Published March 8, 2022 | Version v1
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Left Cardiac Sympathetic Denervation for Long QT Syndrome: 50 Years' Experience Provides Guidance for Management

  • 1. Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin, Milan, Italy; bDivision of Cardiology, Cardiovascular and Thoracic Department, Azienda Ospedaliera Universitaria "Città della Salute e della Scienza di Torino", Turin, Italy;
  • 2. General Surgery, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
  • 3. Division of Cardiology, Cardiovascular and Thoracic Department, Azienda Ospedaliera Universitaria "Città della Salute e della Scienza di Torino", Turin, Italy
  • 4. Via Del Carmine 5, 20121 Milan, Italy
  • 5. Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin, Milan, ItalyIstituto Auxologico Italiano, IRCCS, Laboratory of Cardiovascular Genetics, Cusano Milanino (MI), Italy; Istituto Auxologico Italiano, IRCCS, Department of Cardiovascular, Neural and Metabolic Sciences, San Luca Hospital, Milan, Italy; University of Milano-Bicocca, Department of Medicine and Surgery, Milan, Italy
  • 6. Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin, Milan, Italy
  • 7. Arrhythmia and Electrophysiology, and Coronary Care Unit, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy;
  • 8. Department of Cardiology, Peking University People's Hospital, Beijing, P.R. China
  • 9. Centre for Cardiac Arrhythmia, Pirogov Russian National Research Medical University, Moscow, Russian Federation.
  • 10. Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin, Milan, Italy; Istituto Auxologico Italiano, IRCCS, Laboratory of Cardiovascular Genetics, Cusano Milanino (MI), Italy

Description

Abstract

Objectives: This study sought to report our single-center experience with left cardiac sympathetic denervation (LCSD) for long QT syndrome (LQTS) since 1973.

Background: LCSD is still underutilized because clinicians are often uncertain whether to use it versus an implantable cardioverter-defibrillator (ICD).

Methods: We performed LCSD in 125 patients with LQTS (58% women, mean QT interval corrected for frequency [QTc] 527 ± 60 ms, 90% on beta blockers) with a follow-up of 12.9 ± 10.3 years. They were retrospectively divided into 4 groups according to the clinical/genetic status: very high risk (n = 18, symptomatic in the first year of life or with highly malignant genetics), with aborted cardiac arrest (ACA) (n = 31), with syncope and/or ICD shocks on beta blockers (n = 45), in primary prevention (n = 31).

Results: After LCSD, 17% in the very high risk group remained asymptomatic, compared with 52%, 47%, and 97% in the other 3 groups (P < 0.0001), with an overall 86% decrease in the mean yearly cardiac event rate (P < 0.0001). Among 45 patients with only syncope/ICD shocks before LCSD, none had ACA/sudden death as first symptom after LCSD and a 6-month post-LCSD QTc <500 ms predicted excellent outcome. Patients with a QTc ≥500 ms have a 50% chance of shortening it by an average of 60 ms. LCSD results are not affected by common genotypes.

Conclusions: We provide definitive evidence for the long-term efficacy of LCSD in LQTS. The degree of antiarrhythmic protection is influenced by patient's specificity and amount of QTc shortening. This novel approach to the analysis of the outcome allows cardiologists to rationally decide and tailor their management strategies to the individual features of their patients.

Notes

Funding: Drs Schwartz and Crotti were partially supported by Leducq Foundation for Cardiovascular Research, grant 18CVD05, "Towards Precision Medicine with Human iPSCs for Cardiac Channelopathies." All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. ACKNOWLEDGMENTS The authors are grateful to the late Prof Ugo Ruberti for his initial surgical interventions, to the many physicians from all over the world who sent us their patients for LCSD, with a special word to Kristina Haugaa and Thomas Paul. We also thank Sergey Termosesov with Rukizhat Ildarova and Dayi Hu with Wenling Liu for their help in the implementation of the LCSD in Russia and China, respectively.

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Journal article: 35331422 (PMID)