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Severe Direct Hyperbilirubinemia as a Consequence of Right Heart Failure in Congenital Heart Disease
Joseph Philip1, Ravi S Samraj2, Dalia Lopez-Colon1
11 Congenital Heart Center, Department of Pediatrics, University of Florida, Shands Children's Hospital, Gainesville, FL, USA.
Insights
Pediatric right heart failure can cause severe cholestasis (elevated direct bilirubin) due to venous congestion. This finding highlights right heart dysfunction as a key factor in infant liver issues.
Area of Science:
- Pediatric Cardiology
- Neonatal Hepatology
- Congenital Heart Disease
Background:
- Cholestasis is documented in adult right heart failure but poorly understood in pediatric cases.
- Congenital heart disease (CHD) can lead to complex hemodynamic challenges in infants.
Observation:
- Three infants with CHD presented with significantly elevated direct bilirubin levels (43, 23, 12 mg/dL).
- These infants showed no signs of primary hepatic dysfunction.
Findings:
- The common factor was right heart dysfunction with moderate to severe tricuspid regurgitation and low perfusion.
- Severe conjugated hyperbilirubinemia in these infants is linked to venous congestion from right heart dysfunction.
Implications:
- Right heart dysfunction can serve as an indirect marker for assessing right heart dynamics in infants.
- This study underscores the importance of monitoring liver function in pediatric patients with CHD and right heart issues.
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.
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