Liver Transplantation for Extra-Hepatic Manifestation of Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic disorder distinguished by multiple arteriovenous malformations that can affect the liver and lungs, and additionally cause high-output heart failure. Effective medical treatment for HHT-related heart failure is limited. While most types of heart failure are contraindications in liver transplants, HHT-related high-output heart failure is an indication for a liver transplant. However, this is rarely performed as it poses a higher-than-average intraoperative risk. We present a case of a 57-year-old female patient with HHT and high-output heart failure from HHT who underwent a successful orthotopic liver transplant to significantly improve her heart function. Incidentally, the patient had a concomitant diagnosis of primary biliary cholangitis (PBC) from her explanted liver. We review the literature on liver transplants related to HHT and perioperative risks associated with heart failure and pulmonary hypertension that may be associated with both HHT and PBC.