**9. Conclusions**

Currently, SQTS is still a relatively unknown disease. First described in 2000, the small number of families diagnosed with SQTS worldwide makes the establishment of a risk stratification scale difficult. Clarification of electrophysiological and clinical abnormalities associated with the disease and the genetic origin, has only been carried out in recent years. However, only four genes (*KCNH2*, *KCNQ1*, *KCNJ2, SLC4A3*) have been definitively associated with the disease and a comprehensive genetic analysis only identify the causative alteration in no more than 30% of diagnosed families. It is crucial to perform more genotype-phenotype analyses in diagnosed SQTS families as well as segregation studies and *in vitro/ion vivo* functional tests that will allow clarification of the pathophysiological mechanism involved this lethal arrhythmogenic syndrome. Survivors of SCD have a high recurrence rate of episodes, thus implantation of an ICD is recommended in this group of patients. The pharmacological approach may also be effective in some cases, especially in the pediatric population, and the use of personalized medicine is becoming increasingly feasible. Personalized clinical evaluation, genetic analysis and the adoption of effective therapeutic measures by specialists help to improve the evolution of diagnosed patients with an increasingly positive long-term outcome.
