右位心形成相关分子机制及调控

Molecular mechanism and regulation in the formation of dextrocardia

右位心的形成主要与胚胎心脏左-右体轴的不对称发育异常有关。Nodal、Pitx2、Cited2等基因的不对称表达是脊椎动物左一右体轴不对称分化的重要影响因子，其表达异常会导致器官左右不对称分化异常，表现为右位心、中位心、异位心及其他内脏异位。右位心常伴有多种类型的先天性心脏结构畸形，治疗难度大，预后不良。深入研究右位心形成的影响因素及分子机制，旨在进一步探索右位心的基因诊断及早期干预措施，以提高患者的成活率和生存质量。该文对右位心形成的分子机制及其调控机理进行综述。

Dextrocardia is formed by the disruption of the normal left-right （L-R） asymmetry of the thoracoabdominal organs. Asymmetric expression of Noda1, Pitx2, Cited2 have affected L-R asymmetry of verte- brate significantly, and generated dextrocardia, mesocardia, ectopia cordis, beterotaxy and so on. Dextrocardia is often accompanied by congenital cardiac malformation. Therefore, it is so difficult to be treated that it has poor prognosis. This paper reveiws the molecular mechanism and it＇s regulation of dextrocardia for proceeding the ge- netic diagnosis and early intervention of dextrocardia sequentially in order to improve the survival rate and life quality of patients.