A Successful Case of Liver Transplantation in an Adult With Congenital Hepatic Arteriovenous Fistulae Associated Cardiac Dilatation and Heart Failure

Congenital hepatic arteriovenous fistulae (CHAVF) are direct communications between the hepatic artery and portal vein or hepatic vein. Clinical symptoms of CHAVF depend mainly on the location, duration, and blood flow volume of the fistulae, which are manifested by portal hypertension, hepatic fibrosis, cardiac enlargement, and eventually heart failure. Here we report a female patient aged 54 who was first admitted to our hospital due to recurrent chest tightness and palpitations in March 2014. Metoprolol tartrate and diltiazem hydrochloride were prescribed to control the symptom since nothing unusual was found in coronary angiography and abdominal ultrasound. Until April 2015, the patient's syndrome relapsed and abdominal computed tomography angiography and digital subtraction angiography revealed diffuse arteriovenous fistulae between the branches of hepatic artery and vein. Subsequently, 3 attempts at hepatic arterial embolization were performed; however, her abdominal pain aggravated and her heart discomfort could not be relieved eventually. Therefore, orthotopic liver transplantation as the salvage treatment was performed using a hepatic graft from a 19-year-old cardiac-death donor performed on January 1, 2017. Upon operation, the enlarged right hepatic artery whose diameter was approximately 1.5 cm in this recipient. And we also demonstrated a novel manner that the graft's celiac artery patch was anastomosed to the recipient's proper hepatic artery and gastroduodenal artery patch, which could reduce the blood flow successfully. The patient recovered uneventfully and was discharged home on the postoperatively 15th day. Since her liver transplantation, she has not complained of cardiac discomfort and abdominal pain, and her heart size has returned to normal on echocardiography. The hepatic artery peak velocity reduced to normal and the heart shadow also recovered. Nevertheless, for complex and diffuse intrahepatic vascular fistulae after failed hepatic artery embolization, liver transplantation should be strongly considered as the definitive treatment of choice.