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Intravenous lidocaine in malignant long QT type 3: A bridge over troubled waters

Published:October 02, 2021DOI:https://doi.org/10.1016/j.hrthm.2021.09.038
      Long QT syndrome (LQTS) represents one of the most successful bench-to-bedside stories in medicine. Today, most patients with LQTS can expect to live long and relatively event-free lives and physicians expect a relatively uncomplicated patient population that follows the rules. But, the majority of the therapeutic successes are in patients with LQTS type 1 or type 2 who are more likely to play by the rules. Elegant basic science research illustrates disturbed channel function, the consequence of genetically determined variants. Clinical research, informed by the results of scientific inquiry and effectively applied to patients in a gene-directed manner, helps inform guidelines. The expert consensus guidelines
      • Priori S.G.
      • Wilde A.A.
      • Horie M.
      • et al.
      HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013.
      outline optimal patient management. Therapeutic options have expanded over the last 2 decades and now include a number of pharmacological agents, denervation therapy, and/or device therapy. Yet, occasionally one is confronted with a patient who does not follow the rules, in whom all conventional therapy fails. There are rare patients with such profound LQTS that they can only be saved by heart transplantation.
      • Kelle A.M.
      • Bos J.M.
      • Etheridge S.P.
      • et al.
      Cardiac transplantation in children and adolescents with long QT syndrome.
      Some of the most problematic patients with LQTS are those with LQT type 3 (LQT3), some who do not play by the rules. This is certainly true of the patients in the article by Bains et al in this issue of Heart Rhythm Journal.
      • Bains S.
      • Lador A.
      • Neves R.
      • et al.
      Role of chronic, continuous intravenous lidocaine in the clinical management of patients with malignant type 3 long QT syndrome.
      This small case series outlines the difficult journey in 4 children with malignant LQT3 and the efficacy of intravenous (IV) lidocaine as a bridge to transplant, a bridge over very troubled waters.
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