先天性心脏病（congenital heart disease, CHD）是最常见的出生缺陷，发病机制复杂，与遗传、环境及信号通路异常等多种因素相关。Hippo信号通路是进化高度保守、调控器官大小与组织稳态的关键通路，其核心组分哺乳动物不育系20样激酶1/2、大肿瘤抑制激酶1/2可通过磷酸化Yes相关蛋白/转录共激活因子PDZ结合基序蛋白，调控细胞增殖、分化及凋亡。Hippo通路功能异常与多种CHD的发生发展密切相关。该文综述Hippo通路的核心组成与调控机制，阐述该通路在心脏发育过程中的作用，以及其诱发室间隔缺损、法洛四联症、心肌致密化不全等CHD的分子机制，以期为CHD的早期诊疗提供新的理论依据。

Congenital heart disease (CHD) is the most common birth defect, with a complex pathogenesis involving genetic, environmental, and signaling pathway abnormalities. The Hippo signaling pathway is an evolutionarily conserved key regulator of organ size and tissue homeostasis. Its core components mammalian sterile 20-like kinase 1/2 and large tumor suppressor kinase 1/2 phosphorylate the downstream effectors YAP/TAZ, regulating cell proliferation, differentiation, and apoptosis. Dysfunction of the Hippo pathway is closely related to the occurrence and development of multiple CHD types. This review summarizes the core components and regulatory mechanisms of the Hippo pathway, describes its role in cardiac development, and elucidates the molecular mechanisms by which it contributes to ventricular septal defect, tetralogy of Fallot, and left ventricular noncompaction cardiomyopathy, aiming to provide a new theoretical basis for the early diagnosis and treatment of CHD.