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Title: Ankyrin-B syndrome: a case of sinus node dysfunction, atrial fibrillation and prolonged QT in a young adult
Authors: Ford, Tom ;Robaei, D.;Ooi, S.Y.
Issue Date: Feb-2015
Source: Volume 24, Issue 2, e31 - e34
Journal title: Heart, Lung & Circulation
Abstract: Ankyrin-B protein is involved in regulating expression and localisation of cardiac ion channels and transporters. Mutations of the ANK2 gene in the rare condition Ankyrin-B syndrome result in loss of function of the ankyrin-B protein which in turn leads to abnormal regulation of intracellular sodium and calcium and a predisposition to cardiac arrhythmia including torsades de pointes. We describe a rare case of this condition characterised by sinus node dysfunction, atrial fibrillation and prolonged QT syndrome in a young patient with a family history of sudden death. The management of Ankyrin-B syndrome may include avoidance of QT prolonging medications, insertion of a permanent pacemaker for sinus node dysfunction, or a cardioverter defibrillator for those at high-risk of sudden death from torsades de pointes.
DOI: 10.1016/j.hlc.2014.09.013
ISSN: 1443-9506
Publicaton type: Journal Article
Keywords: Cardiology
Heart Disease
Study or Trial: Case Series and Case Reports
Appears in Collections:Cardiology

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