Abstract
Long QT syndrome (LQTS) is one of the most common inherited cardiac channelopathies with a prevalence of 1:2000. The condition can be congenital or acquired with 15 recognized genotypes; the most common subtypes are LQTS 1, 2, and 3 making up to 85%-90% of the cases. LQTS is characterized by delayed ventricular cardiomyocyte repolarization manifesting on the surface electrocardiogram (EKG) by a prolonged corrected QT (QTc) interval. The mainstay of treatment for this condition involves in part or combination medical therapy via β-blockers as first-line (or other anti-arrhythmic), left cardiac sympathectomy, or implantable cardiac defibrillator placement. Given the high rate of adverse cardiac events (ACE) or sudden cardiac death (SCD) in this population of patients with this disease, this review seeks to highlight the genotype-specific treatment consensus in β-blocker therapy of the most common subtypes.A database search of PubMed, PMC, and Medline was conducted to ascertain the most recent data in the last five years on the management of LQTS types 1-3 and the role of β-blockers in reducing ACE in these types. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were adhered to in the study selection, and selected studies focused on humans, written in the English Language, and within the last five years of LQTS subtypes 1, 2, and 3.Eleven relevant studies were selected after considering inclusion criteria, exclusion criteria, and quality appraisal within the last five years, focusing on β-blocker selection directed based on the subtypes of LQTS. Two meta-analyses, one cohort study, and eight reviews provided significant data that non-selective β-blockers unequivocally are of benefit in these LQTS types. Summary of findings suggested nadolol followed by propranolol yields the best results in LQTS 1, while nadolol would yield the best effect in LQTS 2 and 3.
Highlights
Long QT syndrome (LQTS) is both an inherited and acquired condition that is characterized by an abnormal prolongation of the QT-interval on electrocardiogram (EKG) monitoring and action potential duration (APD), which increases the risk of fatal ventricular, arrhythmias, sudden cardiac death (SCD), and milder adverse cardiac eventsACE such as syncope [1,2]
Databases used for study retrieval were PubMed, PMC, and Medline, which were searched to ascertain the available data within the last five years in the use and effectiveness of β-blockers in LQTS in reducing the risk of cardiac arrhythmias in LQTS depending on the genotype of the more common LQTS subtypes (1, 2, and 3)
Given the relatively limited studies on LQTS within the last five years to ascertain a broad view of the data, we were able to identify 4592 papers in the initial database search on the topic, which was narrowed to 1757 after Medical Subject Headings (MeSH) criteria were applied
Summary
Long QT syndrome (LQTS) is both an inherited and acquired condition that is characterized by an abnormal prolongation of the QT-interval on electrocardiogram (EKG) monitoring and action potential duration (APD), which increases the risk of fatal ventricular, arrhythmias, sudden cardiac death (SCD), and milder adverse cardiac eventsACE such as syncope [1,2]. This condition was first officially coined and supported by EKG evidence in 1957 and has since been recognized as one of the most commonly inherited channelopathies/arrhythmia syndromes with a prevalence of 1:2000 [2].
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
