Pulmonary arterial hypertension (PAH) is a multifactorial cardiopulmonary disease characterized by an elevation of pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR), which can lead to right ventricular (RV) failure, multi-organ dysfunction and ultimately to premature death. Despite the advances in molecular biology, the mechanisms underlying pulmonary hypertension (PH) remain unclear. Nowadays, there is no curative treatment for treating PH. Therefore, it is crucial to identify novel specific therapeutic targets and to offer more effective treatments against the progression of PH. Increasing evidence suggests that epigenetic modification may play a critical role in the pathogenesis of PAH. In the present paper, we provide an overview of the epigenetic mechanisms, specifically, DNA methylation, histone acetylation, histone methylation, and non-coding RNAs. As the recent identification of new pharmacological drugs targeting these epigenetic mechanisms has open new therapeutic avenues, we also discuss the importance of epigenetic-based therapies in the context of PH.
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