HEREDITARY HEMORRHAGIC TELANGIECTASIA AND SECONDARY BILIARY-CIRRHOSIS

We report the case of a 65-year-old woman with a 10-year history of bi liary disease and a 3-year history of anaemia, lethargy, angina and re current ascending cholangitis; she had hereditary haemorrhagic telangi ectasia (HHT) with aneurysms in the splanchnic circulation, and was as sumed to have secondary biliary cirrhosis with portal hypertension and hypertrophic obstructive cardiomyopathy. Her portal hypertension was caused by secondary biliary cirrhosis induced by intrahepatic gallston es; this was associated with left ventricular hypertrophy and a high c ardiac output, caused by increased splanchnic circulation, and with an eurysms in the superior mesenteric and pancreaticoduodenal arteries, w hich were visualized by angiography. We conclude that HHT can produce abnormal abdominal vascular anatomy, which affects haemodynamic perfor mance and makes it difficult to manage a patient with portal hypertens ion caused by secondary biliary cirrhosis. HHT has been shown to be as sociated with intrahepatic biliary stones and might have a causal role . When assessing patients with this type of condition for liver transp lantation, particular attention should be paid to haemodynamic and cir culatory abnormalities.