| Literature DB >> 31311787 |
Akinori Sato1, Toshiki Takano1, Masaomi Chinushi1,2, Tohru Minamino1.
Abstract
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. © BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.Entities:
Keywords: arrhythmias; cardiovascular medicine
Mesh:
Substances:
Year: 2019 PMID: 31311787 PMCID: PMC6663187 DOI: 10.1136/bcr-2019-229628
Source DB: PubMed Journal: BMJ Case Rep ISSN: 1757-790X