Usefulness of the intravenous flecainide challenge test before oral flecainide treatment in a patient with Andersen-Tawil syndrome
Usefulness of the intravenous flecainide challenge test before oral flecainide treatment in a patient with Andersen-Tawil syndrome
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DOI：
10.1136/bcr-2019-229628
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发表时间：
2019-07
期刊：
影响因子：
0.9
通讯作者：
A. Sato;Toshiki Takano;M. Chinushi;T. Minamino
中科院分区：
文献类型：
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作者：
A. Sato;Toshiki Takano;M. Chinushi;T. Minamino
Andersen-Tawil综合征（ATS）是一种遗传性疾病，其特征是室性心律失常（VA）、周期性麻痹和畸形特征三联征。一名31岁女性被诊断为KCNJ 2突变（p.R228ins）引起的ATS，在运动期间晕厥发作后紧急入院。心电图显示频繁的室性早搏和非持续性室性心动过速（VT）与多形性QRS模式。在静脉注射氟卡尼（30 mg）试验期间，频繁的VA被完全抑制。开始口服氟卡尼（100 mg）后，除短暂的二联律外，运动试验期间VA均受到抑制。在24小时霍尔特记录中，VA从50 133次/天降至13 363次/天（-73%）。在3年的随访期内未观察到持续性VT和晕厥。静脉氟卡尼激发试验可能有助于预测ATS患者口服氟卡尼的疗效。
Andersen-Tawil syndrome (ATS) is an inherited disorder characterised by the triad of ventricular arrhythmias (VAs), periodic paralysis and dysmorphic features. A 31-year-old woman diagnosed with ATS caused by a KCNJ2 mutation (p.R228ins) was urgently admitted to our hospital following an episode of syncope during exercise. Electrocardiography revealed frequent premature ventricular complexes and non-sustained ventricular tachycardias (VTs) with pleomorphic QRS patterns. During the intravenous flecainide test (30 mg), the frequent VAs were inhibited completely. After oral flecainide (100 mg) was started, VAs, except for a brief bigeminy, were suppressed during the exercise test. On 24-hour Holter recordings, the VAs decreased from 50 133 to 13 363 beats/day (−73%). Sustained VT and syncope were not observed during a 3-year follow-up period. Intravenous flecainide challenge test may be useful in predicting the efficacy of oral flecainide treatment for patients with ATS.