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Heart Rhythm. 2015 Mar;12(3):596-603. doi: 10.1016/j.hrthm.2014.12.009. Epub 2014 Dec 10.

Efficacy and safety of flecainide for ventricular arrhythmias in patients with Andersen-Tawil syndrome with KCNJ2 mutations.

Author information

1
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan; Department of Advanced Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
2
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan. Electronic address: aiba@hsp.ncvc.go.jp.
3
Department of Cardiovascular and Respiratory Medicine, Shiga University of Medical Science, Otsu, Japan.
4
Department of Cardiology, Omura Municipal Hospital, Omura, Japan.
5
EP Expert Doctors-Team Tsuchiya, Kumamoto, Japan.
6
Department of Pediatric Electrophysiology, Osaka City General Hospital, Osaka, Japan.
7
Department of Cardiovascular Medicine, Nippon Medical School, Tokyo, Japan.
8
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan.
9
Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Suita, Japan.
10
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan; Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
11
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan; Department of Advanced Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan; Department of Cardiovascular Medicine, Nippon Medical School, Tokyo, Japan. Electronic address: wshimizu@nms.ac.jp.

Abstract

BACKGROUND:

Andersen-Tawil syndrome (ATS) is an autosomal dominant genetic or sporadic disorder characterized by ventricular arrhythmias (VAs), periodic paralyses, and dysmorphic features. The optimal pharmacological treatment of VAs in patients with ATS remains unknown.

OBJECTIVE:

We evaluated the efficacy and safety of flecainide for VAs in patients with ATS with KCNJ2 mutations.

METHODS:

Ten ATS probands (7 females; mean age 27 ± 11 years) were enrolled from 6 institutions. All of them had bidirectional VAs in spite of treatment with β-blockers (n = 6), but none of them had either aborted cardiac arrest or family history of sudden cardiac death. Twenty-four-hour Holter recording and treadmill exercise test (TMT) were performed before (baseline) and after oral flecainide therapy (150 ± 46 mg/d).

RESULTS:

Twenty-four-hour Holter recordings demonstrated that oral flecainide treatment significantly reduced the total number of VAs (from 38,407 ± 19,956 to 11,196 ± 14,773 per day; P = .003) and the number of the longest ventricular salvos (23 ± 19 to 5 ± 5; P = .01). At baseline, TMT induced nonsustained ventricular tachycardia (n = 7) or couplets of premature ventricular complex (n = 2); treatment with flecainide completely (n = 7) or partially (n = 2) suppressed these exercise-induced VAs (P = .008). In contrast, the QRS duration, QT interval, and U-wave amplitude of the electrocardiogram were not altered by flecainide therapy. During a mean follow-up of 23 ± 11 months, no patients developed syncope or cardiac arrest after oral flecainide treatment.

CONCLUSION:

This multicenter study suggests that oral flecainide therapy is an effective and safe means of suppressing VAs in patients with ATS with KCNJ2 mutations, though the U-wave amplitude remained unchanged by flecainide.

KEYWORDS:

Andersen-Tawil syndrome; Flecainide; Long QT; Mutation; Ventricular arrhythmia

PMID:
25496985
DOI:
10.1016/j.hrthm.2014.12.009
[Indexed for MEDLINE]

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