The present study evaluated whether the tetrameric form of pyruvate kinase muscle isoform 2 (PKM2) regulates PAEC mitochondrial metabolism through peroxisome proliferator-activated receptor gamma coactivator 1 alpha (PGC-1alpha) and mitochondrial transcription factor A (mtTFA), thereby influencing arterial intimal remodeling in CTEPH. This evidence concerns the gene PPARGC1A and chronic thromboembolic pulmonary hypertension.