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肺动脉高压是由多种先天性及后天性病因和发病机制所导致的一种临床综合征，表现为肺血管阻力增加和肺动脉压力升高，继而导致右心功能衰竭，甚至死亡。表观遗传学是研究在DNA序列不发生改变的情况下，通过化学修饰来改变基因的表达与功能，并产生可遗传表型的一门学科，包括 DNA 甲基化、组蛋白修饰以及非编码RNA 作用，它是环境与遗传之间关系的桥梁。越来越多的证据表明，表观遗传学修饰在肺动脉高压的发生发展中扮演着重要的角色。本文主要阐述表观遗传学参与肺动脉高压发生发展的机制及其相关进展。

Pulmonary arterial hypertension（PAH）is a clinical syndrome characterized by increased pulmonary vascular resistance and elevated pulmonary arterial pressure，which subsequently leads to right heart failure and even death.PAH is caused by a variety of etiologic and pathogenic mechanisms，including congenital and acquired.Epigenetics is the study of the altered expression and function of genes that produce heritable phenotypes without changes in DNA sequence，and it bridges the relationship between environment and heredity.There is growing evidence that epigenetic modifications，including DNA methylation，histone modifications，and non-coding RNA，play important roles in the development of PAH.This review focused on the mechanisms and progress of epigenetic involvement in the development of PAH.