Therapeutic Efficacy of Mexiletine for Long QT Syndrome Type 2: Evidence From Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes, Transgenic Rabbits, and Patients.
Lia CrottiRaquel Almeida Lopes NevesFederica DagradiGiulia MusuFederica GiannettiJ Martijn BosMiriam BarbieriPaolo CereaFulvio L F GiovenzanaMargherita TorchioManuela MuraMassimiliano GnecchiGiulio ConteAngelo AuricchioLuca SalaKatja E OdeningMichael J AckermanPeter J SchwartzPublished in: Circulation (2024)
Mexiletine significantly shortens cardiac repolarization in LQT2 human induced pluripotent stem cells, in the LQT2 rabbit model, and in the majority of patients with LQT2. Furthermore, mexiletine showed antiarrhythmic efficacy. Mexiletine should therefore be considered a valid therapeutic option to be added to conventional therapies in higher-risk patients with LQT2.