Cardiac cirrhosis and hepatocellular carcinoma in a 13‐year‐old treated with doxorubicin microbead transarterial chemoembolization

Abstract

Increasing numbers of children are surviving into adulthood following surgery to correct or palliate congenital heart disease. This surgery can occasionally result in long-standing elevated right heart pressures and chronic hepatic venous congestion leading to cardiac cirrhosis. We report the first paediatric case of hepatocellular carcinoma in the setting of cardiac cirrhosis. A 13-year-old girl developed inoperable hepatocellular carcinoma and was treated with transarterial embolization with lipiodol and doxorubicin eluting microbeads. Promoting awareness of this association, even in younger patients, will hopefully result in better surveillance and screening of hepatic complications in survivors of complex cardiac surgery.

Liver disease secondary to congenital heart disease in children

“…However, the prognosis of children and adolescents with HCC after a Fontan operation is poor. Of the 4 patients in Table 2 who were <18 years old upon diagnosis of HCC [8,10,16,17], 3 had died in the 20 years since the operation [8,10,16]. These reports suggest that surveillance for liver disease at regular intervals is essential to improve the prognosis of children with HCC after a Fontan operation.…”

“…A PubMed interface search revealed that as of February 2019, at least 33 patients had been diagnosed with HCC after Fontan palliation (Table 2) [5][6][7][8][9][10][11][12][14][15][16][17][18][19][20][21]. Although this number is small, gender seems not to be associated with HCC.…”

“…In Table 2, the shortest interval of time between the Fontan operation and the diagnosis of HCC was only 2 years [8]. It is thus difficult to determine the appropriate time elapsed since a Fontan operation for the initiation of surveillance in a Fontan population.…”

“…However, since the first report of HCC following Fontan palliation surgery was published in 2005 [5], the reported incidence of HCC in patients who have undergone Fontan palliation has increased [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25]. Fontan palliative surgery was introduced in 1968 for patients with congenital heart disease; this surgery separates the systemic and pulmonary circulations, producing a single-ventricle physiology [26].…”

Hepatocellular Carcinoma in an Adult with Repaired Tetralogy of Fallot

“…3 Liver fibrosis in adults with congenital heart disease (CHD) is a growing concern, and a few cases of HCC have been reported. [4][5][6][7] Currently, only adults who have undergone a Fontan procedure are considered at risk for liver fibrosis. 8 The Fontan procedure is the last surgery in a series of palliative operations for children born with single ventricle heart defects.…”