Plotse dood en wegrakingen door een erfelijke hartritmestoornis; mogelijke diagnose bij wegrakingen en plots overlijden van familieleden

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited cardiac arrhythmia syndrome. CPVT is characterised by polymorphic ventricular arrhythmias induced by exercise or emotion. These arrhythmias may lead to sudden death. We describe 2 patients with CPVT: a 38-year-old asymptomatic male with a family history of sudden death at a young age, and a 28-year-old woman who suffered from recurrent syncopal episodes triggered by exercise or emotion. In both of these cases the diagnosis CPVT was missed initially, even though the typical arrhythmias were present during exercise tests. CPVT should be considered in young patients who present with syncopal episodes during exercise or emotions, or who display polymorphic ventricular arrhythmias. Family history can also be indicative, particularly if family members have died suddenly at a young age under similar circumstances