Pulmonary arterial hypertension (PAH) is a progressive and a life-threatening condition characterized by high pulmonary blood pressure, remodeling of small pulmonary blood vessels and increased vascular resistance leading to right heart failure. According to the 6th World Symposium on Pulmonary Hypertension, PAH is defined by concomitant elevation of three parameters: mean pulmonary arterial pressure (mPAP) > 20 mmHg; pulmonary arterial wedge pressure (PAWP) ≤15 mm Hg; pulmonary vascular resistance (PVR) ≥ 3 Wood Units.

The right heart failure is due to a persistent remodeling that gradually obstructs and obliterates the peripheral pulmonary arteries that causes vasoconstriction and increases right ventricle afterload. Many efforts have been made to treat PAH. However, there are still few successful and promising pharmacological treatments for this devasting disease, as a consequence, PAH-related survival remains disappointing. Thus, several researches on pulmonary hypertension pathophysiology have focused on a better comprehension of the angioproliferation process and plexiform lesions. The role of increased blood flow in the pulmonary vascular bed is considered one of the main triggering factors to the development of pulmonary vascular remodeling.^–

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