In this paper we demonstrated that endothelin-1 (ET1) is regulated in important ways in infants with SVHD compared to healthy controls. We also showed an association between failure to suppress ET1 level before and after Stage 2 palliation and multiple adverse clinical outcomes related to pulmonary hypertension including low oxygen saturations, need for targeted PH medication, and need for invasive cardiac catheterization within 3 months of surgery. This association remained significant in multivariable analysis controlling for several clinical variables.