The pathology of pulmonary artery hypertension.

The pathologic features of primary pulmonary hypertension (PPH) are well known but its pathogenesis remains uncertain. In the first section, this chapter outlines the characteristic structural changes of PPH. The second section deals with the pathogenesis of these changes drawing on animal models of chronic pulmonary hypertension. The third section deals with phenotypic alterations in cells from the wall of the pulmonary artery of hypertensive animals. The recent identification of a germlike mutation in the BMPR2 gene in patients with PPH provides a novel opportunity to further our understanding of the pathogenesis of this disease.