ABSTRACT. Examination of a 2-month-old male golden retriever presented to the hospital revealed malnutrition, ascites, cardiac murmur and hyperammonemia. Identification of subaortic stenosis and hepatic arteriovenous fistula was made through ultrasonography and angiocardiography. In addition, intrasurgical mesenteric portography showed an intrahepatic portosystemic shunt. The dog did n ot show portal hypertension and secondary multiple extrahepatic portosystemic shunts. Surgical correction was attempted after medical treatment. The hepatic artery branch which was connected to the hepatic arteriovenous fistula was separated, and completely ligated using silk ligature. However, the separation of the intrahepatic shunt blood vessel was unsuccessful and the dog died 15 hr postoperatively. KEY WORDS: canine, hepatic arteriovenous fistula, intrahepatic portosystemic shunt.J. Vet. Med. Sci. 66(3): 299-302, 2004 Hepatic arteriovenous fistula is a condition characterized by a communication between the portal or the hepatic vein and hepatic artery. All previous case reports of dogs regarding this condition showed vascular abnormality with communication between the portal and hepatic artery [1,3,6,7,9,13]. Dogs with hepatic arteriovenous fistula usually exhibit ascites and secondary multiple portosystemic shunt (PSS) due to portal hypertension [8,12]. This report presents an extremely rare case of a dog with hepatic arteriovenous fistula accompanied by congenital intrahepatic PSS and aortic stenosis.A two-month-old male golden retriever was presented to the Koide Animal Hospital, Okayama with a history of ascites which appeared suddenly two weeks before and malnutrition. The dog had been previously diagnosed with aortic stenosis accompanied by hypoalbuminemia and hyperammonemia in another hospital two weeks before and had been undergoing treatment by administration of furosemide (2 mg/kg, twice a day[bid]), spironolactone (1 mg/ kg, bid), alacepril (2 mg/kg, once a day[sid]) and feeding with a protein restricted diet (Hill's Prescription Diet l/d, Hill's pet Nutrition, Inc., Topeka, KS, U.S.A.). A male littermate of the dog had undergone surgical correction of intrahepatic PSS previously.Physical examination revealed that the dog was slightly emaciated, and the body weight was 5.55 kg, systolic ejection murmur over the aortic valve area and continuous murmur (bruit) with thrill through the abdominal wall over the area of the liver. However, there was no sign of ascites during examination.Laboratory examination revealed mild microcytic (mean cell volume = 59 fl), hypochromic (mean corpuscular hemoglobin concentration = 31.5 g/dl) anemia (packed cell volume = 25%), mild prolongation of clotting time by hepaplastin test (25.0 sec; normal, 13-18 sec) and activated partial thromboplastin time (26.9 sec; normal, 18-22 sec), serious panhypoproteinemia (2.5 g of protein/dl, 1.2 g of albumin/dl), high of serum alkaline phosphatase activity (1,513 U/l), slightly high fasting serum bile acid concentration (7.2 µmol/l) and blood am...