Severe Direct Hyperbilirubinemia as a Consequence of Right Heart Failure in Congenital Heart Disease.

Adult literature documents increased cholestasis in right heart failure yet is poorly documented in the pediatric population. We describe three infants with congenital heart disease who developed significantly elevated direct bilirubin levels of 43, 23, and 12 mg/dL, respectively, in the absence of hepatic dysfunction. The common hemodynamic pathophysiology in these infants is right heart dysfunction with moderate to severe tricuspid regurgitation in the setting of low perfusion state. Right heart dysfunction in infants can result in severe conjugated bilirubin, likely as a consequence of venous congestion and can be used as an indirect marker of right heart dynamics.