Left cardiac sympathetic denervation in the therapy of congenital long QT syndrome. A worldwide report.

PJ Schwartz, EH Locati, AJ Moss, RS Crampton… - Circulation, 1991 - Am Heart Assoc
PJ Schwartz, EH Locati, AJ Moss, RS Crampton, R Trazzi, U Ruberti
Circulation, 1991Am Heart Assoc
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. METHODS AND RESULTS We identified
85 LQTS patients worldwide who underwent LCSD, and we provide here the first large-scale …
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.
METHODS AND 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.
Am Heart Assoc