Diffuse hepatocellular carcinoma secondary to cardiac cirrhosis in heterotaxy syndrome

Abstract

Heterotaxy syndrome is a rare congenital defect of left-right laterality of major visceral organs, often categorised by the presence of symmetric left or right atrial heart chambers with a single effective ventricle. Known as left or right atrial isomerism, these conditions may present with a distinct pattern of extracardiac anomalies. Heterotaxy is often palliated with the Fontan procedure and is suggested to be subject to similar long-term sequelae of congestive hepatopathy and ischaemia, increasing the risk for hepatocellular carcinoma. Few works document primary, localised hepatocellular carcinoma in patients with heterotaxy syndrome. We present a case of diffuse, multifocal metastatic hepatocellular carcinoma in a young patient with left atrial isomerism and dextrocardia. We also review suggested guidelines of surveillance for liver disease and hepatocellular carcinoma in this patient population.

Keywords: congenital disorders; hepatic cancer; palliative procedures.

Figure 1

Axial CT image of (A) dextrocardia with single effective ventricle and (B) multifocal, diffuse hepatocellular carcinoma (white arrows), with the largest mass measuring 12×8 cm (red arrow).