Pulmonary arterial hypertension (PAH) is an incurable disease characterized by an intense thickening of the pulmonary arteries leading to their progressive obliteration. This vascular remodeling is a direct consequence of an aberrant accumulation of α-smooth muscle actin (α-SMA)-positive cells in the intravascular area. This process is multifactorial and complex. Recent studies have demonstrated that part of α-SMA-positive cells from intimal and plexiform lesions have an endothelial origin through a process called endothelial-to-mesenchymal transition (EndoMT). Interestingly most of molecular pathways implicated in PAH are known inducers of EndoMT. This review describes how EndoMT appears to play a crucial role in PAH progression.

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