A mitochondrial metabolism study of PAH-induced rightventricular fibrosis revealed that increased pyruvate dehydrogenase activityinhibited mitochondrial superoxide dismutase 2 (SOD2) and H2O2production; activated hypoxia-inducible factor-1α (HIF-1α);increased pyruvate dehydrogenase kinase isoforms 1 and 3, TGF-β1 and CTGFexpression; increased CF proliferation and collagen production; promoted fibrosisformation and reduced right ventricular function. Here, SOD2 is linked to pulmonary arterial hypertension.