Hepatology Management of Adults with Fontan Circulation: An Interdisciplinary Protocol

Document Type

Conference Proceeding

Publication Date

10-1-2024

Publication Title

Am J Gastroenterol

Abstract

Introduction: The Fontan procedure is a palliative treatment for complex congenital heart disease that reduces cardiac output and raises venous pressure, leading to hepatic and cardiac complications. This study describes the clinical course and current management of adult patients with Fontan circulation under an interdisciplinary protocol. Methods: A retrospective review was done on adult patients with Fontan circulation after data collection on fibrosis evaluation, laboratory/imaging surveillance, and clinical follow-up. Monthly meetings helped establish the protocol and review challenging cases. Data was analyzed for describing hepatic, oncologic, cardiac, and renal outcomes. Results: Forty-three patients were included, average age 30.4 ± 7.2 years. Lateral tunnel (60.5%) and extracardiac (30.2%) Fontan were predominant; 46.5% were fenestrated. Indications included arterial malposition (25.6%), tricuspid atresia (23.3%), and hypoplastic left heart (20.9%). Elevated liver enzymes and total bilirubin were noted in 23% and 13.9%, respectively. Transient elastography (TEG) was done on 31 patients showing median stiffness of 14.3 kPa, with 51.7% having F4 fibrosis and 37.9% F3. All patients underwent liver Ultrasound or Magnetic resonance imaging within the last 2 years, revealing cirrhosis in 34.9% and congestion in 23.3%. Despite lacking imaging evidence of fibrosis, 21 had F2 or higher fibrosis on TEG. Liver biopsies in 13 patients showed congestion (53.8%), no/mild fibrosis (23.1%), and cirrhosis (15.9%). 4 patients had congestion without advanced fibrosis, despite advanced TEG or imaging findings. Cirrhotic complications included varices (11.9%) and ascites (7%). There were no cases of hepatocellular carcinoma or hepatic encephalopathy, and 1 patient was referred for liver transplant evaluation. Median ejection fraction was 55%. Heart catheterizations were done in 79.1%, 35.3% of whom had high Fontan pressures. Over half (53.9%) required repeat catheterization and 16.3% got additional heart surgery. 1 patient was referred for heart transplant. None developed chronic kidney disease (CKD). Conclusion: Our results show that fibrosis evaluation via TEG screening and laboratory/imaging surveillance may be confounded by congestive hepatopathy, commonly necessitating liver biopsy. Frequent heart catheterizations are crucial for surveillance in those with advanced fibrosis or congestion. Risk of liver or heart transplant as well as CKD remains low. An interdisciplinary protocol is imperative to establish clearer guidelines on surveillance techniques/intervals to reduce morbidity. (Table Presented).

Volume

119

Issue

10

First Page

S1235

Last Page

S1236

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