title: Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome.
abstract: 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.
authors.affiliation: Department of Lung, Blood and Heart, University of Pavia, Pavia, Italy. PJQT@compuserve.com
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Schwartz
authors.forename: Silvia G
authors.initials: SG
authors.lastname: Priori
authors.forename: Marina
authors.initials: M
authors.lastname: Cerrone
authors.forename: Carla
authors.initials: C
authors.lastname: Spazzolini
authors.forename: Attilio
authors.initials: A
authors.lastname: Odero
authors.forename: Carlo
authors.initials: C
authors.lastname: Napolitano
authors.forename: Raffaella
authors.initials: R
authors.lastname: Bloise
authors.forename: Gaetano M
authors.initials: GM
authors.lastname: De Ferrari
authors.forename: Catherine
authors.initials: C
authors.lastname: Klersy
authors.forename: Arthur J
authors.initials: AJ
authors.lastname: Moss
authors.forename: Wojciech
authors.initials: W
authors.lastname: Zareba
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Robinson
authors.forename: W Jackson
authors.initials: WJ
authors.lastname: Hall
authors.forename: Paul A
authors.initials: PA
authors.lastname: Brink
authors.forename: Lauri
authors.initials: L
authors.lastname: Toivonen
authors.forename: Andrew E
authors.initials: AE
authors.lastname: Epstein
authors.forename: Cuilan
authors.initials: C
authors.lastname: Li
authors.forename: Dayi
authors.initials: D
authors.lastname: Hu
mesh_terms: 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