The best studied of the endothelial cell specific growth factors, VEGF, is present in alveolar and bronchial epithelial cells, in vascular smooth muscle cells, and in macrophages. The gene encoding VEGF is abundantly present in lung tissue and is induced by short-term and long-term hypoxia, as well as by prostacyclin, prostaglandin E2, and cyclic AMP. In the case of prostaglandin-stimulated gene upregulation protein kinase A (PKA) is involved. Suramin, an inhibitor of growth factor receptor binding inhibits the development of chronic hypoxic pulmonary hypertension in rats. Further evidence is necessary to link VEGF with pulmonary hypertensive vascular remodeling. This requires the development of antibodies directed against VEGF or against VEGF receptors and gene transfection or antisense strategies.