Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome? [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. 2018; 46(8): 718-722 | DOI: 10.5543/tkda.2017.77856

Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome?

Yakup Ergül1, Senem Özgür1, Sertaç Hanedan Onan2, Volkan Tuzcu3
1Department of Pediatric Cardiology and Arrhythmia, Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Turkey
2Department of Pediatric Cardiology, Bağcılar Training and Research Hospital, İstanbul, Turkey
3Department of Pediatric Cardiology and Arrhythmia, Medipol University Hospital, İstanbul, Turkey

Andersen-Tawil syndrome (ATS) is a disorder that causes episodes of muscle weakness (periodic paralysis), changes in heart rhythm, and developmental abnormalities. QT prolongation and ventricular arrhythmias, including bidirectional ventricular tachycardia (VT) and polymorphic VT, may occur. About 60% of all cases of the disorder are caused by mutations in the KCNJ2 gene. A 13-year-old female patient was referred for frequent premature ventricular contractions. Suspicion of ATS due to dysmorphic findings, electrocardiogram changes, and periodic muscle weakness was genetically confirmed. Beta-blocker therapy was initiated as a first-line treatment for bidirectional VT and frequent polymorphic premature ventricular contractions. Despite proper treatment, the VT attacks were not brought under control. Flecainide was added to the treatment regime. The number of premature ventricular contractions was dramatically reduced with flecainide and the VT attacks completely disappeared. This patient is a rare example of ATS in our country. This article provides a description of successful management of rhythm disturbance in a patient with ATS

Keywords: Bidirectional ventricular tachycardia, flecainide; long QT.

How to cite this article
Yakup Ergül, Senem Özgür, Sertaç Hanedan Onan, Volkan Tuzcu. Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome?. Turk Kardiyol Dern Ars. 2018; 46(8): 718-722

Corresponding Author: Yakup Ergül, Türkiye
© Copyright 2018 Archives of the Turkish Society of Cardiology
LookUs & Online Makale