79. Increased Recruitment and Proliferation of Intrapulmonary Arterial Perivascular Precursor Cells in Experimental Congenital Diaphragmatic Hernia

Abstract

Congenital diaphragmatic hernia (CDH) is associated with specific pulmonary vascular abnormalities including decreased pulmonary arterial branching, increased muscularization of intrapulmonary arteries, and more distal muscularization within the pulmonary arterial tree. These abnormalities result in varying degrees of pulmonary hypertension, which along with lung hypoplasia, contribute to respiratory distress in neonates with CDH. The process of vascular wall maturation is dependent upon cell signaling interactions between endothelial cells of nascent vessels and the surrounding mesenchyme that lead to the recruitment of perivascular progenitor cells to endothelium tubes and subsequent proliferation and differentiation of these cells to form the vascular smooth muscle cells and pericytes of the arterial wall.