Arteriovenous Intrahepatic Shunting in Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is a vascular disorder characterized by the development of visceral arteriovenous malformations (AVMs), which result in recurrent episodes of epistaxis, mucosal bleeding, and iron deficiency anemia. Hepatic AVMs, when untreated, may lead to high-output cardiac failure, pulmonary hypertension, and hepatic cirrhosis. While not routinely done, a shunt run performed during right heart catheterization (RHC) can provide valuable physiologic data to guide the diagnostic evaluation of patients in complex cases of suspected pulmonary hypertension. We present a case in which an abnormal shunt run during RHC revealed intrahepatic shunting and ultimately led to the diagnosis of HHT.