663 Hepatic Adenoma Masquerading as a Hepatocellular Carcinoma in a Patient With a Fontan Procedure on the Oral Contraceptive Pill

Abstract

The oral contraceptive pill (OCP) has traditionally been contraindicated in the Fontan circulation due to its thromboembolic profile [[1]Gordon-Walker T.T. Bove K. Veldtman G. Fontan-associated liver disease: A review.Journal of cardiology. 2019; 74: 223-232Abstract Full Text Full Text PDF PubMed Scopus (87) Google Scholar]; though our centre uses it with caution with concomitant anticoagulation. Our case suggests that the OCP may be a potential confounder in Fontan patients with liver lesions and this possibility should be discussed with patients. A 21-year-old female was found to have an incidental liver nodule on routine hepatocellular carcinoma (HCC) screening on a background of an extra-cardiac conduit Fontan procedure for tricuspid atresia and pulmonary stenosis and Crohn’s disease. Medications included the OCP, Warfarin and Sulfasalazine. Liver ultrasound with acoustic radiation force impulse elastography suggested cirrhosis. Subsequent magnetic resonance imaging demonstrated a 25mm liver lesion consistent with a HCC. Percutaneous biopsy and microwave ablation was performed. Histology was initially described as HCC or a high-grade dysplastic nodule. Further opinion with immunohistochemistry however revealed an inflammatory subtype hepatic adenoma(HA). Cirrhosis and HCC are well described in patients with Fontan palliation postulated due to increased central venous pressure and reduced cardiac output [[2]Kuwabara M. Niwa K. Toyoda T. Shirai T. Tateno S. Ohuchi H. et al.Liver Cirrhosis and/or Hepatocellular Carcinoma Occurring Late After the Fontan Procedure- A Nationwide Survey in Japan.Circulation journal : official journal of the Japanese Circulation Society. 2018; 82: 1155-1160Crossref PubMed Scopus (21) Google Scholar]. Fontan patients often have benign liver lesions confirmed via imaging. Unlike HCC that is often associated with a poor prognosis [[3]Oh C. Youn J.K. Han J.W. Kim G.B. Kim H.Y. Jung S.E. Hepatocellular carcinoma after the Fontan procedure in a 16-year-old girl: A case report.Medicine (Baltimore). 2016; 95: e4823Crossref PubMed Scopus (30) Google Scholar], HAs are rare benign liver tumours [[4]Babaoglu K. Binnetoglu F.K. Aydogan A. Altun G. Gurbuz Y. Inan N. et al.Hepatic adenomatosis in a 7-year-old child treated earlier with a Fontan procedure.Pediatric cardiology. 2010; 31: 861-864Crossref PubMed Scopus (20) Google Scholar] that are rarely associated with cardiac hepatopathy [[5]Ponnatapura J. Kielar A. Burke L.M.B. Lockhart M.E. Abualruz A.R. Tappouni R. et al.Hepatic complications of oral contraceptive pills and estrogen on MRI: Controversies and update - Adenoma and beyond.Magnetic resonance imaging. 2019; 60: 110-121Crossref PubMed Scopus (6) Google Scholar]. However, HAs are strongly associated with the OCP, anabolic steroids and conditions that cause impaired glycogenolysis [[6]Abarbanell G. Tepper N.K. Farr S.L. Safety of contraceptive use among women with congenital heart disease: A systematic review.Congenital heart disease. 2019; 14: 331-340Crossref PubMed Scopus (11) Google Scholar]. We highlight need for care by expert centres and for specialised opinion in interpretation of investigations. We reinforce need for on-going HCC surveillance in Fontan patients and careful consideration of the OCP.