Please use this identifier to cite or link to this item: https://hdl.handle.net/1/1572
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.
URI: https://elibrary.cclhd.health.nsw.gov.au/cclhdjspui/handle/1/1572
DOI: 10.1016/j.hlc.2014.09.013
Pubmed: https://www.ncbi.nlm.nih.gov/pubmed/25456501
ISSN: 1443-9506
Publicaton type: Journal Article
Keywords: Cardiology
Heart Disease
Study or Trial: Case Series and Case Reports
Appears in Collections:Cardiology

Show full item record

Page view(s)

22
checked on Feb 6, 2023

Google ScholarTM

Check

Altmetric


Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.