Does a Short QT Interval Presage a Short Life Span?
Keywords:syncope, primary electrical disease, atrial fibrillation, sudden cardiac death, potassium channels, short QT syndrome
AbstractA case of a 16-year old adolescent is presented who was referred for investigation of a syncopal episode. The ECG displayed a very short QT interval of 280 ms, compatible with the newly described short QT syndrome, which is genetic primary electrical disease of abnormal potassium currents responsible for the repolarization process of the cardiac muscle. The prognosis is similar to the other well known familial cardiac channelopathies, such as the long QT or the Brugada syndromes which are associated with a high risk of sudden cardiac death, requiring an implantable cardioverter defibrillator device for life-long protection.
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