Pulmonary arterial hypertension (PAH) is a fatal and rapidly progressive disease that ultimately leads to right ventricular (RV) failure and death. RV function is the major determinant of prognosis in PAH, but no effective therapy directly targets the RV in PAH. One important fact is that there is a sex paradox in the epidemiology of PAH: although PAH occurs 2-4 folds more frequently in women than men, women with PAH have better RV function and thus survival than men. Better RV function in female PAH patients is associated with less RV and insertion point fibrosis. However, the sex-dependent RV and insertion point fibrosis in PAH is not well studied. Such sex difference is thought to be linked to sex hormones, especially estrogen.

This project will investigate the underlying mechanism for the sex-dependent RV fibrosis in PAH with the goal of identifying promising therapeutic targets and developing treatment strategies for PAH.