Hepatic Arteriovenous Malformations from Hereditary Hemorrhagic Telangiectasia Presenting as High-Output Cardiac Failure with Biliary Necrosis

Abstract

Purpose: Case: A 47-year-old man presented with worsening dyspnea on exertion, unremitting epigastric pain, jaundice, and fevers. He denied any epistaxis, hemoptysis, gingival or gastrointestinal bleeding. Exam showed icteric sclerae, a loud systolic ejection murmur over the apex, a loud bruit over a tender epigastrium, and hepatosplenomegaly. Initial labs showed total bilirubin 3.7 mg/dL, AST 43 U/L, ALT 55 U/L, alkaline phosphatase 579 U/L, and INR 1.1. Blood cultures grew Streptococcus anginosus. Abdominal CT showed diffuse angiomatous tissue, early opacification of all hepatic veins, and biliary necrosis in segment 7. Aortogram showed hugely dilated celiac, replaced left and common hepatic arteries. Cardiac output was 18.2 L/min. Patient was positive for ACVRL1 gene mutation. Endoscopies and additional imaging did not show any other telangiectasias. Despite 4 weeks of IV antibiotics, patient had recurrent fevers, worsening pain, and increasing jaundice. Repeat labs now showed total bilirubin 21, AST 129, ALT 104, ALKP 738, and blood cultures again grew Streptococcus anginosus. Repeat abdominal CT showed progressive biliary necrosis now affecting segments 2-4. Urgent liver transplantation was successfully performed, with resolution of patient's heart failure symptoms and abdominal pain. Discussion: Hereditary hemorrhagic telangiectasia, or Osler-Weber-Rendu syndrome, with hepatic involvement is rare. Diffuse hepatic arteriovenous shunting can result in high-output heart failure, as well as progressive biliary ischemia with necrosis and sepsis. Epigastric pain can occur due to enteric arterial “steal” syndrome. Urgent liver transplantation is indicated and therapeutic for cardiac failure and biliary sepsis.Figure: [758] Aortogram with hugely dilated celiac and replaced hepatic arteries.Figure: [758] Abdominal CT with diffuse angiomatous tissue, early hepatic vein opacifcation, and biliary necrosis.