Thrombospondin-1 in pulmonary arterial hypertension: What ails NOS?

摘要

Pulmonary arterial hypertension (PAH) is a debilitating progressive disease with a poor prognosis. The median survival for idiopathic pulmonary hypertension was 2.8 years: advances in specifically targeted therapeutics have improved the prognosis significantly, but mortality remains high. irrespective of the cause of PAH, there is evidence of severe vascular endothelial dysfunction within the pulmonary circulation with characteristic findings of vascular medial hypertrophy and intimal fibrosis, occasionally with luminal narrowing. This leads to increased pulmonary vascular resistance, persistent vasoconstriction, and eventually right ventricular hypertrophy and dysfunction.