{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T23:36:45Z","timestamp":1790552205981,"version":"4.1.0"},"reference-count":38,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2020,11,21]],"date-time":"2020-11-21T00:00:00Z","timestamp":1605916800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>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\u2019 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.<\/jats:p>","DOI":"10.3390\/jcm9113750","type":"journal-article","created":{"date-parts":[[2020,11,23]],"date-time":"2020-11-23T11:50:34Z","timestamp":1606132234000},"page":"3750","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["The Role of Liver Imaging in Hereditary Hemorrhagic Telangiectasia"],"prefix":"10.3390","volume":"9","author":[{"given":"Joelle","family":"Harwin","sequence":"first","affiliation":[{"name":"Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA 94143, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2154-3990","authenticated-orcid":false,"given":"Mark D.","family":"Sugi","sequence":"additional","affiliation":[{"name":"Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA 94143, USA"},{"name":"Department of Radiology, Zuckerberg San Francisco General Hospital, San Francisco, CA 94110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven W.","family":"Hetts","sequence":"additional","affiliation":[{"name":"Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA 94143, USA"},{"name":"Department of Radiology, Zuckerberg San Francisco General Hospital, San Francisco, CA 94110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miles B.","family":"Conrad","sequence":"additional","affiliation":[{"name":"Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA 94143, USA"},{"name":"Department of Radiology, Zuckerberg San Francisco General Hospital, San Francisco, CA 94110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A.","family":"Ohliger","sequence":"additional","affiliation":[{"name":"Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA 94143, USA"},{"name":"Department of Radiology, Zuckerberg San Francisco General Hospital, San Francisco, CA 94110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,11,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1148\/rg.281075037","article-title":"Abdominal Findings in Hereditary Hemorrhagic Telangiectasia: Pictorial Essay on 2D and 3D Findings with Isotropic Multiphase CT","volume":"28","author":"Siddiki","year":"2008","journal-title":"Radiographics"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"W350","DOI":"10.2214\/AJR.17.18100","article-title":"Primary Hepatic Neoplasms of Vascular Origin: Key Imaging Features and Differential Diagnoses with Radiology-Pathology Correlation","volume":"209","author":"Choi","year":"2017","journal-title":"Am. J. Roentgenol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"66","DOI":"10.3121\/cmr.4.1.66","article-title":"Hereditary Hemorrhagic Telangiectasia, a Vascular Dysplasia Affecting the TGF- Signaling Pathway","volume":"4","author":"Blanco","year":"2006","journal-title":"Clin. Med. Res."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1016\/j.jhep.2015.07.040","article-title":"EASL Clinical Practice Guidelines: Vascular diseases of the liver","volume":"64","author":"Null","year":"2016","journal-title":"J. Hepatol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1016\/S0008-6363(02)00852-0","article-title":"Hereditary hemorrhagic telangiectasia: An update on transforming growth factor \u03b2 signaling in vasculogenesis and angiogenesis","volume":"58","author":"Driesche","year":"2003","journal-title":"Cardiovasc. Res."},{"key":"ref_6","first-page":"830","article-title":"Hereditary Hemorrhagic Telangiectasia","volume":"25","author":"Metze","year":"2009","journal-title":"Encycl. Mol. Mech. Dis."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1136\/jmg.2009.069013","article-title":"International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia","volume":"48","author":"Faughnan","year":"2009","journal-title":"J. Med. Genet."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"860","DOI":"10.1038\/ejhg.2009.35","article-title":"Hereditary haemorrhagic telangiectasia: A clinical and scientific review","volume":"17","author":"Govani","year":"2009","journal-title":"Eur. J. Hum. Genet."