The present report concerns a 32-year-old woman with complex univentricular congenital heart disease (pulmonary and tricuspid atresia) treated with an extracardiac Fontan procedure who was being assessed for heart transplant (HTx) suitability; all relevant consent forms were signed. Vena cava and pulmonary conduit and artery pressures were 15mmHg while the transhepatic gradient was 4mmHg. Angiography revealed multiple venovenous and aortopulmonary collaterals that were not amenable to percutaneous occlusion due to their small size. The patient had moderate Fontan-associated liver disease (Child-Pugh B8) and posthepatic portal hypertension (small esophageal varices, splenomegaly, and thrombocytopenia). Liver magnetic resonance showed multiple hepatocellular adenomas; the largest was 60 × 80 × 78mm (figure 1A, arrow). Biopsy demonstrated METAVIR stage F3-4 fibrosis in the liver parenchyma and no malignancy of the main adenoma. After evaluation by the hepatology department, the patient was considered suitable for isolated HTx.

Figure 1

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The transplant was conducted via the bicaval technique but was complicated by significant surgical bleeding that required delayed sternal closure after 48hours. Subsequently, the patient was stable with a well-functioning graft.

Liver resonance 1 year after the transplant showed a significant reduction in adenomas; the largest measured 35mm in size (figure 1B). Three years after the transplant, the main adenoma measured 30mm and there was no significant portal hypertension while liver function was similar (Child-Pugh A6). The adenoma regression was probably at least partly due to the restoration of the biventricular circulation and normalization of the posthepatic pressure. Although the liver biopsy was not repeated, it seems that the Fontan-associated liver disease probably stabilized after the HTx and even showed signs of reversing.