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Low Incidence of Sudden Cardiac Death in a Swedish Y111C Type 1 Long-QT syndrome Population
Author(s) -
Annika Winbo,
UllaBritt Diamant,
EvaLena Stattin,
Steen M. Jensen,
Annika Rydberg
Publication year - 2009
Publication title -
circulation cardiovascular genetics
Language(s) - English
Resource type - Journals
eISSN - 1942-325X
pISSN - 1942-3268
DOI - 10.1161/circgenetics.108.825547
Subject(s) - long qt syndrome , sudden cardiac death , incidence (geometry) , cardiology , medicine , sudden death , population , qt interval , physics , environmental health , optics
A 10% cumulative incidence and a 0.3% per year incidence rate of sudden cardiac death in patients younger than 40 years and without therapy have been reported in type 1 long-QT syndrome. The Y111C-KCNQ1 mutation causes a severe phenotype in vitro, suggesting a high-risk mutation. This study investigated the phenotype among Y111C-KCNQ1 mutation carriers in the Swedish population with a focus on life-threatening cardiac events.

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