Several clinical studies have provided evidence that the pulmonary endothelium is essential for the production of various mediators of vascular remodeling including vasoactive peptides, nitric oxide (NO), prostaglandin -I2 (PGI2), endothelin-1 (ET-1), fibroblast growth factor (FGF), angiotensin II (Ang II), cytokines (IL-1, IL-6), and cross talk between endothelial and smooth muscle cells; which are crucial to the development and progression of pulmonary hypertension [11,18]. Here, EDN1 is linked to pulmonary arterial hypertension.