Molecular biology of primary pulmonary hypertension.

Primary pulmonary hypertension (PPH) is a rare but often fatal condition characterized by pulmonary artery remodeling leading to chronic elevation of pulmonary artery pressure in the absence of causes. The pathophysiology of PPH is not completely understood, but a number of recent studies have elucidated many possible gentic, hormonal, and environmental factors. Current treatment options slow the progression of the disease but do not halt it. The study of molecular mechanisms that result from mutations in onmental and hormonal modifiers holds great promise for the development of novel therapies that may halt the progression of the disease.