The Role of Liver Imaging in Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by spontaneous epistaxis, telangiectasia, and visceral vascular malformations. Hepatic vascular malformations are common, though a minority are symptomatic. Symptoms are dependent on the severity and...

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Bibliographic Details
Published in:Journal of clinical medicine 2020-11, Vol.9 (11), p.3750
Main Authors: Harwin, Joelle, Sugi, Mark D., Hetts, Steven W., Conrad, Miles B., Ohliger, Michael A.
Format: Article
Language:English
Subjects:
Ascites
Clinical medicine
Epistaxis
Flow velocity
Heart
Hypertension
Intubation
Kinases
Liver
Magnetic resonance imaging
Monoclonal antibodies
Mutation
Pulmonary arteries
Review
Veins & arteries
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Summary:Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by spontaneous epistaxis, telangiectasia, and visceral vascular malformations. Hepatic vascular malformations are common, though a minority are symptomatic. Symptoms are dependent on the severity and exact type of shunting caused by the hepatic malformation: Arteriosystemic shunting leads to manifestations of high output cardiac failure, and arterioportal shunting leads to portal hypertension. Radiologic imaging, including ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), is an important tool for assessing liver involvement. Doppler ultrasonography is the first-line screening modality for HHT-related liver disease, and it has a standardized scale. Imaging can determine whether shunting is principally to the hepatic vein or the portal vein, which can be a key determinant of patients’ symptoms. Liver-related complications can be detected, including manifestations of portal hypertension, focal liver masses as well as ischemic cholangiopathy. Ultrasound and MRI also have the ability to quantify blood flow through the liver, which in the future may be used to determine prognosis and direct antiangiogenic therapy.
ISSN:2077-0383
2077-0383
DOI:10.3390/jcm9113750