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Vascular Communication
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Shunt Type
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main portal vein to inferior vena cava
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extrahepatic
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intrahepatic branch of the portal vein and hepatic vein
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intrahepatic
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intrahepatic branch of the portal vein and inferior vena cava
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intrahepatic
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intrahepatic branch of the portal vein and a patent ductus venosus
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intrahepatic
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Clinical features may include:
(1) intrauterine growth retardation
(2) hypergalactosemia not explained by an enzyme deficiency
(3) neonatal cholestasis
(4) hepatic encephalopathy and/or hyperammonemia
Some patients may have concomitant congenital heart disease.
Complications may include:
(1) pulmonary hypertension
(2) mental retardation
(3) disorders of cognitive function
(4) tremors
(5) benign or malignant hepatic tumor
(6) congestive heart failure
(7) hepatopulmonary syndrome
(8) splenomegaly
Imaging studies (color Doppler ultrasound, CT, MRI) should be performed to look for the shunt if the diagnosis is suspected.
