Development of Cirrhosis and Portal Hypertension in Fontan-Associated Liver Disease 2225

• Published in: The American journal of gastroenterology Vol. 113; no. Supplement; p. S1263
• Main Authors: Zhang, Hao Chi, Tung, Poyee, Machicao, Victor I.
• Format: Journal Article
• Language: English
• Published: New York Wolters Kluwer Health Medical Research, Lippincott Williams & Wilkins 01.10.2018
• Subjects: Gastroenterology; Hypertension; Liver cirrhosis; Liver diseases; Pulmonary arteries; Stents
• ISSN: 0002-9270; 1572-0241
• DOI: 10.14309/00000434-201810001-02224

Introduction: The Fontan procedure is a cardiac surgery performed in certain complex congenital heart disease (CHD) cases including tricuspid atresia associated with single-ventricle anatomy. However, Fontan-associated liver disease (FALD), including cirrhosis, may result. Case Description: A 20-year-old man with a history of tricuspid atresia and hypoplastic right heart syndrome managed surgically with Fontan procedure was evaluated for portal hypertension after discovery of variceal hemorrhage as the cause of recent melena. By the age of 3 years, he had undergone the Fontan procedure, which shunts systemic venous return towards the pulmonary arteries for oxygenation. A bidirectional Glenn procedure and extracardiac Fontan procedure were performed to link the inferior vena cava to the right pulmonary artery. Thirteen years later, a Fontan baffle stent was placed for correction of stenosis. The patient had no remarkable personal or family history of liver disease. On physical exam, vitals were normal. He did not exhibit stigmata of chronic liver disease, audible murmurs, jugular venous distention, or peripheral edema. Laboratory testing showed normal liver enzymes and bilirubin, with mild thrombocytopenia; results were negative for commonly-tested etiologies of chronic liver disease and viral hepatitides (Table 1). Abdominal ultrasound showed normal portal vein diameter without ascites. Liver elastography showed liver stiffness correlating with moderate-to-severe fibrosis (F3-F4). The patient was diagnosed with cirrhosis in the context of FALD. He underwent a right heart catheterization for restenosis of the Fontan baffle stent, requiring angioplasty, and underwent undergoing serial endoscopies for variceal eradication. Discussion: FALD is a well-described phenomenon. The pathophysiology is thought to be related to liver injury secondary to passive hepatic congestion, and a component of hypoxic hepatopathy secondary to low-cardiac output states. Complications arise from elevations in pulmonary vascular resistance, pulmonary thrombi, narrowing and scarring of the Fontan pathway or pulmonary arteries, and failure of systemic ventricle which lead to a phenomenon termed "failure of Fontan circulation." Advanced fibrosis or cirrhosis may develop within ten years of Fontan operation, introducing the risk of hepatocellular carcinoma. Our case highlights the importance of early diagnosis of FALD and portal hypertension, with ongoing care by a hepatologist.