The Role of Stem Cells in Vascular Remodeling in CTEPH

Chronic thromboembolic pulmonary hypertension or CTEPH is a serious complication of a venous embolism that fails to resolve. Its persistence leads to a complete blockade of the pulmonary artery and development of severe pulmonary hypertension. Mutations in fibrinogen that lead to a resistance to fibrinolysis are one explanation for the prolonged existence of the clot. It is thought that the fibrotic clot provides a unique micro-environmental niche that attracts stem and progenitor cells exacerbating the associated pulmonary vascular remodeling. This chapter focuses on what is currently known about the presence and role of such stem and progenitor cells in the pathogenesis of CTEPH.