Reduction of complex ventricular ectopy and improvement in exercise capacity with flecainide therapy in Andersen-Tawil syndrome.
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.
Fox
David J
DJ
Department of Medicine, Division of Cardiology, University of Western Ontario, London, Ontario, Canada. david.j.fox@talk21.com
Klein
George J
GJ
Hahn
Angelika
A
Skanes
Allan C
AC
Gula
Lorne J
LJ
Yee
Raymond K
RK
Subbiah
Rajesh N
RN
Krahn
Andrew D
AD
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*
D000889:Anti-Arrhythmia Agents; D005424:Flecainide; D011188:Potassium