Phenylketonuria (PKU) is the most common disorder of amino acid (AA) metabolism, resulting from severely reduced activity of the liver enzyme phenylalanine hydroxylase (PAH), which leads to abnormal accumulation of the essential amino acid L-phenylalanine (Phe) in the blood plasma (Güttler et al., 1969; Madden, 2004; Regier and Greene, 2017). Here, PAH is linked to pulmonary arterial hypertension.