| Pulmonary vascular disease forms an ongoing threat to children with congenital heart disease. Pulmonary plexogenic arteriopathy (PPA) is a specific form of pulmonary arteriopathy that occurs in the presence of heart defects with flow-associated pulmonary hypertension. Although early vascular changes may show similarities with other pulmonary vasculopathies, the advanced lesions, including concentric laminar intimal fibrosis and plexiform lesions, are characteristic for PPA. These specific lesions are of clinical relevance because they are associated with irreversible disease, despite correction of the underlying cardiac defects.The pathogenesis of these lesions is poorly understood. R ecent investigations have suggested that endothelium derived vasoactive mediators, such as prostacylin (PGI2) and nitric oxide (NO), play a role.However, research is hampered by the lack of an appropriate animal model. Therefore, the aim of this project is to develop a suitable animal model to study pathogenesis and (preventive) therapy of the advanced plexogenic lesions associated with PPA. |
