Mouse models of human arrhythmia syndromes

Sudden cardiac death stemming from ventricular arrhythmogenesis is one of the major causes of mortality in the developed world. Congenital and acquired forms of long QT syndrome (LQTS) are in turn associated with life threatening arrhythmias. Over the past decade our understanding of arrhythmogenic mechanisms in the setting of these diseases has increased greatly due to the creation of a number of animal models. Of these, the genetically amenable mouse has proved to be a particularly powerful tool. This review summarizes the congenital and acquired LQTS and describes the various mouse models that have been created to further probe arrhythmogenic mechanisms.