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1002\/(SICI)1096-8628(20000306)91:1<66::AID-AJMG12>3.0.CO;2-P","article-title":"Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)","volume":"91","author":"Shovlin","year":"2000","journal-title":"Am. J. Med. Genet."},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"Faughnan, M.E., Mager, J.J., Hetts, S.W., Palda, V.A., Lang-Robertson, K., Buscarini, E., Deslandres, E., Kasthuri, R.S., Lausman, A., and Poetker, D. (2020). Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia. Ann. Intern. Med.","DOI":"10.7326\/L21-0067"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2166","DOI":"10.1007\/s10620-011-1585-2","article-title":"Natural History and Outcome of Hepatic Vascular Malformations in a Large Cohort of Patients with Hereditary Hemorrhagic Teleangiectasia","volume":"56","author":"Buscarini","year":"2011","journal-title":"Dig. Dis. Sci."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1016\/j.jhep.2006.12.008","article-title":"Liver involvement in hereditary hemorrhagic telangiectasia (HHT)","volume":"46","year":"2007","journal-title":"J. Hepatol."},{"key":"ref_13","unstructured":"Vernon, H., Wehrle, C., and Kasai, A. (2020). Anatomy, Abdomen and Pelvis, Liver (updated 31 July 2020). StatPearls [Internet], StatPearls Publishing."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1055\/s-2004-813549","article-title":"Doppler Ultrasonographic Grading of Hepatic Vascular Malformations in Hereditary Hemorrhagic Telangiectasia\u2014Results of Extensive Screening","volume":"25","author":"Buscarini","year":"2004","journal-title":"Ultraschall der Med. Eur. J. Ultrasound"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1007\/s10396-020-01022-w","article-title":"Hereditary hemorrhagic telangiectasia: How to efficiently detect hepatic abnormalities using ultrasonography","volume":"47","author":"Naganuma","year":"2020","journal-title":"J. Med. Ultrason."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"931","DOI":"10.1056\/NEJM200009283431305","article-title":"Liver Disease in Patients with Hereditary Hemorrhagic Telangiectasia","volume":"343","author":"Korzenik","year":"2000","journal-title":"N. Engl. J. Med."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1346","DOI":"10.1093\/ndt\/11.7.1346","article-title":"Symptomatic intrarenal arteriovenous fistula detected 25 years after percutaneous renal biopsy","volume":"11","author":"Alcazar","year":"1996","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1016\/j.jacc.2016.05.043","article-title":"High-Output Heart Failure","volume":"68","author":"Reddy","year":"2016","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s12876-020-01311-1","article-title":"Liver cirrhosis in a patient with hepatic hereditary hemorrhagic telangiectasia and Budd\u2013Chiari syndrome: A case report","volume":"20","author":"Xu","year":"2020","journal-title":"BMC Gastroenterol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1920","DOI":"10.1007\/s00261-018-1671-4","article-title":"Liver involvement in hereditary hemorrhagic telangiectasia","volume":"43","author":"Buscarini","year":"2018","journal-title":"Abdom. Radiol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"2384","DOI":"10.1007\/s00261-019-01976-7","article-title":"Abdominal manifestations of hereditary hemorrhagic telangiectasia: A series of 333 patients over 15 years","volume":"44","author":"Welle","year":"2019","journal-title":"Abdom. Radiol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"486","DOI":"10.3748\/wjg.v23.i3.486","article-title":"Macro- and microcirculation patterns of intrahepatic blood flow changes in patients with hereditary hemorrhagic telangiectasia","volume":"23","author":"Schelker","year":"2017","journal-title":"World J. Gastroenterol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"E8","DOI":"10.3171\/2019.9.FOCUS19624","article-title":"The role of contrast-enhanced ultrasound in neurosurgical disease","volume":"47","author":"Kearns","year":"2019","journal-title":"Neurosurg. Focus"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1186\/2040-2384-2-25","article-title":"Contrast-enhanced sonography as a novel tool for assessment of vascular malformations","volume":"2","author":"Oe","year":"2010","journal-title":"J. Angiogenes. Res."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"283","DOI":"10.3233\/CH-151962","article-title":"Perfusion quantification of vascular malformations using contrast-enhanced ultrasound (CEUS) with time intensity curve analysis before and after treatment: First results","volume":"62","author":"Wiesinger","year":"2016","journal-title":"Clin. Hemorheol. Microcirc."