Abstract
Purpose: To describe the angiographic features of hepatic involvement in hereditary hemorrhagic telangiectasia (HHT), particularly the presence of portovenous shunts. Methods: We reviewed the angiographic findings of seven patients with HHT. The patients comprised three women and four men with a mean age of 51 years. Results: Intrahepatic telangiectasias were found in all seven patients and shunts between three vascular channels were found in six of seven patients. In the four patients who had portovenous shunts combined with arterioportal shunts, the portovenous shunts were large. Three patients had no portovenous shunts. Two of these patients had arteriovenous shunts, and one had no shunt. The mean age (69 years) of the patients with portovenous shunts was older than those without portovenous shunts (26 years). Conclusions: Hepatic vascular lesions in HHT are varied, ranging from telangiectasias to large shunts between three vascular channels. In an advanced stage of involvement, large portovenous shunts are present.
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T Bauer P Britton D Lomas DG Wight PJ Friend GJ Alexander (1995) ArticleTitleLiver transplantation for hepatic arteriovenous malformation in hereditary haemorrhagic telangiectasia. J Hepatol 22 586–590
PJ Reilly TT Nostrant (1984) ArticleTitleClinical manifestations of hereditary hemorrhagic telangiectasia. Am J Gastroenterol 79 363–367
T Cooney EC Sweeney R Coll M Greally (1977) ArticleTitlePseudocirrhosis in hereditary haemorrhagic telangiectasia. J Clin Pathol 30 1134–1141
G Bernard F Mion L Henry H Plauchu P Paliard (1993) ArticleTitleHepatic involvement in hereditary hemorrhagic telangiectasia: Clinical, radiological, and hemodynamic studies of 11 cases. Gastroenterology 105 482–487
PL Zentler Munro ER Howard J Karani R Williams (1989) ArticleTitleVariceal haemorrhage in hereditary haemorrhagic telangiectasia. Gut 30 1293–1297
BJ Derauf DW Hunter JW Cardella W Castaneda-Zuniga K Amplatz (1987) ArticleTitlePeripheral embolisation of diffuse hepatic arteriovenous malformations in a patient with hereditary hemorrhagic telangiectasia. Cardiovasc Intervent Radiol 12 236–237
JH Gothlin K Nordgard K Jonsson U Nyman (1982) ArticleTitleHepatic telangiectasia in Osler’s disease treated with arterial embolisation: Report of 2 cases. Eur J Radiol 2 27–30
N Nikolopoulos E Xynos JS Vassilakis (1988) ArticleTitleFamilial occurrence of hyperdynamic circulation status due to intrahepatic fistulae in hereditary hemorrhagic telangiectasia. Hepatogastroenterology 35 167–168
M Caselitz S Wagner A Chavan et al. (1998) ArticleTitleClinical outcome of transfemoral embolisation in patients with arteriovenous malformations of the liver in hereditary haemorrhagic telangiectasia (Weber-Rendu-Osler disease). Gut 42 123–126
D Brohee P Franken M Fievez et al. (1984) ArticleTitleHigh-output right ventricular failure secondary to hepatic arteriovenous microfistulae: Selective arterial embolisation treatment. Arch Intern Med 144 1282–1284
G Garcia-Tsao JR Korzenik L Young et al. (2000) ArticleTitleLiver disease in patients with hereditary hemorrhagic telangiectasia. N Engl J Med 343 931–936
JS Odorico MN Hakim YT Becker et al. (1998) ArticleTitleLiver transplantation as definite therapy for complications after arterial embolization for hepatic manifestations of hereditary hemorrhagic telangiectasia. Liver Transplant Surg 4 483–490
UP Neumann M Knoop JM Langrehr et al. (1998) ArticleTitleEffective therapy for hepatic M. Osler with systemic hypercirculation by ligation of the hepatic artery and subsequent liver transplantation. Transplant Int 22 323–326
E Buscarini L Buscarini C Danesino et al. (1997) ArticleTitleHepatic vascular malformations in hereditary hemorrhagic telangiectasia: Doppler sonographic screening in a large family. J Hepatol 26 111–118
H Naganuma H Ishida M Niizawa K Igarashi T Shioya O Masamune (1995) ArticleTitleHepatic involvement in Osler-Weber-Rendu disease: Findings on pulsed and color Doppler sonography. AJR Am J Roentgenol 165 1421–1425
M Matsuo M Kanematsu H Kato H Kondo K Sugisaki H Hoshi (2001) ArticleTitleOsler-Weber-Rendu disease: Visualizing portovenous shunting with three-dimensional sonography. AJR Am J Roentgenol 176 919–920
JF Trotter PV Suhocki JR Lina LW Martin JL Parrish T Swantkowski (1998) ArticleTitleHereditary hemorrhagic telangiectasia causing high output cardiac failure: Treatment with transcatheter embolization. Am J Gastroenterol 93 1569–1571
N Bourgeois C Delcour J Deviere et al. (1990) ArticleTitleOsler-Weber-Rendu disease associated with hepatic involvement and high output heart failure. J Clin Gastroenterol 12 236–238
J Ludwig CH Kim RH Wiesner RA Krom (1989) ArticleTitleFloxuridine-induced sclerosing cholangitis: An ischemic cholangiopathy? Hepatology 9 215–218
K Suga Y Ishikawa N Matsunaga N Tanaka H Suda T Handa (2000) ArticleTitleLiver involvement in hereditary haemorrhagic telangiectasia: assessment with 99Tcm-phytate radionuclide angiography and 123I-IMP transrectal portal scintigraphy. Br J Radiol 73 1115–1119
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R.I.W. was supported in part by March of Dimes grant HHT-Fy-2002-677.
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Hashimoto, M., Tate, E., Nishii, T. et al. Angiography of Hepatic Vascular Malformations Associated with Hereditary Hemorrhagic Telangiectasia . CVIR 26, 177–180 (2003). https://doi.org/10.1007/s00270-002-1507-Y
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DOI: https://doi.org/10.1007/s00270-002-1507-Y