TY - JOUR
T1 - Left cardiac sympathetic denervation in long QT syndrome
T2 - Analysis of therapeutic nonresponders
AU - Bos, J. Martijn
AU - Bos, Katy M.
AU - Johnson, Jonathan N.
AU - Moir, Christopher
AU - Ackerman, Michael J.
N1 - Copyright:
Copyright 2014 Elsevier B.V., All rights reserved.
PY - 2013/8
Y1 - 2013/8
N2 - Background-Long QT syndrome (LQTS) is a potentially lethal but highly treatable cardiac channelopathy. Treatment options include pharmacotherapy, device therapy, and left cardiac sympathetic denervation (LCSD). Here, we sought to determine the characteristics of LQTS patients who have had ≥1 LQTS-related breakthrough cardiac event (BCE) after LCSD. Methods and Results-We performed retrospective chart review for 52 consecutive patients (24 males; mean age at diagnosis, 10.0±10 years; mean QTc, 528±74 ms) with LQTS who underwent LCSD between 2005 and 2010 (mean age at LCSD, 14.1±10 years) and have been followed up for 3.6±1.3 years. A BCE was defined as either (1) an appropriate ventricular fibrillation-terminating implantable cardioverter defibrillator shock or (2) arrhythmogenic syncope, seizures, or aborted cardiac arrest after LCSD. Thirty-three patients (61%) had LCSD as primary prevention because of either high-risk assessment or β-blocker intolerance. So far, 12 of 52 (23%) patients (7 males) have experienced ≥1 BCE post LCSD. The clinical phenotype of patients with BCEs was significantly more severe than patients without a BCE. No BCEs were seen in patients undergoing LCSD for β-blocker intolerance (0/12 versus 17/40; P<0.001). Conclusions-Although a marked reduction in number of cardiac events is usually seen after LCSD, ≈50% of high-risk LQTS patients have experienced ≥1 post-LCSD breakthrough. Therefore, LCSD must not be viewed as curative or as an alternative in implantable cardioverter defibrillator for high-risk patients. Prophylactic LCSD may provide another option to counter a suboptimal quality of life resulting from medication-related side effects.
AB - Background-Long QT syndrome (LQTS) is a potentially lethal but highly treatable cardiac channelopathy. Treatment options include pharmacotherapy, device therapy, and left cardiac sympathetic denervation (LCSD). Here, we sought to determine the characteristics of LQTS patients who have had ≥1 LQTS-related breakthrough cardiac event (BCE) after LCSD. Methods and Results-We performed retrospective chart review for 52 consecutive patients (24 males; mean age at diagnosis, 10.0±10 years; mean QTc, 528±74 ms) with LQTS who underwent LCSD between 2005 and 2010 (mean age at LCSD, 14.1±10 years) and have been followed up for 3.6±1.3 years. A BCE was defined as either (1) an appropriate ventricular fibrillation-terminating implantable cardioverter defibrillator shock or (2) arrhythmogenic syncope, seizures, or aborted cardiac arrest after LCSD. Thirty-three patients (61%) had LCSD as primary prevention because of either high-risk assessment or β-blocker intolerance. So far, 12 of 52 (23%) patients (7 males) have experienced ≥1 BCE post LCSD. The clinical phenotype of patients with BCEs was significantly more severe than patients without a BCE. No BCEs were seen in patients undergoing LCSD for β-blocker intolerance (0/12 versus 17/40; P<0.001). Conclusions-Although a marked reduction in number of cardiac events is usually seen after LCSD, ≈50% of high-risk LQTS patients have experienced ≥1 post-LCSD breakthrough. Therefore, LCSD must not be viewed as curative or as an alternative in implantable cardioverter defibrillator for high-risk patients. Prophylactic LCSD may provide another option to counter a suboptimal quality of life resulting from medication-related side effects.
KW - Arrhythmias
KW - Cardiac
KW - Death sudden, cardiac
KW - Left cardiac sympathetic denervation
KW - Long QT syndrome
KW - Syncope
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U2 - 10.1161/CIRCEP.113.000102
DO - 10.1161/CIRCEP.113.000102
M3 - Article
C2 - 23728945
AN - SCOPUS:84884508378
SN - 1941-3149
VL - 6
SP - 705
EP - 711
JO - Circulation: Arrhythmia and Electrophysiology
JF - Circulation: Arrhythmia and Electrophysiology
IS - 4
ER -