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 …

Background Congenital long QT syndrome (LQTS) is a potentially lethal, highly treatable
genetic heart disease affecting an estimated 1 in 2000 individuals. Treatment strategies
generally include beta blockers (BBs), left cardiac sympathetic denervation (LCSD), and/or
implantable cardioverter defibrillator (ICD). However, there are numerous patient-specific
and genotype-directed therapeutic options ranging from intentional non-therapy (INT) to
multimodal combinations of pharmaceutical, surgical, and device therapy. Herein, we …