This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Schwartz, P. J. Articles by Ruberti, U. Search for Related Content PubMed PubMed Citation Articles by Schwartz, P. J. Articles by Ruberti, U.

Circulation, Vol 84, 503-511, Copyright © 1991 by American Heart Association

ARTICLES

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

PJ Schwartz, EH Locati, AJ Moss, RS Crampton, R Trazzi and U Ruberti
Dipartimento di Medicina, University of Pavia, Italy.

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.