Congenital short QT syndrome: A review focused on electrocardiographic features

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.

Left septal fascicular block: Evidence, causes, and diagnostic criteria.

The existence of a tetrafascicular intraventricular conduction system is widely accepted by researchers. In this review, we have updated the criteria for left septal fascicular block (LSFB) and the differential diagnosis of prominent anterior QRS forces. More and more evidence points to the fact that the main cause of LSFB is critical proximal stenosis of the left anterior descending coronary artery before its first septal perforator branch. The most important characteristic of LSFB that has been incorporated in the corresponding diagnostic electrocardiographic criteria is its transient/intermittent nature mostly observed in clinical scenarios of acute (ie, acute coronary syndrome including vasospastic angina) or chronic (ie, exercise-induced ischemia) ischemic coronary artery disease. In addition, the phenomenon proved to be phase 4 bradycardia rate dependent and induced by early atrial extrastimulus. Finally, we believe that intermittent LSFB has the same clinical significance as "Wellens syndrome" and the "de Winter pattern" in the acute coronary syndrome scenario.