Circulation, Vol 84, 503-511, Copyright © 1991 by American Heart Association
PJ Schwartz, EH Locati, AJ Moss, RS Crampton, R Trazzi and U Ruberti
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.
ARTICLES
Left cardiac sympathetic denervation in the therapy of congenital long QT syndrome. A worldwide report
Dipartimento di Medicina, University of Pavia, Italy.
This article has been cited by other articles:
 |
|
 |
|
  C. Spazzolini, J. Mullally, A. J. Moss, P. J. Schwartz, S. McNitt, G. Ouellet, T. Fugate, I. Goldenberg, C. Jons, W. Zareba, et al. Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy. J. Am. Coll. Cardiol., August 25, 2009; 54(9): 832 - 837. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. C. Ahrens-Nicklas, C. E. Clancy, and D. J. Christini Re-evaluating the efficacy of {beta}-adrenergic agonists and antagonists in long QT-3 syndrome through computational modelling Cardiovasc Res, June 1, 2009; 82(3): 439 - 447. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Y. Tan, S. Zhou, M. Ogawa, J. Song, M. Chu, H. Li, M. C. Fishbein, S.-F. Lin, L. S. Chen, and P.-S. Chen Neural Mechanisms of Paroxysmal Atrial Fibrillation and Paroxysmal Atrial Tachycardia in Ambulatory Canines Circulation, August 26, 2008; 118(9): 916 - 925. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. M. Vincent Genotyping Has a Minor Role in Selecting Therapy for Congenital Long-QT Syndromes at Present Circ Arrhythm Electrophysiol, August 1, 2008; 1(3): 227 - 233. [Full Text] [PDF]
|
|
|
|
 |
|
 A. Y. Tan, S. Zhou, B. C. Jung, M. Ogawa, L. S. Chen, M. C. Fishbein, and P.-S. Chen Ectopic atrial arrhythmias arising from canine thoracic veins during in vivo stellate ganglia stimulation Am J Physiol Heart Circ Physiol, August 1, 2008; 295(2): H691 - H698. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Chinushi, D. Izumi, K. Iijima, S. Ahara, S. Komura, H. Furushima, Y. Hosaka, and Y. Aizawa Antiarrhythmic vs. pro-arrhythmic effects depending on the intensity of adrenergic stimulation in a canine anthopleurin-A model of type-3 long QT syndrome Europace, February 1, 2008; 10(2): 249 - 255. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. L. Lujan and S. E. DiCarlo T5 spinal cord transection increases susceptibility to reperfusion-induced ventricular tachycardia by enhancing sympathetic activity in conscious rats Am J Physiol Heart Circ Physiol, December 1, 2007; 293(6): H3333 - H3339. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
W. Shimizu The long QT syndrome: Therapeutic implications of a genetic diagnosis Cardiovasc Res, August 15, 2005; 67(3): 347 - 356. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J R Skinner Is there a relation between SIDS and long QT syndrome? Arch. Dis. Child., May 1, 2005; 90(5): 445 - 449. [Full Text] [PDF]
|
|
|
|
 |
|
 A J Turley, J Thambyrajah, and A A Harcombe Bilateral thoracoscopic cervical sympathectomy for the treatment of recurrent polymorphic ventricular tachycardia Heart, January 1, 2005; 91(1): 15 - 17. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. Villain, I. Denjoy, J.M. Lupoglazoff, P. Guicheney, B. Hainque, V. Lucet, and D. Bonnet Low incidence of cardiac events with {beta}-blocking therapy in children with long QT syndrome Eur. Heart J., August 2, 2004; 25(16): 1405 - 1411. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. J. Schwartz, S. G. Priori, M. Cerrone, C. Spazzolini, A. Odero, C. Napolitano, R. Bloise, G. M. De Ferrari, C. Klersy, A. J. Moss, et al. Left Cardiac Sympathetic Denervation in the Management of High-Risk Patients Affected by the Long-QT Syndrome Circulation, April 20, 2004; 109(15): 1826 - 1833. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. M. van den Bergh, A. Algra, and G. J.E. Rinkel Electrocardiographic Abnormalities and Serum Magnesium in Patients With Subarachnoid Hemorrhage Stroke, March 1, 2004; 35(3): 644 - 648. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. N. Mazzadi, X. Andre-Fouet, J. Duisit, V. Gebuhrer, N. Costes, P. Chevalier, C. Rodriguez, J.-J. Schott, H. Le Marec, P. Guicheney, et al. Cardiac retention of [11C]HED in genotyped long QT patients: a potential amplifier role for severity of the disease Am J Physiol Heart Circ Physiol, August 7, 2003; 285(3): H1286 - H1293. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. D. Booker, S. D. Whyte, and E. J. Ladusans Long QT syndrome and anaesthesia Br. J. Anaesth., March 1, 2003; 90(3): 349 - 366. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Toft, J. Aarøe, B. T. Jensen, M. Christiansen, L. Fog, P. E. B. Thomsen, and J. K. Kanters Long QT syndrome patients may faint due to neurocardiogenic syncope Europace, January 1, 2003; 5(4): 367 - 370. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B Campanelli and J-M Chaudron Long term follow up of long QT syndrome treated by overdrive pacing Heart, November 1, 2001; 86(5): e14 - 14. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S.G. Priori, E. Aliot, C. Blomstrom-Lundqvist, L. Bossaert, G. Breithardt, P. Brugada, A.J. Camm, R. Cappato, S.M. Cobbe, C. Di Mario, et al. Task Force on Sudden Cardiac Death of the European Society of Cardiology Eur. Heart J., August 2, 2001; 22(16): 1374 - 1450. [PDF]
