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

  • Sahej Bains
    Affiliations
    Medical Scientist Training Program, Mayo Clinic Alix School of Medicine, Mayo Clinic, Rochester, Minnesota

    Department of Molecular Pharmacology and Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota
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  • Adi Lador
    Affiliations
    Division of Cardiac Electrophysiology, Department of Cardiology, Houston Methodist DeBakey Heart & Vascular Center, Houston Methodist Hospital, Houston, Texas
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  • Raquel Neves
    Affiliations
    Department of Molecular Pharmacology and Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota

    Department of Cardiovascular Medicine, Division of Heart Rhythm Services, Windland Smith Rice Genetic Heart Rhythm Clinic, Mayo Clinic, Rochester, Minnesota

    Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota
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  • J. Martijn Bos
    Affiliations
    Department of Molecular Pharmacology and Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota

    Department of Cardiovascular Medicine, Division of Heart Rhythm Services, Windland Smith Rice Genetic Heart Rhythm Clinic, Mayo Clinic, Rochester, Minnesota

    Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota
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  • John R. Giudicessi
    Affiliations
    Department of Cardiovascular Medicine, Division of Heart Rhythm Services, Windland Smith Rice Genetic Heart Rhythm Clinic, Mayo Clinic, Rochester, Minnesota
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  • Bryan C. Cannon
    Affiliations
    Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota
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  • Michael J. Ackerman
    Correspondence
    Address reprint requests and correspondence: Dr Michael J. Ackerman, Division of Heart Rhythm Services, Department of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Genetic Heart Rhythm Clinic and Sudden Death Genomics Laboratory, Mayo Clinic, Guggenheim 501, Rochester, MN 55905.
    Affiliations
    Department of Molecular Pharmacology and Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota

    Department of Cardiovascular Medicine, Division of Heart Rhythm Services, Windland Smith Rice Genetic Heart Rhythm Clinic, Mayo Clinic, Rochester, Minnesota

    Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota
    Search for articles by this author
Published:September 15, 2021DOI:https://doi.org/10.1016/j.hrthm.2021.09.016

      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

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