Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome.

Background—The management of long-QT syndrome (LQTS) patients who continue to have cardiac events (CEs) despite
-blockers is complex. We assessed the long-term efficacy of left cardiac sympathetic denervation (LCSD) in a group
of high-risk patients.
Methods and Results—We identified 147 LQTS patients who underwent LCSD. Their QT interval was very prolonged
(QTc, 54365 ms); 99% were symptomatic; 48% had a cardiac arrest; and 75% of those treated with -blockers
remained symptomatic. The average follow-up periods between first CE and LCSD and post-LCSD were 4.6 and 7.8
years, respectively. After LCSD, 46% remained asymptomatic. Syncope occurred in 31%, aborted cardiac arrest in 16%,
and sudden death in 7%. The mean yearly number of CEs per patient dropped by 91% (P0.001). Among 74 patients
with only syncope before LCSD, all types of CEs decreased significantly as in the entire group, and a post-LCSD QTc
500 ms predicted very low risk. The percentage of patients with 5 CEs declined from 55% to 8% (P0.001). In 5
patients with preoperative implantable defibrillator and multiple discharges, the post-LCSD count of shocks decreased
by 95% (P0.02) from a median number of 25 to 0 per patient. Among 51 genotyped patients, LCSD appeared more
effective in LQT1 and LQT3 patients.
Conclusions—LCSD is associated with a significant reduction in the incidence of aborted cardiac arrest and syncope in
high-risk LQTS patients when compared with pre-LCSD events. However, LCSD is not entirely effective in preventing
cardiac events including sudden cardiac death during long-term follow-up. LCSD should be considered in patients with
recurrent syncope despite -blockade and in patients who experience arrhythmia storms with an implanted defibrillator.