|
|
|
|
|
|
P.-S. Chen, L. S Chen, J.-M. Cao, B. Sharifi, H. S Karagueuzian, and M. C Fishbein Sympathetic nerve sprouting, electrical remodeling and the mechanisms of sudden cardiac death Cardiovasc Res, May 1, 2001; 50(2): 409 - 416. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 O. Aramburu-Bodas, A. Cayon-Blanco, J. C. Garcia-Rubira, and R. Perez-Cano Syncope in a Patient With Cervical Tumor and Prolonged QT Interval Chest, March 1, 2001; 119(3): 992 - 992. [Full Text] [PDF]
|
|
|
|
|
|
E.A. Whitsel, T.E. Raghunathan, R.M. Pearce, D. Lin, P.M. Rautaharju, R. Lemaitre, and D.S. Siscovick RR interval variation, the QT interval index and risk of primary cardiac arrest among patients without clinically recognized heart disease Eur. Heart J., January 2, 2001; 22(2): 165 - 173. [Abstract] [PDF]
|
|
|
|
|
|
P. J. Schwartz, S. G. Priori, C. Spazzolini, A. J. Moss, G. M. Vincent, C. Napolitano, I. Denjoy, P. Guicheney, G. Breithardt, M. T. Keating, et al. Genotype-Phenotype Correlation in the Long-QT Syndrome : Gene-Specific Triggers for Life-Threatening Arrhythmias Circulation, January 2, 2001; 103(1): 89 - 95. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. G. Priori, R. Bloise, and L. Crotti The long QT syndrome Europace, January 1, 2001; 3(1): 16 - 27. [PDF]
|
|
|
|
|
|
A. J. Moss, W. Zareba, W. J. Hall, P. J. Schwartz, R. S. Crampton, J. Benhorin, G. M. Vincent, E. H. Locati, S. G. Priori, C. Napolitano, et al. Effectiveness and Limitations of {beta}-Blocker Therapy in Congenital Long-QT Syndrome Circulation, February 15, 2000; 101(6): 616 - 623. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. C. Dorostkar, M. Eldar, B. Belhassen, and M. M. Scheinman Long-Term Follow-Up of Patients With Long-QT Syndrome Treated With {beta}-Blockers and Continuous Pacing Circulation, December 14, 1999; 100(24): 2431 - 2436. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. M. Broka, S. J. Lustik, J. P. Eichelberger, A. K. Chhibber, and O. Bronsther Perioperative Torsade de Pointes Ventricular Tachycardia • Response Anesth. Analg., April 1, 1999; 88(4): 966 - 966. [Full Text] [PDF]
|
|
|
|
 |
|
 M.-D. Drici, I. Arrighi, C. Chouabe, J. R. Mann, M. Lazdunski, G. Romey, and J. Barhanin Involvement of IsK-Associated K+ Channel in Heart Rate Control of Repolarization in a Murine Engineered Model of Jervell and Lange-Nielsen Syndrome Circ. Res., July 13, 1998; 83(1): 95 - 102. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. Coronel, T. Opthof, P. Taggart, J. Tytgat, and M. Veldkamp Differential electrophysiology of repolarisation from clone to clinic Cardiovasc Res, March 1, 1997; 33(3): 503 - 517. [PDF]
|
|
|
|
|
|
D. M. Roden, R. Lazzara, M. Rosen, P. J. Schwartz, J. Towbin, and G. M. Vincent Multiple Mechanisms in the Long-QT Syndrome: Current Knowledge, Gaps, and Future Directions Circulation, October 15, 1996; 94(8): 1996 - 2012. [Abstract] [Full Text]
|
|
|
|
|
|
S. J. Compton, R. L. Lux, M. R. Ramsey, K. R. Strelich, M. C. Sanguinetti, L. S. Green, M. T. Keating, and J. W. Mason Genetically Defined Therapy of Inherited Long-QT Syndrome: Correction of Abnormal Repolarization by Potassium Circulation, September 1, 1996; 94(5): 1018 - 1022. [Abstract] [Full Text]
|
|
|
|
 |
|
 R. R. Liberthson Sudden Death from Cardiac Causes in Children and Young Adults N. Engl. J. Med., April 18, 1996; 334(16): 1039 - 1044. [Full Text] [PDF]
|
|
|
|
|
|
H. K. Naver, C. Blomstrand, and B. G. Wallin Reduced Heart Rate Variability After Right-Sided Stroke Stroke, February 1, 1996; 27(2): 247 - 251. [Abstract] [Full Text]
|
|
|
|
|
|
M. R. Rosen Long QT Syndrome Patients With Gene Mutations Circulation, December 15, 1995; 92(12): 3373 - 3375. [Full Text]
|
|
|
|
|
|
P. J. Schwartz, S. G. Priori, E. H. Locati, C. Napolitano, F. Cantu, J. A. Towbin, M. T. Keating, H. Hammoude, A. M. Brown, L.-S. K. Chen, et al. Long QT Syndrome Patients With Mutations of the SCN5A and HERG Genes Have Differential Responses to Na+ Channel Blockade and to Increases in Heart Rate : Implications for Gene-Specific Therapy Circulation, December 15, 1995; 92(12): 3381 - 3386. [Abstract] [Full Text]
|
|
|
|
 |
|
 C. Drott, G. Gothberg, and G. Claes Endoscopic Procedures of the Upper-Thoracic Sympathetic Chain: A Review Arch Surg, February 1, 1993; 128(2): 237 - 241. [Abstract] [PDF]
|
|