Shirali et al. [28] described much lower values of Ang-2 in six Fontan patients under normal conditions and could not find a relationship between ANG2 and pulmonary arteriovenous malformations (PAVMs), suggesting that the role ANG2 plays in Fontan physiology is likely unrelated to PAVM formation. This evidence concerns the gene ANGPT2 and Pulmonary arteriovenous malformation.