Hereditary Hemorrhagic Telangiectasia (HHT) results in a variety of vascular malformations that can lead to significant complications.


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



(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.


To read more or access our algorithms and calculators, please log in or register.