Efficacy of flecainide in bidirectional ventricular tachycardia and tachycardia-induced cardiomyopathy with Andersen-Tawil syndrome

• Published in: European journal of medical genetics Vol. 65; no. 6; p. 104499
• Main Authors: Ünal Yüksekgönül, Ayşe, Azak, Emine, Akalın, Akçahan, Ertuğrul, İlker, Kılıç, Esra, Utine, Gülen E., Karagöz, Tevfik
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Masson SAS 01.06.2022
• Subjects: Andersen-Tawil syndrome; Flecainide; Left ventricular dysfunction; Novel mutation; Ventricular arrhythmias; Ventricular arrhythmias; Left ventricular dysfunction; Flecainide; Andersen-Tawil syndrome; Novel mutation
• ISSN: 1769-7212; 1878-0849
• DOI: 10.1016/j.ejmg.2022.104499

Andersen-Tawil syndrome is a rare autosomal dominant genetic or sporadic disorder characterized by periodic paralysis, ventricular arrhythmias and dysmorphic features. Ventricular arrhythmias can include frequent premature ventricular complex, polymorphic ventricular tachycardia, and less frequently bidirectional ventricular tachycardia. Left ventricle function has been reported in only a few individual cases of Andersen-Tawil syndrome. A 14-year-old female patient was referred to our clinic from another center with documented arrhythmia and left ventricular systolic dysfunction. Andersen-Tawil syndrome was suspected and the diagnosis was confirmed after detection of a previously unreported mutation in children. We report the successful use of flecainide in bidirectional ventricular tachycardia and tachycardia-induced cardiomyopathy in a case of Andersen-Tawil syndrome associated with a novel mutation.