title: Severe Direct Hyperbilirubinemia as a Consequence of Right Heart Failure in Congenital Heart Disease.
abstract: 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.
authors.affiliation: 1 Congenital Heart Center, Department of Pediatrics, University of Florida, Shands Children's Hospital, Gainesville, FL, USA.
authors.forename: Joseph
authors.initials: J
authors.lastname: Philip
authors.affiliation: 2 Division of Critical Care, Department of Pediatrics, University of Florida, Shands Children's Hospital, Gainesville, FL, USA.
authors.forename: Ravi S
authors.initials: RS
authors.lastname: Samraj
authors.affiliation: 1 Congenital Heart Center, Department of Pediatrics, University of Florida, Shands Children's Hospital, Gainesville, FL, USA.
authors.forename: Dalia
authors.initials: D
authors.lastname: Lopez-Colon
authors.affiliation: 3 Division of Pediatric Gastroenterology, University of Florida, Shands Children's Hospital, Gainesville, FL, USA.
authors.forename: Regino
authors.initials: R
authors.lastname: Gonzalez-Peralta
authors.affiliation: 1 Congenital Heart Center, Department of Pediatrics, University of Florida, Shands Children's Hospital, Gainesville, FL, USA.
authors.forename: Arun
authors.initials: A
authors.lastname: Chandran
authors.affiliation: 1 Congenital Heart Center, Department of Pediatrics, University of Florida, Shands Children's Hospital, Gainesville, FL, USA.
authors.forename: Mark S
authors.initials: MS
authors.lastname: Bleiwies
mesh_terms: D006330:Heart Defects, Congenital / Q000150:complications / Q000175:diagnosis*; D006333:Heart Failure / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D006932:Hyperbilirubinemia / Q000175:diagnosis / Q000209:etiology*; D007223:Infant; D007231:Infant, Newborn; D014262:Tricuspid Valve Insufficiency / Q000150:complications / Q000175:diagnosis
keywords: CHD; congenital heart surgery; heart failure; hyperbilirubinemia; tricuspid regurgitation