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Abernethy malformation is a congenital extra-hepatic porto-systemic shunt. This malformation is characterized by an abnormal connection between the portal vein or its branches and one of the systemic veins. Though rare, this anomaly can lead to pulmonary hypertension. Drainage of Abernethy malformation into coronary sinus is extremely rare. We describe a child with Abernethy malformation with unusual drainage into coronary sinus. The abnormal channel was successfully closed by trans-catheter technique with normalisation of pulmonary arterial pressures.

We describe a child with tricuspid atresia associated with a porto-systemic shunt and complete absence of the portal vein, the latter known as the Abernethy malformation. As far as we are aware, this association has not previously been reported. We review the various cardiac defects that have been reported in children with the Abernethy malformation, emphasizing the interesting clinical presentations of this rare malformation of the portal venous system.

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Trans-catheter closure of an unusual type of Abernethy malformation in a child presenting with severe pulmonary hypertension

Cardiac anomalies in the setting of the Abernethy malformation of the portal vein

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2 results

Trans-catheter closure of an unusual type of Abernethy malformation in a child presenting with severe pulmonary hypertension

Cardiac anomalies in the setting of the Abernethy malformation of the portal vein