Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited 
arrhythmogenic disorder that causes syncopal episodes related with stress or 
emotion and even sudden cardiac deaths. Signs and symptoms usually begin in 
childhood. A suspicion of CPVT should be kept in mind when a child or an 
adolescent suddenly loses consciousness, particularly if this happens upon 
physical exercise or sudden mental stress. During the past decade, the knowledge 
of CPVT genetics and physiology has increased. Exercise testing is essential 
when suspecting arrhythmogenic origin of syncope, and in the case of CPVT, it 
may be even more sensitive than Holter monitoring. Beta-antiadrenergic 
medication can substantially decrease the mortality associated with CPVT. 
Asymptomatic patients with known CPVT gene defects should also be treated 
because sudden cardiac death may be the first manifestation of the disease. An 
implantable cardioverter-defibrillator may also be required in the most severe 
CPVT cases. In this review, we summarise the current knowledge on the clinical 
characteristics, diagnostic, genetic and prognostic features of CPVT in 
children. In all, 133 publications covering 60 years were checked, and those 
written in English and containing ten or more, mainly paediatric CPVT cases, 
were included. In addition, a CPVT family with three members and delayed 
diagnoses until late childhood and adulthood is presented.