title: Implantable defibrillators in long QT syndrome, Brugada syndrome, hypertrophic cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy.
abstract: Sudden death is often the first manifestation in inherited cardiac arrhythmia syndromes. Patients with long QT syndrome who have an episode of syncope while on beta-blockade should be offered an implantable cardioverter-defibrillator (ICD). In Brugada syndrome and hypertrophic cardiomyopathy, ICDs are often the most effective treatment of primary and secondary prevention of cardiac arrest. Risk stratification is crucial in identifying those at greatest risk to provide lifesaving therapy with an ICD while avoiding complications in those unlikely to receive benefit.
authors.affiliation: Department of Medicine, The Cardiac Arrhythmia Center, Tufts Medical Center, 800 Washington Street, Box #197, Boston, MA 02111, USA.
authors.forename: Mustafa
authors.initials: M
authors.lastname: Dohadwala
authors.affiliation: Department of Medicine, The Cardiac Arrhythmia Center, Tufts Medical Center, 800 Washington Street, Box #197, Boston, MA 02111, USA. Electronic address: mlink@tuftsmedicalcenter.org.
authors.forename: Mark S
authors.initials: MS
authors.lastname: Link
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis / Q000628:therapy*; D053840:Brugada Syndrome / Q000175:diagnosis / Q000628:therapy*; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis / Q000628:therapy*; D016757:Death, Sudden, Cardiac / Q000517:prevention & control; D017147:Defibrillators, Implantable; D006801:Humans; D008133:Long QT Syndrome / Q000175:diagnosis / Q000628:therapy*; D011474:Prosthesis Design; D011475:Prosthesis Failure; D018570:Risk Assessment / Q000379:methods
chemical_list: D000319:Adrenergic beta-Antagonists
keywords: Arrhythmogenic right ventricular cardiomyopathy; Brugada syndrome; Channelopathy; Genetic syndrome; Hypertrophic cardiomyopathy; Long QT syndrome; Sudden death