Figure 1.

Pathogenesis of pulmonary hypertension. PAH is associated with vascular and cardiac remodeling. In PAH, vascular remodeling is characterized by smooth muscle and endothelial cell dysfunction. Indeed, both cell types contribute to muscularization, obstruction, and constriction of distal pulmonary arteries (PAs) which progressively increase the vascular resistance to induce right ventricle (RV) hypertrophy and ultimately RV dysfunction. Representative pictures of the vascular remodeling in pulmonary arterioles have been previously published by Dr Hadri and collaborators (185). PAH, pulmonary arterial hypertension; PASMC, pulmonary artery smooth muscle cells; PAEC, pulmonary artery endothelial cell.