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• W2977648755 abstract "The Fontan procedure has been of particular benefit to patients with functional single-ventricle complexes. Fontan procedure has been shown to cause chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. Pulmonary arteriovenous (AV) malformations occur frequently in patients with advanced hepatic failure, as the hepatopulmonary syndrome. But systemic arteriovenous malforamtion has never been documented in cardiac cirrhosis. A 45 year old male, on intermittent hemodialysis for acute on chronic kidney disease was admitted with worsening fatigue and shortness of breath. He had a double inlet left ventricle for which he underwent Fontan procedure in 1980. He was on anticoagulation with warfarin for atrial fibrillation.On physical examination he was tachypneic and had central cyanosis. He had anasarca with grade 3 pedal edema. He had multiple spider angiomas in the both upper limbs and trunk. His right hand showed 2 soft tissue masses adjacent to the 3rd and 4th inter-phalangeal joint. His abdominal examination revealed marked abdominal wall edema and moderate ascites. An ultrasound of the digital masses was consistent with low resistance arteriovenous malformations. A CT scan of the abdomen showed cirrhotic changes in the liver with marked splenomegaly and venous collaterals in the upper abdomen, retroperitoneum and abdominal wall. Systemic AV malformations with advanced liver disease has been reported very rarely in alcoholic cirrhosis. Right-sided heart failure results in transmission of increased central venous pressure directly to the liver through the inferior vena cava and the hepatic veins. Fontan procedure has been shown to cause chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma.In retrospection the patient had the digital swelling for almost nine months prior to this presentation, but unfortunately it was overlooked. To the best of our knowledge this is the first case report in the literature, where a patient with Fontan procedure has presented with systemic AV malformation as the initial manifestation of cardiac cirrhosis. Fontan procedure or similar operations must be carefully followed for the development of chronic liver disease, which could ultimately result in HCC and a fatal outcome." @default.
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• W2977648755 date "2006-09-01" @default.
• W2977648755 modified "2023-09-26" @default.
• W2977648755 title "Cardiac Cirrhosis and Systemic Arteriovenous Malformation" @default.
• W2977648755 doi "https://doi.org/10.14309/00000434-200609001-00958" @default.
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