant determinant to return for BiCSD. Over 60 combined years of follow-up, 4 patients have not had a CE post-BiCSD while the other 7 patients average 3.6 nonlethal CEs. Conclusion: Less than 5% of all patients receiving denervation therapy underwent BiCSD. When BiCSD was chosen, it was almost always done in a staged sequential manner beginning with LCSD first and when driven by the arrhythmogenicity of the LQTS substrate, despite otherwise optimized guideline-directed therapies.
KW - Arrhythmias
KW - Bilateral cardiac sympathetic denervation
KW - Left cardiac sympathetic denervation
KW - Long QT syndrome
KW - Outcomes
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U2 - 10.1016/j.hrthm.2023.03.016
DO - 10.1016/j.hrthm.2023.03.016
M3 - Article
C2 - 36934983
AN - SCOPUS:85152587754
SN - 1547-5271
VL - 20
SP - 1033
EP - 1038
JO - Heart rhythm
JF - Heart rhythm
IS - 7
ER -