An 11-year-old boy with congenital hepatic fibrosis presented with cyanosis at the National University Hospital. Echocardiogram revealed a structurally normal heart with good ventricular function. A pulmonary cause of his cyanosis was suggested on macroaggregated albumin scan and selective pulmonary artery angiogram. Arterial hypoxaemia secondary to intrapulmonary arteriovenous shunting in chronic liver cirrhosis can lead to permanent cyanosis. The potential for a complete reversal of this condition after liver transplantation indicates that arterial hypoxaemia, rather than being a contraindication, should be a reason for early liver transplantation.
Children with chronic liver disorders may present with dysfunction of other organ systems. Encephalopathy, gastrointestinal bleeding, failure to thrive and impaired renal function are some such findings in these children1 Mild to moderate hypoxaemia is also well described in children with chronic liver disease.
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