title: Pharmacotherapy for inherited arrhythmia syndromes: mechanistic basis, clinical trial evidence and practical application.
abstract: In the absence of structural heart disease, sudden cardiac death is frequently caused by inherited arrhythmia syndromes, such as long QT syndrome, Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia. Managing these conditions often requires a combination of lifestyle modification, pharmacotherapy and less frequently, invasive therapy. Over the past decade, patient management has been greatly enhanced by tailored pharmacotherapy as a result of a deeper appreciation for arrhythmia mechanisms and supportive evidence from multicenter cohort studies. This article reviews current knowledge regarding drug therapy for inherited arrhythmias. Anti-arrhythmic mechanisms and available clinical evidence are highlighted while maintaining a practical perspective on patient management.
authors.affiliation: Electrophysiology service and Cardiovascular Genetics Center, Montreal Heart Institute, Université de Montréal, 5000 Bélanger St. E., Montreal, Quebec, H1T 1C8 Canada.
authors.forename: Rafik
authors.initials: R
authors.lastname: Tadros
authors.forename: Julia
authors.initials: J
authors.lastname: Cadrin-Tourigny
authors.forename: Sylvia
authors.initials: S
authors.lastname: Abadir
authors.forename: Lena
authors.initials: L
authors.lastname: Rivard
authors.forename: Stanley
authors.initials: S
authors.lastname: Nattel
authors.forename: Mario
authors.initials: M
authors.lastname: Talajic
authors.forename: Paul
authors.initials: P
authors.lastname: Khairy
mesh_terms: D000200:Action Potentials; D000889:Anti-Arrhythmia Agents / Q000494:pharmacology / Q000627:therapeutic use*; D001145:Arrhythmias, Cardiac / Q000188:drug therapy* / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D007473:Ion Channels / Q000187:drug effects / Q000502:physiology; D032383:Myocytes, Cardiac / Q000187:drug effects / Q000502:physiology
chemical_list: D000889:Anti-Arrhythmia Agents; D007473:Ion Channels
keywords: Andersen Tawil syndrome; Brugada syndrome; catecholaminergic polymorphic ventricular tachycardia; channelopathy; early repolarization; idiopathic ventricular fibrillation; long QT syndrome; pharmacology; short QT syndrome; therapy