Evolution of Intrahepatic Shunts in a Patient With Hereditary Hemorrhagic Telangiectasia
Authors: Leila Haghighat, Eric J. Brandt, Deborah D. Proctor, Guadalupe Garcia-Tsao, Jeffrey Pollak, Lawrence H. Young
Journal: Annals of Internal Medicine (2018)

Letters2 October 2018Evolution of Intrahepatic Shunts in a Patient With Hereditary Hemorrhagic TelangiectasiaLeila Haghighat, MD, MPhil, Eric J. Brandt, MD, Deborah D. Proctor, MD, Guadalupe Garcia-Tsao, MD, Jeffrey Pollak, MD, and Lawrence H. Young, MDLeila Haghighat, MD, MPhilYale New Haven Hospital, New Haven, Connecticut (L.H., E.J.B., D.D.P., G.G., J.P., L.H.Y.), Eric J. Brandt, MDYale New Haven Hospital, New Haven, Connecticut (L.H., E.J.B., D.D.P., G.G., J.P., L.H.Y.), Deborah D. Proctor, MDYale New Haven Hospital, New Haven, Connecticut (L.H., E.J.B., D.D.P., G.G., J.P., L.H.Y.), Guadalupe Garcia-Tsao, MDYale New Haven Hospital, New Haven, Connecticut (L.H., E.J.B., D.D.P., G.G., J.P., L.H.Y.), Jeffrey Pollak, MDYale New Haven Hospital, New Haven, Connecticut (L.H., E.J.B., D.D.P., G.G., J.P., L.H.Y.), and Lawrence H. Young, MDYale New Haven Hospital, New Haven, Connecticut (L.H., E.J.B., D.D.P., G.G., J.P., L.H.Y.)Author, Article, and Disclosure Informationhttps://doi.org/10.7326/L18-0036 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Hereditary hemorrhagic telangiectasia (previously Osler–Weber–Rendu syndrome) is an autosomal dominant disease characterized by telangiectasias in the skin and mucous membranes and arteriovenous malformations in the lungs, brain, gastrointestinal tract, and liver (1–5). The prevalence of this disease is 1 in 5000 persons. Up to 78% of affected patients have liver vascular malformations, but only 8% are symptomatic. Patients with such malformations have 3 types of intrahepatic shunts: hepatic artery–hepatic vein, hepatic artery–portal vein, and portal vein–hepatic vein (Figure).Figure.The 3 possible intrahepatic shunts in hereditary hemorrhagic telangiectasia.HA = hepatic artery; HV = hepatic vein; PV = portal vein.Figure. The ...References1. Garcia-Tsao G, Korzenik JR, Young L, Henderson KJ, Jain D, Byrd B, et al. Liver disease in patients with hereditary hemorrhagic telangiectasia. N Engl J Med. 2000;343:931-6. [PMID: 11006369] CrossrefMedlineGoogle Scholar2. Buscarini E, Plauchu H, Garcia Tsao G, White RI, Sabbà C, Miller F, et al. Liver involvement in hereditary hemorrhagic telangiectasia: consensus recommendations. Liver Int. 2006;26:1040-6. [PMID: 17032403] MedlineGoogle Scholar3. Shovlin CL, Guttmacher AE, Buscarini E, Faughnan ME, Hyland RH, Westermann CJ, et al. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet. 2000;91:66-7. [PMID: 10751092] CrossrefMedlineGoogle Scholar4. Trotter JF, Suhocki PV, Lina JR, Martin LW, Parrish JL, Swantkowski T. Hereditary hemorrhagic telangiectasia causing high output cardiac failure: treatment with transcatheter embolization. Am J Gastroenterol. 1998;93:1569-71. [PMID: 9732949] CrossrefMedlineGoogle Scholar5. Bauer T, Britton P, Lomas D, Wight DG, Friend PJ, Alexander GJ. Liver transplantation for hepatic arteriovenous malformation in hereditary haemorrhagic telangiectasia. J Hepatol. 1995;22:586-90. [PMID: 7650340] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: Yale New Haven Hospital, New Haven, Connecticut (L.H., E.J.B., D.D.P., G.G., J.P., L.H.Y.)Disclaimer: All authors had access to patient records, take responsibility for the accuracy of the analysis, and had authority over manuscript preparation and the decision to submit the manuscript for publication.Funding Source: None.Disclosures: Disclosures can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=L18-0036.This article was published at Annals.org on 5 June 2018. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited bySubaortic Membranes in Patients With Hereditary Hemorrhagic Telangiectasia and Liver Vascular MalformationsACG Clinical Guideline: Disorders of the Hepatic and Mesenteric Circulation 2 October 2018Volume 169, Issue 7Page: 508-509KeywordsCatheterizationDiureticsEstrogensHeartHeart failureHemorrhageLiverLungsOxygenVeins ePublished: 5 June 2018 Issue Published: 2 October 2018 Copyright & PermissionsCopyright © 2018 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...