Precision therapy in congenital long QT syndrome

Abstract

Long QT syndrome (LQTS) is a potentially life-threatening, but highly treatable genetic heart disease. LQTS-directed therapies often consist of beta-blockers (BBs), left cardiac sympathetic denervation (LCSD), and/or an implantable cardioverter defibrillator (ICD). However, in clinical practice, many patient-specific and genotype-directed permutations exist. Herein, we aim to review the spectrum of treatment configurations utilized at a single, tertiary center specializing in the care of patients with LQTS to demonstrate optimal LQTS-directed management is not amenable to a "one-size-fits-all" approach but instead benefits from patient- and genotype-tailored strategies.

Keywords: Genetic testing; Long QT syndrome; Precision therapy; Sudden cardiac death.