Role of TRPM4 ion channel in pediatric arrhythmic syndromes

TRPM4 is a member of transient receptor potential (TRP ) ion channel group which greatly contributes to cardiomyocyte function and physiology. It controls the dynamic stabilization of calcium level and is involved in sinus node regulation and cardiac conduction. Most of pathogenic variants in TRPM4 gene are associated with conduction disorders and various types of arrhythmic syndromes presenting in children and early adolescents. In addition, TRPM4 gain- and loss-of-function variants are discussed in connection to Brugada syndrome and long QT-syndrome. In this review we summarize current knowledge on the role of TRPM4 variants in pediatric arrhythmic syndromes, discuss molecular mechanisms of TRPM4 dysfunction, provide clinical illustrations and case presentations underlining the role of TRPM4 in molecular pathogenesis of arrhythmic syndromes presented in young age.