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 beta-blockers is complex. We assessed the long-term efficacy of left cardiac sympathetic denervation (LCSD) in a group of high-risk patients.


RESULTS
We identified 147 LQTS patients who underwent LCSD. Their QT interval was very prolonged (QTc, 543+/-65 ms); 99% were symptomatic; 48% had a cardiac arrest; and 75% of those treated with beta-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% (P<0.001). 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% (P<0.001). In 5 patients with preoperative implantable defibrillator and multiple discharges, the post-LCSD count of shocks decreased by 95% (P=0.02) 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 beta-blockade and in patients who experience arrhythmia storms with an implanted defibrillator.
Schwartz
Peter J
PJ
Department of Lung, Blood and Heart, University of Pavia, Pavia, Italy. PJQT@compuserve.com
Priori
Silvia G
SG
Cerrone
Marina
M
Spazzolini
Carla
C
Odero
Attilio
A
Napolitano
Carlo
C
Bloise
Raffaella
R
De Ferrari
Gaetano M
GM
Klersy
Catherine
C
Moss
Arthur J
AJ
Zareba
Wojciech
W
Robinson
Jennifer L
JL
Hall
W Jackson
WJ
Brink
Paul A
PA
Toivonen
Lauri
L
Epstein
Andrew E
AE
Li
Cuilan
C
Hu
Dayi
D
D000293:Adolescent; D000328:Adult; D002648:Child; D004562:Electrocardiography; D005500:Follow-Up Studies; D015171:Ganglionectomy; D005838:Genotype; D006321:Heart / Q000294:innervation; D006801:Humans; D008133:Long QT Syndrome / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D008297:Male; D012307:Risk Factors; D013562:Sympathectomy