The congenital long qt-syndrome (lqts) is an important cause of syncope and sudden cardiac death in children and young adults. Although it was originally considered a rare genetic disorder, current insight has shown that the incidence is higher than expected. The identification of mutations in genes encoding cardiac ion channels has strongly increased the knowledge on the pathofysiology of this disorder. This has increased the knowledge on the variability, natural history and therapeutic options for this disease. Identifying lqts patients is important because of the relatively good therapeutic options and the importance of lifestyle advice. Goal of this paper is to review the genetics, clinical characteristics, diagnostic and therapeutic options for children and adults.
