Role of chronic continuous intravenous lidocaine in the clinical management of patients with malignant type 3 long QT syndrome

Heart Rhythm. 2022 Jan;19(1):81-87. doi: 10.1016/j.hrthm.2021.09.016. Epub 2021 Sep 16.


Background: Type 3 long QT syndrome (LQT3) is caused by pathogenic, gain-of-function variants in SCN5A leading to a prolonged action potential, ventricular ectopy, and torsades de pointes. Treatment options include pharmacotherapy, cardiac denervation, and/or device therapy. Rarely, patients with malignant LQT3 require cardiac transplantation.

Objective: The purpose of this study was to evaluate the role of chronic continuous intravenous (IV) lidocaine as a therapeutic option for select patients with LQT3 refractory to standard therapy.

Methods: We performed a retrospective review of patients evaluated and treated at Mayo Clinic and identified 4 of 161 patients with LQT3 (2.5%) who were refractory to standard therapies and therefore treated with IV lidocaine.

Results: There were 4 patients (2 female [50%]). The median age at first IV lidocaine infusion was 2 months (interquartile range 1.5-4.8 months), and the median cumulative duration on IV lidocaine was 11.5 months (interquartile range 8.7-17.8 months). The main indication for IV lidocaine in all patients was persistent ventricular arrhythmias. Before IV lidocaine, all patients received an implantable cardioverter-defibrillator, and while on intermittent IV lidocaine, all patients underwent bilateral cardiac sympathetic denervation. Additionally, 2 (50%) patients had cardiac ablation for premature ventricular complexes. In all patients, lidocaine infusion resulted in a significant reduction of LQT3-triggered cardiac events. The main side effects of IV lidocaine observed were dizziness (n = 2, 50%) and seizures (n = 2, 50%). During follow-up, 3 of 4 (75%) patients underwent orthotopic cardiac transplantation. The remaining patient continues to receive IV lidocaine bolus for rescue as needed.

Conclusion: For patients with LQT3 who are refractory to standard treatment, chronic IV lidocaine infusion can be used as a potential "bridge to transplant."

Keywords: Arrhythmias; Genetics; Lidocaine; Long QT syndrome; Sudden cardiac death.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anti-Arrhythmia Agents / administration & dosage*
  • Cardiac Conduction System Disease / drug therapy*
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Infusions, Intravenous
  • Lidocaine / administration & dosage*
  • Long QT Syndrome / drug therapy*
  • Male
  • Retrospective Studies


  • Anti-Arrhythmia Agents
  • Lidocaine

Supplementary concepts

  • Long QT syndrome type 3