Localized biliary ischemia in patients with hepatic arteriovenous malformations, a newly recognized syndrome occurring in Hereditary Hemorrhagic Telangiectasia Diagnosis and management

by Khoury, Rasha

The clinical manifestations of hepatic arteriovenous malformations (HAVMs) were elucidated. A review of the literature was undertaken to better understand how HAVMs specifically affect the biliary system. A retrospective review of the 50 patients with HAVMs seen at the Yale University HHT Center was done, including clinical manifestation, intervention and outcome analysis. Of 50 adults with HAVM, median age was 64 (range 17-73) and 84% were female. Initially 74% were classified as Type 1, symptomatic heart failure, 16% as Type 2, portal hypertension, and 10% as Type 3, biliary abnormalities. In Type 1 conversion to Type 3 was associated with the highest mortality and in Type 3 invasive procedures precipitated rapid decline and need for transplant +/- death. Of the Type 3s, case reports of two sisters with localized biliary ischemia were presented. To our knowledge this is the first description of localized biliary HHT involvement, diagnosis and management.