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(Circulation. 2004;109:1826-1833.)
© 2004 American Heart Association, Inc.

Clinical Investigation and Reports

Left Cardiac Sympathetic Denervation in the Management of High-Risk Patients Affected by the Long-QT Syndrome

Peter J. Schwartz, MD; Silvia G. Priori, MD, PhD; Marina Cerrone, MD; Carla Spazzolini, PhD; Attilio Odero, MD; Carlo Napolitano, MD, PhD; Raffaella Bloise, MD; Gaetano M. De Ferrari, MD; Catherine Klersy, MD, MS; Arthur J. Moss, MD; Wojciech Zareba, MD; Jennifer L. Robinson, MS; W. Jackson Hall, PhD; Paul A. Brink, MD; Lauri Toivonen, MD; Andrew E. Epstein, MD; Cuilan Li, MD; Dayi Hu, MD

From the Department of Lung, Blood and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., M.C., A.O.); IRCCS Policlinico S. Matteo, Pavia, Italy (P.J.S., M.C., C.S., A.O., G.M. D F., C.K.); IRCCS Fondazione Maugeri, Pavia, Italy (S.G.P., C.N., R.B.); Departments of Medicine (A.J.M., W.Z., J.L.R.) and Biostatistics (W.J.H.), University of Rochester Medical Center, Rochester, NY; Division of Cardiovascular Disease, University of Alabama, Birmingham (A.E.E.); Department of Internal Medicine, University of Stellenbosch and Tygerberg Hospital, Tygerberg, South Africa (P.A.B.); Department of Medicine, University of Helsinki, Helsinki, Finland (L.T.); and Department of Cardiology, People’s Hospital of Peking University, Beijing, China (C.L., D.H.).

Correspondence to Peter J. Schwartz, MD, Department of Cardiology, Policlinico S. Matteo IRCCS, V. le Golgi, 19-27100 Pavia, Italy. E-mail PJQT{at}compuserve.com

Received October 30, 2003; de novo received December 23, 2003; accepted February 2, 2004.

Background— The management of long-QT syndrome (LQTS) patients who continue to have cardiac events (CEs) despite ß-blockers is complex. We assessed the long-term efficacy of left cardiac sympathetic denervation (LCSD) in a group of high-risk patients.

Methods and 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 ß-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 ß-blockade and in patients who experience arrhythmia storms with an implanted defibrillator.

Key Words: death, sudden • long-QT syndrome • nervous system, sympathetic • genetics

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