Short QT syndrome

Short QT syndrome refers to a condition based on a shortened QT interval on the ECG, resulting in a high risk of syncope or sudden cardiac death from malignant ventricular arrhythmias.

It is an autosomal dominant disease based on the mutation of several genes coding for potassium channels. Clinically, despite mutation, it can also lead to asymptomatic or milder courses such as atrial fibrillation.

The implantation of an automatic defibrillator is the only effective therapy.

Short QT syndrome is a disease characterized by a shortened ECG QT interval, which results in a high risk of syncope or sudden cardiac death from malignant ventricular arrhythmias. This is an autosomal dominant disorder based on the mutation of several genes coding for potassium channels. Clinically, despite mutation, there may also be asymptomatic or milder episodes such as atrial fibrillation.

Implanting an automatic defibrillator is the only effective therapy.