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

Background—The management of long-QT syndrome (LQTS) patients who continue to have cardiac events (CEs) despite β-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 β-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 β-blockade and in patients who experience arrhythmia storms with an implanted defibrillator.

[1]  Carlo Napolitano,et al.  How Really Rare Are Rare Diseases?: , 2003, Journal of cardiovascular electrophysiology.

[2]  Calum A MacRae,et al.  Risk stratification in the long-QT syndrome. , 2003, The New England journal of medicine.

[3]  A. Moss,et al.  Implantable Cardioverter Defibrillator in High‐Risk Long QT Syndrome Patients , 2003, Journal of cardiovascular electrophysiology.

[4]  S. Viskin Implantable Cardioverter Defibrillator in High‐Risk Long QT Syndrome Patients , 2003, Journal of cardiovascular electrophysiology.

[5]  P. Schwartz QT Prolongation, Sudden Death, and Sympathetic Imbalance: The Pendulum Swings , 2001, Journal of cardiovascular electrophysiology.

[6]  U Ravens,et al.  Task Force on Sudden Cardiac Death of the European Society of Cardiology. , 2001, European heart journal.

[7]  P. Schwartz Another Role for the Sympathetic Nervous System in the Long QT Syndrome? , 2001, Journal of cardiovascular electrophysiology.

[8]  G. Breithardt,et al.  Life-threatening Arrhythmias Genotype-phenotype Correlation in the Long-qt Syndrome : Gene-specific Triggers for Genotype-phenotype Correlation in the Long-qt Syndrome Gene-specific Triggers for Life-threatening Arrhythmias , 2022 .

[9]  M. Fishbein,et al.  Nerve sprouting and sudden cardiac death. , 2000, Circulation research.

[10]  W. Allan,et al.  Long QT Syndrome , 1998, Pediatrics.

[11]  S. Priori,et al.  Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. , 2000, Circulation.

[12]  S. Priori,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. , 1995, Circulation.

[13]  A. Moss,et al.  Long QT Syndrome: An Indication for Cervicothoracic Sympathectomy , 1995, Cardiovascular surgery.

[14]  Peter J. Schwartz,et al.  Diagnostic Criteria for the Long QT Syndrome An Update , 1993, Circulation.

[15]  P. Schwartz,et al.  Prevention of Sudden Cardiac Death After a First Myocardial Infarction by Pharmacologic or Surgical Antiadrenergic Interventions , 1992 .

[16]  A. Moss,et al.  Left Cardiac Sympathetic Denervation in the Therapy of Congenital Long QT Syndrome: A Worldwide Report , 1991, Circulation.

[17]  J. Jalife,et al.  Cardiac Electrophysiology: From Cell to Bedside , 1990 .

[18]  P. Schwartz,et al.  Idiopathic long QT syndrome: progress and questions. , 1985, American heart journal.

[19]  R. Winkle,et al.  Efficacy of left cardiac sympathectomy in the treatment of patients with the long QT syndrome. , 1984, Circulation.

[20]  P. Schwartz THE RATIONALE AND THE ROLE OF LEFT STELLECTOMY FOR THE PREVENTION OF MALIGNANT ARRHYTHMIAS , 1984, Annals of the New York Academy of Sciences.

[21]  H. L. Stone,et al.  Effects of Unilateral Stellectomy upon Cardiac Performance during Exercise in Dogs , 1979, Circulation research.

[22]  A. Moss,et al.  Unilateral cervicothoracic sympathetic ganglionectomy for the treatment of long QT interval syndrome. , 1971, The New England journal of medicine.

[23]  J. B. Preston,et al.  Functional Distribution of Right and Left Stellate Innervation to the Ventricles: PRODUCTION OF NEUROGENIC ELECTROCARDIOGRAPHS CHANGES BY UNILATERAL ALTERATION OF SYMPATHETIC TONE , 1966, Circulation research.

[24]  Copyright , 2005, DFT.

[25]  P. Schwartz,et al.  Sympathetic nervous system and cardiac arrhythmias , 1990 .

[26]  P. Schwartz,et al.  Effects of unilateral cardiac sympathetic denervation on the ventricular fibrillation threshold. , 1976, The American journal of cardiology.