Journal
ARCHIVES OF CARDIOVASCULAR DISEASES
Volume 101, Issue 11-12, Pages 779-786Publisher
ELSEVIER MASSON, CORPORATION OFFICE
DOI: 10.1016/j.acvd.2008.08.009
Keywords
QT interval; Short QT syndrome; Idiopathic ventricular fibrillation; Sudden death; Ion channel disorder
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The short QT syndrome, a recently discovered ion channel disorder, combines shortened repolarization, a predisposition to atrial and ventricular fibrillatory arrhythmias, and a risk of sudden death. Few cases have been reported, but the prevalence may be underestimated. This syndrome might account for some cases of unexplained ventricular fibrillation in patients with otherwise healthy hearts. Patients have abnormally short QT intervals and refractory periods, and atrial/ventricular fibrillation can be triggered during investigations. Gain-of-function mutations have been detected in three genes encoding potassium channels. Treatment is based on defibrillator implantation, sometimes as a preventive measure. Quinidine may be beneficial in certain cases. (c) 2008 Elsevier Masson SAS. All rights reserved.
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