Congenital short QT syndrome: A review focused on electrocardiographic features.

ABSTRACT

Congenital short QT syndrome is a very low prevalence inherited primary arrhythmia syndrome first reported in 2000 by Gussak et al., who described two families with a short QT interval, syncope, and sudden cardiac death. In 2004, Ramon Brugada et al. identified the first genetic type of this entity. To date, a total of nine genotypes have been described. The diagnosis is easy from the electrocardiogram (ECG), not only due to the short QT duration, but also based on other aspects covered in this review. During 24-h Holter monitoring, paroxysmal atrial fibrillation spontaneously converting to sinus rhythm may be found. Even though the T wave may appear symmetric on the ECG, the T loop of the vectorcardiogram confirms that the T wave is constantly asymmetric due to the presence of dashes closer to each other in the efferent branch. In this review, we also describe the minus-plus T wave sign that we have described in a previously published article. In addition to congenital causes, we briefly highlight the existence of numerous acquired causes of short QT interval.