Heart is made from different mesodermal tissue cells under gene regulation through precise and complex processes, so the role of genes involved in the regulation is critical. Current known congenital heart disease-related transcription factor genes include BHLH, NKX2.5, GATA, and TBX. In recent years, foreign literature has reported hand gene, one of the BHLH family, was related to congenital heart diseases. This article briefly introduced hand gene and describes its mechanism in congenital heart diseases.