An 8 day old girl with infradiaphragmatic total anomalous pulmonary venous connection presented with severe unconjugated hyperbilirubinaemia; exchange transfusion resulted in haemodynamic deterioration. Hyperbilirubinaemia is a rare complication of this condition, however exchange transfusion should be avoided. The diagnosis can be made on the neonatal unit by ultrasound demonstration of dilated hepatic portal veins.
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