Direct hyperbilirubinemia in infants with congenital heart disease

Background: The association between congenital heart disease (CHD) and infantile cholestasis, a key finding for the diagnosis of biliary atresia (BA), has not been previously investigated. The aim of this study was therefore to investigate the characteristics of direct hyperbilirubinemia (D-HB) in infants with CHD.

Methods: All neonates admitted to the present hospital and diagnosed with CHD in 2015 and 2016 were included. D-HB (direct bilirubin ≥ 2.0 mg/dL) at ≤60 days of age and other clinical parameters were retrospectively reviewed. Statistical analysis according to presence of D-HB was performed using chi-squared test or Wilcoxon rank sum test.

Results: Seventy-six patients (M:F, 36:40) were included in this study. CHD consisted of ventricular septal defect in 17, patent ductus arteriosus in 10, and other in 49. Thirteen patients (17.1%) had D-HB at ≤60 days of age. Resolution of D-HB (DB < 2.0 mg/dL) occurred in 10 of the 13 patients during the hospital stay, and this occurred in ≤7 days in eight of the 10 patients. Sex, gestational age, birthweight, chromosomal anomalies, need for Fontan operation for CHD repair, and/or cardiac operation were not associated with D-HB at ≤60 days of age.

Conclusion: While D-HB was frequently observed in infants with CHD, the majority of D-HB cases resolved spontaneously in ≤1 week. Neonatal clinical parameters or CHD status was not predictive of D-HB. D-HB lasting >1 week in infants with CHD should be evaluated for the cause.