Congenital heart disease (CHD) refers to abnormal development of the heart in the foetus during the embryonic period, usually accompanied by abnormal development of the large blood vessels. The pathogenesis of CHD is complex and has not yet been elucidated. Studies have shown that the Notch signaling pathway is involved in the whole process of cardiac development, including the formation and development of the primitive heart, and the maintenance of normal cardiac function after birth, and the abnormalities in this pathway can lead to the development of CHD. In this paper, we review the mechanisms of Notch signaling pathway involved in cardiac growth and development, with a view of providing reference for the early diagnosis of CHD.