PDP1 and pulmonary arterial hypertension: DCA is an investigational drug that was first successfully used in human patients to reduce plasma levels of lactic acid [52] and since then, DCA has been used to treat lactic acidosis, particularly in patients with inherited mitochondrial diseases associated with reduced PDH activity [53,54,55,58], as an antitumour agent [55,59], and in other human pathologies like pulmonary hypertension [56,60] or endometriosis [61,62].