Flecainide suppresses bidirectional ventricular tachycardia and reverses tachycardia-induced cardiomyopathy in Andersen-Tawil syndrome

Oscar A. Pellizzón, Luis Kalaizich, Louis J. Ptáček, Martin Tristani-Firouzi, Mario D. Gonzalez

Research output: Contribution to journalArticle

34 Citations (Scopus)

Abstract

BVT and Andersen-Tawil Syndrome. Bidirectional ventricular tachycardia (BVT), although a rare arrhythmia in the general population, is frequently observed in patients with Andersen-Tawil syndrome and long QT interval. However, the pharmacologic treatment of this arrhythmia remains unknown. In the present study, we documented the favorable antiarrhythmic action of flecainide in a young woman with sustained BVT and Andersen-Tawil syndrome. She presented with incessant BVT that could only be terminated with flecainide. During sinus rhythm, a prolonged QT interval was observed. Genetic studies revealed a mutation in the K+ channel gene KCNJ2. Over a 4-year follow-up period, recurrence of her arrhythmia occurred twice. The first episode was due to noncompliance and resolved with resumption of flecainide therapy. The second recurrence was associated with a tachycardia-induced cardiomyopathy and resolved when the dose of flecainide was increased from 200 to 300 mg daily. This report suggests that flecainide can be effective in controlling BVT associated with Andersen-Tawil syndrome and indicates that the left ventricular dysfunction is secondary to the arrhythmia and not due to an associated phenotypic manifestation of the disorder.

Original languageEnglish (US)
Pages (from-to)95-97
Number of pages3
JournalJournal of cardiovascular electrophysiology
Volume19
Issue number1
DOIs
StatePublished - Jan 1 2008

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Andersen Syndrome
Flecainide
Cardiomyopathies
Tachycardia
Cardiac Arrhythmias
Recurrence
Left Ventricular Dysfunction
Bidirectional tachycardia
Mutation
Therapeutics
Population
Genes

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

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abstract = "BVT and Andersen-Tawil Syndrome. Bidirectional ventricular tachycardia (BVT), although a rare arrhythmia in the general population, is frequently observed in patients with Andersen-Tawil syndrome and long QT interval. However, the pharmacologic treatment of this arrhythmia remains unknown. In the present study, we documented the favorable antiarrhythmic action of flecainide in a young woman with sustained BVT and Andersen-Tawil syndrome. She presented with incessant BVT that could only be terminated with flecainide. During sinus rhythm, a prolonged QT interval was observed. Genetic studies revealed a mutation in the K+ channel gene KCNJ2. Over a 4-year follow-up period, recurrence of her arrhythmia occurred twice. The first episode was due to noncompliance and resolved with resumption of flecainide therapy. The second recurrence was associated with a tachycardia-induced cardiomyopathy and resolved when the dose of flecainide was increased from 200 to 300 mg daily. This report suggests that flecainide can be effective in controlling BVT associated with Andersen-Tawil syndrome and indicates that the left ventricular dysfunction is secondary to the arrhythmia and not due to an associated phenotypic manifestation of the disorder.",
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Flecainide suppresses bidirectional ventricular tachycardia and reverses tachycardia-induced cardiomyopathy in Andersen-Tawil syndrome. / Pellizzón, Oscar A.; Kalaizich, Luis; Ptáček, Louis J.; Tristani-Firouzi, Martin; Gonzalez, Mario D.

In: Journal of cardiovascular electrophysiology, Vol. 19, No. 1, 01.01.2008, p. 95-97.

Research output: Contribution to journalArticle

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