Pulmonary Arterial Hypertension and Its Types:
The Penn Cell Center oversees the isolation and growth of vascular cell culture from explanted lungs from patients with pulmonary arterial hypertension (PAH) and unused donor lungs. PAH is a primary pulmonary vasculopathy characterized by endothelial proliferation and dysfunction, smooth muscle hypertophy, and adventitial thickening.
PAH occurs in several forms which are currently categorized by the Nice Classification. These forms include idiopathic PAH, heritable PAH (an inherited form which is associated with certain genetic mutations), drug and toxin-induced PAH (caused by anorexigens, methamphetamine, and amphetamine), and PAH associated with connective tissue disease (including systemic sclerosis and systemic lupus erythematosus) and congenital heart disease. Cells from donor lungs which are not used for transplant are also collected and stored.
The cell core currently holds more than 7000 vials of endothelial cells, smooth muscle cells, and adventitial tissue cultured from three regions along the pulmonary vasculature. Cells are harvested from the large pulmonary artery (>5 mm), medium size pulmonary artery (3 mm), and small muscular pulmonary artery (<2 mm). Each vial of cells is highly phenotyped and characterized before distribution for research by qualified researchers.