id author title date pages extension mime words sentences flesch summary cache txt cord-019001-ralxw4ad Oishi, Peter Diseases of the Pulmonary Vascular System 2008-11-15 .txt text/plain 12794 669 27 In addition, physical expansion of the lung results in the release of vasoactive substances, such as PGI 2 , which increases pulmonary blood fl ow and decreases pulmonary vascular resistance in the fetal goat and lamb independent of the changes in oxygen tension [35] [36] [37] [38] [39] [40] . In humans, endothelial dysfunction, including histologic abnormalities of the endothelium, impairment of endothelium-dependent pulmonary vasodilation, and increased plasma ET-1 concentrations have been described in children with congenital heart defects and pulmonary hypertension before the development of signifi cant vascular remodeling [22, 98, 101] . In addition, neonates with PPHN and adults with advanced pulmonary vascular disease have evidence of endothelial dysfunction, impairment of endothelium-dependent pulmonary vasodilation, increased plasma ET-1 concentrations, and decreased prostacyclin production [23, 24, 62, 99] . The oral formulation is currently being investigated for chronic pulmonary hypertensive therapy, and recent short-term studies demonstrate benefi cial effects in children with advanced pulmonary vascular disease [164] . ./cache/cord-019001-ralxw4ad.txt ./txt/cord-019001-ralxw4ad.txt