Types of vascular malformations with shunting:
(1) hepatic artery to hepatic vein
(2) hepatic artery to portal vein
(3) portal vein to hepatic vein
Key effects of these vascular malformations:
(1) high output heart failure
(2) fibrosis of the portal triads with portal hypertension
(3) biliary injury and ischemia with elevated alkaline phosphatase
Complications:
(1) secondary sclerosing cholangitis
(2) regenerative nodules
(3) focal nodular hyperplasia (FNH)
(4) encephalopathy
(5) acute hepatic disintegration with hepatic failure
(6) cirrhosis
(7) intrahepatic lithiasis
(8) abdominal angina secondary to vascular steal
(9) hepatocellular carcinoma
In the past repeated transfusion resulted in increased risk for viral hepatitis.
