Cirrhosis in Patients Following the Fontan Operation: Incidence and Long-Term Outcomes

Hepatic complications including cirrhosis are increasingly recognized after the Fontan operation. We examined the incidence and predictors of cirrhosis after Fontan operation. We reviewed records of all patients who had a Fontan operation from 1973-2012 at our institution (n = 1052). 449 patients had available post-operative liver data. Patients were classified as having cirrhosis only if they had a diagnostic liver biopsy or characteristic findings on magnetic resonance imaging (MRI) or magnetic resonance elastography (MRE). Patients with isolated blood liver function tests or ultrasound abnormalities were not considered to have proven cirrhosis. The overall 10, 20, and 30-year freedom from cirrhosis was 99%, 96%, and 76%, respectively. Among the 25 patients with cirrhosis, the mean age at Fontan operation was 11.4 ± 9.7 years. The average duration from the Fontan operation to diagnosis of cirrhosis was 19.5 ± 7.8 years. Of these 25 patients, 13 had an atriopulmonary connection, 6 had a lateral tunnel, 2 had an extracardiac conduit, and 4 had other Fontan connections. Only 2 patients had a fenestration. Atrial or ventricular arrhythmias were present in 20/25 patients (80%), diagnosed a mean of 9.7 ± 5.0 years prior to cirrhosis. MRE-derived mean liver stiffness data was available on 8/25 patients with an average of 6.0 ± 1.8 kPa (>5 kPa interpreted as stage 4 fibrosis or cirrhosis). Using multivariate analysis, a diagnosis of asplenia and preop digoxin use were the only factors associated with the development of cirrhosis. Survival following the diagnosis of cirrhosis was 71%, 25% and 17% at 1, 5 and 10 years respectively. The cause of death was known for 4 patients (3 multi-organ failure, 1 metastatic hepatocellular carcinoma). Patients with asplenia are at the highest risk of developing cirrhosis during follow-up, though some heterotaxy patients may have liver disease prior to Fontan. Long-term outcome after diagnosis of cirrhosis is poor, warranting noninvasive hepatic screening strategies in patients after Fontan. Ultimately our study may be underestimating the burden of liver disease in these patients. Therapy is difficult and novel multiorgan transplant strategies may benefit this patient population.