Review Article International Journal of Basic & Applied Physiology

Pulmonary arterial hypertension (PAH) is a multifactorial life-threatening disease, characterized by high pulmonary artery pressure, with an ultimate right heart failure. Without treatment, death occurs within 3 years of diagnosis. PAH is characterized by abnormal remodelling of small, peripheral resistance vessels in the lung involving proliferation and migration of vascular smooth muscle, endothelial cell and fibroblasts. Current therapeutic approaches in controlling PAH largely depend on symptomatic relief while the prognosis rate is lower due to the lack of specific molecular targets and the involvement of several factors in the development of PAH. Several studies have indicated direct involvement of matrix metalloproteinase (MMPs) during development and disease progression and associated matrix remodelling in vasculature. PAH can be present in an idiopathic form, usually called idiopathic pulmonary arterial hypertension (IPAH), viral infections, portal hypertension with or without cirrhosis, and anorectic drug ingestion. Increased MMP activity has been demonstrated in experimental animal models of PAH, and MMP inhibition has been shown to either attenuate or enhance vascular remodelling. In the present article recent advancement in the areas of MMPs in progressive PAH has been reviewed with possible therapeutic intervention for MMP inhibition. The present review also addresses the impact of therapeutic strategies on achieving possible PAH reversals and scopes for future research.