Referenced in: none

Automatically associated channels: Kir2.1

Title: [QTU pattern in a patient with the Anderson-Tawil syndrome]

Authors: Michał Ciurzyński, Piotr Bienias, Anna Kostera-Pruszczyk, Piotr Pruszczyk

Journal, date & volume: Kardiol Pol, 2010 Mar , 68, 339-41; discussion 342

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/20411461

Abstract
The Andersen-Tawil syndrome (ATS) is an autosomal dominant or sporadic disorder characterised by periodic paralysis, cardiac arrhythmias and dysmorphic features. Mutations in KCNJ2, which encodes the inward rectifier K+ channel Kir2.1 can be found in approximately 70% of ATS cases. The genetic mechanism in the remaining 30% of patients is still unknown. We present ECG of a 19-year-old man with the Andersen-Tawil syndrome. The standard ECG revealed prominent U wave with the ATS TU pattern.