Hepatic Arteriovenous Malformations from Hereditary Hemorrhagic Telangiectasia: Treatment with Liver Transplantation

A 47-year-old handyman presented to the emergency department with worsening exertional dyspnea and persistent lassitude at rest for 2 weeks. He had experienced moderate unremitting epigastric pain over the preceding months requiring narcotics, as well as intermittent jaundice and low-grade fever. His epigastric pain frequently worsened after meals, resulting in anorexia and weight loss, but also was present at rest and radiated through to his back. He denied epistaxis, hemoptysis, and gingival or gastrointestinal bleeding. Although he admitted binge drinking of alcohol in the past, without obvious social or medical complications, he had been sober for several years. He continued to chew tobacco daily. His mother reportedly had alcoholic liver disease, and his two children were healthy. Physical examination revealed a slender man with a fever of 102 F but in no obvious distress. His conjunctivae were anicteric, and his oropharynx did not reveal any lesions or bleeding. His breath sounds were normal. Cardiac examination revealed tachycardia, a 3/6 systolic ejection murmur best heard over the apex, and jugular venous distention. His abdomen was soft and not distended, but tender over the epigastrium, where a loud bruit was also present. The liver and spleen were both enlarged. No jaundice, palmar erythema, or rashes were present. The patient was fully alert and oriented, without asterixis. Comprehensive laboratory studies were significant for a normocytic anemia with a hematocrit of 35%, mild thrombocytopenia with a platelet count of 142,000/mm, and abnormal liver tests with a total bilirubin of 3.7 mg/dl, aspartate aminotransferase (AST) 43 U/l, alanine aminotransferase (ALT) 55 U/l, and alkaline phosphatase of 579 U/l. His international normalized ratio for prothrombin time (INR) was normal at 1.1. B-type natriuretic peptide was elevated at 1,067 pg/ml. Blood cultures grew Streptococcus anginosus (a member of the Streptococcus milleri family). Echocardiogram revealed moderate left ventricular enlargement with normal left ventricular systolic function, and mild right ventricular enlargement with mildly decreased right ventricular systolic function. There was trace aortic regurgitation and mild mitral regurgitation, with increased aortic flow velocity with a mean gradient of 19 mmHg. There was also mild tricuspid regurgitation with an estimated right ventricular systolic pressure of 51 mmHg. No other valvular lesions or vegetations were found. Abdominal computed tomography (CT) revealed diffuse angiomatous tissue within the liver, early opacification of all hepatic veins, and low-attenuation tissue in segment 7 of the liver likely representing biliary necrosis (Fig. 1). Abdominal magnetic resonance imaging (MRI) confirmed the diffuse heterogeneous enhancement of the liver and presence of multiple arteriovenous malformations (Fig. 2). Angiography was performed, and the initial aortogram revealed a hugely dilated celiac artery and replaced left hepatic and common hepatic arteries (Fig. 3). The proper M. Lee (&) T. J. Daugherty Division of Gastroenterology and Hepatology, Department of Medicine, Stanford University Medical Center, 300 Pasteur Drive, Alway Building, M211, Stanford, CA 94305-5187, USA e-mail: maxlee@stanford.edu