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先天性心脏病是胎儿常见出生缺陷之一，是儿童非传染性疾病死亡的主要原因。先天性心脏病是由于心脏发育过程出现错误而引起的畸形，是一个由多种基因、信号通路和调节因子共同参与的过程。人类基因组的测序以及ENCODE项目表明，基因组中有80%以上的基因被转录，但这些转录物中只有3%对应于翻译蛋白质的RNA，非编码RNA和编码RNA一样重要甚至更重要。越来越多的研究证据表明，不仅蛋白质编码基因参与心血管发育的调控，蛋白质非编码基因也在心脏发育过程中发挥着重要作用。该文主要介绍了长链非编码RNA的生物学特性和功能，以及一些代表性的LncRNA在心脏发育和先天性心脏病中的研究进展。“,”Congenital heart disease is one of the most common birth defects, which is the main cause of death in children.Cardiovascular development is a complex process involving multiple genes, signaling pathways and regulatory factors.According to the sequencing of human genome and encode project, more than 80% of the genes in the genome are transcribed, but only 3% of these transcripts correspond to protein-encoding RNA.It is pointed out that non-coding RNA is as important or even more important as encoding RNA.More and more evidences show that not only encoding genes are involved in the regulation of cardiovascular development, but also protein non-coding genes play an important role in the development of heart.This review will summarize the biological characteristic and function of long noncoding RNAs, and introduce the role of some representative lncRNA in heart development and congenital heart disease.