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CIRCULATIONAHA/2008/825547/R4 1 Low incidence of sudden cardiac death in a Swedish Y111C-LQT1 population
"... Background: A 10 % cumulative incidence and a 0.3%/yr incidence rate of sudden cardiac death below 40 years of age and without therapy have been reported in the type 1 long QT syndrome. The Y111C-KCNQ1 mutation causes a severe phenotype in vitro, suggesting a high-risk mutation. Focusing on life-thr ..."
Abstract
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. Twenty-four mutation-carriers <40 years of age experienced syncope (30%). One mutation-carrier had an aborted cardiac arrest (1.25%). No case of sudden cardiac death was reported during a mean non-medicated follow-up of 25±20 years. This corresponds to a low incidence rate of life-threatening cardiac
