title: Reduction of complex ventricular ectopy and improvement in exercise capacity with flecainide therapy in Andersen-Tawil syndrome.
abstract: Andersen-Tawil syndrome (ATS) is a rare inherited autosomal disorder characterized by the clinical triad of ventricular arrhythmias, hypokalaemic periodic paralyses, and skeletal developmental abnormalities, resulting in dysmorphic features. Although ATS patients have a high incidence of ventricular arrhythmias, the occurrence of sudden cardiac death is rare. In this report, we describe the successful use of flecainide in an ATS patient with a considerable ventricular arrhythmia burden who had not demonstrated any response to conventional beta-blocker therapy used in conjunction with potassium (K(+)) supplementation.
authors.affiliation: Department of Medicine, Division of Cardiology, University of Western Ontario, London, Ontario, Canada. david.j.fox@talk21.com
authors.forename: David J
authors.initials: DJ
authors.lastname: Fox
authors.forename: George J
authors.initials: GJ
authors.lastname: Klein
authors.forename: Angelika
authors.initials: A
authors.lastname: Hahn
authors.forename: Allan C
authors.initials: AC
authors.lastname: Skanes
authors.forename: Lorne J
authors.initials: LJ
authors.lastname: Gula
authors.forename: Raymond K
authors.initials: RK
authors.lastname: Yee
authors.forename: Rajesh N
authors.initials: RN
authors.lastname: Subbiah
authors.forename: Andrew D
authors.initials: AD
authors.lastname: Krahn
mesh_terms: D000889:Anti-Arrhythmia Agents / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005080:Exercise Test; D017079:Exercise Tolerance; D005424:Flecainide / Q000008:administration & dosage*; D006011:Glycogen Storage Disease Type IV / Q000188:drug therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011188:Potassium / Q000008:administration & dosage*; D016896:Treatment Outcome; D018879:Ventricular Premature Complexes / Q000188:drug therapy*
chemical_list: D000889:Anti-Arrhythmia Agents; D005424:Flecainide; D011188:Potassium