Left cardiac sympathetic denervation in the therapy of congenital long QT syndrome. A worldwide report.
BACKGROUND
Long QT syndrome (LQTS) is a congenital disorder accompanied by a high incidence of sudden cardiac death. beta-Adrenergic blockade is the therapy of choice, and it is successful in 75-80% of patients. For those in whom cardiac events (syncope or cardiac arrest) are not prevented by beta-blockade, experimental studies suggest that left cardiac sympathetic denervation (LCSD) may be useful.


RESULTS
We identified 85 LQTS patients worldwide who underwent LCSD, and we provide here the first large-scale evaluation of its efficacy. The time interval between the first cardiac event and LCSD and the follow-up period after LCSD were similar (5.6 +/- 6.1 versus 5.9 +/- 5.7 years). The mean age of the patients at surgery was 20 +/- 13 years. LCSD was followed by highly significant (p less than 0.0001) decreases in the number of patients with cardiac events (from 99% to 45%), in the number of cardiac events per patient (from 22 +/- 32 to 1 +/- 3), and in the number of patients with five or more cardiac events (from 71% to 10%). There were seven sudden deaths (8%), and the 5-year survival rate was 94%. The marked reduction in the incidence of tachyarrhythmic syncope suggests that LCSD has also reduced the risk for sudden death in this high-risk population.


CONCLUSIONS
The present findings demonstrate that for LQTS patients who continue with syncope or cardiac arrest despite the use of beta-blockers, LCSD is a very effective therapy.
Schwartz
P J
PJ
Dipartimento di Medicina, University of Pavia, Italy.
Locati
E H
EH
Moss
A J
AJ
Crampton
R S
RS
Trazzi
R
R
Ruberti
U
U
D000293:Adolescent; D000328:Adult; D004562:Electrocardiography; D005500:Follow-Up Studies; D006329:Heart Conduction System; D006801:Humans; D007391:International Cooperation; D008133:Long QT Syndrome / Q000151:congenital / Q000503:physiopathology / Q000628:therapy*; D011184:Postoperative Period; D011446:Prospective Studies; D013562:Sympathectomy