ACE2 overexpression [21] and ACE2 activation by 1-[(2-dimethylamino) ethylamino]-4-(hydroxymethyl)-7-[(4-methylphenyl) sulfonyl oxy]-9H-xanthene-9-one (XNT) [22] ameliorate RV systolic failure, decrease the adverse effects of Ang II, and attenuate pro-inflammatory cytokines in PAH mice. This evidence concerns the gene ACE2 and pulmonary arterial hypertension.