This pseudo neoplastic phenotype of PAH relates to several acquired, epigenetically regulated mitochondrial abnormalities including a) an upregulation of enzymes that promote Warburg metabolism (pyruvate dehydrogenase kinase (PDK) and pyruvate kinase muscle isoform 2 (PKM2) and b) dysregulation of large GTPases that regulate mitochondrial dynamics, namely an increase in mitochondrial fission due to activation of dynamin-related protein 1 (DRP1) and an inhibition of fusion, related to downregulation of mitofusin 2 (MFN2). Here, PKM is linked to pulmonary arterial hypertension.