Complications and clinical outcome of hepatic artery embolisation in patients with hereditary haemorrhagic telangiectasia
Hepatic artery embolisation (HAE) in patients with hereditary haemorrhagic telangiectasia (HHT) is controversial because of the associated complications and unproven long-term benefit. We present our results in 20 such patients over a time span of 17 years.
Staged HAE was performed using polyvinyl alcohol (PVA) particles and coils. Complications, clinical symptoms and cardiac output were assessed before and after therapy as well as at the end of follow-up (median 92 months, range 26–208 months).
Two patients died within 30 days following HAE (10 %). Four further deaths resulted from causes unrelated to HAE. Ischaemic cholangitis, cholecystitis and focal hepatic necrosis with biliary sepsis necessitated re-intervention in four patients. In all but one patient, clinical symptoms resolved with mean cardiac output falling from 11.84 ± 3.22 l/min pre-treatment to 8.13 ± 2.67 l/min at the end of follow-up (P < 0.001). One patient required liver transplantation for de novo symptoms of portal hypertension 4 years after primary symptoms had been cured by HAE.
The 30-day mortality of HAE in patients with HHT is 10 %. The rate of complications requiring re-intervention is 20 %. Clinical response at long-term follow-up is satisfactory.
• Hepatic artery embolisation (HAE) in hereditary haemorrhagic telangiectasia (HHT) provides long-term benefit.
• Mortalities of HAE and liver transplantation in HHT patients are comparable.
• In HHT, complications of HAE are lower than those of liver transplantation.
• Complications of HAE can be further reduced by refinement of technique.
• Complications include ischaemic cholangitis, hepatic necrosis, biliary sepsis and death.
- Garcia-Tsao (2007) Liver involvement in hereditary hemorrhagic telangiectasia (HHT). J Hepatol 46:499–507 CrossRef
- Buscarini E, Leandro G, Conte D, Danesino C, Daina E, Manfredi G et al (2011) Natural history and outcome of hepatic vascular malformations in a large cohort of patients with hereditary hemorrhagic teleangiectasia. Dig Dis Sci 56:2166–2178 CrossRef
- Buscarini E, Plauchu H, Garcia Tsao G et al (2006) Liver involvement in hereditary hemorrhagic telangiectasia: consensus recommendations. Liver Int 26:1040–1046 CrossRef
- Govani FS, Shovlin CL (2009) Hereditary hemorrhagic telangiectasia:a clinical and scientific review. Eur J Hum Genet 17:860–871 CrossRef
- Faughnan ME, Palda VA, Garcia-Tsao G, et al. (2009) International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia. J Med Genet. doi:10.1136/jmg.2009.069013
- Odorico JS, Hakim NM, Becker YT et al (1998) Liver transplantation as definitive therapy for complications after arterial embolization for hepatic manifestations of hereditary hemorrhagic telangiectasia. Liver Transplant Surg 4:483–490 CrossRef
- Whiting JH Jr, Korzenik JR, Miller FJ Jr et al (2000) Fatal outcome after embolotherapy for hepatic arteriovenous malformations of the liver in two patients with hereditary hemorrhagic telangiectasia. J Vasc Interv Radiol 11:855–858 CrossRef
- Shovlin C, Guttmacher A, Buscarini E, Faughnan M, Hyland R, Westermann C et al (2000) Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet 91:66–67 CrossRef
- Chavan A, Galanski M, Wagner S, Caselitz M, Schlitt HJ, Gratz KF et al (1998) Hereditary hemorrhagic telangiectasia: effective protocol for embolization of hepatic vascular malformations—Experience in five patients. Radiology 209:735–739
- Chavan A, Caselitz M, Gratz K-F et al (2004) Hepatic artery embolization for treatment of patients with hereditary hemorrhagic telangiectasia and symptomatic hepatic vascular malformations. Eur Radiol 14:2079–2085 CrossRef
- Lerut J, Orlando G, Adam R et al (2006) Liver transplantation for hereditary hemorrhagic telangiectasia. Report of the European liver transplant registry. Ann Surg 244:854–864 CrossRef
- Azoulay D, Precetti S, Emile JF et al (2002) Liver transplantation for intrahepatic Rendu-Osler-Weber’s disease: the Paul Brousse hospital experience. Gastroenterol Clin Biol 26:828
- Dupuis-Girod S, Chesnais A-L, Ginon I et al (2010) Long-term outcome of patients with hereditary hemorrhagic telangiectasia and severe hepatic involvement after orthotopic liver transplantation: a single-center study. Liver Transpl 16:340–347
- Garcia-Tsao G, Korzenik JR, Young L et al (2000) Liver disease in patients with hereditary hemorrhagic telangiectasia. N Engl J Med 343:931–936 CrossRef
- Pfitzmann R, Heise M, Langrehr JM et al (2001) Liver transplantation for treatment of intrahepatic Osler’s disease: first experiences. Transplantation 72:237–241 CrossRef
- Flieger D, Hainke S, Fischbach W (2006) Dramatic improvement in hereditary hemorrhagic telangiectasia after treatment with vascular endothelial growth factor (VEGF) antagonist bevacizumab. Ann Hematol 85:631–632 CrossRef
- Mitchell A, Adams LA, Macquillan G, Tibballs J, van den Driesen R, Delriviere L (2008) Bevacizumab reverses need for liver transplantation in hereditary hemorrhagic telangiectasia. Liver Transpl 14:210–213 CrossRef
- Dupuis-Girod S, Ginon I, Saurin J-C et al (2012) Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output. JAMA 307:948–955 CrossRef
- Complications and clinical outcome of hepatic artery embolisation in patients with hereditary haemorrhagic telangiectasia
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Volume 23, Issue 4 , pp 951-957
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- Arterio-venous malformations
- Cardiac failure
- Biliary disease
- Liver transplantation
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- Author Affiliations
- 1. Department of Diagnostic and Interventional Radiology, Klinikum Oldenburg, Rahel Straus Strasse 10, 26133, Oldenburg, Germany
- 2. Department of Gastroenterology and Hepatology, Hannover Medical School, Hannover, Germany
- 3. Department of Abdominal and Transplantation Surgery, Hannover Medical School, Hannover, Germany
- 4. Department of Gastroenterology, Hepatology and Diabetology, Klinikum Oldenburg, Oldenburg, Germany
- 5. Department of General and Visceral Surgery, Klinikum Oldenburg, Oldenburg, Germany
- 6. Department of Radiology, Hannover Medical School, Hannover, Germany