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Archives of Medical Science
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1/2018
vol. 14
 
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abstract:
Letter to the Editor

Propafenone is not effective for severe ventricular arrhythmias in Andersen-Tawil syndrome

Piotr Bienias, Anna Kostera-Pruszczyk, Maria Miszczak-Knecht, Michał Ciurzyński, Piotr Pruszczyk

Arch Med Sci 2018; 14, 1: 248–250
Online publish date: 2016/06/30
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Andersen-Tawil syndrome (ATS), which is sometimes named long QT syndrome type 7, is a rare genetic potassium channelopathy combining frequent and sometimes serious ventricular arrhythmias (VA), a prominent U-wave in electrocardiography, periodic paralysis and various dysmorphic features. In most ATS patients a mutation in the KCNJ2 gene was found (encoding potassium channel Kir2.1) [1 3]. Since ATS is a rare disease, no evidence-based recommendations for VA management are available either. Successful -blocker, calcium channel blocker or flecainide medication has been described in many cases [1, 2, 4–6]. However, limited data on propafenone treatment and its efficacy in ATS are available [7].
We present an 18-year-old woman with genetically confirmed ATS (G146R mutation of KCJN2 gene) in whom severe VA were observed since her childhood. She presented typical ATS dysmorphic features (hypertelorism, mandibular hypoplasia and syndactyly) and suffered from periodic paralysis. Andersen-Tawil syndrome was also diagnosed in her mother (the same mutation), but she presented only slight dysmorphic features and no VA. The patient’s father and her two brothers were healthy. At presentation the patient’s echocardiography was normal. In many Holter recordings asymptomatic typical VA were observed including frequent pairs, bigeminy or trigeminy and non-sustained bidirectional ventricular tachycardia (BVT), up to 50 60 thousand ventricular beats daily. After VA diagnosis, various antiarrhythmic drugs were prescribed and finally flecainide was found to be the most effective, with significant VA reduction to 5–10 thousand beats daily and 5–20 short BVT. Due to logistic problems with flecainide supply in our country, we decided to replace flecainide with readily available propafenone, which is also a Vaughan-Williams class IC antiarrhythmic agent and is registered for VA treatment. After admission to our department the patient received propafenone 150 mg t.i.d. and metoprolol succinate 50 mg daily with a small increase of VA in initial clinical observation. Unfortunately, subsequent Holter recordings showed stable intensified VA (15–20 thousand beats daily), and a substantial increase of the number, length and heart rate of BVT (400–800 episodes daily, up to 20–25 s). Typical BVT and standard ECG of our patient are presented in the Figure 1. Moreover, the patient became pregnant with further exacerbation of VA (especially BVT), so we decided to...


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