Abstract

Neointima Development in Pulmonary Arterial Hypertension: A Possible Mechanism

Author(s): William Sam, Rebecca Davis

Pulmonary arterial hypertension (PAH) is a significant complication of a variety of systemic disorders, such as cardiac, inflammatory, and autoimmune diseases. Endothelial instability and dysfunction are important in the onset and course of the disease. Although current medicine has improved quality of life, the underlying vascular disease continues to worsen, and the survival rate has not considerably improved. Impaired vascular relaxation response, medial hypertrophy, and increased pulmonary pressure are the predominant pathogenic alterations; subsequent neointimal development resulting in disease irreversibility. The loss of endothelial Caveolin-1 (cav-1), a membrane protein, has been linked to the reciprocal activation of proliferative pathways and the onset of PAH.  Endothelial cell and endothelial cav-1 disruption is followed by increased expression of cav-1 in smooth muscle cells.