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Congenital absence of portal vein presenting as hepatopulmonary syndrome
Author(s) -
Cheung KM,
Lee CY,
Wong CT,
Chan AKH
Publication year - 2005
Publication title -
journal of paediatrics and child health
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.631
H-Index - 76
eISSN - 1440-1754
pISSN - 1034-4810
DOI - 10.1111/j.1440-1754.2005.00542.x
Subject(s) - hepatopulmonary syndrome , medicine , portosystemic shunt , shunt (medical) , splenic vein , right to left shunt , superior mesenteric vein , radiology , abdomen , portal vein , cardiology , surgery , portal hypertension , cirrhosis , patent foramen ovale , percutaneous
A 9‐year‐old girl presented with cyanosis and decreased exercise tolerance. Chest X‐ray, lung function and echocardiogram were normal. Contrasted echocardiogram showed intrapulmonary right to left shunt and computerized tomography of the thorax showed dilated pulmonary vasculature. There was no arteriovenous malformation. Computerized tomography of the abdomen revealed absence of the intrahepatic portion of the portal vein. The superior mesenteric and splenic veins joined as a common trunk, bypassed the liver and drained into the right atrium. We concluded that the patient had hepatopulmonary syndrome secondary to absence of the portal vein. This is the first report of hepatopulmonary syndrome in a female paediatric patient with a congenital absence of the portal vein. As all portal blood entered directly into systemic circulation, the condition was equivalent to congenital portosystemic shunt. Cases of congenital portosystemic shunt complicated by hepatopulmonary syndrome were also reviewed.