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1007\/s00261-017-1392-0","article-title":"Contrast-enhanced ultrasound of the liver: Technical and lexicon recommendations from the ACR CEUS LI-RADS working group","volume":"43","author":"Lyshchik","year":"2018","journal-title":"Abdom. Radiol."},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Schelker, R.C., Andorfer, K., Putz, F., Herr, W., and Jung, E.-M. (2019). Identification of two distinct hereditary hemorrhagic telangiectasia patient subsets with different hepatic perfusion properties by combination of contrast-enhanced ultrasound (CEUS) with perfusion imaging quantification. PLoS ONE, 14.","DOI":"10.1371\/journal.pone.0215178"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1007\/s11547-011-0688-3","article-title":"Dynamic 4D MR angiography versus multislice CT angiography in the evaluation of vascular hepatic involvement in hereditary haemorrhagic telangiectasia","volume":"117","author":"Scardapane","year":"2011","journal-title":"La Radiol. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"W399","DOI":"10.2214\/AJR.05.1068","article-title":"Liver Involvement in Hereditary Hemorrhagic Telangiectasia: CT and Clinical Findings Do Not Correlate in Symptomatic Patients","volume":"187","author":"Wu","year":"2006","journal-title":"Am. J. Roentgenol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1002\/ccr3.141","article-title":"Putting a new spin: MRI monitoring of hepatic artery and portal vein flow for response to bevacizumab in hereditary hemorrhagic telangiectasia","volume":"3","author":"Kutaiba","year":"2014","journal-title":"Clin. Case Rep."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"e20193206","DOI":"10.1542\/peds.2019-3206","article-title":"Monitoring Arteriovenous Malformation Response to Genotype-Targeted Therapy","volume":"146","author":"Edwards","year":"2020","journal-title":"Pediatrics"},{"key":"ref_32","first-page":"948","article-title":"Bevacizumab in Patients With Hereditary Hemorrhagic Telangiectasia and Severe Hepatic Vascular Malformations and High Cardiac Output","volume":"307","author":"Ginon","year":"2012","journal-title":"JAMA"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s13023-018-0982-4","article-title":"Safety of thalidomide and bevacizumab in patients with hereditary hemorrhagic telangiectasia","volume":"14","author":"Buscarini","year":"2019","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1007\/s10456-018-9646-1","article-title":"Pazopanib may reduce bleeding in hereditary hemorrhagic telangiectasia","volume":"22","author":"Faughnan","year":"2019","journal-title":"Angiogenesis"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"951","DOI":"10.1007\/s00330-012-2694-9","article-title":"Complications and clinical outcome of hepatic artery embolisation in patients with hereditary haemorrhagic telangiectasia","volume":"23","author":"Chavan","year":"2013","journal-title":"Eur. Radiol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1134","DOI":"10.1136\/jcp.30.12.1134","article-title":"\u2018Pseudocirrhosis\u2019 in hereditary haemorrhagic telangiectasia","volume":"30","author":"Cooney","year":"1977","journal-title":"J. Clin. Pathol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1089","DOI":"10.1016\/j.ultrasmedbio.2004.08.004","article-title":"High prevalence of hepatic focal nodular hyperplasia in subjects with hereditary hemorrhagic telangiectasia","volume":"30","author":"Buscarini","year":"2004","journal-title":"Ultrasound Med. Biol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s11547-012-0789-3","article-title":"Hepatic nodular regenerative lesions in patients with hereditary haemorrhagic telangiectasia: Computed tomography and magnetic resonance findings","volume":"118","author":"Scardapane","year":"2012","journal-title":"La Radiol. Med."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/9\/11\/3750\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T10:35:21Z","timestamp":1760178921000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/9\/11\/3750"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,11,21]]},"references-count":38,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2020,11]]}},"alternative-id":["jcm9113750"],"URL":"https:\/\/doi.org\/10.3390\/jcm9113750","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,11,21]]}}}