BCKDHB and hydrops fetalis: ISO/PE perfusion led to more pronounced transcriptional alterations in metabolism by downregulating genes coding for enzymes involved in branched amino acid catabolism (Bckdhb), oxidative energy generation i.e. enzymes of the tricarboxylic acid cycle (Pdh, Cs, Csl), and mitochondrial respiratory chain (Sdh, Nudf, Cox, Uqcrc1), and hence to a profile rather associated with progressive HF than compensation [19, 33].