The Short QT Syndrome is a recently described new genetic disorder, 
characterized by abnormally short QT interval, paroxysmal atrial fibrillation 
and life threatening ventricular arrhythmias. This autosomal dominant syndrome 
can afflict infants, children, or young adults; often a remarkable family 
background of cardiac sudden death is elucidated. At electrophysiological study, 
short atrial and ventricular refractory periods are found, with atrial 
fibrillation and polymorphic ventricular tachycardia easily induced by 
programmed electrical stimulation. Gain of function mutations in three genes 
encoding K(+) channels have been identified, explaining the abbreviated 
repolarization seen in this condition: KCNH2 for I(kr) (SQT1), KCNQ1 for I(ks) 
(SQT2) and KCNJ2 for I(k1) (SQT3). The currently suggested therapeutic strategy 
is an ICD implantation, although many concerns exist for asymptomatic patients, 
especially in pediatric age. Pharmacological treatment is still under 
evaluation; quinidine has shown to prolong QT and reduce the inducibility of 
ventricular arrhythmias, but awaits additional confirmatory clinical data.