Introduction: Fontan procedure is the palliative treatment for univentricular heart diseases. Fontan-associated liver disease (FALD) develops almost universally. Here we present a case of combined heart-liver transplantation in a pediatric patient with FALD and hepatocellular carcinoma (HCC).

Case Report: A female patient with left ventricular hypoplastic syndrome and previous Fontan procedure was diagnosed with FALD at 9 years of age. Three years later (7 years post-Fontan), 6 liver nodules were diagnosed, two of which showed early washout. The lesions increased in size over months. Biopsy showed high-grade dysplasia of the dominant nodule, which, combined with the radiological findings, was considered compatible with an early HCC. The patient was listed for heart-liver transplantation at the age of 14, and chemoembolization was performed on the dominant nodule. Combined sequential heart-liver transplant was mainly uneventful, except for prolonged reperfusion times secondary to a complicated pulmonary artery suture. After surgery, normalization of liver enzymes was delayed by high-dose vasopressors and transient, mildly decreased right ventricular function. The patient rapidly recovered and was dismissed with normal liver function tests. Low-grade dysplasia was confirmed in all nodules at histology, with complete necrosis of the dominant lesion post-chemoembolization.

Conclusion: We report here the first successful pediatric heart-liver transplant in Canada. Combined transplantation for FALD-induced HCC during adolescence offers the advantage of a still satisfying heart function and absent or negligible liver failure. Nevertheless, diagnosis of HCC in FALD patients is complex, best timing for surgery is unclear, and organ allocation in these patients remains an open question.