IMAGE | LIVER Hereditary Hemorrhagic Telangiectasia With Liver Vascular Malformation Presenting With High-Output Heart Failure

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disease manifested by formation of tel-angiectasias and visceral vascular malformations of organ systems, including the skin, lungs, gastrointestinal tract, brain, and liver. Hepatic involvement may lead to portal hypertension, high-output cardiac failure, and biliary strictures.1 Diag-nosis requires patients to have 3 of 4 clinical characteristics: epistaxis, telangiectasia on the skin, visceral lesions, and a first-degree relative with HHT.2 Management involves controlling the source of bleeding by packing the nasopharynx, endoscopic therapies, or pulmonary or hepatic embolization.3 A 48-year-old female with HHT diagnosed at age 21 years presented with worsening shortness of breath and lower ex-tremity edema over a 6-month period. She had heart failure with high cardiac output of 8.2 L/min. Prior chest and brain CT showed no AV malformations and she had no previous history of upper or lower gastrointestinal bleeds. Abdominal computed tomography showed markedly dilated portal vein, hepatic vein, and inferior vena cava with heterogeneous enlarged liver and innumerable hyperdense nodules (Figure 1). Vascular ultrasound of the liver showed high velocity and low resistive indices of the hepatic arteries with multiple hepatic intraparenchymal shunts. The patient develope