Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited cardiac arrhythmia disorder that is characterized by emotion- or exercise-induced polymorphic ventricular arrhythmias and may lead to sudden cardiac death (SCD). CPVT plays an important role in SCD in the young and therefore recognition and adequate treatment of the disease are of vital importance. In this thesis entitled “Risk stratification and genetics in catecholaminergic polymorphic ventricular tachycardia” we aimed to develop effective strategies for rationalizing therapies based on an individual’s risk profile, thereby reducing morbidity and preventing SCD in patients with CPVT. To achieve these aims, we established an international CPVT registry to correlate phenotype with genotype, to generate a risk prediction model for clinical use and to enable identification of relevant new genes.