Pulmonary arterial hypertension (PAH) has been frequently compared with a type of malignant disease and has an incredible number of pathogenic mechanisms similar to cancer (1–3). A cancer model for PAH was first proposed in 1998 by Voelkel and colleagues (4) and had been confirmed and expanded by later studies from many research groups. A critical role for a cancer-like metabolic shift from oxidative phosphorylation to glycolysis despite adequate oxygen supply (the Warburg effect) in PAH pathogenesis had been demonstrated; multiple cancer-shared abnormalities in mitochondrial metabolism and dynamics have been reported as key modulators of pathogenic changes in PAH pulmonary vasculature and right ventricle (RV), including mitochondrial hyperpolarization, altered activity of mitochondrial pyruvate dehydrogenase, superoxide dismutase 2 deficiency, fragmentation and/or hyperpolarization of the mitochondrial reticulum, and dysregulated mitochondrial dynamics due to down-regulation of the fusion protein mitofusin 2 and upregulation of the fission protein dynaminrelated protein 1. The benefits of mitochondria-targeting strategies (ie, pyruvate dehydrogenase kinase inhibitor, dichloroacetate) have been demonstrated for both experimental pulmonary hypertension (PH) and human cancer, suggesting applicability of cancertargeting therapies to human PAH (5–8). Another line of evidence supporting similarities between PAH and cancer came from the microRNA (miR) field. Dysregulation of numerous microRNAs, reported in human cancers, appeared to play an important role in multiple features of PAH pathogenesis, including pulmonary vascular remodeling, inflammation, impaired angiogenesis, and RV hypertrophy (reviewed in depth by Courboulin and colleagues [9]).