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Background The Fontan operation is performed in individuals born with a single functional ventricle. The procedure separates venous return from the heart and allows normal arterial oxygen saturations. It extends the life span of these patients, who are now surviving into adulthood. However, the Fontan circulation results in chronic hepatic congestion and reduced portal blood flow. Fontan-associated liver disease (FALD) is an increasingly recognized complication, and can lead to cirrhosis, portal hypertension (PHT) and hepatocellular carcinoma. Screening for liver disease is a critical part of long-term follow-up, although assessment of fibrosis in this cohort is not clear-cut. Here we describe a model for outpatient hepatology review, and report baseline parameters of liver disease in new referrals. Methods All patients referred to hepatology at the Royal Infirmary of Edinburgh from Dec 2017 to Aug 2019 were included. Demographic data, blood tests and echocardiogram results were recorded in a database at the time of clinic review. Results 21 patients were included. Mean age was 29 years old, 62% were male. Average age at the time of Fontan completion was 9 years. Median Fontan duration was 20 years (range 8–34). 5/21 patients had evidence of moderate LV impairment. The most common liver enzyme abnormality was isolated raised GGT (87.1U/l, range 30–326). 20/21 had abdominal ultrasound, 9 had normal liver appearance. Transient elastography (Fibroscan) results were available in 18 patients. Median liver stiffness was 15.4 kPa (range 7.9–34.3). 11/21 patients had clinical features suggestive of possible PHT (varices/ascites/splenomegaly/thrombocytopenia). None of the patients had features of decompensated cirrhosis. Serum bilirubin was higher in patients with features of PHT (29.2μmol/l vs.16.9μmol/l p Conclusions Individuals with Fontan physiology are surviving longer with the current literature suggesting almost universal development of FALD. Evaluation of FALD is challenging with traditional markers of liver fibrosis being unreliable. We recommend assessment for FALD 10-years post-Fontan including clinical assessment, abdominal ultrasound, laboratory investigations, calculation of fibrosis scores and elastography. Following these individuals over time, and development of a UK registry, would help to improve our understanding of how best to assess fibrosis and predict severity of FALD in unbiased cohorts. |
